As most of us know, lack of sleep can cause a lot of issues in our daily lives. From hallucinations, mood swings, to having trouble thinking. So of course, doctors recommend you get plenty of beauty rest, but what if a disease can prevent you from going to sleep? It is called Fatal Familial Insomnia and (spoilers) eventually leads to your death.
How it works
FFI is a type of prion disease. Prion diseases are usually rare but known for their capabilities of “wasting the brain.” It’s normally infectious either through contaminated meat, genetic, or airborne. It permanently affects the brain and results in death eventually.
When you body enters REM sleep, your body is actively repairing itself. There are three stages of sleep. FFI is fatal because it actually prevents the individual from going past stage 1 on sleep.
- Non Rem Stage 1&2 is light sleeping
- Non REM Stage 3 is a deep slow wave sleep (SWS)
- REM stage where dreams and reparation occurs.
Because FFI prevents you from going into REM sleep, your body is slowly being worn down every day. It is also genetic and normally occurs after child bearing age which means by then most have children that they have passed the gene on to.
The disease has four stages. People can die from either 7 or 36 months after the onset.
- Person has insomnia that keeps worsening which results in panic attacks and disorders. There are also sudden phobias that are present. This stage only lasts 4 months.
- Hallucinations and panic attacks worsen due to continuos sleep deprivation. This continues for 5 months.
- This stage is a complete loss of sleep along with rapid weight loss. The patients mental capabilities are severely limited. This stage lasts 3 months.
- They appear to have dementia but they still understand what is happening to them. The patient also becomes mute and unresponsive. A coma usually follows along with death and this stage lasts 6 months.
The patients are usually stuck in stage 1, where they can repeatedly jerk their limbs as if they are dreaming.
You are probably thinking, “Well they could just take some sleeping pills to force them into REM sleep, right?” Well, no. It about 3/4 of cases, taking any type of sleeping medicine actually hastens the conditions progress and makes the symptoms worse.
One famous case is actually that of Michael Corke. He was a music teacher from Illinois and started having trouble in the year 1991- a little before his 40th birthday. He sought help at a local hospital when sleep started become non-existent. The doctors there were actually unsure of his disease and diagnosed him with multiple sclerosis. They tried to relieve his suffering in the later stages of the disease by putting him in a coma using sedatives. However, his brain continued shutting down and he passed in 1993, a month after his 42nd birthday. By this time, he was completely sleep deprived for about 6 months.
One person actually exceeded the average life expectancy by reaching one year. He used multiple techniques such as meditation and vitamin supplements. He used different stimulants and complete sensory deprivation in order to stay alert during the day. He wrote a book and travelled during the disease but eventually he reached the later stages and died.
How rare is this disease?
In 1998, there were 25 families affected by this disease. This was spread out over Germany, Italy, America, France, Britain, and Japan. In 2011, a man in the Netherlands also succumbed to FFI but he was originally from Egypt.
This disease is 100% fatal and progresses quickly. It is a poorly understood disease that most are originally misdiagnosed. There are no treatments whatsoever for this disease. Any kind of attempt to relieve the symptoms typically worsens them and it is an extremely debilitating disease. While is a 50% chance of passing it off to their offspring, there is now a genetic testing that can determine whether or not you have FFI and hopefully the future of gene therapy will provide a cure.