1Fatal Insomnia & the Psychology of Sleep

In this episode about a rare disease that plagues a single family line for centuries, Michael Drane, Unpopular Culture's resident psychotherapist dives into the Psychology of Sleep and REAL stories of people who suffered from Fatal Familial Insomnia. A guided, 60-second sleep meditation ends this podcast. Please tune in for the last three minutes of the show, ONLY if you are prepared to fall asleep, or in a safe and comfortable space.

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This is the VERY real story of a man who literally lost the ability to sleep and then died from it. When I tell it to people like that, their jaws are on the floor immediately. The amazing part is this Fatal insomnia is an extremely rare disease that has cursed a single family line for centuries. Well talk about what happens to your brain when you sleep, why our bodies even need sleep, and dive into the hellish existence of the eternal insomniac. 

Here's where it gets creepy, although this one family is cursed with a genetic form of this disease, there's also a version that spontaneously appears in people out of nowhere and could effect any of us. 



The Story of Silvano

Silvano moves through the dance floor with grace and style. He easily catches the attention of those around him. He is handsome, and immaculately dressed. He prides himself of his success in life and, in many ways, he is in the prime of his life. He's 53 years old. He travels on a luxury cruise ship, surrounded by friends, without a care in the world. While dancing, he looks down to discover he is drenched in sweat, as if someone had poured a bucket of water over his head...He doesn't feel right. He rushes to the nearest bathroom. He examines his shirt. As he is drying off his shirt with a towel, he happens to catch a glance up at himself in the mirror. That's when he notices the pupils of his eyes have shrunk into the size of 2 black pins. Thats when he realizes he knows he has the same look in his eyes that his father and sisters had before everything went to hell. 

Silvano sees his doctor as soon as he gets home. The doctor notices the dread on Silvano's face and attempts to put him at ease. "No need to worry!" The Doctor says cheerfully. "I haven't even taken a look at you yet! I'm sure everything is fine!"

"No" Silvano says as he stares at nothing "You don't understand. Within 8 or 9 months, I'll be dead".

In a small office Silvano and his doctor stand in close proximity. Silvano sits perched on the edge of an examination bed with a look of mindless and hopeless terror on his face. He has just told his doctor that he believes he will be dead in 9 months. "How could you possible know that?" the doctor asks skeptically. Silvano locates a piece of paper and begins drawing a diagram of his family tree going back to the 18th century. All the way back to a Venetian doctor who fell into a continuous, paralyzed stupor over 200 years ago. Soon after, a nephew named Giuseppe succumbed to a similar fate, and from there, the illness passed through his sons Angelo and Vincenzo to their children and great grandchildren, until it reached Silvano’s father Pietro, who died during World War Two. And finally Silvano. "It's my family's curse" Silvano explains. "My father had it. My sisters had it. And now their all dead. And I'm next." For centuries Silvano's family line kept their "family curse" a guarded secret. And largely thanks to Silvano, this curse has a name: Familial Fatal Insomnia.

As Silvano predicted, he wouldn't live long.  

Knowing his fate is sealed, Silvano offers himself up to science, and volunteers to spend the remainder of his days being observed and studied. His final wish, to help science find a cure for those that can still be saved. 

Silvano is watched for several 24 hour periods with great scrutiny under camera surveillance. As he deteriorates, the cameras capture his descent into the madness of a paranoid and sleepless hell, living in a kind of purgatory, neither asleep nor awake. Caregivers walk Silvano back and forth around the room, as he shuffles mindlessly, led by the arm, eyes dead and exhausted. In all that time, Silvano never slept, but incredibly, he did appear to dream. One day, researchers were astonished to see Silvano laying in bed,  performing simple gestures like combing his hair or buttoning his shirt, while in this dreamlike state. A researcher once saw Silvano give a salute gesture out of nowhere and apparently to nobody. When the researcher asked "What was that Silvano?"  Silvano replied, "I was dreaming that I was a god at the Coronation of Queen Elizabeth."

Medical Efforts could do nothing to save Silvano, extensive testing eventually found the transgressors known as Prions. Doctors are baffled to find that, For some reason it is only when a person hits middle age that the prions begin to ferociously infest the brain. These prions sit deep in the middle of the brain, Poisoning and damaging you from the inside.

In part due to Silvano's sacrifice, the scientists can now explain why damage to this small walnut of neural tissue unleashes such a perplexing mine field of symptoms. For example, the Thalamus, manages all our “autonomic” responses to the environment – things like temperature control, blood pressure, heart rate, and the release of hormones. When the Thalamus is bored open like swiss cheese, your body is thrown into chaos, which is why Silvano and all other victims early symptoms were profuse sweating and shrunken pupils and impotence.

The closest they get to normal sleep is a kind of mindless zombie mode – not quite asleep, but not quite aware, which is why they mindlessly play out ordinary activities like combing your hair, even while in a dream like state.



The Brain and Sleep

There's a lot we know about the brain and we're learning more all the time.

We know the Average human spends about 1/3 of their lives asleep.

We know that sleep is as important to us as food or oxygen.
The average adult requires 7-9 hours of sleep

Even though it only weighs 3 pounds, We KNOW The human brain uses about 25% of the energy our body requires every day.

 What we don't know is WHY we need sleep. If I were to ask you to take your best guess as to why we need sleep, what would say? Gun to your head, what's your best guess? You might say that we sleep so that we can rest right? That's a logical guess. But the neurons of the human brain are just as active when you're asleep as when you're awake.

Modern science now knows that when we dream, our brain acts out what we're doing in that dream as if we were doing it in real life. So when Silvano was dreaming he was saluting the queen of England, this is why he was acting out that salute in the real world. We have a kind of switch in our brain that disconnects our brain from our body, to prevent us from acting out our dreams. This switch doesn't always work, which is why sleepwalking is a thing.



Your Brain is HIGHLY active while you sleep

While you sleep, your brain is doing all of these things:

  1. Makes decisions.

  2. The researchers asked participants to categorize spoken words that were separated into different categories — words referring to animals or objects; and real words vs. fake words — and asked to indicate the category of the word they heard by pressing right or left buttons. When the task become automatic, the subjects were asked to continue but also told that they could fall asleep (they were lying in a dark room). When the subjects were asleep, the researchers began introducing new words from the same categories. Brain monitoring devices showed that even when the subjects were sleeping, their brains continued to prepare the motor function to create right and left responses based on the meaning of the words they heard.

  3. Organizes memories

  4. Some people think of sleeping doing to your brain what defragging does to a computer, organizing the events of your day, filing the important things into your long term memory and discarding the useless stuff, keeping the basic highlights of your day and boiling it down to the basic gist. Think about it, do you remember every moment to moment of what you did yesterday? But you can recall the basic events.

  5. Learn how to remember and perform physical tasks

What happens during REM sleep is that the brain transfers short-term memories stored in the motor cortex to the temporal lobe, where they become long-term memories.

One of my favorite experiments involves a snowboarding video game. Participants would get 1 practice attempt to get the best score possible when playing a snow boarding experiment. The participants are split into 2 groups: Group 1 got to try again later that day, while Group 2 got to sleep and try again in the morning. The results: Group 2's game performance had improved over group 1.

Sleep also: Clears out toxins, repairs and grows muscle and tissue, and makes creative connections. How many of us have had a moment of inspiration while dreaming?

I say all of these benefits of sleep to highlight the importance sleep for you and the devastation a lack of sleep can play on you. 



Now remember, there are 2 kinds of Fatal insomnia, there's the genetic version that mostly has ravaged this 1 particular family line, and then there's the kind of fatal insomnia that anyone of us can spontaneously acquire. Let's start with the kind that effected Silvano, Fatal Familial Insomnia 

  1. Fatal familial insomnia (FFI). (I'm gonna call this "FFI" for the rest of the show because I suck at saying words. So stay with me. Once again, Fatal Familial Insomnia = FFI. FFI It is almost always caused by a mutation of the Thalamus. (A part of your brain that I'll get into in a sec)

  2. FFI is EXTREMELY RARE, thought to have affected just 100 people on the planet. If 1 parent has the FFI gene, then each child has a 50% chance of inheriting it. And if right now you're signing up for Ancestry.com to see if your in this family, also know that FFI has a non genetic strain as well- a strain that will spontaneously appear in any of us- out of nowhere!

  3. This is called Sporadic fatal insomnia (sFI). And you, me, or any of us could obtain this affliction.

  4. Both are considered a prion disease. A prion disease is a mutated protein, sort of like how mutated skins cells replicate to become a tumor or a mole. These mutated proteins act like nasty little worms that burrow deep into your brain, leaving it looking like Swiss cheese.



What Happens to Your Body in the first week with this disease:

A 2010 Study from the International Journal of Occupational Medicine and Environmental Health.

After 24 hours.... The consequences of sleep deprivation are comparable to the cognitive impairment of someone with a blood-alcohol content of 0.10 percent, according to a 2010 study in the International Journal of Occupational Medicine and Environmental Health. “Judgment is affected, memory is impaired, there is deterioration in decision making, and a decline in hand-eye coordination,” Cralle says. “You're more emotional, attention is decreased, hearing is impaired, and there is an increase in your risk of death from a fatal accident.”

After 36 Hours... Now your health begins to be at risk. High levels of inflammatory markers are in the bloodstream, said Cralle, which can eventually lead to cardiovascular disease and high blood pressure. Additionally, hormones are affected — your emotions can be all over the place. You can go through entire events and not remember where you just were or what you just did.

After 48 hours... Cralle says, the body begins compensating by shutting down for microsleeps, episodes that last from half a second to half a minute and are usually followed by a period of disorientation. “The person experiencing a microsleep falls asleep regardless of the activity they are engaged in,” she says. This could include driving, walking across the streeet, anything, You will involuntarily pass out for a brief terrifying moment. Microsleeps are similar to blackouts, and a person experiencing them is not consciously aware of what's occurring.

At 72 Hours... Expect significant deficits in concentration, motivation, perception, and other higher mental processes after many sleepless hours, Cralle says. “Even simple conversations can be a chore. The mind begins seeing objects that don't really exist..objects that aren't really there" He even bought a sensory deprivation tank, An egg-shaped chamber filled with warm salt water, which would make him buoyant, and making him feelings as if he were floating through space. This method would sometimes provide him with a euphoric 4.5 hours of sleep




The Story of Elliott (Anonymous)

One remarkable patient, however, has hinted that there may be some unusual ways to alleviate the misery... I'm going to tell you a story about a real man, who chooses to remain anonymous, but for the purposes of this podcast, we'll call Elliott.

His case was described in The Medical Journal Mescape General Medicine. His story is awesome because he valiantly tried to beat the disease, discovering ways to induce sleep for short amounts of time.

When Elliot was a kid, he remembered his mom talking about some illness in his father’s family, but had decided not to worry her son with the details. Now in his 50's, Elliot had started to experience extreme bouts of insomnia. Genetic testing revealed that he had what his father, paternal uncle, and 2 male cousins died of.... Fatal Insomnia.

Alarmed that he is rapidly deteriorating, Elliot decides he will not give up without a fight. His response to this diagnosis is to buy a motorhome and travel across the US - he wasn’t just going to sit there and die. Elliot sets out on the adventure of his life, open road and open possibility. As he drives he considers his battle strategy to fight his impending demise.

Elliot has been on the road for 5 months and his symptoms are becoming more extreme. He often finds himself too sick an incapacitated to drive himself, so Elliot employs a driver to take over the steering wheel and a nurse to look after him when he gets too sick. He continues to drive, but only after he manages to get enough sleep. Elliot is determined to try as many potential treatments as possible,he tries everything he can think of, from vitamin supplements and exercise to anesthetics such as ketamine and nitrous oxide, and any sleep medicine he can find- He even tried electroconvulsive therapy to see if the sharp electric shock could knock him out. Some of these treatments offer him as long as 15 precious moments of uninterrupted sleep.He is thin and weak.. his mind is ripe with hallucinations and delusions. He lays in the back of the RV while it rocks back and forth against the road. His temperature spikes around 102 degrees and he has cardiac arrhythmia. His nurse checks his blood pressure, as his driver carries him onward.

15 months into his diagnosis and vitamins are no longer helping. After another insomnia bout of 5 consecutive days awake. Elliot is irritable and delusional. An evaluation at the Massachusetts General Hospital in Boston, found that he had suffered a minor stroke; he was anesthetized until he fell asleep. While hospitalized, he slept for 3 consecutive days and was fully alert and refreshed afterward. He feels ok enough to continue his roadtrip. Elliot has already survived longer than anyone with FFI ever has.

Despite these (relative) successes, Elliot experienced regular relapses that became more intense and the disease progressed. His friend said “When the symptoms reared themselves, he couldn’t do anything, There were times when he lost the whole day – it takes over your consciousness. He could sit there without the initiative to move; he’d be frozen in time.”. After a few years of this struggle, like those before him, he too finally passed away.




Over the centuries victims of FFI have not had the ability to know if they had the disease until it was too late. And even though there is still no cure, thanks to modern medicine, some family members now have the horrifying choice of being genetically tested to see if they have it. With a 50 percent chance of finding out if you were going to die of insanity and lack of sleep. With chances no better than a coin toss. How long have you stayed up on your own before? Have you even come close to truly feeling what this is actually like?

If you've been listening, you'll know this is dangerous. Maureen Weston holds the Guinness world record for longest amount of time with no sleep (intentionally) with 449 hours (18 days, 17 hours), followed by Randy Garner at 264 hours (11 days).

As far as FFI is concerned, Some family members have chosen to find out, while others have refused, worried it would taint what time they did have left. Which would you choose?