“Insomnia is a kind of torture because while the world is fast asleep, you’re up all alone. Your mind buzzing with every random thought in the universe and sometimes the thoughts will reach a standstill and your mind goes blank. You become more aware of the silence and it is during this moment that you realize how alone you are.”
Death is natural, death can be terrifying, and death can come in many forms. One of them happens to be sleep.
Fatal Familial Insomnia is a rare genetic condition that exist among 25 families and less than million people around the world.
This condition was first discovered in a family from Venice, Italy, who suffered from the condition for the next 200 years.
What is Fatal Familial Insomnia (FFI)?
FFI is a prion disease. Prion diseases are mutations to the protein that naturally exist in the human body, causing it to form a clump of proteins.
These mutations regenerate, killing the cells that exist around the area forming a hole in the area. In the case of FFI, protein around an area of the brain known as thalamus is affected. The thalamus is the part of the brain that controls the sleep-wake cycle, somewhat like a switchboard. Hence, when the area is exposed to prions, the body goes into a state of insomnia. Meaning, the thalamus switched on the awake switch, forever.
What happens to people living with FFI?
Now, we all know what a day, or a few days in the case of some of you out there does to your body. Imagine living with not even a second of shuteye.
The onset is anytime for those carrying the gene, it can be mid-age 20 to somewhere in your forties. However, the minute the onset begins, the time bomb starts ticking.
Their body physically and mentally deteriorate, to the point where they cannot communicate anymore. They would occasionally doze off but just for a split second before their body jerks awake.
Those afflicted would remain sleepless for about 6 months before they die.
Is there a cure for FFI?
No amount of sleeping pills or barbiturates cold cure this condition. Neither did sleep clinics.
However, while you are sitting there how scary and unfortunate this condition may be, thorough studies are being conducted in different regions of the world, seeking a cure.
Lesson to be learned
Be grateful.
Do not take sleep for granted. Be sure to get at least 8 hours of sleep a day and if you face troubles sleeping, visit your nearest sleep clinic or general practitioner.
There are people around the world who would love to be in your shoe.
Goodnight!
References:
- https://edition.cnn.com/2017/09/19/health/fatal-insomnia-family-curse-somethings-killing-me/index.html
- http://www.bbc.com/future/story/20160118-the-tragic-fate-of-the-people-who-stop-sleeping
- http://www.dailymail.co.uk/news/article-3568158/Nine-News-Hayley-Webb-brother-inherited-Fatal-Familial-Insomnia.html
- https://www.goodreads.com/book/show/32264.The_Family_That_Couldn_t_Sleep
- https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3296552/
- https://www.youtube.com/watch?v=IJzZcFoT-1A
- https://www.youtube.com/watch?v=Co94aQDs3ek
