Davyn M.
Diagnosis: Histiocytosis
During one of Davyn’s routine MRI’s it was found that he had a Chiari type 1 malformation and a Syrinx. Not really knowing what this was we didn’t think very much about it, but our Oncologist suggested we speak with his neurosurgeon about it. At the neurosurgeon we got some pretty devastating news. First when we walked in he was kind of confused as to why we were there. We told him it was to discuss the chiari malformation and syrinx, he didn’t know anything about it. Well apparently in between our hospital visits to Vanderbilt and ETCH and our switching from neurology to oncology information got lost. Basically, Davyn should have seen this Dr. sooner and Davyn’s condition should have been monitored and we didn’t know. I was under the assumption that Davyn had been born with the chiari, which had in turn caused the syrinx and, although somewhat serious, it wasn’t that big of a deal and we should just bring in a Dr. and have it monitored. I figured Davyn wasn’t gonna be a big football star, but that was the extent of it. Well I was wrong, terribly wrong. Apparently, at the time of Davyn’s brain biopsy the chiari malformation and syrinx were not there, which means somewhere between 08/2008 and now this developed and it developed because of the lesions and the pressure it was putting on Davyn’s brain. We know this pressure caused Davyn’s brain to shift and somehow caused the chiari malformation, what we don’t know is what caused the syrinx. Sometimes the syrinx can be caused by the chiari malformation, sometimes it can be caused by brain tumors/ trama/ swelling (lesions) and sometimes it can be another disorder altogether. The problem with not having Davyn followed by the neuro is we don’t know what the source of all this was.
The bigger problem here is the syrinx. Apparently, Davyn’s is of substantial size (not small and not horribly big, but of substantial size). It crosses two vertebrae (T5 and T6) length wise (which is really not the problem), the problem is the width of it (which we weren’t really given exact measurements) but it is substantial. A syrinx is a fluid filled cyst or cavity that forms in the spinal cord, it can cause damage to the spinal cord over time or, like a balloon, with a certain amount of pressure either from the brain or by injury can burst and cause total paralysis. At this point Davyn is very fragile. He cannot, absolutely cannot have an injury to that area of the spine or he could be paralyzed. Which means not only is he not gonna be a big football star, but he cannot be in any contact sports, no bike riding, trampoline, playground equipment, nothing to where he can fall or be hit, no recess and no PE, no scooter…..nothing.
Another complication to this is the lesions, they absolutely cannot grow. His spinal cord cannot take any more pressure. We really didn’t realize the seriousness of this and have been very blessed with Davyn b/c he is our little superhero and, although careful with him, we let him do whatever he felt like he could do, not knowing what was at risk. God’s hand has been on him. There are two surgeries that most likely down the line he will have. The problem is we waited so long to bring in the Dr. and we don’t know the source of the problem so we don’t know which surgery to do first or if at all. If the chiari is causing the syrinx (while there is no cure for chiari) they can perform a brain surgery to open up that spinal cord to relieve some pressure on the spinal cord, if the chiari is not the culprit they can go in and put a shunt in the spinal cord and drain the fluid. Both are risky procedures, with no guaranteed outcomes. So the plan for now is to wait six months and see what happens and monitor very closely. The neurosurgeon needs to know what is going on with the lesions first to know what is going on with the syrinx.
Right now we just have to put some safety measures into place to keep Davyn safe and life is gonna change a lot. Even with the surgeries he is gonna be fragile and we will have to protect the back of his head and his spinal cord the rest of his life. We had started to think that somehow we were at the end of this thing, but realized today we were somewhere in the middle of it still and it was a hard realization to come to. We also realized it was pretty important that these lesions not grow, but we didn’t realize they absolutely can’t. The problem with the lesions in Davyn’s brain is he is only showing symptoms from one of them (the brainstem), it is devastating to think this but if one of the other begins to grow (and we don’t know) and puts just enough pressure on his brain his spinal cord could shatter or if he falls in just the right spot his spinal cord could shatter. Thankfully Davyn’s last MRI showed no growth. We are going to repeat the MRI in late July or early August.
We also met with his psychologist to go over Davyn’s complete medical history and about 8 hrs worth of testing that he has had done over the last month. It was A LOT of information to go over to say the least. We went in with a lot of questions, a little hesitation and a hope for answers. We came out with more than we had hoped for. As we knew Davyn does have some residual brain damage related to the lesions. He also has a very higher than normal IQ. His therapist likened it to the horse power of a car, Davyn’s IQ is the horse power of let’s say a corvette. Even with the fastest horse power a car cannot run at it’s capacity with an oil leak or a flat tire or some other problem. That analogy really helped put what we are dealing with in perspective. We knew Davyn was very bright, what we didn’t know was the specific areas he struggles with and how to help him. Apparently, he struggles highly with verbal memory. He has almost no ability to remember things he has heard or recall things he has heard without having them written down or repeated constantly. We knew he had an amazing memory when it came to spelling and charting and we also knew we lost him during story time or movies, when it was something he was unfamiliar with. We thought it was just disinterest or an attention problem. Apparently, it is very normal for the lesion on his brain stem and cerebellum to cause problems in memory.
The thing that makes Davyn unique is that he is actually aware of it. During testing he did become oppositional, but he also stated he could not remember it unless it was written down. For a five year old to know the way he remembers things is highly unique. We also learned that because of the damage that has been done he has a lot of traits that resemble Aspergers syndrome and he does also suffer from some OCD traits. His awareness of his deficit is causing some anxiety which will then manifest itself into OCD traits, which explains why we see them coming and going and not always being there. So with all that said, yes it is hard to come to the realization that Davyn will have some residual damage from all this, but we now have the beginnings of a plan. A plan that will be specifically tailored to Davyn and will work with his strengths to help him maximize his horse power. We also now have a liaison who is going to help us coordinate with the schools, therapist, doctors etc. Right now specific protocols will be put into place for Davyn regarding what he will require from a school to help him learn and also to keep him safe. She will then contact the school, we will begin an IEP plan and come up with an environment that is conducive to Davyn. He will definitely require occupational therapy and could benefit from some type of social therapy also. I still do not know if he will be in public school, home schooled or if we will do a co-op. It will really depend on if the school can provide 100% of what Davyn needs.
Other than that our summer is in full swing and we are just focusing on enjoying every day God gives us. Thank you so much for your cards, letters and support. When the bad news comes in it is easier to receive knowing we have the love and support of so many. Please continue to keep Davyn in your prayers…pray for no growth of the lesions, continued healing, and answers as to how best to deal with Davyn’s secondary medical conditions and for continued strength as we go through this journey.
Stephanie (Davyn’s mom)
