Cerebellar ataxia and me: trying to live while the brain is dying
Spinocerebellar ataxia sucks the joy out of another day…
As I have mentioned before, I have a degenerative brain condition. It’s called spinocerebellar ataxia, and is essentially an atrophying of the portion of the brain that coordinates and regulates muscular activity. If you read the details at NIH you’ll probably understand pretty quickly just how nasty it really is. It has taken away a lot of what I love in life and is, for now, uncurable. For the most part, there is also no treatment for the symptoms.
My doctors at the University of Colorado Health Center are some of the best in the business, and we have had frank conversations about what this disease means for me. They have encouraged me to join some online support networks and to have a DNA test, which will hopefully confirm the diagnosis and tell us for sure which variant of the condition I have (there are several). They believe I have Type 6, but can’t be certain without the test.
The reason they want me to have the DNA test is … well, it’s a bit cold, but also obvious. While there’s no hope for me, probably, whatever data can be gleaned from my case might help researchers improve their understanding of ataxia and perhaps even help effect a cure someday. (I should note that one of the specialists in question is more hopeful about a cure or treatment in my lifetime than the others. I hope she’s right, but I suspect she isn’t.)
I’m perfectly fine with their motivations. I don’t expect to survive this damned thing, but if I can do anything to help others I’m all-in. This is true generally, and it’s especially important considering that ataxia is genetic. It may be stalking my sisters. My cousins. My nieces and nephews. This is, in all likelihood, the disease that killed my grandmother and her sister, and maybe her brother, as well. If I thought my death would save those near me, I’d pull the plug today.
There’s a problem, though. The doctor’s office got back to me yesterday on what goes into the testing process. I had expected that the test would probably require some out-of-pocket expense on my end, and I was thinking the hit could be a few hundred dollars. As it transpires, the testing lab is out-of-network. My insurance will cover 60% of the cost. Which means that my investment would be – brace yourself – $7,000.
That’s a lot of money. Specifically, it’s more than I can possibly pay.
Visits to the doctor to discuss my progress – okay, my digress – are always hard. I have accepted my lot and vowed to wring every last drop of joy from whatever time I have left. And in truth I have probably been happier over the past year and a half, if you can believe it, than at any point in my life. Normally I can plow through the day dealing with work, reveling in my photography, enjoying time with friends and loving every second of my time with my new girlfriend and her psychotic Blue Heeler. My reality is my reality and every time I stumble or fall, every time I see someone playing basketball or soccer and know that I can’t anymore, every time I have one drink and it hits me like I’ve had five, I’m reminded of how different my future will be from my past. But I don’t dwell and I don’t let it depress me. To paraphrase Zen master Bodhidharma, it is what it is.
The doctor visits, though … For an hour or so every few months the whole world boils down to what I can expect. Yesterday, for instance, there was a moment where the doc and I were talking about the normal advance of ataxia. I haven’t noticed any worsening lately, which is great, but the way it usually works is that you hit a tipping point where it begins going downhill rapidly.
I remember how my grandmother’s illness took her out. She went from being perfectly functional to being a vegetable, unable to move, unable to communicate, unable to even let us know if she was still there, in a matter of two or three months.
There’s a moment of awkward silence at this point of the conversation. How can there not be?
I don’t know how long I have. Maybe 20 years. Maybe five. Maybe a bus hits me tomorrow and it doesn’t matter. But in my mind I begin planning for that moment where I feel myself falling off the cliff. Planning. Thinking thoughts nobody in their right mind ever thinks.
My sense of humor has always embodied a dark streak, and the past few years have honed it to a positively vorpal edge. I sit there and crack jokes about wasting, about dying, about having my brain sawn out of my head before I’m cremated. It’s inappropriate in the extreme and those who hear it have to feel horrid when they catch themselves laughing.
I think you get to a certain point where you use whatever you have at hand to deflect talk away from what you’re really thinking about.
It kills me that I can’t afford this test. The doctors understand, and we talked about keeping tabs on research trials that sometimes, because they’re having trouble rounding up enough subjects, will offer to pay for the testing. I plan on doing that. We also talked about what I need to do to assure that my brain is donated to the right places when I die, and soon I need to get serious about writing up a will. I don’t have much to leave anyone in the way of material things, but if you know me, you understand that nothing I could ever dream of owning would be of more value to me than my brain.
I never feel quite so helpless as I do on days like yesterday. And I don’t deal well with helplessness. Please bear with me…