History

Journal History

Thursday, January 6, 2011 9:35 PM PST

January 6, 2011

Grandma Cindy has decided to take this over with help from Mommy since Melanie truly has her hands full with 3 energetic and busy boys. We’re not sure how many dear friends still check in on Jacob since the updates have been so sporadic but plan to turn over a new leaf in the New Year and update quarterly or as things in Jacob’s life emerge.

Sending you all a belated Merry Christmas and a very healthy and happy 2011! I have asked Melanie to get a couple of current photos published as well.

Well since September Jacob has slowly recovered from his femur break. He was in the partial Spica cast for 6 weeks and once again it’s been a very slow process to get him walking again. These days he does pretty well but he is still struggling, however, they are back to keeping the doors locked or he’d be down the block in the blink of an eye. He can move quickly when motivated by escape! As far as therapies, so far it’s been primarily hydro therapy since the leg break which he really enjoys. Melanie takes him on Wednesdays and it does seem to be helping. We all appreciate Melissa, one of Jakie’s caregivers, who spends lots of time helping us all out. A big thank you to “Lissa” as Jake would say!

On another note, as you know Jacob has always been a bit of a mystery. Along that line he has been experiencing some sort of pain since Thanksgiving Day. He will cry for anywhere from 15 minutes to an hour. It is so sad to watch and especially difficult because he can’t tell us where it hurts. He has gone through about every test known to the medical community. We initially thought it could be his shunt, but after an MRI of his head that was pretty much ruled out. He has had labs, an abdominal ultrasound, an echocardiogram, a DEXA Scan, EKG, and he’s been seen by all of his specialists including the dentist. The only solid result is that he has low bone density and will be starting on IV Pamidronate at the end of February.

He is enjoying being back in school, loves his teacher, aides and the other students. Hopefully we can keep him in school for a while.

Matt is getting so tall, eats like his stomach is a bottomless pit, is excelling in school and sports and starting indoor soccer on Sunday. Noah loves his Pre-K program and even has his own posse. Very cute!

That’s it for now! Again Happy New Year to you all!

Friday, September 3, 2010 3:00 PM PDT

Well it has been way too long since I posted an update on Jacob! Time just seems to get away from me and while I have good intentions, they don’t always materialize. So where to begin.

It seems forever since Jake’s c-spine surgery in November, 2008. His c-spine healed well throughout 2009 and life was fairly normal. The next major event was in January of this year. Jacob had bilateral hip osteotomy at Seattle Children’s performed by his great orthopedic surgeon, Dr. Klane White. Jakie was in a full spica cast for 8 weeks. In addition to the affects of the surgery, his muscles atrophied and the poor little guy spent almost 7 months in a wheelchair. With lots of therapy (thanks to Grandpa Mark for taking him to most of his therapy sessions) his walking was almost back to normal. Then last Friday Jacob fell in our kitchen. My Mom and Dad met me and all 3 boys at the Seattle Children’s ER. After several hours we learned what I suspected all along, Jacob broke his femur right at the site of the instrumentation from his hip surgery. This is the first time the doctors had seen the break occur like this. But, of course, we’re talking Jacob here!

His surgery was last Saturday morning as the anesthesiologist on duty Friday night wasn’t comfortable with Jake’s airway issues and intubation history. Friday night Steve stayed with Jake while I went out to dinner with Klane and Amy White and then I spent the night with Jake in his room. Matt and Noah spent the night with my parents. Saturday morning my parents traded the boys off to Steve’s parents who took them camping for the week end. We had all been planning this camping trip and the boys would have been terribly disappointed if they hadn’t been able to go camping. A big thanks to Grandma Patty and Grandpa Steve for taking Matt and Noah camping and to Grandma Cindy and Grandpa Mark for all the other help!! Saturday night Steve stayed with Jake at the hospital and I spent the night in the White’s guest bedroom. We couldn’t ask for a better surgeon or for better friends. Thank you Klane and Amy for everything!! Jake was able to come home on Sunday in a partial spica cast with no weight bearing for another 6 weeks. Wednesday he started spiking a fever and by yesterday I took him to Children’s where they checked the incision site for infection. Fortunately the incision looks great and it may be he has a virus. Last night was a sleepless night with Jake having diarrhea every 30 minutes to an hour. I called my Mom this morning to ask for her help and she’s been here all day helping with the boys and running errands for me…and spurring me on to do an update. I’m still deciding if I may need to take Jacob back to Children’s if I can’t keep him hydrated. So far he’s taking Pedialyte through a syringe and my Mom and I both think his coloring is a bit better.

On a happier note, Matthew started 4th Grade on Wednesday at English Crossing Elementary and Noah will start Pre-K this coming Tuesday. Jacob will be house bound again for a while and I am working with his school & teacher to come up with a plan for either a partial return to school or some home-based school.

I’ll try to update more quickly next time or perhaps have my Mom do some updates in the future!

Love ~ Melanie, Steve, Jake, Matt, & Noah (& Grandma Cindy!)

Friday, March 20, 2009 9:52 PM PDT

New photos!

Jacob is doing great! His c-spine is healing up very nicely. I will do a more thorough update when I have more time. :-) Take care ~ Mel, Steve, Jake, Matt, & Noah

Monday, December 8, 2008 10:56 PM PST

Jacob is 2 weeks post c-spine decompression and fusion surgery today. He is doing amazingly well. He had x-rays and his stitches removed this afternoon. The x-rays went well and we think they look good although his orthopedic & neurosurgeon have not officially looked at them yet. Removing the stitches was not fun at all. Jake was not happy about it...I think the worst of it was having to hold him down as usual. He hates that but the other option was putting him under anesthesia and taking him to the OR to have them removed and after how he responded to the anesthesia a couple of weeks ago I really didn’t want to go through that again!

We are still keeping him home from school and I am really uncertain about the when/how/etc. of sending him back to school. He is getting around really well but he is in the neck brace and as far as we know he is still very fragile. I am going to be sending an e-mail off to his doctors tomorrow in the hopes that they have had a chance to look at the x-rays from today and perhaps they will have an idea on all of this for us. He is dealing great with the neck brace and as long as we keep the co-flex on it he does not mess with the velcro straps. In fact I think he actually feels more 'protected' with the neck brace on. We are very happy he is not in the halo but I think we are all still unsure about what all is OK and how strong that fusion actually is although we have been assured many times by Dr. Krengel (ortho.) that Jake’s fusion is very strong!

That is all for now. I will try to get some pictures up shortly and I will post again soon. Thank you all for your thoughts and prayers for Jacob and the rest of us through all of this...

Love ~ Melanie, Steve, Jake, Matt, & Noah

Monday, December 8, 2008 10:56 PM PST

Monday, November 24, 2008 11:05 PM PST

Friday 10:50am

We are going home! Jacob has been officially discharged. His medication from the pharmacy should be here in about an hour and we will be on our way. :-) Jake took a walk around the floor today and although his endurance is very low he is moving around very well. Steve and I are going to have our work cut out for us keeping his activity level down to the level they want it to be but we will manage. I will update again in the next couple days when we get settled back in at home. Thank you all for your wonderful words of kindness, thoughts, & prayers. They worked!

Thursday 7:45pm - Happy Thanksgiving!

What a difference a day makes! Jake has been moved out of the ICU and into a regular room. His breathing is back to normal and he is tolerating the c-spine brace fairly well. The stitches there and on his hip (where they took the piece of bone for his spinal fusion) look great. There is a good chance he will actually be discharged home tomorrow which would be wonderful. He needs a consult with the physical therapist and the orthotics people need to make sure the brace is fitted right and modify it so Jake can't get it off and if all that goes well then we are out of here. I do most of the work anyhow and both Jake and I would be happier at home! I will update more when I can...

Thursday 12:10am - sleeping is something we will do at a later date...

What a roller coaster! Jake thankfully made small improvements throughout the day which resulted in a very nice, comfortable evening. Breathing although still labored is under control and the incision site looks good...still bleeding but no open stitches. Talk of taking him back into the OR to put on the halo has been thrown around BUT it is sounding like this is less and less likely. Jake is pissed off but more his old self rather than the groggy, drugged out, raging bull of last night. We are still in the ICU as Jake is in need of that level of care. Speaking of.....Steve and I stepped out of the room and into the family lounge to have dinner this evening while Jake was sleeping very soundly. His nurse apparently 'looked away' and Jake FELL OUT OF BED! Yes, only us...in the ICU and the Jakester falls out of bed. Long story short...multiple doctors, very chastised and apologetic nurses, a head CT, several x-rays, and a 1:1 nurse sitting at the end of Jake's bed later and all is well thankfully. Does someone think we needed more excitement!?!?

******************************************************************************

****Morning Update****Wednesday 8:45am

Unfortunately, Jake is having some post-operative issues. We remain in the ICU after a very rough night last night. Jake is incredibly agitated, uncomfortable, scared, and in pain. His breathing is not great. He is still on a large volume of oxygen and after a middle of the night chest x-ray it appears that Jake's lungs are very wet. He is getting some Lasix as I type this to try to get some of the fluid out of his puffy little body. The other issues is with the incision site. With how agitated Jake became over the past 12 hours his cervical collar has rubbed the incision area and it is now bleeding quite a bit. We are waiting on neurosurgeon for a dressing change and a visual of the site to see if he has opened the stitches. Due to the breathing issues and bleeding he will remain in the ICU for awhile. Please keep him in your thoughts and prayers!

***Morning Update***Tuesday 7:50am

Jacob had a fairly restful night. He only woke up a couple of times and was not thrilled with the vent breathing for him but his nurse was able to quickly settle him down with some morphine & ativan. Pain is being managed well. The plan for the day is to extubate and if all is stable tranfer him out of the ICU and to the floor which would be great. I will update again when I can.

******************************************************************************

Just wanted to post a quick update on Jake's surgery today. Jacob's c-spine decompression & fusion surgery went very well. He is resting peacefully in the ICU on the ventilator. The surgery took about 6 1/2 hours today. We expected him to come out in a 'halo' but the fusion portion of the surgery went very well and the surgeons are incredibly confident with the strength of their fusion so Jake is in a 'miami collar' which is basically a neck brace. This will make the next 3 months much easier IF we can make him not mess with it. Everyone who knows Jake knows this may pose a problem but we are willing to attempt to work this out if it allows us to avoid the halo. If things get really crazy they will take him back in the OR and put a halo on. He is not thrilled with the vent so they are managing both his comfort & pain with morphine which seems to actually be working this time which is great. Not having the halo may mean he gets to come home sooner as well so stay tuned for another update and hopefully some pictures soon. Thank you all for keeping Jake in your thoughts and prayers!

Monday, November 17, 2008 10:27 AM PST

Just wanted to post a quick update and when I have more time I will post more. Jacob will be undergoing c-spine decompression and fusion surgery next Monday (Nov. 24TH) at Seattle Children's Hospital. He will be in the PICU initially and then a regular room...if all goes well he will be discharged the first week of December. Jake will be discharged in a halo which he will be in for 3 months...ugh... We will be spending Thanksgiving in the hospital this year. I will update more after the surgery. Please keep Jake in your thoughts and prayers!

Tuesday, September 16, 2008 10:56 PM PDT

Once again I have let Jake's website go! I start to get gentle reminders from friends and family and know it's time to get a new post up here!

We had a very fun and busy Summer. Our wonderful friends the Wigglesworth's came and spent some time with us at the end of June. We attended the International MPS Symposium in Vancouver, BC with them and had an absolutely amazing time. Jacob was literally 'adopted' by the UK MPS Society and they generously gave us the use of one of their amazing caregivers, Beth, who took care of Jake the entire time we were there. Jacob is now an honorary Brit and we are hoping that Beth will be at Disney World in December 2009 as well. Noah fell in love with his caregiver in the nursery, Michele, and I think she fell in love with him too. And, Matt was such a big boy with his group from the Camp Canada program...making friends and going on outings. Wonderful Beth and the UK MPS Society even took Matthew on the final day so he could spend time with Jake swimming in Stanley Park. Having the boys all taken care of freed Steve and I up to attend the conference and really gather some interesting and cutting edge information on MPS disorders. We also were able to socialize with many of our MPS friends from around the world. It was truly a global experience.

We went camping to Ocean Shores and eastern Washington. The boys attended Camp Prov which they always love. Matthew attended golf camp at Harbour Pointe (golf is a passion of his). Jacob turned 8 at the end of July and Matt turned 7 at the end of August. Jacob and I ended the Summer with a trip to Duke for his 6 year post-transplant check up (more on that in a minute). While Jake and I were in North Carolina Steve worked, Noah spent time with both sets of grandparents and Matthew went camping with his good buddy Daniel, his mom, and brother.

Jacob was such a big boy traveling alone with his mommy. I was incredibly relieved. He handled 9 hours each way on airplanes and in airports like a champ and I was shocked with how well he tolerated being poked and prodded at Duke. I was truly impressed. He got through his ECHO and EMG un-sedated and even tolerated 6 HOURS of developmental testing with very little resistance. I can honestly say that Jake is growing up. He may not be developmentally appropriate for an 8 year old but he is certainly doing the best he can. Most of Jake's test results came back good. We came home with one minor issue and one major issue. Minor issue is his esotropia is worse (this is his 'lazy' eye(s)). The eye doctor understands that we can't keep glasses on him so we are going to try some eye drops to see if they may help. The next step is Botox...it figures my child will get Botox before me! We have an appt. with a local eye surgeon in mid-October to monitor the drops and possible Botox injections.

The major issue is the doctors now believe we are putting Jake at risk for paralysis if we don't get his c-spine operated on soon. So..........I have spent the past couple of weeks trying to figure out where and who is going to do this procedure. I am hopeful that we have a team in Seattle that is able and willing to do it although the complex spine surgeon on staff at Children's is new and I have yet to meet him so I am working on getting an appt. set up with him. Thankfully, we have a connection in Seattle who is very knowledgeable about Hurler Syndrome and is willing to assist us in this difficult decision. We did not want to have to do this surgery as it is just a scary procedure but we are going to have to. My understanding is Jake will spend at least one night in the PICU, and a week in-patient at either Harborview or Children's and then come home in a halo (external bracing system screwed into his skull) for a minimum of 6 weeks but possibly longer. When I have more details on all of this I will be sure to post them here.

Jacob has been back to school now for 2 weeks and things are going well. He loves going to school and is very happy to be back in the routine. He is in a general ed 2nd grade classroom with a one-to-one aide to help support and transition him through his day. He is with the same kids from his 1st grade class as they and their teacher looped to 2nd grade. Mrs. Strittmatter is a kind, caring teacher for Jake which makes things easy. He also is able to go to his special ed teacher, Mrs. Stringer, for academics which helps him to not get frustrated. The really cool thing is that Matthew is in the 2nd grade classroom on the other side of the wall and has our awesome neighbor, Mrs. Yarnell, as his teacher. He and Jake are able to interact during the day because their teachers like to combine their classes when they can. I really couldn't ask for a better scenario for my boys! I will be meeting with Jake's teachers tomorrow to iron out some of the details but once we do that all should be well.

That is the latest Jacob news. I am going to try to get some new pictures up as well. Thank you for continuing to check in on our family.

Love ~ Melanie, Steve, Jacob, Matthew, & Noah

Friday, June 6, 2008 9:13 PM PDT

OK, I realize I have given Jake’s website out a few times lately and it doesn’t look very good that there isn’t a current journal entry!

Jake is doing great. He had surgery number 18 on Tuesday...crazy he’s actually had that many surgeries and he’s only 7 1/2, isn’t it!?! He had 2 of the 4 ortho-fix eight plates removed from his legs. His orthopedic surgeon removed the femoral staples but left the tibial staples. At this point, Jake’s hips to knees are straight although he needs a bit more time on the tibia (knees to ankles). He came through the surgery with flying colors...our only problem is keeping him down so that he can recover. He was literally up and running as soon as we got home from the hospital! In fact he had the surgery on Tuesday and was back to school on Thursday...wow!

Jake has seen several doctors in the Seattle area and we have consulted with both Duke and Minnesota doctors in regards to Jake’s c-spine. There is a slight difference of opinion but at this point we are going with the ‘watch things very closely’ recommendation. We will be seeing Dr. Goldberg at Seattle Children’s every 3 months with MRI of the problem area every 6 months. We are just not ready to put Jake through c-spine stabilization and fusion. It is likely the most major surgery Jake will have had to date and until we are 100ure he needs it we are going to wait. Of course, it’s always a balancing act when we do this because we don’t want to wait so long that too much damage has been done. Ugh! If only these things were a little more black and white...

School for Jake this year has been really great. His development is still pretty much in the age 2 – 3 year old range but he has learned so much this year and when he is willing he shows/tells us. Socially he is doing even better...sometimes we even see glimpses of a more age appropriate Jake. He literally has the best classmates in Mrs. Strittmatter’s First Grade Class at Lakewood Elementary School (little shout-out to his classmates!). This is a general ed classroom (as opposed to special ed) and although Jake may be different than his classmates they have been nothing but kind and accepting of him. This class is a “looping” class so we anticipate he will spend next year with many of these same kids for Second Grade which is just wonderful. We are gearing up for the big zoo field trip next week and of course the anxiously anticipated Field Day. I am dreading school being out for Jake in a week and a half because that routine is so important for him. I guess it will be up to me...’Mommy Routine’ for the Summer...

Thankfully, we have the International MPS Symposium at the end of June in Vancouver, BC which we are really looking forward to, not to mention our wonderful friends the Wigglesworth’s will actually get a chance to stay with us for a few days during this time. :-) The boys will attend the Camp Canada program while we are taking part in the conference. We also have a couple of camping trips planned for Summer and of course all THREE boys will be attending Camp Prov this year. In addition, Matt will be going to Golf Camp and then he starts PeeWee Football at the end of July. Matt is our little athlete. He just finished his 3RD baseball season. He has a really great swing and is both good at and enjoys both baseball and golf. This will be his first year playing football so we shall see how that goes. Mommy is a bit nervous but Matt is completely nonchalant about the whole thing. At the end of August we are supposed to head back to Duke for Jake’s 6-year post-transplant studies. This is still in the planning phase and if the price of gas keeps going up who knows if we will even be able to afford the airfare. It’s getting ridiculous!

The other big Summer news is that after Jake’s Make-a-Wish we were contacted by the Marysville Strawberry Festival Committee which is a very big local event. They asked Jake to be the President’s Marshall of the Grand Parade so on June 21ST all 5 of us will be riding in a vehicle in the parade. We really think Jake will think it’s cool and it is quite an honor to be asked to be in the parade and be a part of this event. We have been interviewed by all the local papers and the boys and just Jake have had their pictures taken for the program and other articles. Our Jake is quite famous around here!

I think it’s time to end this for now. We really appreciate all of you who still check-in on us. It’s really great to hear from all of you in Jake’s guestbook!

Love ~ Melanie, Steve, Jacob, Matthew, & Noah

Friday, January 18, 2008 10:44 PM PST

Wow, it's been SO long I don't even know where to begin! First and foremost, Jake is doing well and life is moving along fairly 'normally' for us.

Jake's every day health is really good. I don't want to jinx it but it seems that Jake has gotten fewer colds thus far this season and although he's had his usual runny nose it comes and goes rather than the constant we've seen in the past. Jake does have a 'new' medical issue unfortunately. Jake had an MRI at Duke at the end of August that showed an area of concern to Jake's c-spine (back of the neck where the spine meets the brain). He has some narrowing/tightening of the area and so we are in the process of establishing some local doctors to follow this issue. He saw a great orthopedic surgeon at Children's in Seattle, has another MRI scheduled for the beginning of February, and will see a neurosurgeon at the beginning of March. We are going to have to follow this issue closely...we don't know if surgical intervention will be necessary BUT if there is even the remotest sign of neurological damage then something will need to be done as soon as possible. His legs appear straighter and we'll have that rechecked with x-rays in the next few months and possibly have the staples removed from his knees as well.

Jake's doing great in school. He has a group of friends that he seems to really enjoy and they seem to really love Jake. I had the pleasure of going in to Jake's class at the beginning of the year and explaining to all the kids about Hurler Syndrome and Jake's issues specifically and since then the kids have been very involved in Jake's day-to-day activities. It's great! I spend one morning a week volunteering in his classroom and Matt's classroom so I feel like I get to really see and know what's going on with both Jake & Matt's educations.

Jake's development is about the same. Some days he seems more verbal than others but all and all things are pretty consistent. We wish that we would see more obvious improvements with Jake's development but his improvements are subtle. The good thing is we are not seeing any regression and he is either holding steady with his development and at times improving. His therapists at school and privataly are working hard with him and that's very helpful as well.

The latest BIG news is that Jake was granted a Wish from Make-A-Wish which we received this past Saturday. Because Jake doesn't say much we waited until we thought we really knew what he wanted and we KNEW water was going to be a big part of Jake's Wish because water whether in the bath, on the beach or even to drink is pretty much Jake's favorite thing. We came up with some pretty cool vacation ideas that involved pools, beaches, sun, sand, etc., of course they also involved airplanes which are not a favorite of Jake's. We kept going back to the hot tubs that I had heard Make-A-Wish sometimes grants and I showed them to Jake online and he kept pointing and saying "pool" so...Jake got a big hot tub delivered and installed last week. It is amazing with a waterfall feature, stereo & speakers, LED lights, and all kinds of other cool features. Then on Saturday his wonderful MAW volunteer Dana had a big party for him with all his family and friends. The fire department came and filled the hot tub, the kids all had McDonald's happy meals --- it was all about Jake and he absolutely loved every minute of it! Needless to say we've been in the hot tub every day! It's cold out so we just run from the back door to the nice warm hot tub...such fun! I'm going to post some pictures of all our party & hot tub fun. Hope you like them.

I think that's all for now. I'll try to update sooner next time. We're kind of 'boring' lately which is just fine with me! Take care!

Love ~ Mel, Steve, Jake, Matt, & Noah

Sunday, July 29, 2007 0:34 AM CDT

HAPPY 7TH BIRTHDAY, JACOB!!!

Mommy, Daddy, Matt-man, & Baby LOVE you!

Tuesday, May 22, 2007 9:22 AM PDT

May 22, 2007
HAPPY 5 YEAR POST-TRANSPLANT DAY JACOB!!!

Five years ago today Jacob received treatment for his Hurler Syndrome...it's hard to believe we're at the 5 year mark...this means my boy will be 7 in July...wow! Jake & Matt wore their Duke 'Kids Rule' t-shirts to school today and brought snacks to share with the class this afternoon to honor this special day.

Medically Jake is doing really well. January, February, & March were a tough 3 months as far as illness this year. It just seemed like one cold, infection, virus after another but not just for Jake...for Matt & Noah as well. I think that might just be part of having 3 kids who pass their colds amongst each other! Jacob did have surgery in March to put his knee staples back in. This time he had both tibial & femoral instrumentation put in on both sides. He got through the surgery amazingly well and was up and walking within a day! He was only out of school for 1 week and then half days the second week. Now we wait and see if they work. He appears to be growing in height still and that is the way these 'staples' do the job so we will hope that this growth continues.

Development continues to be an area of concern. We will be heading back to Duke in August for Jake's 5 year post-transplant studies and hope to get some answers to some of our questions/concerns at that time. He does not seem to be following the same developmental progress as many of the post-transplant Hurler kids. He is still very delayed and we just don't know why. He receives extensive amounts of therapy and has since his transplant and we make sure he gets everything possible out of his educational experience. We don't know if there's something more going on or if this is just the way Jake's Hurler is manifesting itself. He continues to be incredibly oral and this has turned into a huge problem with nail biting. In fact, in the past week has almost bitten off 2 of his nails and we are very concerned about this and are searching for something to do to make him stop. More on this when we return from Duke...

As you can see from the pictures we spent a wonderful week at Disneyland with Grandma Patty & Grandpa Steve which was such fun. Matthew is playing baseball again this year. His team, the Reds, have 2 games a week and they are so good. He is playing coach pitch and loves it! He wants to play soccer & football as well...I'm OK with soccer but not so sure about football...we shall see... We have several fun activities planned for this Summer as well. The boys will go to Camp Prov again, Jake will have 4 weeks of Summer school, Matt will have a week of theater camp, Matt & Noah will spend the week with both sets of grandparents when Steve & I take Jake to Duke, we'll spend the weekend at Camp Primetime with other MPS families from our area, and we have a few camping trips planned...phew...I'm tired just thinking about it all!!!

That's all for now...thanks for checking in on Jake!

Love ~ Mel, Steve, Jake, Matt, & Noah

Monday, May 7, 2007 10:56 AM PDT

We spent the past week at Disneyland with Grandma Patty & Grandpa Steve. We had a wonderful time! I posted some pictures from our trip and will post more of an update on Jakie soon.

Love ~ Mel, Steve, Jake, Matt, & Noah

Wednesday, January 31, 2007 1:28 PM PST

Wow, time flies...can't believe it's been so long since my last update!

Jacob is doing well medically besides the major limp. He has actually had much fewer colds/viruses this year which is wonderful. He typically is sick from about December to May but so far so good. Hopefully this is an indication that his immune system is working at its full potential! As far as the legs, he will have x-rays again on Feb. 20TH as things weren't healed enough when he saw his orthopedic surgeon at the end of December. We will work out a plan after Dr. Dales takes a look at the x-rays. We will not be doing any major surgery right before or during Summer as this really limits Jacob's activities and Steve and I do not want this...he has several programs he participates in during the Summer months that are critical for his growth and development. We'll just have to see what the x-rays show and go from there...

Jacob continues to adore school. We have had some kinks in the road along the way but I believe we continue to have a good plan in place for Jake...sometimes it just takes a little more effort on Mommy's part than it should! Sadly, one of the major changes is the teacher we were so thrilled to have has had to take a medical leave for the entire year. We are so very sorry to hear this and know that Jake's education has suffered because of her absence. We really hope she feels better and is able to return to LES next year! It appears that Jake will now have 2 teachers during the week and his one-on-one aides who will remain the same. I am hoping this will work out and hope these new teachers see as much potential in Jake as Colleen. He also continues with his school-based speech and OT. His OT sessions at school are going very well. He seems to have a very good connection with his school occupational therapist which is great. His speech sessions are going OK but we are adjusting them this week to try to better meet Jacob's needs. Jake's development continues to cause us some concern. He is just not able to express himself the way he would like to and his lack of speech although improving is still pretty significant. Steve and I had hoped that we would see Jake's development getting closer to that of his peers. We believe this is happening but it is a much slower process than we had anticipated and it is hard to imagine that Jake will catch up developmentally like most of the post-cord blood transplant Hurler kids who have gone through the process prior to Jake. We have not lost hope...we know Jake is taking it all in...we just can't wait to see it come out!

Matthew is doing great in kindergarten and loves being in the same class as his brother. This has truly worked out amazingly well. They both seem to be thrilled with the time they are able to spend together in the classroom. We know this won't always be the case so we are taking it while we can get it!

Jacob is doing well in his private therapy sessions. Again, speech continues to be a problem and we are trying to come up with a new therapy plan that might motivate him further. He is doing really well in physical therapy. It is shocking what he can do with a messed up leg. His latest achievements have been climbing the rope ladder to the ceiling (I can barely do this!) and hanging/swinging from a bar. The positive to being in that spica cast for 6 weeks was the amazing strength to his upper body. He has always struggled with low muscle tone so these are amazing accomplishments. We have recently added a private OT session as well. This did not go so well last time so we are hoping this gets better over time and it is just Jake's unfamiliarity with the therapist.

Noah is great. He is a year old in a month...I can't believe it has already been a year. He has been a wonderful addition to our family. He is a happy, healthy boy who gets along so well with both his brothers. They are a treat to watch and spend time with!

Thank you for checking in on our family. I continue to check in on all of our 'on-line' friends as well. Know that you all remain in our hearts...

Love ~ Mel, Steve, Jake, Matt, & Noah

Saturday, November 18, 2006 11:20 AM PST

The healing process for Jacob's broken femur has had its share of ups and downs and continues to...who knew a broken leg would cause such problems? He spent 6 weeks in the spica cast and then an additional 3 weeks in a brace that resembled a spica cast although we could take it on and off for bathing & toileting which was nice. A couple things appear to have gone wrong somewhere along the recovery process. Jacob's knee instrumentation loosened and started to back out so Jacob had surgery right before Halloween to remove the instrumentation for awhile. The good news with that is Jake's left leg looks great. He has grown 2 inches and this has allowed his leg to straighten out nicely. Unfortunately, his right leg appears to have healed at an angle and is now rotating in even worse than it was prior to having his knee instrumentation placed so all the straightening that came with the growth over the past year on the right side is ruined! He walks with a severe limp and keeps his right foot turned out when he walks. He is using a walker to get around. The current plan is to let things heal completely and take x-rays again at the end of December to see how his right leg looks then have an additional surgery to put his knee instrumentation back in and possibly a right femur osteotomy (surgically re-break his femur). This time they would set it and put pins in to ensure it stays straight then back in a spica cast for 6 to 9 weeks. Needless to say, we are less than thrilled about this. His physical therapist has been working with him but is very concerned about strengthening it at the angle it’'s at now so we are waiting to see if we should do some x-rays sooner and see where things are internally. We are at that wait and see point...

Jacob was finally able to return to school and did so last Monday. I am very happy with the teacher Jake has this year and am so happy that he is in school after missing the first 2 1/2 months! Jake is in a developmental kindergarten/general education combo. class which is wonderful. He had a great week and has thoroughly enjoyed being back amongst his peers. He is talking, singing, & participating in all his school activities with very little intervention from his education assistant. He really thrives in school and it broke my heart to have to keep him out for so long. Having his teachers and therapists to the house is just not the same...for Jake the interaction with his peers is critical. Jake & Matt also have the added benefit of being in class together 2 or 3 days a week. Matthew is one of the "typically developing peer models" in kindergarten which is great for both of them. Matthew was very excited to introduce his brother to all his friends. I'm sure this will be the only year they are in the same class but the teachers are doing a very good job of making this time special for them. We are realistic that Jake will probably be out of school once again when he has the second surgery on his leg but are hoping for some sort of miracle that he won't need the additional surgery because pulling him out of school again will be incredibly difficult for both he and Matt.

Matthew adores school and does a very good job. He is the youngest student in kindergarten as he had just turned 5 a couple of days before the cut-off for kindergarten but he is definitely a mature 5 in many ways and seems to be adapting well. He has made a lot of progress since the beginning of the year. He's doing well with his numbers and letters. Loves writing & is trying to read. He is also learning sign language as a second language which is wonderful. He is a physical boy (he & Jake are very alike in this way!) and loves PE...they have a new PE teacher and she is teaching them yoga which he thinks is great.

Noah is doing great too. He continues to be a happy, easy baby. He is so adaptable and just kind of goes with the flow. He must have figured out, being born into this zoo, he needed to be easy going! He crawls all over the place and pulls himself up on furniture. He's starting to cruise around furniture and wants to transition between things. He's a little piggy and loves to eat...he wants nothing to do with baby food and wants to eat what we eat!

Anyhow, now that Jake is back in school we are back to our busy routine of school, therapy, and doctor appointments. Now that we have Melissa (Jake's caregiver), we are able to give individualized time to Matt & Noah between all the Jake 'stuff' and this has worked out wonderfully. Melissa has been kind enough to watch Matt & Noah a few times as well so Steve and I have had a couple of nights out which is unheard of for us! In fact, we went out last night for Steve's 35TH birthday with a group of friends which was so fun. Happy Birthday, Steve/Daddy!

We have a busy month coming up with the holiday's right around the corner. Matt & Jake are very excited about Santa this year! I probably won't update until after unless something changes with Jakie's leg situation. I will try to get some new pictures up today or tomorrow as well. Hope all is well with everyone and thank you all for your continued support to Jacob & us.

Love ~ Mel, Steve, Jake, Matt, & Noah

Tuesday, September 5, 2006 10:20 PM PDT

Wow, I can't believe how long it's been since I updated! I wonder if anyone even checks in anymore...we'll see I guess! We have had a very busy, fun-filled summer with a very bad past week but we'll get to that...

Medically, Jake had been doing fair. He has had a few viruses over the past few months and one virus/infection combo. that warranted an ER visit. He recouped from all of that and had his PE tubes replaced at the beginning of August as they had fallen out. That surgery went really well and he was up and playing shortly thereafter. The colds/viruses/infections have been worse this summer and we are really hoping these new tubes stay in place for awhile as they really help keep the infections and congestion away.

The more major medical news happened on Aug. 23RD(Matthew's 5TH birthday!), Jacob climbed up on a chair in the kitchen, lost his balance, fell off and broke his right femur (thigh)! This was absolutely horrifying. I was by myself with my 3 boys and Matthew's friend, Isaac, who lives next door. I immediately called 911 and sent the boys next door to get Isaac's mom, Joanne. I called Steve who rushed home. The paramedics were unable to put Jake in traction which is what they typically do for a broken femur because of his knee & spine instrumentation. They decided to start an IV to get him comfortable for transport...it took an hour to get the IV started as Jake's veins kept rolling over and collapsing. They finally got some morphine in him and he and I were on our way to the ER in Everett. After some strong discussion there (and some insisting by Mommy & Daddy) a second ambulance was called to transport him to Children's in Seattle. My parents thankfully took Matthew & Noah so we were able to focus solely on Jake. Our wonderful friends Klane and Amy paved the way for our arrival at Children's (Klane & Amy have an adorable daughter with Hurler and Klane is our newest addition to the Children's pediatric orthopedic department --- how lucky are we! --- thanks Dr. Klane!). Jacob was admitted and after some discussion the decision was made to set & cast Jake the next day. Morphine & valium were used to keep him comfortable and he and Steve got settled into a room for the night (I am nursing Noah and needed to get to him to take care of that...). The next day Jacob was put into a spica cast which is pretty much a full body cast. His right leg is completely covered and the cast comes up and around his waist and up to his chest. He will be in this cast for 6 to 9 weeks. He is very unhappy with the situation although I highly doubt it will 'teach' him not to climb chairs! He won't be able to begin school as planned and some other changes will have to be made to our lives until the little guy is out of this big cast. Not fun but we're handling it...I have to say it is strange to be homebound again...reminds me of those long days when Jake was immunocompromised post-transplant...

Matthew had drama camp for one week at the beginning of summer which he loved. He is definitely our little actor. Jake & Matt then had a week of Camp Prov which they love and I love! Their time at Camp Prov is filled with fun, games, and therapy for Jake and I was able to spend some solo time with Noah. Matthew is a wonderful peer model to many of the kids and adores spending the week with his brother and the staff & volunteers that work with the boys are just amazing. We spent a week camping in Eastern Washington with Grandma Patty & Grandpa Steve. We played in the water, rode the SeaDoos, and took the boat out. Noah absolutely loves camping and adores going out in the boat. We went to Camp Prime Time and spent time with some wonderful MPS families from the Northwest. We all had a great weekend and a great time catching up with friends we only see once or twice a year. We have celebrated many birthdays including Jacob's 6TH birthday on July 29TH and Matthew's 5TH birthday that we’re actually still celebrating (making up for Mommy & Daddy leaving him on his b-day to take Jake to the ER!). Jake went to his summer school program a few times but wasn't able to attend much as that was one of the times he was ill prior to his PE tube surgery.

We have just recently added a new member to 'Team Jake' and we are all thrilled to have her. The State has agreed to give Jake some respite hours and one of the boys' counselors from Camp Prov is working as Jake's caregiver. She is a wonderful person and Jake seems to really enjoy the time he spends with her. This has given me more time to get things done around here, run errands, & go school clothes shopping! She is a sophomore at the University of Washington and will be heading back at the end of Sept. but hopefully we'll be able to keep her around a bit.

My baby Matthew will be starting kindergarten on September 12TH. I really can't believe it! I am so nervous for him...he'll be one of the youngest in his class but everyone keeps saying how ready he is so we shall see... He and Jake will be in the same class once Jake is allowed to return to school but I think it will be good for Matt to establish some friendships prior to Jake getting there. My newer baby, Noah, is wonderful and amazing! We are having so much fun with him! He is a completely easy going doll baby. He is pretty much fat and happy all the time. I truly couldn't ask for a better baby.

I will post some new pictures of all of them. Hope you enjoy!

Love ~ Mel, Steve, Jake, Matt, & Noah

Monday, May 22, 2006 2:48 PM PDT

HAPPY 4 YEAR’S POST-TRANSPLANT, JACOB!!!!!!

Four years ago today, Jacob received his life-saving unrelated umbilical cord blood transplant. Once again this day brings back many memories and very mixed emotions. Jacob is such a joy in our lives and we are thrilled that we were able to take him to Duke and get him the treatment he needed. Of course, we can't help but wish that he never needed this treatment in the first place. Jacob is doing very well at this 4 year mark. He is no longer on any medications. He is happy and healthy for the most part. He has multiple orthopedic issues that we are attempting to remedy with surgical procedures. He tends to get sick easier and longer than a typical child. He is developmentally delayed...more like a 2 to 3 year old as opposed to the 5 1/2 year old that he really is in age. We take things one day at a time and happily anticipate the milestones, both big & small, yet to come!

Our trip to Duke for Jake's 4 year post-transplant check-up went well. We had some flight delays/cancellations on our departure but the boys were all troopers and we muddled through. Jake's appointments once we arrived went OK. I have most of the results back although we are still waiting on a few things.

Jake's eyes look good with very minimal corneal clouding. He has strabismus (a lazy eye) which could be helped by glasses but we are going to wait a little longer to fight the glasses battle. We tried it at one point and it did not go well...he just takes them off and chews on them...Dr. Greene (his eye doctor at Duke) thinks we can wait until Jake understands that the glasses will help him before we try again.

Development testing was fine. Jacob really hates all the people asking him to do things and tends to rebel against it. He was doing his typical crying, hitting, & dropping on the floor. He did do a few things that were asked of him but for the most part he ignored everyone and did his own thing. It was great getting Dr. Escolar's input on things, especially on some behaviors we've been dealing with. Dr. Escolar actually seemed happy with some of Jake's behaviors as they are very age appropriate for him at this point in time. We do not have the full report yet but should have something in the next week or so.

Jake's appointment with Dr. Fitch went well. We were able to get the x-rays the day before which really cut down on our waiting for Dr. Fitch. We are waiting on him to look at Jake's MRI results for the official word on things but we are hoping for the best.

Jake's labs look great. His enzyme level is normal. Lung capacity is great. Heart looks good. Donor cells are still at greater than 98 His immune system looks good although his titers for diphtheria & tetanus are low even though he has been fully immunized for them so he will need another DPaT booster. His bone density might not be very good although he didn't tolerate the scan for this very well. We have started him on calcium & vitamin D supplements just to be on the safe side.

Lastly, his audiology exam & ENT appointment went OK. Everything checked out fine except his left ear tube is working its way out so we will be scheduling an appointment here to have his tubes replaced. This always presents a challenge as the local docs don't believe that without ear tubes Jake will get a continuous ear infection that won't respond to antibiotics. We prefer & believe it is better for Jake to be proactive on this issue as opposed to reactive. We'll see if I can get this accomplished...

We ended the week with the MRI and a great appointment/visit with Dr. K. The MRI was pretty tough on Jake & Steve! I stayed with Matt & Noah and Steve took Jake for a peripheral blood draw & 2 hour MRI. Poor Jake hated the blood draw of course and needed extra medicine to keep him asleep for his long MRI so he was VERY nauseous afterwards. We took him back to the hotel room for a bath which helped a bit then off to see Dr. K. She thinks Jake is doing really well and is turning into quite the big boy. He was pretty whacked out from his sleepy meds from his MRI still so he wasn't all that friendly but she did get another chance to see him on Saturday which was good.

We then got to have some fun and enjoy the beautiful North Carolina weather. We spent a few days with our friends, the Wigglesworth's. We have such a good time together and can't wait for them to come visit us! I don't want to wait another 12 months to see them! We went to the Duke PBMT Rainbow of Heroes Walk together on Saturday. We went to this 4 years ago right before Jake's transplant; this Walk has turned into a huge event and was such fun for all of us. This is also a very emotional event...it is a fundraiser for the family support program but also a time to remember those who've lost their fight as well as honor those who have been through transplant. Sadly, we released balloons for many of the children who have made such a profound impact on our lives.

We made it home safe and sound. I, of course, caught a nasty bug from the airplane and was really sick for about a week. I think Jake then caught it from me but we are all feeling better now.

Thank you all so much for your continued support to our family over the past 4 years. It has been very uplifting to be able to come to this website and read your wonderful guestbook entries and know you're out there keeping track of Jacob.

Love ~ Mel, Steve, Jake, Matt, & Noah

Friday, April 28, 2006 8:58 AM PDT

Thought an update was in order prior to our departure to North Carolina. We fly out on Sunday for Jacob's 4 year post-transplant studies at Duke. Of course, we have had a bit of excitement around here in the past week which may impact some things while we're at Duke. Jacob broke his arm last Sunday. He fell off the boys toy John Deere in the backyard (which is a very common occurrence) on the grass and he must have hit just right (or wrong!) and broke his proximal humerus bone. The humerus runs from the elbow to the shoulder and Jake's fracture is basically a shoulder fracture. They do not cast this type of fracture but he is in an immobilization sling for the next 4 weeks or so. Thankfully it heals pretty quickly and well so this is just a little bump in the road for our tough guy Jake. We didn't even know it was broken...he only cried for about 15 minutes and then pretty much resumed his activity while being very protective of his arm. I took him in for x-rays just to make sure everything was OK...thank goodness!

Anyhow, like I said, we fly out Sunday and arrive in Durham late Sunday night/early Monday. Jake's schedule is:

Monday -
2pm - Dr. Greene (eye doctor)
?shoulder x-ray

Tuesday -
8:30am - Dr. Escolar & team (development testing)
2pm - Dr. Fitch (orthopedic surgeon)
After Fitch - Clinic to see a nurse practitioner

Wednesday -
Noon - chest x-ray, height & weight
1pm - PFT (pulmonary function test)
2pm – Echo
2:45pm - audiology
3:30pm - Dr. Hulka (ENT)

Thursday -
8am - labs
9am - spine, brain, & hip MRI (sedated)

Friday -
Appt. with Dr. K in the afternoon (not sure what time yet)

We won't be flying home until Sunday. We decided to spend an extra couple of days so we could attend the Rainbow of Heroes Walk at Duke. This is a fundraiser for the pediatric stem cell transplant family support program. We also will be spending some time with our wonderful friends, the Wigglesworths's. I think we are going to impose on them once again and stay with them at the end of the week. We have such fun when we're all together!

I will be sure to update when we get home with results.

Take Care ~ Mel, Steve, Jake, Matt, & Noah

Thursday, March 23, 2006 1:07 PM PST

All is going well here. As you can see from the pictures, Noah was born on February 27TH at 2:34am. He weighed 7lbs. 4 oz. and was 19 inches long. He looks so much like Matthew when he was a baby it's amazing although I think he has Jake's nose and eye color. We have been busy trying to get back into our routine and have been fighting colds for the past month which is not fun. Matthew and Noah probably got the worst of it and we were a little worried Noah would have to be admitted back to the hospital but we made it through and are all on the mend. It's super busy around here with three boys but we are loving it!

Jacob is doing well medically and managed to only catch a mild form of Matt's flu/cold/bronchitis. He is loving school and anxiously awaits the bus every morning. Between the colds and all we have missed some of Jake's therapy appts. but will get back into them tomorrow. Jake is learning how to be gentle with the baby but it definitely takes some reminding! We are supposed to be heading to Duke the first week of May for Jake's 4 year post-transplant studies & the Rainbow of Heroes Walk. I'm a little nervous to travel with Noah but I think we're going to give it a try!

Congratulations to our good friends Karin, Bryan, & Ava on the birth of their son, Ryder Matthew (born 5 days after Noah). Jacob, Matthew, & Noah also welcome their newest cousin Braxton Gray. Congratulations to Aunt Steph, Uncle Ben, Nathaniel, & Alec! In other baby news, Cousin Christian is doing really well...gaining weight like a champ...he does not appear to be a preemie anymore! Four new baby boys in three months...fun!

Love ~ Melanie, Steve, Jacob, Matthew, & Noah

Saturday, February 25, 2006 11:13 AM PST

Today is National MPS Awareness Day... Please take the time to think about those in your life who are affected by this group of rare diseases! Jacob and the rest of us will be wearing purple to honor his COURAGE and the COURAGE of our MPS friends! More information can be found at www.mpssociety.org and a new website www.jointhesearch.org.

Jacob is doing well. I am doing fine although very ready for Baby Noah to come out and play. He certainly is taking his own sweet time. I am in the beginning stages of labor and have been for over 2 weeks! Any day now...

Love ~ Mel, Steve, Jake, Matt, & 'almost' Noah

Monday, January 16, 2006 2:02 PM PST

I have been gently reminded to update Jake's website & it has been awhile so here goes... :-)

Medically, Jake is doing OK. He saw his local ortho. surgeon last week. According to the x-rays his knees and hips look good. It actually looks as if he has grown a small amount and the knee instrumentation is holding steady which should allow his legs to straighten. We have been questioning his hips for awhile but they still look about the same...not good but the same if not slightly better when looking at the trend over the past couple of years. His hips just don't fit in the sockets correctly and they never have. The one area of concern is once again Jake's spine. I'll have to address this more later as our local ortho. wants Jake's Duke ortho. to look at them and see what he thinks. The area of concern is beneath his current fusion and instrumentation and we are just hoping it is a non-issue. I should know shortly as the x-rays were over-nighted to Dr. Fitch (Duke ortho.) on Friday. Jake is now fully re-immunized which went well except for the very last vaccine --- the varicella (chicken pox) vaccine appears to have given Jake the Chicken Pox. He came home from school Thursday with two bumps on him which I wasn't so sure about but when he woke up Friday with more we decided he officially has the Chicken Pox. Matt has been fully immunized so we're hoping he doesn't get them but we'll see. Thankfully, Steve and I have both had them so we should be safe as well. Of course, now we are pretty much confined to the house until things start to scab over...it's a pretty mild case so we're hoping this doesn't take too long.

School and therapies are going exceptionally well for Jake. He is in a really wonderful learning pattern right now and is responding well to everyone and everything involved with his education. The school has made a real effort where Jake's learning is concerned and they have really been willing to work with me and others in getting things set up well for Jake. He actually seems to enjoy going to school. He loves the bus ride and if/when I pick him up from school he gives his teachers and therapists hugs good-bye. It's so nice to see him actually enjoying adults for once...he was so wary of adults for so long! His private therapy at Prov. is wonderful as well. Again, he is enjoying his therapists and doesn't seem to care if I'm around or not. He is verbalizing things more although his receptive language still is much stronger than his expressive language. The other big development news is he is FINALLY starting to respond to potty training. It has been a long time coming and I have been hesitant to push him but he appears to be on the verge. He is very aware when he goes to the bathroom and he loves to use the potty. He hasn't gotten it all coordinated yet but this is a huge step for him. He actually went potty at school on Thursday which his one-on-one aide, Martha was thrilled about. I am hoping this keeps up because it is great to see this advance in his development.

The boys (and us) had a wonderful Christmas. They got spoiled as usual! We have been re-doing there rooms and moving things around so we could have the Nursery back for when Noah arrives. This entailed new beds for both Matt and Jake. They are now both in full-size (double) beds and they absolutely love them. Matthew was moved to the big room (former guest room) and he has a space for his special toys. We spent some time at Ikea and got them new furniture including chairs that look like eggs and hang from the ceiling. They're very cool and the boys love them! We thought it was important they both have their special places before the baby arrives. We know this is going to be a big transition for them so we're doing the best we can to make their environment comfortable. We would still like to do some painting but I'm not sure we'll get to it before Noah makes his appearance...we'll see...

Speaking of baby, I am doing really well with my pregnancy. Thankfully, this has been a pretty easy one for me...just anxiously awaiting his arrival. I am at 33/34 weeks and as Jake and Matt both came at 38 weeks I figure I can start expecting something to happen in about a month or so. I feel very calm and at peace with it...I'm hoping that translates into a nice calm labor & delivery...

My sister and her husband finally got to bring Christian home from the NICU last night right after her Baby Shower. He is up to 6 lbs. and doing really well. He loves his Mommy and I know he was as anxious to get home with them as they were to bring him home! He continues to be adorable and health-wise is doing really well. As far as anyone can tell he doesn't appear to have any issues from being 6 weeks pre-mature...only time will tell. My sister is still struggling to get her health back to normal but the doctors say this is going to take more time than most after being on in-patient bed rest for over 2 months and then having a very difficult labor & delivery.

I think that's everything for now. Check out the pictures as I am updating them as well! Thank you all for your continuing support to Jake and the rest of us!

Love ~ Mel, Steve, Jake, Matt, & (in my tummy) Noah

Tuesday, December 27, 2005 10:00 AM PST

Merry Christmas and Happy New Year to all!

Jake is doing well. I will post an update on him with pictures within the week.

Welcome to the world Christian Shay VanDenburgh! He was born this morning around 6:10am by c-section. He weighs 5lbs. 4oz. and is 18 1/2in. He came about 6 weeks early but seems to be doing OK. He is in the NICU and will remain there for a bit. He was having trouble breathing on his own but seems to be doing better now. My sister was a trooper and tried her hardest to push him out on her own but 3 hours later things just weren't happening the way they should. She is in a considerable amount of pain and exhausted. As soon as she is able she will be taken to the NICU to be with her son. Please keep them (Marlene, Jason, & Baby Christian) in your thoughts!

Love ~ Melanie, Steve, Jake, & Matt

Monday, November 7, 2005 2:17 PM PST

Not a whole lot to report but I did post new pictures to the photo album. Be sure to check them out...there's a Halloween one of the boys.

Medically, Jake has been doing well. He's pretty much recovered from his knee stapling surgery. We have all been sick with a nasty cold for a little over a week and of course Jake got it worse than the rest of us so he's on antibiotics. He now seems to be clearing up quickly which is good. He missed a week of school so he wasn't thrilled to go today. It's so hard for him to get back into the routine!

His therapies have been going well both at school and at Providence. He is so routine oriented if I can keep things on schedule (barring colds & surgeries!) he does really well with everything.

I would like to ask that you all send a good thought in my sisters direction. I know I mentioned I was pregnant in my last post, my sister is pregnant as well. She is at 27 weeks and went into pre-term labor a little over a week ago. She has been in the hospital ever since and will remain there until the baby comes. We're hoping and praying she can keep the little guy (Christian Shay VanDenburgh) in there until at least 32 weeks! It's her first (and probably last!) baby and I know she is scared as well as being incredibly upset to be on bedrest & in the hospital for so long.

Take care and thank you for continuing to check in on us! :-)

Love ~ Melanie, Steve, Jacob, Matthew, & Baby Noah Charles (we named him!)

Wednesday, October 12, 2005 2:00 PM PDT

Jacob is doing a little better every day. I have to say though that this recovery has been much more difficult than we initially anticipated! He is finally standing and attempting to walk although the walking is VERY difficult for him. He has been out of school all week and won't be returning until next Monday. I am really hoping by then he will be able to go back as it's so hard for him to switch routines. He has physical therapy on Friday and I'm hoping that will help Jake with some of the stiffness he seems to have in his knees since the surgery. The bruising is pretty severe as well poor guy!

In other news, Jacob & Matthew will be welcoming a baby brother in late February/early March. I am pregnant and we have gone through extensive pre-natal testing. We can safely say that this baby is unaffected by Hurler Syndrome. He appears to have a normal enzyme level. Of course, they can't say the pre-natal testing is one hundred percent accurate but we feel very confident with the results. The baby will again be tested once he's born. I just wanted to share our wonderful news with those of you who check in on Jake. Matthew is just thrilled and Jake says "baby" at my tummy. So cute!

That's all for now. Take care!

Love ~ Mel, Steve, Jake, Matt, & Baby Boy

Tuesday, October 4, 2005 9:37 AM PDT

October 7, 2005
Surgery Update

Jake's surgery yesterday went well. The anesthesia worked out just fine and the surgeyr itself only took about 45 minutes. Unfortunately, Jake is not feeling well at all today. He is in a considerable amount of pain. We are giving him pain meds regularly and he is groggy but still seems to be hurting. He has slept alot today and has not walked or stood since his surgery. We are really hoping he starts to feel better soon. It is so hard to see our baby in such pain!
******************************************************************************

All has been going fairly well with Jake. He seems to be doing pretty well with school. Trying to get back into the swing of things is never easy for him but Steve and I have been thrilled with how well he seems to be slipping into the routine of it. For the most part he is excited to get on the bus in the morning and of course, thrilled to get off in the afternoon. He has his one-on-one aide for 4 hours (10am-2pm) of his 6 hour (9am-3om) day and I think this is working out well for him. She is able to help him with lunch and assist in much of the educational components of his day. Jake continues to get OT and Speech at school which he is getting every day at this point which is great. I think he probably dislikes Speech the most (sorry Theresa!). It is so cute though --- when his ST drops him off at his classroom he tries to shut the door on her while saying "bye bye beech, bye bye beech" --- she thought he was talking about the beach or possible calling her a naughty name --- we figured out it's how he says "Speech!" :-)

Medically, He got a runny nose the second week of school which we expect will last until March but that's just the nature of Jake's immune system and this time of year! Thursday he will be having his knee 'stapling' surgery. We're not looking forward to another surgery but it really sounds like this one will be fairly simple. We'll just be keeping our fingers crossed and hoping that this surgery doesn't cause him any problems. It will be our first surgery at Swedish so we are hoping that goes OK as well.

Matthew absolutely adores school. He wants to go every day and doesn't think 3 days is enough! It is so wonderful to see him spreading his wings and becoming such a big boy. We couldn't be more proud of him!

I think that's it for now... Please keep Jake in your thoughts on Thursday for his surgery. We hope all is well with all of you!

Love ~ Mel, Steve, Jake, & Matt

Sunday, September 18, 2005 3:07 PM CDT

I have very sad news to share... Our very good friends the DeVon's lost their son, Derek, yesterday evening to leukemia. I know I have mentioned him before on Jake's website and I just ask those of you who check in on Jake to offer your kind words and prayers to the DeVon's. Derek's website is www.caringbridge.org/wa/derekd (direct link below)... Derek battled leukemia for 6 years and was such a fighter through it all. He will be greatly missed by all those who love him and his family. Denise, Dante, Dylan, and Angel Derek --- we love you and are thinking about you...

Friday, September 2, 2005 8:37 PM PDT

I figure it must be time for an update... Not sure if anyone still checks in on Jake but there must be someone out there! We have had a very busy Summer between traveling a bit and the boys various activities. The boys had a great time at Camp Prov this year. They didn't want it to end which is wonderful! Jake made it through his right carpal tunnel release and port removal with flying colors and only had to miss one day of swimming at Camp due to his cast. We then headed off for a week of camping with Steve's family. The boys loved it! We rode the SeaDoos, went for boat rides, & swam every day.

Jake's 5TH Birthday was July 29TH. We were able to celebrate while we were camping and Jake was able to do all the things he loves (going 'fast' on the SeaDoos and swimming). On our way home we stopped for dinner at a Mexican food restaurant which is Jake's favorite food. My parents also had a BBQ for him a couple of days after his birthday which he loved. All-in-all I think he had a wonderful 5TH birthday. This birthday was a very big milestone for us...when Jake was diagnosed we were told he had a very severe form of Hurler Syndrome and we would be lucky if he lived to the age of 5. Well, that is no longer a concern to us! Jake is doing very well and reached the age of 5 with flying colors.

Sadly, we lost my Dad's Mom, my Grandma Charlotte, to a rare blood disease similar to cancer. This has been very sad for us as Grandma Charlotte always seemed much younger than her age. She was my "saucy" grandma. I was able to spend the last couple of days of her life with her and was there with the rest of our family when she passed away. I'm glad my boys got to know their Great-Grandma Charlotte as she had so much love and family history to give them. The boys still have 3 great-grandmas and 1 great-grandpa that they are able to know which is wonderful.

We spent a very fun weekend at Camp Prime Time with other Washington and Oregon MPS families. This is such a great weekend for us where we can truly be ourselves with people who understand everything we go through on a day to day basis living with this horrible disease. The group that goes to Prime Time is such a wonderful group of caring people. I honestly think it is one of the times of year when Steve and I can really let go and relax. The boys absolutely love it there playing in the dirt (or for Jake...eating the dirt!), swimming, and hiking. The last day of the weekend the boys even went horseback riding which was so great. Last year Matt was too scared and Jake didn't want to wear a helmet. This year Matt wanted to do it and the minute Jake saw Matt up on the horse, he grabbed a helmet and went with the people to the horse. I am seriously considering hippo-therapy for Jake now that I know he enjoys horses!

Matthew celebrated his 4TH birthday on August 23RD. He had a very fun day spending it with my Mom, Grammie Cindy (I had to take Jake to 2 doctors appts.). I baked a cake for him that night and he picked his favorite meal for dinner...salmon and corn on the cob. He's such a little man! We also had the whole family over for a BBQ this past Sunday and we went to the Fair which is another one of Matthew's favorite things. We are making Matthew's bedroom into a big boy bedroom (full-size bed, Jimmy Neutron theme and all) and he got a new bicycle. Fun stuff for a 4 year old!

Medically, Jake continues to do really well. We are switching to a new local orthopedic surgeon for Jake. We weren't entirely comfortable with the surgeon at Children's and have switched Jake to Dr. Dales at Swedish Pediatric Specialties. I feel this is a better match for us and Jake will be having his knee surgery at Swedish at the beginning of October. I actually might be moving some of Jake's other specialist care if needed to Swedish as well.

Developmentally, Jake is hanging in there. He does a lot for us at home...not as willing to practice these things outside of his comfort zone. We are looking into having an autism specialist work with Jake to help him with his social communication. Jake's school has one and I have heard that there are some around that I am looking into as well. This is one of the recommendations given to us by the development team at UNC and I figure we're willing to try almost anything to get Jake where he needs to be developmentally. Things just don't always happen quite as quickly as I sometimes like them to...keeping up with Jake's developmental needs can be a full-time job!

I think the last thing is that I will have a chunk of free time here in the next week. Not sure what I'm going to do with my time! Matthew will be going to pre-K 3 days per week from 9am-11:30am. I will be taking him to Bethlehem Lutheran in Marysville Monday-Wednesday. If all goes well with this for Matt he will begin Kindergarten the following year. His little buddy Isaac, our next door neighbor will be attending the same pre-K so hopefully his Mommy and I will be able to do some carpooling. Jacob will be attending Developmental Kindergarten in the morning and pre-school in the afternoons at his school, Lakewood Elementary. He'll be going Monday-Thursday from 9am to 3pm! I've just found out that he will have the same teacher all day which I think will be good. Ms. Honey Niemann will be teaching Developmental Kindergarten in the morning then some of her students will go to the mainstream Kindergarten in the afternoons and a couple of the younger ones like Jake will stay with her for her afternoon pre-school class. Jake will also have a one-on-one aide assigned to him...hopefully he'll get Ms. Sherri again. He's going to need additional help with lunchtime, recess, and just hanging in there for a full day. I'm a bit nervous about this but know that the teachers at Jake's school really have his best interest at heart.

I think that's about all this time. I am updating the pictures finally so enjoy those as well! Our thoughts and prayers are with those dealing with the aftermath of Hurricane Katrina especially our MPS friends in the affected areas...

Love ~ Melanie, Steve, Jake, & Matt

Monday, July 11, 2005 10:20 AM PDT

Just wanted to give a quick update. All has been well around here since we've been back from North Carolina. Jake is getting some wonderful Physical Therapy and Speech Therapy from Cris & Eileen at Prov and home Occupational Therapy from Sarah. I am so thrilled we're able to hang onto this through the Summer. We are working really hard on getting him caught up. We also just got back from a week in Whistler,BC with my parents, Grammie Cindy & Grampie Mark. We had a wonderful time. Jake wanted to walk everywhere and really avoided using his stroller which is great for his strength and we've learned that Matthew is quite a climber. He had Steve climb a pretty steep mountain at the top of Whistler! I will try to get the pictures downloaded in the next week or so...

Please keep Jake in your thoughts tomorrow. He will have his Right Carpal Tunnel Release Surgery and his Port Removed in the afternoon. We hate putting Jake under anesthesia because of his airway issues but we have to get him all fixed up! He'll be in a cast for 7 days and we're hoping everything goes as smoothly as his Left Carpal Tunnel Release Surgery went last November.

Thursday of this week Matthew will be attending his first SibShop. This is a program for siblings of special needs kids. We're hoping Matthew enjoys it...he will probably be one of the yougest kids there but the activities they have planned sound like things Matt will love. Then next week both boys will be going to Camp Prov which ia a wonderful day camp at Forest Park for special needs kids and their typically developing siblings. The boys went last year and loved it so I'm sure they'll love it this year as well.

That's it for now. Hope you are all having an amazing Summer. Take care!

Love ~ Mel, Steve, Jake, & Matt

Wednesday, June 8, 2005 8:46 AM PDT

***PLEASE MAKE A NOTE OF OUR NEW E-MAIL ADDRESS BELOW***

***CHECK OUT THE NEW PHOTOS - UPDATED 6/13/05***

3 - year Post-Transplant Studies Update

We have been home for a few days and have been busy getting things back in order. It's somewhat difficult when you only plan to be gone for 5 days and end up being gone 10!!! Thank goodness for our wonderful neighbors who took care of things around our house, especially the Yarnell's for taking care of Matt's fish, Oreo our kitty, and our mail and Joe for mowing the front lawn! We really have the best neighbors! :-) On to Jake's results in the order of his appointments...this might be a bit lengthy!

Jake's development testing Tuesday morning went OK. His behavior this time was MUCH better than last time (no hitting biting, spitting, etc.). That said, the development testing is always long and tedious for all of us but Jake was a trooper. We don't have any results from them yet and probably won't for a few weeks. They are in agreement with Jake doing a split schedule with AM kindergarten and PM pre-school next year so that's the direction we're heading. I'll be meeting with Jake's teachers next Tuesday to discuss and work out a plan.

Next Jake had an appointment with Dr. Fitch his orthopedic surgeon. Jake is out of his body brace --- yippee! His spinal fusion looks good although it's not healed up all the way yet it is good enough to not need the bracing anymore. It should be all healed up at the 6 months mark at the end of July. We still need to be cautious with him and utilize the same safety precautions we have since January just without the brace. He looks SO tall and skinny now! He has officially gained 3 inches in height since January 26TH! His hips look OK but his knees are not so good and Dr. Fitch is now in agreement with our local ortho. surgeon that they need to be fixed. Dr. Fitch feels that Jake's ortho. in Seattle should be able to handle this one so hopefully we won't have to travel for it. The surgery involves 'stapling' one side of Jake's knees and leaving the staples in place for 9 to 12 months to allow the other side of his knees/legs time to grow and straighten. We hope this works...if it doesn't we'll have to go with a more aggressive procedure that involves cutting bone and realigning things surgically. All in all Dr. Fitch seemed very happy with Jake's spine and doesn't seem to think it will be that big of deal to go ahead with the knee surgery.

Off to Clinic so Jake could be seen by a Nurse Practitioner. We were so happy to see one of our favorite nurses, Andre waiting to see us. Andre is such a wonderful guy and a great nurse --- soon to be Nurse Practitioner. Steve went golfing with him once during Jake's transplant and we make an effort to see him every time we're in Durham. As we're talking to him we see our wonderful Nurse Practitioner, Jen, is there that night and she's going to take Jake on for the week! We are truly SO lucky to have such wonderful people who love our Jakie! Jen did an exam on him and got all the information from me to give to Dr. K so that we were all prepared for our meeting with her on Friday morning.

The next day Jake's dental appointment went well. He wasn't thrilled but they were able to get a good look in there as well as clean them and apply fluoride. Jake's teeth look great with no chemo damage visible. His teeth are very strong with good spacing although the bottom set is a bit close together so we're supposed to try to floss them (good luck!). He STILL has one tooth coming in --- the poor guy has been teething for 4 years!!! It is popping through and they would expect it to be in within the next year --- no wonder he's still so oral!

Dr. Hulka, Jake's ENT was next and this is where our plans took a drastic turn. First Jake saw his audiologist and I expressed our concerns with his hearing although his ABR had been perfect in October we couldn't figure out why he appeared not to be hearing as good as he should also the development team at UNC was questioning his hearing as well. Dr. Hulka and Gwen (the audiologist) stood by their previous findings but wanted to repeat the ABR so that everyone concerned knew they were accurate. When Dr. Hulka looked in Jake's ears he found that they were both compacted with wax and "debris" with a non-functioning tube on one side and no tube on the other. He couldn't even tell if there was an ear infection present because they were so full of stuff pressing right against his ear drums. Dr. Hulka wanted to get him in ASAP for PE tubes, "debris" clean out, and an ABR, unfortunately & due to the holiday, the next available was Tuesday so we quickly made the decision to extend our time in Durham. As you know from my previous journal entry, all went well with the surgery and our flight home went very well with a happy Jake the entire flight!

Jake's ECHO went well. He was SO good he didn't even need to be sedated! The ECHO results aren't perfect this time which is a little strange as Jake's heart has always looked good. The ECHO showed some fluid and mild valve issues. This can happen from chemo/transplant or from Hurler's but it typically would have shown up long before now. It can also show up after a person has had a virus so we're thinking Jake just recently had a virus. It would fit with all the other symptoms he's been having, i.e. runny nose, plugged ears, etc. We are going to do another ECHO here in Seattle in 6 months to see what's going on...

We decided to head over to Jake's Nerve Conduction Study next without any sedation (they had ordered some Ativan for him in Clinic) because he did so well with the ECHO. He didn't love the NCS but he handled it OK. The neurologist doing it was a Mommy so she just kind of ignored his outbursts like I would! Results show the left carpal tunnel is better although not perfect and the right carpal tunnel has gotten significantly worse. So I am currently in the process of scheduling him right carpal tunnel release surgery at Children's with Dr. Hanel. I should know when the surgery will be by Tuesday.

The next day Jake had his labs, chest x-ray, and pulmonary function test. All looks good. He had his CT without sedation (I think our boy is growing up :-)!). His CT results as far as his brain look good. His sinuses were completely filled which would again go along with a recent virus and non-functioning ear tubes --- one more reason to get those PE tubes back in there and allow things to start draining again ASAP.

Our last appointment of the day was with Dr. Greene, Jake's eye doctor. Jake hates this appointment so Matt and I sat in the waiting room and visited with Claudina & Caterina Bonetti (another Hurler Mommy and adorable post-transplant little Cati) and made Steve put him through the torture. Jake's corneal clouding is still very mild and his vision results were great so no more glasses. Dr. Greene thinks his far-sightedness has repaired itself which can sometimes happen in toddlers. This is not to say that he won't need glasses again someday but at this point in time we don't have to struggle with them. One less thing...

The next day we were able to sit down with Dr. K and go over everything. She was very impressed with Jake and can't believe how great he looks (last she saw he was laying in bed very unhappy after spine surgery!) --- she thinks he looks even better and older than he did when she saw him at Disney World in December. She thinks all his results look fine just wants us to repeat the ECHO in 6 months, repeat spine x-ray in 4 months, get the right carpal tunnel release, and the knee stapling. If all goes well with all of this we won't have to head back to Durham until May of 2006 --- 12 MONTHS!!! That will be the longest we've gone between Duke visits since we began this process in March and we couldn't be happier although we will severely miss our Duke medical team as we always do!

We had such a wonderful Memorial Day weekend and subsequent week with the Wigglesworth's. We couldn't ask for better friends! I also have to mention how wonderful Matthew was the entire trip. He is SUCH a trooper sitting through all Jake's appointments and being Jake's best friend. I really think Matt's the reason Jake did so well this time. No one ever believes me when I tell them Matt is only 3 years old! We truly couldn't ask for better kids! Even though we had to add some extra time onto Jake's 3 - year studies it ended up working out for the best.

Thank you all for checking in on Jakie! Take care...

Love ~ Mel, Steve, Jake, & Matt

Wednesday, June 1, 2005 9:23 AM EDT

Surgery Update:

After waiting in pre-op for 5 HOURS!!! yesterday, Jake was finally taken in for his procedure. Apparently, there were several major traumas yesterday and the OR was short-staffed so Jake kept getting bumped...Dr. Hulka was not going to let them cancel him so we waited... Needless to say, Jake was not a happy guy by the end of the night...BUT...he has a new set of PE tubes and a very acute ear infection was discovered behind his right eardrum so we are very fortunate that we were able to get this done prior to flying with him again! He is on antibiotic ear drops to make sure the ear infection is all gone. Also, Jake's ABR once again showed his hearing is within the normal range...yippeeee!!! We can safely say now that Jake's hearing is normal and there truly is no more need for hearing aids. We definitely need to keep on top of the PE tubes and make sure that things stay clean in there. I will update more when we return home. Thank you all for checking in on Jake!

****Surgery Time: check-in at 4pm; surgery at 6pm****

Hope you all are having a wonderful Memorial Day weekend. We are! Even though we've had to stay in North Carolina, Kym, Craig, and Maddy have made sure we are having a good time. They have opened up there home to us once again and made us feel more than welcome. We've gone swimming, the boys went on a hike on an old tobacco trail, Steve was able to join in on one of Craig's poker nights, we've gone to the movies, and today they've invited us to join in on the neighborhood holiday party. At this point we don't even feel like we're here for anything medical! Of course that will change tomorrow...

Jake has to be NPO (no food or Pediasure) at 6am. We won't know exactly what time his surgery is until tonight but we are expecting it to be around 2pm as Dr. Hulka is doing surgeries in the AM at the Ambulatory Care Center which is for low risk patients...because of Jake's history his surgery has to be done at the hospital so Dr. Hulka will be heading back to the hospital to take care of Jake when he's done with his morning. We expect everything to go well although it will probably be a couple of hours due to the ABR (brain stem hearing test). The tubes will only take about 10 minutes to put in so this is the easy part. Also, he won't need anything stronger than Tylenol after the procedure so this will cut down on the problems Jake has experienced with narcotics after surgeries. Anyhow, we feel like we are doing the best thing possible for Jake and that's why we are remaining here for this procedure. Jake will have some very consistent hearing exams as well and we will know for sure how well he is hearing (we believe the results from October which said his hearing was good but these results have been questioned by a few people so we need to double check this information).

Thank you so much to the Yarnell's for taking care of our house, our fish, and our kitty! We really appreciate it. We fly home early Thursday moring and I will update on all Jake's 3-year post-transplant results when we return home. Everything checked out OK although this tube surgery is not the only surgery Jake will need in the next couple of months...but more on that later...

Love ~ Mel, Steve, Jake, & Matt

Friday, May 13, 2005 11:03 PM EDT

****QUICK UPDATE****
Wednesday, May 25, 2005

Slight change of plans... We will not be returning home until Thursday, June 2ND. Jacob has to have surgery on Tuesday afternoon, May 31ST. We saw Dr. Hulka (ENT) today. Jake's been having some ear infections and possible trouble with his hearing. Dr. Hulka said his ears are filled with "debris" and neither of his tubes are functioning. The debris (aka wax) is very close to his ear drums and has to be removed under anesthesia. Dr. Hulka will put tubes back in at this time and this will hopefully keep things good for the next 3 years or so like the last ones did. His audiologist will also be performing an ABR (brain stem hearing exam) while Jake's under to double check the good hearing results from last October. We've decided to remain here for several reasons. Dr. Hulka has done all Jake's ENT surgeries and has assisted with his small airways during anesthesia. He has a very specific way he does things with our Hurler kids. Also, Jake would experience a significant amount of pain during the take off and landings of our flights if this is not taken care of (which we experienced on our flight here!). We are choosing the safe way to take care of Jakie! So, we're off to the Wigglesworth's, our wonderful friends, on Friday. We will be spending the Memorial Day weekend with them and it sounds like they have some fun-filled events planned! I'll try to update again when we get to Kym and Craig's... Mel

Well, it's been awhile since my last update and I figured it's about time for those of you who still check in on our little man.

First and foremost, Jacob's spine seems to be healing up well. He is still in his body brace but has become increasingly more sturdy & steady. He is VERY used to his brace and the things that were difficult in the beginning for him (and us) are no longer difficult. He continues to get physical therapy once a week which I think is helping him in many ways. His PT, Ilene, is pretty firm with him which he really needs in this situation. Jake likes to run the show and Ilene has to work really hard to get him to stay on task and focused.

Pre-school is going OK for him as well. Jake has his good and his bad days. Everyone who knows Jake knows he has a bit of a control issue (wouldn't you if the first several years of your life were both painful and out of control!!!)...anyhow...this unfortunately is very challenging in school and therapy situations. His behavior is 100etter since the beginning of the school year. There is no longer any hitting, biting, kicking, etc. incidents. He seems to really enjoy and respond to his one-on-one aid, Sherrie, which we love! He's more age appropriate now with his refusal to do what he is told. I guess it's nice that he's being more age-appropriate though, right!?! Anyhow, we are currently trying to decide what the best approach is for him next year as far as DD Pre-School again, DD Kindergarten, or a combination of the two. I'm going to address this issue when we're back at Duke and UNC and hopefully will get some insight into what has worked for other post-transplant Hurler children. It doesn't help that Jake's birthday falls during the Summer so it becomes a bit more difficult to make a decision as we have a few more options than those with birthdays that fall during the school year.

Speaking of Duke, we will be flying out at 6am on Monday, May 23RD. I think we will stay at a hotel at SeaTac Sunday evening as we have to be to the airport between 4:30am & 5pm. EARLY! We arrive in Durham around 5pm Monday evening and will be staying at The Millenium while we're there. Jake's schedule is as follows:

Tuesday, 5/24
8:30am to 1pm – Dr. Escolar @ UNC for Development testing
2pm to 5pm – Dr. Fitch, ortho. surgeon (hopefully Jake will come out of his brace!)
Clinic at Duke to see a Nurse Practitioner before 8pm

Wednesday, 5/25
8am to 9:30am – Dr. Neal, pediatric dentist
10am to 11am – meeting with Dr. K
11:15am to 12:45pm – Dr. Hulka, ENT
1pm to 2pm – ECHO, sedated so must be NPO at Noon
2:30pm to 4:30pm – EMG/Nerve Conduction Study

Thursday, 5/26
NPO at 6am
10am to 11am – labwork
11am to Noon – PFT
12:30pm to 1:30pm – CT, sedated
1:30pm to 2:30pm – Dr. Greene, eye doctor
3pm – chest x-ray

Fun, huh!?! We fly home on Friday the 27TH and should arrive in Seattle around 7pm. We are SO looking forward to seeing Kym, Craig, & Maddy while we're back there. We really miss them! We also hope to see some of Jake's nurses and of course Stacy. The one really great part of going back to North Carolina is being able to see some of the friends we've made along the way.

The other really big thing is my sisters wedding which is Saturday, May 21ST. Matt is getting so incredibly excited to be the ring bearer. Jake could really care less but they both look totally adorable in their tuxedos! Matthew keeps asking me when he gets to wear his tuxedo like his Uncle Jason (my sisters' future hubby). Jason is a sweet guy and super good with our boys. He has a son and a daughter of his own and I think Jake & Matt can tell that he's a good Daddy. I will be sure to post pictures of them in their tuxedos as well as a Duke update when we get home.

Thank you for checking on our Jake and for signing the guest book. We like to know your still out there! :-)

Love ~ Melanie, Steve, Jake, & Matt

Monday, March 28, 2005 10:53 AM PST

Hope you all had a very Happy Easter yesterday. We certainly did. :-)

It's been awhile since my last update and everything has been going surprisingly well. Jake continues to amaze us with his agility considering he is about 4 pounds top heavy! Medically he has been great. His incision on his back is finally healed although the scar is pretty nasty looking still. The scar itself doesn't seem to itch as much although he is pretty itchy in general under his brace. He is so used to his brace now that he actually helps me take it off and put it back on during diaper changes and bath time!

As far as school, Jake is getting back into the swing of things. He's not perfect but for the most part he does really well. He is warming up to (more like getting used to) his one-on-one aid, Sherri. I really like her and although she is in the position as a substitute, we're hoping she continues to the end of the year as Jake's aid. I believe she is willing and I don't think the school wants to try to train someone else for the position. I feel good about it because Sherri (as well as Jake's teacher, Miss Kristine & the classroom aid, Ms. Vicki) are very familiar with Jake's needs at this point. I feel comfortable that he is being kept safe which was the most important thing for us in returning Jake to school post spine surgery. That said, he's off for the next two weeks due to parent/teacher conferences and Spring Break. These breaks are both good and bad in my opinion...it's nice to get a break but it's SO hard for Jake to get back into the routine of school. Hopefully, with it being only two weeks it won't be too bad on him. I have his parent/teacher conference tomorrow afternoon and I'm sure all will be well.

It looks like we will be heading to Duke the week of May 23RD for Jake's 3 year post-transplant check-up and if all goes well his spine will be all fused and he can come out of the brace. I don't have a confirmed schedule yet but once I do I will post it. Once Jake is healed from his spine surgery we will have a small surgery here in Seattle to have his port removed. This will be nice as we won't have to worry about flushes every 30 days as well as the risk of infection. He has received his second round of immunizations and has a few more to go before he will be all the way immunized. He doesn't enjoy the shots but gets over it pretty quick. Once all this is done...Jake will only have to see the doctor on an every 6 months basis...WOW!!! What will we do with all our time!?! :-)

The last thing on Jake is his therapies. He is now seeing a PT (physical therapist) and a Speech Therapist at Providence Children's Center outside of what the school provides. This is going amazingly well and is supplementing Jake's needs nicely. He continues to have a very small number of items he eats and still gets all his nutrients from PediaSure. We will address this again at Duke in May and decide if it's time to become more aggressive about his eating. He has started to drink out of a straw which was a big step for Jake as he has low muscle tone in his mouth area. He loves his little Juicy Juice boxes now.

I want to congratulate my sister, Marlene (Auntie MoMo) on her engagement to Jason. They will be getting married on May 21ST. I will be the Maid of Honor (or is that Matron of Honor because I’m married?...whatever!) and the boys will be the ring bearers. Matthew is so excited to wear a tuxedo! If he was a girl, he’d be such a little diva!!!

Finally, I want to thank all of you so much who recognized and did something on February 25TH for National MPS Awareness Day...ESPECIALLY...my Dad's (Grampie Mark's) co-workers at The City of Everett Public Works Department who all wore purple in honor of Jacob and MPS. Jake, Matt, and I went in to visit Grampie that day and I have to say that the affection and generosity that these people show for my family brought me to tears!

Take care all!

Love ~ Mel, Steve, Jake, & Matt

Monday, February 21, 2005 12:19 PM PST

Jake is doing SO WELL considering he had posterior spinal fusion only one month ago. His orthopedic surgeon and his team were so right, they said the first 2 to 3 weeks would be the worst and you wouldn't think things could possibly get any better but they do! Jake definitely has his limitations but they are minimal and we can live with them for the next 4 to 6 months. He is mobile, eating, and pretty much back to his routine. We've even gone shopping and out to eat a couple of times!

I had a meeting with Jake's school on Friday about his return on the 28TH. Steve and I had several concerns about Jake returning as there are some precautions and some pretty devastating results if these precautions are not followed. Our main area of concern is for Jake's safety and I was very happy to find all the professionals who work with Jake present at the meeting as well as those who are able to make decisions (Jake's Teacher, Teachers Assistant, Speech Therapist, OT, Speech Therapy Assistant, OT Assistant, school Principal, District Nurse, & Director of Special Education were all present) concerning Jake's care. I had the opportunity to really explain the situation to everyone, especially the areas I believe caution must be taken. The outcome of this meeting was very positive. Jake will return to school with a one-on-one aid. This individual will be assigned to Jake and only Jake. Jake will have this aid for the remainder of this school year as precautions will need to be enforced until he is medically stable (4-6 months). I will attend with Jake his first week back in order to train this individual. There's a chance that Miss Vicki, Jake's current classroom aid, will become his one-on-one aid which would be great as he already knows her and as most of you know Jake does not do well with transitions! If not, he will begin with a substitute until someone can be hired for this position. Thank you to Lakewood School District for seeing this need and for acting on it...also...thank you to Jake's teacher, Miss Kristine, for voicing your concerns and for your love of Jake. :-)

Please remember that February 25TH is National MPS Awareness Day. Find out more at www.mpssociety.org. We will be taking the opportunity to honor Jake and his MPS friends that day by wearing our purple ribbons and our purple Courage bracelets.

We hope you enjoy the new pictures. I hope you don't mind the one I included of Jake right after his surgery. He just looks so peaceful (a.k.a drugged :-)!) to me!

Love ~ Melanie, Steve, Jake, & Matt

***Please continue to keep our friends the DeVon's in your thoughts and prayers. Find out more at www.caringbridge.org/wa/derekd.***

Friday, February 11, 2005 11:33 PM PST

***Update 2/17/05***
I just wanted to let you all know because you have been so wonderful to Jacob that I just started a website for our friends and their son Derek. I have mentioned him a few times here before and I just ask that you offer Derek your thoughts and prayers during this very difficult time for he and his family. His website is: www.caringbridge.org/wa/derekd

Thank you! Mel :-)
********************************************************

Well, as I said, we arrived home...safe and sound. Matthew was absolutely wonderful on the flight --- he loves to fly --- Jake on the other hand was very uncomfortable to say the least! The first leg of the flight (about 2 1/2 hours) Jake was pretty good. He just fussed at the end like he usually does...the second leg of the flight (3 1/2 hours) was a different story. Jake screamed and cried for about 2 hours of the flight --- the other 1 1/2 he slept very fitfully. I gave him his pain medicine every 3 hours but it just didn't seem to take the edge off as it usually does. It truly broke my heart to see him so uncomfortable!

Now that we are home, Jake is a changed little guy. He is happy and comfortable. He is pretty much back to doing what he was doing before with some limitations simply due to the bulk of his brace. He is still in a small amount of pain and probably will continue to have some pain for another week or so from both the incision site and the instrumentation in his spine. He has had nothing but sponge baths for the past couple of weeks and is looking forward to a 'real' bath when Steve gets home from work tonight (it will take both of us as Jake will have to lay on his back and remain as still as possible).

Before we left Duke, Jake had a physical therapy appointment and a post-op with Dr. Fitch. The PT appointment went really well. I got all the paperwork and diagrams on how to lift and move Jake around so as not to disturb his fusion and I got recommendations on gross motor activities to help strengthen his spine and the muscles that he will be using more now that he is top heavy. They also gave me instructions on exercises I need to do in order to keep myself strong enough to lift him!!! Looks like I'll be running stairs and doing lunges --- I don't think I've ran stairs since high-school P.E.!!! His appointment with Dr. Fitch was long but good. The x-rays of Jake's spine while braced look great. The incision site is heeling up well and they were happy to see him in a much better mood than he had been in while in-patient. We will be going back to Duke in May for Jake's 3-year post-transplant studies and another post-op with Dr. Fitch. At that time, they will do x-rays without the brace and if things look good he might be able to get out of his brace a month early...we'll have to wait and see though. Dr. Fitch feels that he got the instrumentation and the banked bone fusion in very "tight" which apparently is a good thing.

The one thing we will miss as always when leaving North Carolina are the friends we have made along this journey. Thank you so much to Craig, Kym, and Maddy for making our stay so comfortable. Steve thinks our phone bill is going to be a lot more expensive now and he's right! :-) Also, thank you so much to Stacy for being such a great friend. The lasagna was wonderful and you are going to make an amazing doctor whatever path you choose!

Please mark February 25TH on your calendar as National MPS Day. I will be posting more on this in a future post. Also, I'll be putting new pictures up in the next day or so...

Love ~ Mel, Steve, Jakie, & Matt-man

P.S. The turtle border is in honor of Jake's "tortoise-shell" brace! :-)

Thursday, February 10, 2005 9:31 AM PST

After a difficult day of flying yesterday we arrived home! Jake is thrilled to be back! I'll post an update and some pictures in the next day or so...once I get back on West Coast time. Thank you all for your continued support and prayers.

Love ~ Mel, Steve, Jake, & Matt

Friday, February 4, 2005 9:12 AM EST

Just a quick update to let you know that all is going well here in sunny...but cold North Carolina. We relaxed around the house yesterday which seemed to get Jake all rested and ready to WALK yesterday evening. He and Matt got a package in the mail from Jake's wonderful teachers' assistant Ms. Vicki and Steve and Matt were opening it at the kitchen table while Jake was out on the couch. Jake heard them talking about all of Jake's favorite things that Ms. Vicki sent (paper, crayons, glue, scissors, etc.) so he rolled himself off the couch and walked...unassisted!...out to the kitchen table to participate. Thank you SO much to Ms. Vicki for motivating Jake to get moving with your gift. You're the best! Medically, Jake continues to need pain medicine occasionally. I would say he has begun to tolerate his brace and assists us with putting the front piece on and off when we're changing his diaper or giving him a sponge bath as these are the only times it really comes off. I think he is beginning to understand that if he's not wearing it we can't pick him up and he can't move around which is no fun for an active 4 year old!

Last night Jake's 'Best-Buddy' from transplant, Stacy, came to see us and joined us for dinner. It was wonderful to see her! Jake wasn't too interested but then he thinks pretty much anyone besides those he knows well are going to do something bad to him.

I have to once again mention how thankful we are to the hospitality of our friends...Kym, Craig, & Maddy. I truly think that being in a home environment for Jake's recovery has made all the difference in the world! I don't know if we'll ever be able to fully show our appreciation!

Steve will be heading home tomorrow morning which will make things much more difficult. The boys will miss their Daddy terribly but if all goes as planned we'll be joining him 5 days later.

Take care and take time every day to enjoy your children to the fullest!

Love ~ Mel, Steve, Jake, Matt, Kym, Craig, Maddy, Grandma Patty, & Grandpa Steve

Wednesday, February 2, 2005 9:13 AM EST

Jake was discharged from the hospital yesterday and we are now comfortably ensconced with Kym, Craig, & Maddy. Jacob has seemed so much happier since being discharged. He has even tried to get around on his own a bit. He is still not able to walk on his own but he has been more willing to attempt to get around and much more willing to sit up which in turn allows his lungs to continue to repair themselves. He is still in some obvious pain and discomfort a lot of that being from his big bulky brace. He actually looks like a football player when I put a shirt on over the thing --- a shirt at least one size bigger than his normal size!!! Before being discharged Jake had an x-ray done on his spine and his fusion looks great as well as his spine. He also had an EEG done which Dr. K ordered because Jake had had a little incident his first day back to school after the holiday break. His speech therapist was concerned that he had a seizure although I thought he was very tired that day as he hadn't slept the night before. I think he might have been tuning her out and/or dosing off a bit! Regardless, we wanted to err on the side of caution and check to make sure everything was OK and his EEG results were perfect...no current or past seizure activity present. That's what we like to see! We are also waiting on the results of his cortisol level to see if we can finally take him fully off his steroids. Besides all that, Jake is definitely in the recovery phase of his surgery and we are just here to assist him however we can --- I think today we might try to get him out and get him some fresh air and a change of environment...

Grandma Patty and Grandpa Steve have arrived as well which is very nice. The boys were thrilled to see them!
That's it for now...I'll continue to let you know how things are going...

Love ~ Mel, Steve, Jake, Matt, Kym, Craig, Maddy, Grandma Patty, & Grandpa Steve

P.S. Cris - I would love for Jake to get hooked up with the PT at Prov. --- especially one who is familiar with orthopedic issues.

Sunday, January 30, 2005 7:39 PM EST

Jake is slowly but steadily improving. He's still in a considerable amount of pain and discomfort but much improved from the past several days. We seem to have a good combination of medications keeping him comfortable. He's eating and drinking a bit more...he ate the meat sauce off the top of his spaghetti tonight and appeared to really enjoy it! :-) He still does not really want to walk with the physical therapist but when I tried it alone tonight he was more willing to walk for me. I did have to support his whole body but he was trying really hard for me. It is very obvious that the brace is going to take A LOT of getting used to...it is heavy and cumbersome for him. He did not have a great center of balance to begin with and this has really thrown things off for him. Once he's discharged he will be receiving some out-patient physical therapy here at Duke and I will more than likely be setting some up at home once we get there. It sounds like he is going to be discharged either tomorrow or Tuesday but I think they're leaning more towards tomorrow. It's a bit scary to think of taking him home so soon as he is still very much in the recovery phase of things and we have to be very careful with him.

Our wonderful friends the Wigglesworth's stopped by to visit Jake yesterday. It was great to see them again and they brought gifts for all of us. It is so nice to have their support during our time here. Steve and my Mom think Kym and I are related! They think we look similar and have very similar personalities. It cracks me up because we do get along really well...it must be that MPS genetic thing!!! :-)

My Mom, Grammie Cindy, flew home to Washington this afternoon. We were very sad to see her go as she has been a huge help to us this whole difficult week. We appreciate everything she did for us but especially trying to keep a sense of normalcy for Matthew...Thank You, Mom...Matt-man wants you to e-mail him! :-)

Tomorrow evening Grandma Patty and Grandma Steve arrive to help us out. We are so appreciative of both our parents' willingness to be here for us. It provides us such help and support during a very stressful and intense time.

Love ~ Mel, Steve, Jake, & Matt

Friday, January 28, 2005 7:50 PM EST

Jake had a bit of a rough night last night but things have calmed down somewhat tonight. His lungs and breathing have improved alot but his bowels started to act up last night and most of the day today. Things just weren't moving like they should (this can sometimes happen from a surgery) so poor Jake had to be given some 'things' to help. He finally went poopy this afternoon which hopefully helped him feel better. He appeared to be having some very bad cramps. The dressing was changed on his incision today and it looked really good. His brace came this evening and he is not so thrilled about it but Steve and I feel much more comfortable holding him and moving him around. The physical therapist and I tried to get him to walk with it and he took about 5 steps which was good. He cried the whole time and was miserable which was hard but we've got to try to get him moving to make his system start working more properly. He is still in a considerable amount of pain and discomfort so we're just doing our best to try to keep him comfortable. He is resting right now after a dose of phentynol. Thank you all for continuing to check in on Jake and keeping him in your thoughts and prayers. He's having a tough time with things and I can tell that he just doesn't understand why he's here and why he's feeling the way he feels. I wish I could explain it to him...

Love ~ Mel, Steve, Jake, Matt, & Grammie Cindy

Thursday, January 27, 2005 6:00 PM EST

So sorry for not updated sooner...I know many of you are checking in and worrying about Jake! We have had a bit of drama around here so I was not able to get to the computer or the phone. Jake is stable now and much more comfortable. His surgery yesterday went very well. It took about 4 1/2 hours. Jacob was VERY hard to intubate (which we've dealt with in the past) and this took a long time. His throat is very raw and sore! Dr. Fitch (Jake's ortho. surgeon) felt that he was able to get Jake's spine straightened and fused very well which is excellent. The anesthesiologists were able to extubate Jake after the surgery and he was able to move all his extremities which is very important after a spinal surgery. He was taken to the PICU (pediatric intensive care unit) to be monitored after the surgery. We went up to him and were pleasantly surprised at how good he looked. He had very mild facial swelling and his coloring looked good. He had not needed any transfusions during the procedure...he actually lost hardly any fluids. There was no need for a drain at the incision site because it was so easy to stitch him right up. He has a big dressing on his back and we haven't seen what the incision looks like yet. The PICU nurse was keeping him comfortable with some dilauded (a narcotic) and he was resting. We stayed with him and were just beginning to gather our stuff together as the parents are not allowed to stay in the PICU between 7pm and 8pm, when Jake's breathing started to sound very loud and 'junky' - we watched him for a minute or two and I saw his oxygen saturation level decreasing rapidly. Jake turned a shade of blue and was not breathing well...the nurses and doctors were able to bring his oxygen intake back up with medications and position changes but his breathing was still VERY wet. It appears that he had been loaded up with too much fluid during the surgery and he did not have enough going out as he should so his lungs had become wet. His chest x-ray showed fluid in both lungs and a small area of collapse on the left side. He also was not metabolizing the medication as rapidly as normal and was a bit over-medicated and this is why he de-sated. This happened a few more times throughout the evening and it was a long night trying to get him comfortable. All the narcotics were causing him to de-sat so he was beginning to really feel the pain from his surgery which broke my heart. I was not able to pick him up to comfort him which was incredibly difficult as well. We finally were able to find a hallucinogen (ketamin) that helped him sleep and took the edge off. I stayed with him all night (parents are not allowed to sleep in the PICU) and tried to comfort him the best I could. He was given diuretics and was able to get rid of a lot of the fluids that were making his lungs wet which was good. He was taken to a regular room this morning but is still being monitored closely. His breathing sounds much better and he's had several chest x-rays since he de-sated. The last one looked much better although there is still some cloudiness and a small area of collapse. This should clear up as he begins to move around more although he will be having chest x-rays several times a day until his lungs return to normal. He's still on oxygen but only 2 L which is a pretty small amount. He is definitely not happy at all to be here in the hospital and he is in some pain from the surgery. He was fitted for his brace today (he was too sick yesterday) and it should be here by tomorrow evening. The physical therapist has been by to see him already and taught Steve and I how to pick him up without hurting his spine which was great. We actually had him standing up for a couple of seconds (he was REALLY pissed!) and he is taller! We have to try to keep him moving and get him to stand several times a day as this will help with his lungs and the joint stiffness that can come with this type of surgery. Steve's going to stay with him tonight and I'm going to go back to the hotel room to spend some time with Matthew. He's taking this pretty hard and is having a very tough time figuring out what is going on with his brother. Thank you all for thinking of Jake and praying for him. We know that all your good thoughts and prayers being sent our way is what helped him get over this latest hurdle.

I also want to say to our friends the DeVon's that we love you and are going to be home soon and will be there for you as soon as we can. I had asked for all of you to send your good thoughts and prayers their son Derek's way and I need to ask you to do that again. They are an amazing, strong family that has been hit by another devastating blow. Derek has relapsed once again and they are only left with one option for treatment...he will be going through bone marrow transplant very soon.

Love ~ Melanie, Steve, Jacob, Matthew, & Grammie Cindy

Tuesday, January 25, 2005 11:07 PM EST

We had a long but productive day here in Durham. We started the day with a pre-op appointment to go over everything related to Jake's surgery and subsequent hospital stay. We found one of our old transplant Nurse Practitioners, Bridget, is now the pre-op Nurse Practitioner which was great for us because we didn't have to explain Jake's history to her...she knew the majority of it! We then met with the Child Life Specialist who explained Jake's surgery to him (very cute!) and went over policy, procedure, etc. with us. It was a nice refresher course as it's been awhile since we've been admitted for any length of time her at Duke. Jake then had an Evoked Potential study which is actually a nerve conduction study of the whole body. They gave him some chloral hydrate to help him sleep which he fought tooth and nail. It took an hour to get him to fall asleep but he finally did. His nerves in his body showed normal response which is beneficial for the surgery tomorrow as they will be monitoring his nerves closely to guard against nerve damage. Jake then had one spine x-ray and we were finally out of there (9:15am – 4:15am). Jake is first on Dr. Fitch's surgery schedule tomorrow so we have to check-in at 7am (4am for those at home) and his surgery is scheduled to begin at 8:45am (5:45am at home).

Tonight we relaxed a bit then went out for dinner. The boys were a bit crazy. They definitely know something is going on tomorrow!!! We then went swimming for a very long time and in the hot tub here at the hotel. We met another family with an adorable child named, Zach, who are beginning the transplant process. Zach has Morquix-Lamy (?sp) which is in the same family as Jake's disease. We brought the boys back to the room and gave them a big long bath. This will be Jake's last swim and real bath for the next 4 months so we thought we better do it up right!

We're off to sleep as we have to be up bright and early tomorrow. Please keep Jake in your prayers --- especially between 8:45am and 2:45pm as this is probably how long our precious baby will be in the hands of his surgeons, doctors, and nurses. Thank you!

Love ~ Melanie, Steve, Jake, Matt, & Grammie Cindy

Monday, January 24, 2005 11:23 PM EST

We have arrived and settled into Durham, safe and sound. Our flights yesterday were pretty much on time which is somewhat shocking due to the inclement weather situation. We went swimming in the hotels indoor pool (way too cold outside!) this morning, which the boys loved...we'll swim tomorrow too as this will be Jake's last chance for 4 months. We then went to one of the best shopping places around, Streets of Southpoint, and the boys played in the cool play area there. We also went to Build A Bear so Jake could make something to take to the hospital and Matt could have something for the hotel room. Jake picked Elmo and Matt picked a monkey he named Magic and dressed in a basketball uniform! Jake's Elmo is wearing denim shorts and a Duke t-shirt. They're really cute! We then headed to the Wigglesworth's to visit with them and have dinner. They are just wonderful and so good to us. Jake and Matt can't wait to stay with Maddy! Tomorrow the pre-op appointments begin at 9:45am so I best be off to bed. Please keep Jake in your thoughts and prayers tomorrow as he is not going to enjoy all the poking and prodding! Also, please keep our friends the DeVon's in your prayers...especially their son, Derek, as he ends his chemo and has some procedures done.

Love ~ Mel, Steve, Jake, Matt, & Grammie Cindy

Sunday, January 16, 2005 11:10 AM PST

We hope your holidays were wonderful, ours certainly were. We had an amazing time at The National MPS Society Conference at Disney World (as you can see by the pictures!). We met so many amazing people...I am so proud to count myself a part of such a strong, compassionate community of people dealing with this horrific group of diseases. I wear my purple 'COURAGE' bracelet every day for my son and for all those who share this commonality. I wouldn't be honest if I didn't say that there were difficult times for me personally at this conference...the families who have lost those they love and the ones who are more involved in their diseases...this was very hard to see... But to see my son so incredibly willing to interact with children he KNEW he shared a bond with was priceless. It was great to see Dr. K and hear her presentation at the conference. Her perspective on treatment options for MPS kids is so important and we've felt that she hasn't had a good opportunity to give her opinion to this group so to be there when she did was very important to us.

We also, of course, had an amazing time outside the conference. If Jacob were only a little taller he would have gone on absolutely every ride even the ones Mommy gets scared on! He is so fearless and seems to love the excitement and the rush. We also made sure and went to all the 3D shows as Jake was incredibly fond of them as well. Matthew on the other hand is not so interested in the rides (I think scared to death might be the best term!). He loved the characters, he loved the face painters, and he loved hanging out with all of us...in particularl, his cousin Nate! We had a very enjoyable time just all being together and seeing what a wonderful time our kids were having. The one crazy part of our trip was the weather. It was literally ARCTIC! The night of Mickey's Very Merry Christmas Party the wind chill was in the 20's! We actually all ended up buying more clothes, blankets, even stocking caps and gloves! My Mom and Matthew actually headed back to one of our rooms early that night as they were just too cold! It was never really warm enough to go swimming or anything plus we were so busy I'm not sure if there would have been time. By the time we spent all day at the parks, all evening at the conference, Steve and I would get the boys & Lauren back to our room and pass out! Having Lauren with us was great! She was very helpful with the boys and fun for me to have another female perspective (you know...someone to gang up on Steve with!).

Once we got home we had two days to finish our shopping, unpack, and clean our house. Christmas Eve we went to my grandma's place for my family Christmas party with all extended family. It was very nice as we haven't been able to attend the last couple of years because of Jake's weakened immune system. Christmas Day we decided to have everyone over to our house so we had 16 people throughout the day and Steve and I made Christmas dinner (turkey and all the stuff that goes with it) for our first time. We actually loved having everyone come to our house. We both love cooking so that was no problem for us and I felt like we could relax more because we didn't have to run from place to place.

Once we got through Disney and the holiday, we began preparing for our upcoming return to Duke. We (Steve, Jake, Matt, my Mom, and I) will be flying out on Sunday, Jan. 23RD. We will be staying at the Millenium hotel right by Duke the first week we're in Durham. Jake has pre-op appointments on Tuesday, Jan. 25TH and surgery on Wednesday, Jan. 26TH. They'll let us know what time the surgery will be at his pre-op appointments. His surgery will take a minimum of 3 hours and the entire time he will be facedown with his legs slightly elevated. This will cause all the fluid in his body to drain to his face and he will not be looking very good. After the surgery, he will be taken to the PICU (yes, I am scared!!!) and will probably remain intubated through the night. He'll go to a regular room the next day if all is well and will remain in-patient until Sunday, Jan. 30TH or Monday, Jan. 31ST just depending on how things are going. My Mom will be flying home on Sunday, Jan. 30TH and Steve's parents will be flying in on Monday, Jan. 31ST. Once Jake is discharged we will be checking out of the hotel and staying with the Wigglesworth's near Durham. I think having Matthew and Maddy around will be great for Jake and hopefully help him recover. Jake will have a few appointments the remainder of our time there to check on how his cast/brace is fitting and see how he's doing. Steve will be flying home to resume working on Saturday, Feb. 5TH. Jake will have a final appointment with Dr. Fitch (his ortho. surgeon) on Tuesday, Feb. 8TH and we (Jake, Matt, Steve Sr., Patty, and I) will be flying home on Wednesday, Feb. 9TH. Jake will remain in the brace for 4 months and we will have to return to Duke in late April/early May for an additional post-op appointment as well as for his 3 year post-transplant testing. We're hoping this will become a trend for us and we'll only have to return to Duke once a year! I plan to take our laptop and will be updating the journal more frequently during our time in North Carolina. I'll be sure to let everyone who still checks Jake's website know how things go with his surgery.

One other piece of Jake's medical news, we restarted his immunizations on Friday. He was not thrilled but I am so glad for the chance to return to normalcy. Now I have to make an appointment for Matthew to get all his live vaccines. He's really going to hate this as he was there on Friday when Jake got his...kicking and screaming!

Well, I better get to cleaning and packing as we leave in a week! A special 'thank-you' to our friend Craig for house/pet-sitting for us while we're gone.

Love ~ Melanie, Steve, Jakie, & Matt-man

***Check out the photos from our Disney trip while you're here!***

Sunday, January 16, 2005 12:18 AM PST

An update will be coming in the next day or two... In the meantime, I've put up some pictures from our wonderful trip to Disney World last month for the National MPS Society Conference. Enjoy!

Sunday, December 5, 2004 12:40 AM PST

Happy Holidays!

It's been awhile since my last update and things have been exciting around here. Jake has had one cold after another...'tis the season! His first carpal tunnel surgery on October 28TH was cancelled due to a head cold. He ended up in the hospital for a couple of days at the beginning of November because of a high fever and cold symptoms and maybe an ear infection. He then had his left carpal tunnel surgery a couple of days before Thanksgiving. He did exceptionally well with this surgery and was using his hand, cast and all, within hours. We were thrilled! A few days after Thanksgiving I got a horrible stomach virus that the entire family came down with after me! Of course, it lasted much longer in Jake and just today he has finally started to seem better. Also, he's back on antibiotics because he had some green drainage coming out of his nose on Friday when he had his routine appointment with Dr. Hawkins at Children's and we don't want to take any chances as we leave for DisneyWorld in 8 days!

Jake has been doing great at school when he's there...unfortunately...he's had to miss a lot due to illness and appointments. His teachers and therapists really feel like he's settling in and getting the hang of things. Last Monday (before he got sick) he was so excited to go out and meet the bus that he actually put on his coat, hat, & bus harness with very little assistance from me! He went on his first official field trip to the pumpkin patch before Halloween and he brings me home his art projects almost every day. Art is absolutely Jake's favorite thing to do at school!

We got a few more results from our last trip to Duke. Jake's immune function is normal and alpha-L-iduronidase level is normal. Don't you just love the word 'normal'!?! Also, it looks as if we will be heading back to Duke in January. Steve and I have decided to have Dr. Fitch at Duke perform Jake's spinal fusion surgery for his kyphosis. We feel much more comfortable with someone who has performed this surgery on kids with treated Hurler Syndrome and we are also comfortable within the Duke system. Logistically and financially, this will be a bit of a struggle but we'll figure it out. The pre-op is scheduled for Tuesday, January 25TH and the surgery will be Wednesday, January 26TH. Jake will spend approximately 4 days in-patient and then we will have to stay in Durham until Wednesday, February 9TH if all goes as planned. We will be staying in a hotel near the hospital for a portion of this time but will also be relying on the kindness of our friends the Wigglesworth's who have graciously offered us a place to stay in their new home outside of Durham. I'll let you know what's going on with all of this as we get nearer the surgery date.

Last but not least, we are very excited to be heading off to DisneyWorld on December 13TH to December 21ST for The National MPS Society Conference. We can't wait to meet new friends and actually come face-to-face with those we consider friends although we've never actually met in person! We will be traveling with my Mom (Grammie Cindy) and our niece Lauren (who's 13 and will be a big help with our boys). Steve's parents (Grandma Patty & Grandpa Steve) will be arriving the same day and bringing our nephew, Nathaniel. And, my sister (Auntie MoMo) and Dad (Grampie Mark) will be joining as for a few days as well. We are really looking forward to this experience and I will be sure to post all about it including pictures when we return.

We hope your holidays are wonderful! We hope you all get a chance to spend time with family, friends, and all those you're close to as we certainly plan to this holiday season!

Love ~ Melanie, Steve, Jake, & Matt

!!!UPDATED PHOTOS!!!

Monday, October 18, 2004 5:59 PM PDT

Well, I have so much info. running around in my head from the appointments Jake just had this past week at Duke. I have to say that I sometimes use this journal just to get my thoughts on 'paper'! Hope you don't mind! :-) Now, I feel like I need to say, "do you want the good news or the bad news first?" I probably don't need to say it but the 4 days Jake and I were in Durham were VERY hectic, exhausting, informative, overwhelming, etc. and of course our pre-arranged schedule did not go as planned as other tests and procedures were added/changed on an hourly basis (sorry Rebecca :-)!!!). I guess I'll just start from the beginning...

I don't think I'll be attempting the 'red-eye' flight with Jake again (for that matter I am once again going to say I am never doing this alone again!). He was not able to sleep well until the very end of our flight and then we had to meet our connection in Atlanta and pop on another plane for an hour. Jake did not like that and cried the whole flight from Atlanta to Raleigh/Durham. We had a very unpleasant man sitting next to us who proceeded to shake his head and sigh loudly the entire flight. Trust me...if I hadn't been so exhausted and trying to calm Jake down I would have given him a piece of my mind...I thought it best to hold my tongue at the time! Anyhow, we arrived...and headed off for Duke...Jake had his labs drawn and the results we've gotten back so far look good. We're still waiting on his immune function and alpha-L-iduronidase level. Jake had his pulmonary function test (lung capacity) and got 104hich is wonderful as the highest he's gotten in the past was 94 Jake saw Dr. Greene (eye doctor) and was very uncooperative (I came to find that this was the theme of our whole 'trip'). They were able to get an OK look at his eyes and they look about the same as last time. He still needs to wear his glasses (far-sighted) and he still has mildly clouded corneas.

The next day we were off to UNC to see Dr. Escolar and her team for Jake's development testing. Do you remember when I said "uncooperative" before...well, that was putting it mildly!!! Jacob was probably the worst I've ever seen him during development testing. He was surly, he would not do anything asked of him, he SPIT on the 2-way mirror, he threw toys at the Speech therapist, and the list goes on... I have no idea what type of results we're going to see when we get the report in 4 weeks. Also, I wanted to discuss some of the behavioral issues Jake is having at school (hitting kicking, & biting at the teachers, therapists, & others in authority positions a.k.a those who are trying to get Jake to do what they want him to do) with them which I ended up not having to do because they got to see them first-hand and then some! Anyhow, like I said I don't have the full report but Dr. Escolar wants us, Jake's teachers, & therapists to start using parts of a method that is used for kids with autism. Dr. Escolar believes that Jake has some similar characteristics (his behavior & sensory issues primarily) that are similar to those of children with autism and believes that one of the two methods used in treating kids with autism might help Jake. This is not to say Jake has autism but apparently children with cognitive delays like Jake tend to have "autistic tendencies." I made them clarify to me that he is NOT being diagnosed with autism they are just thinking that the TEACCH or ABA approach might be beneficial to Jake and us. I can't help but wish he would act a bit more like himself when we're at these development appointments because I don't really feel that they get a good overall look at Jake...they just get a window into some of what makes Jake who he is. I have already spoken to Jake's pre-school teacher about this and she is going to utilize the information given to help Jake any way she can. Kristine (Jake's teacher) is very willing to do what we need her to do (within the constraints of the public school system of course!) to help Jake reach his full potential. After a stop at Jake's favorite restaurant (YES, McDonald's) we were off to Dr. Fitch's office (orthopedic surgeon). Jake's kyphosis (spinal curvature) has gotten much worse. A little over a year ago Dr. Fitch measured it at a 40 degree angle, he is now measuring it at a 66 degree angle. This is a HUGE increase and the recommendation is for Jake to have spinal surgery soon. He actually has the kyphosis in 2 areas of the spine (L2-3 & C7) and the areas will be fused and straightened using metal/titanium rods. This apparently won't affect his growth in these areas which we are thankful for. Now the question is whether or not to have it done here or at Duke. I had hoped to be able to have it done here but Jake's is pretty severe and Dr. Fitch has done several kyphosis surgeries on post-transplant Hurler's patients whereas his local ortho. surgeon has never actually performed kyphosis surgery on a child with Hurler's...so...we are leaning towards having it done at Duke...which means 4 days in-patient and at least 2 weeks in North Carolina and 4 months in a back brace! We are planning to put it off until after we go to Disney World in December and at this time this is OK with Dr. Fitch but Jake would probably need to have it done VERY soon after that time. I'll keep you all updated on this issue as we don't have everything figured out at this time. Then we headed over to Clinic to see our old Nurse Practitioner, Jen Condron. I absolutely adore her and am so happy that I was able to see her and catch up with her. Jen is 8 months pregnant with a baby boy --- CONGRATULATIONS to Jen and her husband Steve! We then saw our new Nurse Practitioner, Gil, and went over everything with him. Jake thankfully was wiped out by then and slept almost the whole time.

Wednesday morning bright and early Jake had a brain & spine MRI and an ECHO under sedation. Jake's ECHO looks the same...pretty much normal with some very minimal swelling from steroid use. Jake's brain MRI was amazing. There is no longer any fluid present. Jake's brain is the size of a normal 4 year old AND the areas that had fluid in the past where the brain tissue wasn't able to grow have filled in with brain tissue!!! This is HUGE news for us and tells us two things: the stem cells from the unrelated umbilical cord given to Jake at transplant have crossed the blood brain barrier and have re-grown damaged brain tissue AND with all these new areas of brain tissue, Jake is ripe for learning. He has new brain tissue present that is waiting to be filled up and we should start to see Jake's development curve increase! Yippeee!!! On the other hand, Jake's spine MRI showed the 2 areas of kyphosis and some narrowing of the spinal column. Thankfully there is no spinal compression yet but this is why it is critical for Jake to have the spinal surgery as we don't want there to be any spinal compression. We then headed up to Clinic so Gil could examine a very cranky, drugged up, vomiting Jake. Apparently, the sedation did not sit well on Jake's tummy and he was not a happy little guy. Here's where Jake's schedule changed drastically as Gil then walked us down to the operating room where Jake was put under anesthesia for 4 HOURS to have an ABR/BAER hearing exam and an EMG (carpal tunnel exam). Needless to say, this turned into a LONG day! I have to be honest though and say that after how uncooperative Jake was acting about everything, it was nice to sit back and let some of the tests be performed while he was 'asleep' so I didn't have to hold him down and take a beating! I was even able to read a book, get some sushi from the cafeteria, and watch Oprah. :-) Here's some more GREAT news. Jake's hearing is fine. He has NO hearing loss. He no longer needs to wear his hearing aids. Jake had a sensory-neural hearing loss (a brain hearing loss) and the transplant has repaired his hearing! This is VERY exciting news! The audiologist has never seen this before even in a post-transplant patient although we heard rumors that it could happen. Jake's EMG showed something a bit different this time as well. It showed that the carpal tunnel on his left has gotten more severe but the carpal tunnel on the right was within normal limits. Jake still has carpal tunnel surgery scheduled for October 28TH at Seattle Children's with Dr. Hanel but it looks like he might just be doing the left side this time and we'll continue to monitor the right side.

Our last day there, we met with Dr. K. We went over all the results I talked about above. Dr. K. thinks Jake looks amazing and is just moving in the right direction. She thinks it's taken Jake a bit longer to get to where he is than some of the post-transplant kids but as we know Jake does things at his own pace. She gave me some helpful information on Jake's development and how to work with some of Jake's unique characteristics and we went over absolutely everything. It was an informative meeting as always and I have come home with tons of information to go over. As additional results and info. come in she will be getting it to me and I will share it with you. The last thing we did before our flight was met my friends the Wigglesworth's for lunch. It was GREAT to see them. Maddy looks amazing and is 5 YEARS post-transplant so I just love to see her. Kym and Craig are such great people and even invited us to stay with them if we decide to have Jake's spinal surgery done at Duke. I am once again humbled and overwhelmed by the generosity of those we've met through this process! The flight home went a bit better until the battery on the portable DVD player died --- thankfully this was an hour before we landed so I just had to try to keep Jake occupied and in his seat for a short time.

So sorry for the extra long post but I wanted to try to get everything in for those of you who still check in on Jake. Take care and I'll be updating as we get new information on Jake's surgeries, etc.

Love ~ Melanie, Steve, Jake, & Matt

Monday, October 18, 2004 5:59 PM PDT

Well, I have so much info. running around in my head from the appointments Jake just had this past week at Duke. I have to say that I sometimes use this journal just to get my thoughts on 'paper'! Hope you don't mind! :-) Now, I feel like I need to say, "do you want the good news or the bad news first?" I probably don't need to say it but the 4 days Jake and I were in Durham were VERY hectic, exhausting, informative, overwhelming, etc. and of course our pre-arranged schedule did not go as planned as other tests and procedures were added/changed on an hourly basis (sorry Rebecca :-)!!!). I guess I'll just start from the beginning...

I don't think I'll be attempting the 'red-eye' flight with Jake again (for that matter I am once again going to say I am never doing this alone again!). He was not able to sleep well until the very end of our flight and then we had to meet our connection in Atlanta and pop on another plane for an hour. Jake did not like that and cried the whole flight from Atlanta to Raleigh/Durham. We had a very unpleasant man sitting next to us who proceeded to shake his head and sigh loudly the entire flight. Trust me...if I hadn't been so exhausted and trying to calm Jake down I would have given him a piece of my mind...I thought it best to hold my tongue at the time! Anyhow, we arrived...and headed off for Duke...Jake had his labs drawn and the results we've gotten back so far look good. We're still waiting on his immune function and alpha-L-iduronidase level. Jake had his pulmonary function test (lung capacity) and got 104% which is wonderful as the highest he's gotten in the past was 94%. Jake saw Dr. Greene (eye doctor) and was very uncooperative (I came to find that this was the theme of our whole 'trip'). They were able to get an OK look at his eyes and they look about the same as last time. He still needs to wear his glasses (far-sighted) and he still has mildly clouded corneas.

The next day we were off to UNC to see Dr. Escolar and her team for Jake's development testing. Do you remember when I said "uncooperative" before...well, that was putting it mildly!!! Jacob was probably the worst I've ever seen him during development testing. He was surly, he would not do anything asked of him, he SPIT on the 2-way mirror, he threw toys at the Speech therapist, and the list goes on... I have no idea what type of results we're going to see when we get the report in 4 weeks. Also, I wanted to discuss some of the behavioral issues Jake is having at school (hitting kicking, & biting at the teachers, therapists, & others in authority positions a.k.a those who are trying to get Jake to do what they want him to do) with them which I ended up not having to do because they got to see them first-hand and then some! Anyhow, like I said I don't have the full report but Dr. Escolar wants us, Jake's teachers, & therapists to start using parts of a method that is used for kids with autism. Dr. Escolar believes that Jake has some similar characteristics (his behavior & sensory issues primarily) that are similar to those of children with autism and believes that one of the two methods used in treating kids with autism might help Jake. This is not to say Jake has autism but apparently children with cognitive delays like Jake tend to have "autistic tendencies." I made them clarify to me that he is NOT being diagnosed with autism they are just thinking that the TEACCH or ABA approach might be beneficial to Jake and us. I can't help but wish he would act a bit more like himself when we're at these development appointments because I don't really feel that they get a good overall look at Jake...they just get a window into some of what makes Jake who he is. I have already spoken to Jake's pre-school teacher about this and she is going to utilize the information given to help Jake any way she can. Kristine (Jake's teacher) is very willing to do what we need her to do (within the constraints of the public school system of course!) to help Jake reach his full potential. After a stop at Jake's favorite restaurant (YES, McDonald's) we were off to Dr. Fitch's office (orthopedic surgeon). Jake's kyphosis (spinal curvature) has gotten much worse. A little over a year ago Dr. Fitch measured it at a 40 degree angle, he is now measuring it at a 66 degree angle. This is a HUGE increase and the recommendation is for Jake to have spinal surgery soon. He actually has the kyphosis in 2 areas of the spine (L2-3 & C7) and the areas will be fused and straightened using metal/titanium rods. This apparently won't affect his growth in these areas which we are thankful for. Now the question is whether or not to have it done here or at Duke. I had hoped to be able to have it done here but Jake's is pretty severe and Dr. Fitch has done several kyphosis surgeries on post-transplant Hurler's patients whereas his local ortho. surgeon has never actually performed kyphosis surgery on a child with Hurler's...so...we are leaning towards having it done at Duke...which means 4 days in-patient and at least 2 weeks in North Carolina and 4 months in a back brace! We are planning to put it off until after we go to Disney World in December and at this time this is OK with Dr. Fitch but Jake would probably need to have it done VERY soon after that time. I'll keep you all updated on this issue as we don't have everything figured out at this time. Then we headed over to Clinic to see our old Nurse Practitioner, Jen Condron. I absolutely adore her and am so happy that I was able to see her and catch up with her. Jen is 8 months pregnant with a baby boy --- CONGRATULATIONS to Jen and her husband Steve! We then saw our new Nurse Practitioner, Gil, and went over everything with him. Jake thankfully was wiped out by then and slept almost the whole time.

Wednesday morning bright and early Jake had a brain & spine MRI and an ECHO under sedation. Jake's ECHO looks the same...pretty much normal with some very minimal swelling from steroid use. Jake's brain MRI was amazing. There is no longer any fluid present. Jake's brain is the size of a normal 4 year old AND the areas that had fluid in the past where the brain tissue wasn't able to grow have filled in with brain tissue!!! This is HUGE news for us and tells us two things: the stem cells from the unrelated umbilical cord given to Jake at transplant have crossed the blood brain barrier and have re-grown damaged brain tissue AND with all these new areas of brain tissue, Jake is ripe for learning. He has new brain tissue present that is waiting to be filled up and we should start to see Jake's development curve increase! Yippeee!!! On the other hand, Jake's spine MRI showed the 2 areas of kyphosis and some narrowing of the spinal column. Thankfully there is no spinal compression yet but this is why it is critical for Jake to have the spinal surgery as we don't want there to be any spinal compression. We then headed up to Clinic so Gil could examine a very cranky, drugged up, vomiting Jake. Apparently, the sedation did not sit well on Jake's tummy and he was not a happy little guy. Here's where Jake's schedule changed drastically as Gil then walked us down to the operating room where Jake was put under anesthesia for 4 HOURS to have an ABR/BAER hearing exam and an EMG (carpal tunnel exam). Needless to say, this turned into a LONG day! I have to be honest though and say that after how uncooperative Jake was acting about everything, it was nice to sit back and let some of the tests be performed while he was 'asleep' so I didn't have to hold him down and take a beating! I was even able to read a book, get some sushi from the cafeteria, and watch Oprah. :-) Here's some more GREAT news. Jake's hearing is fine. He has NO hearing loss. He no longer needs to wear his hearing aids. Jake had a sensory-neural hearing loss (a brain hearing loss) and the transplant has repaired his hearing! This is VERY exciting news! The audiologist has never seen this before even in a post-transplant patient although we heard rumors that it could happen. Jake's EMG showed something a bit different this time as well. It showed that the carpal tunnel on his left has gotten more severe but the carpal tunnel on the right was within normal limits. Jake still has carpal tunnel surgery scheduled for October 28TH at Seattle Children's with Dr. Hanel but it looks like he might just be doing the left side this time and we'll continue to monitor the right side.

Our last day there, we met with Dr. K. We went over all the results I talked about above. Dr. K. thinks Jake looks amazing and is just moving in the right direction. She thinks it's taken Jake a bit longer to get to where he is than some of the post-transplant kids but as we know Jake does things at his own pace. She gave me some helpful information on Jake's development and how to work with some of Jake's unique characteristics and we went over absolutely everything. It was an informative meeting as always and I have come home with tons of information to go over. As additional results and info. come in she will be getting it to me and I will share it with you. The last thing we did before our flight was met my friends the Wigglesworth's for lunch. It was GREAT to see them. Maddy looks amazing and is 5 YEARS post-transplant so I just love to see her. Kym and Craig are such great people and even invited us to stay with them if we decide to have Jake's spinal surgery done at Duke. I am once again humbled and overwhelmed by the generosity of those we've met through this process! The flight home went a bit better until the battery on the portable DVD player died --- thankfully this was an hour before we landed so I just had to try to keep Jake occupied and in his seat for a short time.

So sorry for the extra long post but I wanted to try to get everything in for those of you who still check in on Jake. Take care and I'll be updating as we get new information on Jake's surgeries, etc.

Love ~ Melanie, Steve, Jake, & Matt

Sunday, October 10, 2004 10:06 AM PDT

We are off to Duke this evening for Jake's 2 1/2 year post-transplant visit. I will post an update on everything when we get home and some new pictures of course! :-) Jake's had some issues crop up over the past month and hopefully when we get back we will have a better idea of how to proceed...at this point Jake has carpal tunnel surgery scheduled for Oct. 28TH and possibly knee stapling surgery at the same time to fix his genu valgum (knock knees). Here's our schedule at Duke just in case some of our North Carolina friends want to track us down:

Mon. 10/11
8am - arrive NC
noon - pulmonary function test
12:30pm - labs
2:30pm - eye doctor

Tues.
8am - Dr. Escolar & team
2:30pm - Dr. Fitch
after Fitch - Clinic

Wed.
7:30am - MRI
10:30am - Echo
1pm - nerve conduction study

Thurs.
8:30am - Dr. Kurtzberg

Just Jake and I are going this time so wish us luck!!!

Love ~ Mel, Steve, Jakie, & Matt-man

Wednesday, September 8, 2004 11:12 AM PDT

Today is Jacob's first day of pre-school and I couldn't think of a more appropriate day to do an update! As a matter of fact he is there right now...Matt and I took him this morning and he will be riding the bus home mid-day. We decided to start out by doing the bus just one-way to see how it goes and get him used to it...more than likely in the next couple weeks he will be riding the bus both ways. He was not too thrilled with being at school today which is typical Jake behavior...he pretty much wants to be with his Mommy &/or Daddy ALL the time! We're really hoping that this transition to pre-school helps a bit with this and he becomes more comfortable being around others. There are 7 kids in his pre-school class – 4 with developmental delays and 3 typically developing kids which I think is a good mix. My hope is that Jake is able to not only learn from his teacher but more importantly from the other kids in his class. He will be attending school Mon.-Thurs. 9am-11:30pm. Matthew is very envious and really wants to start school so our goal is to get him potty-trained so he can start going to pre-school a couple days a week as well (what am I going to do with all this free time!?!). Matthew and I will be starting MOPS (Mothers Of Pre-Schoolers) on Monday mornings at Smokey Point Community Church which should be fun and educational for Matt...hopefully this will make up for Jake 'getting' to go to school (if only Jake felt the same way Mattie does!). We also start swim lessons (both boys) next week – twice a week in the evenings. I'm looking forward to this and hope they enjoy it --- Jake adores being in the pool so I know he'll like it --- Matt is sometimes a bit fearful but I think he'll like it as well.

Our Summer has been great and somewhat 'normal' which is, of course, wonderful! The boys had a week of day camp which was amazing. They both were upset when I dropped them off but when I picked them up would be smiling and telling me how much they loved it. Jake and Matt were in different groups which was nice for them and got to do tons of fun, age-appropriate stuff. Since Summer Camp, Jake has been eating so much better with his favorites being cheeseburgers, french fries, and pizza (VERY typical kid food!!!). I think eating lunch around the other kids at camp made a big impact on him! The last day of camp they both ran in hand-in-hand with not even a backwards glance to spare!

Jake had some Summer School which was hit or miss as it was during the same time as one of our vacations and a doctors appointment. I think this was a nice mid-Summer refresher as far as therapies go though. He got both Speech and OT during these sessions as well as an Education component. Jake has also continued with his one hour of private OT and one hour of private PT through the Summer. He won't be getting the one hour of private PT now as we are hoping to add some private Speech therapy and think the activity he'll get from both the swim lessons and being in school will more than make up for the PT. He will get his OT (90 minutes per week) from his school therapist and his Speech (90 minutes per week) from a school therapist as soon as they hire someone. Unfortunately, the school seems to be having trouble getting this position filled and I am worried this will adversely affect Jake as his greatest weakness is his Speech, hence the need for us to try to line up some private Speech therapy. I'll be working on finding him a Speech therapist today and getting this scheduled into our busy weeks!

We also have had the pleasure of going on a few vacations this Summer which has been wonderful. We went camping for a weekend at Ocean Shores with Grandma & Grandpa Irish. The boys love the beach --- Matthew stays firmly on solid ground playing in the sand with his assorted tools and Jakie runs immediately to the big waves in the Ocean to play in the water. He's such a water baby! We spent a week camping at the end of July in Eastern Washington at Wanapum with Grandma & Grandpa Irish and Auntie Steph and cousins Nate & Alec. The boys and Steve & I had such a great time playing in the water all day every day. Our little daredevil Jake took a liking to the SeaDoos and would pretty much try to coax anyone he could to take him out on them. We had a wonderful weekend in August at Camp Prime Time with other families in our area with kids with MPS disorders. Jake was the only child with Hurler's but there were a couple SanFillippo and a couple Hunter's kids. It was so nice to finally meet some familes who are walking in similar shoes to us and we were so happy to meet such amazing, kind, considerate people. Finally, we have just spent the past 4 days at Whistler (British Columbia, Canada - site of much of the 2010 Winter Olympics) with Grammie Cindy & Grampie Mark. They have a timeshare condo and we finally were able to join them. Whistler is amazing and we had a great time! I am so happy we went, the boys loved it, and we all got some fresh mountain air, some great shopping, and some wonderful food! We can't wait to go back (Steve definitely wants to go back in the Winter for some snowboarding!).

The other big Summer event was of course the boys birthdays. Jacob turned 4 on July 29TH and Matthew turned 3 on August 23RD. We had a big joint party for them with family, some friends, & neighbors. The theme was Thomas the Train and we rented a giant train bouncy house/slide for them. They had a great time, got tons of gift, ate too much, played really hard, and had an all-around good time! Seeing them having such a good time was perfect for Steve and I to experience.

As you can see we've had a very fun-filled, healthy Summer. Jake is no longer getting monthly infusions of IVIG and Pentamadine and only has to see the doctor every 3 months now. He is scheduled to see Dr. K & team at Duke Oct. 12-14 (he and I will be going alone this time) and I am hoping and praying that will be nothing but good news. Our goal at this time is for him to get through cold season with nothing more than the normal kid type colds and then he can be re-immunized and have his port removed. Please pray for a healthy Fall & Winter then Steve and I will really feel like we're in the clear!!!

Thank you all for continuing to keep up on Jake and the rest of us and for all your thoughts and prayers over the past 2 1/2 years. Our thoughts and prayers continue to be with all of the other families we have encountered along this journey, especially those of you whose children are no longer with us on this Earth but who are our special Angels in Heaven.

Love ~ Melanie, Steve, Jake, & Matt

Monday, August 23, 2004 10:32 PM PDT

I am so sorry it's been so long since an update...Jacob has been having a fun, activity filled Summer and I will be posting on entry on everything in the next few days. In the meantime, I've put up some new photos. Enjoy!!!

Love ~ Mel, Steve, Jakie, & Matt-man

Saturday, May 22, 2004 10:50 PM PDT

*************************************
Additional Update May 29, 2004:

New pictures are posted! Enjoy!

Many of you know already, but if you don't...Jake's treatment for Hurler's has all been experimental and part of an on-going study at Duke. The data to this point has finally been published and Jakie is one of the children discussed in the article (not by name, by number) in The New England Journal of Medicine. Here'e the web address to the abstract: http://www.content.nejm.org/cgi/content/short/350/19/1960 To view the whole article you have to either pay $10 for it (that's what we did) online, buy a copy of that issue (I think Barnes & Noble sells them), or go to your local library. Also, I do have a copy of it and can e-mail or fax it (e-mail me directly if you want...). We are very proud that our son and what he has been through is helping doctors and other medical professionals and in turn helping other children and families dealing with this dreadful disease.

Enjoy the pictures & the article! Mel

**************************************

May 22, 2004 --- TWO YEARS POST-TRANSPLANT

HAPPY TRANSPLANT BIRTHDAY TO JACOB!!!

Wow, I can't believe it's been 2 whole years since Jake's transplant. I think I've said this before but it seems like yesterday in some ways and like a lifetime ago in others! I haven't had a chance to post anything for quite awhile...I apologize to the faithful website readers :-)...life has been so busy for us lately and it just seems to get busier. I'm sure that doesn't have anything to do with chasing after 2 very active toddlers, does it!?!?! :-)

Jake's health has been wonderful. He has had no adverse reactions to his medication taper and is almost fully off the Cellcept. He's still getting labs and IV meds every 4 weeks at Children's and I know his IVIG really helps to keep him healthy as Matt has had a few colds and Jake has not caught them at all. Jake is kept really busy with all his therapies and school (and I'm kept really busy keeping it all figured out!!!). We have added another therapist to the mix, a private physical therapist who Jake sees in her office in Marysville. He really seems to enjoy going there (so does Matthew) and is doing really well for her. He is also going to school a few times a week now for some of his therapy sessions and education when there are no children present. The school has worked out some scheduling to get Jake in a side entrance and have the teachers and therapist rotate in and out of a room that is not being used. This is working out OK but would be much better if his separation anxiety was not so bad. He cries REALLY hard when I leave him and from what I am told this lasts awhile (20-25 minutes). It breaks my heart to leave him but I know he enjoys it once he settles down. When I pick him up he's usually covered in paint and wet from the water table! Art is definitely his favorite 'subject' and he really is quite the little artist! He's talking more and even when he doesn't talk it is very obvious that he's taking in everything that is being said. He follows directions (when he wants to!) so we know he knows what's going on. He is wearing his glasses some of the time and they really seem to help with his vision. He'd be wearing them more often if I didn't have to give him 100f my attention when they are on his face. If I am not watching him like a hawk they end up in his mouth being chewed on. He needs new lenses already and the frames need to be tightened and adjusted again! Ugh!!! The hearing aids are a whole other issues that I won't even go in to...suffice it to say that they are not worn often and when they are he takes them out the minute I turn my back! He has had a couple appointments with the orthopedic surgeons at Children's. We've been told to monitor things and have him checked every 6 months which is what we've been doing. I was happy to get him re-established with some ortho. surgeons here as I prefer to have his surgeries closer to home now rather than having to go to Duke for every little thing, as long as we're comfortable with the doctors of course. We can expect at least 4 surgeries in the future for him...genu valgum (knock-knees), kyphosis/scoliosis, hip dysplasia, and carpal tunnel. These will all happen at different times...we've just got to watch as he grows and time the surgeries right so that his growth is not adversely affected.

Other than the medical and health stuff, Jake and Matt got an early birthday present from us and their grandparents. We all split the cost and got them a big play system for the backyard. It is wonderful with swings, a slide, a climbing wall, picnic table, monkey bars, sandbox, ladder, and fort. They absolutely love it and would be outside from the time they wake up to the time they go to bed if they could --- especially Matthew. We also planted a vegetable garden and the boys are very interested in helping with this as well. Besides this, we have a pretty busy Summer planned. Jake's out of school in mid June but will be attending Summer School for 4 weeks. Also, he and Matthew will be going to day camp for one week in July for developmentally delayed children and their siblings. We have a couple family camping trips planned and will be going to a weekend camp for Northwest MPS families in August. We are also supposed to be going back to Duke at the end of August so Jake can get a check-up before school starts in the Fall. As far as school in the Fall, I am really trying to work out a combination of public school so Jake can get his therapies and such and private school so he can be with typically developing kids. The problem we're running into is the potty-training issue. Most of the private schools can't/don't take kids in diapers and Jake is not potty-trained yet. I think I'm just going to try to potty-train both of them this Summer at the same time!!! Don't be alarmed if you come to our house and see a couple of bare bottoms running around --- I have a feeling that's what it will come to! :-)

Thank you to those who continue to check on Jake through this website. I will try to do a better job of keeping it current! I plan to add some new photos in the next couple of days so make sure and check back for those! Take care!

Love ~ Mel, Steve, Jake, & Matt

Friday, March 19, 2004 11:23 PM PST

HAPPY SPRING! :-)

Jacob is doing really well medically. He has been a happy and healthy little guy! He is amazingly active and wants to climb things all day long! He took a bit of a spill on his head last week which put us in quite a panic as he hit about 1 cm. beneath his shunt. It swelled up huge and broke a blood vessel. We immediately called his doctor at Children's but they felt all would be OK and they were right. It's been about a week and the bruising and swelling is pretty much gone. His home school and therapies are going OK. I have to say, he is really not interested in doing what his teacher or therapists want him to do --- Jake wants to do what Jake wants to do --- which makes their job somewhat difficult. And to be honest, I don't really feel that Jake has taken to his school therapists as much as I have seen him take to others in the past. I think it's maybe because they don't come as often and their schedules aren’t always the same. Anyhow, it's a bit of an ongoing struggle. I'm really hoping that getting him in school (rather than the comforts of him surrounded by all of his 'stuff') in the Fall will help!

We've gotten a few additional test results from Duke but still not everything. One of the biggest is Jake's immune function is NORMAL --- as in --- like a NORMAL person NORMAL! This means that once he has been off his Cellcept for 2-3 months all should be well as far as Jake being able to go to school and spending time around other children, etc. It's taken almost two years but we're finally back to normal! His enzyme level is in the normal range and he is 100% engrafted with donor cells!!! Jake has carpal tunnel syndrome in the mild to moderate range, worst on the right than the left. Dr. Fitch (Jake's North Carolina orthopedic surgeon) does not believe we need to do anything about ANY of his orthopedic issues right now. He feels we need to retest everything in 6 months and monitor it. Jake has an orthopedic consult here in Seattle with Dr. Song at Children's at the end of May to get a local opinion and I am waiting for an article from the orthopedic surgeon at the University of Minnesota that sees all the Hurler kids there for their carpal tunnel syndrome so we're researching Jake's ortho. issues to make sure we're doing the right thing for him. Still no results from UNC on his development testing --- if I don't get anything from them by next week I'll have to give them a call!

I have been calling pre-schools and setting up appointment times to visit them. I visited and observed a Montessori school (Small World Montessori) on Thursday and really liked it. I have to admit that I get scared thinking about putting Jake there --- I mean the kids that I watched were so active, verbal, and 'normal' --- it scares me a bit to know that Jake won't exactly fit in. On the other hand, I think about these kids and know that if Jake is allowed to spend time with them he'll be able to learn so many things and be forced to utilize skills that he is not forced to use pampered by Steve and I at home! Anyhow, I am going to an Open House tomorrow at a local Waldorf School and I have a couple more Montessori schools to visit. I've discovered that none of these private school options are cheap BUT we do whatever we have to for Jake! I still have to visit the public school as well and I feel like I am running out of time! Ugh! Who knew that you practically need to have your child enrolled the day they are born to ensure a spot in a good class!!! :-)

Well, I think that's all for now. Thank you so much for all your continuing support of Jacob and his family (us!) and thank you for signing his Guestbook. We love to hear from you all!

Love ~ Mel, Steve, Jakie, & Matt-man

P.S. Our wonderful neighbors the Anderson's moved last week. We were so sorry to see them go. Matthew in particular has had a really hard time --- he misses his 'big sister' Alyssa SO MUCH! He can't understand why she hasn't knocked on our door in a week. Keep in touch Mark, Ginger, and Alyssa!!!

Tuesday, March 2, 2004 12:44 PM PST

****NEW PICTURES****

Well, I was trying to wait to get some additional results before I posted but it doesn't look like that's going to happen so you're going to get some results now and some later! :-)

All in all, our trip back to Duke went well. Jacob was SO GOOD on the airplane. I will admit this is probably the part I was dreading the most...8 hours on an airplane with a 3 year old would bring fear to anyone!!! We had 3 across seats on all legs of the journey and my Mom and I took the aisle & window and put Jake in the middle. He was happy to sit there and watch his new personal DVD player (I highly recommend one of these!!!) and snack on pretzels (OK --- lick the salt off of them and give the soggy pretzel to me to dispose of) and even ate (chewed, swallowed, everything!) a quarter of a turkey and cheese sandwich. I felt good because we had Jake somewhat blocked in, surrounded, and protected from 'germ-ridden' people. Also, I would recommend American Airlines because they really do have the extra leg room they advertise.

Our hotel room at the Homewood Suites was OK. I, of course, bought Lysol and wiped all the surfaces down. I just have not been real satisfied with the cleanliness of the accommodations in Durham! They also made me REALLY MAD the last day of our stay as they started 'construction' on our room about an hour prior to our check-out time. We walked in to finish up our packing after our meeting with Dr. K to find a horrible mess, random workmen in our room, and the kitchen floor torn up! I freaked out to no avail but since our return home I wrote a letter to the manager of the hotel as well as the Hilton Corp. who owns Homewood Suites and just this morning received a call from the Asst. Manager apologizing profusely and crediting the credit card for the entire amount of our stay. Of course, my first concern was Jake's health (he can't be exposed to construction sites or excessive amounts of dust, molds, & bacteria) but I will admit to being pretty happy that I actually received results from the letter I wrote. I honestly wasn't expecting anything to come of it!

Now on to the medical stuff...Jake's first day of appointments was HORRIBLE! It was like he knew what was going on the minute he saw the Children's Health Center. He's just too smart for his own good!!! He cried when they took his weight, height and vitals (which of course made his blood pressure look high which it's not!). He screamed, cried, kicked, and thrashed when they accessed his port for his big blood draw. It was horrible and I don't know what happened but the blood got messed up and made his labs look funny so he had to be re-accessed and have another blood draw the next day. He had his pulmonary function test which we thought would go good because he was already crying but he was not letting enough oxygen out so we just did that until his reached the minimum passing of 81 I know this isn't accurate (he was at 96 think last time he had a PFT) but we just weren't willing to push it any further with him --- at least he passed. He cried through the whole echocardiogram even with a Dora video (his ABSOLUTE favorite right now) playing. They never got a good look at his heart because they couldn't see it through his lungs! He did OK with his x-rays, just cried a little --- I think he was so exhausted by then that he couldn't fight it that hard. We then met with a nurse practitioner in clinic, Ann, and she examined Jake. His skin was pink and mottled by then and he was just totally done for the day. She didn't push things with him, thank goodness, and we were out of there. We let Jake relax and have some fun for the remainder of the day!

The next day was Jake's development testing at UNC. I was prepared for the worst as far as his behavior goes but he did really well. He was much more relaxed and calm on day 2! I don't really have any full results from this appointment yet and will post that info. when it comes in. I usually get the report from them about 4 weeks after he is seen. I do have a few 'results' one of those having to do with his valgus deformity (knock-knees). Dr. Escolar believes they are much worse (16cm.) and the shoe inserts (orthotics) aren't going to help any further. He needs to be seen by his orthopedic surgeon and some decisions have to be made in regards to surgery versus bracing/casting. His North Carolina surgeon (Dr. Fitch) is being consulted and we are waiting for an appointment time with an orthopedic surgeon at Children's in Seattle. I also asked a lot of questions this time about Jake's level of delay and his future opportunity in regards to his delays. I have again been told that it really depends on the level of Jake's cognitive delays prior to transplant and we have no way to know what those really were. That said they are seeing the transplanted Hurler kids getting pretty close to 'normal' if not actually getting to 'normal'. Bottom line is that he could be mildly 'retarded' (I hate that word but it's the one that was used in this situation) BUT if we give him every opportunity as far as therapy and the best schooling possible in these early years post-transplant he has every chance for being 'normal' and having an average or even an above average IQ. So...this is what we are shooting for...we will pass this information on to all those that work with Jake and make sure they know that our expectations in this area continue to be very high. After Jake's development testing (4 ½ hours) we headed to Duke for his Nerve Conduction Study (carpal tunnel test). He did great with this. It was a pretty long test (1 ½ hours) but he just laid on the table and watched Dora DVDs while the neurologist hooked him up to electrodes and shocked his little hands, wrists, and arms. We do not yet have results from this test although the neurologist did say he felt Jake did have carpal tunnel syndrome but nothing official yet or the severity of the syndrome.

Day 3 began with the eye doctor. Jake did pretty good here and they were able (finally) to get a good look at his eyes. Jake is far-sighted (he can't see well up close) and needs glasses. His corneal clouding continues to be very mild to non-existent which is good. It turns out the reason he has been crossing his eyes is because he is trying to focus on things up close and not succeeding. Anyhow, I have his prescription and am getting him glasses ordered this week. I was waiting to talk to his audiologist on the best glasses for a child who wears hearing aids so I am now armed with that info. and know what kind of glasses to get. I am somewhat dreading trying to get him to wear them as I just now got him back to wearing his hearing aids full-time. I'm just hoping he realizes how much they help (his prescription is pretty strong) and he wants to wear them so he can see! After that, the brain CT was TERRIBLE...my child from Monday had returned!!! It took two different groups of techs and CT rooms to finally get a good CT after taping him to a table and wrapping his arms so he couldn't move. He was so exhausted by this that we took him back to the hotel room and he slept for quite awhile with Grammie Cindy (who wasn't feeling that well either)!

Thursday morning we met with Dr. K to talk about Jake and go over the information we had thus far. As always, I love meeting with Dr. K and feel so reassured that everything is going OK after these meetings with her. She lowered Jake's Cellcept and we have a schedule to continue weaning it. If all goes well he will be off his Cellcept in June and then we can start to remove some of the other medications. Although, we do not have the results from his immune functions yet, Dr. K felt that in beginning to wean him off the Cellcept and on the assumption that his immune functions will be continuing to head in the right direction...Jake will begin school in the Fall!!! This news was huge and took up the majority of our conversation. Dr. K has a lot of concerns about Jake being put in a special ed. program through the public school. She feels that this could be very detrimental to Jake's future development and believes it is very important for Jake to be around typically developing kids as this is the only way for him to reach his full potential. She recommended possibly a Montessori program where the focus isn't so much on age but on abilities so that has been my 'homework' since we've been home. I have been researching pre-school programs for both Jake and Matt. I do not plan to put them in the same pre-schools and Mattie will only be going twice a week whereas Jake will be going more often so I have been busily trying to figure all of this out. I have some options lined up and now have to set up times to go visit them. We also haven't ruled out the public school if they are willing to put him in their mainstream pre-school class. Anyhow, Dr. K thought Jake looked wonderful and is happy with his progress. She addressed all the questions I had and as always talked to me Mom to Mom where appropriate as opposed to Doctor to Patient. This is very important to me!

Well, the length of this post is already way too long so I'm going to have to say TO BE CONTINUED...

Love ~ Mel, Steve, Jake, & Matt

P.S. My family and friends have been dealing with a lot of not so fun stuff lately... Please send some good thoughts and prayers their way...

My sister, Auntie MoMo, has to undergo surgery to remove some suspicious 'cancer' cells from her body on Thursday. We love you Auntie MoMo and know everything will be fine.

My friend Jessica is supposed to leave for Panama on Thursday to work in an orphanage for 6 months but she is having some strange medical issues. She is being tested for Hodgkins Lymphoma today and we are thinking of her and hoping it's something much more minor.

My Grandma Charlotte is dealing with some unidentified blood cancer symptoms and hopefully the medicine they have started her on will clear everything right up!

Finally, my sister-in-law, Auntie Steph, had to go back to work today after being off on her Maternity Leave. We understand that you're sad but know that baby Alec will be just fine! He is having fun with the other little babies at daycare right now. :-)

Monday, February 9, 2004 5:06 PM PST

FRIDAY, FEB. 20, 2004 --- QUICK UPDATE

Just want to let everyone know that we made it home safe and sound from Duke. Things went OK although we've got a lot of new information to share with you all (Anne - yes on the glasses, but no on the yellow lenses!)! I will be posting a thorough update soon as I need to get it all down so I don't forget everything!!! New pictures and update VERY soon!

21-months post-transplant...

I know...I know...FINALLY…new pictures and an update!!! I finally figured out how to make the photos the right size. They're from a couple months ago but I really wanted to put the Christmas photos up for you all to see. Hopefully I won't have this problem again now that I have it all figured out! ;-)

Jacob is doing really well. He keeps busy (as do we) between school and therapy. He definitely has a mind of his own and really wants to control his environment (I guess that comes from having absolutely no control for almost 3 years of his life!) which does create some conflict with him doing what his teacher and therapists want. He really enjoys the words "NO" and "MINE" when it comes to things he does and does not want to do. He has his favorite parts to all his sessions and the parts he doesn't like so much. He really enjoys doing his exercises with Miss Dana (OT/PT), Buddy Bear with Miss Sue (Speech), art projects with Miss Kristine (pre-school teacher), and all Sarah's (Elks OT) fun Discovery brand toys. For the most part I am happy with his therapy and school although I would like more but until he is allowed to be around other children I don't really think this will be an option. At least they haven't tried to reduce any of his therapy time! Besides his formal therapy we spend time reading books and working on various art projects which he loves. Also, Jake is finally wearing his hearing aids again which is wonderful. He really hated them for awhile and it was almost impossible to get him to keep them in --- now he tolerates them and gives me this VERY suffering look when I put them in his ears in the morning but he doesn't try to pull them immediately out.

The rest of us are doing OK too. I am busily getting things ready for Jake and me to head to Duke on Saturday. Steve and Matt will be staying home so Steve doesn't have to take any time off from work. Grampie Mark is going to take care of Matt on Monday and Grandpa Steve & Grandma Patty are going to spend Tuesday & Wednesday with him. What a lucky boy! My Mom (Grammie Cindy) will be heading back to North Carolina with Jake and I. A special thank you to all the Grandparents for all that you do --- we couldn't do it without you!

Well, wish us luck for Jake's 18 month post-transplant check up at Duke. I'm going to post our schedule as I know some of you (Kelly, Lori, Andria, Keri, Andre', any other 5200 Nurses!, Jen Condron, etc. --- you guys might want to see this as we'd love to see you ALL):

2/14 --- Saturday --- Arrive Durham 10:30PM
2/15 --- Sunday --- no plans (get used to the time change, maybe visit with Stacy!?!)
2/16 --- Monday --- 9AM - lab draw, height & weight, appt. w/ Caryn, NP, 10AM – ECHO, 11AM – pulmonary function test, chest, spine, & hip x-ray
2/17 --- Tuesday --- 8AM – Dr. Escolar (ALL development studies), 2:30PM – Nerve Conduction Study (?carpal tunnel)
2/18 --- Wednesday --- 9AM – Dr. Greene (eye exam), 11:15AM – brain CT
2/19 --- Thursday --- 8:30AM – Dr. Kurtzberg, Depart Durham 2PM

I'll update with all the results when we get home. Take care and ENJOY the new pictures!

Love ~ Melanie, Steve, Jacob, & Matthew

Saturday, January 3, 2004 12:11 PM PST

1/13/04 UPDATE --- The pictures are coming...I promise! It looks like I'm going to have to buy new photo software or upgrade my old software to make the pictures do what I want them to do. Hopefully, if I can get to Costco, this will happen some time this week. I want to look and see what they have because they usually have the best prices. Anyhow, the pictures are coming soon! A quick Jake update...he is doing well. Still dealing with dry skin/rash, but we're used to that! He started back to 'school' after the Holiday Break and seems to be happy getting into the old routine. I've been busy getting us scheduled to head back to Duke. It looks like (if we don't get a private flight) we'll be flying out Feb. 14TH and flying home Feb. 19TH. Take care, Mel :-)

UPDATE 1/5/04 --- I know you all are waiting for the pictures...I'm having a little trouble getting them sized right to fit the page. I'm trying to learn my new camera and software! Anyhow, I will get them on the page as soon as I can figure out how to make them small enough (right now they take up the entire page!). Sorry for the delay! Mel :-)

Happy New Year! And so 2004 begins...

The past couple weeks have been very hectic around our house but fun nonetheless. We made it through Christmas Eve and Christmas Day and I think the boys loved it. This is the first year either of them really knew what was going on. We tried to make it as fun for them as possible and believe me, Santa (and Daddy & Mommy AND Grammie Cindy & Grampie Mark AND Grandma Patty & Grandpa Steve AND their aunts & uncles, etc.!!!) were VERY good to them. Matthew and I ended up going to my families Christmas Eve Party for about an hour to say hello and exchange gifts with my cousins' kids and then hurried home to spend the remainder of the evening with Steve and Jake. Christmas morning dawned bright and early (OK, OK Steve and I were more excited than the boys and ended up waking them up!!!) and I think I got some good pictures (with my new digital camera :-) Merry Christmas to me from Steve!) of the boys on their new John Deere Gator...way to go Santa! We then headed off to my parents for the morning and early afternoon and then to Steve's parents for the rest of the day --- they only live about 10 minutes from each other and 25 minutes from us so it's not tough to do both. Honestly, the boys really weren't that interested in opening their gifts. Jake just liked tearing off the wrapping paper and Matt just wanted to play with whatever came in the box. They actually ended up watching their new Dora DVD and running in and out of the rooms for the majority of the day. They also had fun playing with their cousin Nathaniel and their new baby cousin Alec Tristan. We were very cautious of everyone's health and thankfully no one was ill so we were able to join in most of the festivities with Jacob. Anyhow, by the end of the day the boys had absolutely had it --- I think Matthew was asleep by 6:45pm and didn't wake up until morning --- on the other hand Jake has become quite the night owl and wants to take evening cat naps so he can stay up all night! We're working on that one!

Steve and I have been able to get out twice by ourselves since Christmas...can you believe it!?! We were able to go see a movie and do some shopping while my parents watched the boys and then Steve's parents decided to come over on New Year's Eve so we could go out and celebrate the arrival of the new year with Chris & Rene' (Steve's brother and his wife). We had a good time both evenings and have enjoyed having some time for ourselves as that seems to happen so rarely when you have kids --- especially with medical issues! The boys, of course, have LOVED spending time with their grandparents.

Jacob's health has been pretty consistent through it all. He was a bit under the weather for a couple of days this past week as was Matt but nothing severe. We monitored it closely as we are very fearful of the flu this time of year but I think they just picked up a little virus and were both able to fight it off with no problems. Jake's labs look great. He got his IVIG, Pentamadine, & Synagis infusions yesterday so he's all protected for another month. We are scheduled to go to Duke the week of Feb. 16TH and I think the big issue we will need to address with Dr. K is Jake's on again off again rashes. Is it really GvH? Is it time we find out by a skin biopsy? Dr. Hawkins is not convinced that it is GvH and we are a family of sensitive skin so we aren't convinced either. I'll admit that Steve and I are ready to start to try to wean Jake's Cellcept (MMF) again and maybe drop down on his steroid but we've been at a standstill for months because of the rashes. Hopefully we'll be able to figure it out. If any of our transplant friends or nurses are going to around Durham that week, please let us know, we'd love to see you all! I actually think it will be my Mom, Jake, and I heading back East. Steve is going to stay home to work and we're going to try to line up baby-sitters for Matthew for the week. We'll see how that goes!

Well, I think that's enough for now. I need to try to get this house cleaned up and all our Christmas decoration safely put away until next year. I think my boys are going to be spending the weekend watching football --- Go Seahawks --- that's for you Horton Family (www.caringbridge.org/il/jordan <--- Packer's Fans)!!! :-) Take care; check out the new photos, and once again --- Happy New Year!

Love ~ Mel, Steve, Jakie, & Matt-man

Monday, December 22, 2003 6:39 PM PST

Just a quick update to let you know that all is well with Jake. He has avoided the flu and all the other illnesses that seem to be floating around thus far and we HOPE this continues. We will be spending a low key Christmas Day with our immediate families as we need to keep Jake isolated again this year. Hopefully next year Jake will be doing better in regards to his immune system and we'll be able to join in the festivities with the extended family and friends on Christmas Eve!

We are hoping you all have a wonderful Holiday! This time of year can be difficult for some of our friends who have lost their loved ones and we want you to know that our hearts are with you. Thank you for continuing to check in on Jacob!

Merry Christmas and Happy New Year!!!

Love ~ Melanie, Steve, Jaocb, & Matthew

Saturday, December 13, 2003 9:28 PM PST

19 months post-transplant...

I finally forced Dell to send me back my computer so I am able to update Jacob's site. Long story short...I got my computer back unfixed and am going to have to figure out what I am going to do about it...return it all together or wait months for it to be repaired. Needless to say, I have not been real happy with Dell Technical Support!

Anyhow, all is well with Jakie. He has just been getting better and better in our opinion. His development continues to amaze us. He is talking so much and becoming more and more understandable to everyone, not just us. He is growing in both height and weight (he's FINALLY hit the 30 lb. mark!) and I have been able to box up all of the boys 2T and smaller clothes which is a huge step because Jake has been wearing the same size for a year and a half. His health has been pretty steady. He continues to have very mild GvH symptoms off and on so his doctors have chosen to keep his medications the same. This means he will continue to be immuno-suppressed for many months to come but if this is all we have to deal with right now then no big deal. We are keeping Jake more confined due to the fear of flu and other colds that everyone seems to have right now. This makes it a bit difficult to get stuff done (grocery shopping, running errands, Christmas shopping etc.) but we manage --- Steve and I spend a little less time together because we're always running off doing something when the other is at home with the boys!

I updated the photos. Hope you enjoy them!!!

Have a wonderful Holiday...we plan to! :-)

Love ~ Mel, Steve, Jakie, & Matt-man

Thursday, December 4, 2003 1:41 PM PST

Our computer has been out of commission for several weeks and I have been unable to access my e-mail and Jake's website as well as all the other kids websites I keep track of... I am hoping to have my computer back in the next few days and will do a more proper update at that time. I just want to let everyone know that Jacob is doing wonderful. All is well here in the Irish household...busy preparing for Christmas. Take care and as I said I will do a better update and new pictures very soon.

Love ~ Mel, Steve, & Jake, & Matt-man

Sunday, November 2, 2003 6:49 PM PST

As promised...here's a Jakie update & new pictures in the photo album!

Honestly, there isn't really much new to report on Jake. He is plugging right along...just like any other little guy. His health has been pretty good. He continues to experience skin rashes off and on which may or may not be GvH symptoms. Because the doctors are unsure of what the rash is, we have continued his medications at the current doses. The consensus at this time is to err on the side of caution as far as the medications go which is just fine with Steve and me. The last thing we want is to cause Jake any medical complications because we are in a hurry to get him weaned from his medications. The only adverse effect the medications have on Jake is that he continues to be immuno-compromised which means we still have to remain somewhat confined. Needless to say, we are all getting flu shots in the hopes of keeping ourselves somewhat healthy and in turn keeping Jake healthy through the cold and flu season.

The only other thing on our agenda has been preparing for the holidays. The boys had a wonderful Halloween and were so cute in their Elmo (Jacob) and Winnie the Pooh (Matthew) costumes. We even took them trick-or-treating to a few of the homes in our neighborhood. I think they really enjoyed the idea of getting candy by just knocking on a door. I'm afraid they might want to go every night! :-)

We hope you all are enjoying the Fall (although it feels more like Winter here!) and the beginning of the Holiday Season.

Love ~ Mel, Steve, Jakie, & Matt-man

*******BE SURE TO CHECK OUT THE NEW PHOTOS*******

Thursday, October 30, 2003 2:47 PM PST

I received an e-mail message from our friend Anne Himes, Andrew's Mommy, and I wanted to post the message on Jake's site. Andrew has Hurler's like Jake and was transplanted at Duke. His website is www.caringbridge.org/page/andrewh. I've seen this quote in the past but it really touched me this time and I think it's a wonderful way to explain things...

________________________________________________________
WELCOME TO HOLLAND

by Emily Perl Kingsley

I am often asked to describe the experience of raising a child with a disability -- to try to help people who have not shared the unique experience to understand it, to imagine how it would feel. It's like this:

When you're going to have a baby, it's like planning a fabulous vacation trip -- to Italy. You buy a bunch of guidebooks and make your wonderful plans. The Coliseum. Michelangelo's "David." The gondolas in Venice. You may learn some handy phrases in Italian. It's all very exciting.

After months of eager anticipation, the day finally arrives. You pack your bags and off you go. Several hours later, the plane lands. The flight attendant comes and says, "Welcome to Holland."

"Holland?" you say. "What do you mean, Holland? I signed up for Italy! I'm supposed to be in Italy. All my life I've dreamed of going to Italy."

But there's been a change in the flight plans. They've landed in Holland and there you must stay.

The important thing is that they haven't taken you to a horrible, disgusting, filthy place full of pestilence, famine and disease. It's just a different place.

So you must go out and buy new guidebooks. You must learn a whole new language. And you will meet a whole new group of people you would never have met.

It's just a different place. It's slower-paced than Italy, less flashy than Italy. But after you've been there for a while and you catch your breath, you look around and you begin to notice that Holland has windmills, Holland has tulips, Holland even has Rembrandts.

But everyone you know is busy coming and going from Italy, and they're all bragging about what a wonderful time they had there. And for the rest of your life, you will say, "Yes, that's where I was supposed to go. That's what I had planned."

And the pain of that will never, ever, ever go away, because the loss of that dream is a very significant loss.

But if you spend your life mourning the fact that you didn't get to Italy, you may never be free to enjoy the very special, the very lovely things about Holland.
_________________________________________________________

I'll post an update on Jake and some Halloween pictures this weekend! Take care...

Love ~ Melanie, Steve, Jacob, & Matthew

Sunday, October 5, 2003 2:00 PM PDT

One year ago we arrived home (or "home home" as some of our transplant friends call it!) from North Carolina and entered our more local phase of post-transplant life. I can't believe it has been a full year already...sometimes it seems like it was yesterday, other times it seems like a lifetime ago!!!

Jacob is doing really well. He has started school in our home and seems to really enjoy his teacher and new therapists. The insurance company has continued to deny further coverage for private therapies, so although I am not at all thrilled about this we are making do with what we have at the time. Jake's teacher, Kristine, is so sweet and kind to both Jake and Matt and Sue (Speech) and Dana (OT/PT) are really working hard with Jake and introducing some new ideas and things to him. Kristine is trying to introduce structure with "circle time" and a specific order to how her time with him is spent and he is doing surprisingly well with this concept although he does want to get up and run around still somewhat. We are also enjoying our once a week visit from the Elks therapist Sarah. She is helping Jake with a lot of his functional skills which I am thrilled with. She is working on teaching him how to dress himself and get up and down the stairs which are areas that would make my life much easier!

Jacob has FINALLY started to show more of an interest in eating and SWALLOWING his food. Although, he is not anywhere close to where he should be for his age he has started to actually swallow a few small bites of food at each meal which is a huge improvement. Maybe just the threat of feeding therapy made him start to figure it out on his own!

Jacob's physical health is good for this stage of his transplant except for the skin rashes. The poor guy is still fighting mild skin GvH but it is enough to keep him on immuno-suppresents with no sign of coming off anytime soon. This will continue to keep us somewhat confined especially as we get into the cold and flu season. As a matter of fact, Matthew currently has Croup and I caught it from him and mine is a terrible cold and laryngitis. It is not fun at all and we are just hoping that Jake stays healthy and doesn't catch what we have.

We hope all is well with you and your families. We continue to keep track of all the friends we have made since Jacob's diagnosis and subsequent treatment and wish you all the best.

Love ~ Melanie, Steve, Jacob, & Matthew

Tuesday, September 2, 2003 9:40 AM PDT

15 months Post-Transplant........

So sorry for the time between updates, August has been a very busy month for us. And, to be truthful, it has felt almost normal with Jake being such a typical 3 year old during the Summer months and Matthew reaching that big 2 year old mark! We actually started the month with a small family birthday party for Jake. We wanted to do something to mark the momentous milestone for Jake but as he is still immuno-compromised we couldn't do much. We had a very enjoyable afternoon with Grandmas, Grandpas, Aunts, an Uncle, and a couple Cousins who all doted on Jake as it should be on his big day! We made sure everyone was healthy and thankfully Jake absolutely enjoyed being the center of attention.

Steve spent 4 days on a fishing trip to the Canada/Alaska border with his Dad, Brother, & Grandpa. They didn't have much luck catching anything unfortunately so came home a bit disappointed from there. A few days after Steve's return we all took off for 4 days at Ocean Shores on the Washington Coast for a family reunion on Steve's side of the family. Steve and I were a tad anxious about how Jake would react to the beach. The last time we took him to the beach was prior to his transplant in North Carolina and he freaked out. He wouldn't touch the sand AT ALL even with shoes on his feet and he screamed if we got him anywhere near the water so we didn't know what to expect! Well, what a changed boy we have. He LOVED the beach!!! He ran around in the sand with shoes on and OFF. He sat in the sand and played with a shovel and bucket. And, he went to the water with the waves crashing in and waded out to his thighs with his Grandma Patty. It was so wonderful to see...you can't imagine how happy Steve and I were by this. The reunion was held outdoors and there were only a few children there. We tried to keep Jake away from areas where there were groups of people which was really no problem as there was a huge field where he, Matthew, their cousin Nathaniel, and Steve's cousin's daughter Rylee ran around. All in all it was a wonderful trip, especially for Jake and Matt!

Matthew turned 2 on August 23RD. We had a very special day planned just for him. My Mom and Dad watched Jake for us and we took Matt-man to the Evergreen State Fair in Monroe. He had a great time seeing all the animals, watching people, eating junk food, and topping things off with a pony ride. He is still an inch too short for the kiddie rides although I think they would have let him go on them, Steve and I aren't quite ready for that yet. Frankly, we don't think Matt is ready for them yet either. As crazy as Mattie can be he has a somewhat serious nature and gets frightened by things pretty easily. One minute he is fearless and the next minute he is hiding his face in your chest because something scares him! HAPPY 2ND BIRTHDAY MATTIE!!!

We celebrated two other birthdays in August as well... HAPPY 4TH BIRTHDAY TO COUSIN NATHANIEL (Aug. 21) and HAPPY BIRTHDAY TO GRANDMA PATTY (Aug. 24). Aunt Stephy (Matthew has started calling her that...how cute!) and Uncle Ben had a party for Nathaniel at their house. I went back and forth about taking Jake as they expected a few kids there. I finally relented when we heard the weather was going to be nice and it would be outside (Dr. K. has told us it is much safer for Jake to be around people outdoors as opposed to indoors) although I have to say that between Steve, me, and Auntie Ne' we kept Jake pretty close to us and away from the other kids.

Finally, the end of the month saw us sending off Grammie Cindy for a 3 week trip to Italy! We have heard from her several times and they are having a wonderful time seeing all kinds of historic things and doing all kinds of fun stuff. She went with my Aunt Sharon and another women friend of my Aunts'. We miss her and can't wait for her to get back so we can hear all about it. We're taking care of Dad for her while she's gone (he'll love that!). I even took him to Costco the other day so he could get some things for their house. :-)

Jake's medical stuff this past month hasn't been quite as fun. Jake was assigned a new case manager at our insurance company and they made the decision after our return from Duke to deny coverage for Jake's home therapies. We were absolutely devastated to get this news as we know this is the only way to ensure that Jake reach his full potential. He had such a wonderful rapport with Cara (PT), Mindi (OT), and BJ (Speech) and we have missed them tremendously. They fit so well with Jake's temperament and our family dynamics and when you have someone coming in your home this is so very important. Anyhow, I gathered letters and data from all Jake's doctors both here and in North Carolina and have formally appealed the insurance companies decision. I have yet to hear back from them although we got a letter saying they would get back with us soon. In the meantime I have taken on the therapy responsibilities and am doing the best I can. I think I am doing fine with the PT and OT stuff but the Speech stuff takes a skilled therapist in my opinion and I am very worried he is going to fall even further behind if I don't get someone in here soon! In saying that...Jacob starts school this week at Lakewood Elementary School...in our home as he is not released by his doctors to be in that type of environment yet. He will receive 90 minutes of education, 60 minutes of Speech, and 90 mintues of OT/PT per week so at least he will be getting about half of what he needs. We've also spoken to the local Elks and they keep a pediatric therapist on staff that comes to the kids' homes to do therapy. She is an OT and has offered to come out once a week which will be great as well. I've just got to figure out a time with her.

Jacob also had an Upper GI about a week ago. I think I mentioned he was going to have one in my past journal entry. It was to rule out a stricture or web (blockage). If he had something like that it could explain his strange eating habits of sucking the food but not actually swallowing it. The test was not fun at all for Jake. He did not like the barium he had to swallow and he really did not like lying on the x-ray table under a giant camera and being held down but we got through it and there is no blockage present and no signs of reflux. Everything is clear and the same as it would be in any typical 3 year old. So...Jake's lack of eating is behavioral and we have to now approach it as such. For the time being I am too continue to offer him meals and just hope he starts to eat more. I think this will be an issue we address once again back at Duke in January.

I've got a couple other test results from our July trip to Duke but still don't have the formal report which will include everything. Jake continues to be 100% engrafted with donor cells and his enzyme level is at the high end of normal. These are the most critical things and we are thrilled with his results.

Jake has had rashes on and off throughout the past month so we have tried our hardest to keep him smothered in ointments and creams. We fear he is exhibiting some mild GvHD symptoms so we have not taken him off his prednisone yet and are slowing the Cellcept taper down. This will keep him immuno-compromised for longer BUT the alternative of a more severe GvHD is not even worth thinking about! So for now Jake will continue to be somewhat confined from the outside world and as the Summer turns to Fall and weather gets cooler (we're going on two months without rain here in Washington State and I for one am hoping for some!!!) this confinement will be even more necessary. I am dreading cold season this year and am just hoping I can keep my boys healthy!

One last big milestone to mention...the boys are out of their cribs (well, Jake's out of his crib...Matt's out of our bed) and into toddler beds thanks to Grandma Patty & Grandpa Steve. Both a fire truck bed and a race car bed arrived on Matthew's birthday. They are so cute! For the time being, Jake is in the fire truck and Matt is in the race car although I might switch them around. Jake sleeps in his all night although his naps are a bit shorter during the day now. Matthew is going to be a bit tougher I think but we're gradually working up to a full night in his new "vroom vroom" as he calls it!

Thank you for continuing to check in on Jacob! As always, we appreciate your support more than you can imagine.

Love ~ Melanie, Steve, Jacob, & Matthew

NEW PICTURES ARE POSTED!!!! CHECK THEM OUT!!!

Friday, August 1, 2003 9:56 AM PDT

NEW PICTURES --- NEW PICTURES --- NEW PICTURES --- NEW PICTURES

First & foremost we must THANK Corporate Angel Network and The Boeing Company for providing us a one-way flight from Seattle to Washington, DC. I can't tell you how comfortable and safe I feel about having Jake on a private airplane and in private hangars with no concern for germs and random illnesses that people might be harboring. The Boeing flight crew were absolutely wonderful...thank you so much to Bonnie, John, & Don!!! The executives on board, Fiona & John, were so nice and everyone was so interested in Jacob and understanding of his needs (see photos!). Once again, I must say this Mommy was very spoiled as well --- this is the ONLY way to fly in my opinion --- I guess I should have been a rock star or something!!! :-) Speaking of the famous, MEL GIBSON, yes, I said MEL GIBSON passed within two feet of me at the private airport in DC. Can you even believe it!?! He looked right at me and did I say anything? NO --- for once in my life words escaped me!!!! Not even, "can I have your autograph," I pretty much just stared at him with my mouth hanging open ---- real smooth on my part!!! :-) In my defense, Fiona was standing right there as well and she didn't say anything either --- although she definitely didn't look as 'starstruck' as I did!

Our friend Stacy also deserves a huge amount of THANKS not only did she drive us all the way from DC to Durham but she did this with a crying Jake for just about the entire time. There was no rhyme or reason to the crying but it was pretty constant and I'm sure as troubling to her as it was to me. I think he'd had it for the day and was letting us know! We didn't end up arriving in Durham until 3AM and were all exhausted! We were up by 7AM with a tired Stacy heading back to DC and us hurriedly trying to get to an 8AM CT scan!

Anyhow, two of Jake's wonderful nurses from 5200 joined us for most of Jake's appointments Monday morning and it was so nice to see them and great to have someone to chat with as we waited! Jake's brain CT went well (no sedation necessary) and the results look good as well. It looks like all of the chemo and steroid related swelling is gone and his shunt is functioning perfectly. Chest x-ray is clear. We have some, but not all, of the blood draw results back and everything looks pretty good. His cortisol level is low which we expected and means he will continue on the steroids for the time being and one of his white blood counts is very slightly elevated which could be a sign of chronic GvH. I don't really want this to be the case and at this point we'll just need to monitor it and him to watch for any further GvH signs beyond what he already has with the skin rashes that come and go. The fact though is that Jake has shown GvH symptoms since his transplant, some more severe than others, we're just hoping that if this is chronic GvH it is a very mild form. His pulmonary function test went great and his lung capacity is up to 94% from 92% in February.

After a nap back at our hotel room we headed out again to visit with our old Nurse Practitioner, Jen. It was so nice to see her as we just love her dearly. She thought Jake looked amazing which was wonderful to hear! We also got to visit with Maddy Wigglesworth and her parents, Kym & Craig. We have been e-mail correspondents since Jake's diagnosis so it was very nice to finally meet in person. Maddy is 4 years post-transplant and looks amazing. She is going to be entering mainstream kindergarten in the Fall and gives me such hope for Jake to do the same. We ran into the Wigglesworth's throughout our stay as Jake and Maddy's appointments seemed to correspond to each others.

Tuesday began at UNC for Jake's development studies...Jake spent 5 HOURS being tested and observed and in my opinion he handled it like a trooper! We do not have any official results yet but unofficially they all thought Jake looked great and has definitely made many developmental advances. They are concerned about his eating or lack thereof and want us to pursue this further. Dr. K wants us to hold off for another 6 months and relax about it so that's what we're going to do. Even with the advances Jake has made he is still significantly behind especially in the gross motor skill area (equivalent to about 17 months of age) because of his balance and strength issues. Cognitively he seems to really know what's going on (21 – 27 months of age), it's just a matter of getting his body to do what he needs it to and of course getting everything up to where he should be at his true age of 36 months. Jake saw Dr. Fitch, the orthopedic surgeon in the afternoon. Jake's kyphosis is the same at 40 degrees so there is still talk of surgery in the next couple years. His hips look good. He has a valgus deformity to his knees (knock-kneed) which we've been aware of since he began walking independently in October. This is why he wears orthotics in his shoes. There is no intervention necessary at this point. There is a surgery than can be done about 2 years before he stops growing (at about age 16) where they snip a couple growth plates and it straightens his legs out so this is another issue we will discuss at a much later date. There is a possibility that his transplant might fix these issues but no guarantee that the enzyme can repair the bone damage that had already been done pre-transplant. After Jake's appointment with Dr. Fitch we headed to Clinic for an exam by Jake's new Nurse Practitioner, Caryn, in preparation for his Dr. K appointment on Wednesday.

Wednesday began with an eye exam that Jake absolutely hated. I have never seen him so resistant to a doctor in my life and Dr. Greene was a very nice man...Jake just did not want him anywhere near his eyes. He basically screamed and said NO the whole time! Dr. Greene said Jake's corneal clouding was clear to mild --- basically normal with maybe a slight cloudiness around the edges which is great. He tried to get a picture of Jake's eyes so he has something to look at in the future but that was not happening! I think poor Maddy could hear him screaming the whole time as she was in the waiting room and then she did not want to have anything done either. Poor Dr. Greene! Jake then had an ECHO which he laid perfectly still for. We have no results on this yet. We headed up to the temporary Unit on the 9th floor (while 5200 is be remodeled) and visited with Andre --- another of our nurse friends. We love him and can't wait for him to come visit us in Washington in the Spring! Then we headed over to meet with Dr. K which was great as always. Jake loves playing with the toys in her conference room and pretty much let us discuss everything thus far. Dr. K made some medication changes and planned some taper schedules --- yes there is a light at the end of the medication tunnel!!! Due to his medications and GvH issues he continues to be on immuno-suppresents and probably will be for the next 6 to 9 months which means he will continue to be confined for at least this amount of time. No major public outings yet but soon, I promise. I have to say we've been enjoying the nice weather because Jake is able to be around people in outdoor setting much more safely than indoors! One thing Dr. K recommended that Jake will get done here in Seattle is a barium swallowing study to rule out any physical abnormalities that might be causing his inability to swallow food. He has had a regular swallowing study in the past, the barium will look further down in his throat to see if there's a stricture or web preventing swallowing. Jake saw Dr. Hulka after this (his ENT) and he checked his tubes which look great. Jake had some wax build up which Dr. Hulka removed but all in all said everything looked good from his standpoint.

Jake and I made it to the airport to catch our commercial flight home. I have to say that I love the Raleigh/Durham airport because it is so empty, especially at 8PM. We were on a full flight from Raleigh/Durham to Memphis but Jake and I had the front two seats right behind first class and I felt pretty good about this. Jake did very well on this leg of the flight. Memphis to Seattle was not so great. We were stuck in a three across row because that's all there was on this plane. Thankfully, our seatmate was a very nice girl who was happy to give me her medical history (yes, I'm a little crazy!). All was going OK until Jake vomited ALL OVER his seat, mine, & a bit on the girl next to us!!! Ugh!!! With no help from the flight attendants, I cleaned everything up and begged a nice man up in first class for his blanket so I could cover Jake's wet seat. Jake rested fitfully for the next 4 hours until we landed at SeaTac. The stroller did not make it from Memphis until three days later so I lugged Jake and our carry-ons to the baggage claim area where Steve and Mattie were anxiously waiting for us. Let me tell you I was VERY happy to finally see them!!!

All-in-all, everything went fairly well at Duke. I wish we would have had more time to spend with friends but that is not the nature of our trips to Duke. Maybe when Jake's immune system is stronger we'll be able to spend some more time there and maybe act a bit like tourists but for now we are there for medical reasons and that is what we focus on. Dr. K says we will be thrilled with the progress Jake is going to make in the next 6 months with his therapies and just overall as this continues to be a very critical time post-transplant. We will be heading back to Duke again in January.

I'm so sorry about the length of this update but I wanted to get everything on here from our recent visit. More results will be coming over the next 3 to 4 weeks and I'll be sure to include those as we get them. Take care and thank you all for your continued support to Jacob and us!

Love ~ Mel, Steve, Jake, & Matt

P.S. I almost forgot to mention that although Jake's weight has stayed about the same he has grown about 5 cm (2 in.) since Februaury! This is a huge step as most Hurler kids do not grow in height at all --- they tend to have characteristics of dwarfism --- so we are seeing one more sign of his transplant working and that enzyme getting in there and doing it's job!

Tuesday, July 29, 2003 2:04 PM PDT

I PROMISE I will get an entry up in the next day or two with all the info./results from our Duke visit!!! But first...I need to put this up...

HAPPY 3RD BIRTHDAY JACOB!!!

Today is Jacob's big day and we are trying to have as much fun as possible ALL DAY (a trip to the park, a swim in the pool, running through the sprinkler, etc.). We will top off his special day with a trip through the McDonald's (Jake's favorite!) drive-thru!!! No big party this year but we'll have a little celebration with our immediate family on Sunday. I'm hoping next year we'll be able to have a big party with tons of kids running around...we'll have to wait and see though...

HAPPY, HAPPY 3RD BIRTHDAY to our BIG BOY, JAKE!!!

We love you ~ Mommy, Daddy, & Matt-man

Wednesday, July 16, 2003 11:06 PM PDT

Well, it looks like we finally have a firm (I use that term lightly!) schedule of what our next week is going to entail…

Corporate Angel Network came through for us on one leg of our journey which we greatly appreciate. It did make for a bit of rearranging on my part today but no big deal as long as we make things safe and easy for Jake. Jacob and I will be flying from Seattle to Washington, DC on Sunday at noon with The Boeing Company (Thank you to Boeing!). We arrive in DC at 7:40pm EDT and our WONDERFUL friend Stacy will be picking us up and driving us ALL the way to Durham. She is absolutely one of the best people in the world and we can’t begin to tell her how much we appreciate all she has done for us over the past 14 months!!! Anyhow, approximately 4 ½ hours later (between midnight and 1AM) we will arrive in Durham, pick up our rental car from the airport, and head to our hotel (Candlewood Suites). Wonderful Stacy is going to stay the night (or what's left of it!) with us and then head back to DC Monday morning for work...isn't she the greatest!?! And Jake's appointments begin...

Monday 7/21 –
8:10AM – brain CT, chest & spine x-rays, BIG lab draw
11AM – pulmonary function test

Tuesday 7/22 –
8AM – development tests (Dr. Escolar & team at UNC)
3PM – orthopedic surgeon (Dr. Fitch)

Wednesday 7/23 –
8:15AM – eye doctor (Dr. Greene)
9:30AM – height & weight check
10AM – echocardiogram
12:30PM – meeting with Dr. Kurtzberg
4PM – ear, nose, & throat (Dr. Hulka)

Probably between our meeting with Dr. K on Wednesday and Jake's appointment with Dr. Hulka we'll check out of the hotel. Immediately following Dr. Hulka's appointment we head to the airport to drop off the rental car and catch a 7:50PM flight from Raleigh/Durham to Seattle with a brief stop and plane change in Memphis to arrive home EARLY Thursday morning (12:30AM PDT!). What a whirlwind!!! Somewhere during all of this we are going to visit with some families we have 'met' along the way and some nurses and other Duke staff we miss and can't wait to see again!

Meanwhile, back in the state of Washington, Steve and Matthew will be camping in Eastern Washington with Grandma & Grandpa Irish, Cousin Lauren, Aunt Steph, Uncle Ben, & Cousin Nathaniel. Fun in the sun for the boys...be careful near the water Matt-Man...Mommy loves you and will miss you!

Wish us luck on our journeys!

Love ~ Mel, Steve, Jakie, & Matt-Man

Tuesday, July 8, 2003 10:21 AM PDT

I want to let everyone know that if they get a chance today --- go buy a Wall Street Journal or get your hands on a copy (Tuesday, July 08, 2003). There is a wonderful front page article on our friends the Birmingham's and their story. For those of you who don't know, their daughter Molly was diagnosed with Hurler Syndrome and went through an unrelated umbilical cord blood transplant. Their story mirrors ours in so many ways it's amazing! I went out to our mailboxes first thing this morning and 'borrowed' our neighbors' paper so I could read it (I returned it within 15 minutes!). I was, of course, quite emotional by the time I was finished --- it is very difficult to relive those emotions and the feeling of uncertainty that we still feel at times about Jacob's future. We want to thank the Birmingham's for once again sharing their story with the world. It is articles like this that help bring more awareness to these rare diseases like Hurler Syndrome.

The second Yard Sale attempt was thankfully much more successful than the first! We even sold Steve's pool table which, believe me, took up a lot of space. There's a chance we may actually be able to park a car in the garage!!! :-) There will be one more Yard Sale/Fundraiser before the Summer ends at my sister's house in Everett. I will be sure to post that one as well so if you're in the area you can check it out. If you're unable to attend but are interested in making a monetary donation, please feel free to follow the link at the bottom of this page for COTA or contact Jake's fundraising chairperson Cindy Wellman (my Mom) at 425-252-2066. Jacob's medical treatment is ongoing and the financial aspect of it does not end and frankly does not appear to have an end anywhere in the near future. We appreciate this more than we could ever say...

Speaking of expenses, we finally have a confirmed schedule for our 'trip' back to Duke. Jake has appointments scheduled Monday through Wednesday, July 21 through 23. I have calls into both Corporate Angel Network and the travel agent they recommend to hopefully find us a flight. Obviously, we are hoping Corporate Angel Network will be able to find something for us but if not the travel agent is usually able to find the best price and we don't have to pay for it until 48 hours in advance. As of right now it will be just Jake and I traveling this time but it's only for a few days so no big deal! I'm also hoping we'll get to finally meet the Wigglesworth Family in person as they will be there for Maddy's 4 year post-transplant check up! They were very instrumental in getting us connected with Dr. K at Duke and we will forever be indebted to them.

We hope you all are enjoying your Summer as much as we are! Jacob is actually able to experience a somewhat 'normal' summertime routine and for this we are so thankful!

Love ~ Mel, Steve, Jake, & Matt

P.S. Remember to check out the Wall Street Journal for today, Tuesday, July 08, 2003!!!

Tuesday, June 24, 2003 8:04 AM PDT

***NEW PICTURES --- updated Thursday (6/26/03) AM***

REMINDER:

YARD SALE/FUNDRAISER ---- YARD SALE/FUNDRAISER
Saturday, June 28, 2003
10AM-4PM
Our Driveway -
4030 143RD ST NW
Marysville
***Can't wait to see you here***

Did I say things had been BORING in my last post??? Someone remind me never to do that again!!! :-) The day after that post one of two things happened...either Jacob's body decided it DID NOT like the wean to every other day on his steroid OR he had some sort of stomach virus. Needless to say, after about 4 hours of vomitting we finally got a big steroid dose in him per Dr. K (yes, basically I call her any time day or night --- she is there for Jake 100 percent) and the vomitting stopped. He was pretty weak and lethargic for the next day or two and he had an issue with his equilibrium which was quite scary (not fun at all to watch your child stand up and immediately topple over backwards!). Anyway, all is back to 'normal' now although he is on a higher steroid dose again...still every other day but 3 mg. as opposed to 1 mg. Oh-well, we'll just try the taper again!

Jake's therapies are going great. He continues to receive two, hour to hour and a half, sessions per week from each of his therapists through Rehab Without Walls in our home (180 min. Speech, 180 min. PT, and 120 min. OT per week). He is showing such improvement in ALL the areas of his therapies...it is just wonderful to see. His vocabulary is huge --- he can say almost anything (if he wants to!) it might not be as clear as other children his age but if you really listen you'll know what he's saying. He plays with playdoh and colors. We're working on stringing beads although he isn't quite there yet. He's doing obstacle courses to improve his gross motor abilities and is walking better all the time. We're trying to get him to sit cross legged which is his latest challenge. He DOES NOT like to feel 'tippy'!!! In September he will begin school at Lakewood Elementary School, although for the time being it will be in our home, as soon as Dr. K says he can - he will be going to the school. While it's in our home he will get 90 min. per week Education, 90 min. per week PT/OT combined, and 60 min. per week Speech. We will continue to supplement this with his Rehab Without Walls therapists as the school cannot meet Jacob's therapy needs fully. We are just praying that our insurance continues to pay for the therapies as it is VERY important to Jake. Dr. K says it is the only way Jake will reach his full potential and possibly/probably be able to mainstream into a 'normal' classroom by Kindergarten. This is absolutely what we are shooting for!

Well, this post ended up being longer than I had planned... I truly hope all is well with everyone reading Jake's updates. We appreciate you!!!

Love ~ Melanie, Steve, Jacob, & Matthew

Monday, June 16, 2003 10:56 AM PDT

Thank you all for continuing to check in on Jacob and for sending us your good thoughts and prayers. We appreciate it more than we can ever say!

The Garage Sale/Fundraiser didn't go as well as we had hoped. We did it on the day of our area-wide Garage Sale thinking this would be good because there would be more people up here. Unfortunately, I think people were overwhelmed by the amount of 'stuff' to be had! Also, we live back on a cul de sac and I think that deterred people from driving back here when there was so much going on on the main road! Anyhow, we have so much stuff still that my Mom and I will be doing another Garaqe Sale here on June 28TH and then one at my sister's house some time in July. I want to thank my Uncle Glennie for all the stuff to sell to help raise money for Jacob's medical expenses. I also want to thank my wonderful friends Karin and Gina for spreading the word about the Garage Sale and then coming up here and spending most of the day keeping us company and helping out with the kids and moving stuff back into the garage!!! You are both wonderful friends!!! Anyhow, better luck next time we hope.

Jacob is doing really well medically. Actually, since he had his port placed and his hernia surgery things have been quite boring and in our 'world' boring is GREAT! The weather has been pretty hot and sunny here which makes it a little tough to keep Jake safe from rashes and sunburns. We've been chasing him around with SPF 45!!! He has a bit of heat rash under his armpits and some exzcema/dry skin around his mouth. We are keeping him moisturized and trying to ward off the dreaded GvH rash that can occur with these symptoms. Jake had has LAST dose of FK506/Tacrolimus one week ago and is down to 1mg of prednisone every other day. I can't wait until he is off the dreaded medications! We are scheduled to head back to North Carolina for his one year studies (two months late!) the week of July 21ST and hopefully we can begin to wean him off his Cellcept after that visit. The Cellcept is the last immunesuppressing medication he is on --- once he is off the Cellcept all should be pretty much back to normal as far as his immune system goes although we will still have to be careful with him getting illnesses for the next several years due to damage from chemo and transplant.

HAPPY FATHER'S DAY (one day late...sorry!)!!! I especially want to wish a Happy Father's Day to my husband - Steve, my Dad - Mark, my father-in-law - Steve, and my brother-in-laws - Chris & Ben!!! Hope you all had special days! :-)

Love from sunny Washington State ~ Melanie, Steve, Jacob, & Matthew

Monday, June 9, 2003 10:45 PM PDT

ANNOUNCEMENT*******ANNOUNCEMENT********ANNOUNCEMENT

GARAGE SALE/FUNDRAISER

Saturday, June 14, 2003
10AM-5PM

Our House:
4030 143rd Street NW
Marysville, WA
Call for directions: 360-654-1996 or 425-252-2066

***All proceeds from Garage Sale will go to Jacob's medical expenses and travel expenses***

***If you are interested in making a donation to help out with Jake's expenses and aren't able to make it to the Garage Sale...you can follow the link for COTA at the bottom of this page or call Cindy Wellman at 425-252-2066 (all donations are tax deductible)***

ANNOUNCEMENT*******ANNOUNCEMENT*********ANNOUNCEMENT

Thursday, May 22, 2003 9:12 PM PDT

Day +365 ~~~~ ONE YEAR POST-TRANSPLANT ~~~~

I could not let this day go by without an entry to Jake’s journal... Happy ‘Birth’day, Jakie!!! I feel it is important for us to mark this day in some way so we will be enjoying cake and ice cream, taking pictures, and making a little production out of things just as we do on ‘real’ birthdays...but...on this special day instead!

Jake is feeling well and doing great. I love weaning him from his steroids...they distort his look drastically when his dose is high and he is now starting to look like such a little boy. Auntie Rene’ saw him today and commented on what a resemblance she sees between Jake and Matt. I absolutely love to hear that as it is not something we hear often!

Anyhow, just a short update to let you all know that Jake is doing great and to wish our baby boy a Happy ‘One Year Post Unrelated Umbilical Cord Blood Transplant’ Day!!!

Love ~ Mel, Steve, Jake, & Matt

Sunday, May 11, 2003 9:15 AM PDT

****UPDATE Tuesday, May 13, 2003****
Sounds like the 60 Minutes II program might not be airing until September...sorry...
______________________________________

Abraham Lincoln once said, "All that I am or hope to be I owe to my Mother."

Happy Mother's Day to ALL of the Moms who read Jacob's website...with special Mother's Day wishes for my Mom – Cindy (I love you and hope you are having a wonderful time in Mexico!), my mother-in-law – Patty, my Grandmas – Bernie & Charlotte, my sister-in-law – Stephanie, and my friends who are new Moms - Staci (who had her second daughter last week) & Karin (who had her first child ~welcome Ava Grace~ yesterday)!!!!!!!!!!!!!!!!

Please forgive me if I must once again point out a 'milestone' in our transplant journey... Last year, Mother's Day was the day Jacob was admitted to the hospital to begin his chemotherapy in preparation for his unrelated cord blood transplant. I felt both fear and hope on this day. As Jacob's Mom, I was doing the only thing possible to keep my child alive yet in trying to keep him alive the opposite could happen and I could lose him even sooner than the Hurler's would have taken him. What a horrible decision we were forced to make! I remember lying in the hospital bed with him (watching Elmo videos of course!) and just hoping that everything would work out for the best. I also remember thinking about Matthew and hoping (as I have so many times in the past year) that Matthew doesn't resent me someday for having to spend so much time away from him… On this Mother's Day I am thankful to be spending the day with BOTH of my boys and I couldn't be happier that they are here with me --- happy and (for the most part in Jake's case) healthy.

Speaking of that, Jacob is recovering nicely from his surgeries last week. He still has a bit of pain from time to time but that is easily taken care of with a Tylenol. Yesterday he was a bit fussier than usual so we spent some time cuddling --- I think the healing process can be painful and irritating at times. He does not have to have his new port accessed until next Friday so that should give it ample time to heal. The hernia incisions look wonderful as far as I can tell and the bandages will be able to come off on Tuesday.

For those of you who have been reading Jake's journals for awhile you might remember that 60 Minutes II interviewed us for a story they were doing on rare genetic disorders and transplants right before we left Duke to return home. I have heard a rumor that this story will air this coming Wednesday night (May 14TH) and I had promised I would let you all know when it was going to be on TV. I'm not sure if our little interview will even be in it --- we might be on the 'cutting room floor'--- but I think it will be an interesting story nonetheless as the focus is a family that has 3 children with SanFillipo Syndrome with their youngest being the only one eligible for a transplant.

Once again HAPPY MOTHER'S DAY from one Mommy to another and another and so on...

Love ~ Melanie

Thursday, May 8, 2003 11:32 AM PDT

I should know by now that when we are told the 'typical' way things will happen that where Jacob is concerned that will not be the case, shouldn't I!?! Anyhow, I'll get to that in a bit...

We met with the surgeon, Dr. Healy, on Monday to discuss the foreign object Steve and I felt in Jake's testicle. He did a very thorough pre-op exam and determined that the end of Jake's shunt WAS making its way through a hernia 'hole' in to his groin area (we knew it!). On further examination he actually found hernia's on both sides so after a consult with neurosurgery it was decided that Jake would need to have bilateral (meaning both sides) inguinal hernia surgery as well as the port placement surgery on Tuesday.

Jake and I made our way to the hospital Tuesday AM to await his surgery. It was scheduled to take about an hour and a half. Jake was given a little oral sedative to relax him and his wonderful anesthesiologist, Dr. Lynn, carried him back to the OR. She wanted to intubate him in the OR because of the possible complications due to his Hurler's. I went to the waiting room to...you guessed it...wait... Steve and Matthew showed up about an hour after Jake was taken to the OR. We were trying to plan things so that Mathew wouldn't have to spend too much time waiting around --- Matthew is 21 months old and 'waiting' is not in his vocabulary! Well, Jake's surgery took about 2 and 1/2 hours... Dr. Healy came out and talked with us when he was done and told us that the hernia surgery went well (he repaired two large hernia holes). The port surgery took him much longer because he encountered a lot of scar tissue from Jake's old central lines. He had to 'dig' around the scar tissue and find a good vein to use for the port. He said Jake would probably be pretty sore because of the added trauma to the site. He said Jake was doing fine and we'd be able to see him soon. The recovery room Nurse came in and gave us the room number where he would be spending the night (neurosurgery requests a mandatory 24 hour in-patient stay when working that close to the shunt to administer IV antibiotics to protect against infection to the shunt) and told us they were going to transport him directly to the room so we could meet him there. We sat in his room for about 45 minutes when Dr. Lynn showed up. She said she had a lot of trouble intubating him (those horrible tiny Hurler airways!) but managed to do it without using the fiber-optics after a few tries. She said his throat would probably be pretty sore for a few days. She then told us that they had given him some morphine in the recovery room (he's had morphine lots of times in the past) because he was experiencing a lot of pain from the port site and he desated (oxygen desaturation) so he was put on blow-by oxygen and would remain in the recovery room until they were sure he was stabilized but would probably come to the room still on oxygen. My heart dropped after this conversation --- as all you Hurler parents out there know...these are things that send our kids to the PICU! Anyhow, she said he should be coming to the room shortly. So we continued to wait... When he finally got to the room he was miserable. His throat was so sore he drank two glasses of ice water immediately --- the cold seemed to make his throat feel better. He was crying, sore, and miserable for quite awhile but his sats (oxygen saturation levels) looked great (98-100%) which was wonderful so we didn't have to have him on oxygen in the room. Daddy and Matthew stayed as long as Matthew tolerated and then they left Jakie and me to spend the night. Jake ended up having a pretty good night and was able to sleep fairly well with the help of some oxycodone! He was discharged within 24 hours and is home with me right now watching Barney and relaxing on the couch. He's definitely still tender in areas but has been pretty comfortable with the help of some Tylenol. Matthew is spending some time with Grandma & Grandpa Irish (THANK YOU FOR ALL YOUR HELP WITH MATT-MAN) so that Jake can recuperate on his own. Matthew sometimes likes to 'help' a little too much. :-) He'll be back home later this afternoon and everything should be back to 'normal' by then. Steve and I are hoping this is the last of it for Jake for awhile. We want him to stay out of the hospital from now on!!!

In non-medical news, before we went to the hospital on Tuesday we were able to watch the USS Lincoln make its way to its homeport of Naval Station Everett from my parents front deck. It was truly an amazing site to see and something I really wanted the boys to take part in. I have to say they seemed more interested in all of the airplanes and helicopters flying overhead...oh-well! Anyhow, the sailors lined the railing in their dress whites and it was so emotional to see them coming home from war. I am proud to say that the citizens of Everett (and surrounding areas) came out in full-force to welcome these brave women and men home! These people were away from their families for 10 full months --- I can't even begin to imagine how difficult that must be! Welcome Home USS Lincoln!!!

Love ~ Mel, Steve, Jakie, & Matt-man

Saturday, May 3, 2003 10:04 PM PDT

Jacob is free of all external lines! It is such an amazing thing and has been one year since he has been virtually line free. He of course still has his shunt but this is all internal and doesn't hinder him in any way. He and Matthew took a bath together and were able to splash and play and dunk under the water --- it was such a joy to watch them having such fun and doing stuff they haven't been able to do together because of Jake's 'hardware'! Anyhow, we got through the IV antibiotics once again on Thursday and Dr. Hawkins pulled Jake's PIC line out at Clinic on Friday. I think Jake and I were both incredibly happy to see the last of the PIC line --- it was kind of a pain for all of us to deal with. He is scheduled to have his port placed on Tuesday. He has had one interesting and somewhat strange issue which could impact the surgery on Tuesday. It appears that his hydro-seal (fluid in his testicles) is possibly pushing the abdomen end to his shunt in and out of his testicle as well as the fluid we've always noticed. Steve and I were quite alarmed when we felt what appears to be the end of a tube in his testicle yesterday evening and the only tube in his body is his shunt! Although this is not an emergency thing, I believe we will be heading in to Clinic on Monday to try to get squeezed in for a surgical consult. He might need to have this hole (like a hernia) that is causing the fluid to go in and out surgically repaired. We've always known this is a possibility but were able to put it off until absolutely necessary. If it is affecting his shunt we might be to the point of 'absolutely necessary'. I guess we'll know more on how to proceed after the surgeons get a look at him.

Sadly, our network of Hurler 'family' continues to grow. I say sadly because I would not wish this horrible disease my child has on anyone - but - I can't tell you how much the other parents and children mean to us. It truly is an amazing bond and I think the fact that our children often times look like they could be related just intensifies that feeling of camaraderie. We'd like to send out big hugs from Jacob to our new friend Allison (www.caringbridge.org/nd/allison) as she begins her treatment for Hurler Syndrome. She will be receiving Aldurazyme infusions very soon. Aldurazyme was JUST approved by the FDA to treat Hurler syndrome. It is basically a manufactured alpha-L-iduronidase (the enzyme Jake is lacking). Just in case some of you are wondering, we probably would have still chosen to have an unrelated umbilical cord blood transplant for Jake even if the Aldurazyme would have been available. Aldurazyme is a safer option but is not shown to help with any brain issues which Jake obviousely had due to his slower development in comparison with other children his age and the hydrocephalus that was discovered pre-transplant. Kids with Hurler Syndrome have a VERY broad range of symptoms so what is right for one child with Hurler's might not be right for another. Anyhow, we know Allison is going to do great with this and wish her and her family the best. There are also a couple Hurler babies (that we're aware of) at Duke going through transplant --- Nolan and Jack are in our thoughts and prayers as well!

Take care! Love ~ Melanie, Steve, Jacob, & Matthew

Saturday, April 26, 2003 2:00 PM PDT

DAY + 339 (can you believe I still keep track!?!)

Jacob's procedure on Wednesday went OK. Of course, it wasn't routine, but it wouldn't be Jake if it was! It ended up taking a bit longer than expected so they had to continue to re-sedate him throughout the procedure. Needless to say, he was pretty snowed for the remainder of the day! His central line came out smoothly and the PIC nurse found a good vein to use, unfortunately, she got the tubing in and began threading it through to his heart and it got stuck near his shoulder, probably on his clavicle. Once he woke up I carried him down to x-ray so they could see if the location of the line was OK and to make sure it wasn't twisted. It looked OK although we are not able to draw anything out, just push his flushes and antibiotic in so if he needs a blood draw it will have to be taken peripherally. He's not going to like that but will probably only need to happen once before he gets his port placed. I'm not sure exactly when that will take place --- it should be sometime the week after next. He gets his last dose of IV antibiotic this coming Thursday so more than likely Dr. Hawkins will pull the PIC in Clinic on Friday. Jake is really not fond of the PIC line and will be thrilled to have it out. Basically, it takes up the whole lower part of his left arm from his elbow to his wrist (it's much bigger than I expected for some reason). Anyhow, Jacob seems to be feeling wonderfully and as long as I keep him in long-sleeved shirts he is happy (out of sight out of mind apparently). He and Matthew are running around like little boys should! They truly appear to be best-friends and love to play together. We are so blessed to have them both!

The meeting we had with the school district on Tuesday turned out to be just a fact gathering meeting. There was really no discussion about the amount of services they can offer Jacob yet --- just that they will be able to offer him something due to his delays. They will be in contact with us to continue the discussion on Jacob's needs and their availability. We did meet the therapists who work at Lakewood Elementary School and the developmental pre-school teachers and they all seemed very nice and interested in Jacob. I'm sure we'll know more in the near future.

We hope you all are having a wonderful weekend. Enjoy!

Love ~ Mel, Steve, Jake, & Matt

Tuesday, April 22, 2003 11:43 AM PDT

Thank you all for your kind thoughts and prayers! We appreciate it more than you could ever know!

Jacob was discharged from the hospital yesterday (Monday) evening so I am writing this journal entry from home! I tried to update over the weekend to let you all know what was going on but there just never seemed to be enough time in the day (or night)! Jake's blood cultures came back positive for gram negative rods (bacterial infection) in the middle of the night Friday. Steve was awake monitoring Jake's blood pressure as it was still very low throughout Friday night, dropping to 65/35 at one point. Anyhow, this is the same type of infection Jake had a few weeks ago but they had to get the bacteria isolated to know for sure if it was the exact same strain of bacteria as last time. We were all assuming it would be the same thing though. Well, Sunday afternoon rolls around and they finally get the bacteria isolated --- turns out it is a different bacteria (Klebsiellia). I report all this to Dr. K through e-mails and a phone call and she assures us this is a bacteria that hangs out inside the body nothing he 'caught' externally from someone or something. I repeat ---- this is not something he caught from someone or something but something his own body gave to him ---- so those of you who visit Jake in person do not think it was anything you did! Although this is a different bacteria Dr. K. wants us to proceed with the line removal (his single lumen Hickman catheter placed one year ago still remains in his chest), PIC (temporary IV line in arm) placement for the remaining 10 days of IV antibiotics (we’re administering these at home), PIC removal, and then port (access site directly to vein under the skin). She feels that we are starting to see a trend with these infections and the next one might not be as easy to treat. His line can play a roll in the continuation of these bacteria infections because it is very easy for the bacteria to 'hide' in the material of his line not to mention it is a foreign object in the body which is infection causing in itself. Jacob still remains immuno-compromised and certain infections can lead to death --- thankfully we have not encountered one of these but with the line his chances are greater for repeat infections and who knows what the infection might be. Anyhow, it took a bit of convincing Monday morning to get Dr. Hawkins 'on board' with this plan but I managed...the only problem is all of these procedures are considered 'elective' because he does not have the same bacterial infection so we were not able to get things done as immediately as I would have preferred hence the discharge yesterday evening. Jacob will have his line pulled and PIC placed Wednesday in Clinic under mild anesthesia, he will have the PIC pulled next Friday in Clinic, then we will have to schedule the port placement with the surgeons in the OR sometime after the PIC is pulled. Geeze....what a schedule!!! The good thing through all of this is Jacob remains in wonderful spirits. He felt better within a day of starting the IV antibiotics and that is truly all that matters to us. We just want him to feel good and remain healthy!

We celebrated Easter in the hospital which was not our preference but we made do. The hospital Easter Bunny came by to visit Jake on Saturday which he enjoyed. Matthew and Daddy stayed Saturday night with Grammie Cindy & Grampie Mark so Matt could color eggs Saturday night with Grammie and Easter Sunday they all (Daddy, Mattie, Grammie, & Grampie) came down to bring me Brunch and Jacob’s Easter Basket. We had celebrated Easter the weekend prior with Grandma & Grandpa Irish and Uncle Chris & Aunt Ne as they are in Hawaii right now and Aunt Stephanie and Cousin Nathaniel. Auntie MarMar was planning to come to the hospital yesterday but due to Jake's discharge will be coming out to our house on Thursday. Daddy took a nap with Jake in his hospital bed on Sunday so Matthew, Grammie, and I ran to U Village so I could get Starbuck's and Matt played in their little play area there. I had to drag him away when it started to rain --- he was having so much fun. Anyhow, all in all we had a very nice Easter.

One last thing to mention, this afternoon is Jake's IEP meeting with the school district. We are hoping everything goes smoothly and they agree to Jacob's needs. I've heard both good and bad from other parents about these meetings. We could use something going our way this week and are hoping this IEP is it (I'm not holding my breath though!). I'll let you know how it goes when I post again as well as how Jake is doing!

We hope you all had a wonderful Easter (or upcoming Easter for those who follow the Orthodox calendar) and were able to celebrate in your own way. Thank you so much for thinking of and praying for Jacob as he continues through this transplant process. Take care of yourselves and hold you children close!

Love ~ Melanie, Steve, Jake, & Matt

Friday, April 18, 2003 9:55 PM PDT

I only have time for a quick update right now (because I am tired and going to bed!!!) but wanted to get this posted... Please send prayers and good thoughts Jake's way. He was admitted to the hospital today! It began with irritability and vomitting late last night and turned into an entire day of a fluctuating fever (from normal up to 102.5), vomitting, and a dramatic drop in blood pressure (78/35 which is VERY low for Jake!). His labs look OK although he is a bit dehydrated. His white count and platelets, although not out of the normal range, were a bit high and low respectively. He had blood cultures done which are not back yet but I would not be at all surprised if our little Jakie-bug is battling some sort of infection again! The doctors are treating him with IV antibiotics in anticipation of an infection and fluids to bump his blood pressure up. Anyhow, he is pretty miserable right now and we just hope he gets better soon! I am home with Matthew tonight and plan to try to get some sleep as I didn't get much last night and spent the whole day in Clinic with Jake. Daddy is at the hospital snuggling with Jake for the night! Thank you in advance for your prayers and well-wishes. I truly believe the more people that are thinking of our 'baby' the quicker he will recover!

Love to all ~ Mel, Steve, Jake, & Matt

Wednesday, April 9, 2003 11:00 PM PDT

DAY + 322

Jacob is doing wonderfully. I have been putting off posting a journal entry because we just really don’t have anything much to report. He is no longer on IV antibiotics and his infection did not come back --- yipeeeee! Health-wise, Jake has not had any medical issues. We continue to wean his steroid and FK506 and thus far have had no problems with this. I have to say that I have really come to enjoy the ‘uneventful’ moments of our lives. Frankly, if things remain uneventful for awhile (or forever for that matter) I will be thrilled.

We have had some nice Spring weather lately and myself and the boys have been spending as much time possible outside. I feel like we’ve been cooped up in the house forever and I’m sure Jakie & Mattie feel the same. I’ve been doing some gardening and the boys love playing with their outside toys. They are both learning to ride their new tricycle (thank you so much Great-Grandma Bernie!) which is pretty much the cutest thing in the world --- see the photo album for proof of this fact! :-) Steve and I are looking at ‘swing-set’ systems as we think the boys would love something like this and because we have to continue to stay away from other kids this seems like the perfect thing to have as it keeps us away from playgrounds.

Well, I think that’s all for now. Thank you for checking in on us and for signing Jake’s Guestbook. I continue to check it every day just to see if anyone has ‘stopped by.’

Love ~ Mel, Steve, Jake, & Matt-man

***I POSTED NEW PHOTOS TODAY***

Wednesday, March 26, 2003 9:14 PM PST

DAY + 308

***NEW PICTURES***NEW PICTURES***NEW PICTURES***

I think I can safely say that no news is good news. Jake was discharged from the hospital last Tuesday as expected and eased back into his home routine without too much difficulty. On the other hand, I have had to get used to administering IV medications again --- every 8 hours --- which inevitably means I am sneaking into Jake’s room around 11:30pm every night, trying to hook him up to his pump, all without waking him up (yeah, right!). Thankfully the IV antibiotic only runs over 15 minutes so it’s not too tough on Jakie. He’ll receive his last IV dose on Saturday and then have blood cultures done both 48 and 72 hours after the last IV dose. If he remains infection free we’ll be truly happy --- if he has a positive culture, he’ll be back in the hospital and will have to have his line removed BUT we’ll cross that bridge if we’re forced to!!! Besides that, Jake appears to be feeling really well. He has had some wonderful therapy sessions which we feel are very important right now because his hospital stays have once again caused him some delay in his development. This is to be expected and easily fixable through therapy.

On to what we think is pretty exciting news --- Jacob’s immune function tests came back and the antibodies in his immune system are functioning at 100%!!! This is huge post-transplant news as 308 days ago his immune system was functioning at 0%. This does not mean he is able to completely fight infection and other nasty things nor does it mean he is completely off isolation. He is still unfortunately on two immune suppressing medications and he still has his central line which all cause him to be more susceptible to things, especially those things that one child passes to another child. He by no means could be put into a group setting with other children at this point and probably won’t be allowed to do this for some time yet. It does mean that once he is weaned from his FK506 and Cellcept AND once his line is removed we will pretty much be able to stop isolating him so much. The fact is that for the next few years we’re going to have to be a little more cautious with him as far as illnesses and such. If all goes as planned and he stays healthy…he should be off the FK506 by early June and then we can begin weaning the Cellcept which will probably take a couple months to completely come off of so maybe by August......... We celebrated by taking Jake on his first ‘official’ non-medical outing to Home Depot. He rode in his stroller with no mask and really enjoyed helping Mommy pick out some flowers for the flower beds! It was great to do something semi-normal with him!

We also got the report from the development people at UNC. Jake showed improvement in all areas since his last tests were done. We are so proud of him! I was also happy to see this because I will need it at the end of April when we have our meeting with the Lakewood School District in anticipation of Jake starting ‘school’ in the Fall. Depending on his immune system his schooling might take plus in the home and will hopefully be very similar to the therapy he is currently getting. We’ll see...

This week has been a bit of a time for reflection for me. It was one year ago this week that I was frantically packing Jake and myself up to move to Durham, NC for what I thought would be 3-4 months (little did I know). I had spoken to Dr. Kurtzberg at Duke and Steve and I made the decision to go ahead with things as quickly as possible due to the progression of Hurler Syndrome so Jake and I were on an airplane within 7 days of this phone conversation (we arrived in NC on Easter Sunday 3/31/02). Steve and Matthew were going to follow as soon as Steve could get all his jobs wrapped up and the house closed up. Matthew was only 7 months old. It was such a difficult, scary time for all of us --- yet, I can honestly say I had a feeling of such hope when I boarded that plane. We were going to make Jacob better and that was and still is all that matters!

Our thoughts and prayers are with the women and men in our armed forces --- we support our troops now and always. Please hold your loved ones close...

Love ~ Melanie, Steve, Jacob, & Matthew

P.S. Happy 12TH Birthday to our cousin (& niece) Lauren! Jacob and Matthew send you hugs and kisses… Hope you had a wonderful and special day!

P.S.S. If you get a chance, please check out Jacob's online quilt at www.smilequilt.com/jacobi.html. This is a wonderful organization that makes these adorable virtual quilts for kids dealing with terminal/life-threatening illnesses. Thank you to Smile Quilts for this --- it is perfect!

Tuesday, March 18, 2003 9:49 AM PST

Well, here Jake continues to be......in-patient at Children's Hospital Seattle. We have reason to believe, though, that he will be discharged today. It sounds like we are just waiting on one more negative blood culture which was drawn yesterday morning. The results from that should be back anytime so...we wait. Sunday mornings culture came back negative so we have no reason to believe Mondays will show anything different. We are so happy that the antibiotics got in there and did their job! Jake will be going home with two new medications unfortunately. Both I will give him by IV twice a day --- Merepenum & Gentomyacin --- which are both strong antibiotics. I don't know how long yet but the normal courses for both of them are 7-14 days so I'm sure it will be somewhere in that range. One of the concerns we have now is that once the antibiotics are stopped the infection could possibly come back. This could happen because he still has one of his central lines. The infection can become trapped in the line and will only show up negative while the antibiotics are running through the line, once they stop the infection comes back within a day or two. This would mean Jake would have to have surgery to remove his line, then 2 weeks with a temporary line, then new line placed (or port possibly), AND more time in the hospital. This is something we'd love to avoid so our hope is this infection is truly gone!

Jake has had some visitors while he's been here which has been great! Jake loved seeing Grandma & Grandpa Irish on Sunday and Auntie MarMar on Sunday and Monday (although I think Monday was more for Mommy as Auntie brought lunch and watched Jake so I could shower!). Auntie Rene' is supposed to come by today but I might have to call her and tell her we won't be here --- I'm sure she'll be fine with that! Once again, Steve and I want to thank our parents and Auntie Stephanie for helping out with Matthew. It was definitly easier on us that the majority of this hospital stay for Jake was over a weekend that Steve didn't have any work scheduled so we were able to keep the boys together a little more. We really hate to separate them as they have such a nice bond!

Anyhow, I will update again soon --- especially if there are any changes. Take care and thank you all for checking in on us and for leaving messages in Jake's Guestbook. As always, it is such a pleasure to read all your wonderful messages!

Love ~ Mel, Steve, Jake, & Matt

P.S. Hope you all had a great St. Patrick's Day yesterday! You would think I would make a big deal out of it with a last name like 'Irish' wouldn't you??? The Nurses here definitly had fun with it yesterday --- and the clowns came by and kept telling the other kids how it was Mr. Irish's day and pointing at Jake's room. Jake of course got a kick out of all the other kids looking at him and put on a little show for them from his hospital bed!

Saturday, March 15, 2003 1:32 PM PST

Just wanted to post a quick update to let everyone know that Jake is back in the Hospital. He has not really seemed himself this week but we have been unable to pinpoint anything specific...so...he had some tests done yesterday at Clinic to see if the doctors could figure out what was going on with him. Steve and I got a call at 10PM last night from the doctor on-call saying that "gram negative rods" grew in his blood cultures which means he has a bacterial infection. Hmmmm...I guess that's why he hasn't been himself this week! She said we needed to come in right away to be readmitted (through the ER because it was after hours - UGH!) so we packed up out little ones, dropped Mattie at Grammie Cindy and Grampie Mark's and brought Jake to the Hospital. The infection he has is called Citro Bactera and it is easily treatable with antibiotics which is wonderful news. We're not sure how he got it exactly. It is one of those bacterias that is already in your body --- they think he either has a urinary tract infection which is a common area for Citro Bactera OR while he had Rotavirus he had some tears in his gut and it got into his blood that way. Regardless, he's got it --- it's being treated --- and hopefully we'll have our Jakie back to normal before you know it! We will definitly be in-patient for a couple of days as he can't be released until his blood cultures are negative for 48 hours. Please send good thoughts Jake's way and pray that these antibiotics do their job and we are able to get home and back to normal sooner rather than later.

Thank you all for checking in on our Jakie-Bug.

Love ~ Mel, Steve, Jakie, & Mattie

Thursday, March 6, 2003 8:09 AM PST

DAY +288

Jacob was discharged from the hospital Monday afternoon! It was great to get him out of there although I admit to being pretty nervous about it after our experience last week (the discharge and re-admittance all within hours of the other!) but thankfully all went well. Jake seems to be feeling OK since then. He is much less irritable and although I wouldn’t say his ‘poopies’ are normal they definitely aren’t the ‘water’ they were for the past week and a half!

He restarts his therapies beginning on Saturday and he certainly needs it after spending a little over a week in bed. The poor little guy remembers what he is supposed to do; he’s just not strong enough to do it now. He gets frustrated so easy. I’m sure it will only take a very short time to get him back to where he was pre – Rotavirus though

I think that’s all for now. Jake sees Dr. Hawkins Friday and depending on how his labs look we think everything will be just about back to ‘normal as we know it.’

Take care ~ Mel, Steve, Jake, & Matt-man

Thursday, March 6, 2003 8:09 AM PST

Monday, March 3, 2003 9:57 AM PST

DAY +285

I think I finally have time for a more detailed journal entry. Jake has kept us very busy these past several days...

Jacob spent most of the day Wednesday, and all day Thursday and Friday crying/moaning/sobbing. It was horrible and it seemed like there was little we could do that helped --- from comforting him to medicating him. Finally, Saturday the Dilauded kicked in and he became VERY drugged....but not crying finally! I should probably backtrack a little though.....the thought on Wednesday was he was upset because he wanted to get out of the hospital. His diarrhea had slowed down and he had not vomited in a day and a half --- so he was discharged. I brought him home and things just got worse. He cried harder and was inconsolable and the diarrhea started up again --- we were back to the ER within 4 hours where they performed tons of tests to see what might be going on (labs, x-rays, brain CT, enema to check his bowels). This took almost all night Wednesday night and was exhausting. Steve ended up dropping Mattie off at his parents’ house and coming to the hospital with my Mom arriving right before him around 1am Thursday morning. All the tests came back negative for anything bad and we were finally admitted back to the cancer unit around 4:30am. Steve and my Mom headed home as they both had to work and Jake and I slept for 3 hours before the crying started up again. The doctors just seem to think he has a REALLY bad case of rotavirus and the stomach cramps and general pain associated with this were really intense for him. Anyhow, finally Sunday morning he seemed to be more himself except still somewhat drugged. They stopped the pain meds and all continues to go well. He had one dose of oral oxycodone yesterday at 2:30pm and has needed nothing since then! The diarrhea persists which is a problem. There is a concern as to the absorption of his medications and of course the possibility of dehydration which is much more common post-transplant. They had hoped to discharge Jake today (and we had hoped to be discharged) but I am not positive if that is going to happen. The doctors will be by in the next couple hours and we’ll see what they think. I certainly do not want to get discharged too soon as I do not want a repeat of Wednesday but it definitely would be nice to get home. I miss Mattie terribly when we are separated (I did spend Friday night and most of Saturday with him though as Steve stayed at the hospital with Jakie). Anyhow, we’ll see what happens and I’ll try to update when we make it home! :-)

I big thank you to our Parents for helping out with Matthew during this difficult week +. Also, an extra big thanks to my Mom for coming down in the middle of the night twice! I know I’ve said it before but we really could not do it without the support we get from our families! Jacob also wants to thank all his visitors for taking the time to come see him --- Mommy, Daddy, & Mattie; Grammie Cindy & Grampie Mark; Mindi (one of his therapists); Auntie Ne’; and of course Athena (Lukie’s Mommy). The phone calls and e-mails by pretty much everyone else are very much appreciated as well!

In other news, many of our Hurler Friends are having a tough time right now dealing with everything from infections to the more severe issues that plague our recently transplanted kids. Please send special thoughts and prayers their way...Taylor in Minnesota, Reese in North Carolina, Isobel in Canada, Henry in Texas, and finally Carter’s family in Canada for the loss of their beautiful Hurler Angel this past Saturday. Our thoughts and prayers are certainly with you all!

Love ~ Melanie, Steve, Jake, & Matt

Thursday, February 27, 2003 2:00 PM PST

Jacob remains in the hospital. He is doing OK but VERY uncomfortable and dealing with some pain. He was actually discharged yesterday evening but we were back to the ER 4 hours later. I'll explain in more detail later. I've got to get back to my little sicky! Thank you all for your nice guestbook messages --- keep them coming!

Love ~ Mel, Steve, Jake, & Matt

P.S. I posted our hospital information below as I'm pretty sure we're going to be here for several more days (just in case you're thinking about it --- there are no flowers or latex balloons allowed in the Unit!).

Monday, February 24, 2003 10:23 PM PST

Just wanted to get a quick update out to everyone. Not long after my last journal entry Jacob started to get sick...vomitting and diarrhea...just like Matthew. Exactly what we were hoping wouldn't happen was happening, Jacob appeared to be coming down with Matthew's sickness/stomach virus. By 3AM I had talked to Dr. K (I think I woke her...oooops) and the Hem/Onc Fellow on-call at Seattle Children's and was driving to Children's Hospital in Seattle (one hour away from our house) where we were admitted through the ER (went straight to a room --- did not have to wait in the ER waiting room thank God!) to the BMT/Cancer Unit of the hospital. My Mom (Grammie Cindy) thankfully popped in her car and met me at the hospital to help me out as Steve had to stay home with a still sick but recovering Mattie. We've been here ever since and will be here a day or two more for sure if not longer. Jake's stool sample came back positive for rotavirus. For more information on rotavirus go to http://kidshealth.org/parent/infections/bacterial_viral/rotavirus.html. In a nutshell it is very common in children, most will catch rotavirus at some point before the age of 4 or 5. Symptoms are vomitting, diarrhea, and fever which Matt and Jake both experienced. Matthew obviously has been dealing with it and fortunately he is able to do this at home without the need for a hospital visit. Jacob on the other hand is not able to fight things on his own like Mattie...he needs the help of IV fluids as well as converting all his other medications to IV form in order to keep them inside of him. The good news is he is going to get through this and actually seems to be getting better already. He continues to have diarrhea but the vomitting appears to have stopped. He had a fever all night last night but it broke early this morning. He is not interested in eating or drinking anything on his own at this point so IV fluids continue to be necessary. There are no medications that can be given for rotavirus so we are in that wait and see what happens phase. Once Jake starts consuming liquids on his own and the diarrhea has gone away or at least slowed down we will get to come home. Steve is taking care of Matthew and I am staying with Jake. It is so hard when both kids are sick in this situation because Matthew can't come to the hospital so Steve has to find someone to watch him so he can see Jake and at this point I'm pretty much not able to see Matthew at all. Hopefully, this whole thing will be short-lived and we will be back to 'normal as we know it' before long. Thank you all for checking in on us!

Love ~ Mel, Steve, Jake, & Matt-man (this is what he calls himself...how cute is that!?!)

Saturday, February 22, 2003 3:49 PM PST

DAY +276 (9 months post-transplant)

Well, we made it home... I considered starting this entry...we’re back from hell week...but it truly wasn’t THAT bad.... ;-)

Anyhow, things started on the wrong foot...on our way to the airport last Sunday I called to check to make sure our flight was departing on time which it was, then on a whim I decided to check our connecting flight as well which I’ll admit I never do, I hear the recording say the flight has been cancelled of all things! I immediately get a ‘live’ person on the phone and we try to figure out what we can do (Raleigh/Durham was experiencing freezing rain/sleet/snow and almost all flights in to the area were cancelled due to this). Of course, everything is booked up out of Seattle until late Monday night so we make the decision (really our only option at that point if we wanted to get there in time for Jake’s appointments) to go ahead and keep the flight we have and get from Seattle to Minnesota Sunday evening and then they re-book us on two flights out of Minnesota on Monday (7am & 1:15pm) in the hopes that the weather conditions will be better and the flights will not get cancelled. Because we knew before the first leg of our flight took off that the second leg was cancelled we technically were not ‘stranded’ in Minnesota so we did not receive any reimbursement from the airline for our hotel/food which was somewhat annoying but whatever... We of course tried to make the best of the situation and contacted Jen & Taylor (www.caringbridge.org/mn/taylor). I have posted Taylor’s website in the past...she is on her second transplant for Hurler’s in Minnesota and they are from Washington State as well. I feel like Jen and I already know each other but we have never actually met in person so it was wonderful to finally meet face to face. Taylor is completely adorable and the boys were quite taken by her. Jen was nice enough to drive me to a grocery store so I could get some milk, Pediasure, diapers, and wipes (I had enough to get me through the day but had planned to buy these things on our arrival in Durham!). She was truly a lifesaver as the hotel was right by the airport and Mall of America (closed by the time we arrived in Minnesota!) but no grocery stores in the vicinity. I took some photos of Jake and Taylor and will post them today or tomorrow. Taylor is doing really well medically which is great to see...thank you so much for visiting with us! Anyhow, we were able to get out of Minnesota on the 7am flight (9 degrees in Minneapolis/St. Paul at this time of day....brrrrrrrrrrr....) and arrived in Durham with no additional problems. The boys were excellent on the flights both getting there and coming home yesterday which made things so much better and easier on Steve and me.

Jacob started the week with some fussiness, a couple vomiting episodes, and some diarrhea which I just thought to be all the traveling and change. These symptoms in Jake don’t really alarm me as they are some of the many medical things we deal with on a day-to-day basis with him. On the other hand, Wednesday evening Mattie started acting strange. He has never been sick in his whole 18 months of life and I’ll admit we’ve come to take his overall healthiness for granted. Anyhow, Matthew vomited Wednesday evening (all over!) and had a fever (up to 105) throughout the night. For the most part he slept from 5pm Wednesday evening to 2:15pm Thursday afternoon only waking up to vomit and cry (we think because of stomach cramps). I of course talked with Dr. K and Jen (Jake’s NP) about all of this and they gave me some wonderful suggestions and even examined Matthew to make sure there wasn’t anything else going on at Jake’s appointment with Dr. K Thursday afternoon. They decided there was no reason to separate the boys because at this point Jake would have already been exposed to whatever Mattie had, not to mention Matt could have caught it from Jake during those couple days that Jake exhibited some similar symptoms. Matthew seemed better for awhile and now is sick again. He’s vomited a few times since we’ve been home, has a low grade fever, and horrible diarrhea. If this keeps up it looks like I’ll be taking him to see the pediatrician next week!

Because the boys were sick we weren’t able to visit the Unit (5200) nor were we really able to visit anyone in Clinic. We were at Clinic for 6 hours on Wednesday to get Jake’s IVIG and Synagis (I know this should only take a couple of hours...don’t get me started!) and didn’t really see anyone we knew during this time. Two of our wonderful nurses from the Unit, Kelly and Andria, came to visit us on Wednesday after Clinic which was great. It was so nice to see them and we got to show off how wonderful Jake is doing (he was feeling perfect by this point; it was Matthew who was starting to feel sick). Anyhow, I think they were thrilled to see how Jake was doing too...I think the last time they saw him he was pretty yellow from his jaundice and just not himself! Thank you so much to Kelly and Andria for taking the time to track us down so we could visit with you --- Jakie was thrilled! We also got to see Jayne Cash on Wednesday before Clinic which was excellent as well. We have such fond pre-transplant memories of Jayne. I can honestly say she got me through my first month in Durham!

Another WONDERFUL visit we had was with Jake’s ‘old’ Best Buddy Stacy. She is now another child’s Best Buddy but has become a close friend of ours! She is one of the nicest people I know and Jacob continues to totally adore her. She brought us dinner two of the nights we were there and on a third night her and I went and picked up dinner for us and while we were waiting we had a margarita in the bar ---- it was great to have some girl talk time!!! Her boyfriend Stu came with her one of the evenings as well and it was great to see him too. We really miss Stacy when we are gone from there and so this was definitely a highlight of our trip back to Durham. We can’t wait for Stacy to come visit us ---- your room is ready for you!!!

Another highlight of our time back at Duke was our Thursday meeting with Dr. K and Jen. We absolutely adore Jen (Jake’s NP) and it was great to see her again and of course Dr. K! And now for the excellent news...Jake checks out wonderfully!!! He has either stayed the same or improved in all areas of the testing. His brain CT looks better which was big news for me to hear and his spinal x-ray (kyphosis) looks the same but possibly slightly improved! His liver is much smaller although his heart remains slightly enlarged but not any more so than in his past ECHO’s. It is obvious to everyone that he has improved greatly in the area of his development although we won’t have the official report on this for 2 to 3 weeks. We also will have to wait 2 to 3 weeks for his immune studies and enzyme level. I will post these results as we learn them. His chest x-ray looks clear and his eye exam remains unchanged. With all this good news Dr. K feels we can make some medication changes...yippee...we will be weaning him off his FK506 and his steroid simultaneously. If all goes well with this he could actually be off some medications in the near future. We have a list of possible reactions to coming off these drugs to look for so we’ll see how it goes. She did increase Jake’s Cellcept because his weight has gone up (little fatty) and won’t start weaning this until he’s off the FK506 and prednisone. This is true for most of his other medications as well --- he pretty much won’t be coming off anything else until we can get him off the FK and steroid. I can’t wait for the rest of our test results to get in because I am sure they will be just as good as everything else! I think Dr. K was pleasantly surprised and impressed with how well Jake is doing which of course makes Steve and me so very happy.

So...as you can see there were many good points to our return visit to Duke. I wish things had been a little smoother with our flights and that Jake and Matt hadn’t gotten the stomach virus or whatever it is....hopefully Mattie will be feeling better soon. He’s not a very good ‘sicky’ and I feel a bit panicked when he doesn’t feel good. Dr. K says we are "on another level" as far as dealing with illness and I am just not good at this ‘normal’ kid stuff anymore!

I think that’s it for now...sorry about the length...just wanted to get everything out there! Take care...

Love ~ Mel, Steve, Jake, and Matt

Sunday, February 16, 2003 12:32 AM PST

HAPPY belated VALENTINE'S DAY!!!! We hope you all were able to spend a little time with your loved ones! :-)

I just wanted to quickly update to let everyone know that we are leaving tomorrow (actually today --- geez I need to get to bed) for Durham. We are unfortunately flying commercial. Corporate Angel Network tried their hardest but were unable to help us out this time although they thought they had something for us a few times. Anyhow, we're just going to try to make sure Jake wears his mask as much as possible and also try to make sure that those around us aren't ill.

Healthwise, Jacob is doing really well. His magnesium was pretty low on Friday so he got an IV boost. Dr. K says it's his FK506 catching up with his kidneys...we're going to talk about it further this week but she says it's nothing out of the ordinary for post-transplant kids.

Wish us luck --- flying with two little ones in the middle of the day ought to be interesting!!!

Love ~ Mel, Steve, Jake, & Matt

P.S. We won't have our laptop with us (we are bringing SO much stuff I just couldn't see adding another thing!) so we will check in when we return and hopefully have a fun update to post.

Thursday, February 6, 2003 3:22 PM PST

DAY +260

I’m really not sure if I have much medical news to report since my last entry. Medically, things have been quite boring which in our case is a very good thing! Jacob continues down the path to full recovery. His doctors have weaned his steroids a little bit since we had to bump them back up and hopefully we can continue with this trend. He is still on the higher dose of Cellcept just to ward off any GvH symptoms. His super chubby steroid cheeks are back as you can probably tell by the pictures I posted the other day. It’s funny because he had started to slim down and look more like a little boy...now he’s getting his chubby cheeks back which I think makes him look more like a baby! Either way I think he’s totally adorable!

Jacob’s therapy sessions are going superbly! He adores all his therapists and although he is pretty adamant about doing things on his own time (what 2 1/2 year old isn’t!?!) he continues to reach many milestones set before him. I can’t wait for his North Carolina development team to see how much he has improved!

Speaking of N.C., we have our official 9-month studies schedule and as I suspected we will be pretty busy. We are still anticipating that all 4 of us will travel to Durham whether that be commercially or privately we do not know and probably won’t know until right before we leave. Corporate Angel Network has a way of finding flights right before (as in a day or so) you need them! Anyhow, Tuesday - 2/20 - Jake spends the morning with the Dr. Escolar and her development people at UNC Chapel Hill, then early afternoon with Dr. Fitch his orthopedic surgeon. After that, we’ll try to get to Clinic to see some of our friends. Wednesday morning – 2/21 - Jake will have a brain CT scan and a chest x-ray then we will probably head to Clinic so Jake can get his IVIG which he is due for that week. Thursday - 2/22 - he will have his big lab draw, pulmonary function test, an appointment with Dr. Enyedi his eye doctor and to finish everything off an afternoon appointment with Dr. K. This of course is all subject to change! We are hoping to get in some time to see some of our transplant friends who are there, visit the Unit to see Jake’s Nurses, and visit with his Nurse Practitioners at Clinic. We also must see Jake’s Best Buddy Stacy and maybe even her boyfriend Stu. It is very important for us to make this trip as ‘fun’ as possible for Jake as we will be making trips back to Duke for quite some time and we don’t want him to associate these ‘trips’ with too much awfulness and medical stuff. You do what you can...you know...

In other news.......the past week or so Steve and I have had a chance to get a couple evenings off so to speak. My parent’s (Grampie Mark and Grammie Cindy’s) house has been somewhat under construction so they stayed with us for a few nights. This came in handy as one of the nights was my birthday (yes...I turned 30...ugh!!!!) so Steve and I were able to get out for dinner and another evening we were able to get to dinner and a movie with Steve’s brother Chris and his wife, Rene’ which was very nice. I can’t remember the last time we saw a movie! I think we’re feeling much more comfortable with Jake’s condition and of course ‘live-in’ help is always appreciated!!! :-)

Well, that’s it for now. I’m not sure if I’ll get a chance to post before we leave for Duke but will be sure to get an update posted on our return. Thank you all for your continued love and support through this process.

Love ~ Mel, Steve, Jake, & Matt

Sunday, February 2, 2003 1:08 PM PST

FINALLY --- FINALLY --- FINALLY!!!!!!

NEW PICTURES HAVE BEEN POSTED TO THE PHOTO ALBUM!!!

CHECK THEM OUT --- THEY'RE PRETTY CUTE IF I DO SAY SO MYSELF... :-)

Wednesday, January 22, 2003 at 02:04 PM (PST)

Hello All! Hoping this entry finds you all happy and healthy. We’ve had a pretty ‘normal’ (normal in our world anyhow!) week and a half since my last entry...

Jacob’s vomiting and diarrhea has completely stopped since the doctors decided to increase his steroids and Cellcept which a quite a relief. Jacob last stool sample again came back negative for c-diff and rotavirus. We are still not sure if we are dealing with gut GvH or a virus that does not show up in the cultures. Regardless, he’s feeling better and that’s all that matters. Now we just need to get his medications weaned back down and hope that we don’t get another flare-up of vomiting, diarrhea, or skin rash!

Jacob’s therapies are still going so incredibly well. I swear with each session I see him learning something new. He still has a long way to go as he is developmentally at about the same level as Matthew - 17 months old - as opposed to the 30 months old he should be but we are working hard to get him back up to speed. We’ve had a minor set-back with the hearing aids though which is NOT Jacob’s fault. He continued to wear them every day with no problems up until this past Saturday when the left one started giving feedback (piercing squeal sound) whenever you touched it or put it in Jake’s ear. We did all the troubleshooting suggestions to no avail so they are going to take a look at it when we are down at Children’s on Friday. I guess this kind of stuff can happen so hopefully it will be an easy fix.

The last thing I want to mention is that we are scheduled to head back to Durham the week of February 17TH for Jakie’s 9-month studies. We don’t have an exact schedule yet but know it will be a busy week. I am going to contact Corporate Angel Network and hope they might be able to come up with a flight for us. I am actually looking forward to heading back to N.C. so Dr. K can see and evaluate Jake and so we can catch up with some of our ‘East Coast’ friends! I am pretty sure Steve is going to be able to come as well this time which will be nice and if we have to fly commercial Matthew is going to come as well which makes me happy as I absolutely hate to leave him. We unfortunately can’t take him if we get a flight through Corporate Angel Network as one of their rules is no siblings allowed --- only the patient and one or two caregivers...we’ll see though...

Take care of yourselves and your families! :-)

All our love ~ Mel, Steve, Jakie, & Mattie

P.S. I've had a couple requests for new pictures and I promise to get some posted very soon. The batteries are dead on the digital camera and every time I go to the store I seem to forget the batteries! They're on my list so I shouldn't forget next time I get to the store!!! :-)

Saturday, January 11, 2003 at 10:45 PM (PST)

Day + 234

The beginning of the New Year has brought us both highs and lows and we’re only on the 11TH day of the year...

Jacob has his Clinic appointments where he sees Dr. Hawkins, gets labs drawn, and gets infusions if needed every Friday. Last Friday morning (the 3RD), I was awoken by a crying Jakie. I went into his room and my poor baby had vomited and had diarrhea in his bed and on himself. I got him cleaned up and took his temperature. Thankfully he had no temperature so we headed off to his normal Friday appointment. He seemed fine – had no more vomiting – but continued to have a bit of loose stools. Dr. Hawkins took some of his stool to be cultured to test for viruses and bacteria but Jake appeared fine after that so we just decided rather than change his medications around to just observe him. Thankfully, all went well the remainder of the day and his stool cultures all came back negative.

Saturday was Kyle’s Memorial Service. Steve and I were both able to attend because my wonderful friend Karin and her excellent Mom (who’s also a Nurse) offered to watch the boys for us. We really appreciated that as we both felt it very necessary to pay our respects to the Cottey Family. Kyle’s Service was beautiful and very befitting a wonderful 4 1/2 year old boy. There were so many touching moments to the Service; you can see one of them (the releasing of the red balloons) on Kyle’s website (www.caringbridge.org/wa/kyle). I have to admit that it was very difficult to be there......grieving is never easy......especially in this situation. We want Angie, Tom, & Casey to know how much we care about them and that they remain in our thoughts and prayers.

Dr. K e-mailed me with Jake’s 6 – month immune study results and Jake’s immune system is 75% of normal!!! This is excellent news and much better than we expected. She made it clear that this is by no means normal and we must continue to be incredibly cautious with him. We are now allowed to go to the park, the grocery store in the middle of the night and early morning (times of the day when we have the least possible chance of running into any other children), and small shops with little to no people in them MASK FREE! If he rides in a shopping cart it has to be wiped down with antibacterial wipes before he gets near it. He must still wear his mask when going to the hospital, doctor’s office, Clinic, etc. He has to continue to avoid other children and crowds. I really don’t plan on taking him to the grocery store as I try to get that kind of stuff done when Steve is home with the boys but nice to know that I have the option if absolutely necessary. Of course if anyone is feeling ‘under the weather’ they have to stay away from Jake. We definitly feel more freedom when it comes to outdoor activities.

Jacob’s therapies continue to go really well. He really seems to pick up on everything that is taught to him. It is really amazing to see him advancing so nicely. He has so many new words and although they’re not completely clear you can definitely tell what he is saying. His two new words this week are ‘football’ and ‘touchdown’. Both he and Matthew are saying these two words and Daddy couldn’t be happier! Jakie also started drinking out of a ‘sippy cup’ which is a big step as well.

Unfortunately, this week ended with more vomiting and diarrhea. Jake was very obviously not feeling well and made for an uncomfortable few days for him and us with very little sleep for anyone. He had a low grade fever (under 101) and a very mild skin rash along with the tummy problems. It started Thursday morning so we decided (after consulting his doctors) to hang in there until his normal Friday Clinic appointment. Dr. Hawkins and Dr. K consulted with each other prior to this appointment and decided to increase Jake’s steroid by a very large amount and add another dose of his Cellcept (MMF) a day. We also got another stool sample to test. We should have results on this by Monday. The worse case scenario is that he is suffering from gut GvHD which would not be good. Dr. K and Dr. Hawkins chose to increase the steroid and Cellcept just in case this is the problem but if it is just a virus these increases will help as well. It’s kind of one of those wait and see situations so that’s what we are doing...and I have to say that Jake seems to be feeling much better today.

The last thing is that Jacob FINALLY got his hearing aids yesterday. They are blue and he looks totally adorable with them in. I’m going to have to take a couple new pictures and get them posted. Jacob is handling the new hearing aids wonderfully – he doesn’t even seem to care that they’re in. They told me to be happy if I could get him to wear them 5 minutes at a time...well...he wore them all day yesterday besides nap time and has worn them all day today besides during his nap (don’t be too jealous Anne but I don’t think he’ll be throwing them into any bodies of water :-)!). I honestly think he likes them and I think he can tell that they are helping him hear better. I am really hoping that over the next couple weeks he starts speaking clearer. That would be excellent.

Thank you all for checking in on Jacob and for signing his guestbook (hint hint)! :-)

Love ~ Mel, Steve, Jake, & Matt

New Year's Day, Wednesday, January 01, 2003 at 03:30 PM (PST)

Wishing you all a happy, healthy 2003. I feel a lot of hope that with this change from 2002 to 2003 comes a better year for us and many of the families we have met along the way. 2003 IS going to be a good year!

Our Christmas celebrations this year were very low-key and spent in small groups due to Jacob's weakened immune system. Christmas Eve was spent quietly at home --- just the 4 of us. Christmas Day was spent with Grammie Cindy, Grampie Mark, and Auntie MarMar (or MoMo as both Jake and Matt like to say) exchanging gifts and socializing. Our Christmas then extended to the 26TH which was spent with Grandma & Grandpa Irish, and Aunt Rene'. The boys had a wonderful time and definitly mastered the fine art of wrapping paper removal! Steve and I have been joking about how we need to buy a whole other house for the Christmas presents. Both sets of granparents, aunts, & uncles really know how to spoil the boys not to mention Santa Claus! Oh-well, after what they've been through this past year they deserve it!

Besides the holiday festivities, Jacob is doing really well medically. He is no longer taking Methadone and both his steroid and FK506 doses have been reduced. His donor test came back and he continues to be 100% engrafted. We continue to wait on a few test results and then should have some answers from Dr. K on how Jake's doing in regards to where he should be/is during this point in the transplant process. The holidays delayed some of the results which should be back in the next week or so. Jacob's therapies are going wonderfully --- he is showing such amazing improvement and we are just thrilled with the level of therapies he is getting. He has about 25 words he uses spontaneously and about 35 he imitates. He still prefers to point, gesture, and grunt to communicate with us but he has the words in him and uses them when he really needs to...I'm hoping when he gets his hearing aids on the 10TH we'll see even more improvement in this area.

It is with a heavy heart and a great deal of sadness that I must pass on the loss of Kyle (www.caringbridge.org/wa/kyle). He went to Heaven on December 23RD. I thank all of you who prayed for him at our request, unfortunatly his little body had had enough and he went to be healed in Heaven. His Memorial Service is scheduled for this coming Saturday in Sumner. I will be attending, hopefully with Steve. It is a difficult day for childcare and so Steve might be needed at home with the boys. I am not looking forward to going alone but must be there for Kyle. I also need to mention that a Hurler baby named Brittney (www.caringbridge.org/nc/brittney) passed away on December 30TH. She was about a month post-transplant at Duke and was having the same gallbladder problems as Jacob had during his transplant. She was in surgery to have a drain placed (same as Jake had done), sadly she didn't make it through the procedure. Her family is having a very difficult time with this loss and could use your thoughts and prayers sent their direction. We feel such an incredible bond with other Hurler families and feel their losses very deeply.

All our love ~ Melanie, Steve, Jacob, & Matthew

New Year's Day, Wednesday, January 01, 2003 at 03:30 PM (PST)

Wishing you all a happy, healthy 2003. I feel a lot of hope that with this change from 2002 to 2003 comes a better year for us and many of the families we have met along the way. 2003 IS going to be a good year!

Our Christmas celebrations this year were very low-key and spent in small groups due to Jacob's weakened immune system. Christmas Eve was spent quietly at home --- just the 4 of us. Christmas Day was spent with Grammie Cindy, Grampie Mark, and Auntie MarMar (or MoMo as both Jake and Matt like to say) exchanging gifts and socializing. Our Christmas then extended to the 26TH which was spent with Grandma & Grandpa Irish, and Aunt Rene'. The boys had a wonderful time and definitly mastered the fine art of wrapping paper removal! Steve and I have been joking about how we need to buy a whole other house for the Christmas presents. Both sets of granparents, aunts, & uncles really know how to spoil the boys not to mention Santa Claus! Oh-well, after what they've been through this past year they deserve it!

Besides the holiday festivities, Jacob is doing really well medically. He is no longer taking Methadone and both his steroid and FK506 doses have been reduced. His donor test came back and he continues to be 100% engrafted. We continue to wait on a few test results and then should have some answers from Dr. K on how Jake's doing in regards to where he should be/is during this point in the transplant process. The holidays delayed some of the results which should be back in the next week or so. Jacob's therapies are going wonderfully --- he is showing such amazing improvement and we are just thrilled with the level of therapies he is getting. He has about 25 words he using spontaneously and about 35 he imitates. He still prefers to point, gesture, and grunt to communicate with us but he has the words in him and uses them when he really needs to...I'm hoping when he gets his hearing aids on the 10TH we'll see even more improvement in this area.

It is with a heavy heart and a great deal of sadness that I must pass on the loss of Kyle (www.caringbridge.org/wa/kyle). He went to Heaven on December 23RD. I thank you all who prayed for him at our request, unfortunatly his little body had had enough and he went to be healed in Heaven. His Memorial Service is scheduled for this coming Saturday in Sumner. I will be attending, hopefully with Steve. It is a difficult day for childcare and so Steve might be needed at home with the boys. I am not looking forward to going alone but must be there for Kyle. I also need to mention that a Hurler baby named Brittney (www.caringbridge.org/nc/brittney) passed away on December 30TH. She was about a month post-transplant at Duke and was having the same gallbladder problems as Jacob had during his transplant. She was in surgery to have a drain placed (same as Jake had done, sadly she didn't make it through the procedure. Her family is having a very difficult time with this loss and could use your thoughts and prayers sent their direction. We feel such an incredible bond with other Hurler families and feel their losses very deeply.

Friday, December 20, 2002 at 10:41 PM (PST)

DAY +212

One year ago Jacob was diagnosed with Hurler Syndrome and so the journey began... I can remember every detail of the week prior to Christmas 2001 like it happened yesterday. There are so many times when I just wish I could block it out of my mind and then maybe it wouldn’t be true but as we all know that’s not possible. The first time the geneticist saw Jake she knew he had an MPS disorder, she thought either Hurler or Hunter Syndrome, but wasn’t 100% sure. Of course Steve and I had never heard of either and didn’t really believe she could have any clue just by looking at him --- we needed something more concrete. The urine test came back positive for MPS but a blood test was still needed to confirm this and pinpoint the exact disorder. Steve and I were still not convinced --- we needed the blood test to know for sure. The Friday before Christmas, December 21, 2001, the geneticist called and confirmed that our 17 month old baby had Hurler Syndrome. We were told that he had 10 maybe 15 years to live. There was no cure. There was the possibility of some very dangerous treatments. Then we were told that we would need to get our 4 month old baby, Matthew, tested because he could have it as well. This was truly the worst day of my life. We made it through Christmas, just barely, had Matthew tested immediately and thankfully found that he was a carrier but not affected by Hurler’s and started researching a way to fix our son. If you’ve been following Jacob’s journal then you know the rest… I’m sure many of you have similar stories of your children’s diagnosis and I know that you can completely understand the devastation.

This Christmas is going to be so different. Although not ‘cured’ of Hurler Syndrome, Jacob is healthy (for a child 7 months post unrelated umbilical cord blood [stem cell] transplant can be) and happy. He has received a treatment that is as close to a cure as possible and we have high expectations for a long, healthy, happy future. The weight that was smothering me last Christmas is so much lighter this year. No, it’s not gone completely --- someday maybe it will be --- for now though it is manageable. Jacob has beaten the odds and is thriving. His development is excelling and his weekly doctor’s appointments result in good news. We continue to deal with side effects of his transplant and it is a slow road we are on but, nonetheless, we are heading in the right direction.

We hope you all have a blessed, peaceful Holiday. We ask that you continue to keep our friend Kyle (www.caringbridge.org/wa/kyle) and his family in your thoughts as he continues to struggle with the side effects of his transplant. Thank you all for checking in on Jacob and keeping him in your thoughts. We would not have made it through the past year without the support of our family and friends.

Happy Holidays!

Love ~ Melanie, Steve, Jacob, & Matthew

Sunday, December 08, 2002 at 9:25 PM (PST)

~~~~~~PICTURES HAVE BEEN UPDATED~~~~~~ (Hope you enjoy them, Alyssa!)

DAY +200!!!

It’s hard to believe, 200 days ago Jacob received his life-saving transplant...it feels both like it was just yesterday AND like it was a lifetime ago. Is that possible? I guess so...it’s how I feel. :-)

We had a very nice, low-key Thanksgiving celebration. Just Steve, the boys, my sister, and I --- Jacob cannot be around bigger groups of people for awhile yet. I cooked my first turkey and all the fixings which was pretty fun and amazingly everything seemed to turn out well!

Jake thankfully continues to feel pretty good despite the cold/nasal congestion that seems to be hanging on --- unfortunately, I have a feeling it is going to be around most of the winter months! His skin is also quite sensitive to the climate this year as well as the carpeting so we keep him well moisturized and try to keep his legs covered so he doesn’t get too much ‘rug burn’. His therapies are going incredibly well and it appears to me that he is developing by leaps and bounds! He has a list of 20-25 words that he uses and he has really gained a lot of strength in areas of his body that were weakened from both the Hurler Syndrome and the extended hospital stay. The therapists are here about 17-18 hours per week working with Jake and I think this is making a big difference.

We have some preliminary results from Jake’s 6-month studies lab work --- it showed that Jake’s immune system is still pretty much non-existent which means he is still not able to fight anything (germs, infections, etc.) on his own. One area did show that he has a higher concentration of immature antibodies which apparently means he is in the beginning stages of starting to grow mature antibodies which means his body is trying to bring his immune system back, but as of yet --- nothing. Hopefully, this means when he is checked at 9-months he’ll have a bit of an immune system and we won’t have to be quite so cautious about everything. We are still waiting for more results from his lab work on his enzyme level and chimerism (donor cells). Next week he will have his ECHO, brain CT, and chest and spine x-rays as part of his 6-month studies. He also will be going under general anesthesia (which I’m not too thrilled about) for his BAER hearing exam. We will then know if he still needs hearing aids or if his hearing has improved or weakened since his transplant. I will be sure and update when I have results on all of these things.

Please continue to pray for Kyle and his family (www.caringbridge.org/wa/kyle) as they continue to deal with a very tough medical situation. And, all our other friends in North Carolina and the surrounding areas as they deal with very difficult weather situations. I can only imagine how hard it is to do ‘day-to-day things’ with no power, icy roads, etc.

Happy Holidays!

Love ~ Melanie, Steve, Jacob, & Matthew

Tuesday, November 26, 2002 at 03:20 PM (PST)

DAY +188

Hello Family & Friends! All I can say is, “WOW we sure have been busy!” Steve has been very busy with work (shameless plug: if you’re in need of any floor covering installation, i.e. carpet, vinyl, laminate, etc., give us a call at 360-654-1996) and the boys and I have been super busy with appointments, both with the doctor & therapists. As a matter of fact, I am currently waiting for noon to roll around as I am to participate in a conference call with Jake’s therapists, case manager, insurance company, & Dr. K (if she can make it as she’s on vacation). Jake and Steve are on their way back from Seattle Children’s as Jake had to have his blood drawn down there today --- they drew some of the blood that is needed for his 6 – month studies which will then be sent to Duke. We also have a ‘Rehab Specialist’ coming today to work with Jake, this person is part of his therapy team and will work on the things his main therapists have set-up. We haven’t met her yet but I’m sure she’ll be great and she is scheduled to come to our home 6 hours a week which I think is wonderful! His O.T. spends a couple hours a week with him --- P.T. & Speech spend 3-4 hours per week each with him...so...as you can tell much of our time is spent working on Jake’s development. This is all to ensure that he will reach his full potential and I must admit my expectations are pretty high. He is doing so incredibly well thus far I really don’t see why they shouldn’t be!!! :-)

Medically, Jake is doing pretty well. His lab results continue to look great. He is scheduled to have his ECHO, x-rays, CT, & PFT during the next couple weeks as part of his 6 – month studies and I’m sure they’ll all be great as well. His nasal congestion and cough continue which is not fun for him. He had a respitory viral battery done on Friday to rule out viruses and all was negative (infection has been ruled out at this time as well) so there is nothing major wrong --- just some cold symptoms that are, unfortunately, hanging on. This really doesn’t surprise us --- with no immune system cold symptoms are bound to stick around. He also had an outbreak of GvH rash over the weekend but we caught it right away, restarted the creams and ointments, and appear to have gotten things under control once again. My hopes are that this rash stays gone so we will continue with the creams & lotions for the time being.

I hope those of you in our local Seattle area were able to catch the health segment on KIRO Channel 7 5PM News yesterday evening (I wanted to post something sooner but didn’t know exactly when it would air). It was about Dr. K and unrelated umbilical cord blood transplants on children with Sanfilipo Syndrome. They interviewed two of our friends the Sarantinos’ (www.caringbridge.org/wa/luke) and the Cottey’s (www.caringbridge.org/wa/kyle). Sanfilipo is another of the MPS disorders (like Jake’s Hurler Syndrome) and they have just begun to perform transplants on children with this genetic disease. I think Luke is the 3RD child transplanted and Kyle is the 4TH. Kyle is still at Duke and is experiencing some problems (to find out more, please check out his website) --- he and his family could use your good thoughts and prayers sent their direction.

Just two more days ‘til Thanksgiving and we have so much to be thankful for this year! First and foremost, we are all happy and healthy --- including Jacob --- truly what more could we ask for? The other big ‘thank-you’ is we are together as a family and HOME! We are certainly saddened by some of the things we and our ‘transplant and Hurler’s families’ have endured, especially Jacob the past 8 months, BUT are so thankful for the people we have met and many of the new experiences shared. We have definitely gained some insight and a new understanding. We are so truly blessed and thankful for these things and I feel it is important to share these things with everyone we know.

Happy Thanksgiving to you all!

Love ~ Melanie, Steve, Jacob, & Matthew

P.S. I will get some new pictures posted in the next couple days --- sorry about the delay!

Sunday, November 17, 2002 at 11:52 PM (PST)

DAY +179

Well, I’ve got quite a bit of Jacob news to report this week. First and foremost, we (Jake and I) will NOT be flying to Durham tomorrow. I started to get a bit worried about it at the end of the week and talked to Dr. K a couple of times. Jake has been dealing with some nasal congestion for the past couple weeks. On Thursday he started getting a cough and now his congestion has worsened. He does not have a fever and all the drainage has been clear besides first thing in the mornings which are all good things...but...we were really starting to get concerned about flying cross country with Jake in this condition. Dr. K understood our concern and ‘let us off the hook’ if we promised to be there for his 9 - month evaluation in February 2003. I waited until today before I made a definite decision in the hopes that Jake would get better but if anything he’s gotten worse so our ‘trip’ to North Carolina is officially cancelled. I am both happy and sad about this. It is nice that I don’t have to expose my child with no immune system to dirt, germs, and unknown illnesses (especially when he already appears to be fighting something) but it is a little scary that Dr. K won’t be seeing Jacob now until February. She is our expert on both Jake’s stem cell transplant AND his Hurler Syndrome and it is a little scary not having her in the picture for such a long period of time --- we’re quite attached! She continues to be completely available to us by telephone and e-mail which is wonderful. Anyhow, as far as Jake’s cold symptoms go --- we just have to continue to keep our eye on him and make sure he doesn’t get a fever. If he gets a fever, we’re off to the hospital so we want to avoid this at all costs. Dr. K is going to contact our Seattle doctor, Dr. Hawkins, to schedule all of the testing he needs at the 6 month mark and I will let everyone know how it goes.

Therapies have officially been started! We are very excited about this...Jake now has physical therapy (PT), occupational therapy (OT – for fine motor skills), & speech therapy twice a week --- 6 sessions total. He appears to completely adore all his therapists and they seem thrilled to be working with him. I think it’s all going wonderfully. Jacob is such a little show-off when the therapists are here not to mention Matthew who thinks he’s the center of attention. The therapists all include Matthew in everything which makes it easier on me. I personally just love to watch Jake overcome his developmental delays and surprise us all with his abilities!

Steve and I took an evening off last week (thank you to Grandma & Grandpa Irish!) and went out to dinner and did a little shopping. It was very nice to be back in the world of adults for a few hours. We were able to celebrate Steve’s birthday early...today is the actual day (Happy 31ST Birthday, Daddy!).

We want to thank everyone immensely for the donations to Jacob’s medical expenses account through COTA. This helps more than you can ever know. There is a link at the bottom of the page for information on how to donate or you can contact my Mom (Grammie Cindy) at 425-252-2066. We want to send a special thank you to Margaret for raising awareness and funds for Jacob at her Retirement Party. We also want to send a very special thank you to Alyssa, a very special young girl. I inadvertently found out that she asked for donations for Jacob instead of gifts for her 6TH Birthday Party! We find this so amazing and are so honored to know such a wonderful girl. The kindness of everyone during this difficult time has been incredible. We are blessed with incredible family and friends and appreciate all that you have done for us!

As we approach this holiday season please take a minute to remember all you have to be thankful for..........................it is something that I now do on a daily basis.

Love ~ Mel, Steve, Jake, & Matt

Saturday, November 09, 2002 at 01:10 PM (PST)

DAY +171

Jacob continues along the road of good health. He had his transplant 5 ½ months ago and we think he is doing great considering ALL he has been through. We will have a more ‘scientific’ look at how he is doing November 19-21. These are the 3 official dates for his 6 month post-transplant evaluation. Jacob and I (and MAYBE Daddy) will be traveling back to Duke & Dr. K. for these appointments. We expect to leave Washington on Monday the 18TH and return home the evening of Thursday the 21ST or Friday the 22ND. I have given all of this information to the organization (Corporate Angel Network) that helped us find the private flight home from N.C. at the beginning of October and am hoping they might be able to help us out again. Please keep your fingers crossed this works out for us again!!! Jacob continues to be very immune-compromised which is expected at this stage of the game, but, I just hate to put him on a commercial flight during ‘cold season’ in his condition. I know that expecting him to wear a mask the entire time 7 -8 hours is completely unreasonable so...we’ll just keep hoping for a private flight!

Jacob’s GvH rash seems to have cleared up which we are just thrilled about. He saw Dr, Hawkins for his routine weekly appointment yesterday and he thought Jake’s skin looked great. I think it was his dry skin that brought it on so we continue to keep him well moisturized but were able to discontinue the steroid creams. We also were able to wean his steroid dose by a small amount yesterday. All good things! The other medication change we’ve made in the past week was to lower his Methadone dose a small amount. He should be off the Methadone completely by mid December which will make me feel less like we have a little drug addict on our hands... :-) It makes us very happy to see some of his medication doses going down as this gets us closer to bringing back his immune system (the steroids, FK506, & CellCept are all immune-suppressants). Jake is now receiving 9 oral medications morning and night --- some of you might remember how I was so proud of how great he was at taking them --- unfortunately that is no longer the case. It has turned into quite the struggle, almost worse than it was at the beginning! So, as you can see, we can’t wait for these medication decreases to happen.

In other Irish Family news, Matthew continues to act as therapist for Jake. The two of them are like little wild men all day long. They’re running me ragged --- but I love it!!! They have their moments but for the most part they adore each other. They kiss and hug and hold hands as they walk through the house together. It’s pretty adorable. They also bite, kick, hit, and push each other around --- not so adorable! We truly couldn’t ask for two better little boys though... :-)

In closing, please remember to hold your children close. They are a gift...

Love to all ~ Melanie, Steve, Jake, & Matt

Wednesday, November 06, 2002 at 11:50 PM (PST)

Once again, it has been a tough couple of days. Jacob is doing fine, but on Monday we lost another dear friend. Andrew (www.caringbridge.org/md/andrewlee) has lost his battle not with his luekemia as he was 100% donor cells but with his weakened body due to both his disease and his transplant. We are so incredibly saddened by this as we felt such a strong bond with Andrew and his family. They were the very first people we met when my Mom, Jacob, and I arrived at Duke Hospital and Steve had many conversations with Andrew's Daddy while we were on the Unit together so we ALL felt a special connection. I have to say that the loss of Amanda a couple weeks ago and Andrew a couple days ago has been absolutly the worst. We were very close with them both and although they did not share Jacob's disease they all went through the same thing as far as their transplants. We do believe that Andrew is free from his suffering which is a blessing. Please say a prayer for his family this week as they are dealing with something no parent should have to deal with. Thank you...

Love ~ Melanie, Steve, Jacob, & Matthew

Thursday, October 31, 2002 at 03:49 PM (CST)

DAY +162

HAPPY HALLOWEEN TO ONE AND ALL!!!

Well, the boys are dressed in their orange Halloween t-shirts and are enjoying their Halloween thus far. My Dad, Grammpie Mark, is coming by after work to pick Matthew up as he is going to spend his Halloween evening with him & my Mom, Grammie Cindy. He might even get to go trick-or-treating at a couple places even though he doesn’t really have a costume this year. Jacob will be staying home with Steve and me and spending a quiet evening together. We are planning to keep the porch light off as even answering the door to trick-or-treaters could be potentially dangerous for Jakie. My hope is that next year all will be different and I will be taking my two little ‘goblins’ trick-or-treating around the neighborhood and answering the door to hand out tons of candy! It’s truly a small price to pay to keep Jacob healthy though!

Jacob continues to do pretty well. He still has some GvH rash which we are treating aggressively with his oral steroid, keeping his FK506 at the right level, and applying several different creams & ointments 4 + times per day. The poor little guy is slippery he’s so lubed up! Because it is necessary to keep his steroid, FK506 (chemo-like med.), and Cellcept (immune suppressant) levels up due to his rash, his immune system is still being significantly suppressed. He does continue to receive IVIG once a month which is an immune system booster as well as his assorted prophylaxis medications. Steve and I are getting our flu shots next week and we are just hoping that with his prophylaxis medications and us doing everything we can to keep illness away from him he will remain cold & flu free throughout the winter months.

In other Jacob news, we continue to pursue therapy for him. As of yesterday, I think I’ve found a place that is willing to come to us in our home AND provide the full amount of therapies Jacob needs. We will be meeting with someone from this rehab organization in the next couple of days and hopefully get things started up as early as next week. For the time being, Matthew acts as interim therapist!!! It is truly amazing what both boys will do as long as they’re together.

Big Halloween hugs to all of you from Jacob & Matthew!

Love ~ Mel, Steve, Jake, & Matt

P.S. Please keep our friends the Lee's in your thoughts. We met them the very first day we spent at Duke (April 1, 2002). Their son Andrew had his transplant before Jacob, returned home the same week as us, but has now had to return to Duke due to some complications. He is having a pretty tough time and I just know they can use the extra prayers and good thoughts sent their direction. You can learn more about Andrew on his website: www.caringbridge.org/md/andrewlee

Thursday, October 24, 2002 at 04:47 PM (PDT)

DAY +155, approx. 5 months post-transplant…

So sorry about the delay in updating Jake’s site! I know that there are some of you out there still checking in on him…especially our friends outside of our ‘home’ area. Hope this journal entry finds all of you healthy & happy and thank you for sending your prayers and good thoughts to Amanda’s family. They certainly can use it during this trying time.

Well, we have BIG Jakie news --- Jacob started WALKING INDEPENDENTLY this past weekend!!! We are SO proud of him and happy about this as it will help so much in his overall development. Walking independently is going to strengthen many of the areas in his body that are weakened from his Hurler Syndrome and will greatly improve his gross motor skills. I can’t wait for Dr. K and Dr. Escolar back in North Carolina to see him --- I just know they will both be happily surprised by his progress. He has this adorable little shuffle and is so steady on his feet! Besides the walking everything else continues along normally. We are only going to Children’s once a week on Fridays to get labs drawn and see Dr. Hawkins. I draw labs Tuesday mornings and drop them off at Providence Hospital in Everett. This makes for a much shorter drive on Tuesdays and no need to find a baby-sitter for Matthew and Jake’s labs always look so good that he never needs to go in for anything (i.e. blood, platelets, IV mag., etc.). We hope this wonderful trend continues. As far as his therapies go…we are still trying to get these set-up…he apparently needs more therapies than the home therapy organizations in our area are able to offer us so we are trying to get something worked out. Dr. K and Steve and I are adamant that he get his therapies on our home as taking him to an outside program could be incredibly dangerous due to his non-existent immune system. We’ll continue to plug away on this until we get what we want!

We want to wish Steve’s brother and his long-time girlfriend/fiance’ (10 years!), Uncle Chris & Aunt Rene’ CONGRATULATIONS on their marriage! We had a busy week last week with the rehearsal, rehearsal dinner, & wedding…not to mention getting all dressed up which none of us have done in such a long time! My wonderful Dad, Grampie Mark spent Thursday evening and half the day and most of the night Friday taking care of Jacob for us as he, unfortunately, could not go to the wedding (way to BIG a group of people for Mr. Jake to be around). Both of my parents, Grampie Mark and Grammie Cindy, had planned to take care of Jakie together but my Mom came down with a horrible cold the day before and therefore could not be around Jake so my Dad managed on his own! Way to go Grampie Mark! It is tough to keep up with a 2 year old!!!

Thanks again for checking in on Jacob!!!

Love ~ Mel, Steve, Jake, & Matt

Sunday, October 20, 2002 at 04:27 PM (PDT)

Jacob is doing just fine. I have been struggling to put together a post on him and us but just can't do it right now. That will have to wait for another day. We'd like to ask that you all say a prayer and send your good thoughts to our friends Kathy and Gary. Their beautiful daughter Amanda went to Heaven yesterday. Our hearts are truly broken for them. I have mentioned Amanda and them in some of Jake's past entries and want you all to know what wonderful people the Massey Family is and how saddened we are by this news. Please keep them in your thoughts as we will. I've posted it before but will do so again, Amanda's web address is www.caringbridge.org/nc/amanda.

Love ~ Melanie, Steve, Jacob, & Matthew

Thursday, October 10, 2002 at 02:16 PM (PDT)

Jacob is settling into his ‘new’ home life wonderfully. His days are spent playing with his brother (and Mommy & Daddy) and all his new (old) toys. We’ve had a few visits from healthy friends and family and of course Jakie basks in the attention! We brought his dog, Sampson, home Tuesday evening and he loves watching him play in the yard. Sampson is now an outdoor dog due to the necessity for cleanliness. We can’t begin to thank my cousin and her family (Tiffany, Kyle, Alex, Nick, & Sam) for taking such good care of Jake & Matt’s dog for the past 5 months!!! We owe you guys big time --- make sure and let Steve know when you need him!!! Sampson is getting used to being here again and his new outdoor home --- thank you to our neighbors for bearing with the barking --- he’s just getting used to his new environment and seems to settle down more with each passing hour.

Jakie had his first appointment with his Seattle doctor this past Tuesday, Dr. Hawkins at Children’s. He seems very nice and competent which is a relief. We have also been assigned a nurse practitioner as well to help us with Jake’s needs. Everything went great with his Tuesday appointment, his labs look good and his exam went well. His weight is up a bit to 13.1 kilos (a little over 29 lbs.) so he is getting pudgy! His face is so cute and chunky from his steroids. I have to say I am thrilled he is starting to walk more because he is getting HEAVY to carry around all the time! Steve says he looks like a little linebacker from the waist up! He still manages to totter around on his little chicken legs though! Anyhow, he will have labs draws twice a week for the next couple weeks and exams by Dr. Hawkins once a week (Tuesdays & Fridays). We also were able to schedule and appointment with the audiologist at Children’s for November 1st so hearing aids are in the near future. We really expect and hope this will help with his talking and verbalization.

For those of you who are keeping up with the local media…the Marysville Globe ran a little blurb on our homecoming yesterday (Wednesday - 9/9/02). They had forgotten to include the fundraising information in the first article they did so they wanted to get it in this time. There is a picture of Jakie’s first nap at home with the article which is pretty cute!

Love from Washington ~ Melanie & Steve
XOXOXOXOXOXOX ~ Jacob & Matthew

P.S. PICTURES ARE UPDATED!!! Check them out --- I know I’m a ‘little’ biased but they’re pretty cute!

Sunday, October 06, 2002 at 10:32 AM (PDT)

DAY +137

We made it home safe and sound! Steve, Jacob, and I had a wonderful flight from Charlotte, NC to Seattle, WA. I have to say I think we are truly spoiled now...your own private plane is the only way to go! We were met in Charlotte by Weyerhaeuser’s two pilots and Mr. Bill Baird a Weyerhaeuser V.P. They were all so incredibly wonderful to us, showing such interest in Jacob and making our flight as comfortable as possible. We can’t thank Corporate Angel Network and Weyerhaeuser enough for this opportunity and Mr. Baird for letting us impose on him during his flight home. Jakie loved not having to wear his mask as well! Grammie Cindy, Auntie MarMar, and Matthew met us at the Weyerhaeuser hangar and it was so great to see all of them especially Mattie although I think he is a little mad at his Mommy. He pretty much wanted nothing to do with me until today. He has warmed up to me more and is actually coming to me for comfort now as well as Daddy (Friday & Saturday all he wanted was Daddy). I have to say I was a little upset but understand. I just have to work a little harder with him now --- he’ll like me again soon!!! Matthew is walking all over the place which he was not doing 5 weeks ago when he was in North Carolina. He is such a big boy although he has somewhat of a ‘drunken sailor’ look to his walk. It is adorable! And, just like Dr. K said would happen, Jacob is starting to walk more independently as well after only two days of being around Matthew! He has taken 7 or 8 steps on his own and he is totally steady on his feet (no ‘drunken sailor’ look to him!). It is just a matter of time with him being around Mattie that he starts walking --- it is really very exciting to see these developmental milestones in both of the boys!

Health-wise Jacob continues to do really well. We’ve had a little diarrhea issue the past couple days and are going to bump up his steroid for a couple of doses to see if that does the trick. Dr. Kurtzberg is truly an e-mail/phone call/pager away and is helping take care of Jakie from afar. We don’t have an appointment scheduled yet with his doctor at Children’s but anticipate this will be on either Tuesday or Wednesday. Hopefully I will know more on that tomorrow.

We are back in the ‘normal’ world again and so far so good. Our family, friends, and neighbors have been so wonderful to us throughout the past 6 months and continue to be there for us now. Both the inside and the outside of our house looks great thanks to our parents and Steve --- everything is just perfect for Jake and for this I am so thankful. Jacob and I haven’t really left the house yet and I think this is fine for now. I know the first couple times we take Jake to Clinic in Seattle will be a little stressful. It always is when we take him out though! Tonight my parents and sister came for dinner and it was so nice to get together as a family again after so long. I have also been able to talk to several friends and family members on the phone that I wasn’t able to talk with as much as I would have liked over the past 6 months.

We want to thank everyone who has donated to Jacob’s COTA account. This money goes directly to Jacob’s transplant expenses and is VERY appreciated by all of us. I want to send a special thank you to Luci at Nordstrom’s for organizing the bake sale for Jacob and Bayside Marine for keeping the canister out on the counter and keeping it full of money for Jake! If you are interested in finding out more on how to donate you can follow the COTA link at the bottom of this page or call Cindy Wellman at 425-252-2066.

Lastly, we miss many of the people we met in North Carolina very much. We want to send our thoughts and prayers to Amanda (www.caringbridge.org/nc/amanda) right now and ask that you do the same. She is having a really tough time of it and her and her family holds a special place in our hearts. We are thinking of you Amanda and praying that you get better very soon!

All our love ~ Melanie, Steve, Jacob, & Matthew

Thursday, October 03, 2002 at 11:02 PM (EDT)

DAY +134

Our bags are packed, the apartment is empty and clean, WE ARE COMING HOME! It has been 6 months and 4 days since Jacob and I have been home...I truly can’t believe it’s been that long. I just know Jakie is going to be as thrilled as I am once he sees his house, room, toys, BROTHER, family, etc. We will be leaving Durham around noon tomorrow, catch our flight in Charlotte, NC at 5:45pm and arrive at SeaTac at 7:50pm. My Mom/Grammie Cindy, Dad/Grampie Mark, sister/Auntie MarMar, and MATTHEW are meeting us at the airport! As excited and happy as I am about coming home --- I am so incredibly sad to be leaving all of the wonderful friends we have made here in Durham. We want to thank you all for making us feel so welcome. Jacob couldn’t have had better doctors, nurses, nurse practitioners, ALL the staff at Duke Hospital and the Children’s Health Center. We have met so many wonderful families going through this transplant process with us and believe we have made lifelong friends. We also have to thank Jacob’s incredible ‘Best Buddy,’ Stacy who we couldn’t have done without. She stuck with us throughout this whole process and helped in every way possible (you’re great too Stu)! So...like I said...we leave North Carolina with great happiness and some sadness as well.

There are some guidelines or ‘rules’ if you will that need to be followed upon our arrival home to ensure Jacob’s continued health & wellness. We ask that you all follow them as Jacob has no immune system to speak of and must be kept in a germ-free environment.
-----> Jacob can have visitor, 2-3 people at a time, no large groups.
-----> No kids who are in daycare, pre-school, or elementary school are allowed to visit Jake at this time (I hate this rule but children often times pick things up in those settings that if Jake got could kill him!).
-----> Anyone coming to visit Jacob MUST be healthy. If you have been sick or felt ill in any way in the past 48 hours do not visit us.
-----> No smoking.
-----> No pets.
-----> When you come to our home, please be prepared to leave your shoes at the door and wash your hands thoroughly throughout your visit.
-----> Please do not send/bring us any fresh flowers or plants. They must remain outside.
-----> No latex balloons, Mylar are OK.
Thank you for your willingness to follow these guidelines where Jakie is concerned. They’re for his safety and protection.

I’m off for now but will be SEEING many of you very soon!

Love from Durham, North Carolina ~ Melanie, Steve, & Jacob

Tuesday, October 01, 2002 at 09:32 PM (EDT)

DAY +132

The clock is ticking and we’re getting closer and closer to coming home!!! Have you ever noticed how time seems to slow down when you get anxious!?! I am learning a lesson in patience here --- 3 days to go!

Jacob is doing great. All his home medications are now taken orally and he seems OK with this thus far. He had his last Clinic visit yesterday for his IVIG infusion and a magnesium infusion as his mag. was a bit low...so he is ‘medically’ done here at Duke for the time being. We don’t even have to draw anymore labs until we get home!

Grandma & Grandpa Irish left this morning for Washington with all our stuff. Jacob was so sad to see them go and a little fearful that they were taking his Daddy with them...when he realized that wasn’t happening he was much better with the whole situation. They are staying in Knoxville tonight and Nashville tomorrow night for their wedding anniversary. Happy Anniversary Mom & Dad a.k.a. Grandma & Grandpa Irish!

This afternoon Dr. K had us stop by the Clinic so she could see Jacob. Part of the reason she wanted him and us there is that 60 Minutes II is here doing a story on Dr. K and unrelated umbilical cord blood transplants for metabolic disorders. Jacob fits in this category and is doing so well (and is so adorable!) that Dr. K wanted them to see him. The 60 Minutes II people were able to get some great film of Jakie and his buddy Lukey playing together and some of him walking down the hallway pushing his ‘truck walker.’ They also filmed Dr. K and I talking and a few other things. They have our contact information and might want to pursue us further. My hope is this gets more information out about metabolic disorders like Hurler Syndrome, Sanfillipo, ALD, Krabbe, etc. and what cord blood transplants can do for our kids. We had to do our own research to find Dr. K and Duke but maybe others won’t have to...Dr. K always says, “knowledge is power” and I couldn’t agree more. Anyhow, I’ll be sure to let you all know when this is going to air.

Hope all is well with all of you. Matthew, we ALL miss you horribly and can’t wait to see you again...so glad you are having such fun with Grammie Cindy & Grampie Mark. We’ll see you soon!

Love from NC ~ Melanie, Steve, & Jake

Sunday, September 29, 2002 at 09:59 AM (EDT)

DAY +130

Daddy, Grandma Patty, & Grandpa Steve have arrived. Jacob and I picked them up at the airport yesterday morning and are so happy to see them. Jakie warmed right back up to Daddy even though he hasn’t seen him for a month. Now he won’t let him out of his sight...I think he’s afraid Steve’s going to leave him again! Our family is ALMOST whole again --- just 6 days to go! Our missing piece, Matthew, is having a great time --- spending the whole week with Grammie Cindy & Grampie Mark. Grammie Cindy took the week off from her work so she could be with him 24/7. Thank you so much Mom & Dad for taking care of our little guy for us!

Health-wise, Jacob is doing remarkably well. He continues to take his oral meds with little to no complaint and his IV meds don’t even faze him. We had our big discharge meeting with Dr. K & Jennifer on Friday evening (Jayne popped in to say hello as well). It was exciting and a little scary for me. We went over everything we need to know to effectively take care of Jacob’s needs at home. Dr. K will keep in contact with both us and his new doctor at all times which is a huge relief to me. She made some medication changes and took Jake off all his home IV meds as of Monday (tomorrow) and switched them to oral. So, we’ll have more oral medications to give him but no IV. He’ll continue to get IVIG & Pentamadine monthly in Clinic and will need to see his doctor at home (at Seattle Children’s) weekly for lab draws & check-ups. We will continue to flush his line daily & change his dressing every few days at home. Dr. K also stressed the importance of therapy several times a week --- so we will be scheduling speech, physical, & occupational therapies as soon as we arrive home --- these will all need to be done in our house due to Jacob’s non-existent immune system. Therapies are the only way Jacob will ever get up to speed developmentally so they are our main goal at this point in time. So --- we have basically been released by Dr. K and are free to leave for home on Friday --- we are expected back at Duke on November 19TH for a few days for Jake’s 6 month evaluation --- Dr. K will continue to follow Jake’s progress for years to come. I feel good about all of this as we have a ‘plan’ in place...this will be a smooth transition!

The Herald ran a story on Jacob in Friday’s paper. It was a very nice story and can be found on the front page of the local section (Julie Muhlstein’s column). If you didn’t get a chance to see it in Friday’s Herald. It can also be found at www.herladnet.com. You have to scroll down the page a little and click on the ‘Columnist Julie Muhlstein’ link OR follow this link, www.heraldnet.com/Stories/02/9/27/15869197.cfm, directly to the story (Sorry, you have to actually type it in the navigation bar --- I used to know a little html but can’t remember it anymore!).

Well, I better get to packing. Grandma & Grandpa Irish will be leaving Tuesday morning to drive our vehicle home loaded with all the belongs we’ve accumulated for the past 6 months here in Durham. Thank you so much to them for getting this stuff home to us. We hope you have some fun on the way!

Love ~ Melanie, Steve, & Jake

Thursday, September 26, 2002 at 12:36 AM (EDT)

New photos have been posted to the photo album so if you get a chance, check them out!

Also, wanted to let everybody from home know that the Marysville Globe that came out today (Wednesday) has an article about Jacob in it and I believe, Friday, The Herald is running an article on Jacob. The Marysville Globe article is not viewable on-line unless you have a subscription to the paper and I'm not sure if The Herald article will be available on-line. I'll be sure to let you know if it is. Jacob's scrapbook just keeps growing and growing!

Take care of yourselves and cherish your children...

Love ~ Mel & Jakie

Tuesday, September 24, 2002 at 06:33 PM (EDT)

DAY +125

Jacob's doing really well...anxiously awaiting the arrival of his Daddy (and Grandma & Grandpa Irish) Saturday morning and the reunion with his brother (and Grammie Cindy & Grampie Mark) in about a week and a half! He got his Pentamadine and IVIG infusions in Clinic today, saw his Nurse Practitioner Jennifer, and Dr. Szcabolz(?sp) as Dr. K is out of town today. We will be meeting with her Friday evening to go over all Jake’s discharge information so not too much needed to be done today as we’ll be making necessary changes to medications as well. He only has one Clinic visit left for his final ‘North Carolina’ IVIG infusion on Monday the 30TH. He will continue to need weekly IVIG infusions for some time --- not sure if he will get those at Seattle Childrens’ or if a home health nurse will administer it in our home (I’m hoping for the second scenario). We’ll have to wait and see...

Everything is still on for an October 4TH departure and I found out Monday morning that we will be able to stay in our apartment until the 4TH so there will be no need to be uprooted prior to that date. A big thank you to Athena for offering to let us stay with her and Luke…Jakie would have loved it and we’ll come over to play before we leave for sure!

I think that’s everything new for now. Take care of yourselves. Thank you all so much for posting to Jake’s guestbook, it lets us know you’re out there, and for keeping Jake in your thoughts and prayers.

Love ~ Mel & Jakie

Sunday, September 22, 2002 at 09:19 AM (EDT)

DAY +123

Jacob continues to amaze me with how well he is doing. He has not had any vomitting or diarrhea for over a week (knock on wood as my Grandma Bernie would say), he is happy the majority of the time, and he seems to do something new every day which is big news for a child with developmental delays. He becomes more verbal and more physical as time goes by. It makes me so very happy to see. We really work for these milestones practicing things his assorted therapists have taught us over the past several months. His lab results (blood counts, electrolytes, organ function numbers) look great at each blood draw so we're only doing lab drops a couple times a week now. We are having trouble regulating one of his medications (FK506). I draw levels for this when I drop labs and he has been a bit low ever since we switched him to the oral form of the medication. He appears to have a bit of an absorption issue which I am assured is quite common in post-transplant Hurler kids so we just keep bumping his dosage up until we get him to a theraputic level. He has his moments of crankiness and can be VERY demanding...I blame this on his steroids but probably it is as much that as just being 2 years old!

We have a little change of plans as far as timing for our return home. I received a call Friday evening from Corporate Angel Network and there is a company that wants to fly us home! Yippeeee!!! Unfortunetly, they will not be in the area until Ocotber 4TH. We can't really pass up this opportunity because it is the MUCH safer situation for Jacob so we will remain in North Carolina an additional week. Jacob, Steve, and I will be flying out of Charlotte, NC (about a 2 hour drive from Durham I'm told) on Friday, October 4TH on the Weyerhaeuser corporate jet with the CEO. I'm afraid I might never want to fly commercial again! :-) Anyhow, this means that Steve, Grandma Patty, & Grandpa Steve will be flying to North Carolina from Washington on Saturday, September 28TH to help me get everything packed up...we have to be out of our apartment on September 30TH...we will then stay with friends (Athena & Luke) for those 4 days or in a hotel room depending on how the boys are feeling...AND Grandpa Steve & Grandma Patty will be driving our vehicle home to Washington with a U-haul trailer towing all our newly accumulated (and things we shipped from home) belongings. They're going to take their time --- just need to be home in time for Uncle Chris & Aunt Rene's wedding on October 18TH! This is the plan as it stands although some wonderful people at Nordstrom's (namely my sister, Auntie MarMar and Erik Nordstrom) are working on seeing if the Nordstrom corporate jet will be in the area any time sooner than the 4TH. Thank you to the White Family for recommending Corporate Angel Network (www.caringbridge.com/page/susannah)!

The last thing I want to report on is that Jake and Luke (www.caringbridge.com/wa/luke) had a great play-date yesterday. They were so adorable running around the apartment. It was just so incredibly nice to see them playing with each other as they both miss their brothers so much! Lukey is more advanced developmentally than Jakie (he's also about 7 months older) and I think this really helps Jake to see what he could be doing. Thank you Athena & Aunt Diana for bringing Luke over to play!

We'll see many of you soon!

Love ~ Melanie & Jacob

Thursday, September 19, 2002 at 08:54 PM (EDT)

I received two quotes today from friends that I feel the need to share with all of you. They really touched me as I hope they do you, both as a parent and as a parent dealing with a child with a terminal illness... The first is:

"Having children is like watching you heart walk around outside of you body." ~Eleanor Roosevelt~

This one can be found in Jacob's guestbook but I wanted to bring it out to the 'main page' for a couple days:

Most women become a mother by accident, some by choice, a few by habit. Did you ever wonder how mothers of children with cancer [Jacob does not have cancer but I feel this applies to any child with a life-threatening disease] are chosen?

Somehow, I visualize God hovering over the earth selecting his instruments for propagation with great care and deliberation. As he observes, he instructs his angels to make notes in a giant ledger...

"Armstrong, Beth, son, patron saint Matthew."
"Forrest, Marjorie, daughter, patron saint Cecilia."
"Rutledge, Carrie, twins, patron saint Gerard. He's used to profanity."

Finally, he passes a name to an angel and says, "Give her a child with cancer." The angel is curious. "Why this one, God? She's so happy."

"Exactly," smiles God. "Could I give a child with cancer a mother who does not know laughter? That would be cruel."

"But does she have patience?" asks the angel.

"I don't want her to have too much patience or she will drown in a sea of self-pity and despair. Once the shock and resentment wears off, she will handle it."

"I watched her today," said God. "She has that feeling of self-independence that is so rare and necessary in a mother. You see, the child I'm going to give her has it's own world. She has to make it live in her world and that's not going to be easy."

"But Lord, I don't think she believes in you," said the angel. "No matter. I can fix that. This one is perfect. She has just enough selfishness."

The angel gasps, "Selfishness? Is that a virtue?"

God nods, "If she can't separate herself from the child occasionally, she'll never survive. Yes, here is a woman I will bless with a child less than perfect. She doesn't realize it yet, but she is to be envied. She will never take anything her child does for granted. She will never consider a single step ordinary. I will permit her to see clearly the things I see...ignorance, cruelty, prejudice...and allow her to rise above them."

"And what about her patron saint?" asks the angel, his pen poised in mid-air.

God smiles and says,..."A mirror will suffice."
~author unknown~

Thank you so much for these beautiful quotes Kathy and Crystal.

I ask that you all say an extra prayer and send your good thoughts to Kyle and his family (www.caringbridge.org/wa/kyle). They received some devastating news today and need everyones prayers and warm words to get them through. It can be so hard here at times and this is one of the many reasons. They have found out that Kyle's first transplant has failed and have some very difficult decisions to make.

Love to you all ~ Melanie & Jacob

Wednesday, September 18, 2002 at 02:32 PM (EDT)

DAY +119

Yesterday was our big Clinic day and all went really well. We started the morning with Jacob’s little surgery. He was such a big boy. He went to the operating room (OR) without me which is a first. Dr. Kurtzberg & Jennifer (his Nurse Practitioner) went with him and Dr. K actually performed the procedure with Jenn assisting. He got a little gas and then some IV anesthesia which made for an interesting day but the procedure was pain-free which was important for me AND him. I guess when the anesthesiologists wheeled him in to the OR. Dr. K took his mask off and he waved and said “bye, bye” to it and was smiling and laughing with them prior to getting put to sleep. How cute is that!?! Anyhow, it took about 10 minutes to pull his double line and re-bandage his single line and then I went into post-op and sat with him until he woke up. It’s the first time I’ve been in before he wakes up and they still had the oxygen in his mouth and the mouthpiece that holds his tongue out of his throat in place --- pretty weird to see --- the minute he woke up he yanked that thing out and threw it off the bed! The rest of the day and last night he was pretty loopy and out of it. He’d go from being super happy and playing like crazy to screaming and being pretty unhappy...both very normal reactions to the anesthesia. After his procedure we went to Clinic where he had his IVIG and Dacluzimab infusions. If all goes well that was his last Dacluzimab infusion which is great news (Dacluzimab is for GVHD which at this point in time Jacob does not have). He will remain on the Cellcept which is also a GVHD medication for quite some time just in case his liver starts acting up again but this infusion I give at home, two hours morning and night, so no need to go to Clinic which is nice. Dr. K lowered his steroid dose and took him off one of his oral medications. As far as it stands today, we are still on target to come home the last weekend of the month! Although, we are still waiting and hoping to hear from Corporate Angel Network about a flight home…we do have a commercial flight booked for Saturday the 28TH just in case we need to use it. Please continue to keep your fingers crossed that Corporate Angel Network comes through for us!

In non-medical Jacob news, his Best Buddy Stacy stops by and plays with him almost daily and his primary nurse from 5200, Keri came to visit Monday afternoon/evening. Jacob adores the company and the attention. This morning he even had a bit of a ‘play-date’ with his buddy Lukey. We met at Athena & Luke’s home away from home (aka apartment) and drove to The Streets of Southpoint indoor/outdoor Mall. We stuck to the outdoor part before any people came around and walked the boys and window shopped. We also had a bit of ‘adult’ conversion which I miss at times! We left when the temperature started to increase and more people started to come around, about mid-morning. Now Jakie and I are back at the apartment, he’s playing with Stacy as I complete this entry. Stacy is a life-saver if you couldn’t tell already. I don’t know what I would do without her, especially while I am here alone...plus she brought me homemade chocolate covered strawberries today which are always VERY appreciated!!! I better go for now so I can join in the playing...

Love ~ Mel & Jake

Saturday, September 14, 2002 at 02:06 PM (EDT)

DAY +115

Jacob continues to do well. He is taking his oral medications morning and night without too much kicking and screaming and being hooked up to his IV medications doesn't even faze him. He has truly adapted to his situation and for this I am so very thankful and proud of him. It makes life much easier! I found out this morning that Jake will have to undergo a minor surgical procedure Tuesday morning. They are going to pull his double line from his chest. He will get a very small dose of anesthesia through his single line which will make him sleep for a few minutes and then snip a couple stitches and pull the double line right out. Amazing! It will be much easier to do dressing changes and flush only one line as opposed to two. This also puts us one step closer to coming home. Yippeeeee!

In other North Carolina news, it is pouring down rain and I am loving it. I hate to open the door though as it ruins my rainy Washington images...you see...it is like 83 degress...and you Washingtonians all know that when it rains at home it is NOT 83 degrees. Apparently, it is some sort of tropical storm --- I couldn't be happier! My friend Athena, Lukey's Mommy (see website link at the bottom of this page), is enjoying it just as much as me. We spoke on the phone earlier and she even made soup for lunch to help with the fantasy of home (they're from Seattle if you didn't already know). Simple pleasures...

Love from rainy Durham ~ Mel & Jakie

P.S. We miss you just as much as ever Steve & Matt!

Thursday, September 12, 2002 at 10:29 AM (EDT)

Jacob is feeling well...but...last night we had a bit of excitement. Jacob spiked a fever around 8pm so I had to call the on-call doctor. It was after 8pm so we had to go to the Unit (5200) to get Jake’s lines cultured to check for infection and have a bit more blood drawn to check his levels. His fever was only 101.4 but if he goes over 101 I’m supposed to call. He held on right around 101 until I got him in the car to go to the Hospital and once we got there he was totally fine! I knew when I pulled up to the Hospital all was well as he was no longer flushed and his skin temperature felt normal to me. We got up to the Unit and sure enough, when they took his temperature he was totally fine. Next time I’m just going to take him for a ride in the car! I’m beginning to think he might have been teething and got a little fever from that... He has yet to get all of his teeth due to his condition but the swelling of his gums is really starting to go down and I can see where some teeth are starting to pop through. I have to say it was kind of a fun visit to the Unit. Jake got to play with Keri, Andria, & Michelle...three of his favorite nurses (wish you would have been there too Kelly!)...and Jo, our favorite nighttime doctor from our days in-patient got to play as well. Everyone was so impressed with how great Jakie looks and he put on a real show for them! He’s such a little superstar!

Yesterday afternoon Jake had a visit from the physical therapist and she will be coming 3 times a week until we go home. She says Jake has a lot of strengths and a few areas of weakness that need to be worked on. She’s going to work with him on balance, protective reflexes, and walking independently (as well as a bunch of other things I can’t remember right now). I’d LOVE to get him walking on his own because he is getting heavy!

A special congratulations to our friends Victor & Jennifer on their new arrival. Welcome Baby Grace! There son Henry was transplanted a couple months prior to Jake for Hurler Syndrome (www.caringbridge.org/wa/henryharten) here and Duke.

Love to all ~ Melanie & Jacob

Tuesday, September 10, 2002 at 04:55 PM (EDT)

DAY +111

WE’RE COMING HOME!!!!!!!!!!!!!!! I’m euphoric (I don’t think I have ever used that word in my life)! I am trying not to get too excited, as everyone who has been through this knows, things can change at any second...BUT...if Jake continues to do well and tolerates the medicine changes Dr. K is making over the next couple weeks, then we will be home by the end of the month. She will see him for her final examination of him on the 26TH or 27TH of September and we will be able to fly home the weekend of the 28TH. Yippeeee!!! It’s hard to believe that this time has finally come. There are many families who have been here much longer than us so I feel very blessed that Jacob is now doing so well. We don’t have a flight arranged yet, but, I am working with the people at Corporate Angel Network and am hoping they will be able to get us home. This is an organization that works with corporations with private jets so we would basically be hitching a ride with some CEO’s or something. There are apparently 4 potential companies that fly out of this area to the Seattle area, Boeing, Microsoft, Nordstrom’s, and J.P. Reynolds. Keep your fingers crossed that one of them comes through...this would make for a comfortable mask-free germ-free flight! Many of the kids go home with a port (access site straight to a vein) rather than central lines but because of the problems Jake had and the need for a few more medications than kids are normally sent home with Jake will keep one of his central lines. They are going to pull the double lumen out and leave the single so we will continue to flush this line daily and change the dressing weekly. He will also probably need to see the doctor Dr. K has recommended at Seattle Children’s Hospital twice a week as opposed to once a week for awhile. I am perfectly fine with this as long as we are HOME. Jacob seems to be feeling well, he vomited once Sunday morning but nothing since then so Dr. K decided to lower his steroid dose slightly today. She also changed his FK506 from IV to oral which is a big step and one less home infusion. The results of his last engraftment test were back today and he continues to be greater than 99% engrafted and there are no sign of his own cells. All in all everything is going very well.

Jacob has two new quirky little things that he loves to do around the house. He loves hiding things in drawers. I searched for my keys for 20 minutes the other morning and finally found them in the bottom drawer (closed) of the dresser in my bedroom. He also loves to go into one of the bedrooms and shut the door. He then knocks on it until I knock back. It’s his new favorite pastime, but hey, it keeps him occupied and happy!

Please send some happy thoughts to our friends Jen and Taylor in Minnesota. Taylor’s link is at the bottom of the page. Her first transplant has failed and she will need a second transplant. This means her and her Mommy will be in Minnesota through the holidays rather than home in Washington State where they belong. We’re thinking about you guys and sending our prayers and good thoughts for a successful second transplant your way!

Love from North Carolina ~ Melanie & Jacob

Saturday, September 07, 2002 at 09:25 AM (EDT)

DAY +108

I’m happy to report; Jacob seems to be doing better. The additional testing for viruses and bacteria that were done on him have come back negative thus far so...I guess we can assume all is well. Either increasing his steroid helped out whatever was going on or it just went away on its own! He hasn’t vomited since Wednesday and although he still has diarrhea it has decreased by quite a bit. He still sometimes has a strange gag-e/reflux-e (like he has a bad taste in his mouth) look to him which panics me...so I keep bowls around the apartment which I immediately shove in his face if I see this look just in case he is going to vomit. It’s actually somewhat amusing --- he shoves me away every time I get near him with a bowl now. I’m sure I’m giving him a complex!

Jacob had an EEG yesterday as part of his 100-day evaluation. He hates it when they attach all the electrodes to his head (this is his third EEG) but once they get them all attached he’s fine. He actually fell asleep for about a half hour while they had him hooked up which apparently is good for the test as it would show if he was having any seizure activity while sleeping. Anyhow, I took the opportunity to dose a little with him until the technician came in and turned all the lights up. Jake was not real thrilled with getting woken up (nor was I)! After that we went up to 5200 to visit out friend Kyle and his family (www.caringbridge.com/wa/kyle) and say hello to Jake’s primary Nurse Keri and his primary Nurse Practitioner Jennifer who is on the Unit for two weeks so we don’t get to see her in Clinic right now. Anyhow, we enjoyed our visit and if Keri doesn’t get called in to work Monday she is going to come to our apartment for a visit. Jake will be thrilled to see her outside the Hospital.

That’s all for now... We miss you all very much---ESPECIALLY Matthew & Daddy!

Love ~ Mel & Jake

Wednesday, September 04, 2002 at 10:57 PM (EDT)

DAY +105

Jake hasn’t had a very good few days. His vomiting and diarrhea have continued and actually worsened. Dr. K is running some additional tests (above and beyond the norm) to rule out viral infection, gut Graft vs. Host Disease (GVHD), and pancreas irritation. He also has a swallowing study and possible upper GI scheduled for next week. He has always had a bit of acid reflux but we need to get an idea of the severity of it. She increased his steroid yesterday due to these issues. Hopefully, we’ll get somewhere with these tests and the steroid increase as vomiting & diarrhea is not fun for Jakie or me! The problems are a bit disappointing...when I asked Dr. K yesterday when she expected Jake and I to be released out of the area...she said 3 to 4 weeks. We have to do what’s best for Jacob but I am REALLY ready to leave North Carolina. Anyhow, we’ll see how things go... Jacob did have his ABLC (anti-fungal) discontinued and was put on a new oral anti-fungal to be administered twice a day at home. This is a big step as it cuts out our Thursday and Saturday Clinic infusions and takes one med. off Tuesday’s infusion schedule.

In other Jacob medical news, we spent yesterday morning (4 hours) with the development people (Dr. Escolar and her gang of psychologists, speech therapist, physical therapists, nurses, audiologists, pediatricians, residents, med students, & interns!) at UNC. Dr. Escolar will be calling me Friday morning with the results. I think Jacob did a wonderful job with them and was such a trooper. It is a crazy evaluation with Jakie in a room with people testing him and other people watching and listening to him through one-way glass. It’s like he’s in a fish bowl for 4 hours! He also saw the orthopedic surgeon and had x-rays done on his spine and hips. His kyphosis is still there (obviously) but Dr. Fitch does not think there is any need to rush into surgery. He thinks we should wait until he’s 4 which is just fine with me. Jakie does have some hip dysplasia but in the x-rays the sockets look good so I guess that means all is well in that regard. Just one more area we’ll need to keep our eye on.

Steve has been working non-stop since he got home to get the house in order. He’s been painting, cleaning, making a play room for the boys, putting up a fence to keep Sampson in the yard, and cleaning like crazy! Thank you for all your hard work, honey! Also, the grandparents (both my parents & Steve’s parents) and my sister (Auntie MarMar) have been helping out with Matthew as well as helping Steve with the house. Thank you all so much for taking care of my baby and helping Steve out! I’m sure Mattie has been having a fun time. I hear he got a new pair of Nike’s in a size 6...Jake only wears a size 5!!! What a big boy! Jacob and I miss you all!!!

Love ~ Mel & Jake

P.S. I added one new photo in the photo album so take a look if you get a chance!

Sunday, September 01, 2002 at 09:06 PM (EDT)

DAY +102

Just wanted to do a quick post to let you all know that Jake is doing OK. We have pretty much just been hanging around the apartment together playing and relaxing. He had Clinic yesterday for a couple hours then Stacy came over and brought me dinner and played with Jakie. Isn't she great!?! Jake is still experiencing some vomitting episodes so I'm keeping an eye on this...if it continues we will have to check his weight more often and keep an eye on his electolyte levels. Other than that all is well.

Thank you for posting in Jake's guestbook! We love to know that you're out there thinking of us, especially sending good thoughts and prayers Jacob's way.

We miss and love you Matthew & Daddy!!! We'll be home soon...I just know it!

Love ~ Mel & Jake

Friday, August 30, 2002 at 02:02 PM (EDT)

DAY +100!!!

WE MISS YOU SO MUCH MATTHEW AND STEVE/DADDY!!! Just wanted to get that out of the way at the beginning... :-)

Jacob is doing really well through his additional testing. So far everything looks good although some of the results won't be in for a week or so. His pulmonary functions test yesterday showed improvement over his initial testing which was done prior to his hospital admittance --- he went from 87% intake to 94% --- which is great. This morning he had a brain CT scan and an echocardiogram. We were anticipating the need to sedate him for both of these but he is such a big boy now he just layed there calmly through both procedures. It amazes me what he is able to put up with now. He had to be sedated for both of these things prior to his hospital admission! I think he's just been through so much in the past several months that a 'little' CT scan and ECHO is just no big deal. Tomorrow he has Clinic for a couple of hours to get his ABLC infusion and then we are done until Tuesday so we have a couple days off to play --- around the apartment of course!

I hope some of you were able to check out the article in the Mukilteo Beacon --- www.mukilteobeacon.com --- or you were able to pick up a copy. Apparently, there is a pretty cute picture of Jakie & Mattie included with the article.

Take care of yourselves!

Love from Durham ~ Melanie and Jacob

P.S. If any of you want to get a hold of Steve at HOME, our new home phone number is (360) 654-1996

Wednesday, August 28, 2002 at 11:46 PM (EDT)

DAY +98

Well, it’s official...Jakie and I are on our own...only half a family again After almost 5 months, Daddy & Mattie left this afternoon with the help of Grandma & Grandpa Irish (3 to 1 were better odds, hence calling in the reinforcements for a ‘pleasant’ flight home). We are SO sad to see them go! Jakie and I will miss them so VERY much and can’t wait until we get to come home too. Hopefully, it will be within the next month...nothing for certain yet though. Jacob’s Best Buddy, Stacy, was nice enough to come over and baby-sit him so I could take everyone to the airport and extend my time with Matthew. Every little bit counts...don’t want him to forget his Mommy!

I think Jennifer, Jake’s Nurse Practitioner, wanted to keep my mind off missing Matt & Steve so she scheduled all Jake’s 100-day evaluations for the next week and a half which means we will be very busy! Tomorrow he has a huge blood draw, pulmonary functions test, & a chest x-ray as well as going to Clinic for his ABLC infusion. It will be a busier day than we’ve had in a while. This is all good with me because it just means we are getting closer to coming home and will give us some valuable information on how Jakie is doing medically. Yesterday at Clinic we talked further with Dr. K about Jake’s medication weaning schedule and in the next week or so he will be taken off two of his GVHD medications and switched from his ABLC to an oral drug that can be administered at home. Yippeeee! The fewer drugs my baby is on the better. Keep sending good thoughts Jake’s way to stay healthy so we can continue taking medications away!

Jacob is getting some local (Washington State) press. The Mukilteo Beacon did a wonderful article on Jacob in the paper that came out today. My Mom (Grammie Cindy) was interviewed and quote’s were used from this website made by me. Please pick up the paper if you’re in the Mukilteo area, if you’re not, the article can be found on their website ~ www.mukilteobeacon.com ~ under the heading “Life’s priorities change drastically for Mukilteo grandparents...”. Also, a couple weeks a go the Voice of the Valley (Maple Valley paper) included a small piece on Jacob. We actually didn't find out about this one until after the fact.

Thank you so much for your continued interest in Jacob and us - his family. We could not have done this without your support. We miss you all, especially Mattie & Daddy!

Love ~ Melanie & Jacob

Saturday, August 24, 2002 at 09:38 PM (EDT)

DAY +94

It is the week of birthdays for our family. Today, we want to wish Grandma Patty a very HAPPY BIRTHDAY! We'll see you Monday to celebrate in person! Grandma Patty & Grandpa Steve are flying here and will arrive Monday morning. They will be flying home with Steve and Mattie on Wednesday. It takes more than one person to keep track of a VERY active one year old on a six hour flight!!!

Jacob is doing well. Still having some stomach upset from his medications in the morning but the rest of the day goes smoothly. He had Clinic today...got his normal medication (ABLC) and needed some GCSF to boost up his white blood count. Nothing to be concerned about though.

We're all just trying to spend as much time with each other as possible in preparation for Steve & Mattie's departure...

All our love ~ Mel, Steve, Jake, & Matt

Friday, August 23, 2002 at 10:57 AM (EDT)

DAY +93

Today is a VERY special day for us. Matthew is 1 year old! HAPPY 1ST BIRTHDAY, MATTIE!!! I can’t believe our BABY is 1...Wow. He is such a big guy........a happy, HEALTHY one year old. Daddy is taking him to Toys R’ Us this morning to pick out whatever he wants (within reason and not too big so we can get it home, of course!) and then cake and ice cream this evening. We’re trying to make this day as special as possible for Mattie! Today is Matthew’s day! Yippeee!

Jacob is doing well. We’re still struggling with some vomiting and diarrhea issues and he did not sleep well last night at all. We think he had an upset tummy...the vomit & diarrhea this morning led us to that assumption! Other than that all is well and with the time of day he has these issues it really seems like the medications are the culprit. Hopefully, the new tummy medicine will begin working for this. Jakie had his first out-patient speech therapy session yesterday. His therapist was really impressed with many of the sounds he makes. She says he really seems to know what he wants to say, it’s just a matter of getting the actual words out of him. His hearing loss obviously doesn’t help! We are going to work on some sign language and continue introducing new sounds to him. Hopefully, when we get home-home we can work on getting him some hearing aids. Maybe this will help with his verbalization.

This has been a very sad week for everyone going through transplants. Two of our ‘neighbors’ on 5200 have passed away. Please say an extra prayer for their families during this difficult time. We will miss our precious friends.

Love to all ~ Melanie, Steve, Jacob, and the Birthday Boy - Matthew

Wednesday, August 21, 2002 at 10:31 AM (EDT)

HAPPY, HAPPY BIRTHDAY TO COUSIN NATHANIEL!!! He turns 3 years old today...what a BIG GUY! We miss you and wish we could be there for your birthday, buddy. You should be getting a little something in the mail today or tomorrow. Also, Nate, check out the photo album…the boys did something for you (with some help from Daddy). We love you!

Jacob & Daddy had their long Clinic day yesterday. Dr. K said Jake is doing well. His counts all look really good. He is still having a bit of a vomiting/diarrhea issue so his steroid has been switched around a little bit more. We’re just looking for that ‘perfect’ dose and time of day…both of these things matter. We are also going to switch his tummy medicine (Zantac) to a different one (Prilosec) in the hopes that it will help with these symptoms a little better. Steve talked with Dr. K a little bit further about how we (Jacob and Mommy) are going to get home. If we fly commercial Jacob will have to wear a mask for a full 5 ½+ hours (that’s if we drive to Charlotte to get a direct flight, longer if we fly out of Raleigh-Durham)...anyhow, we’re looking into our options! Jake’s social worker is looking into a company that might have their corporate plane flying our direction or something and would be willing to donate it. We’ll see...anyone know Bill Gates? :-)

PHOTOS ARE UPDATED!

Love ~ Mel, Steve, Jake, & Matt

Monday, August 19, 2002 at 02:31 PM (EDT)

Jacob is doing well again. Bumping his steroid back up a little helped get rid of the vommitting, tummy ache, & crying. This must have been the problem and the slight change did the trick. It's amazing how these medications Jakie is on are sustaining him in so many different ways! Him and Mattie have been playing like little crazy boys the past couple days. They are so cute...even though Jakie & Mattie are pretty much equal physically...Jakie still lets Mattie know who the big brother is! :-)

Well, it's official...Steve & Matt are flying home Wednesday, August 28TH in the afternoon. We are all sad to have to split up but know, if all goes well, it will only be for about 4 weeks. I'm trying to be strong and not be too sad about my baby leaving! Steve & I are going to try to get a night out (just the two of us) before they head home, too. We've got to get a hold of our wonderful 5200 nurses, Keri, Kelly, & Andria, they offered to watch the boys for us a long time ago and we're finally going to take them up on their generous offer! Anyhow, this is just one step closer to us ALL being home-home together.

Thank you all for continuing to check in on us and for signing Jake's guestbook. We love to hear from you!!!

Love ~ Mel, Steve, Jake, & Matt

Saturday, August 17, 2002 at 10:46 PM (EDT)

DAY + 87

Jacob is doing pretty well. We've had a couple bad mornings with irritability (2 hours of crying!), upset tummy, & vomitting. We thought it was from the steroid reduction that happened Thursday morning and Dr. K agrees, so we have bumped his steroid dose back up a bit. Hopefully he will feel better tomorrow morning as we gave him a little extra steroid this evening to get him through the night. His body isn't producing any of it's own steroids yet and this can cause these types of symptoms.

If Jacob continues to do well, Steve and Matthew will probably head home at the end of the month. Steve is going to call the airline Monday to confirm a flight date and time. We are really sad to have to do this but with Jacob doing so well, Steve really needs to get home and prepare the house for our arrival (everything has to be sanitized and SPOTLESS --- pretty mugh germ free) and get back to work. Absolutely no income for 5 months is tough but we've managed with help from our family & friends! I can't thank you all enough for your support!!! By the way, if you need any carpeting or vinyl installed, give Steve a call (like my shameless plug...just trying to drum up some business!). ;-)

Take care of yourselves...

Love ~ Mel, Steve, Jakie, & Mattie

P.S. If you get a chance, check out our friend Amanda's website: www.caringbridge.com/nc/amanda. There are two reasons I am sending you to her site...1) her and her family are very nice people --- her Mom even sits with Jake so I can use the bathroom at Clinic, and 2) she has a link at the bottom of her website which will give you a tour of where we've spent the majority of our time the past 6 months! It helps put things into perspective...

Wednesday, August 14, 2002 at 05:29 PM (EDT)

DAY +84

Well, yesterday was our super long Clinic day...we spent 8 ½ hours there. Jacob received his ABLC (anti-fungal), IVIG (immune system booster), and Daclizumab (GVHD medicine) infusions which take about 4 ½ hours and the remainder of the time we waited to see Dr. Kurtzberg. This is pretty normal for a Tuesday as Dr. K sees all her Clinic patients in one day as well as fields her phone calls from all over the world and deals with any emergencies that pop up throughout the day. It is somewhat annoying to have to wait that long with a 2 year but Jacob handled it wonderfully as usual. Just as long as he has his toys, his new Wiggles video (Ugh!), Teddy Grahams, & Pediasure...we’re good! And, frankly, Dr. K is worth the wait! We even had a few visitors while we were waiting...3 post-transplant Hurler’s patients...Hannah is one of the first (?maybe is the first) Hurler’s patient to be transplanted with unrelated umbilical cord blood about 6 years ago, her sister Cassidy 4 years ago, and Anderson 5-6 years ago. They are all adorable little girls doing so well. Hananh and Anderson are both in kindergarten – physically & MENTALLY doing so well and Cassidy is just your normal 4 year old. They were all transplanted at very young ages, Anderson & Hannah around 7 months and Cassidy at 12 weeks. Jake had his transplant at 22 months, but we have high hopes (and medical data supporting) that his outcome will be just as good as Hannah, Cassidy, & Anderson! It is one of the best feelings in the world for us to see such positive outcome from what we decided to put our baby through!

Anyhow, Dr. K made several changes to Jake’s medicine. Unfortunately, it is still looking like Jacob will need to remain in Durham an additional 25-30 days beyond the normal 100 days post-transplant required. There is just no way to safely wean Jakie from the medications he’s on in a hurry...it takes time...especially the GVHD medications he was put on while having the liver/gallbladder problems. The amount of medications he is on is just amazing. I look at the list and just shake my head! With the reductions and changes that Dr. K made yesterday, Jakie is on the following on a regular basis:
Actigall (orally twice a day – to flush things through his gallbladder & liver)
Amlodipine (orally twice a day – for high blood pressure)
Multi-vitamin
Acyclovir (orally twice a day – anti-viral)
Methadone (orally once a day – for pain)
Prilosec (orally twice a day – for tummy problems)
IVIG (in Clinic once a week – to boost immune system)
Pentamidine (in Clinic once a month – for pneumonia)
Daclizumab (in Clinic every other week – for liver GVHD)
Infliximab (in Clinic every other week – for liver GVHD)
ABLC (in Clinic 3 times per week – anti-fungal)
Cellcept (at home by IV twice a day – for liver GVHD)
Solumedrol (at home by IV once a day – steroid)
FK506 (at home by IV twice a day – mild chemotherapy type drug...immuno-suppressant)
Magnesium (orally twice a day – because he is low)
Rocephin (at home by IV once a day – antibiotic)
This is just a list of the things he gets regularly...it does not include the things he gets as needed!!! Many of these things he will be on for the next year...maybe even longer depending on how his tests turn out throughout the next year or so.

Today, Jacob had to go to Clinic again. He normally doesn’t go on Wednesdays but...........we had a little mishap this morning. I was changing Jakie’s diapers and looked at his lines and noticed the cap was missing from one of them! When his lines aren’t in use they are clamped off and they all have ‘caps’ on the ends of them for extra protection. It appears that Jake somehow managed to unscrew his cap sometime during the night. I actually found it in his crib when I went looking for it. He’s never done this before and I think I didn’t get that cap on very tight or something when I changed them yesterday. Regardless, it is like having a line break so Steve took him to Clinic to have his lines cultured for infection and to get a dose of Vanc (super power antibiotic) which is a 2 hour infusion. Oops!

I think that’s it for now! This entry is much longer than I had planned...I just get carried away sometimes. Take care of yourselves.

All our love ~ Melanie, Steve, Jacob, & Matthew

Sunday, August 11, 2002 at 11:13 PM (CDT)

HAPPY BIRTHDAY TO GREAT GRANDMA CHARLOTTE & GREAT GRANDMA BERNIE!!! Yesterday G.G. Charlotte turned 80 and today G.G. Bernie turned 90...WOW!!! What wonderful milestones to reach. Jakie & Mattie (and us) send their love and birthday wishes to you both. We wish we could have been there today for the big joint birthday party! We miss you all and hope everyone had a fun time...

Jacob continues to do well. Nothing much new to report on him...hopefully we'll have some more information on Tuesday after he see's Dr. K. Speaking of Dr. K, hope some of you were able to catch her on the Today Show last week (Monday I think). She was on the show to 'fight' an insurance company for a life-saving unrelated cord blood transplant (what Jakie had) for a family with 3 kids with Sanfillipo (in the MPS disorder family like Hurlers). It is too late for their oldest daughter...the disease is too far progressed...but, their two sons still have a chance yet their insurance company won't pay for the procedure as they say it is too risky (yet they're going to die from their disease anyhow...hmmmm...). We think it is HORRIBLE and Dr. K did a wonderful job getting her point across on the program. As you can tell, we are thrilled she is Jacob's doctor and just know he is getting the best possible care from her and her team.

Auntie MarMar leaves tomorrow and we are very sad to see her go. The boys will miss her terribly! They adore her. We all had a fun last night together though. Jakie's 'Best Buddy' Stacy and her boyfriend Stu came over and played with the boys for a few hours with us. It was great and the boys love the audience (Auntie MarMar, Stacy, & Stu!)...they're such performers!

Take care of yourselves and thank you for checking in on us...

Love ~ Mel, Steve, Jake, & Matt

Friday, August 09, 2002 at 08:04 AM (EDT)

DAY +79

Nothing new to report medically which in Jacob's case is a good thing. He continues to feel well and show improvement and stabilization on his lab reports. His platelets are hanging out around 250 which is within the normal range and his white blood count is holding between 5 and 6. All is well! Dr. Kurtzberg is trying to get his medications weened quicker than she first planned so we might be released out of the area sooner than we had hoped. We'll just have to wait and see.

In many ways Jacob acts like your typical 2 year old. He is happy most of the times and loves to play and get into mischief with his brother. I have yet to figure out who the little instigator is...I think they want it that way! They both want Mommy and Daddy's attention all the time. If Daddy is holding/playing with/paying attention to Jacob, Matthew wants to be a part of it and vis-a-versa. It is great to finally see a more normal type of interaction between the boys. They both love music...toys that play music, music on TV or the radio...they dance and 'sing'! It is so cute! They have their moments though like when they wrestle and get into little shoving matches...Jake is a bit of a biter (UGH!) and Matt is a hitter --- we're working on these things though! Mattie is starting to take steps independently (he's up to 3 or 4 at a time!) and Jakie watches him intently...maybe it will rub off on him.

We have all been enjoying our week with my sister here (Auntie MarMar). When Jake and Daddy were at Clinic on Tuesday we went to the brand new Mall in town. It's great and as many of you know --- Auntie MarMar and I enjoy the Mall! Mattie has even gone swimming at the pool once this week with his Auntie...it has finally cooled down a bit here (80's instead of 90's and 100's) so it is easier to be outside.

We hope you are all enjoying your Summer! Children are a blessing, hold them close...

Love ~ Mel, Steve, Jake, & Matt

Saturday, August 03, 2002 at 11:50 PM (EDT)

DAY +72

Grandma & Grandpa Irish left Thursday evening...it was great to have them here this past week. The boys (and us) miss you! Jacob enjoyed having them here for his birthday.

Auntie MarMar arrives tomorrow morning for 9 days! We can't wait to see her... Fly safe Auntie!!!

Jacob continues to do well and amazes his doctors and nurses with his improvement. He has not needed a blood or platelet transfusion since he was released from the Hospital. This is a huge deal...he is growing platelets (he has gone from 32 to 189 in 2 weeks) and is mantaining his hemoglobin count. It is wonderful for us to see these numbers going up and/or staying the same. This is just one step closer to us coming home!

I finally loaded the birthday pictures so take a look at the photo album *** NEW PHOTOS ***.

Love ~ Mel, Steve, Jakie, & Mattie

Wednesday, July 31, 2002 at 09:21 PM (EDT)

We have some wonderful news to share...we got the results back from Jacob's enzyme test and it has gone from <0.1 to 26.6!!! Yippeee!!! Most of you know, but for those of you who don't, Jacob WAS missing an enzyme and the reason for the stem cell transplant was simply to bring this missing enzyme into his body. The enzyme level of a 'normal person' (I hate that term!!!) is usually 18 to 50 so...Jacob now falls within the normal range! What this means to us is that the damage that has been done to Jacob's body over the past 2 years has hopefully stopped...not necessarily reversed…but hopefully no more damage will occur. There have been children where the problems they have from Hurlers prior to transplant have reversed and we can hope this will happen with Jakie. This will occur over the next several years. A stem cell transplant in a child with Hurlers is not considered a cure, rather a treatment, but we just know that our tough guy will make it! Now that Jacob has engrafted and has the missing enzyme he is officially on the road to recovery. This is a very long road……..…no immune system until at least May of 2003, continued physical, occupational, & speech therapy, weekly (if not more) doctor visits at home-home, trips back to Duke at 6 months, 9 months, 12 months, 18 months, 2 years, 3 years, 4 years, etc…………but we are on that road and heading in the right direction! :-)

Best regards from Durham, North Carolina…

Love ~ Mel, Steve, Jake, & Matt

Monday, July 29, 2002 at 11:18 PM (EDT)

HAPPY 2ND BIRTHDAY JAKIE-BUG!!!

Today is Jacob’s big day --- he turned 2 years old at 4:02 am, today, July 29TH. He has been through more in the past few months than any 2 year old should have to go through…but he made it this far...and we are SO proud of him! Birthdays are special for every body, but, just a little more important and special for people, particularly kids, who have experiences like Jacobs. Way to go buddy...Mommy & Daddy love you so very much and hope you had a wonderful 2ND Birthday!

Jacob has been feeling really good all day and had fun in the apartment. He spent time with his Grandma & Grandpa Irish and his ‘Best-Buddy,’ Stacy, came by to play and give Jacob a gift. Of course he also spent time with his brother, Mommy, & Daddy. He got several new toys (Matthew did too because Grandma & Grandpa won’t be here for his 1ST Birthday) and loved playing with them all! After dinner he even got some birthday cake --- didn’t really eat much...maybe a little frosting...but loved playing in it! We took pictures and I’ll definitely post one in the photo album when I get them downloaded. All in all I think Jakie had a good day.

Tomorrow is our long Clinic day so I better be off to bed. Take care of yourselves...

Love ~ Mel, Steve, Jake the Birthday Boy, & Matt

P.S. Jacob is probably lactose intolerant due to the chemotherapy...most the kids are...so no ice cream with his cake. :-( Actually, he probably shouldn’t have the cake either as he is on a very strict diet (Neutropenic Diet – to reduce the risk of infection) but that was more for playing in than eating!!!

Sunday, July 28, 2002 at 1:10 PM (EDT)

DAY +67

I’d like to share with all of you the story behind the cranes that my friend Jessica mentions in her last guestbook message. She is currently teaching English to Japanese students in Japan. Everyone has been so kind to us in so many different ways and to me this just encompasses the essence of all this kindness. Jacob received a box in the mail while he was in the Hospital filled with origami (folded paper) cranes with a letter sitting on top. I am going to share the letter with you all and I truly hope you don’t mind, Jess. Here goes…

“Steve, Melanie, Jacob, & Matthew ~
Perhaps you know the story behind the making of 1000 paper cranes. But in case you don’t….
Years ago there was a young girl in Hiroshima, Japan who became very sick from the fallout after the A bomb. While she was sick in the hospital she began folding 1000 paper cranes. She believed ~ as many do ~ that if she completed them all, any wish she made would come true.
Her wish was for peace. My wish for you is health and healing. From this side of the world there is little that I feel I can physically do to help you at this time. But please know that in each and every crane that I folded I thought of you. Prayed for you. Loved you.
I’m not responsible for folding all 1000. About 250 of them came from friends, co-workers, and even a few people I’ve never met. When they saw what I was doing they wanted to help. So I told them about you and how incredible you are. They not only send cranes ~ they send thoughts and prayers as well.
I ask one favor of you. After you have finished with these cranes could you please pass them on to someone else? Perhaps another family, perhaps leave them at the hospital. There is so much love in this box and I would love to see it passed on.
May you always know that I carry you in my heart and in my thoughts. Even though I’m done folding cranes I will continue to think of you each and every day.
May all your wishes come true.
Love Always, Jessica”

I have been stringing cranes together into chains and will do as Jessica asks and share this gift with the families we have met and continue to meet on this journey. It’s so amazing to see that people are sending good thoughts Jacob’s way from all over the world! Cranes were folded by Yoshimi, Naomi, Emiko, the Maeda Family, Yukihiro, and Shuoko from Osaka, Japan; Steve from New York; Betty from Atlanta; Julianne from Sydney, Australia; Nick from Prince Edward Island, Canada; John from Derrygoolin, Ireland; Yukiko from Nara, Japan; Gavin from New Zealand; Randall from Philidelphia; Amanda from Perth, Australia; and Liam from Cork, Ireland. Wow!

Just recently Jessica traveled to Hiroshima and actually placed a crane in honor of Jacob there. She sent me a picture and I’ve added it to the photo album so you all can see the cranes as well.

Thank you to everyone who reads Jacob’s site. We hope you enjoyed the story of the cranes as much as we did. I really wanted to share it with all our family and friends and felt this was the best way to do it. Hopefully these cranes can touch your lives as they have Jacob’s…

Love ~ Melanie, Steve, Jacob, & Matthew

***PHOTOS HAVE BEEN UPDATED***

Saturday, July 27, 2002 at 06:28 AM (EDT)

DAY +66

Hello All...

We're happy to report that Jacob is still doing great! He loves to play & WRESTLE with his brother, laugh, dance, 'talk', crawl, & walk with his push toy. He seems to really thrive away from the Hospital where he can be his own little man! He continues to amaze us with his ability to recover.

His 3 hours Clinic day on Thursday turned in to about 5+ hours by the time we got done...par for the course where Jakie is concerned! :-) The nurse hooked him up to his meds and the line running from one of his meds to his central line broke in half. Steve and I were looking at him and noticed what looked to be a ton of blood (probably only 4 cc), of course we panicked until we figured out it was just a line break. Unfortunately, having a line break with an open clamp is a conduit for infection (picture a vein straight to the heart wide open) so he had to have his line cultured for infection (we don't have the results yet but expect them to be normal) and a dose of the strongest antibiotic available (Vanc) administered to him via IV to that line. Well, he's gotten Vanc before but for some reason this time he had a reaction to it...they call it Red Man's Syndrome...you get the picture. So then he had to have a big dose of Benadryl to stop the reaction! Crazy!

Friday morning Jacob had an appointment with Dr. Rice, one of the general pediatric surgeons to take a look at his gallbladder tube. We have really been wanting to have it removed...try telling a 2 year old and an incredibly inquisitive 11 month old to leave the big tube alone that is hanging out of your belly (or your brothers belly)! It does not work real well. And, his bilirubin number has been decreasing steadily...it's not quite normal yet but not alarmingly high as it was...so really no reason to keep the tube. We took him in and Dr. Rice decided to just remove it so they snipped the stitch and just pulled it out! I should have just let the boys do it themselves a week ago! Now Jakie has a hole in his belly that we have to keep clean and covered, we're told it should be closed and beginning to heal within a week. We're happy this annoyance is out of the way. Hopefully, his gallbladder will just heal up correctly then we don't have to worry about having it removed down the road.

Grandma Patty & Grandpa Steve arrived in town yesterday morning. They came to celebrate Jake's 2nd Birthday with us (Monday, July 29) and will be here for a week. We're so happy to see them and the boys are excited to spend some time with them!

Take care of yourselves!

Love ~ Mel, Steve, Jake, & Matt

Thursday, July 25, 2002 at 07:03 AM (EDT)

Jacob has become quite the early riser so we've been up for a couple hours (Matthew is somewhat of a night owl and didn't go to bed last night until 11:20pm!) and he wanted me to get a couple things on his website...

HAPPY BIRTHDAY TO UNCLE CHRIS!!! He turned the big 2-9 yesterday! Hope you had a wonderful day...wish we could have been there to celebrate with you. We love you and miss you!

Also, HAPPY BIRTHDAY to Normie!!! Norm is one of our doggies and turns 8 today...56 in dog years. He lives with Grammie Cindy and Grampie Mark right now and probably will forever...they're all around the same age so they get along well! :-) He likes it there! Thank you Grammie Cindy & Grampie Mark for taking care of Normie!!!

While we're at it...Thank you so much to Tiffany, Kyle, Alex, Nick, & Sam (Mommy's cousin and her family) for taking care of our other doggie Sampson while we're away. We appreciate it more than you know...Sampson is Jake & Matt's puppy and they miss him very much! And thank you VERY much to our friend Angie & Corey and their kids Trey, Kylie, Kassidy, & Trevor for taking care of our kitty Oreo. Hopefully she is being a good girl! Thank you all for taking such good care of the members of our family we couldn't bring with us!!!

Anyhow, Jacob has to go to Clinic for a few hours today to get a couple medicines administered. His body seems to be making its own platelets and hemoglobin right now so hopefully he won't need any blood products. This is a very good sign as it means his body is starting to grow red blood cells which is very important in his recovery process.

Thank you all for your guestbook posts and for being as happy as us with Jacob's progress in the right direction!

Love to all from our 'Summer Home' ~ Mel, Steve, Jake, & Matt

Tuesday, July 23, 2002 at 11:16 PM (CDT)

All is well with Jacob. He seems to just get better and better each and every day. His labs were so good yesterday morning that we didn't have to take him to Clinic. We did have to go today as Tuesday is our big Dr. Kurtzberg day...we were there from about 11am to 8pm getting meds and waiting to see Dr. K. She is very happy with the way Jacob is improving and says that we only have to go to Clinic Tuesday, Thursday, & Saturdays from now on. This will be very nice for us to get this break as Clininc days are somewhat long and tiring. We had a nice surprise at Clinic today though...sometimes celebrities drop by the Unit and Clinic to visit with the kids and today the band Sister Hazel came by and sang a couple of their songs. If you don't know by the name of the band who they are, you might know some of their songs --- www.sisterhazel.com to hear them --- from the radio. It was pretty fun and I got some good pictures. Anyhow, it is great to have Jakie somewhat back to his old self...smiling, laughing, & playing! Take care of yoursleves and thank you for keeping Jake in your prayers.

Love ~ Mel, Steve, Jake, & Matt

Sunday, July 21, 2002 at 12:35 AM (EDT)

DAY +60

Jacob was discharged from the Hospital on Thursday as planned. It was both a very happy and exciting time but a bit sad and scary as well. After spending 2+ months with ‘our’ Nurses, Doctors, fellow patients & parents, support staff, etc. it was sad to leave them behind as well as the feeling of safety they provided us. We left the Unit to cheers, well wishes, bubbles, confetti, & silly string from all the above mentioned wonderful people into the big, bad, germ-ridden world! We got back to our apartment with our grocery bag filled with oral medications, had a couple hours to rest, and then the home health nurse arrived with all of Jake’s IV medications and boxes of medical supplies...and so it begins...

We have a good organization system in place to prepare the medications (things have to be mixed and hooked to the pumps) and get them flowing into Jacob...it’s takes about 5 hours a day --- ½ hour to get things together and 2 hours to run through the pumps into his lines twice a day plus giving him 7 oral meds twice a day and blood draws every morning. We also took Jacob to Clinic Friday and Saturday. We thought we would have to go today as well but Jacob is doing so well that we got the day off. Yippeeee! We have to go again tomorrow and then we have our possible 12+ hour Clinic day on Tuesday. That’s the day he will be seeing Dr. Kurtzberg, making changes to meds when necessary, and getting several additional IV meds.

Besides all of that Jacob has loved being at the apartment. He scoots around a bit and plays with his brother. I’m sure it must seem huge to him here compared to the little room he has spent the past couple months in. Yesterday, his Best-Buddy (I think I’ve mentioned her before --- the Family Support program assigns a volunteer to each family to help out with things and spend time with the patients), Stacy, came by for a visit. He absolutely adores her and loves to play with her...she had him giggling like crazy. Thank you for everything you do for us Stacy!!!

Hope you all are enjoying you weekend...Take care of yourselves!

Love ~ Mel, Steve, Jake, & Matt

P.S. Fundraising efforts on behalf of Jacob have begun. For more information, go to www.cota.org, click on the 'Patient Campaign' link on the left side of the page, then click on Jacob's name on the list. Thank you!

Wednesday, July 17, 2002 at 10:11 PM (EDT)

All is well with Jacob. He has loved his 'passes' out of the Hospital the past few days and is still scheduled for discharge tomorrow. Many of his medications have been switched to oral the past couple days in anticipation for his release from the Hospital. He absolutely hates them! He's on 6 or 7 oral meds twice a day at this point which he loves to spit in our faces...we just deal with it and persist although the screaming and crying make you feel pretty bad. He will also be on several IV meds which we will administer at the apartment and several IV meds which will be administered at Clinic as well as blood or platelets if needed. Steve and I will also do blood draws every morning and deliver them to the Hospital between 7am and 9:30am, flush his lines, change caps, do dressing changes on his lines & his gallbladder drain tube, etc. It's a pretty hectic schedule but we don't really care...we're just thrilled he is getting out of the Hospital! Tomorrow is a big day so I'm off to get some sleep...Matthew has been aspleep for an hour (ah...the luxury of being a baby) and Daddy is spending Jacob's last night at the Hospital with him so hopefully they're getting some rest as well.

Thank you for all your prayers and kind words throughout this in-patient part of our journey. We'll begin on our next leg tomorrow...wish us luck!

Love ~ Mel, Steve, Jakie, & Mattie

Monday, July 15, 2002 at 10:03 PM (EDT)

DAY +54

Jacob stuns us with his ability to recover. He is gaining strength and looking better every day. Today, we were offered a chance for a ‘pass’ off the Unit. I, of course, jumped at this opportunity! Logistically, it was a bit tricky though…Steve was golfing this morning with a nurse on the Unit, Andre’ (Jacob adores him) and Kayla’s (a transplant patient like Jake) Daddy, Johnnie. Jacob’s primary nurse, Keri, was baby-sitting Mattie and Jake & I were at the Hospital with no vehicle! This was the first opportunity Steve had to do something relaxing and for himself but I knew he would want to get Jakie off the Unit just as much as I did. Unfortunately, he was not getting the pages I was sending him (our borrowed pager doesn’t always work) and he had our vehicle at the golf course. So…wonderful Keri loaded Matthew in her car and came and picked Jake and I up so we could have our outing. He is not allowed in public (and won’t be for at least the next year) but is allowed to spend time at the apartment so we brought him ‘home.’ He LOVED it! He played with Matthew and even walked a bit. He ate better here and talked up a storm. It was just great to see. Also, Steve finally received my pages and rushed home to spend time with him as well. I was pretty happy it worked out the way it did…it was really nice to have Keri here because if anything happened with Jake she was here to help. It’s pretty scary the first time he leaves the Unit a.k.a. the cocoon. A big THANK YOU to Keri for all your help --- YOU ARE A WONDERFUL PERSON!!! When we got back to the Hospital (he was out for 4 ½ hours!) Dr. Mustafa came in and told us that we get a ‘pass’ off the Unit tomorrow and Wednesday AND if all goes well Jake will be discharged from the Hospital on Thursday…we can hardly believe the time has finally come! It is such a wonderful feeling to know he is finally well enough to leave the Hospital for our home-away-from-home. Once he is discharged we will be going to Clinic everyday, usually for the full day (8 – 12 hours), and then will be sleeping at the apartment. Eventually, his Clinic visits will be reduced to every other day and so on until it’s time to come HOME. Normally, patients are released from the area around day +100 which would be at the end of August for us. I really am not sure if we will be released from the area at that time due to the many complications that have plagued Jakie. We’ll just have to wait and see how things go for him…he’s such a little tough guy he might amaze us once again! He truly is his Mommy & Daddy’s HERO and amazes us on a daily basis.

The pictures have been updated so take a look if you get a chance!!!

All our love ~ Mel, Steve, Jake, & Matt

Saturday, July 13, 2002 at 11:30 PM (EDT)

DAY +52

No news is good news! Jacob has been getting increasingly better...it is so wonderful to see! He is still somewhat jaundiced but his bilirubin is decreasing a bit each day. His other liver enzyme numbers have yet to decrease, in fact some even continue to increase, but, if you could see him compared to how he was a week ago you can just tell he is healing. His nurses and doctors have been amazed by his turn-around. We think Jakie just got tired of being sick and decided to get better on his own!

Jacob continues to get a large chunk of his day 'off his pole & pumps' which is so nice. He has gotten a pass off the Unit the past couple days and we have been allowed to take him to the fish tank near the elevators for 10-15 minutes. It is quite an outing! We took him and Matthew to the fish tank today and they had such fun looking at the fish together...it has been so nice getting the boys together again as well.

We also had some visitors today. Some NASCAR 'celebrities' came to visit the kids on the Unit (you're jealous aren't you Grampie Mark!?!). They brought the kids all goodie bags filled with all kinds of NASCAR stuff. I think Jacob just liked being surrounded by men for a change. I think he gets a bit tired of all us women (Mommy & his nurses) fussing over him. He gave the guys lots of big smiles and they were very taken by him (can you blame them?).

Hope you are all well. Take care.

Love ~ Mel, Steve, Jakie, & Mattie

Wednesday, July 10, 2002 at 08:15 PM (EDT)

DAY +49

Jacob has had a happy day today. He played most of the morning and this afternoon he was off his pole for about 2 hours. For those of you who haven't been through this, Jacob is hooked up to 3-5 pumps on an IV pole 24 hours a day, 7 days a week. He constantly has medications and fluids pumping in to him. Being off his pole (detached from medications, fluids, nutrition, etc.) for any period of time is something that is done when the patients are starting to feel better. It means that he doesn't need fluids pumping in to him all of the time and the nurses are able to lump his medications together so we get a nice chunk of time off the pole. It is a wonderful feeling of freedom. You would be surprised at how confined he truly is when hooked up --- a 2 year old just shouldn't have to go through this! As I've said before, if Jake wasn't still having liver/gallbladder issues, we'd have been discharged long ago and I think this 'freedom' is just one more step closer to possibly becoming out-patient. Anyhow, he wore himself out with all of his freedom and has already fallen asleep. It will probably be an early morning tomorrow!

We did have a small medical issue today. Jake's nurse was changing the dressing on his gallbladder tube and the stitches have all pulled loose. He was crying and the tube was actually coming out so we quickly held the tube in and taped it back up. We are still awaiting the Surgeons (they come in packs - Duke is a teaching Hospital) arrival to 5200 to take a look at it. Hopefully, that will be sometime before midnight!

Take care...

With love ~ Mel, Steve, Jake, & Matt

Monday, July 08, 2002 at 04:51 PM (EDT)

DAY +47

Nothing new to report on the Jakie front. His organs are still not cooperating, but, his demeanor is happy & playful most of the time.

Grammie Cindy just stopped by the hospital to say her final 'good-bye' to Jake. He (and us) are so sad to see her go. Steve and Mattie are taking her to a hotel close to the airport this evening so she can catch her flight out in the early hours of the AM tomorrow. Have a safe flight Grammie Cindy...we already miss you VERY MUCH!!!

Thank you all for the guestbook posts. It's wonderful to be at the receiving end of such warmth and kindness from family and friends. Take care of yourselves and we miss you!

Love ~ Mel, Steve, Jake, & Matt

Friday, July 05, 2002 at 11:18 PM (EDT)

DAY +44

As you can see, I didn’t post a WBC number like I usually do…now that we have confirmation that Jake is 99% engrafted, the white blood count isn’t really all that important. I mean we want a normal count but that will come in time. And, his WBC number has started to fluctuate as his GCSF (a white blood cell growth stimulant) has been reduced. Anyhow, I won’t be posting a number any longer unless something crazy happens with it.

Jacob’s internal organs continue to be a problem for him. His liver is still not working as it should be. His gallbladder seems to be working well... now though…the surgeons have some concerns about removing the tube. Hopefully, they will decide what they want to do soon. With all of this going on inside him, amazingly, Jakie’s demeanor continues to improve. He has wanted to play more, eat more, smile MUCH more, etc. It is so nice to see after such a long time! Last night for the 4TH of July, we were even allowed to leave the Unit for a short time and take him up to the 9TH Floor of the hospital to watch the firework display at Duke University. Of course, Jacob was not at all interested in the fireworks…was just incredibly interested in being out and about. We made the most of this unexpected freedom and enjoyed our little journey to the 9TH Floor as much as possible!

A little over a week ago, one of Jacob’s excellent nurses left her position at the hospital to pursue her new life, in a new town, with her future husband. Jakie asked me to send a special message to Jami to tell her that her little peanut misses her VERY much. He hopes she is having a good time in Georgia but if she wants to come back and take care of him, he’ll let her!

Please take a look, I have updated the pictures. Enjoy!

Love to all ~ Mel, Steve, Jakie, & Mattie

Wednesday, July 03, 2002 at 11:59 PM (EDT)

DAY +41
WBC = 12.7

DAY +42
WBC = 8.0

Wow, Jacob has been in the hospital for 53 days…that’s an awful long time! Things are heading in the right direction for him which means he is getting that much closer to being released. His bilirubin count is down for the 4th day in a row which means we can consider it a trend…yippee. We just want to see this number continue to decrease and hope that his liver decides to heal itself. He still has the tube in his gallbladder but we clamped it off today to see if he really still needs it. After a few days, if he is still OK, he will probably have a little surgery to have the tube removed. This will be great so he can have more freedom to move around.

Grammie Cindy sat with Jake for about 4 ½ hours this evening. We were offered a couple free tickets to a Durham Bulls game --- not a pro team but very big here nonetheless --- so Steve and I thought it would be fun to take Matthew. It was box seats and there were several other transplant families there. Matthew had such fun watching the other kids. The game ended with a fireworks display for the 4th of July. It was Matt’s first fireworks…he was very excited and a little scared all at the same time. It was very nice to get out and do something ‘normal’ for a change and the first time Steve and I have been together outside the hospital in 53 days! Thank you Grammie Cindy for taking care of our little Jakie for us!

I added a couple new links below. Check out the websites of some of Jacob’s new friends, Taylor and Luke. We still love Henry (www.caringbridge.com/wa/henryharten) and Emma (www.caringbridge.com/page/emmaq) but they are at the end of the transplant journeys and are heading back to their homes. Congratulations to both of them and please continue to check the websites as their parents have promised to keep them updated so we know how they are doing. Once a child has a transplant their journey is truly not fully over for many years.

Happy 4th of July to you all!

Love ~ Mel, Steve, Jake, & Matt

Monday, July 01, 2002 at 10:56 PM (EDT)

DAY +40
WBC = 10.7

Jacob’s bilirubin has finally started to decrease over the past couple days. We can’t call it a trend until it goes down another day or two but we are praying that we are seeing the beginning of a trend in the right direction. He still looks terribly jaundiced but if the bilirubin continues to decrease the jaundice should begin to clear up. We still don’t know if he is going to have long term liver damage from this but will cross that bridge when we come to it. The gallbladder tube will remain in for the time being to make sure it does not get clogged up again as well. There is apparently still some question as to whether the gallbladder will just need to be totally removed or they might be able to just pull the drain tube and allow it to heal up on it’s own. Again, we’ll just have to wait and see. Jake had another chest CT today…the last one was about a month ago…to make sure the small spots they saw in his lungs on the last CT have cleared up. We should have those results tomorrow some time. Also, he was restarted on his TPN last night (his liquid nutrition). All kids who go though transplants (and anyone who is in the hospital for extended period of time without eating) are on TPN. Jacob’s was stopped about a week and a half to two weeks ago because there was a question as to whether or not it was causing some of the problems he’s been experiencing but as he has been off it for so long and he does not eat anything on his own, he was starting to become malnourished. His poor little arms and legs are tiny and his bones are sticking out everywhere…he still has a big Buddha belly though! Anyhow, hopefully the TPN won’t cause him any (more?) problems and he will become a bit more energetic as he will be taking in some protein and calories.

Grammie Cindy has decided to stay an additional week to help us out which we are so happy about…she will remain until the 9TH of July now! She can’t stand to leave her little Jakie-poo!

Love to all from NC ~ Mel, Steve, Jake, & Matt

Saturday, June 29, 2002 at 09:42 PM (EDT)

DAY +37
WBC = 10.2

DAY +38
WBC = 8.0

Nothing much new to report. Jacob continues to suffer from the unknown ailment. His doctors continue to try to figure out what is going on with him. They have switched around some meds and added a few new ones. We'll see if any of these changes do the trick. There is still some talk of possibly removing his gallbladder but not enough evidence it is the culprit. Also, still some talk of liver GVHD but again not enough evidence that this is what he has... So we continue to play the waiting game. It is very frustrating...all Steve and I want is for Jake to get better. At this point we really don't care what the diagnosis is or what they do --- just fix it!

Hope all is well with all of you. Thank you so much for all the guestbook posts...it's so wonderful to hear from family and friends, new and old!!!

Love ~ Mel, Steve, Jake, & Matt

Thursday, June 27, 2002 at 09:45 PM (EDT)

DAY +36
WBC = 7.8

Jacob continues to have severe internal organ problems. There appears to still be a problem with his liver and or gallbladder although there is some confusion as to what is really causing the problem. As of yesterday afternoon, his medical team wanted to remove his gallbladder and today the story has changed somewhat. We are really getting a mixed message which is incredibly frustrating. My hope is that they get their story straight by tomorrow…frankly; I just don’t think anyone really knows what’s going on with him though. He is incredibly uncomfortable and is continuously getting pain medication pumped into his central lines and a sedative every 2 to 4 hours. Also, I hadn’t mentioned in the past because I didn’t want Jakie to be embarrassed when he reads this journal when he’s older but the entire time he has been in-patient he has had a ‘hydro-seal’ (not sure how it’s spelled…that’s how it is pronounced though) which is simply a fluid filled…ummmm…testicle sac. This continues to be an annoyance and for some reason has picked right now to become even larger. His little pee-pee has actually disappeared inside of it. If he was a ‘normal’ child they would simply go in and drain it but in his condition they are not real keen on doing this. Anyhow, it has become so large that we believe and he makes pretty clear by his action that it is causing him a great degree of discomfort and pain. Well, I think I’ve been depressing enough for the time being. Will keep you all updated on what the final consensus is…

Love~Mel, Steve, Jake, & Matt

Wednesday, June 26, 2002 at 08:40 AM (EDT)

DAY +35
WBC = 7.8!!!!!!!!!!!!!!!!!

Well…let’s start with the great news and move on to the not so great news and then back to more good news…

Jacob had the test last week to see if he has donor cells. The test came back and he is 99% engrafted!!! Ultimately, this means the transplant was a success. Someday he might actually be 100% engrafted, basically though, 99% is just as good as 100%. We were all thrilled to hear this news.

As far as his liver/gallbladder issues go, Jake’s team of doctors is still somewhat unsure as to what is actually going on. They’ve been treating him for GVHD of his liver but don’t really believe that is what he has…just treating for it to be on the safe side. The feeling I get is that they continue to think the gallbladder is the culprit by clogging up and backing up into the liver. This means it is just a wait and see situation and hope the tube they placed in his gallbladder continues to drain. Dr. K said that this issue could take 2-4 weeks to resolve which means Jacob remains in the hospital. He’s been in-patient for 46 days now and we are very anxious to become a ‘complete’ family again. The other alternative is to go back in and remove his gallbladder which is being discussed as well. I guess we’ll just have to wait and see… (My goodness, I’m beginning to sound like Jake’s medical team!!!)

Grammie Cindy arrives this afternoon and Jacob (and the rest of us) can’t wait to see her. Daddy & Mattie will be picking her up at the airport later on today. Have a safe flight Grammie Cindy!

Love from Duke University Hospital ~ Mel, Steve, Jakie, & Mattie

Sunday, June 23, 2002 at 11:16 PM (EDT)

DAY +32
WBC = 5.3

Nothing much new to report since my last post... Jacob continues to have problems with his liver and/or gallbladder. The doctors continue to try to figure out what is going on. At this point no one really seems to know, they are just making some educated guesses. We just try take one day at a time and hope that things begin to clear up soon! Maybe I'll have more to report on Jacob the next time I post...

Also, if any of you are interested, there is a wonderful article in the July 16TH issue of Woman's Day magazine about Jacob's friend Molly Birmingham, her Mom-Jenny wrote it. She has Hurler Syndrome like Jake and was transplanted here at Duke about a year ago. The article really describes and explains some of what we and many of the other families are going through here. I strongly urge you to get a copy and read the article about Molly (www.caringbridge.com/page/mollyb). I checked the Woman's Day website and the article, unfortunatly, does not appear to be on-line.

Take care...Love ~ Melanie, Steve, Jacob, & Matthew

Friday, June 21, 2002 at 08:47 PM (CDT)

DAY +30
WBC = 5.1!!!

Wow…once again…check out that white blood count!!! It’s been at 5.1 for the past two days…can’t wait to see what it will be tomorrow. Jake continues to have problems with his liver and gallbladder…and we continue to wait for the medicine to start kicking in. He has been interested in taking a small amount of fluids by bottle for the past couple days so we’re trying to interest him in the Pediasure so he gets some vitamins. He smiles a couple times a day and plays with his ‘gently used’ blocks from his buddy, Henry, once a day. These are big steps for him so we’re very happy to see this.

Auntie MarMar leaves for home tomorrow and we’re very sad to see her go… Jakie and Mattie will miss her horribly!!! It’s been great to have her around this past week…we were even able to get some good shopping in today! I took her to a great Mall here…she was impressed which is tough, for those who know her, to imagine.

Love ~ Mel, Steve, Jake, & Matt

Wednesday, June 19, 2002 at 11:36 PM (EDT)

DAY +28
WBC = 3!!!

Can you believe that white blood count!?! I think I already mentioned this but a normal white blood count for Jake is 10-15 so will are really climbing up there. Actually, if he was not experiencing so many transplant related complications, we would be looking at being released from the hospital very soon. As it is now, it is still going to be quite some time before we become out-patients. We are still waiting on the test results to make sure these are donor cells but we have no reason to believe they’re not.

Jake’s liver biopsy and last ultrasound results are in. He still has clogged bile ducts in his liver which are being treated with the tube that was placed in his gallbladder and a medication to help flush out his system. He also appears to have GVHD (Graft vs. Host Disease) of the liver. This is being treated with a medication as well. He continues to be jaundiced with a high biliuribin count. The GVHD medication takes about a week to begin working. We are really looking forward to this happening as Jacob is still experiencing quite a bit of discomfort from these problems and we really want his discomfort to subside. The other thing we unfortunately do not know is how much damage is truly being done to his liver and what type of long-term damage are we looking at, if any. It is horrible not knowing…we just try to take things one day at a time and try not to dwell on the unknown. We’re doing everything we can to cheer Jakie up. Daddy and Auntie MarMar had him smiling and laughing a little bit yesterday and that was just about the best thing I’ve ever seen in my life. I won’t go into details but they were singing to him and it was incredibly amusing. He smiled for me again this morning and took a small amount of Pedialyte out of a bottle. This made my week!

Grandma Patty and Grandpa Steve fly back to Washington first thing tomorrow morning. We (Mommy, Auntie MarMar, & Mattie) took them to a hotel closer to the airport this evening so we didn’t have to get up at the crack of dawn to drive them to the airport tomorrow. Jacob is going to miss Grandma and Grandpa terribly and can’t wait for them to come back and see him again. We are so very thankful they were able to come and help us out during this difficult time.

Thank you all for you guestbook entries. It is wonderful to hear from our family and friends back home as well as the friends we have made all over the world. I would love to respond to you all individually but right now that is just too difficult…one of these days I will though..I promise!!!

Love ~ Mel, Steve, Jake, & Matt

Monday, June 17, 2002 at 11:08 PM (EDT)

DAY +26
WBC = 1.6!!!

HAPPY FATHER'S DAY (sorry...one day late) to all the Daddys out there, especially Daddy Steve, Grampie Mark, Grandpa Steve Sr., Uncle Chris, & Uncle Ben! Jacob loves you all!!!

Jacob is still recovering from his surgery last week. He is pretty weakened from the chemo and all his ailments over the past couple months but appears to be feeling a little better. As you can see above, his white blood count has jumped to 1.6 from 0.5. According to the doctors this is good news although it could potentially drop down again. We'll see what it is tomorrow... They are actually going to do a test with his 4am lab draw to find out if the cells coming in are his or the donors. The results will be back in a few days and we are keeping our fingers crossed for donor cells! We are still awaiting the results of the liver biopsy and the latest ultrasound of his belly. He is still experiencing some complications having to do with his gallbladder and/or liver and we're anxiously awaiting some of these test results to find out what is going on.

Take care! Our love to you all ~ Melanie, Steve, Jacob, & Matthew

Saturday, June 15, 2002 at 10:46 PM (EDT)

DAY +24
WBC = 0.4

Jacob is looking a bit better today. He appears to be feeling a little better and his pain meds were lowered a small amount. He is still pretty out of it though due to the pain meds even though they've been lowered and is still very itchy due to the jaundice and the pain meds. We're hoping things continue to subside as more fluid is drained from his gallbladder.

Jakie and Mattie were both thrilled to see their Auntie MarMar! She arrived in North Carolina today and had not seen her 'boys' in 2 months. We are all very happy she is here to spend some time with us!

Thank you all for your continued kind words and prayers. We are so lucky to have you all for our friends and family.

Love from Duke University Hospital ~ Melanie, Steve, Jacob, & Matthew

Friday, June 14, 2002 at 07:58 PM (EDT)

DAY +23
WBC = 0.3

Just wanted to do a quick post to let everyone know how Jakie is feeling the day after his surgery. He's pretty itchy from the pain meds and the jaundice, but besides that he appears to be resting pretty comfortably. The tube from his gallbladder is draining well and as the doctors say...everything is as expected. We are now just waiting for the liver biopsy results which will hopefully be here on Monday. Thank you all for your wonderful posts. It's such a connection to home for us...one that we greatly need right now.

Love to all...Melanie, Steve, Jake, & Mattie

Thursday, June 13, 2002 at 10:16 PM (EDT)

Yesterday ~~~ DAY +21 ~~~ WBC = 0.4
Today ~~~ DAY +22 ~~~ WBC = 0.3

As of yesterday Jacob's liver and gallbladder were not improving at all...in fact they were getting much worse. So...this afternoon he had exploratory surgery. Dr. Skinner, Jacob's surgeon, went in and took a biopsy of his liver and placed a tube in his gallbladder to drain the sludge and bile out of him. Basically, the tube is put in to by-pass the gallbladder and the reason they wanted to drain it is because it could be infected and/or poisoning his body. It could also possibly be causing the problems in his liver but not necessarily. We won't really know what is going on with his liver until the biopsy results come back on Monday. He really did well with his surgery considering he has very little white blood cells and pretty much no immune system. They pumped him with platelets prior to the procedure and gave him a very stong antibiotic (only used in hospital settings). They were able to keep him stabalized during the procedure and he was able to come back to his regular room on 5200 where his wonderful nurse, Jami, was waiting to take care of him. We were so happy to have her here as she babies her little 'peanut.' Jacob was also assigned his own nurse to focus on him all night, Andria, who is wonderful as well. There was a chance that he might have had to go to the PICU but thankfully did not. He is resting comfortably on strong pain meds in bed with Daddy. I am in the room as well. Grandma Patty and Grandpa Steve caught a red-eye last night from Seattle and arrived in Durham this morning to be with us during this difficult time. They are watching Matt right now so we can both focus our attention on Jacob. We are very appreciative of this as we weren't sure how we were going to juggle things. Jacob will be spending the next several days recuperating from his surgery and we hope he does this with as much ease as he seemed to have throughout his procedure.

Thank you for all your great guestbook posts! We really appreciate them.

Love ~ Melanie, Steve, Jake, & Matt

Tuesday, June 11, 2002 at 11:37 PM (EDT)

DAY +20
WBC = 0.3 ~~~~GROW CELLS GROW~~~

Well, if it's not one thing it's another... Jakie just can't get a break! It seems that almost every 'side-effect'/'complication' that was described to us prior to Transplant has happened to our poor baby. Yesterday morning, Jacob's labs came back with an elevated biliuribin (?sp.) and when Steve turned on the lights in his room that same morning Jake was totally jaundiced (yellow). This led us and his doctors to believe that something must be going on with his liver which can sometimes happen in transplant situations (VOD, GVHD, etc.). An ultrasound was ordered and happened this morning. It showed that Jake's gallbladder and liver are severely enlarged. His gallbladder appears to be accumulating sludge (nice word...means it's storing garbage in it) and is now plugged up which will in turn affect the liver. They have started him on medicine to try to clear it out and temporarily stopped his TPN and lipids (his 'food' as he doesn't eat or drink really anymore). They are also consulting with the surgeons to see if they have to possibly go in surgically and clear things out. We are really hoping this does not have to take place as surgery for Jacob right now is extremely dangerous. His rash has pretty much cleared up but he is now itching from the jaundice which is no fun for him. He also has a very swollen belly due to the enlarged organs and is experiencing some pain in this area as well. One good thing is that his cells continue to grow as you can see by his increasing WBC number. We try to focus on this to keep us positive right now.

If you could say an extra little prayer for India and her family we would appreciate it. India lost her battle last night and our hearts go out to her parents during this difficult time.

With love ~~~ Melanie, Steve, Jacob, & Matthew

Sunday, June 09, 2002 at 11:07 PM (EDT)

DAY +17 ~~~~~~~ WBC = 0.1
DAY +18 ~~~~~~~ WBC = 0.2

Hi All,

Jacob seems to be doing pretty well right now. He has not had another 'episode' like last Thursday....thank goodness. Hopefully, that was just a one time thing and we will never have to deal with it again! His urine, blood and nasal secretion tests have so far (they have to grow them) been negative for infection. He had a chest x-ray this morning and there are still spots visible on his lungs but apparently don't look quite as obvious as they did on past scans. Maybe the antibiotics are working... His blood pressure has been fairly high so he has been put on medicine for this as of yesterday. He's still pretty itchy from his rash and his breathing is somewhat labored but besides these things he is doing well. We are trying to get him to play with his toys again and have even gotten a few smiles out of him over the past day or two. We are really hoping that the worst of it is behind us...keep your fingers crossed!!!

Take care and Love to all ~ Mel, Steve, Jakie, & Mattie

Friday, June 07, 2002 at 10:53 PM (EDT)

DAY +16 ~~~~~~~ WBC = 0.2

Jacob is not feeling so well right now. It has been a pretty tough couple days for all of us. Yesterday, a chest CT was ordered for Jake because the doctors noticed a few spots in his lungs in his last chest x-ray. The CT was ordered as it will give a better picture of the lungs. The thought is that he has some type of infection in his lungs...not sure yet if it's bacterial, fungul, or viral. I (Mommy) was at the hospital to go with Jacob for his chest CT...it's one of the few times Jacob has to leave our room on 5200, a.k.a our cocoon, because they do not have portable CT scanners. Anyhow, all was going well with the CT until the very end. I noticed Jacob's breathing was not right and immediately notified the tech to get a doctor in the room. Jacob turned completely white and a bunch of nurses and doctors came into the room and put him on oxygen right away. Our transplant doctors were then called to the CT department to help evaluate the situation and stabalize Jakie. It took a couple hours to stabalize him fully and he continues to be on 'blow by' (oxygen blowing near his face). We can't be sure exactly what happened...the feeling is that he either had a seizure OR he was dehydrated and having trouble breathing with his mask on (he has to be masked any time he leaves his room) and had somewhat of a panic attack. We might never know exactly what happened but he appears to be recovered from that drama now. Jacob's Primary Nurse, Keri, was visiting at the time (not working...just dropped by to say hello) and stayed the whole time to help comfort Jacob (and me!). We can't thank her enough for this!!! Anyhow, Jacob had some further testing today to try to find out what type of infection if any is in his lungs. In the meantime, they are treating him with very high doses of antibiotics to be on the safe side. He also had an echocardiogram today to make sure his heart was not involved in this problem with his lungs and the echo came back normal so that is very good news. We are now just praying the antibiotics clear up the infection or at least mantain it at its current level so his white blood cells can grow some more so they can come fight it. Also, Jacob is still very itchy with his rash but we can't be too upset about this as it is a sign that the donor cells are growing. We just wish he was a little more comfortable.

I'll update again in a couple days and maybe we'll know more about his lungs.

Love ~ Melanie, Steve, Jacob, & Matthew

Wednesday, June 05, 2002 at 010:11 PM (EDT)

DAY +14 ~~~~~~~ WBC = 0.2

We think Jacob is feeling a little better every day. Maybe it has something to do with that rising white blood count!?! Thank you Grammie Cindy for keeping everyone updated!!! Jacob still has quite a rash but now we can assume it is engraftment rash (donor cells are taking over...hence...a rising WBC). He is still somewhat lethargic and irritable but actually had his eyes open a lot more yesterday and today than he has in days. He has watched his Elmo videos a little, but is not yet ready to play with his toys or spend any time working with his therapists (physical, occupational, & speech). Also, he had an MRI yesterday and everything looks pretty good. There is a very small area that shows some blood between the skull and the skin which is probably from his shunt being tapped and there is also a small amount of fluid visible...again, not on his brain but between the skin and the skull. Jake's transplant team will be consulting with his neurosurgeon on this fluid but the feeling is that it is no big deal. Possibly, all this stuff the poor little guy has been through over the past week or so has been the donor cells trying to take over! We really are hoping and praying that this is the case and we will continue to see an improvement over the next several days!

Grandma Patty and Grandpa Steve are beginning their journey back to Washington tomorrow afternoon (they fly out early Friday morning) so we will be on our own again. We will ALL miss them terribly as they have been such a huge help through this very difficult time in Jacob's transplant process. They have been here for about 3 1/2 weeks and it will be very tough to see them go.

Thank you all for all the wonderful posts to Jake's guestbook. Thank you for keeping Jacob in your thoughts and prayers!

Love ~ Mel, Steve, Jakie, & Matt

***P.S. New photos have been posted to the photo album. Check them out! Oh, and a special sorry to Nate...there isn't one of Mattie right now but your Grandma & Grandpa will see you on Friday and they have lots of pictures of both your Cousins for you to see!

Monday, June 03, 2002 at 11:17 PM (EDT)

DAY +12 ~~~~ WBC 0

I'm happy to report that Jakie is doing a little bit better. He's not crying quite as much and the fevers appear to be gone for the time being. He has received 4 very large doses of antibiotics and he is completely 'poofed' up. He looks like a little dough boy and weighed 28 lbs. 6 oz. this morning which is about 3 1/2 lbs more than his 'normal' weight! He still has a rash covering his entire body. Steve & I and our wonderful nurses, Jami & Keri, have been slathering lotion on him like crazy...trying to get him some relief from the itching. He is still very irritable and lethargic, although, he actually cracked his eyes open twice today as opposed to once yesterday. His doctors are still somewhat concerned about some of the symptoms he has exhibited and have ordered an MRI (he has already had his shunt tapped, 2 CT scans, and an EEG in the past 3 days) of his brain which will take place either tomorrow or Wednesday. We are praying it will be normal as all the other tests have been. We just believe Jake's doctors are being extra cautious and making sure they rule everything out which is a good thing in our opinion. We are also hoping we will see a white blood count (WBC) on his lab sheet tomorrow morning so then we can blame all this misery on engraftment and will feel that it has all been worth it!

Thank you for all your kind words and prayers. We know they are working!

Love ~ Mel, Steve, Jake, & Matt

Saturday, June 01, 2002 at 09:27 PM (EDT)

DAY +10; WBC 0

Hello to all. It has been a hectic several days...Jacob has been feeling awful and on top of that, we moved to our new apartment (brand new - no one has lived in it so we feel much safer bringing Jacob home to a place like this once he is released from the hospital).

Jacob's troubles started with high fevers several days ago. He tested positive for a bacterial infection at that time which they treated swiftly with very strong antibiotics. Because his infection apparently cleared up, it was expected that he would snap back to 'normal' (keep in mind that 'normal' on 5200 is very different to normal in the 'real world'). Unfortunatly, he has not recovered at all, if anything, he has gotten worse. At this point he is severely irritable and completely lethargic. He is still experiencing high fevers. Basically, he does not want to be touched...he pretty much just wants to lay there...when he's awake he cries almost constantly. It is very difficult as parents to see our baby in so much pain and discomfort. They have switched his pain meds 3 times now and he is receiving the maximum dose possible for his weight. Last night, the doctors decided to tap his shunt to see if it was infected. This process is simply a needle inserted into a valve right under the surface of the skin on his head. A nuerosurgeon came and did this last night and everything checked out OK (low pressure and no infection). The problem with this procedure came because his WBC is still zero and his platelets are very low...his blood did not clot correctly after his shunt was tapped and he ended up with blood under the surface of the skin covering most of his bald head (basically a big purple bruise). He had a CT scan today and it showed that a small amount of the blood got caught in an area at the front of his skull. This should not cause any problems and will clear up with platelet transfusions...but caused us a lot of concern today until we were able to speak with a nuerologist who explained what happened should not affect him in any way. Now we're back to square one though and still don't know what is causing the severe lethargy and irritability. They have now decided to switch around some of the medications he is receiving to see if maybe he is having a reaction to something. At this point we are just waiting and praying that he feels better very soon.

I have to say that one of the symptoms he is having right now could possibly be good. He has a nasty, speckly, sun-burned looking rash covering a large part of his body. His nurses think this might be engraftment rash which would be a good thing. It would mean the new cells are growing in Jakie's body! Might explain the fevers as well... Grow Cells, Grow! (the 'motto' on 5200)

It saddens us to tell you that Jacob's 'hallway friend' Shay passed away the other day. Please say a prayer for her and her family during this difficult time. We will all miss Shay and her Mommy, Norma, especially during our walks, very much. They are in our thoughts daily.

Love ~ Melanie, Steve, Jacob, and Matthew

P.S. Please see slight change in address and new phone number below.

Tuesday, May 28, 2002 at 03:40 PM (EDT)

DAY +6

Jacob is still pretty much the same. The first day of the increased pain meds seemed to really help but now he's not feeling too well again. Dr. K just increased his pain meds again today. He has a fever that seems to come and go and is still dealing with the mucositis. Yesterday during the day he had some fun playing in his room and even pushed his walking toy around a bit. It's tough for him to breath right now in his mask, plus his WBC is still zero, so we stick pretty close to our room.

Hope all is well with all of you...Love ~ Mel, Steve, Jake, & Matt

Sunday, May 26, 2002 at 10:44 PM (EDT)

DAY +4

Jacob is still having a tough time with the mucositis. He is in a considerable amount of pain. Dr. K thankfully upped his pain medication today so hopefully he will rest better tonight. All he really wants to do right now is cuddle with Mommy or Daddy and he even went to Grandma Patty for a little while today. Basically, we are just trying to keep him comfortable at this point. He did get to see his buddy Henry today (from a distance as his WBC is zero) and this made him very happy!

On the lighter side…Daddy and Grampie Mark have shaved their heads bald as cue balls to support Jacob in his beautiful baldness. I am waiting for pictures to arrive of Grampie Mark and as soon as Jake is feeling up to it, I will take a picture of Daddy and Jakie and their bald heads with the digital camera for all to see. Daddy sure has some ears on him! And, I hear that Grampie Mark somewhat resembles Jesse Ventura!

***Here’s a special message to Cousin Nathaniel: Grandma Patty tells me that you are mad at me because there are no pictures of Matt on the website. Well, I posted one just for you buddy so take a look! Jake and Matt miss you and send you bunches of hugs and kisses. Love, Auntie Mel***

Sorry…I had to get that in here…3 year olds can be tough!!!

Finally, we will be moving to a new apartment on the 29th so I have posted our new address below. We do not have a phone number yet but once we do, I will post that as well. Our new apartment is actually brand new, never been lived in, and makes all of us feel more comfortable for when Jacob is released from the hospital with a very weakened immune system and low blood count. The place we are currently living just isn’t cutting it!

Thanks for all your guest book comments. When we’re feeling down, all we have to do is read your posts and it lifts us up!

Love ~ Melanie, Steve, Jacob, & Matthew

Friday, May 24, 2002 at 11:37 PM (CDT)

DAY +2

Jacob has been doing pretty well since his transplant. He definitly has mucositis...hard to tell how much as it is mostly internal for him. This is not much fun for him and we are definitly using the pain medication to try to keep things under control. This really seems to help him rest better. We're told that the mucositis usually hangs around for about 10 days. Besides the mucositis and some lingering ATG hives, he's doing extremely well. His WBC (white blood count) has now dropped to zero which was expected so now we're playing the waiting game for when it begins it's gradual climb upward. His platelets dropped below 20 today (again, this is to be expected) and he needed to have a transfusion.

We had somewhat of a big event tonight. I took Steve some dinner at the hospital (it's his night) and I was playing with Jakie's hair while I was sitting with him and it started to fall out. So...rather than have it everywhere which was already starting to happen. We decided to shave his head...so...my baby is bald (now I have 2 bald babies!!!). I have to say...he is pretty cute with a naked head. It was somewhat traumatic to see all that beautiful white hair go away, but I saved it in a bag (I know, I'm crazy!) and we'll always have it to remember his pretty white hair. We took some before and after photos as well...next time I update the photos I'll be sure to put one up.

I also want to post a little update on Matthew...I don't think Jacob will mind if I use his website to do this. :-) Yesterday Mattie turned 9 months old. He has been crawling all over the place and is now pulling up on furniture and starting to take steps. He is advancing by leaps and bounds and amazes us everyday with his energy and fearlessness (which has resulted in MANY bumps on the head and pinched fingers). He has 7 teeth and number 8 is on it's way! He is super happy, always smiling and really keeps us active!

Take care everybody, we're thinking about all of you!

Love ~ Mel, Steve, Jake, & Matt

Wednesday, May 22, 2002 at 11:50 PM (EDT)

HAPPY 'BIRTH'DAY JACOB!!!
Today was Jacob's big day and is considered somewhat of a re-birth. Jacob received the new cells that will (once engrafted) save his life. He was administered his unrelated umbilical cord blood transplant from 11:30am to noon. Present in the room were Mommy, Daddy, Matthew, Grandma Patty (Grandpa Steve waited outside of the Unit as he has a bit of a sore throat - sorry Grandpa - hopefully you can visit soon!), Jayne (our wonderful Nurse Coordinator who 'held our hand' through the entire pre-transplant process and continues to be a huge presence in our lives), Emily (the Child Life Therapist who took the digital photos), Keri (Jake's Primary Nurse), and the two Nurses who administered the cord blood - another Keri and Bobbi. We are so happy you were all there to share this with us and we know that the rest of you were there 'in spirit'! I have to admit that Jakie was not quite as excited by the whole thing as the rest of us. The poor little guy has been through so much over the past 10 days that he just wanted to take a nap the whole time everyone was there. The minute the 'party' broke up, Jacob totally sacked out - with a bit of help from some Morphine. It appears that Jacob has the beginnings of Mucositis (we really don't know how bad it is internally) and he is in some pain from this as well as the chemo in his system which apparently causes pain as well. He also has some horrible hives that seem to spread and move around his body to different areas. The crazy thing is they don't seem to really bother him...they just look awful. From now on, we will be monitoring Jake's white blood count (WBC) very closely and will be posting it on the website. Normal range for Jacob, I believe, is between 10.0 and 15.0. Today his WBC was 0.3 and will probably drop even lower (which is a good thing). Today is considered Day 0...if everything goes well and as planned with no complications, we will be released home to Washington on Day +100. That is our target day where Jacob should be engrafted with his donor cells and on the road to recovery! The average stay in the hospital for the babies with Hurler's is between Day +30 to Day +35...so this is another goal we are shooting for...it's so nice to have these things to look forward to! :-) Take care all and thank you for being a part of Jacob's special day, either in person or with your prayers and well wishes.

Love ~ Melanie, Steve, Jacob, & Matthew

P.S. Check out the photos! They are all of Jake on Transplant Day.

Tuesday, May 21, 2002 at 12:16 AM (EDT)

Jacob is having a tough time right now. He received very large doses of Cytoxan (chemo)yesterday (Sunday) and his last dose today (Monday). He also received doses of ATG which he will get one more of tomorrow for a total of 3 doses. ATG is an immuno-suppresent drug which is derived from horse serum. It is given to Jacob in very high doses to basically wipe out his entire immune system. It is super harsh on the body and between that and the two different types of chemo given in large quantity, Jacob is a hurting little guy . ATG can cause high fevers and hives. Jacob's fever spiked to 104.2 Sunday night and I just left him at the hospital with Steve and his temperature was again climbing...it was about 103.2 and hour ago. He also has broken out with hives all over his abdomen, back, neck, scalp, and around his eyes. His poor little eyes are almost swollen shut. He is retaining a ton of water which adds to the puffiness as well. He is also now receiving steroids and will be for the better part of the next year or so, and is likely to gain some weight from them. He also appears to have the beginnings of mucositis from the chemo. Mucositis is basically sores in the mouth, throat, stomach...basically the GI tract because the chemo attacks the cells in these areas. Now, I have to say that this is all considered completely normal on 5200. The things that are happening to Jakie are really no different than what many kids go through. If he didn't have these things happening to his poor little body then the drugs would not be working. As awful as it sounds, we want these things to happen so that we know the drugs are wiping out his cells in order to make way for the new, better, healthy cells to make him better. Jake's white blood count (WBC) was down to 1.2 today and his platelets were at 96. These numbers are considered very low but just where we want them (actually we want the WBC to zero which it will probably be tomorrow) so when the new cells are introduced to his body on Wednesday they don't meet with too much resistance. Through this all Jakie managed a couple small smiles today and even watched his Elmo videos for awhile. He almost even acted like he wanted to play for awhile earlier today...maybe it was the Morphine he got right before though...hmmmmm! I hope he is resting well for Daddy right now and can't wait to go back and see him in the morning.

Thank you all for the guestbook entries. We're thinking of you and missing you all as well!

Love ~ Melanie, Steve, Jacob, & Matthew

Saturday, May 18, 2002 at 10:48 PM (EDT)

I haven't updated in a couple days...last night would have normally been Steve's night and I would have posted but he was not feeling that well so we decided he should not come to the Hospital. We have to watch out for that sort of thing as Jacob has to be protected from all germs, illnesses, etc.

First, Jakie wants to say good-bye and "I'll miss you" to Grammie Cindy. She flew back to Washington early this morning. She'll be back at the end of June and Jacob is very excited for her return.

Jacob is still doing very well. He had his last dose of Busulfan Thursday night at 10pm. The Busulfan is the oral chemo which was put through his NG tube straight to his stomach. This made his tummy a little upset but Benedryl seemed to help it stay in his tummy better than just taking it with nothing. Friday morning he began Cytoxan (chemo). This type of chemo goes straight through Jakie's central lines to his bloodstream. He did not tolerate his first dose to well...got very fussy and agitated and his cheeks got very flushed. He was given some Tylenol which seemed to help for awhile but his discomfort came back very strong in the late afternoon. His medical team decided to begin him on some Morphine so he had a half dose (for his size) Friday evening and needed another half dose around 4am Saturday morning. We have decided that there is absolutely no reason for Jake to be in pain if he doesn't have to be and the Morphine really helped in this! This morning he did very well with his dose of Cytoxan and has not needed any Morphine since that 4am dose. We think the Cytoxan might have just been a little hard on his body to begin with and maybe his body got a little more used to it today. He is receiving several other medications through his lines to prevent different things from happening (antibiotics, kidney protection, etc.). Also, he had is NG tube removed today. The only thing he will need to take orally at this point is Tylenol and the tube had sprung a leak so we decided it was fine to remove it...everything else will be put in through his central lines.

Through all of this Jacob is still smiling and happy the majority of the time. Still loving his Elmo videos...he's actually even trying to say 'Elmo.' It's adorable! His nurses appear to love him...we've heard that there is 'fights' over him when the nurses are getting their assignments! It really doesn't surprise us though...he's such a flirt!!!

There are a couple new photos up of Jake's first day in the Hospital. Take a look if you get a chance.

Love to all ~ Melanie, Steve, Jake, & Matt

Saturday, May 18, 2002 at 09:19 AM (CDT)

New pics!! see photo album
love Steve,Mel,Jacob and Matthew

Thursday, May 16, 2002 at 12:15 AM (EDT)

Everything is crazy hectic here and our little Jakie just seems to take it all in stride. He is smiling, clapping, playing, and watching his favorite Elmo videos on TV through this all. It's Steve's night at the hospital and all Jake wants to do is go for walks in the hall of the unit. He likes to walk and push a little Elmo lawnmower and he likes Mommy & Daddy to push him a Flinstone's style (where he can put his feet through the bottom and move himself around) car. I took Steve some dinner tonight and got roped into just one more walk through the halls! How can you resist that face though?

Jacob is still receiving all of the same medications as I posted last time. I did forget 2 though...an anti-fungal and an anti-naseau. He's still doing pretty well with the Busulfan (chemo) although he has thrown-it-up a couple more times. It's very hard on his stomach. We're also having a major butt rash issue. The poor guy has the chemo coming out and it just burns his poor little tushy. We're using some different prescription creams and ointments...trying to decide which one works the best. so far, nothing is helping that much.

He also pulled his NG tube out this morning the little devil. It went back in much smoother this time though. Our wonderful nurses love Jacob so much they try to make everything easy on him! I don't know what we'd do without that NG tube. It would be a nightmare trying to get him to swallow the chemo...some kids do it though. They're all amazing in my opinion.

Jacob wanted me to make sure and tell his Grampy Mark that he misses him. Grampy Mark flew home early Monday morning because he had to go back to work. Jacob is thrilled, though, to see Grandma Patty & Grandpa Steve. They arrived Tuesday morning and are staying indefinitly to help out. Jacob only could wave at Grandma Patty through the window though...she's not feeling well and can't risk subjecting Jake to anything! Grammie Cindy is here for another few days and visiting with Jake every day and helping out with everything else. Jacob doesn't know what he's going to do when she has to go. He knows she'll be back soon though...

I think that's it for now...thank you all for the prayers...Jacob needs every last one of them!

Love ~ Melanie, Steve, Jacob, & Matthew

Monday, May 13, 2002 at 09:04 PM (EDT)

Jacob has officially began his journey as a transplant patient. He was admitted on Sunday morning and I (Mommy) stayed with him his first night in the hospital. Daddy is with him tonight. We're going to try to switch off every other night and see how that works out.

He is doing very well considering he's been thrown into a crazy little world. He settled in very well yesterday...although he is very sensitive to anyone touching him at this point. He seems to be pretty afraid of what they're going to do, even if it's just to comfort him. The nurses tell me this sensitivity will eventually go away. The nurses on 5200 are wonderful. It is considered an ICU so there are only 2 patients to every 1 nurse and they work 12 hour shifts so Jacob is getting wonderful care and really gets to know his nurses. This helps with the sensitivity issue.

He is attached constantly now to a bunch of different meds. He receives his Busulfan (chemo), Dilantin (anti-seizure), and an antibiotic through his NG tube which, thank god, he has not pulled out yet although he has made several attempts the little devil. He has had one blood transfusion already, Benedryl (just in case he had a reaction to the blood), Heparin drip(stops clots from forming), and saline through his central lines. I think that's it right now but, frankly, I'm not 100% sure. He vomitted about half of his first dose of the chemo, but since then has tolerated it just fine. We can tell it is going through his little system and now have to wear gloves to change his diapers. Also, he is constantly having blood draws to check the levels of all these things. I know it sounds like a lot but it keeps us busy which makes the day and night move faster.

He did not sleep well last night (too much to see in a new environment) so I am really hoping he gets some sleep tonight. We're also hoping he doesn't get his days and nights messed up which is quite common in extended hospital stays! I will do my best to keep you all updated on this and the rest of his progress.

Thank you all for caring so much ~ Melanie, Steve, Jacob, & Matthew

Friday, May 10, 2002 at 08:05 PM (EDT)

****SEE UPDATED PHOTO ALBUM****

Enjoy ~ Mel, Steve, Jakie, & Mattie

Thursday, May 09, 2002 at 11:18 PM (EDT)

Spent the afternoon doing 'touristy' stuff with Grammie & Grammpy. It's very nice to have them here, the boys are thrilled, and we're eating well without having to do any of our own cooking! :-) Just trying to spend as much 'normal' time with Jake as we can before he's admitted to the hospital as it will probably be a very long time before we're able to do stuff like this again. Thank you all for your posts in the guestbook. We can tell you were as thrilled with the Hep B results as we were...and thank you for your prayers (calloused knees and all :-), Auntie Sharon)...they worked!

Love ~ Mel, Steve, Jake, & Matt

Wednesday, May 08, 2002 at 02:34 PM (EDT)

We just received some great news. The results from Jake's DNA test are already back (way to go Mayo Clinic on the speedy return!) and he is NEGATIVE for Hepatitis B! Yippeeeee!!! Must have been like Dr. K said...some residual virus left in his system or something. So we now have an admit day of Sunday the 12th...Mother's Day :-)...the only thing that could delay us is if there is not a room available but they don't seem to think that will be a problem.

Jacob is doing really well in spite of yesterdays discomfort and is very happy to see his Grammie Cindy & Grammpy Mark. They arrived around noon today and we are very happy they made it safely!

We also want to say how very happy we are for our friends Victor and Jennifer (their son is Henry...there is a link to his website at the bottom of this page). They received the results to their amnio today and their unborn baby girl is NOT affected with Hurler Syndrome. We wanted to share their wonderful news with all of you!

Love from Durham ~ Mel, Steve, Jake, & Matt

Tuesday, May 07, 2002 at 10:48 PM (EDT)

Well...let me begin by saying all 4 of us are sleeping at 'home' tonight. We went to Clinic this morning. They dropped the NG tube (tube going from Jakie's nose to his tummy), he really loved that (said with heavy sarcasm), and began to administer the anti-seizure medicine in anticipation of admitting him in the afternoon to begin the Busulfan (chemo) at 4am Wednesday morning pending the Hep B test results. At about 2:30pm the results came back positive...again. We were very shocked by this, as were our doctors and nurses. They truely had no reason to believe the test would be positive again...just assumed the first test was a mistake otherwise they never would have dropped the NG tube and began medicating Jake. So...now we are awaiting DNA test results to find out what is going on...the belief is still that he really does not have Hepatitis B. Dr. K thinks that he possibly had a recent minor virus that he made antibodies to and these antibodies are cross reacting with the hepatitis test. We do not know exactly when these results will be in...should have a better idea tomorrow morning, but think it is 10 days from when the blood was drawn which was last Friday so that would put us somewhere during the week of the 13TH. So we've been delayed again which is very frustrating. All we can do is think that things happen for a reason... Thank you all for your prayers...I knew all I needed to do was read your guestbook entries to make me feel better after our 'rollercoaster' day. Thank you for this.

Love ~ Melanie, Steve, Jacob, & Matthew

Monday, May 06, 2002 at 09:40 PM (EDT)

We do not have the results from Jacob's Hep B test yet...won't have the results until tomorrow afternoon. Dr. K wants to get things started tomorrow anyhow...I think she thinks the results will be negative so...we'll be going to clinic tomorrow to start on the anti-seizure medication. Once we get the results from the Hep B test we will either be admitted to 5200 (the transplant unit) that afternoon, or, if the results are positive, be sent 'home' to await DNA testing results (10 days). Needless to say, we are praying for negative.

On a happier note, Grammy Cindy & Grammpy Mark will be here tomorrow or Wednesday. They are making good time driving our vehicle to us. They had to stop a little early today due to thunderstorms/tornado warnings in Kentucky.

Love to all and thank you for your good thoughts and prayers. They really help us get through all of this...

Love ~ Melanie, Steve, Jake, & Matt

Sunday, Cinco De Mayo, 2002 at 04:52 PM (EDT)

Happy Birthday Aunt Stephanie! We love and miss you...Jacob & Matthew (and Mommy & Daddy)

Nothing new to report today. It's 'laundry day' so Jacob & Matthew are helping Mommy & Daddy with their chores.

Love to all...Melanie, Steve, Jake, & Matt

Saturday, May 04, 2002 at 04:24 PM (EDT)

We went to a wonderful event today...the Rainbow of Heroes Walk. It is a fund-raising walk for the Duke Pediatric Bone Marrow and Stem Cell Transplant Family Support Program. It is raining here today (it feels like a normal Washington Spring day to us) so the event was moved indoors but really was nice to take the boys and get out for a little while. We saw many of our wonderful doctors, nurses, and support people and met some new people as well. Once again the kindness of 'strangers' is overwhelming. We truely feel a sense of family here...it is so nice to have that so far from home. We also got to see some adorable pictures of some of Jake's friends...Henry, Emma, Maddy, & Molly (all post-transplant Hurler babies). I've added Emma's web address below in case any of you want to check it out...Jacob has a bit of a crush on her!

Love from Durham ~ Melanie, Steve, Jacob, & Matt

Friday, May 03, 2002 at 03:26 PM (EDT)

HAPPY BIRTHDAY TO GRAMMIE CINDY!!! We hope you are having a wonderful birthday (on the road) and can't wait to see you next week!!! Love you, Jakie & Mattie

We've had a minor delay in Jacob's admission for transplant. Jacob had some labs drawn last week for some tests that are needed pre-transplant...he had already had them done once when we first got here but because it's been so long they had to re-do them in order to admit him. All the tests turned out fine the first time they did them so we had no reason to believe there would be a problem...unfortunatly his Hepatitis B came back positive. We and his doctors have no reason to believe that he has Hep B as it was negative a month ago and there is really no way that he could have contracted it this past month. They believe it is a false positive which happens sometimes...but they have to be 100% sure before they admit him for transplant so he had another blood draw today, we should have the results on Monday or Tuesday and that will move his hospital admission day to either Tuesday the 7TH or Wednesday the 8TH. We do not like the delay but understand that we need to be absolutely sure that he is as healthy as possible before we put him through a transplant...

Love to all...Mel, Steve, Jake, & Matt

Thursday, May 02, 2002 at 06:25 PM (EDT)

Jacob and Matthew (and Mommy & Daddy) want to send a great big CONGRATULATIONS to Grampa Mark on his PROMOTION and new position at the City.
Way to go Grampa! Love, Jake
Great job Grampa! Love, Matt

Besides that,all is well with Jacob. We're expecting a big thunderstorm this evening (it's 83 degrees & humid right now) so we're sticking close to 'home.' Steve took the boys for a walk earlier so they weren't cooped up in the apartment all day long (and I got a chance to do a little cleaning/organizing). We have our final appointment with Dr. K tomorrow morning to sign the consent forms and are looking forward to this...

Take care and Congratulations again to Grampa Mark!!!

Love ~ Mel, Steve, Jake, Matt

Wednesday, May 01, 2002 at 11:07 PM (EDT)

Jacob saw Dr. Hulka, his ENT, today for his post-op appointment. He said his ear tubes and throat (minus tonsils & adnoids) look great. We're still working on getting hearing aids for him...unfortunatly our insurance does not cover hearing aids. If we were in Washington the State would cover them, but we're not so... Maureen, our insurance coordinator with the transplant program here at Duke is working on finding a way to get them paid for. We also will be starting some 'formal' fundraising at home...I'll keep you posted on that.

We miss you all!!!

Love ~ Mel, Steve, Jakie, & Matt

Tuesday, April 30, 2002 at 10:26 PM (EDT)

Jacob saw his neuro-surgeon today. He says that everything in regards to Jake's shunt looks great! They removed his bandages and I think it looks pretty good, although the incisions are a little bigger than I expected...I can live with that though. The important thing is it is draining the fluid! And, Jacob finally got his hair washed which makes me very happy! It was starting to get pretty yucky...

Hope you all have a wonderful day...

Love ~ Mel, Steve, Jake, & Matt

Monday, April 29, 2002 at 11:12 PM (EDT)

Jacob went to clinic today to have a bit of additional lab work done and to get checked out by his Nurse Practitioner, Bridget (Jake has a bit of a crush on Bridget). :-) She says he looks great...his lines look good...which means we're doing a good job taking care of them. This was good for us to hear because it's all new to us! If there's room for us on 5200 (the transplant ward) then we're still on for May 5TH. Yeah! Other than that, we did some grocery shopping today so we actually have food in our new little kitchen. Every day it feels a bit more like home.

Take care...all our love ~ Mel, Steve, Jake, & Matt

Sunday, April 28, 2002 at 11:10 PM (EDT)

Nothing new today...just spent the day relaxing and playing with the kids.

Saturday, April 27, 2002 at 11:18 PM (EDT)

Jacob and I spent 4 hours in the Emergency Room this evening. Steve and I noticed some redness, swelling, and odor to Jake's shunt sites and were concerned about infection/blockage to his shunt...turns out nothing is wrong but that was after some testing and time in the ER (CT scan, blood work, & exam by a neurosurgeon). Thankfully, Steve stayed 'home' with Matthew. Matthew would have gone crazy waiting around that long. Jake and I felt like we were on an episode of ER or something. Duke University Hospital is a major trauma center for the area with a couple of heli-pads. While we were there the helicopters came in with patients several times and ambulances constantly...there were people on gurneys in the halls and everything. It really was just like on TV...

We are just thankful that all is well with Jake's shunt. We can't mess around with stuff like this...it is very important for us to keep him totally healthy!

***PLEASE NOTE ADDRESS/PHONE NUMBER CHANGE BELOW***

Friday, April 26, 2002 at 11:54 PM (EDT)

Jake is doing well today...still has thrush though. It doesn't seem to be bothering him at all, but, we are trying our hardest to get rid of it so he is able to be admitted on May 5TH... We're crossing our fingers that the thrush clears up early next week so he checks out OK!!!

We spent the day packing (it's amazing what one can accumulate in a matter of 26 days!!!) and moving to an apartment. Please see new address and phone number below under the hospital information section. This is most likely where we will be for the remainder of our stay in North Carolina. It is much cleaner and more 'home-y' than the hotel we were in. It's a couple miles from the hospital so we won't be able to walk like we were doing from the hotel. There's a free shuttle from the apartment to the hospital that we will be utilizing between 8am and 5pm. Grampa Mark and Gramma Cindy are driving our vehicle here at the beginning of May. We can't wait for this as it is very difficult to get around here without one.

Thank you to everyone that has posted in the guestbook. Your kind words impact Steve and I every time we read them. I'd like to respond to everyone individually but the boys get somewhat irritated if I spend too much time on the computer...just know that it is wonderful to hear from all of you.

Love ~ Melanie, Steve, Jacob, & Matthew

Friday, April 26, 2002 at 12:09am (EDT)

All's well with Jake today. We flushed his lines this morning and administered his thrush medicine. His thrush doesn't appear to be clearing up yet but maybe he needs a couple more doses of his medicine. We had some free time today so we decided to drive to the Atlantic Ocean (Wrightsville Beach) as Steve and I have never seen the Atlantic. It's a couple hour drive but well worth it for us. It was really fun to take the boys to the beach for a little while and now we can all say we've seen the Atlantic Ocean. Jake was a little cranky during the drive back but is fine now. Tylenol seems to do the trick! He even felt up to eating a few ounces of pureed bannanas when we got back to our room. This was wonderful to see as he has not eaten anything since he had his tonsils & adnoids removed 3 weeks ago, he's been living off whole milk and vanilla Carnation Instant Breakfast. Hopefully this is a sign that his appetite is back!

Wednesday, April 24, 2002 at 09:57 PM (EDT)

Jakie is doing great today. We flushed his lines, changed his dressings, & administered his Thrush medicine this morning with the help of our home health nurse, Allison. She says we pretty much have everything figured out so she won't be returning unless we feel like we need her. It's nice to hear that we're doing well playing amateur doctor! :-) Then we had the rest of the day to ourselves so we went and did some window shopping at the brand new Mall in Durham. I, of course, loved this and the boys love going for walks so they were very happy as well. Steve was even pretty impressed with this Mall... We're taking advantage of the bit of free time we have right now because starting next week Jakie will need to stay away from any area with groups of people. We can't risk him picking up a bug this close to transplant.

Thank you all for your wonderful messages!

Love ~ Steve, Mel, Jake, & Matt

Tuesday, April 23, 2002 at 09:46 PM (EDT)

Today went well. Steve and I 'officially' learned how to flush Jacob's lines this morning. We then pretty much spent the remainder of the day at clinic. Jacob got his Diflucan through his lines to treat his Thrush and we saw Dr. Kurtzberg. She said his lines and shunt incisions look great! I'm happy he's able to get his Diflucan through his lines so I don't have to fight him to take it orally. Jake's still on track to be admitted on May 5TH (Auntie Stephanie's Birthday).

Love to all...Mel, Steve, Jakie, & Mattie

Monday, April 22, 2002 at 10:02 PM (EDT)

Today was a very nice day. We rented a car that we plan to keep for a week and were able to run some much needed errands --- went to WalMart and took Steve to see the apartment we are probably going to rent. We have one more apartment to look at tomorrow, we'll make a decision, then we'll be moving! We can't wait to have a space of our own...

We had to go to clinic again, as well, to have Jake's lines flushed and his dressings changed. The home health nurse will begin tomorrow rather than today as we previously expected. No big deal though --- we don't mind going to clinic as Jakie loves to flirt with all his new friends! Also, his dressings are only supposed to need to be changed once a week but we are having a moisture issue with our sweaty little monster. They tried something a little different this time so maybe it will work. He really does NOT like having the dressings changed though!!! Other than that all is well...

We love and miss you all...Melanie, Steve, Jacob, & Matthew

Sunday, April 21, 2002 at 05:45 PM (EDT)

Jake is doing really good today. Doesn't seem to really even need the Tylenol. We took him to clinic today to have his lines flushed and his dressings changed. He did not like that at all! I sure am glad we have a home health nurse coming to help us out tomorrow. We also took the boys for a long walk today to Duke Gardens and the Duke University campus. Those two love to go for walks --- we made sure they wore their sunblock and hats as it's 86 degrees and overcast today.

Take care...Love ~ Mel, Steve, Jake, & Matt

Saturday, April 20, 2002 at 07:55 PM (EDT)

Jacob did so well that he has already been released from the hospital. They sent us 'home' about 2pm today. Can you believe it...only one night in the hospital!!! He's such a tough guy. :-) He really seems to be doing wonderfully. He's taking some Tylenol for the pain but other than that doesn't seem to really need anything. He's still a bit sleepy but was feeling well enough to play with Matthew several times throughout the day. We'll have to go to the clinic tomorrow to have his lines flushed, Monday a home health nurse will come to us to do this...then Steve and I will be handling it on our own when we're comfortable doing it. Both of us are looking forward to learning how to take care of everything on our own. We really appreciate all the prayers and good thoughts for Jake's surgery --- they really did the trick.

Love ~ Melanie, Steve, Jacob, & Matthew

Friday, April 19, 2002 at 06:16 PM (EST)

Jacob's surgery went very well today. We had a lot of concerns about his airway but there did not seem to be a problem with it at all this time around! We are VERY happy about this because they might have had to trach him if there was a problem and we did not want that! He's at the hospital right now recovering with Daddy. I came back to the room for a bit so Mattie could take a nap. We're going to go back over once more this evening, Steve is going to stay the night (we'll be rotating nights) and Matthew and I will come back to our hotel room. Jacob appears to be doing really well...seems less fussy than after his tonsils and adnoids came out, but is still really groggy. The poor little guy went through a lot today! The lines look really good and are placed close together so they will apparently be easier to take care of and the neuro-surgean only had to shave 2 small areas of Jakie's hair for the shunt placement. Next step is about 2 weeks of recovery, chemo, then TRANSPLANT...we can't wait!

Thursday, April 18, 2002 at 04:57 PM (CDT)

STEVE AND MATTHEW HAVE ARRIVED!!! We are so happy they are here. Their flight arrived on time this morning and all went well with that. They are pretty wiped out from flying all night though. We had a wonderful little reunion this morning and are just so happy that our little family is all together again.

We all went to Jacob's pre-op appointment today. He had to have some blood drawn and get all checked out to make sure it's OK to do the surgery. All is well and it's a go for tomorrow morning at 7:45am (we have to arrive at the hospital at 6:15am!). He'll have his shunt and his central lines put in at that time and we'll be spending 2 or 3 nights in the hospital. There's a bed available in the transplant unit this weekend so they're going to let Jacob recover there...it will be nice to meet some of the nurses, doctors, parents, & kids that we'll be spending so much time with here in the very near future.

We miss you all! I'll do my best to post an update after Jakie's surgery tomorrow.

Love ~ Melanie, Steve, Jacob, & Matthew

Wednesday, April 17, 2002 at 02:04 PM (CDT)

Jacob is having another great day. The weather is gorgeous and HOT (90+ degrees) so we went for a bit of a walk before our appointment with Dr. Kurtzberg (Jake's wonderful transplant doctor) this morning. She went over all the test results from the past two weeks with us and everything looks pretty good from a transplant perspective. It's all a go once we get this shunt surgery out of the way. Jacob has some mild to moderate hearing loss so he's going to be fitted for some hearing aids. This should help him with his speaking because he will finally be able to hear what we are saying to him! This hearing loss is pretty normal in kids with Hurler's Syndrome. Down the road a ways it will be repaired by the new cells in his body and eventually he won't need the hearing aids. We also talked a bit more about his surgery Friday. They are going to go ahead and put his central lines in (necessary for the transplant) during this time period as well so that we don't have to put him to sleep again. Anesthesia is risky for Hurler's kids especially Jakie because his airway is fairly blocked. Dr. Hulka, his excellent ENT doc will be on hand in case there are any airway issues. I feel very comfortable with this surgery and with the way things are progressing thus far. We have a tentative admit date of May 5, 2002 for the transplant with chemo beginning at 4am on May 6, 2002. This will give his body enough time to heal from the shunt surgery. Other than that we are anxiously awaiting the arrival of Daddy and Mattie tomorrow morning at 8:18am. Jakie has been pointing at all of the pictures of them I brought with us for days!

Thank you all for your wonderful, heartwarming, inspirational post to Jacob's Guestbook. We can't thank you enough for all your well wishes, prayers, and good thoughts.

Love, Mel & Jake

Tuesday, April 16, 2002 at 05:07 PM (CDT)

Hello All! Finally, I'm able to post an entry! A great BIG THANK YOU to Grandma Patty & Grandpa Steve for the new laptop computer. It is going to be much easier for me to keep all Jacob's wonderful family & friends updated on his progress! Also, thank you all for the kind words and warm thoughts posted to the Guestbook. Jacob is going to enjoy reading all of those when he's a little older!

Jacob is doing well today, he never acts like anything is wrong though! He is mostly recovered from his T&A surgery a week and a half ago although he has thrush now from the antibiotics & steroids he was on. We're treating that now... We met with Dr. Fuchs, the neuro-surgeon today. Jacob has hydrocephalus (fluid on his brain) and will need a shunt (tube inserted from his brain to his belly under the skin)to drain this fluid. We need to do this prior to his transplant as his body will be too weakened from the transplant to do it after. The surgery is scheduled for this coming Friday. He'll need to recover 10-14 days after this surgery and then they can begin chemo. We are very ready to get going with everything!

Daddy and Matthew arrive Thursday morning and we can't wait. Jacob (& Mommy) misses them soooooo much!!!

I'll be updating more frequently now so please check back soon.

Love, Melanie & Jakie

Friday, April 05, 2002 at 01:25 PM (CST)

Hello all... Thank you all for your kind words and thoughts. It's wonderful to know that you are all out there thinking of us here in North Carolina. I've finally found a computer I can access the Internet on --- yeah! Once we become in-patient at Duke this won't be such a difficult task! Right now, I am in the Medical Library using one of the student computers.

Anyhow, Jakie is in his hospital room with Grammie Cindy. He had his tonsils and adnoids removed yesterday morning and tubes put in his ears to help with the ear infections. The surgery went well, but we will be spending a few days in the hospital to monitor his oxygen level. We need to make sure he is breathing well on his own before they release him from the hospital. He's such a little trooper and is taking things in stride. We have all weekend to recuperate from this surgery...then Monday we begin again with more appointments! Grammie Cindy leaves tomorrow morning and we will be very sad to see her go but Daddy and Matthew will be here soon!!! Thank you all again for checking in on us. I'll update as soon as I can...

Wednesday, March 27, 2002 at 01:50 AM (CST)

Jacob, Mommy, and Grammie Cindy will be flying to North Carolina on Saturday, March 30TH at 11:40pm and will be arriving in Durham, North Carolina Sunday morning. We begin work-ups and pre-transplant testing Monday, April 1ST and will have a very busy couple weeks. If all goes well, Jacob will be getting his cord blood transplant the week of April 22ND. Please check back for updates...I will do my best to keep things somewhat current.

Friday, March 22, 2002 at 03:53 AM (CST)

This page has just been created. Please check back for additional updates.

Click here to go back to the main page.

----End of History----