Saturday, September 21, 2013

Stages & Effects

There are four stages of Fatal Familial disorder (FFI):

Stage One: (typically 4 months): the 1st stage includes sudden onset of sleepiness and insomnia resulting in phobias, paranoia and panic attacks.

Stage Two: (typically 5 months): During the 2nd stage hallucinations and panic attacks become increasingly apparent.

Stage Three: (typically 3 months or more): This stage includes the absolute inability to sleep along with rapid weight loss and diminished cognitive performance.

Stage Four: (Typically lasts for 6 months): The end stage includes ‘dementia’. The patient becomes mute and unresponsive over the course of 6 months after which death occurs.


Writing in a 2006 issue of the Medscape General Medicine journal, Dr. Joyce Schenkein outlined the etiology and characteristics of FFI. She noted that it often begins in middle age (average age of onset being 50 years) and has no cure (even ‘gene therapy has been unsuccessful to date). Unfortunately, the prognosis following initial diagnosis is poor with FFI sufferers’ only living for an average of about a year and a half (with Dr. Schenkein noting that survival ranged from 7 to 36 months from diagnosis of FFI). It originates in the form of unexplained sleeplessness before rapidly developing into a fatal insomnia. 

Further Reading:
Schenkein, J. (2006). Self-management of fatal familial insomnia. Part 1: What Is FFI? Medscape General Medicine, 8(3), 65.
Schenkein, J. & Montagna, P (2006). Self-management of fatal familial insomnia. Part 2: Case report. Medscape General Medicine, 8(3), 66.

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