William's updated diagnosis

We visited our usual neurologist this morning. He observed the decrease in William's abilities and also commented that his hands are curled more than even the first visit we had. His reflexes are missing in his legs and now in his arms, which is new. Due to the unknown onset of this condition (sometimes it can be linked to a surgery, stomach virus, vaccinations, etc, but we have no indication of that in William) and the continuation of symptoms past 8 weeks, he has diagnosed William with the Chronic form of GBS, called Chronic Inflammatory Demyelinating Polyneuropathy. The condition is the same as Acute IDP/Guillain Barre, but does not resolve itself in the brief time that GBS patients experience. This does not mean that he can not spontaneously recover from the condition, but his condition could continue on for a very extended amount of time. Treatment for CIDP includes the IVIG treatment he has been receiving and possibly steroids. Other treatments can include immunosuppressants, but those are not even on the table at this point.

We know that the IVIG is effective in relieving William's symptoms and given the drastic side effects of Prednisone, we'd like to avoid that if possible. Of course, steroid doses can be administered at home and are far cheaper than hospital admittance for IV blood products (which run $30-$50K per treatment) and we will pursue them if necessary. The doctor really wrestled with whether to begin a steroid treatment today or whether to begin a course of regularly scheduled IVIG treatments. Before he continues treatments, he needs to do a nerve conductivity test. This is the test we've been fearing. This test involves electrode stickers placed strategically on his arms, legs and hands, through which they run electricity and measure the reaction times of his muscles to determine where and how the nerves and/or muscles are failing. In a person with healthy nerves, it feels like the shock you get in the winter when you're wearing wool and touch the door knob. For William, who is extra sensitive right now to all stimuli because of his nerve damage and because of his prior diagnosis of Sensory Processing Disorder (SPD makes his clothing tags feel like sandpaper on his skin and walking flat footed on the floor feels like walking on jagged rocks), this will be unbearable. On top of that, the doctor will have to use a higher setting to get a reaction from his nerves at all, since they are damaged. Which begs the question, "WHY exactly are we putting our 4 year old through this??" The doctor and I discussed that very thing at length. This test will not alter the treatments at this time, but now that we know we are dealing with a chronic problem, it is important that we have a benchmark test to assess any future downturns to determine the necessity for drastic treatments, like the immunosuppressants. By now you are thinking what I was when the doctor said it...if this test is the benchmark, we'll have to endure another one at some point to be able to compare. And now you are thinking, like I was, that this guy is just sadistic to put a young child through that not once, but twice (or more). I honestly feel like he would prefer not to perform this test on William and there is no guarantee that we will have to do another one, unless William continually worsens, but it will become an essential tool if we continue down this horrible path over the next months or years. The doctor has prescribed a sedative that should not interfere with the test results, but will make William relax for the test without actually making him unconscious. "Schnookered" as the doctor called it.

William seems to be following a 4 week cycle with the IVIG treatments and subsequent weakening. We will perform this test on Wednesday morning, allowing us to enjoy William's 4th birthday party at the fire station this weekend and also to see how his condition progresses without IVIG intervention every 4 weeks. We will be watching for signs of distress, breathing difficulties, changes in his voice, or his ability to hold up his head, all of which will send us immediately to the hospital for intervention with IVIG. Also, any further weakening even without distress will warrant hospital admittance after the test on Wednesday for IVIG, but if he remains stable through Wednesday morning, we can pursue a path of allowing his own body to balance itself without hospitalization. Regardless of potential intervention he will continue with physical therapy until this condition becomes a thing of the past. Continual exercise of his muscles is essential for preventing permanent paralyzation and weakness.

So, as it turns out, we're in this for the long haul and it won't be pleasant. In a condition which normally repairs itself in its own time frame, there are no easy answers and no rushing things. I'm confident that God will heal William at the appropriate time, perhaps when I've learned the lesson from this that I need. (So, let's get on it with it...hit me over the head with that lesson and let's get better!! ) :-) On second thought, maybe a lesson in God's timing is in order here.

In case you were worried, William is still the brilliant and sweet boy he's always been. He's frustrated by his lack of abilities now, especially when trying to keep up with his sister. But, you've never met a more cuddly boy and he's so forthcoming with expressing his love too.

Thank you for your prayers.