Glycogen Storage Disease – StarchWars

Glycogen Storage Disease and the Subtleties of the Brain

Posted on 11. Nov, 2007 by BMW in Glycogen Storage Disease

Our family is now beginning to dive deep into the mysterious realm of the brain. Since our older boys were diagnosed with GSD 1A there has been an underlying concern for the well-being and health of their brains. It was apparent that due to multiple exposures to hypoglycemia there had been a delay and possible damage to their mental development. Since diagnosis, both boys have made tremendous progress and like their physical appearance they are generally recognized as being “normal”.

Of course, we want them to feel as competent as possible, but as with the physical aspects of GSD the neurological symptoms seem to lie dormant and difficult to detect especially with good metabolic maintenance. I think many a GSD parent is familiar with others not seeing a “problem” because the kid looks normal.

We have recently met with a neuropsychologist that was able to give us some great insight as to how metabolic diseases that impact glucose levels can effect the brain. We were pleased to finally hear someone pinpoint and have a logical explanation for the concerns that we had for our child’s mental health. Apparently, automated processes in the brain that most people take for granted, are the parts that are effected by the glucose deficiencies. In short, a child may gain the ability to write and recognize letters, but the organization of those pieces may be jumbled making comprehension and verbalization complicated and frustrating. This is exactly what we were seeing happen.

We doubt that our other children will have as many of the challenges that our eldest is experiencing, but it became very apparent that because Glycogen Storage Disease disrupts glucose levels, their brains will very likely be effected.We definitely want to know what to look for so that we can minimize their frustration and maximize their learning.

So now that we enter a new dynamic of GSD and the brain we will find out more from Dr. Weinstein as well as our new neuropsychologist . Hopefully we will be able to add some more insight and more accurate information for other parents as we become more educated on the mysteries of neuropsychology.