It is a scary thought to realize how many other kids there are in the world right now who are gene positive and pre-wired with their own “HD personality” who are struggling in school right now. They are already suffering the effects of HD and having the crap knocked out of their self-esteem even though their official diagnosis will not come until they become adults and choose to be tested. Kids who are obviously symptomatic can be diagnosed with the onset of JHD and helped accordingly. But what about the rest of them who are right now struggling as I did through school who will not understand why until years later?
The official diagnosis of “onset” is under hot debate right now. Jane Paulsen did a study where she charted the rate of loss of a particular ability against time as the years prior to onset. They observed that there was a slow steady decline in a particular measure, and then, 10-15 years prior to when typical HD diagnosis would be made, a significant decline in the measurement started and continued to drop at a progressively worse rate. By the time that someone is symptomatic in such a way that the disease can be diagnosed by a doctor as being evident, about 20% of the cortex is gone and a third to a half of the basal ganglia.
That was a really sobering study and it helped me to understand that I was already losing major brain cells even as a young teenager/child. It was not juvenile HD but it was the pre-onset HD symptoms. I remember when I was 16 saying that one day the doctors were going to find out that there was something seriously wrong with me because it could not be normal to feel the way I felt inside. I was a square peg in a round world until I found out I had HD and met some other square pegs at camp.
I remember when I got my first literature on HD back in 1996 when I found out out of nowhere that I was at-risk for it when my father tested positive. I had the whole list of emotional and cognitive symptoms on the pamphlet and had had since I was a teenager. I knew I was symptomatic before the gene test even came back positive. I wondered then if I had not been wired differently from birth. Looking back it seemed I had been predisposed to certain HD personality traits earlier on that became more and more pronounced as the years passed until they were more pronounced and officially was called onset many years later.
School had been very hard for me. I had problems with concentration, retention of information, and comprehension of what I learned. I did well on the class room work but bombed on the tests. I transposed a lot of letters and numbers (which lost me lots of points in math), could not remember math formulas, and word problems were my nemesis; numbers were hidden in the words and I would miss them. Ironically I was the one who asked to be tested for a learning disability at age sixteen.
They did not find anything wrong but we did not know I was at-risk for HD then, let alone that I was both gene positive and had the early pre-HD wiring/symptoms already. No wonder I thought I was crazy when none of my quirks matched any of the known disabilities that they tested for. I figured I had some kind of dyslexia back then. Doctors kept telling me that nothing was wrong, but I knew they were wrong all along.
Needless to say, I knew the moment I saw the list of HD symptoms at age 28 that I had found my answer; I felt vindicated when the gene test confirmed my status. But the next challenge became to get officially diagnosed with “onset”. It was very frustrating because I personally spent 365 days a year with me and I knew what all my body and mind were doing, but I would spend an hour a year with the HD doctors trying to convince them that this disease had already started for me years ago. Even then I could see an obvious path of HD all the way back to my young teenage years. But when they would only go so far as to say it might be HD because I only had the cognitive and emotional symptoms, it made me feel like they thought I was only imagining the symptoms. I knew already that they were very real.
I certainly did understand that they were worried about how some people react when they find out their HD symptom clock has started, especially with the high rate of suicide in Huntington’s. But it was crazy making in the beginning. The first symptom I was able to show them a few years later was the tremors. They had started as an internal pulsing that felt like my body had electricity running through it. I found if I took my arms away from my body and lifted them straight out in front of me and pressed my hands together hard, that by putting someone’s hand in-between mine that I could buzz them. As my arms became more strained the sensation became more intense and became jerky.
Once I was finally diagnosed as symptomatic because the chorea became more obvious I no longer felt I needed to convince my doctors that it was indeed onset. So with that 10-15 year pre-HD brain loss information this whole thing makes sense to me now. It is because it is such a gradual descent that the HD manages to stay under the radar for many years. I am not saying that those early symptoms that I have listed in this written piece apply to everyone but in my case they were definitely early indicators.
Until next time, remember that being happy doesn’t mean everything is perfect. It means you’ve decided to see beyond the imperfections…