Fatal Familial Insomnia: The True Story of The Man Who Never Slept

 

Fatal Familial Insomnia

The True Story of The Man Who Never Slept

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Nov 1, 2007 Rebecca Turner

Fatal Familial Insomnia, or FFI, is a devastating genetic sleep disorder, which strikes during middle age and results in death. This is the story of Michael Corke.

Fatal Familial Insomnia is a rare curse that affects about 40 families around the world. It is caused by prion diseases (mutated proteins) which can affect both humans and animals. Here is one story of Fatal Familial Insomnia, or FFI.

Michael Corke (Michel A Corke)

Shortly after his 40th birthday in 1991, Michael Corke, a music teacher from Chicago, began having trouble sleeping. In the following weeks, the insomnia grew worse and his health rapidly deteriorated. Eventually he couldn’t sleep at all.

The doctors were baffled but could do nothing for him. Michael was physically and mentally exhausted, and wanted nothing more than to be able to fall asleep. But his brain wouldn’t let him.

Video footage of him appearing at a school orchestra concert revealed a frail old man - a far cry from the fit and healthy individual he was just months earlier. Eventually he was admitted to hospital and doctors diagnosed him with an extremely rare genetic disorder discovered just seven years prior: Fatal Familial Insomnia (FFI).

Michael Corke died in hospital after six months from a complete lack of sleep.

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Symptoms of Fatal Familial Insomnia

The FFI sleep disorder is so rare that only 40 families worldwide have been identified as carrying the defective gene. If only one parent has the gene, the offspring has a 50% chance of inheriting and developing the disease.

Fatal Familial Insomnia strikes between the ages of 30 and 60 years, with no apparent trigger that the sufferer can relate to. Patients have been known to survive for up to three years, gradually passing through four stages of illness:

1. The onset of insomnia, creating panic attacks and unfounded phobias, lasting for four months.
2. Severe insomnia, worsening panic attacks and hallucinations, lasting for five months.
3. Complete insomnia and rapid weight loss, lasting for three months.
4. Dementia and unresponsiveness, lasting for six months. FFI is eventually fatal.

Causes of Fatal Familial Insomnia

Ten years ago, scientists discovered that Fatal Familial Insomnia is caused by a dual mutation in a gene that codes for proteins. The tell-tale sign of prion diseases is an insoluble protein that causes plaque to develop in the thalamus. This is the region of the brain responsible for the regulation of sleep, as well as sensory and motor systems.

As the plaque "eats away" at the brain, the sufferer loses the ability to shut down at night. This manifests in the form of insomnia. The resulting symptoms of FFI are caused by the complete lack of REM and NREM sleep, proving that sleep is vital to everyone.

Treatments for Fatal Familial Insomnia

Because of the nature of prion diseases, Fatal Familial Insomnia has been linked with other gene disorders like Bovine Spongiform Encephalopathy (BSE – or mad cow disease) as well as wasting diseases in deer and elk. It is also linked to Kuru (the laughing disease).

There is currently no known cure for FFI. This prion disease is still shrouded in mystery, and sleeping pills only worsen the symptoms. However, there is hope that one day, Fatal Familial Insomnia will be controlled through gene therapy.

Further Reading

If you found Fatal Familial Insomnia interesting, you may also like:

Fatal Familial Insomnia: A History of FFI

The Effects of Sleep Deprivation

Fatal Familial Insomnia: The True Story of The Man Who Never Slept

Roger Waters gives 'The Wall' a new, 'more political' edge for tour - USATODAY.com

 

Roger Waters gives 'The Wall' a new, 'more political' edge for tour

Updated 4/12/2010 8:32 PM | Comments 13 | Recommend 5
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By Jeff Christensen, AP

Roger Waters is asking relatives of war victims to submit photos on his website to be projected in arenas during his tour.

By Elysa Gardner, USA TODAY

NEW YORK — Roger Waters is rebuilding The Wall— and he wants fans to help.

The British rock veteran will launch a North American tour this fall — kicking off Sept. 15 in Toronto, with shows in the USA scheduled through Dec. 13 — revisiting the epic concept album that he introduced in 1979 as principal songwriter of Pink Floyd.

It has been 30 years since that group launched a string of visually ambitious live performances of the songs and 20 since Waters performed them solo in Berlin.

After showcasing another Floyd classic, The Dark Side of the Moon, on a 2006 trek, Waters "realized that the story in The Wall— of a young man who is alienated and defensive, because he's fearful — could be an allegory for a more universal story. We're all frightened of each other, and that makes us behave in ways that are sadly inhumane, like engaging in wars. I wanted to make this show more political."

To do that, Waters, 66, is posting an appeal on roger-waters.com to relatives of those lost in war. He's asking for their photographs to project onto arena surfaces representing the title's metaphorical wall — so that each victim becomes, in effect, another brick.

"I get slight shivers talking about this," says Waters, who lost his father in World War II. "I want people to remember their loved ones as I remember mine, as part of a show that has a strongly anti-war message."

Rolling Stone contributing editor Anthony DeCurtis doesn't expect Waters' approach will prove too controversial. The Wall's songs have always had "a generalized anti-war message," he says. "People are going to go for the spectacle."

They shouldn't be disappointed. Waters says video technology allows for projections twice the size of those in the original Wall dates, in which a huge wall was constructed between the band and the audience. Album illustrator Gerald Scarfe, who worked on the tour and 1982 film, is providing new art and redesigning puppets and inflatable objects.

Waters also is developing The Wall into a stage musical with Billy Elliot librettist Lee Hall. He doesn't plan to collaborate again with surviving Floyd members Nick Mason and David Gilmour, despite fond memories of the band's reunion at Live 8 in 2005.

"But Nick and I have become great friends again," he says. "We had a bad 15 years, so to come back together is fantastic."

Roger Waters gives 'The Wall' a new, 'more political' edge for tour - USATODAY.com

The 4th Kind (Movie)

"The Fourth Kind" Movie Poster (Image: Universal Pictures)

 "The Fourth Kind" is an alien abduction film 'somewhat' based on real life events, with an out-of-this world twist. The story, set in Nome, Alaska, is loosely based on the mysterious disappearances of 24 people in the town. The FBI did investigate the disappearances, which took place between the 1960s and 2004, and concluded in 2006 that "alcohol" was  common factor in most of the disappearances. Before the FBI concluded the investigation, it was believed that the deaths and disappearances were the work of a local serial killer.

The movie is based on the theory that the missing-persons cases were actually alien abductions. "The Fourth Kind" hit theaters on November 6, 2009 and featured "archival footage" of the "most disturbing evidence of alien abduction ever documented." The 'footage' is that of hypnotherapy sessions between a "Dr. Abigail Tyler," played by Milla Jovovich, and patients who claimed they were abducted. The aliens in the movie spoke the long-lost Sumerian language.

True Story: The FBI and Missing Villagers in Nome Alaska

In 2005, the FBI sent homicide detectives to investigate a series of unsolved disappearances and deaths in Nome, Alaska. Most of the victims were Native villagers. Between the 1960s and 2004, over 20 people mysteriously died, or vanished. In 2006, the FBI concluded that "excessive alcohol consumption and a harsh winter climate" were to blame for the disappearances.

Dr. Abigail Tyler and the "Alaska Psychiatry Journal"

In the movie "The Fourth Kind," Milla Jovovich plays Dr. Abigail Tyler, the Nome, Alaska, psychiatrist who stumbles upon the 'alien abduction' link between her patients, during clinical hypnotherapy sessions. If you search for Dr. Abigail Tyler, Nome Alaska, a website called "Alaska Psychiatry Journal" provides a "biography" of Dr. Tyler with "related articles" on the topics of sleep disorders, emotional issues, hypnotherapy and regression therapy. However, the website does not have a homepage or contact information. The website was registered on GoDaddy in August 2009. A real online-medical publication would have such information, so this leads to the conclusion that the website is a viral marketing ploy, much like the promotion for the upcoming "2012" movie and the  for "Institute for Human Continuity." Sorry to burst your bubble, but this doesn't rule out that Dr. Tyler 'could' have been based on a 'real' doctor; but if there were, the true account would have made for a much more interesting find.

Overall, I love a good horror/sci-fi flick whether it is true or not. This one looks promising. Check out the trailer below.