A New Diagnosis... Rolling with the punches

For quite some time we had been concerned with Bryce’s tone. The scissoring of his legs was the worst we had seen it and his tone had been extremely high. Back when we went through SDR, the operation was to address the spasticity in his legs. The operation did eliminate the spasticity and he was able to have full range of motion in his ankles. However as he recovered and grew stronger it became very apparent that his tone was still a big part of the problem. SDR doesn’t solve for the tone, but other operations could. We have had several discussions on surgeries to address and hopefully manage his tone. Recently we opted to go ahead and begin Botox injections again so that Bryce could get more immediate relief while we discuss the next steps.

In late January we had met with his CP doctor for the injections and to discuss what those next steps should be. They sent in a Child Life Specialist to entertain Bryce during the appointment and to also help keep him as calm as possible during the injections. As they usually do they asked what Bryce loves so they could bring in the IPad and show him his favorite cartoon or play a game. We told her his love of sports and that he would be most happy with that. This decision ended up being key for that appointment. Bryce gets very excited when he watches any games so this gave the doctors an opportunity to see Bryce when he was excited (since most times he is NOT very happy to be there). When they witnessed Bryce and how his body responded when he got excited the atmosphere in that room changed. It became very obvious to them that this was dystonia.
Dystonia is a movement disorder that causes a person’s muscles to contract uncontrollably. Over time the contraction can cause the body part that is affected to twist involuntarily and can result in contractures and abnormal postures.

The symptoms of dystonia can range from mild to severe in nature. So the reaction of Bryce’s body when he is excited is the brain sending the response to certain areas of his body. Bryce when watching sports will point his toes and scissor his legs. His arms get tight and go straight out (like Batman arms). He also has times where he will turn his head a bit and grind his teeth. All of these responses are a result of the message from his brain to these muscles. All along we believed it was just his excitement and his tone kicking in which it was. However, I never considered this as being something bigger and more concerning.

There are several other symptoms of dystonia that Bryce has that we were unaware would be related. When he eats he lets his head fall back and we constantly have to adjust his head position when eating. Some days he will keep his head turned to one side and we have to move the chair sideways so that we can get to his mouth. They also asked if Bryce keeps his arms bent and I explained that he did when we would try and change his shirt but definitely when we were putting on his winter coat (which is a very difficult thing to do). The more we looked into the more things became clear. We knew that things had changed but had honestly thought these changes were a result of him growing. Hearing this news was pretty devastating even though I really can’t say that it was completely shocking.

Why so devastating? The diagnosis of Cerebral Palsy was tough but we also knew that it wasn’t a progressive disorder. We knew that over time we would have to manage his tone but I honestly always believed that once we made it through all these phases of growth that we would be able to better manage his tone so that he could at least be comfortable. Dystonia is a completely different ball game. Most times it is a progressive disorder and (as the doctor told us) it is extremely difficult to manage. It never goes away and it can change over time. Some people deteriorate and can lose abilities. For example, someone that could once eat may need a feeding tube placed.

We discussed several different treatment options and we discussed that every person is different so the right treatment for Bryce will be trial and error. Everyone did agree that we needed to address this now because of the impact that it is having on bone development and so forth. There had been a dramatic change in his right hip from August to January alone…nearing but not quite at the point of needing hip surgery…. yet. So we started a plan to gradually bring on board medication(s) and we have also scheduled phenol blocks that can be injected into very specific nerves. Then we will see how things are at that point before determining next steps. Best case scenario – these things alone will be management enough (at least for now).

For a few days I had to exercise my right to be upset, mad and to kick yell and scream. It’s hard when you can’t find a release – when you are so mad and upset that you have no idea how to let it go. How can you keep spinning the positive when you keep getting punched in the gut? So as much as I wanted to throw my hand’s up and let the entire world know how unfair this is to my sweet sweet boy – I just tuck those feelings away again. We will deal with this and find the best way to manage it knowing now what we are up against.

The phenol blocks have been scheduled for early tomorrow morning. We are to arrive at 6AM and the OR is scheduled for 7:45AM. We should see an immediate change when he wakes up (Botox takes a week or two before any change could be seen). If all goes well we should be able to come home tomorrow. For a few days it may be hard for Bryce to adjust and he could seem a little “floppy” but hopefully this works and will last as long as possible before he needs it again. It is temporary but unlike Botox that lasted maybe 3 months for Bryce – this “could” last up to 10 months for him. I hope and pray that it will last that long for him but with it being his first time we won't really know. I am praying for positive results and a prolonging of a much larger surgery.