SCA3 ataxia results in cerebellar degeneration, but its symptoms are not particularly unusual and not what I would call bizarre or horrific. In my family, and with myself in particular, degeneration is so slow that I see it like accelerated aging, as the issues are not much different. Cerebellar degeneration eventually causes a lower quality of life than graceful aging (e.g., speech dysfunction aka dysarthria and swallowing dysfunction aka dysphagia), but mostly it’s causing me to confront aging issues 50+ years early.
This can be hard to grasp
In 2010 at age 43, I put my ataxia-adjusted age at about 85. This is totally nonscientific, of course. I felt that way because I began to rely on the aids in place for the elderly in this country. For example, I was able to climb onto the public train here, with heavy luggage in one hand, only because of the thoughtful placement of handrails everywhere that was beneficial, all the way to a seat. Clearly, the process had been given some thought. These aids are tuned out and unused by people who don’t need them. I am tuned into them and often rely on them. I “scan ahead” for them and anything else I can lean on without breaking, in any new situation.
Contrastingly, in Bora Bora, French Polynesia, there are essentially no such aids for getting around. There are almost no handrails anywhere! It would have been impossible for me to safely travel there without a companion.
Not a river in Egypt
I assume that people who age gracefully are not in denial or oblivious to the fact that they are aging. I assume that aging in “normal” people occurs at a rate that feels somewhat natural. Occasionally, they may even get the upper hand on an issue when they first experience it. For example, if you discover you need reading glasses, you get reading glasses (even though eventually your eyes may get even worse).
It is a bit worse with ataxia. You don’t get time to adjust to, get used to, or fix your degradations before they get worse and multiply. You never get the upper hand or improve. You never get the chance to make the most out of a bad situation. Symmetrical double vision due to ataxia? There’s no solution, but you must reduce dependency on your vision, such as by no longer driving.
The only saving grace is that it’s unlikely you’ll wake up with a totally new issue from one day to the next, though the flipside of that is it generally takes a few years for symptoms to mature and fully realize themselves, during which time you’ll likely feel like an outsider to your own disease.
This can be hard to swallow
I like how Wikipedia puts it: “Old age is not a scientifically recognized cause of death.” I guess dying peacefully in one’s sleep is not a common occurrence.
https://en.wikipedia.org/wiki/Death_by_natural_causes
SCA3 is not a cause of death, either. Death is always due to some malfunction of the body. Interestingly, the causes of death I looked at when I examined the rarity of ataxia apply to the elderly. For example: heart disease. An elderly person in the U.S. is most likely to die of heart disease. When you have SCA3, you skirt some of those statistics. You are more likely to die of something related to your poor swallowing ability, such as aspiration pneumonia.
https://www.cdc.gov/nchs/data/ahcd/agingtrends/01death.pdf
https://www.ninds.nih.gov/health-information/disorders/spinocerebellar-ataxias-including-machado-joseph-disease
Clarification
Life is a fatal condition that everyone has, but diseases we usually refer to as fatal are generally horrific and fast-acting, such as Ebola or smallpox. These horrific diseases are generally contagious and subject to epidemics or pandemics, which further differentiates them from SCA3.
Let’s be clear: SCA3 is an incurable, fatal disease. In my circle, it reduces lifespan by two or three decades. But Ebola kills you in a little over a week. It wouldn’t be right to think of Ebola as accelerating your aging process to bring you to death in one week.
SCA3 isn’t actually accelerating the aging process. But degradation occurs so slowly, having SCA3 is taken care of (as well as it can) using many of the medical and social programs already in place for the aged. SCA3 fits the aging pattern (though a bit accelerated) better than it fits the incurable, fatal disease pattern of something like Ebola.
If it’s raining, let it rain
I think that acceptance is of key importance. Ataxia causes me to go through a lot of what everyone goes through eventually, including the financial aspects of it: Social Security, Medicare, assisted living, and so on. Everyone has to deal with these issues sooner or later; physical independence wanes eventually. True, the U.S. government programs and society as a whole are a bit more fine-tuned for the elderly than for the young and disabled. And having your own kids (I don’t) would complicate matters. I’m not hinting at any panaceas for anybody, young or old.
The flip side to this is that acceptance can be seen as highly pessimistic by some, whereas I see it as highly optimistic. Some feel you should keep pushing yourself to hang on to your old abilities, and accepting their loss means you’ve given up—which is understandable, up to a point. Eventually, it gets to be like pressuring someone in their 90s to go skiing, to go bicycle riding, or to try learning a new musical instrument. Ataxia is reducing my lifespan and my capabilities, so I expect to slow down, scale back, and reapportion how I live my life accordingly.
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