Fatal Familial Insomnia: Bizarre Death Sentence
Not even the Guinness World Records for voluntary sleep deprivation compares with what fatal familial insomnia sufferers experience
.
Fatal familial insomnia demonstrates what it's like to be extremely sleep deprived. Victims of fatal familial insomnia go on for months with totally no sleep which ultimately ends with death.
Extreme sleep deprivation
Fatal familial insomnia (FFI) victims go through the state of extreme sleep deprivation once the disease triggers. Some of its effects may change from patient to patient, these are:
Increased insomnia
Muscle twitching or spasms
Hallucinations
Blurry vision
Dizziness
Paranoia
Dementia
Fatal familial insomnia uncovered
Bizarre deaths in a particular Italian family lead to the discovery of this disease. Fatal familial insomnia has been discovered in the early 90's. Dr. Ignazio Roiter, a specialist in internal medicine and endocrinology at the Treviso Hospital married into a family carrying this rare disease. His wife has been experiencing the loss of relatives approximately every 3 years. He then sought to uncover more information about his wife's family and traced the bizarre deaths as far back as the 1700's. What marked these relatives were periods of insomnia and symptoms due to months of no sleep.
Fatal familial insomnia explained
FFI is an extremely rare genetic prion disease of the brain which 40 families as of 2006 suffer from. Each family member has a 50/50 chance of inheriting this disease. This disease usually triggers when a gene carrier hits their 50's. When it does, mutated proteins called prions start to accumulate around the victim's thalamus. This is the part of the brain which regulates sleep among other functions.
Some experts contend that it is not insomnia that kills the patients. It is these proteins that continue to reproduce that eventually kills them.
FFI is a member of the prion family of diseases. Some of the other prion diseases are:
Madcow disease
Kuru
Scrapie
Gerstmann-Strussler-Scheinker
Creutzfeldt-Jakob disease (CJD)
Recent cases
In 2006, 40 families are documented to be suffering from this rare genetic disease. This means that there could still be more families affected all over the world. Due to the fact that FFI has only been recently discovered, not a lot of people or doctors know about this disease. In a more recent case, a woman was in her deathbed when she was diagnosed to have FFI shortly before she passed away. Her children have mixed emotions but they at least know what they are facing. They can suffer the same fate; even their children aren't completely safe.
Search for a cure
Researchers are relentless in their search of a cure for fatal familial insomnia and prion diseases. So far, gene therapy was tried and did not work. Affected families also hope that a cure for FFI will be found. At the moment, the only thing doctors can do for sufferers of FFI is to improve their quality of life.
Fatal familial insomnia shows everyone how extreme sleep deprivation can debilitate a person. Some people are lucky being able to live with insomnia. FFI victims cannot say the same.
Not even the Guinness World Records for voluntary sleep deprivation compares with what fatal familial insomnia sufferers experience. Fatal familial insomnia demonstrates what it's like to be extremely sleep deprived. Victims of fatal familial insomnia go on for months with totally no sleep which ultimately ends with death.
Extreme sleep deprivation
Fatal familial insomnia (FFI) victims go through the state of extreme sleep deprivation once the disease triggers. Some of its effects may change from patient to patient, these are:
Increased insomnia
Muscle twitching or spasms
Hallucinations
Blurry vision
Dizziness
Paranoia
Dementia
Fatal familial insomnia uncovered
Bizarre deaths in a particular Italian family lead to the discovery of this disease. Fatal familial insomnia has been discovered in the early 90's. Dr. Ignazio Roiter, a specialist in internal medicine and endocrinology at the Treviso Hospital married into a family carrying this rare disease. His wife has been experiencing the loss of relatives approximately every 3 years. He then sought to uncover more information about his wife's family and traced the bizarre deaths as far back as the 1700's. What marked these relatives were periods of insomnia and symptoms due to months of no sleep.
Fatal familial insomnia explained
FFI is an extremely rare genetic prion disease of the brain which 40 families as of 2006 suffer from. Each family member has a 50/50 chance of inheriting this disease. This disease usually triggers when a gene carrier hits their 50's. When it does, mutated proteins called prions start to accumulate around the victim's thalamus. This is the part of the brain which regulates sleep among other functions.
Some experts contend that it is not insomnia that kills the patients. It is these proteins that continue to reproduce that eventually kills them.
FFI is a member of the prion family of diseases. Some of the other prion diseases are:
Madcow disease
Kuru
Scrapie
Gerstmann-Strussler-Scheinker
Creutzfeldt-Jakob disease (CJD)
Recent cases
In 2006, 40 families are documented to be suffering from this rare genetic disease. This means that there could still be more families affected all over the world. Due to the fact that FFI has only been recently discovered, not a lot of people or doctors know about this disease. In a more recent case, a woman was in her deathbed when she was diagnosed to have FFI shortly before she passed away. Her children have mixed emotions but they at least know what they are facing. They can suffer the same fate; even their children aren't completely safe.
Search for a cure
Researchers are relentless in their search of a cure for fatal familial insomnia and prion diseases. So far, gene therapy was tried and did not work. Affected families also hope that a cure for FFI will be found. At the moment, the only thing doctors can do for sufferers of FFI is to improve their quality of life.
Fatal familial insomnia shows everyone how extreme sleep deprivation can debilitate a person. Some people are lucky being able to live with insomnia. FFI victims cannot say the same. You can know more about this rare disease at
http://insomniarevealed.com/fatal-familial-insomnia/. Get more information about insomnia symptoms, causes and cures from
Insomnia Revealed.
Fatal Familial Insomnia: Bizarre Death Sentence
By: Sally Stephenson
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