7 Oct., 2020

Fatal Familial Insomnia

More Fatal Familial Insomnia

The very existence of this terrifies me. I don’t have it. I’m sure I don’t—I’d be dead by now if I did, because you live only a short time after its onset, and I’ve been living with insomnia for almost 40 years. Besides, its age of onset isn’t 16, when I first began having sleep problems, nor 28, which is about how old I was when I first sought help for insomnia.

Nonetheless, reading the list of symptoms of sleep deprivation is frightening:

Some affected individuals may experience double vision (diplopia) or abnormal, jerky eye movements (nystagmus). There may be problems with swallowing (dysphagia) or slurred speech (dysarthria). Some individuals eventually have trouble coordinating voluntary movements (ataxia). Abnormal movements including tremors or twitchy, jerking muscle spasms (myoclonus).

I’ve been sleep-deprived enough at one time or another to have experienced each of these. Sometimes just one at a time, sometimes several at once.

I know that myoclonus as a primary diagnosis is a serious disorder, even a disability. I know people with this diagnosis, and I don’t feel right using that word to describe what happens to me; I get “the twitches.” It’s scary nonetheless. Sitting and quietly writing something, then with no intention, flinging your pencil across the room, is dangerous to anyone else in the room, and hard to explain. No apology is ever enough, and you can’t just offer up the fact that you haven’t been sleeping well; people don’t understand. They think you’re been up partying and being irresponsible; they think you’re exaggerating; or simply inventing an excuse—albeit for what? voluntarily tossing your pencil across the room because it’s so fun? No one believes you spent eight hours in bed last night, but only slept 3 & ½ of them, so it’s not worth trying.

Sometimes with the twitches, you get a sensation out of nowhere that you are falling. You may be sitting, or even lying down, but abruptly the ground opens up underneath you. You may reach your arms out to try to grab hold of something, and knock things over, or you might just startle– if you are standing, you can then fall for real. You get facial tics. I got asked once if I had Tourette’s syndrome.

Double vision is scary too. You struggle against it, and get tension headaches. You’re stupid enough to drive when you’re fighting it, because when you’ve slept 10 hours in the last 6 days your judgment is really poor, and you lose the fight while you’re on the road, or roads, as it suddenly appears. You pull over while your heart pounds, and try to pray it away– just long enough to get you home. Any errands you may have had planned go undone. Medications you were supposed to pick up get put off until tomorrow.

When you are having trouble swallowing, you choke on your own saliva, and go into coughing spasms. I’ve never experienced trouble with deliberate swallowing, just with this swallowing you’re doing all the time without thinking, so you don’t drool.

I’ve never had slurred speech exactly, in the sense that I sound drunk, but what I get is a word-finding problem: I use the wrong word, and say something nonsensical, or something that doesn’t mean what I wanted to say. I remember very specifically one time when I reached for the word “shlep,” and grabbed the similar-sounding, but very different-meaning, “shtup.” If you don’t know Yiddish, I said “fuck” when I meant “bring.” And I vaguely recall another time when someone asked how I was, and my answer was something like “My dog forgot the carpet.” I don’t know what I meant to say.

Or I just can’t find any words at all, except very generic words, so there’s grammar and syntax, but no content to what I say: “I, umm, need the thing that I need to do what you do to umm, get, umm, what makes it do it….” the whole time running frantically around the labyrinth, which my brain that can expand at will, just to screw with me, has created. I grope for words that means anything close to what I want to say, and find only dark and grimy dead ends.

There are people, I’m sure, who are certain that I’m either bipolar, have multiple personalities, or am simply twins to whom they have not been properly introduced. In a sense, I am different people depending on how much sleep I’ve had– I don’t lose time, nor answer to different names, nor is one facet of me unaware of another, but the well-rested me, when she has a chance to appear, is brave, stalwart, able to fix anything, and would kill on Jeopardy. But with my luck, the jittery, weepy, 11-hours-of-sleep-in-a-week me, is the one Alex would hand the buzzer to.

These are also listed as potential symptoms:

[F]ever, rapid heart rate (tachycardia), high blood pressure (hypertension), increased sweating (hyperhidrosis), increased production of tears, constipation, variations in body temperature…. Anxiety and depression are common findings as well.

A lot of days, after cumulative sleep deprivation, I feel hot all the time, and get sweaty even in cool weather. My hands and feet will be like ice sometimes even while my face and torso are burning and sweaty. At random times during the day, my heart will race; this is a horrible feeling—sometimes it pounds my temples, sometimes it’s like a fish flopping in my chest. My blood pressure typically runs a little low– 110/60, or so– but in the middle of an insomnia bout, it climbs to 130/80. I clocked a record 151/92 once when I’d been up almost 48 hours. I’m not generally a crier, but when I’m in really severe sleep deprivation, I can be. In fact, weepiness is what people remark on most often as being out of character for me, so I guess that one comes only with the worst episodes. I get diarrhea more than constipation, but it’s probably a result of the same system being off-kilter.

When I’m having cold hands/hot face moment, you’ll catch me sitting like the poster for the movie Home Alone, with my hands against me face. (But with my mouth closed.)

And, of course, there’s the anxiety and depression. I have said before, and will say again, I think insomnia nearly always comes first. I see insomnia often listed as a symptom of depression, but I think the cause and effect are backwards. My mood is entirely reflective of how well I’m sleeping. If I happen to be sleeping well, I might be the happiest person in the room.

Fatal insomnia. If you Google “How long can a person live without sleep?” you get few definite answers. Apparently the Guinness-brokered record for going without sleep is 11 days, and the medical consensus is that this is about as long as a person can live without any sleep at all. After 11 days, a person is likely either to die in an accident while hallucinating, or from some kind of heart issue, because sleep deprivation causes tachycardia, arrhythmia and hypertension. Guinness won’t report attempts at breaking the record any longer for fear of people dying. However, Australia reports a medically monitored study by sleep scientists that lasted almost 19 days. Unlike the 11-day record holder, who claimed no ill effects, the 18+-day holder claimed hallucinations, paranoia, blurred vision, slurred speech and memory and concentration lapses.

The longest I’ve ever gone with no sleep whatsoever is about 48 hours. But I’ve gone a couple of weeks with sleeping about 4 hours a night. And I’ve gone months with sleeping in fits and starts—nights where I might get 6 hours a night, if I have 8 & ½ hours to spend in bed each night. But sleep comes in no more than 90 minutes intervals. Often intervals are less than an hour.

Fits and starts is how nearly all my nights go. I may be clocking 7 hours a night for weeks, with a few even longer nights, but they are all like this. Short naps, between which I lie there. If I lie there wide awake long enough, I just get up. I might do a load of laundry, clean the bathroom, take the dog for a walk, prepare a Hebrew lesson, take a shower, clean the fishtanks. Basically, kill an hour with a necessary task that I now don’t have to do during the day, and if I haven’t got one, I can play pinball, read a while, or cook something for later. Then I can spend another hour in bed, chasing capricious sleep. Maybe tonight I grab its heel.

Prion diseases on the whole are scary, but I’m a vegetarian, so Mad Cow disease doesn’t trouble me. Kuru is remote and foreign (as well as not on the vegetarian menu), and doesn’t trouble me. Creutzfeldt-Jakob Disease happens to people who have had medications made from harvested neural tissue; I’ve never had that, and am not troubled on that account either. Fatal insomnia I think about all the time.

Published by Chava Freya

Insomnia is a brain-based disorder I’ve had since I was at least 16 years old. Anti-anxiety medicine doesn’t help, except when there’s external anxiety exacerbating the problem. Sleep hygiene is irrelevant, because it’s not the problem, although I have submitted to it five different times, including having sleep specialists actually come to my home and advise me on rearranging furniture, buying special pillows, forbidding TVs in the bedroom, telling me the bed was for nothing but sleep, sex and reading, and when that didn’t work, then nothing but sleep. Period. That was the biggest failure of all.

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