He feels better. Can you see it? Can he feel better than this? Maybe. Will I continue to ask? Absolutely.
Will is showing us that we are right to advocate for him. We are right to ask for the best care available. It’s up to us to insist that the team doesn’t forget that there is a little boy in Maine who relies on them to feel his best.
Do you advocate for yourself the way I advocate for Will? When’s the last time you asked for what you need: Personally, professionally, spiritually?
I posted an update of sorts, a short blurb about the past three months, but in the background, we have been busy coordinating evaluations for therapies and attempting to obtain important equipment for Will.
When we were handed Will’s terminal illness diagnosis, we were devastated. Throughout his short life, we have wrestled with having to fight for services and equipment we need to care for him. For example, he is too big to fit in a baby bath seat, the type you would buy at Target. He can’t sit on his own, we hold him in the shower to bathe him. Our Early Intervention program (CDS) in Maine has given us a bath seat to borrow until we get one of our own. In November we submitted a request for a bath seat to insurance; we still do not have it (March 2019). CDS has given us other equipment to try so we can start to figure out how to get Will in a standing position. The primary service provider, the Physical Therapist, has been amazing and loves our family very much. She has sat with me as I cried with frustration/worry/grief…all of it. She’s been here since the beginning and I adore her. The rest of it has been hard to navigate.
More fight? Don’t give up. He’s worth it. iWill.
Will was seen by his Maine neurologist in December for a regular follow-up visit; he did not realize that we weren’t plugged into speech or communication therapy. He ordered an evaluation to be done by a local expert who sees children needing augmentative communication. This evaluation would be the beginning of a valuable roadmap that helps navigate the functional world of communication for Will. He is nonverbal. He is expressive with his body, mainly his eyes, and he communicates in his own way. The specialist asked us to forward the referral to the Early Intervention team (CDS) because they will be following Will into the preschool setting (ages 3-5) where his direct therapies will take place. To our surprise, the CDS team denied our request. They said, “Will does not have the building blocks of communication appropriate for this type of evaluation.” This is not true. They insisted we have a meeting to discuss their position on the matter. They disagreed with Will’s doctor and dug their heels in. “Not appropriate,” they said. I rebutted, “if Will is not likely to sit, crawl, walk or feed himself, does he not need PT and OT either?” If we are talking about the building blocks of future function, let’s have that discussion too.
We fought with CDS for the next six weeks over this and decided to schedule Will’s evaluation and pray that insurance covers it. Fast forward several weeks, we attended this evaluation: Will surprised us all. Not only did he catch on to the games he was part of, but he followed along. We were embarrassed that we didn’t pursue this earlier. We learned so much about “being Will’s voice.” Our second visit is this week; there is so much more to learn about communicating with a nonverbal child. We’re ready! This lifestyle is exhausting, but when you’re fighting for your kid, it’s worth the effort. I know government run programs are not perfect. However, the CDS Early Intervention program in Maine should do a better job managing complex needs children. Better yet, let another program take on those complex children. Maine, you can do better.
Unexpected Gifts: Our next visit to CHOP is scheduled for the first week of April. There were rooms available for one of two nights needed for the appointment. The harder news was that the room rates were over $300 per night in the usual locations. We reached out to the social work department that assists families visiting the neurology team at CHOP. With their help we applied for the Kolbe Fund, and they approved our request. They gifted us our stay through their “Hopeful Nights” program. We now have a reservation in Philadelphia for our appointment in April. We are so grateful. All they ask is that we pay it forward by donating to the next family who needs help. More about this fund here: www.thekolbefund.org
“In 2011, our son, Max, received a life-altering diagnosis. With support from family, friends, and strangers, Max had the best medical attention and is now a very healthy boy. In awe of the love and help we received from others, we founded The Kolbe Fund to help other families who must travel for care.” – Kate and Aaron Schnittman, founders of The Kolbe Fund
We traveled to CHOP in December for Will’s follow-up appointment with the research team. We reported that he had been experiencing a long stint of interrupted sleep and more irritability over the past two months. The team decided to change the dosing of his Baricitinib; they thought he was experiencing breakthrough symptoms. We traveled home to Maine and started our new plan. Over the past two months Will has been happier, more vocal (laughing and squealing), and tolerating being in his stander better. He has also put on more weight and is thriving. Amazing what happens when you’re feeling better! CHOP recommended that we have Will’s lab work checked because he is anemic again. A side effect of Baricitinib is anemia. Since Will has an affinity for red meat, we have been serving him plenty of that. We will recheck his levels in the next week.
Our team at CHOP also recommended that we add a pulmonologist to Will’s team. We found a pediatric pulmonologist in Maine that specializes in neuromuscular diseases. We met with this doctor about two weeks ago and are thrilled to have him on our team. Not only is he trained to manage a kiddo like Will but he has trained at Children’s Hospitals where he had a plethora of experience. We are in really good hands! We are headed back to CHOP the first week of April, and while we thought we had avoided a crazy week of events in Philadelphia, there must be something else going on because we are having trouble finding a HOTEL ROOM! Wish us luck!
We are headed to CHOP for the 6-month follow-up visit with the research team in January. It has been six months since we started Baricitinib and we will have PT, OT, and neuropsych tests done to measure changes in Will since starting the medication. Will has made slight progress in his movements, but mostly we notice that he is happier. He still cannot sit, roll over, or accurately reach for toys consistently. He is non-verbal and cannot communicate his needs with us.
We have ramped up the physical therapy sessions and asking for two sessions/week whenever we can get on their schedule. We had a follow-up visit with our neurologist in Maine, and he didn’t realize that we were not doing speech therapy yet. We have tried to find a speech therapist but haven’t been unable to find a practice in Maine that can treat Will. We have heard, ‘we don’t have what we need at this practice to provide therapy.’ Early Intervention therapy (for children under 3 years) in some states provide therapies in the home, but Maine does not. Maine’s Early Intervention program has adopted a “coaching model” but does not provide direct care to children…this has been a huge source of frustration for myself and many other families.
Our neurologist called a practice in Portland that provides augmentative speech therapy evaluations for children and asked the provider to see Will. Typically children 3 years and older start this type of speech (assisted and augmentative) therapy in a developmental preschool setting. In the meantime, Will needs therapy to work on the building blocks of communication. It has been 21 months since we started Early Intervention here in Maine and we are finally getting plugged into a practice that can appropriately evaluate Will’s communication needs. It would be ideal for Will to get some of his intensive therapies at the same place. Ideally, I’d like to find two therapies in the same building (OT and Speech). We may have to drive 45 minutes for combined therapies, I’m waiting to learn more about the practices in Southern Maine.
Will enjoys when we take his hands and explore with him. At a restaurant, he likes when I grab a crayon and color while bringing his hands along for the activity. He cannot grasp an object by himself anymore; the neurological insult robbed motor planning from him. He likes when we fill the dump truck with colorful items and then dump them on the floor. He also likes it when I put a snack in his hand and help him reach his mouth. The delight he exudes when he takes a bite catches us both by surprise. For Christmas, Will is going to get fat crayons and a sketchbook!
He is thriving!
Will is close to losing his Failure to Thrive diagnosis. He lost weight during his 10 months of not feeling well, battling neurological flare-ups, etc. I kept saying, ‘when he feels better, he will eat.’ Well, we are making progress because he feels better. Someone said, ‘what are you going to do if someone reports you to DHHS because you won’t allow them to surgically insert a feeding tube?’ Well, here we are: gaining and thriving!
We haven’t done any traveling other than hospital visits over the past year. I miss my family; it isn’t like me to go long periods without a trip to Pennsylvania. Airline ticket prices are obscene, and it’s prohibitive to fly the kids to see my parents. Worse, is worrying that they have overbooked a flight leaving a family like ours scrambling at an airport. Hopefully, we can make a trip happen next summer, even if we have to drive. Speaking of driving, Will allows me to drive him about 25 minutes without crying now. I can go to the grocery store, drive to Falmouth for appointments, and even pick up the kids at school.
Many of our friends helped during the time when I was mostly homebound with Will. Our friends would bake us treats, bring lasagna and mac n’ cheese, and Will’s favorite food with crackers: guacamole! Our village never ceases to amaze me. Even our mailman goes out of his way to help make life a little easier for us, he always asks ‘How’s Will?’ Will has shown me the value of slowing down and checking in with people, being more present.
Our extended family: Scott, RN
We have been blessed with the most amazing registered nurse. When I work, Will is cared for by Scott. Scott has been a nurse for 33 years. He has been a NICU nurse, a Nurse Director, Associate Director of Pediatric Surgery and Anesthesia, Divisional Director of Nursing Services…worn so many hats. He also works per diem at the state prison in Maine. This guy is a sweet as he is intelligent and after a breaking in period, Will gave into the idea of being cared for by someone other than Justin and I. They have become great buddies. It’s such a relief to know that Will is in good hands and happy while I’m gone for my shifts.
Thank you for following along. We are grateful for the friends and family who embrace our journey with us and continually add support along the way.
Is there a better time of year than fall? Some would say the first snowfall is their favorite; and while there is something romantic about the first snowfall, it just isn’t as renewing as a bright, brisk fall day. I love every season, but fall is my favorite. I love watching the first sign of flurries but am always sad to see the beautiful leaves hidden by a blanket of snow.
After a hot summer, I welcome the relief of humidity and find comfort again during outdoor runs that seemed harder just weeks prior. For me, there is that urge to train more often because snow impedes outdoor running and cycling.
As a mom and a nurse, fall reminds me of the illnesses associated with the upcoming months. RSV, parainfluenza, influenza, croup…it’s coming. I’m starting to see youngsters affected by respiratory illnesses at work. It’s a reminder that we all need to do a better job at preventing the spread of respiratory illnesses by washing hands, covering coughs, staying home when feeling ill, and vaccinating if possible.
Here’s the thing: For our most vulnerable population, less kisses! No kisses to the hands, or face during flu season. Doesn’t that make sense?! It’s so hard to old back a kiss when you’re grandma or grandpa or adoring Aunties. Everyone loves to kiss babies and toddlers. It’s a trap! Keep your precious loved ones safe and kiss them in the spring.
Remember, you’re contagious and spreading some viruses before you realize you have one!
It has been ten weeks since we started Baricitinib and we had another follow-up appointment at CHOP this week. We started the day with lab work, then met with the neurologist to review his labs and discuss how Will has been doing since our last visit.
Since Will started Baricitinib we have noticed the following changes:
*He can hold his head up longer than he could before starting the drug.
*His trunk strength has improved, and he can maintain an upright posture when straddling our legs for longer periods of time.
*He has become more vocal: think tiny dinosaur…he is not babbling yet, maybe someday. The neurologist says that vocalizing is the precursor to babbling. We all wonder what his voice would sound like.
*He is easier to console, less irritable, and generally happier.
*We think he is bringing his hands to his mouth more often, which has been tough because he bites himself.
*He is chewing and swallowing better than he was, with a noticeable decrease in gagging on fluids and medicines.
*He is demonstrating reciprocal movements in his legs when assisted to standing and also on his hands and knees. The neurologists and research team is so excited to see this too.
Our team at CHOP recommended custom orthoses and a TLSO braces to use for therapy sessions. In addition to this, we have tried some medications to address the dystonia in Will’s arms and legs. Botox was given on two different occasions but did not seem to make a difference in Will’s tone. We moved onto baclofen, an oral medication but he did not tolerate it at all. He lost his spark and wasn’t eating meals because he couldn’t hold his head up. We have moved onto gabapentin. Gabapentin, like baclofen, has been a drug we are titrating to get to the desired dose. So far, the gabapentin has not made any difference in Will’s tone.
Sleeping (or lack thereof):
Will hasn’t slept through the night in about six weeks. We are a mess, exhausted. No sleep, no energy for exercise, less patience, etc. It’s ugly. We aren’t sure why he isn’t sleeping, but I know that swimming made a huge difference in Will’s sleep cycles. Six weeks ago when we were regularly crashing our friend’s pool, he was sleeping through the night. Could that be all it is, does he just need to move his body? Good grief! Our deck is near completion, and the hot tub is going to be placed in the next week or two. We need to have an electrician do his thing so we can be up and running.
We couldn’t afford to add the pool this summer and have big dreams about what that might be like for Will and the other kids, but it’s expensive and may take another year or more to save. Glamour Pools has an above ground pool with a sunbathers bench option that would be great for Will’s physical disabilities. To sit him in a chair in a shallow area before moving to the deeper water would be more advantageous. We have the sweetest friends’ who give us the open invitation to their beautiful pools; we are very fortunate to have such amazing support.
Many of our friends know that we lost our beloved dog Baxter to lymphoma. A huge presence is lost in our home. Every day one of my children say, ‘I miss Baxter.’ Hunter sleeps with the blanket we covered him with while he was sick. My children will never forget the love they have for Baxter. I miss walking into the kitchen and seeing him sprawled out on the floor, watching me with his big brown eyes. I also miss seeing Mia on the floor with him. She always took Will to him and played. Will loved his long-haired friend.
Daddy works during our trips to Philadelphia. He isn’t used to being away from his construction work as often as we have been, the stress wears on him but he tries to juggle it all as best as he can. Will offers a smile as we wait for dad to finish his phone call.
Our next trip to CHOP is January 8th. We will be there for two days and then fly home. At this visit, we will have labs drawn, see the doctor, and then have research visits with PT, OT, and neurobehavioral psychologists to track Will’s progress on the medication.
We appreciate the GoFundMe gifts, cards, help with the pets and kids so much. PALS pilots fly us to Philadelphia and that makes such a difference for us; we couldn’t afford to go to PA as often as we do without them. Not only does PALS give us the gift of transportation, but it helps Will stay healthy. The germs Will would encounter by flying commercially would undoubtedly impact his health. The monies received from our fundraising helped us with the hotel fees, meals, and Uber rides while we were in Philadelphia. We used all of the donated money for the trip in July, August, and this week. That’s a lot of money in a short amount of time. Having a child with special medical needs is very costly. I just remembered a nice gesture: the night we arrived in Philadelphia we went to the restaurant in our hotel. Our waiter asked what brought us to the city. When we said we were visiting for a medical appointment at CHOP, he apologized and wished us well. At the end of our meal, he brought us a paper bag and said he wanted to send us on our way with some fresh baked cookies. Things like that make me sad and happy all at the same time. Justin ate all the cookies (ok, maybe all but a bite). I ate the lonely chocolate cupcake.
“When I die, I want to know that I helped all the people I could with the time that I had.” J.P.
Arguably the most beautiful person I have met during my life.
We have seen Will move his legs forward, at times bending his knees at the proper time in the gait sequence. This is progress! He wasn’t able to do this until recently. If you look at the video closely, you can see he needs to be held up and also needs supportive ankle orthotics.
We are headed back to Philadelphia next week and I have asked to meet with our beloved physical therapist. We will have his legs casted and orthotics will be made for him. Also, I’d like to pick her brain about assistive devices that we can use at home to challenge Will (gait trainers or gait trainer/standers). He has shown us some new tricks, let’s see what we can do with some more practice. We are upping the ante on PT…if we need to go to Philadelphia to get what we need to help Will, we will. I’m not taking ‘you won’t be able to get that here’ for an answer again.
Click on the underlined links to see some videos of Will.
4 weeks of Baricitinib and what did we discover last evening? A walking William!!👣
This means we have a lot more work to do, but more importantly, we have progress. I have only seen Will walk in my dreams…it was once and it was perfect. I didn’t think I’d see the day he’d take steps on his own (or even with support). 👣
We are headed back to Philadelphia next week and will get some help with orthotics to support Will’s ankles. In the meantime, more swimming, therapies, and practice. I’m also interested in getting Will a gait trainer. This equipment will support Will’s body while we practice ‘walking.’ I’m going to find out what will be best for Will and ask the therapists to write the letter to the insurance company. We’re ready for more walking! 👣
We hit the ground running after being in Philadelphia for ten days, but wanted to write a quick update about Will. We have had a few questions about the drug, so I thought I’d include some information in this update.
Will takes Baricitinb twice a day. It’s a pill that partially dissolves in water; it doesn’t dissolve but breaks down into little particulates. We mix the particulates with other medicines Will takes and orally administer by syringe; he isn’t bothered by the taste or texture when we administer the medication in this way.
Within a couple of days, we noticed that Will had little ‘bursts’ of energy shortly after his afternoon dose; he would become more wiggly and vocal. So cool! He has been more engaging, less irritable, and his appetite is much better. To our surprise, he took some sips from my straw the other day, something he hasn’t been able to do until now. We have been offering him water in a sippy cup throughout the day for the past year but are routinely offering him a cup of milk or water because he enjoys the challenge of drinking from a cup so much. Small steps in the right direction.
We will be going back to CHOP 8/21-8/23 for lab work and a clinic visit. More updates to come!
We headed to CHOP this morning to visit a physical therapist and try out a beanbag chair. She had shown us some things to try with Will, so we asked her to demonstrate for us.
While there we met with one of the techs who casts kids for custom orthotics. We are waiting to hear if insurance will cooperate with them and if so, we will have some important pieces of equipment made for William. He will have some splints for nighttime, and a supportive vest (dragonfly TLSO) to wear during therapies and feeding. We have been hoping to schedule this visit for a while and it seems like it might come together for us. She told us to keep Will swimming and add equine therapy whenever we can.
Tomorrow will be one of our more difficult days. Our day starts at 6:50am. An IV will be placed and Will’s blood will be drawn over the course of the day to see how he is metabolizing the drug. The samples are sent to an outside lab and then once analyzed, we will adjust the medication accordingly.
We haven’t noticed any changes in Will since starting Baricitinib. The goal of the medication is to slow the progression of the illness and allow his brain to further develop. The interferon that Will has in his brain creates a hostile environment and makes him feel lousy. Click the underlined word above to read about JAK1/2 inhibition with baricitinib in the treatment of autoinflammatory interferonopathies. Our best description of what Will has is an ‘interferonopathy.’