doing some additional reading about prions...
i'm fascinated by the idea of a self-replicating nonliving object. as malleable and transformable as the name (protein/protean) suggests, a prion is nothing more than a malformed or mutated protein. yet, unlike a hot dog or a nice omelet, these agents remain communicable for decades, cannot be killed with formaldehyde, resist disinfection via bleach, and can be hereditary, infectious like a virus, or, famously, contracted via food like a bacteria or a poison.

despite this remarkable adaptability in propagation and expression, they're not even as 'alive' as a relic bit of DNA/RNA like a virus. consider this: if you acquired a condition like mad cow/Creutzfeldt-Jakob disease, Kuru or fatal familial insomnia, you would be suffering from gradual brain death caused by an abnormally folded protein that turned your brain tissue into a something like a honeycomb, almost cancerously remade itself, and was nearly impossible to denature, even out in the open, exposed to air, or rinsed in disinfectants, much less inside your brain.

FFI, the prion disease that most enthralls and horrifies me, is my kind of worst-nightmare medical condition. the condition is hereditary, expressing itself around early middle age. this means you have plenty of time to pass it on to your offspring before you ever figure out if you are a sufferer or carrier yourself. around this time, your body's self-regulating mechanisms begin to fail. you sweat profusely, suffer from paranoia, you lose your ability to sleep, and begin to suffer panic attacks as you remain awake day after day, and eventually simply fail ever to fall asleep again. towards the end, speech fails you, and gradually, you descend into madness as your body is racked by convulsions and hallucinations before you finally die (generally three to five years after having first begun to detect symptoms in the first place.)

can you just imagine how lonely that must be? even quadriplegics can sleep... and you can forget about alcohol, ambien, or benadryl. apparently opiates and other sleep drops may make the condition worse, leaving sufferers delirious but unable to lose consciousness, plagued by disorientation, completely vulnerable.

as a lifelong insomniac, i know there are few reliable cures for sleeplessness. my parents tell me that when i was a child, they were forced to put up fences to stop me tumbling down the long stairs that led down to our living room, as even toddler-'nessa apparently paced all night long. i remember many how many nights i spent huddled out on the low roof of our back den, rolled in a comforter, smoking, staring blearily up at the stars, as the sun rose to my right and picked the night threadbare, listening to the trains.

at least i knew this was a condition that went into remission pretty regularly. even now, twice the age i was in those days, i know i won't go for more than a week or so on three or four hours' sleep a night before i fall over and lie comatose for seven or eight hours. imagine if it only ever grew worse, day by day. it's lonelier than hell keeping watch at four a.m, but what kind of agony would a person experience after weeks, months or even years without sleep?



Sidebar:
so, i'm working for this pharmaceutical account, marketing a drug called NUVIGIL, which is indicated to treat excessive sleepiness due to apnea, shift work or narcolepsy. the idea is that you take the pill in the morning and experience 'improved wakefulness' throughout whatever your daytime or work period is. having read and reread the Important Safety Information more than just one or two dozen times, i must report that there are a number of truly worrying 'side effects' covered therein. one potential consequence of taking this stuff is merely the potential of erupting in a rash described as toxic epidermal necrolysis (that's poisonous skin death, in case you're feeling excessively sleepy.) another, far more chilling side effect, is disclaimed in a line i'll quote directly from the website's prescription information:
Persistent Sleepiness
Patients with abnormal levels of sleepiness who take NUVIGIL should be advised that their level of wakefulness may not return to normal.
Can you imagine the overwhelming sense of irony you'd experience if you took a medication to prevent you from falling asleep at work, only to find you couldn't manage to do it anywhere else, ever?