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Can't sleep? Then don't read this horrifying article about KILLER insomnia
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Imagine one night, you prepare for bed, but you cannot sleep. It's the latest in a spat of insomnia that seems to be worsening. The sleepless nights turn into days, then weeks without sleep. Eventually, you lose the ability to sleep altogether. What's happening and why?
Highlights
CALIFORNIA NETWORK (https://www.youtube.com/c/californianetwork)
4/19/2016 (8 years ago)
Published in Health
Keywords: fatal familial insomnia, prion, disease
LOS ANGELES, CA (California Network) - If this is you, then you have the grave misfortune of being one of the few people on Earth to suffer from fatal familial insomnia (FFI). The disease, as the name suggests, is fatal. After a period between seven months to three years, your body will succumb to the lack of sleep and the atrophy in your brain, leading to severe dementia and death.
Fatal familial insomnia is one of the rarest diseases in the world and it is genetic, meaning that the people who get it often have an ancestor who had the disease before them. The disease is caused by a prion, that is a rogue protein that replicates itself in a damaged form. The FFI protein is folded, and it causes other proteins to fold as well. These folded proteins disrupt normal communication within the brain and creates holes in the brain tissue. The brain becomes sponge-life in appearance, similar to what happens with mad cow disease. FFI is a form of encephalitis.
The first recorded case of the disease was of an Italian man in Venice who died in 1756. The disease is found in his descendants, with each generation suffering a 50 percent risk. The disease does not manifest itself until middle age, which commonly results in infected people having children first, and those children can develop the disease.
There are only perhaps 40 families in the world with this genetic mutation and many who are aware they have this mutation refuse to have children.
It strikes on average in middle age, after children are born, with the mutation themselves.The disease is particularly fearsome because it can quickly debilitate an otherwise healthy person. The onset of symptoms is usually quick, and after a night or two without sleep, a person can cease to function in a reasonable capacity. The victim suffers hallucinations and panic attacks. Phobias and paranoia can seize them. This first stage lasts about four months on average, but can progress faster for some patients.
Hallucinations and panic attacks intensify after four months. The victim loses all ability to sleep and will remain awake for the next few months, although they will be in a state of pre-sleep from which they cannot awake. Weight loss is rapid during this phase. Finally, the patient can linger for six months or longer, suffering from worsening dementia and non-responsiveness until they die.
Victims quickly realize what is happening to them and why, and they are aware there is nothing they can do to alleviate their suffering or the suffering of those around them. They become entirely dependent on others for their day-to-day survival because they lose coordination. It's a slow and terrifying descent into death that everyone must agonizingly endure until the bitter end.
There is little doctors can do about the disease. Drugs which induce sleep have been found to worsen and accelerate the progression of the disease, and they don't induce sleep anyway, so they offer no relief.
It is hoped that future gene therapies and genetic testing will make this disease a thing of the past, but for dozens of people with the deadly mutation in their bodies, the clock is ticking on their inevitable, horrifying demise.
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