Found in just 28 families worldwide, Fatal Familial Insomnia (FFI) is a hereditary condition caused by an abnormality in the Prion Protein (PrP) which causes the protein to mutate into a new form called PRPsc. PRPsc is insoluble and builds up plaques in the Thalamus, the part of the brain responsible for regulating sleep.
A person with a single parent suffering from FFI has a 50% chance of developing the disease.
Symptoms typically begin around the age of 50 with increasing bouts of insomnia culminating in complete sleeplessness which is ultimately fatal within 36 months of onset.
FFI occurs in 4 stages.
- Increased insomnia (causing panic attacks, phobias and paranoia)
- Increasing panic attacks and psychotic behavior
- Complete inability to sleep (causing rapid weight loss)
- Dementia (leading to death)
There is no cure or treatment for FFI. Sleeping pills do nothing to relieve the symptoms. Gene therapy seems to be the most promising research avenue to-date.
Due to the horrific course of the disease and it's 100% fatality rate I give this disease a
9 on my "lethality scale"
and a 5 on my "disturbing scale"
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