Dysautonomia

Dysautonomia.  Something we have been dealing with for years and years.  It can be a problem for Shwachman Diamond kids and it is a mitochondrial disease.  At first my kids only showed symptoms of not being able to tolerate the heat.  Anytime they exert energy, they become incredibly flushed and worn right out.  Summer is hard because of the heat.  We don't have central air so sleeping becomes a problem when the house is 85 degrees.  Hence, our living room becomes the bedroom and all three kids crash on the floor and sofa since the bedrooms upstairs are probably 110 degrees. 

The past couple of years, they have had more symptoms.  Some have really baffled doctors but now that we are learning more about Mitochondrial disease I am starting to recognize how much this part of the disease has advanced.  One problem Shelbie and Spencer have had is hives.  A couple of years ago, Shelbie had hives non stop for almost 8 months.  We never did figure out what was causing them.  Shelbie gets hives anytime her skin has pressure against it like when they do a blood draw or start an IV.  Even taking a band aid off will trigger a week of hives. 

Now it's Spencer's turn.  This in addition to his ongoing asthma attacks that we are still dealing with.  Here is an unbelievable picture of his hives.  Just remember, this is not a sunburn.  They are raised hives.

It started on Saturday night when he went bridge jumping with friends.  They showed up after the first jump.  Monday morning when he woke up, they were down quite a bit and his stomach was almost back to his normal, pale colored skin.  After his shower, they came back.  They come and almost go.  It seems like anytime water hits his skin, he breaks out in hives.  Monday, he went bridge jumping again and they were worse.  I am pretty sure he isn't allergic to water but the impact of the water hitting his skin is causing the hives and rash.

I think the 'trauma' of bone marrow biopsies, triggered a flare in his dysautonomia which also explains the asthma attacks.  I have been doing tons of reading tonight to try to figure him out.  I could take him to the doctor but they won't know what to do either. 

As I read tonight, I found an article that said dysautonomia can also cause vision loss.  This totally makes sense for Sam.  The last few months he has been losing his vision in his left eye.  Completely gone.  It's kind of scary.  It comes back after about a half hour. 

I never thought that 20 years later, I would still be learning new things about this disease.  I find the human body so incredibly intriguing.  I really wish I was a doctor because then I might actually know how to take care of them.  Until I get my degree, I hope these problems with Spencer go away soon.  He is most uncomfortable.  Just in case you wanted to know more, go here.