In the past, this debilitating disease, that stops people from sleeping, starts in one family member and then the ugly disease surfaces in other family members. Fatal Familial Insomnia or FFI is as bad as the name suggests and it’s a brain disease that occurs when sufferers get a build-up of infectious proteins called prions in the brain and as this gradually stops the patients from sleeping, it kills them within a year.
Eric Vallabh Minikel likes to call the disease – Prion Disease. Eric’s wife tested positive when tested for the mutation that causes the genetic prion disease, after her Mother died from the disease. Since 2012, Eric and his wife have been re-trained as scientists and are working on a cure at Harvard Medical School in Boston, United States. http://www.cureffi.org/2012/12/03/introduction-to-fatal-familial-insomnia/
Eric stated in his blog, that from the first symptoms to appear, to death, is usually around a year and the average age of onset is around 50.
“As far as we know, everyone who has the mutation eventually gets FFI and dies of it. There’s a lot of variance, with cases having been reported at ages ranging from 20 to 72.
“Our goal at Prion Alliance is to catalyse that science through fundraising to support specific research goals, and our goal at CureFFI.org is to collect the scientific knowledge and promote discussion and discovery. If this sounds awesome to you, get involved,” Eric said.
Rebecca Turner, who is the author of a blog, World of Lucid Dreaming, said the genetic disease has been diagnosed in less than 40 families worldwide. She also wrote about the stages of the disease:
The disease manifests itself in four deteriorating stages:
- STAGE ONE – The sudden and inexplicable onset of insomnia causes panic attacks and unfounded phobias, lasting for about four months.
- STAGE TWO – As sleep deprivation takes hold, the panic attacks and hallucinations become severe, lasting for a further five months.
- STAGE THREE – The total insomnia causes rapid weight loss and limited mental functioning, lasting for up to three months.
- STAGE FOUR – Finally, the patient suffers from dementia and unresponsiveness, lasting for up to six months, before slipping into a coma and dying.
There are sufferers in different countries and in Australia, Queensland siblings Hayley and Lachlan Webb, lost their Mum to the disease and they also carry the gene and are on their mission to find a cure. You can make a donation to Eric Minikel and Sonia Vallabh’s research via: http://www.prionalliance.org/donate/ #60Mins