Jonathan
Forum Replies Created
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I started taking omaveloxolone (Skyclarys) on July 3rd. Because of United Healthcare and Reata’s REACH program, I pay nothing. Maybe 15-20 days after daily use, my wife believes my speech is improving.
I’m 42, and I’ve been using a wheelchair full-time since I was 28.
My son (15) and I have been running our own experiment since day 1 to quantify what functions measurably improve. It involves 5 activities. We record data every 21 days.
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The pharmacy also called (yesterday afternoon) nd confirmed confirmed coverage. The pharmacist was very kind and went over the usual precautions and details for a new prescription.
BTW, the pharmacy is called Biologics.
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Actually, the pre-authorization came from my prescription insurance which is OptumRX. I think that’s a Medicare Part D thing.
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I’m 42. GAA repeats measurements are about 650 and 900. I’ve got United Healthcare/Medicare. I just got the recorded message phone call from either Reata or its pharmacy (Biologique or something) that they got pre-authorization.
Be sure to register with Reata’s REACH program. Their main purpose is to coordinate funding for the patient getting the drug.
BTW the pharmacy isn’t expected to be ready for distribution of prescriptions until about mid-August.
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Coughing’s been annoying more and more lately. I suspect it has something to do with progressive loss of sensory nerve tissues that innervate the epiglottis. Basically, my brainstem or cerebellum is losing it’s ability to sense when the epiglottis should close (rapidly, like any other reflex).
I wonder if I should adopt some sort of swallowing regimen in my daily physical training.
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My home is getting a young German Shepard before the year’s end. A costly one. Yay. We already have 3 ferrets, 2 tarantulas, and a snake. I don’t even actually like animals, but the wife and son do. So after some mental gymnastics, I found a reason for a dog.
The goal(s) is that my son (11 years old) gains more responsibility, the practice of training an animal, and a strong commanding voice. Plus, he (and my wife) really want a dog. I’m more like, whatever — it’s more expenses, but it’s probably gonna make ’em happy and serve a purpose. Fine.
Anyway, everything is being planned: feeding an animal that’ll grow to around 100 lbs., training techniques, training regimen, health insurance…and shedding. Yeah.
My son’s allergic to pet dander. And he’s always refused medication for it. I warned him, though. “You think the ferrets’ shedding is a nuisance? The dog will be way, way worse.” But his want (and why) for this dog is so strong (which is good) that he’s agreed to vacuum regularly and eat allergy medication. Okay, really? Maybe I’ll like this.
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Yeah, the degeneration is mostly among certain afferent nerves that have looong sensory axons reaching from certain nuclei just outside the cord (dorsal root ganglia — DRG). The axons innervate muscles and pass sensory data of when that muscle fiber is relaxed to itself (i.e., resulting in spinal reflexes) and up to the cerebellum, which rapidly maps where the body is in space (i.e., proprioception).
That “map” (proprioception data) is passed from the cerebellum to the motor cortex to plan appropriate voluntary movement. But due to tissue loss, the proprioception data isn’t satisfactory, so the cortex (which doesn’t operate anywhere near as fast as the cord or cerebellum) tries to compensate…but it’s not effective. And we get the wonderful world of ataxia!
Really, though, and I mean extra nerdy specific: it’s the oligodendrocytes. Those are the teeny tiny cells that wrap all along the DRG axons with myelin. The myelin is supposed to act as shielding for the axon so its signal gets smoothly and quickly transmitted up to the DRG and cerebellum. But as those tiny buggers become weak and off themselves (i.e., programmed cell death — step 1 is crappy mitochondria), the axon loses bits and pieces of its myelin.
Imagine a wire with pieces of its cover missing. It’ll be vulnerable to interference and it won’t transmit signal very well either. So, in effect, the cord and cerebellum get crappy signal. It’s a gradual process and I suspect that even certain nuclei in the DRG and (((maybe))) in the cerebellum commit suicide too.
I could be wrong, though. I didn’t read the article. This is what I remember from college and studying other FA research BACK IN THE DAY.
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“…that’s not where garbage belongs, and whoever left the cup made it someone else’s responsibility, which I thought was a cowardly way to pass the buck.”
Agreed. And this irresponsible attitude can and has grown and spread throughout culture. Too many people are contaminated by it, so it’s practically normalized. If you have ataxia or any sort of disability, you especially do NOT want to make your life feel more difficult and have less mental fortitude by carrying around a negative attitude.
Your attitude is your responsibility. Don’t wait for somebody else to take care of your garbage. I mean that both literally and metaphorically.
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I want to see the live action version, but my son Leo (11) isn’t interested. AND HIS NAME LITERALLY MEANS LION! So, yeah, that’s a little disappointing. Anyway, your questions and my opinions (well, not mine but ones I collected and keep shiny in my treasure chest)…
1. Life can seem linear. Birth, childhood, adolescence, adulthood, old-hood, hood-rat (actually, that might be somewhere after adolescence), and death. But that’s a shallow, temporal point of view. There’s much more to life than biology, which (haha) means the study of life. An individual can often experience a few psychological/spiritual deaths and rebirths. He or she transforms. New identity, new life. And it’s a cycle. The circle of life, bruh.
2. Transforming isn’t pleasant. Part of your psyche/spirit must die before there can be rebirth. It is for this reason that most people avoid it (until it might be thrust upon them). Hakuna Matada is hood-rat level and younger. No responsibilities, no deep meanings, not much control (so why bother?), and a lot of things seem random or a matter of luck. The spirit can get stunted and degrade into escapism and nihilism.
But when the individual voluntarily crawls onto the hot coals for transformation, he or she is taking control and maturing. That individual knows it’s scary and it’s gonna hurt, but he or she is gonna pick up and carry the heaviest weight he can (i.e., responsibility), and be king (like his father before him).
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Even with this optimistic spin, yes, nihilism is bad. The video mentions to pursue what is meaningful, which I agree with, but it conflates meaningfulness and happiness. They’re not synonymous. Pursuit of happiness is a slippery slope. You might become a slave to your senses and, i.e., progress into an obese glutton, a heroin addict, a shameless attention seeker…and worse…or a mix. These pitfalls of human nature (there’s all kinds — 7 cardinal sins is a decent breakdown) have been warned about and discussed for a long, long time.
Slaves to their senses and emotions suffer much, much more when their mood consumes them and/or their sources of happiness are gone. When you’re on a mission with meaning, you can happily suffer.
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I live near the big ones in Orlando. And I have kids. Do I participate? Yes. The kids love the rides and the experiences and the souvenirs and food and blah, blah, blah…I do NOT. Why? COST. Sure, I get escorted to the ride through the exit (in a rusty, over-sized wheelchair — can’t use my own), which is fine. Then I crawl and climb onto the roller coaster seat. It’s fun.
But people on vacation are rude, and people are EVERYWHERE. You feel like you’re in a herd of cattle being moved around from one part of the ranch to another. Whatever. Mainly it’s the cost-benefit ratio. In my opinion, it’s a slaughterhouse. When you’re in charge of the household budget, a lot of this looks like one rip off after another.
“Oh, but think about the children, Jonathan, and their wonderful smiles!” Yeah. No. There’s countless other activities to do that are way, way more affordable. If money, though, isn’t your concern, then go for it. Getting on and off rides might be tricky and uncomfortable, but at least you’re not paying for it.
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I live beachside in Daytona Beach, Florida. My home’s on the 5th floor of an apartment building facing the ocean and all the weather she brings. My wife, son, and I have tolerated a few hurricanes. The annoying issue is loss of electricity, which means the loss of a lot of things including the elevator.
Sure, we’re warned (or mandated or whatever) to evacuate…but we’re like, nah, bruh. We’re prepped. We’re committed. BRING IT. It’s a helluva view.
Basically, we camp inside until power’s restored. I think last time took 7 days or something. And when I say camp, I mean heating up cans of rice & beans with a little burner and fuel cells, goofing around with flashlights in the dark building halls, and day drinking. Sure, it was a little uncomfortable, but we made some memories.
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I avoid clothing that has buttons or laces. When the situation demands something nicer than t-shirt and shorts, I get help from my wife. My main advice for getting dressed is use simple clothes and do it on the floor. Getting dressed on the floor means I have to practice getting down from my wheelchair and climbing back on, which is a workout. Literally. I train this task. I might do 10-30 reps a day. I own the floor!
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For me, it’s lectures/podcasts from two specific people that really changed me. Dr. Jordan Peterson (a professor and practitioner of clinical psychology) and Jocko Willink (a retired US Navy SEAL, commander, and combat veteran). Their content is vast, free, and on YouTube. Anyway, these men taught me about psychology, personality transformation, responsibility, suffering, meaning, human nature, leadership, and discipline. These subjects gave me a larger perspective and practical tools that really helped minimize the impact of FA on me. I still review their content.
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If you’re not sweating, you might be too comfortable. Comfort is nice, but it’s a slippery slope towards weakness.
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My thoughts are…not positive. I think like most NPOs working to raise funds to solve a certain problem, they began with a noble cause. But the medical science to solve genetic issues isn’t here. It never will be.
But, hey, they keep hope alive for many patients and families. Some of the funds they raise pay scientists to keep on science-ing. And some of those funds keep a few FARA folks employed.
Is it worth it? I used to think so.
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Struggling is life. Whether or not you have ataxia, struggling and suffering and failure — it’s all unavoidable. Does it make you stronger and more resilient? Does it make you smarter and adaptive? Yes and no.
Yes, it does make you better, but only when you see yourself overcome it. Did you solve problem? Did you tolerate the pain? Good. Do you understand how you did that? Better.
Don’t expect to get tougher by simply struggling through life with ataxia. Look at your struggle, your obstacles, your mistakes and really understand HOW and WHY you grew past them.
The struggle isn’t what changes you into something better. Most folks wilt when there’s resistance. They change into something weaker. Metaphorically or psychologically, they move closer to death. And when you’re mentally weak, which I realize is an abrasive phrase, every little thing feels like a struggle.
Like I already mentioned: it’s overcoming struggle that makes for a good change. Seeing yourself do it and understanding it makes for a better change.
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Be prepared to use your hands, bruh. Or just simply ask the airport.
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I don’t know what kind of labels describe me. They’re not that important.
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Actually, in my opinion, falling over and moving from point A to point B is EXACTLY what physical training’s base should be. Repeat it 10-20 times. BOOM. Workout done. And it’s completely practical.
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As I gradually became more familiar with the science of FA and medicine, as well as, the economics of NPOs and medical research…I decided to stop. A lot of it is a game or trick. If I get proven wrong, then great. I really wouldn’t mind. But there’s a helluva lot more to life. FA isn’t really a priority for me.
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Stop pretendin’, yo. Love is about sacrifice.
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Hahah!