About Me

My name is Jens-Ingo Farley. I was born (in 1967) and live in California, U.S.A. I have an hereditary ataxia, or more specifically spinocerebellar ataxia type 3, also known as SCA3, also known as Machado-Joseph disease (MJD). I share this specific SCA3 diagnosis with between about 700 and 3,400 people in the U.S. but more generally between about 3,000 to 16,000 people in the U.S. with any form of SCA.

See here for more on the rarity of the disease.

I casually refer to what I have as ataxia, which is really just shorthand for its full name. I prefer using SCA3 over MJD when identifying the specific disease. Sometimes I’ll redundantly refer to SCA3 ataxia, just to be clear in the moment.

See here for more on terminology.

SCA3 is dominantly inherited via a parent’s defective gene, in which case that parent also had the disease if they lived long enough. It is a neurodegenerative disease that involves degeneration of the cerebellum and other parts of the brain, which causes gradual loss of control across the spectrum of brain function: physical, cognitive, and emotional. Cognitive and emotional issues are secondary and are largely ignored from pathology considerations, but since I’m experiencing them, I feel compelled to highlight them.

  • Physically, it degrades coordination (muscle control) in the extremities of the whole body, from the eyes to the toes. My biggest problems so far have been vision degradation, mild speaking impairment due to the excessive need to swallow, clumsiness, and walking impairment.
  • My own cognitive decline at this point is hard to separate from what I call mental exhaustion, which has a large physical component, but includes difficulty concentrating and learning new things, as well as memory issues.
  • All of my irrational fears (e.g., phobias and anxieties) are becoming amplified and harder to control, which is what I refer to as emotional issues.

Again, it’s about loss of control. Any ability that requires effort and/or coordination, even subconsciously, degrades over years of time. This includes abilities typically taken for granted, such as walking and swallowing, as well as abilities such as emotionally “holding it together” when at the dentist’s office.

See here for more on what I call this unholy trinity.

I’m starting this blog in July 2014, just prior to age 47, after being overtly symptomatic for over eleven years. Taking into account my genetic makeup, which I know from 1996 DNA testing at age 29, and my family history, I expect to last into my sixties after about a decade of being unable to care for myself.

Why this blog?

A hundred people with early-stage SCA3 will have a hundred different experiences. At endgame time, finally everyone with SCA3 is viewed more or less the same. The health care system and information out there in general is geared towards sloppily and roughly dealing with late-stage SCA3, not so much the one or two decades of struggle leading up to that. This blog is giving one person’s viewpoint on those early decades.

I have felt little connection to what I’ve read about the disease over the last 20 years. My diagnosis and DNA test results (i.e., CAG repeat count of 73) are unequivocal, yet I often feel like an impostor, because my experiences don’t seem to match “what they are supposed to.”

Perhaps it’s like the fictional population consisting of an equal split of people who are 5 feet tall and 6 feet tall and nowhere in between. The average height of that population is 5½ feet tall, but not a single person is that height. I suspect the world of SCA3 is like that: data are given in terms of averages that don’t exist, or perhaps too generally, like saying all people are between one and ten feet tall.

Reading about people with similar experiences is very satisfying. You could say that I’ve been inspired to write this blog by recently reading an article in The New Yorker, where I hung on every word and knew exactly what the author (Michael Kinsley) was writing about every step of the way, plus there was food for thought that made it even more interesting. The author does not have SCA; he has Parkinson’s disease. The article is “Have You Lost Your Mind?” from The New Yorker, 2014-04-28.

My attitude

I hope I never give the impression that I’m expecting different people to have identical feelings or experiences, especially when it comes to problem remedies. I give no credence whatsoever to people whose mantra is, “It worked for me, therefore it will work for you.” If somebody found something helpful, then it’s by all means worthwhile for others to hear about and consider trying.

Similarly, it’s not fair to say, “It didn’t work for me, therefore it won’t work for you.” Something that works for some won’t work for all. That’s the way all things function.

…within reason. I approach everything skeptically. For example, if someone claims that eating eggs cured their genetic disease, I know that is pure nonsense.

That said, I might sometimes overgeneralize. There are dozens of types of ataxia, and I have only one. In most cases, I am probably talking about my type of ataxia, and my own experience with it, unless it’s obvious that I’m trying to address a different type.


12 Replies to “About Me”

  1. A real treat to read such an honest and useful description of ataxia and its effects on a fit person. I am being brave tomorow and trying out an electric bike to see if i can keep up with m y wife. Good luck.

    1. Coasting in a straight line was the hardest thing for me to do, and pedaling was necessary to actively keep myself balanced. Good luck to you as well.

  2. You might be interested in how I use you (your log). My gene profile to ataxia not established. This forum lays out current drug trials and ataxia gene targets. No sense more gene identification unless more effective drugs available. Once more through the breech dear friends!

    1. Word of warning: since I have SCA3, I sometimes only address that variation (without intending to). I was lucky enough to be tested in 1996, after the test patent was filed but before the patent was granted. Testing was dirt cheap during that time.

  3. To Richard Franklin- hope to hear how the bike worked out. Each stage in life is something new. My inherited ataxia is like that. It is snowing again in the mountains of Canada today so no biking but the dog sled calls. Keep in touch .Brian Law.

  4. Jens, Any comment on the use of medical marijuana for the relief of symptoms associated with our baring stages of ataxia. Personally I find stoicism and distraction keeps me “real” but on a sceptical level on not against it. Again, there are many sides to an argument and I would like to hear your thoughts. Brian Law

    1. I don’t think marijuana will improve cerebellar function or help with early stage ataxia, but if it helps relieve the later stage symptoms that are the result of years of cerebellar degeneration and the compounding health issues, I have no quarrel with it. If it offers relief without the side effects of pharmaceuticals (or harder, illicit, feel-good-until-they-ruin-your-life drugs), it’s already coming out ahead. I don’t doubt that it’s the “high” that helps, but if it helps, it helps.

      I have no personal experience with it, but I think that smoking it is considered the most effective method of administration. That’s a barrier for me. I take turmeric every day in capsule form for peripheral neuropathy, and it works wonders. If I had to smoke it, I simply wouldn’t have ever considered it.

  5. Jens-Sara Riggare-Parkinson’s since 13 yrs old. Doctoral student/health informatics at. Karolinska Institude-Stockholm, speaker at. Nuffield Trust health policy summit. Feb 26\27. Article on topic Guardian News Paper website today. Check it out. Brian Law

    1. Today? I think you mean three years ago:


      I like the 1-in-8,765 idea for selfcare. It’s a nice mathematical idea I will remember. I’m currently at 0, because I don’t require/want/need/have-available prescription medication that I need prescriptions for, and I have long viewed my future desire for prescription medicine to be the only future reason to go from 0 to 1.

  6. Jens-just an aside on fatigue associated with sca. A blog comment on the role of neurotransmitters and fatigue in the guardian .com . Just a reference to neurons, hypocretin, and Orexin. Henry Nicholls-Marscolonist, Feb 25, 2018. Brian-guardian. March 4, 2018

  7. I am also a SCA3 patient. Recently I wrote a letter to professor McLoughlin, Hayley(U Michigan) , who is working on a very promising lead using ASO therapy in cooperation with IONIS Pharmaceuticals. She replies to me that it is very much in line with the recent approved ASO therapy to treat Huntington’s disease by IONIS. It is a very hopeful way to treat out disease.
    BTW, why don’t you update your blog now? It is very encouraged to fight with SCA3, for all of us.

    1. I have covered ASOs and Ionis on this blog, and I especially keep the Ionis section updated. In my ASO article, I’ve had a link to ASO research that Professor McLoughlin participated in, for over one year.

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