Most of us have likely felt that at some point in our lives we’ve danced with the devil. In my case, I was dancing with the devil known as Huntington’s disease (HD). HD is a genetic neurodegenerative disease that is sometimes referred to as a combination of ALS, Parkinson’s and Alzheimers all wrapped into one, in that for those who have HD, over time it will destroy your mobility, cognitive ability and emotional stability. It isn’t that you don’t have movement, thoughts or feelings, you just lose the ability to control them. It isn’t a very nice disease and so far there is no cure. The only therapies available can offer just an attenuation of symptoms while the HD progression marches on unabated.
As a genetic disease it is passed down from generation to generation, all of whom live in a 50-50 lottery of having it, depending on if they have the particular gene from the parent with HD or the parent without. The gene was identified in 1983, I think and a test for HD was developed in 1987. In the United States, roughly 30,000 people have been diagnosed with HD and somewhere between 200,000-250,000 are at risk of having HD.
And therein lies the problem for a lot of us. We fall into the at risk category becase many of my generation (I’m 51) had no way to know that one of our parent’s had HD. It wasn’t until my older brother was diagnosed about a decade ago that I’d even heard of Huntington’s, much less understood what happened to my mom. Since he was tested and was diagnosed with HD it became clear that my mom had it, though she died before the gene was identified or the test developed. And with an older brother diagnosed that put a bullseye on my back. It was at that point that I learned that I have a 50-50 chance of having HD. I became one of 250,000 people in the US who find themselves at risk.
Since I’ve already mentioned there is a test available for the HD gene the obvious choice would be to get tested. Oh, were it so simple.
The emotional roller coaster of living ‘at risk’, so to say with the HD bullseye on your back, is offset by some very real world implications. There are some anti-discrimination laws that come into play for health insurance and for employment but if you test positive for HD you will not be able to get life insurance or long term care insurance, both of which are made even more necessary with a positive test and diagnosis. I get the business reasons why this is true, but it is problematic, nonetheless. These are just a couple of the challenges that factor in when decided to get tested, or not.
There is also something to be said for denial, too. It seems ever so simple to get tested but knowing you have HD has profound implications in real life. Spending your life wondering whether you have HD has a certain appeal over knowing you have it. Some have referred to a positive HD test as a death sentence and in some sense that is true but really, we’re all going to die. What differentiates knowing you’re going to die from HD from dying any other way is knowing that you are going to die in a particularly shitty way. Loss of mobility. Loss of emotional control. Loss of ability to speak. The most common cause of death for HD folks is aspirational pneumonia which means you choked on something and got it into your lungs, causing pneumonia. Did I really want to know that is how it ends any earlier than absolute necessity?
Also, I had a fear that I was actually an asshole and if I found out I had HD the veneer that I keep up that makes me seem like a nice guy might come crumbling down. Logical? No but as I hope you’re seeing, HD does not always lend itself to logical processing.
And what about the effect on family, especially my wife and kids? Logically, getting tested should allow for planning. Financial planning, life planning, death planning. Again, there is a certain illogical comfort to be found in denial. Denial isn’t going to change the outcome one way or the other so why live in the middle of the fight before you need to?
My wife was in the middle of all of this, waiting and watching. I suspect it was incredibly difficult for her but that’s her story and I wouldn’t presume to tell it. I can say that she supported me every minute of every day.
Of course if you have kids, and I do, that adds another dimension to the internal debate of getting tested. Or not. Children of at risk people live with a bullseye on their back too. Not knowing whether their parent has HD leaves them in the at risk category as well. Not only do they have to deal with the above implications, they have to make decisions on whether to have kids. This weighed heavily on me.
As did the wondering. Wondering if that little twitch was the beginning of symptoms. Everyone has those weird little hand, arm, leg and foot twitches but when you are wearing an HD bullseye on your back you wonder every time it happens if this is it. Every time. Wondering if the fact you can’t remember something is the beginning of cognitive problems. Wondering if you’d passed the gene on to your kids. Wondering, wondering, wondering.
These are ultimately the reasons I decided to get tested. A combinations of things, specific to me and my family.
Part of the decision stemmed from a desire to help my kids out, allow for family to plan financially and ultimately, I wanted to be done with wondering. I was going to get tested. To be clear, getting tested is an intensely personal decision that defies logic and defies one size fits all. Even so, it was my time to make it happen.
Appointments were made, schedules were set. Finally, test day. There are more details than I’m going into but the test comes down to a blood draw that is sent off for genetic testing. To say the least, it is a stressful time as witnessed by a blood pressure reading of 153/110 rather than my normal 110/78. It is also the time when the HD bullseye on my back became a set of crosshairs that felt like they were centered on my head.
After the blood draw the delay in getting results was about 3 weeks for me. Three weeks of not knowing. Three weeks of non-stop wondering what I was going to find out in just a few weeks. Just a few days. Just a few hours. Three weeks of living with a set of crosshairs that felt like they were centered on my head.
And then, at long last, the results appointment time. Time to dance with the devil in the pale moon light. Turns out that time becomes a surreal dimension when you’re dancing with the devil. It seems to slow down up to the point where the genetic counselor shares the actual results and at that point, at that moment, time stops. Time stops as you wait to hear one of two words. Positive or negative. One word.
In my case, the word was negative. In that one word, the dance with the devil stops and time starts again.
I’m still reeling a bit from that news, which I understand is pretty common. When you live with the bullseye on your back for a decade and with crosshairs centered on your forehead for three weeks; when you dance with the devil and time stops, it takes a little bit to get recentered after the bullesye and the crosshairs go away and when the dance stops and time restarts.
I don’t know yet. I guess I’ll continue holding up the veneer that makes me seem like a nice guy. I can quit wondering about a lot of things, though after a decade this is a slow process so far. We’ll have to see.