Dr. Maria Escolar
Krabbe Disease … we wish more than anything you didn’t exist. These past few weeks have been very tough on Kyle and Jenna, but especially on sweet Bryce. It’s become very clear that the disease is progressing, though how much exactly we don’t know.
Once they returned from Pittsburgh in January, Bryce plateaued once they got his medications in place, which is what the team out there predicted would happen. His movement has been limited since then: He really only moves his arms and legs when he’s experiencing a nerve or muscle spasm: It’s like he’s paralyzed from the neck down.
Since about the first week of March, though, he has been a bit more irritable and has started vomiting after many of his meals. They’ve speculated that it could be because they’re feeding him too much or too fast; that it could be about how he’s positioned and how soon they lay him down after he eats, etc. But there isn’t much consistency to when it happens. That’s what makes them think the disease progressing.
With this in mind, they are anxious to move forward with the Nissen Fundoplication procedure, which will prevent reflux and aspiration: Because Bryce can no longer swallow on his own, there is a greater chance of this happening. Also, due to Bryce’s floppy epiglottis, there’s a greater chance of him getting pneumonia as a result of the vomiting: Because his epiglottis doesn’t seal, every time he gets sick, the bacteria builds up on the epiglottis and could cause infection in his lungs. They are meeting with a doctor at the UW-Children’s Hospital on Monday and hope to move forward with the procedure within a week or so.
Bryce’s vision has also been affected, something that Kyle and Jenna began to notice a couple of weeks ago. He is no longer tracking people across the room or focusing. It’s almost as if his vision is delayed – like it takes a while for things to become visible to him. This change is devastating …
They’re not sure if it’s because of the medication or just the disease, but Bryce is sleeping a lot more now. His digestion is also slow, a result of his lack of movement; they are giving him a supplement with two of his feedings to help him have more regular bowel movements.
In spite of all these things, Kyle and Jenna have been experiencing life with Bryce: They spent a week in Mosinee with the Heckendorf family; they spent the weekend here in Minnesota and went to the Sea Life Aquarium at the Mall of America; yesterday, he laid in his first snow pile 🙂 These among many other things …
We ask for your continued prayers for Bryce, Jenna, and Kyle. Your love, strength, and positivity are what is carrying them, and all of us, through this … Thank you!
It’s hard to believe it’s been more than two months since we learned of Bryce’s diagnosis already. Sometimes I wonder how we’ve gotten through that many days — and if I feel that way, I can’t imagine how Jenna and Kyle are doing it. It’s a reminder that life continues to go on regardless …
Since my last update here, Kyle and Jenna have started the process of meeting with doctors to discuss the procedures they’re considering for Bryce. They won’t do anything until March at the earliest, simply because they can’t replace his G-tube button until then (it needs three months to heal properly), and they want to do all the surgeries at once so he doesn’t have to go through all of that and be in and out of the hospital multiple times. Whether they decide to do all of the procedures or not will likely depend on how much the disease progresses between now and then.
The good news is that Bryce’s meds are working, and he’s quite a bit calmer than he was a month ago. They’re actually getting more coos out of him now than then — and a few more smiles, too! It’s SO good for his Mommy and Daddy to see that beautiful smile and his eyes light up in the midst of all of this. They’ve also been able to lay him down for naps more frequently, though they don’t mind holding him as much as they can (per Dr. Escolar, a lot of children with Krabbe go through a phase where they don’t want to be held). And a few more of us are getting to hold him as well.
Last weekend was the #BStrong for Bryce Ice Fishery in Bagley, and despite the frigid temps and wind, so many people showed up and gave their support. Jenna and Kyle asked me to share this message, which they originally posted to Facebook, for all of you who may not be over on that platform:
Kyle and I would like to thank everyone who came out to support Bryce and our family yesterday. We are so grateful for everything everyone is doing to support us! We would especially like to thank Mike and Frankie for organizing the event, as well as everyone who volunteered their time and/or made donations to make it happen. We feel so blessed to have such an amazing support system and we would not make it through each day without all of you! We ask that you continue to keep Bryce in your thoughts and prayers as he continues to fight this fight! Thank you all!
Please remember to mark your calendars for one month from tomorrow, for the Sky Is Blue Benefit for Bryce on March 1. In the meantime, please keep your thoughts, prayers, and positivity coming for our sweet Bryce.
Kyle, Jenna, and Bryce have been home from Pittsburgh for a week now — what a relief to have that trip over. They spent it at home in Dodgeville with help from Grandma Sue (through Monday morning) and then Grandma Wendy from Monday late afternoon through Thursday midday. Thank goodness for our amazing mothers is all I can say.
They have been adjusting Bryce’s medications since returning, and he has seemed more content the last few days. They were even able to put him in his infant swing for five minutes two times the other day. He also sat on Papa Tom’s lap on Friday for a few minutes, something he hasn’t done since early December.
While Kyle and Jenna expected test results earlier in the week, they learned on Tuesday that they’d be able to talk with Dr. Escolar on Thursday. I’m going to do my best to recap what Jenna relayed to me about all of Bryce’s tests:
- Vision Test: Bryce’s eyesight is still good, and according to the Visual Evoked Potentials test, his brain is correctly interpreting what he’s seeing, which is great news.
- Hearing Test: His hearing is still good, though there is a slight delay in how Bryce is processing what he’s hearing. Dr. Escolar recommended that they just speak in shorter sentences to him and more slowly so he can process their words better.
- Nerve Conduction Test: When a healthy child does this test, their nerve response is typically 100% — meaning they respond immediately. Bryce’s response is about 40%, which Jenna said she felt was a little generous as he is moving his arms and legs very little on his own at this point. Dr. Escolar explained that part of his lack of movement is due to the muscle relaxant medication that he’s on: The medicine relaxes his muscles to a point where even if he wanted to move, it would take him a lot more effort to overcome the effects of it. Why not reduce the amount of medicine, you might be wondering? Without it, Bryce is in pain. It’s a double-edged sword.
- Brain and Lumbar MRI: This test showed that the Krabbe has progressed, meaning that some of the white matter has been effected, and as a result there’s been some slowing of the nerves, but it’s mild at this point.
Dr. Escolar explained that there are four stages to this disease and that Bryce is in stage 2 (there’s often overlap between the stages so how some children progress is different than others):
- Stage 1: Feeding problems; slowing of development/regression
- Stage 2: Tightness and stiffness; extreme irritability; digestive system begins processing more slowly
- Stage 3: Vision begins to go
- Stage 4: Vision and hearing are typically gone; seizures; trouble controlling body temperature
Krabbe is a disease of the brain and peripheral nervous system, and Dr. Escolar was also able to help Kyle and Jenna (and all of us) better understand why certain things are happening with Bryce’s little body. He is constantly clenching his fists, for instance — to the point where he cries when you try to open his hands. She says this is a sign of a “disease of the brain.” His sensitivity to touch and need to be held are signs of a disease of the peripheral nervous system.
Other things that Dr. Escolar told them:
- Most families see the most changes within the first six months of the disease and then it begins to plateau.
- Over the next two months, she expects Bryce to become even tighter and weaker (she wants them working with a physical therapist regularly with the goal of keeping his joints loose and to work on range of motion).
The most important message that she shared with Kyle and Jenna (at least from my perspective), is that with proper care and interventions, and if he stays healthy besides the disease, Bryce could live two to three years.