Today was a very unhappy day, as my wife and I attended the funeral of my aunt, Opal Shrontz.
Not too long after my wife and I were married, Opal was diagnosed with Huntingdon’s Disease — a genetic degenerative brain disorder. Currently there is no cure for Huntingdon’s.
The disease is caused by a defective gene which, to oversimplify a bit, causes the overproduction of a protein in the brain. As the protein accumulates, it gradually destroys brain functions (exactly how this happens is still not understood). The cruelest part of Huntingdon’s, then is that before the victim dies, he or she experiences years of gradual mental deterioration accompanied by symptoms such as involuntary movements and difficulty controlling basic motor skill such as walking or speaking. Typically, death occurs 15-20 years after onset.
My aunt was only 53. One of the more vivid memories I have of her is visiting amusement parks like Cedar Point. At a very young age I concluded that my aunt had inherited the brains in the family since, like me, she refused to go on many of the rides. We agreed, for example, that ferris wheels were clearly the devil’s work.
Unfortunately I didn’t get to know my aunt as well as I would have liked as a child due to some BS family conflicts that had nothing to do with her, and then Huntingdon’s robbed me of the chance to get to know her better as an adult.
Brian… Its christmas eve and i am thining of my mom, and thinking its sad that with all the technology, I have almost no photos of my mother.. I googled her just to see if anyone had ever posted a picture, and I discovered this… Now that I am 42, daily I wonder if this disease will come after me.. I hope you and the family are well… I dont know what I was thinking in my younger days to move so far away from my family, now to find myself stuck out here.. Hugs o Lisa and the kids, merry christmas