Jackson's Journal

Hi friends! Sorry it's taken me so long to update.

Let's see, last time I wrote we had just gotten home from Duke. Shortly after we arrived home Jackson developed a rash over his whole body that got progressively worse over the course of a few days. Despite many calls and emails to Jackson's medical team here, they basically ignored my concerns by putting me off (telling me they would talk to the doctor and get back to me and then not calling back) and giving me ridiculous treatment recommendations (to put baby powder on it?!). It was incredibly frustrating! This rash was clearly NOT RIGHT and nobody was taking me seriously. This went on for a week and a half before we got some resolution.

We happened to have an appointment at a different hospital to see a new orthopedic doctor. While in to see her she noted the rash and sent us right across the hall to the pediatric urgent care clinic. We had excellent treatment there - they took a full history, cultured his rash in a few places, contacted his transplant team for more history, and had the infectious disease team come see Jackson to make sure he was being diagnosed and treated correctly. We were very pleased with the care (and may end up transferring Jackson's transplant care to this facility).

As it turns out, the cultures taken from Jackson's rash grew out two nasty bacteria - MRSA and psuedomonas. Both of these bacteria can be very dangerous in people with suppressed immune systems. Jackson was treated with antibiotics and the rash cleared up, but it was scary that this rash went unchecked for so long. And extremely aggravating that we got such a brush-off from his team here when I was sounding the alarm bells and trying to get them to pay attention.

ANYWAY! That's old news. Jackson is fine now and that's what matters.

We did decide to keep Jackson at home for school after all. If the school district opens up a spot in the augmentative communication class for him then we will reconsider, but for now he's continuing with the same teacher that has been working with him for years now. She's wonderful and they have a great rapport. She challenges him and he really responds well to her. He's also continuing with his speech and physical therapists from last year. We were sad to lose our beloved occupational therapist, Mary Beth, who recently moved to Sacramento. She had been working with Jackson since he was 2 and has been a big part of our lives, and she is greatly missed!

Last week Jackson had a trial of a new communication device that utilizes eye gaze as a form of access. He did very well with it - he was able to play a Whack-A-Mole game with success, and played around with some of the alphabet and core vocabulary pages. He's going to trial a different device in a few weeks so that we can compare the two, and hopefully we'll move forward with trying to secure the funding for one shortly after that. I think it will be much easier for him to use than his current device, which relies much more on neck strength and head control for accuracy.

Alexa and Maya are wonderful little bundles of enery. They're toddling all over and getting into everything . Maya is really starting to talk and loves to repeat everything said to her. Alexa loves to read and help me in the kitchen. They're keeping me busy but it's so much fun. Watching them develop is a whole new experience for Marshall and I and every day we're amazed by the things they can do. They fill the house with so much joy .

On a sad note, Marshall's Uncle Quincy passed away yesterday. He was a wonderful man and he will be greatly missed. We were supposed to see him at Marshall's family reunion this past June, but we had to miss it because of the problems with Jackson's port infections. We are very sad that we didn't get to see him one last time. Rest in peace, Uncle Quincy.

Thanks for checking in. Peace and blessings.

Hi everyone, sorry for taking so long to update after Jackson's second surgery. He's fine now, but he did have a little setback while he was in the hospital after the surgery.

The second surgery was to have a new port put in and also had the tendons in his legs released (they were so tight they were pulling his hips out of socket). Both procedures went well from a surgical standpoint, and we were very grateful to have a room on the transplant unit waiting for him.

After surgery he needed to be on a little bit of O2 to keep his oxygen saturations above 90%, which happened after his previous surgery as well so not very concerning. After we got to his room the nurse started weaning him off the O2 and he was tolerating the wean just fine. I think he started out on 3L via nasal cannula when we got to the room, and the nurse weaned him down to .5L by early morning. He was having lots of pain and we were having a hard time getting it under control again so that was no fun but he appeared to be doing fine otherwise.

Around 5am things started to turn for the worse. His started requiring more oxygen to keep his sats up again. By noon he was back up to 3L of O2 and still having trouble staying above 90%. They did a chest xray that showed a small pneumonia (it was behind his heart and difficult to see), so they started a barrage of antibiotics and switched him to getting his oxygen via mask. At this time he was also still in quite a bit of pain so the docs ordered some higher doses of a couple different things (narcotics and muscle relaxers), and it pretty much zonked him out which made it even harder for him to take deep breaths. This downward cycle continued and by 3pm he was in major respiratory distress... at one point we had 4 docs, 1 NP and 2 nurses in his room discussing whether or not he needed to go to the PICU and get intubated. I've never seen him have such a hard time breathing, and it was incredibly scary.

Luckily things turned around as quickly as they had gone downhill. They switched him to a different kind of oxygen mask and that seemed to help. He also started waking up from his drug haze and was able to get deeper breaths. They gave him some Lasix to remove some of the fluid from his body in case that was making things worse. By 6pm he was much more stable, and talk of PICU had quieted down. That was Friday. Amazingly, by Saturday we were able to start weaning down his O2 and he was completely on room air by Sunday. He was discharged on Monday morning.

We stayed at Duke until the end of the week, and were released to travel this past Friday. You'd never even know Jackson had been so sick - he's just got a little cough left over and otherwise is doing great. His resiliance and ability to bounce back are just amazing to me.

It was a real wake-up call for me though. I've lulled myself into thinking he could just fight off any bug that he was exposed to since he's done such a great job of recovering from colds and such, but this experience showed me how truly fragile he is if he gets a bug in the wrong place (his lungs) or the wrong time (when his resistance is already down). And he got SO sick SO fast - if he had been on a different unit where the doctors weren't right there and on top of things, I don't even want to think about what could have happened. He went from doing fine to practically needing intubation within the span of 12 hours. ::shudder::

We left on Saturday to drive home and arrived home late Wednesday night. The kids all survived the drive (and Marshall and I did too) and we're all very happy to be home. Missing our North Carolina friends and doctors though - they took such outstanding care of us.

Jackson's school is set to start week after next and we're doing some re-evaluating of whether or not we want to put him in public school. I'll update about that when we make our decision. Thanks for checking on us and for your prayers.

I realize that along our journey we've picked up a few new friends that might not know the beginning of the story. When we were going through transplant I blogged nearly every day on this website, but about 1 year after transplant our site got hacked and that portion of the website was taken down (we still have it archived at home). Since that information isn't available on Jackson's website anymore, I thought I would go ahead and post a recap of Jackson's story here for those of you that aren't familiar with what happened, how he was diagnosed, and the early transplant story....

Jackson's Story

Jackson was born on March 30, 2003 after a healthy pregnancy. He was absolutely perfect in every way, and Marshall and I were overjoyed to be first-time parents. We delighted in watching him smile, learn new things, and engage in the world around him.

Jackson always had a very peaceful spirit. He was a content baby that didn't cry much - he was generally happy as long as he had loved ones around him. He developed skills at a typical rate, learning to sit up and crawl well within the normal time frame. Nothing in his early development led us to believe there could be anything wrong with our son.

When he was about 16 months old, we began to notice that he was not sitting as strongly as he had been previously. He began needing to steady himself with a hand on the ground, where he had previously been able to sit and play with a toy with both hands. We took him to his pediatrician and she immediately sent us to a neurologist for a work-up. Within two weeks he had an MRI that showed demyelination in his brain. Based on the pattern of demyelination and his physical symptoms, Jackson was diagnosed with a leukodystrophy - a disease that would rapidly rob him of all his abilities and would eventually take his life. We were told he wouldn't live past his 5th birthday. I can't even begin to describe the pain we felt on hearing those words, and how our world came crashing to a halt that day at the neurologist's office.

There are many different leukodystrophies, and Jackson began to undergo testing to determine which particular leukodystrophy he had. At first we were told that he most likely had a form called MLD (metachromatic leukodystrophy) because of his symptoms and his age of onset, but confirmatory blood tests were negative for this disease. We sent more blood work to a lab in Philadelphia that specializes in this area, and about a month after Jackson's initial MRI we received his final diagnosis... Jackson had Krabbe Disease. He has a rare, late-onset form (most babies with Krabbe Disease begin showing symptoms by 6 months of age), which was why he wasn't tested for it initially. When our neurologist called with the news, he told us that there was no hope - the best we could do would be to make Jackson as comfortable as possible and await his inevitable death.

Despite this grim prognosis, we were determined to find any possible means of treatment.

That night, during an exhaustive internet search for treatments for Krabbe, I found information about a program at Duke University that was having some success in treating leukodystrophies with cord blood transplant. I sent an email to the head of the program, Dr. Joanne Kurtzberg, that night at midnight and I had a response from her by the time I woke up the next morning. She wanted us to fly to Duke for further evaluation to see if he would benefit from a transplant. A flicker of hope was lit.

Within a week we were in Durham, North Carolina. Jackson went through a grueling week of more testing to determine if he was a good candidate for a cord blood transplant. Since Jackson has a rare form of Krabbe Disease, the doctors at Duke had very few patients to compare him with. Because of their limited data they couldn't give us a definite answer as to whether transplant would help him. Would the transplant give him a good quality of life, albeit with some disabilities? Or would we be extending his life only to watch him slip into the clutches of the disease anyway? With hope in our hearts, we chose to pursue transplant.

Transplant Journey

The transplant journey is not an easy one. At the first meeting with the medical team we heard all the risks associated with the process - risk of death from this, risk of death from that. It seemed like there were almost more risks than benefits. This is why transplant is a 'last-ditch' effort to save a life, and only used when every other option has failed. For diseases like Krabbe, there are no other options. Over the past two months we had gone from being a normal, happy family to finding ourselves on the brink of a 'last-ditch' effort to save Jackson's life. Our heads were still spinning.

During the previous two months, Jackson had continued to lose skills. By the time we arrived in North Carolina, Jackson was no longer able to crawl or sit unassisted. He started having difficulties with eating solid foods, although he was still able to nurse. He was speaking less and losing the ability to form some of his words. Even though this loss, he remained a cheerful and sweet child. He gave us so much strength through his gentle spirit.

Jackson was admitted to the hospital to begin his pre-transplant chemo on October 19, 2004 (my birthday). He received 8 days of high-dose chemotherapy and immune suppressants which destroyed his bone marrow. On October 29, 2004 he received his cord blood transplant - a unit of cord blood from a healthy baby boy born in North Carolina. The transplant itself was very uneventful... the cord blood trickled in like a blood transfusion, and Jackson slept through the whole thing. But underneath that quiet process a huge moment was passing - our son was being saved. This blood would give him a chance at life.

Jackson's post-transplant path was remarkably smooth, without major infections or illnesses. We learned on post-transplant day 30 that his donor cells (the term for the cord blood cells in Jackson's body) did not fully engraft, meaning that his body was threatening to reject the transplant. Since then we have aggressively and creatively tinkered with his immune suppressant medications to ensure that enough of those life-giving cells stay in his body to produce the enzyme he needs to survive. So far, we are winning that battle. However, to ensure that his body doesn't completely reject the donor cells, Jackson remains on high doses of immune suppressants and will continue for the forseeable future.

The transplant is considered a success because it has stopped the disease in it's tracks, because the demyelination will not progress further and take more of his skills. Unfortunately the disease did quite a bit of damage to his nervous system before the transplant was able to stop it, and as a result Jackson will always have some level of disability. Although he has fairly good head control, currently he is not able to walk, crawl, or even sit unassisted. He is not able to speak. He is fed through a tube because he lacks to oral motor strength to coordinate eating. This probably paints the picture of a profoundly disabled child, but Jackson's spirit makes all of that seem unimportant. Jackson is very smart and understand everything going on around him - his developmental testing shows him at the cognitive age of a 6 - 6.5 year old (he turned 5 in March). He uses eye gaze, facial expression, and computerized devices to express himself. He will start kindergarten in the fall and is learning to read. He has an amazing smile and laugh, enjoys jokes, and can light up a room with his gorgeous brown eyes.

Through all this, he still has an amazing disposition and positive outlook. He is sweet, funny, kind, and cheerful. He is incredibly laid-back and mellow, yet can be very determined and definite about things when he wants to be. Quite simply, he is an amazing boy and has taught me more about living life and love than I ever thought possible. He fights so hard for things that most of us take for granted, yet he takes all of his difficulties in stride with a smile. He is still medically fragile and on a lot of medications, but we hope that he will grace our lives for years and years to come.

Jackson's first surgery went OK. Not great, but it could have been worse, and he's recovered in true Jackson style. After the surgery he struggled with keeping his airway open and his oxygen levels up, and needed to get some oxygen support for about 24 hours after the procedure. This was not unexpected, but was still scary for me. There was a time during his immediate post-op recovery that I was actually propping his airway open with my hands (holding his chin forward so that his tongue didn't fall back and occlude his breathing) in order to avoid the need to re-intubate him. That only lasted a short while but it was too long for me! He also required continuous IV pain medication for the first 24 hours as well. Thankfully after a good, long nap on Saturday we were able to wean both the O2 and the IV meds down. Currently he's just on Tylenol with codeine for pain and it's keeping him comfortable.

I can already see the positive effects from having his tonsils/adenoids removed. He's breathing much clearer and easier now, and he's had less drooling and coughing/congestion as well. It was such a sudden decision to move forward with the T&A that I was nervous we were being too aggressive, but it looks like we did make the right decision after all. The team here at Duke always seems to steer us in the right direction.

One nice part to our stay at the hospital was that there was a room available on the transplant unit for us, so we were able to see some of the nurses that took care of Jackson during his transplant. Actually, Jackson's primary nurse from transplant was the same nurse assigned to him this weekend! It was nice to catch up with her and give her a chance to see how much Jackson has grown up. He was practically a baby when he went through transplant - he was only 19 months old at the time. I spoke to a few families in the unit going through transplant right now, and it was so wonderful to be able to tell them that we are nearly 4 years out and doing well. They need all the positive and hopeful stories they can get.

Next up is the placement of a new port and a tendon lengthening procedure on his hips. The tendon lengthening surgery will release some of the tightness in his hips and thighs, which will make it easier to position him and will hopefully help keep his hips from being dislocated in the future. It should be a relatively simple surgery that would typically be done as an outpatient, but Jackson may have to spend a night or two in the hospital to make sure he stay stable afterwards. I don't think we have too many appointments between now and the next surgery, which is a welcome break. I hope we'll be able to do some fun things with the kids over the next few days.

Thanks for checking in and for keeping Jackson in your prayers. Peace and blessings.

Hi all!

Just wanted to send out a little update on Jackson and our trip. We had a wonderful time in the Hamptons with my dad and family, where Jackson enjoyed lots of beach and pool time. Then on to the Krabbe Symposium, where Jackson's favorite part was seeing his penpal Gina (another transplanted Krabbe child). It was so adorable to see them together - Marshall directed them in "talking with their eyes" to each other, they would say hello and goodbye with their eyes each time they met. Amazing to see how they both understood immediately what to do, and to watch them looking deeply into each other's eyes to communicate was beautiful.

We're now here at Duke. So far all of Jackson's post-transplant testing has gone well. His developmental testing went very well this year, and he was answering questions in the 6 to 6 1/2 year old range when he finally tired out . His enzyme level was at an amazing 0.7 (the highest we've seen it in over 2 years), even though his donor cell count was down to 8%. We had been operating under the assumption that those two numbers should correllate, but we are being proven wrong and that's fine with us! His MRI and EEG are stable, so all is well from a disease standpoint.

And now the not-so-good news... Jackson has another blood infection . He is back on IV antibiotics, and the plan is to have his current port removed tomorrow and a new one placed next week. We didn't like the location of his current port at all, so this is another reason to remove it. Since he has to be under anesthesia, we are trying to think of all the possible surgical procedures he might need in the coming year and attempt to do them while we are here. Right now it looks like Jackson will be having his tonsils and adenoids removed because they cause breathing problems when he sleeps (he will have this done tomorrow along with the port removal), and will have a tendon lengthening procedure done on his hips (this will happen when his new port is placed next week or early the following week).

It's a lot to process, it's happening fast! But we really trust the team here and feel that we are doing what's best for Jackson. Would you please keep Jackson in your prayers as he goes through these surgeries? The tonsil/adenoid procedure will be particularly difficult for him, as it can be painful and there is a possibility of respiratory problems post-op (both should resolve, but will be tough on him for a few days).

All of this means we won't be home until later in August. We will keep you posted on how things are going. Thanks for your prayers.

Peace and blessings,
D'Anne

Hi all,

Jackson has had a few ups and downs over the past few weeks. It started about a month ago when he spiked a temp and wasn't acting like his usual cheerful self. We took him in to the ED for bloodwork and a chest x-ray, all of which came back clear (except the blood cultures, which take at least 8 hours for preliminary results). They gave him a dose of IV antibiotics and sent us home. The next day they called us to let us know that his blood culture was growing bacteria (meaning he has an infection in his blood), so he had to be admitted for a couple of days. They were able to discharge him quickly and he continued on IV antibiotics at home for 10 days.

Four days after he finished his course of antibiotics, we took him in to clinic to have blood cultures drawn to make sure the infection was cleared. Unfortunately they called us the next day to tell us that the cultures were again growing bacteria - the same bug that had grown the first time. Because of the quick relapse the team felt that the infection was most likely being harbored inside his port... which means that the port had to be removed. Jackson had his port removed a week ago and a PICC line was placed temporarily so that we could continue to administer IV antibiotics.

The plan now is for him to get a full two weeks of antibiotics, then draw cultures and make sure the infection is completely gone, and only then will he go in to have another port placed.

Despite the turmoil of all this, Jackson has been doing really well. He hasn't been bothered by fevers other than that initial fever. He seems to be feeling just fine. He did have some pain after the port removal but we were able to control it well. We are very thankful that most of this has been done on an outpatient basis, which really limits his exposure to whatever germs he might pick up in the hospital. His port placement should also be outpatient. We hope that everything continues to follow the plan we have laid out with his team here and no other bumps pop up.

In other news, we had Part II of his IEP this week. It looks like, as of right now, Jackson won't be going into the wonderful communication-based classroom in the Fall. We put together a very good plan for him that will serve him well in the other class, and there is a chance that we may get a call during the summer to tell us that his placement has been changed to the communication class (we have our fingers crossed for this!). Overall we're happy with his program and excited for him to start Kindergarten in the Fall. Whether or not I'll be able to let go of him is another story .

We hope all is well with everyone checking in with us. If you have a moment, please send up a prayer for all the other Krabbe families. Thanks so much.

Peace and blessings,
D'Anne

Jackson's IEP meeting was today. This is the meeting where all of his team members - teacher, therapists, nurse, administrators - meet to discuss what he has accomplished in the past year and to set goals for the coming year. There's no better way to spend an afternoon than by sitting and listening to people gush over how wonderful and smart he is . He's done an outstanding job of accomplishing the goals set for him a year ago, and I liked the future goals that his team came up with as well.

We also spent some time talking about potential KINDERGARTEN sites for him! I can hardly believe he's about to start Kindergarten. When did my little baby grow into this boy?! There are two possible classes being looked at for him, and we should find out which he will be placed into by the beginning of June. One of the classes is a class specifically for children that use communication devices, and Marshall and I are leaning strongly towards that class for him. Unfortunately we don't get much say - it depends on a lot of other factors, and this class (called "the AAC class") will have it's own application process. It sounds like his team is leaning towards this class as well but we have to wait and see what the district decides after we submit all his information.

It was a very good meeting overall. Our district has definitely done right by him so far, they genuinely seem to care about him and want the best for him. Hopefully that extends to putting him into the communication/technology class!

On a final note, I have to give a shout out to my babies - they were kind enough to nap for the entire 2.5 hour meeting. They are little rock stars .

I cannot believe my baby is FIVE! This birthday held a special meaning for me. Once upon a time, we were given some incredibly bad news. Before it was determined that Jackson had Krabbe disease and not MLD, we were told that Jackson would probably not live to see this birthday. His prognosis changed once we got the true diagnosis and he went to transplant, but that ominous deadline has always stayed in the back of my memory. Well, he made it past that day and is doing better than ever, and it feels like a major victory. Every day feels like a victory .

He's doing incredibly well. The biggest accomplishment recently has been with his communicator. We recently started using a device that enables him to move the mouse cursor with his head, and the whole thing really 'clicked' for him last month. He's still learning how to improve his accuracy, but he's doing much better and is able to say things to us rather than just being able to answer yes or no questions. It's pretty amazing to be in another room and hear him say "Mommy I love you" .

His therapies and school (still in-home) are also going well. We're looking at sending him to kindergarten in the fall! I'm equally very excited and totally terrified by this prospect. The district has recently formed a class specifically for non-verbal kids that use communication devices that would be PERFECT for him, and the creation of this class has been a deciding factor in whether or not he'll start in the fall. We won't know if he gets a spot in this class until after his IEP later in April, but we're hopeful.

The girls are one! This year has gone by so fast. Alexa is starting to walk and Maya still gets around with her funky one-legged crawl (she puts one foot on the ground and scoots the other leg underneath her). They're both signing 'milk' as of this week and Maya just learned to clap. Neither have any real words that I'm convinced of although we have lots of babbling - Maya says the cutest "ducka-ducka-ducka" thing.

They are both very complex little people - there's no "this is the quiet one" or "this is the friendly one". They both have their moments of silliness, seriousness, contemplation, crankiness, etc. I'm just amazed by them and I love watching their personalities emerge.

We had a wonderful party for all three of them last weekend, with a petting zoo and lots of family and friends. Jackson LOVED the petting zoo. We got a bunch of great pictures but I'm having some trouble uploading them to the website photo album. I was able to upload some to photobucket to tide you all over, so here are a few. I'll try and get the rest on his website photo album in the next few days. Enjoy!

Jackson holds a bunny

Maya

Alexa

Happy Birthday, Jackson!!

I haven't done a thorough update in so long! I apologize for that - Jackson is doing so well and I should take a moment to document that.

Aside from the enzyme level scare of last month, things are going very smoothly. We've managed to avoid any hospitalizations or major illnesses for quite a while now, something that we are so grateful for. He's very active with therapy and school. He continues to have the same gentle and happy spirit, which continues to inspire us and give us so much joy.

At just about every session his therapists say something to the extent of "I've never seen him do that before!", which means that steady progress is being made. He's getting better at bringing his hands to midline, and his trunk muscles and postural and protective reflexes continue to improve (being able to right himself when he's tilted, putting his arms out to "catch" himself when he's moved from a center position). He recently got a new trunk support vest that helps him sit up straighter and protects his spine from scoliosis. He didn't love it at first, but as he gets used to it I think he is seeing how it helps him. He's due for another round of Botox injections in his biceps, legs (hamstrings, adductors), and hands which should again help loosen those muscle groups and allow him better functional movement.

He's learning a lot in school these days too. His teacher (in-home) is working from the kindergarten curriculum with him and it's amazing to see him respond and learn things. He's able to recognize about 10 words and can tell you when a sentence sounds right or when it's wrong. He's learned to count by 10s and is now working on counting by 5s. He's learning to count money by getting a penny every day of the month, and gets to buy something from his teacher on the last day of the month. We are doing fun science experiments with him like finding out what things dissolve in water, and the difference between gas/liquid/solid. As soon as the weather warms up a little he's going to plant and care for a little garden - he will pick what he wants to grow and help water and care for it. He's really excited about that.

He's recently enjoyed writing letters to family and has a new pen pal in Ohio! His pen pal is Gina, another child with Krabbe that we get to see every year at symposium. This weekend we've been busy making valentines to mail out to his friends.

Health-wise he's been stable. He had a stomach virus a few weeks ago and it takes him a while to completely bounce back from something like that, but he's pretty much better now. He's been a little more willing to try tastes of foods now that the girls are eating table foods too - we all sit down together and he tries what they're eating. He helped me make cupcakes a few days ago and wanted to lick the beaters .

He still gets IV fluids through his port every other night. I'm anxious to start decreasing that but the medical team here wants to wait until spring to make any changes. I just want things to be as normal for him as possible.

In other medical news, I was recently approved to work as Jackson's home health RN through Medi-Cal! This means I get paid full-time wages to be at home with him, caring for him as I always have. This is a huge blessing financially. I only wish we had found out about this program earlier.

We are tentatively planning another cross-country road trip for this summer. Marshall's family is having their big reunion in Michigan and the timing lines up with the Hunter's Hope symposium, so we'll try and do both of those events and schedule Jackson's 4 year post-transplant follow-up at Duke for just afterwards. I think the girls will have a harder time than last year, but they still seem to be very mellow babies and I think they'll tolerate it OK as long as we make lots of stops.

Speaking of the babies, they are 10 months old now and just so much fun! They are getting to the age where they are starting to be able to do things that Jackson never did (clapping, pointing, standing unassisted), and it's eye-opening to see what normal development looks like from this point on. It's a little bittersweet. I'm realizing that even though I have a 4 year old, there is a LOT about parenting that I don't know! Tantrums? Potty training?? My role as a mother has been very different than the typical one, and I have a lot to learn. Thankfully the girls are patient and gentle with me .

Overall, we are very very blessed. Jackson is going to be 5 in March!! So many families lose a child to this horrible disease, and we are holding on to each precious day and savoring each moment. Thank you to everyone that has held us in their thoughts and prayers. No matter what the future holds, we are forever grateful.

Repeat enzyme level was higher - back up to 0.3. Thank you God!

We received Jackson's most recent enzyme level and donor cell counts back this morning. It's confusing, but potentially really bad news.

His enzyme level is 0.1 . That is very very very very low, the lowest it's ever been except for when he was first diagnosed (it was 0 then). We are going to do a repeat level in the hopes that it's a lab error.

It's hard to say exactly what this means, and I'll do my best to explain why. Nobody knows what the threshold level is for the enzyme, what amount needs to be present in order to prevent myelin destruction and prevent his symptoms from progressing. Previously we had been told that the Duke team feels comfortable with an enzyme level above 0.5, but he's been below that for nearly a year now so we were taking a wait-and-see approach. His enzyme level was 0.3 at last test 3 months ago, and before that was 0.4 for probably the 9 months before that. So it has been dropping but slowly.

His donor cell count further complicates matters. The whole blood (overall) count was 11% donor cells, which is stable from the previous result. They fractionate the whole blood out to different kinds of white cells and do a donor cell count on those (they represent different things happening in the blood production) and those were both 7% which is quite a bit lower than previous tests, but it doesn't really make sense in light of the whole blood count so that may be an inaccurate level too. I don't know though, it seems like too many errors to happen at one time.

The bottom line here, in more understandable terms, is that this enzyme level could be indicating that the transplant is failing. If that's the case, the disease will start progressing again soon if it hasn't already. We could be looking at heading back to Duke for a second transplant in the very near future.

I am so scared. Please, please, please God let this be an error. Please let the enzyme level be higher on the retest. We should get the retest results on Friday.

Please send up a prayer or a good thought for Jackson, and for the rest of our family too. I know God will carry us through whatever is to come, but I am really nervous right now. I just feel sick inside thinking about going through it all over again.

Our holidays were wonderful - so much joy and so many blessings! Everyone is healthy and happy. I've posted some new pictures so check them out if you have time. Happy 2008 to everyone!

It is with a very heavy heart that I make this post. Ashleigh Ryann Gwin, beloved daughter of Michelle and Howard, beautiful sister of Mackenzie, passed into God's arms one week ago. I have been too shocked and sad to write about it. I am still at a loss for words. I wish I could make a beautiful post about her life and her incredible will to live... about her parents' love for her and their tireless pursuit of a better life for her... about the hope her life gave me for Jackson, and the courage she gave me to never give up... but I sit here at the computer and still can't come up with anything other than deep sadness and grief. I hope to find the words to pay proper tribute to this angel in the coming days.

Sweet Ashleigh, you will always be missed.

New pictures posted!

Hi Friends,

Things are good here at Casa White. We're having a blast with these two little babies, they're so much fun! Jackson is a great big brother, and enjoys hanging out with them and rolling on the floor together.

He's had some GI issues recently, similar to what was going on in January when he went into the hospital. It's been frustrating that we can't seem to find a cause for his issues, and nobody on his California medical team seems all that interested in helping with that. I guess as long as his labs stay within normal limits and he doesn't get fevers then we're still OK, but it's still a little scary to have him dealing with something that landed him in the hospital for a month before. Hopefully we'll start seeing some improvement in that area soon.

Jackson's school (in-home) and therapies are going well. He really is getting smart . It's fun to see what he knows and understands. His teacher challenges him and he enjoys it. His therapists have worked with him long enough to know and understand him well, and it's nice to feel like they have a good handle on what he can do. It's not easy to find good therapists... we've been very lucky in putting together this team.

Other than that, things are pretty much the same. We passed the whole diagnos-iversary and the mourning that goes along with that time, and can now get back to enjoying life with our wonderful kids. Back to feeling very, very blessed. Thanks to everyone that sent us good thoughts, a note, an email, or otherwise held us up during that time - your continued support is so needed and we are grateful for it.

And speaking of support, I've unfortunately had to disable some of the comments sections of the blog . It was getting to where I was spending way more time deleting spam comments than doing anything else on the website, and that wasn't much fun. I will leave comments open on new posts until the spam-bots find them, then I'll disable, so if you want to comment you better do it quickly! Please feel free to send me an email if you would like - I can't guarantee I'll answer in a timely fashion (one of my worst personality flaws... that and thank you notes) but that will still be a way to let us know you're thinking about Jackson and our family.

Again, thanks for continuing to check on us. God bless.

Peace and blessings,
D'Anne

P.S. I'll try to get some more pictures up soon!

I've been struggling a little these past few days. Monday was the third anniversary of getting Jackson's first MRI results, when our world basically crumbled in the time it took the doctor to say the words "he will probably die by the time he's 5." As much as our outlook has changed since that day, the trauma of that moment (and the month after it, until we got the firm diagnosis of Krabbe and received our first glimmer of hope in the form of an email from Dr. Kurtzberg) will always sort of define me. I've been feeling this more acutely recently.

Things are good right now, I just need to spend a little time processing everything. This time of year always brings up lots of anxiety and emotions, and I feel that grief all over again. I know I have so much to be thankful for. Regardless, the past three years have been very hard.

I was working on Jackson's communication board (picture symbols) last night and started going through some old photos. For the first time, I looked through the pictures of that time three years ago with the purpose of seeing when Jackson started having symptoms - and it's very clear, and happened very quickly.

Here are two pictures that were uploaded to our computer at the same time - 5/29/04. The previous grouping of pictures was uploaded on 5/18/04, so these two pictures were taken no more than 11 days apart.

A picture from early in the grouping. He's sitting very straight.

Then, later in the grouping of pictures. Notice the difference in his trunk.

Now, here's a picture that was uploaded on 7/2/04. Apparently I had subconsciously noticed his instability - can you tell? I didn't consciously know something was wrong until our vacation to Grand Cayman later in the month.

And this one was uploaded 8/10/04. He needs a boppy to sit upright (and probably falls even with that support, given the pillows around the boppy). At this point we knew something was very wrong and were waiting for his first MRI (done 8/12).

Thankfully, he seemed to stabilize at that point because he still had approximately the same amount of trunk strength in the early pictures at Duke and through transplant. He was very weak after transplant, but it's hard to say what was disease progression and what was weakness from the chemo and steroids. He can't sit as well as those pictures now, but he's also taller and heavier so I think that probably makes a difference. Plus his legs/hips/pelvis are stiffer so they don't give him the "base" he needs to sit properly.

So there you have it. Krabbe progression in photos. I love this sweet boy so incredibly much for who he is. And I hate this disease with every fiber of my being.

Here we are on our "monster road trip", as Marshall calls it! Things are going great, I can hardly believe how well everyone is doing with the long car rides. The babies sleep a lot and cheerfully look out the window the rest of the time. With Jackson we're talking a lot about the different states we go through - looking at them on the map and reading from a book with tidbits of information about the states. He's also watching some DVDs and enjoying looking out the window too. Even Marshall and I are getting along and not fighting over each other's driving techniques . It couldn't be going better.

We're at Duke right now, half way through Jackson's 3 year post transplant studies (doing them a little early this year). So far everything is stable, no evidence of disease progression. Hooray! We have his visit with Dr. Escolar (the developmental pediatrician and her team of specialists) on Monday, and that will be our last appointment. Jackson has visited the nurses on 5200 and seen lots of old friends (most of whom he doesn't remember, but they remember him!). It has been nice to be back and see some familiar faces.

Our next stop is New York for the Hunter's Hope Krabbe Symposium. I'm really looking forward to seeing some of the friends we made last year and meeting some new friends. It's a very emotionally difficult event, but it's also filled with lots of love and laughter. My sister Juli will be meeting us there so it will be nice to have her along.

I added some new pictures to the photo album a few days ago, so check them out if you haven't yet.

Peace and blessings,
D'Anne

Nwe pictures added to the photo album - sorry it took me so long! Everything is going great... I'll try to update again soon...

Introducing Alexa Lillian and Maya Katherine!!

They were born on Friday March 23rd. Alexa weighed 5 pounds 13 ounces and Maya weighed 5 pounds 3 ounces. They're doing great for being 34 week preemies and were going to come home with mama today, but will have to stay another day or two in the ICN because they both looked a little jaundiced this morning.

Jackson LOVES his baby sisters already - it's so amazing to see how he responds to them. He was only allowed to see them briefly on Friday and visit for the first time today. More pictures to follow! We are so blessed!

Just wanted to make a quick post to let y'all know I added a whole bunch of new pictures to the photo album area of the website - 4 pages worth! I realized that I hadn't added any new pictures since Halloween... oops! There are some from the hospital, and even back through Christmas.

Things are going well here. We are slowly increasing Jackson's feeds and he's tolerating it well. No more pain, no fevers, so hopefully we're past the bad stuff. Thankfully Jackson really didn't lose much strength or abilities due to being in the hospital for so long, and he's back to working hard with his therapists. His occupational therapist from Early Intervention (before he turned 3) is working with him again, and seeing them together really shows me how much he has improved over the past year. He's really gained functional movement in his hands and arms, his response time in those muscle groups has improved, and he's more motivated and involved in the play and interaction. It's exciting to see the difference in him.

So check out the pictures, and enjoy! I'll try to update again soon.

Yes, we're finally home! After 4 weeks at the hospital, we were discharged yesterday. Jackson is still on 14 hours of TPN (IV nutrition) and his feeds are only running at 10cc/hr, but we're HOME! We're going to slowly increase his feeds and decrease his TPN over the next few weeks.

We have only Marshall to thank in expediting our discharge. The team here had no real exit plan in place, and seemed to be just spinning their wheels by keeping us inpatient. We never did find out what is causing Jackson's ulcers, but he hasn't had a fever in over a week, he's tolerating the slow rate of feeds, and his bowel habits are more back to normal (sorry to talk about that online, Jackson!) so Marshall began an aggressive push to get us out of the hospital. While we were there both Jackson and I came down with a cold (kind of scary, considering we were supposed to be safe inside the immune-compromised unit), and we didn't feel that it was doing him any good to stay there. At least we can control the environment here at home.

The hospitalization overall was a pretty frustrating experience, fraught with many errors on the part of the staff and difficult situations. I'm just glad it wasn't something more serious going on with Jackson.

We will be taking him in to clinic next Thursday for bloodwork and to be seen by both BMT and GI.

Thanks again for keeping us in your thoughts during Jackson's hospitalization. We're not over this little bump in the road but we're on a good track. He's happy to be back home too, and doesn't appear to be weakened by his month-long stay in the hospital. We're anxious to get his therapy schedule back on track next week so that he doesn't go without it for too long.

I'll update again when I can. For now I'm going to hang out with my boys - it feels great to be home as a family again .

Hi friends, we're still in the hospital . Jackson has spiked a few fevers since my last update, so that's been a major issue in keeping us here. We obviously aren't fixing what's wrong since his body is still fighting something with the fevers.

We do have a new plan as of yesterday. Jackson's main transplant doctor is the one covering inpatients this week, and he seems to be taking a more aggressive approach. Dr. W feels strongly that Jackson's issues are due to an infection rather than a random case of inflammatory bowel disease or Crohn's Disease (which would be pretty rare in a child Jackson's age). So the new plan is to figure out what the heck it is (hey, what a novel idea!). They've ruled out some of the more common sources of infection, so Dr W wants to investigate and look for the more obscure things that could be causing these problems.

Jackson had a CT scan of his chest, abdomen and pelvis yesterday to look for anything that may have escaped notice thus far - those results were clear with the exception of a swollen lymph node in his left armpit (which could have been caused by blood draws on that side or something equally benign - not a cause for concern). He had lots of blood drawn to look for antibodies for different bacteria, viruses, and fungi. He's been taken off his feeds again and will have complete gut rest for 5 to 7 days and new antibiotics with more complete coverage against anything that could attack the gut.

We like the new plan. My fear was that we were going to overlook some bizarre infection and it was going to take over - this has happened to more than one transplant child that I knew, and the outcome was not good . So much of a transplant family's fears revolve around what happened to other transplant kids we've gotten to know over time. We are torn between wanting the tests to find something so that we can treat it, and wanting them to find nothing wrong.

So the bad news is that we'll be here for at least 5 to 7 more days.

In good news, Jackson finally got a good night's sleep last night because he wasn't uncomfortable due to his feed running. Which means mama got a good night's sleep too .

Thanks for keeping Jackson in your thoughts.

Hi friends,

I'm so sorry that I let the wonderful holiday season go by without an update, only to post now that something is not going well. I don't like doing that, I don't want Jackson's journal to be a place where only bad news and prayer requests are posted... but there's not much I can do about that now. (We did have a lovely holiday season though!)

Jackson has been in the hospital for about a week now for some GI issues. He'd been having minor problems for some time, but last week he finally spiked a high fever that caught everyone's attention - and the team here at home finally decided they needed to investigate further (my requests for them to do that had fallen on deaf ears for months).

Testing showed that he has colitis, which is basically inflammation of a portion of his large intestine. He was taken off his feeds and put on IV fluids and meds in order to give his gut a rest. After 2 days of gut rest we slowly started pedialyte through his tube and then progressed to formula, but he didn't tolerate it well so we stopped and returned to IV fluids again for a few days. Subsequent trials of using the tube have not worked either. Tomorrow he is scheduled for an endoscopy to take a look and see if they can find out exactly what's causing his discomfort.

We had hoped that this would only take a few days inpatient, but here we are - it's been a week and we have no idea how long it will take for his gut to heal enough to allow us to restart his feeds. Hopefully not much longer, and we hope that the endoscopy will yield some helpful information.

Please send up a little prayer for Jackson's healing. I'll try to update again soon.

Wishing all of Jackson's Friends a wonderful Christmas and much happiness in the New Year. God bless!!

Hello friends,

Once again I apologize for the lack of updates. Rest assured, everything is fine here.

Jackson is doing well. He had another cold last week which landed us in the ER getting IV antibiotics, but he's recovering from the cold just fine and is feeling good. He's still as happy and joyful as ever . His recent enzyme levels have been stable - low, but stable. We are OK with that.

We are thinking about taking him out of school until the spring time, just to reduce the chances of him getting sick. Even though we knew we were taking the risk of him getting sick when we put him in school, the reality of fevers and ER visits is having an impact. We are still in the discussion phase of this decision, but it seems likely that we'll take him out of the public school setting until cold and flu season is over. He'll go back to having a teacher come in a few times a week to keep him stimulated. He'll miss seeing his friends in class, and that's a difficult thing for us to think about - school has been so good for him socially and developmentally. But his health is important too. We're weighing the options.

Getting ready for Christmas has been a real joy this year, as Jackson is able to understand what's going on and be a part of things. It's so much fun to see him enjoy taking part in decorating the tree and our new Advent calendar. Every day we feel so blessed that he is here to enjoy these moments with us.

Now in other big news, we have a family announcement to make...

In April, Jackson will be a big brother!! We are expecting TWIN GIRLS!!! Early testing has indicated that both babies are free from Krabbe Disease, and they look healthy as can be. We are thrilled . Jackson is starting to understand and likes to talk about his "baby sisters". I know he's going to be a wonderful big brother.

That's all the news from here. We wish everyone a wonderful holiday season. God bless!

Thanks to Ken Roberts for the "nudge" to post our Duke update!

Well, we had our 2 year post-transplant checkup at Duke and overall it went very well. With the exception of his donor cell count, all of Jackson's test results were either stable or improved. The best of all was that he has increased myelin in his frontal lobe!! He also has better nerve conduction. His EEG and brain wave tests were stable, as was his enzyme level. His donor cell count is down again - this time to 12% - but since the enzyme level is the same we are not panicking. It isn't a good sign, but nothing needs to be done about it right now.

While we were there Jackson developed a fever and required a few doses of IV antibiotics. It was interesting that this happened while we were there, since he hasn't had any fevers at all since we came home from Duke initially. If it had to happen, I was very happy that it happened while we were at Duke - at home, the bone marrow team would have admitted Jackson to the hospital for a fever, but at Duke they treat it as an outpatient and trust the parents to be alert to changes in their child's status. We like that procedure much better. Jackson is doing fine now, he started having some mild cold symptoms a day or two after the fever broke so we thing he was fighting a little virus. We kept him out of school last week to make sure he healed up well, and he will be going back tomorrow for Halloween festivities.

I was able to add a few more pictures to the photo album, including this gem:

Happy Halloween, everyone!

Sorry if I lead you to believe that I'd actually be updating once we got the journal back up and running . I promise I'll try harder.

Things are going well here - Jackson is really enjoying preschool, making new friends, and learning things. Marshall or I are still going with him for most of the day, but we feel comfortable leaving for a few hours and we'll slowly increase that until he's going on his own for the full time (he attends from 8:30am to 12 noon, 4 days/week). Although it's so much fun to watch him interact and participate, we might just continue to go with him for quite a while. I swear we'll have cut the cord before he gets to high school .

I have managed to spend some time repopulating the photo albium - take a peek! (I put them in chronological order so you may have to flip past ones you've already seen) The most recent photos are from the "special kids night" at the local Walnut Festival - the Festival committee opens the carnival a night early for families of children with special needs to come and enjoy, and it was so neat. They had all of the kiddie rides running, free pizza and drinks, tee shirts and a stuffed animal for Jackson. I've been going to the Walnut Festival since I was a child myself and I had no idea they did this special event - it really was a wonderful evening. We even saw some of Jackson's friends from school and got to hang out with their families. The only camera we had was our phones, so sorry the pictures aren't great!

I also added some photos from the Krabbe Symposium we attended in New York this past June. Oh my goodness, what an amazing experience that was. I'm not even sure I can put into words how meaningful it was to meet other families who are going through the same struggles we are, who understand this disease and all that goes along with it... to not have to explain ANYTHING. I have never felt so "at home" with total strangers before. Here is an entry in my personal journal from that trip...

"June 27, 2006

The medical presenters talked about some very exciting things on the horizon, which I'm sure you'll hear me post about in the future. Newborn screening for Krabbe is very close to becoming a reality (at least in New York, and other states to follow), so soon we will be able to stop this disease before it steals any more of our children. I am so anxious for that day."

This year's "family photo":

And some excellent news - since that post, New York has officially started screening for Krabbe Disease as a part of their newborn panel! This means that every child born with Krabbe Disease in New York state will now have a chance at life. Legislation is currently underway to bring all states up to the standard that New York has set - I will have updates on this as it gets closer to vote.

Getting back to current events, we're preparing for our return to Duke for Jackson's 2 year post-transplant studies. We'll be getting another enzyme level and donor cell count while we're there - please say a prayer that the numbers are higher or at least stable.

Thanks so much for continuing to care about Jackson and to pray for him.

Peace and blessings,
D'Anne

Welcome to Jackson's journal! We're just in the testing phase right now, so please bear with us and check back soon.

Peace and blessings,
D'Anne