Thursday, April 18, 2019

Fatal Familial Insomnia "Trip Report"

While in one of my weird internet rabbit holes, I stumbled upon the single most interesting & chilling medical study ever.  But first, some background.

Fatal Familial Insomnia story

Fatal insomnia is an exceedingly rare disease that occurs when a mis-folded protein called a prion causes other proteins in the sleep center of the brain (the thalamus) to self-destruct, becoming filled with holes like Swiss cheese.  This results in days, weeks & finally months of sleeplessness until the affected individual eventually dies a horrific death from a combination of brain degeneration & the secondary effects of sleeplessness.  Though it appears death is primarily caused by the physical degeneration of the brain, as other cases of long-term total insomnia have been reported without the patient dying (see:  Thai Ngoc).

Like all prion diseases, Fatal Insomnia is 100% fatal with no known treatments or cures.  Most cases are inherited ("Fatal Familial Insomnia") while a tiny portion happen for unknown reasons ("Sporadic Fatal Insomnia").  I stress again for all you hypochondriacs out there:  this is an exceedingly rare condition, with the sporadic form affecting about 24 people worldwide ever.

Afflicted FFI sufferer known only as "Silvano" who offered himself for recording in the 1980s

While Googling this horrific condition, I came upon a link to a study in which a man with the inherited form of the disease self-treats his condition with a vast array of supplements, stimulants, tranquilizers & anesthetics...some exceedingly rare or even obsolete in America.  This is hands-down the most bizarre & unconventional case study I've ever read in more ways than one.  The case took place in the U.S. in 2001 & was conducted by doctors Schenkein and Montagna.

Here is an excerpt from the study:

"Shortly after his diagnosis, patient "DF" received vitamin therapy at the Clymer Institute for Alternative Health in Quakertown, Pennsylvania.   DF took a combination of niacin, antioxidant powder (comprising vitamins A, C, E, and D), blue-green algae, brewer's yeast, B complex, zinc (sublingually), magnesium, inositol, PABA, grape seed extract, CoQ-10, cholineTiger's Milk nutrition bar, highly concentrated wheat germ oil, tryptophan, and 2 g of melatonin. He also injected a gelatinous form of ± 2 into his nasal cavity.  Within 30 minutes of this treatment, DF reported that he fell into a natural and restful sleep. On this regimen, he experienced 5 out of 6 consecutive nights of sleep, ranging from 5 to 6.5 hours per night." 
However, this regimen only worked for so long before the patient became restless & delusional.  He even suffered a stroke.  Time to up the ante with some anesthetics then:
"By month 15, vitamins alone failed to induce sleep. Following 5 consecutive nights of insomnia, DF became intensely irritable and delusional. An evaluation at the Massachusetts General Hospital in Boston, Massachusetts, found that he had suffered a minor stroke; he was anesthetized until he fell asleep.  In later months, DF continued the strategy of anesthesia. Ketamine and nitrous oxide induced short (15-minute) periods of restful sleep, and were reapplied to offer more prolonged relief. Chloral hydrate in a light alcohol mix and/or chloroform also worked."
Intriguing.  Around this time he also added a hefty load of tranquilizers, including the obsolete ethchlorvynol (Placidyl) as well as diazepam & zolpidem to his regimen.  He claimed that the ethchlorvynol doubled as a "mood elevator".  I'll bet.  😁
His condition continued to deteriorate severely despite these valiant interventions.  By month 16 he decided it was time to try some stimulants & brought out the big guns...  
"By the 16th month of his illness, DF spent much of the day as an akinetic mute with terrible headaches, confusion, mood swings, and myoclonus of the left arm (treated with levodopa). Despite his outward “dementia,” he inwardly pondered approaches to his condition, and, when again able to speak, he requested a regimen of stimulants."


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Brain scans of Fatal Insomnia sufferer
"He was prescribed phentermine HCl 37.5 mg (time-release), which he took twice daily and eventually 3 times daily (before meals and no later than 6:00 pm). The drug had immediate and dramatic effects, promoting not only alertness during the day, but apparently a sleep-inducing rebound when it wore off.  For 4 consecutive nights, DF slept 5 hours each night, was able to drive, and returned to his daily routines, which included writing a novel."
"During his last months, DF received a new stimulant protocol and he took these on a daily rotating basis: diethylpropion + propoxyphene-acetaminophen (for headaches), methylphenidate, phentermine, modafinil, and a benzedrine inhaler (which is illegal in the United States, but which he was nevertheless able to obtain). Benzedrine enabled him to successfully drive 500 miles alone (on several occasions) during the 24th month of his illness. DF believed that the diethylpropion + propoxyphene-acetaminophen combination restored his function to that of a far earlier stage of the disease....In the 26th month, DF began taking time-released benzphetamine (90 mg) and a small amount of methylphenidate to reduce the stress on cardiovascular and renal systems."
Other therapies tried included electroconvulsive therapy, exercise, sensory deprivation, GHB & meditation.  Ultimately, the patient ended up outliving others with the same genetic variant of the disease by an entire year, though it's not known which treatments were responsible due to the total lack of scientific rigor in the experiment.  The fact that he was able to communicate, drive (bad choice!) and write a book as his brain was being ravaged by this disease has to be a first in the annals of medicine.  Most sufferers are rendered unable to speak or function at all shortly after the disease sets in.


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But perhaps the most chilling part of the study is the description of what this man experienced while approaching sleep.  I've never seen anything like it in a scientific study or medical journal, as it definitely borders on the spiritual/supernatural.  Keep in mind, this is a disease that eats away at the brain's physical structures & usually renders patients totally unable to communicate.  From the outside, they look a bit like zombies in great physical distress due to the tortuous lack of sleep.  
The section is titled simply "Serenity of FFI" {Emphasis mine}:
Unlike the typically mute FFI patient whose subjective serenity is unknowable, DF described his oneiric sleep as extremely gentle and pleasant — like entering a room filled with everyone who he would want to encounter, including deceased friends and relatives who would tell him that everything will be all right. In his words, “to the outside world, I am dead and gone, but to myself I'm still here, in this wonderful place and it is they who have disappeared.”
His “waking REM” was multisensory and included images, voices, and scents. It was experienced as a form of knowing everything about himself, with no more hidden secrets. As might be expected from a sustained “handshake” between the right and left hemispheres, DF's conscious mind experienced himself in a global way. He described his unconscious as filled with “wounded children” who bore “poor witness” to events that had injured them — unable to logically evaluate or rise above these damaging experiences. His FFI put him in the unique position to soothe these children with adult insight, which he often did in the form of written letters when he was “off-line.” 
The door that admitted DF into this other world became best defined after long periods of insomnia and was so inviting that he believed others who have been in this place simply gave into it and allowed themselves to die. In fact, DF's fight against FFI specifically centered on this arena, with the wish to surrender to its serenity as opposed to his real life of handicap and degeneration.
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Holy hell.  That's some insight you don't hear every day.  Sort of like being stuck in a near-death experience for months & being able to come back to describe it in great detail over & over.  I've read hundreds of psychedelic trip reports & nothing comes close to the spine-tingling creepiness of this account.  Excuse the pun, but this man did NOT take his death lying down.

While I heavily disagree with his decision to drive a motor vehicle in his impaired state, I give him major respect for fighting this disease to the bitter end & providing scientists with this treasure trove of intriguing data, not just about FFI but about the dying process as well.  Maybe this is what other dementia sufferers experience to a degree?  Many Alzheimer's patients speak of going "home" & claim to see dead relatives in their rooms shortly before dying, so perhaps there is at least some degree of serenity & hope in the dying process with dementia.  

It does raise some questions though.  Where did he get the Placidyl & Benzedrine inhalers from?  And what doctors knowingly let him use these illicit substances without a prescription while agreeing to do a study on him?  I understand he was dying anyway, but no scientific protocol was followed whatsoever and some of the substances administered were extremely rare (chloroform), discontinued for many decades (Placidyl & Benzedrine) or were administered in a way that could've been dangerous (i.e. injected directly into the nasal cavity).  And that's a metric buttload of drugs, maan.

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Benzedrine inhaler, discontinued in 1959
Most importantly:  WHO in the bloody hell cleared this guy to drive a car while dying of a dementia that impairs the ability to think, stay alert & react to stimuli?  And so late into his disease with no sober sitter present?  It's a doctor's job to report dementia patients to the DMV if their families don't take away their vehicles to ensure they don't cause a wreck (I know; I had to report someone myself for a similar reason) so this is extremely troubling.  Under no theory should someone in this state be driving, period.  

Despite all this, I give this report a 10/10 for its insanity & insight.  I hope you other medical nerds enjoyed reading it as much as I did.  Maybe it'll make death slightly less scary for those of us who don't have to face something as agonizing as Fatal Insomnia.  Or any other disease with the word "fatal" in the name.  

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Sweet dreams.




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