Have you ever dealt with insomnia that was so bad that you thought your lack of sleep would eventually kill you? While you’re probably going to be all right once you get your sleep schedule back on track, there is a very small number of people in the world whose genetic insomnia will actually kill them.
These people have an illness called Fatal Familial Insomnia (FFI). This inherited disorder, which has affected around 28 families worldwide, affects a region in the brain called the thalamus, which affects things like sleep and body temperature. People who inherit the disease develop an inability to sleep that gets worse over time. In addition, they start to develop severe dementia and trouble with coordination. They may also experience unexplained weight loss, increased blood pressure and heart rate, and periods of intense sweating due to their brain’s inability to control body temperature. Within a year to eighteen months, most FFI sufferers die.
The illness is caused by prions, which are misshapen proteins in the brain. Prions are also responsible for Mad Cow Disease and Creutzfeldt-Jakob Disease, which are also fatal illnesses. The prions in FFI build up in the thalamus and destroy the nerve cells there, which causes the worsening progression of the disease. Sadly, people with FFI typically don’t show any symptoms until they have passed child-bearing age, so in many cases they have already passed the disease on to their children before they know they have it. Children of people with FFI have a 50% chance of developing the disease themselves.
FFI was not even identified until the late 20th century, though the disease has been traced to its Patient Zero, a Venetian man who lived in the 1700s. He died after suffering from insomnia and other symptoms for over a year. Then, many of his descendants died the same way. For nearly two centuries, people with FFI were diagnosed as having illnesses like encephalitis because the true cause had not been identified.
Unfortunately, there is no cure for this devastating disease. As of now, anyone who develops FFI will die from it, though researchers are working on a way to treat it. But just because you can’t sleep doesn’t mean you FFI, even if your insomnia is severe. Though there is a sporadic form of the disease that develops in those who have not inherited it, there have been fewer than 10 such cases diagnosed. There also aren’t many cases of the inherited form, either. Basically, if you don’t have a family member who has died from the illness, then you really shouldn’t worry. You have a better chance of winning the lottery than you do of inheriting Fatal Familial Insomnia.