Monday, April 26, 2010

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

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