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Exploring the Darker Side of Everything

Fatal Familial Insomnia: The Most Terrifying Disease in the World

Written by Dave Page

Most people, at some point in their lives, have wished that they did not need to sleep. On the face of it, sleep does certainly appear to be tremendously wasteful, with the average person spending approximately one third of their life sleeping. It is certainly understandable that some people wish that this time could be employed in a more productive fashion: Last minute revision, finally getting down to the gym, and writing scripts for YouTube channels, are just a few productive sleep alternatives that spring to mind. Sadly, a sleepless existence is anything but the fabulously productive lifestyle that students, workaholics and habitual procrastinators wish that it could be.


Although scientists still do not fully understand why we need to sleep, it is generally accepted that the brain requires this time to correctly file information, transfer memories from short-term to long-term storage and remove waste products from brain cells. Any new parent will tell you (often at great length) just how detrimental sleep deprivation can be to your daily existence and this information has been utilised by interrogators and torturers throughout history. So, how bad would it be for your health if you literally could not sleep?

Not just periodically or for a few days, but literally never ever sleep again. Today we will try to answer that question as we investigate the truly terrifying condition that is Fatal Familial Insomnia.

The Beginning

From the outside, Hayley and Lachlan Webb appeared to be no different to any other brother and sister that had grown up in Australia. Haley worked as a successful television reporter for 9 News and, although we were not able to find anything to verify this, it is likely that Lachlan was still in school. However, beneath this seemingly ordinary family exterior, there was a curse: a ticking genetic timebomb that the siblings became aware of during their early teens. In an interview with nine news, Hayley said:

“My grandma started getting sick and dying. Her eyesight went, she had signs of dementia, she was hallucinating and couldn’t talk. Eventually she was diagnosed with FFI, that was the first time that we even knew that FFI existed.” 

So, what exactly is Fatal Familial insomnia? Also know as FFI, it is an extremely rare genetic disease that usually remains dormant until around the age of 50. However, once triggered it is completely unstoppable and the patient usually has between 6 and 36 months left to live.

The Cause

Although it contains the word insomnia, FFI is not actually a sleep disorder, it is a prion disease similar to Creutzfeldt-Jakob Disease or CJD (the human form of mad cow disease). These diseases are caused by incorrectly folding proteins in the brain. If one of these proteins interacts with an unaffected protein, then the mutation will spread, and they will both go on to infect more proteins – and thus the process begins. According to web MD:

“A mutation to the PRPN gene causes the prion malfunction in people with FFI. Most of the time, this mutation is passed down from a parent. However, in rare cases, the gene spontaneously mutates. This mutation occurs in the egg or sperm that forms the baby.

The PRPN gene controls the creation of the PrP protein. Experts aren’t sure exactly what this protein does, but when you have this specific genetic mutation, the protein forms in the incorrect shape. This makes it toxic to your body. The malformed protein builds up in your thalamus over your lifetime, damaging it”.

The thalamus is often referred to as the brain’s switchboard, and plays a large part in, amongst other things, transitioning between being asleep and awake, interpreting sensory input, and forming both short and long-term memories. Once this part of the brain starts to break down, sufferers of FFI start to experience symptoms such as: difficulty controlling movement, hallucinations, delirium, difficulty swallowing, and of course a complete inability to sleep.

Living with FFI

When Hayley was 20 and Lachlan 18, they went on holiday with both of their parents and immediately noticed that the mother had lost a lot of weight, another symptom of FFI. Their worst fears were confirmed when, a few months later at Hayley’s 21st birthday party, their mother told them that she had six months to live. One month after that, she had a started to show signs of dementia, would regularly fall, and was suffering from hallucinations.  She would pass away due to complications from the disease a few months later.

Dna. Original public domain image from Wikimedia Commons

After much consideration, both Hayley and Lachlan would undergo genetic testing and, unfortunately, they would both be diagnosed with the disease. Naturally, this had a profound impact on how they perceived some of the most common daily experiences. In an interview for Australian television, Hayley said:

“If I am overly tired, and I get, you know when your brain gets fuzzy when you’re really exhausted? And I think, crap! Could that be a symptom? And I have to make myself think, no, you are just tired. And then I go to bed, and I cannot sleep, and I think crap! Is that a symptom? Subconsciously, you can’t help but be scared. It’s terrifying!” 

In spite of this, neither sibling is prepared to admit defeat. Although there is currently no cure for this disease, they have both enrolled on a study at the University of California being led by US couple Eric Minikel and Sonia Vallabah, both of whom retrained as neurologists when Sonia was herself diagnosed with FFI. In Hayley‘s own words: “I don’t want to sit here while the sands through our hourglass pass waiting for it to trigger and for me to cark it. I want information, I want answers and I want a bloody cure.” 

One of the main reasons behind their decision to go public, was to help dispel some of the myths and factually incorrect reporting that surround the condition.  During a television documentary, Hayley talked about how frustrating it is to read sensationalist headlines such as “meet the brother and sister who have not slept for 30 years”:

“That is so not true” she said, “I can’t even stay awake through a whole bloody movie. So, that is completely untrue”.

Treating FFI

Although, as previously stated, there is currently no cure for FFI, one American man was able to remain alive for considerably longer than the projected 36 months. Known simply as Daniel, he decided that he would attempt any and every possible technique in order to prolong his life. In addition to ordinarily recommended practices such as vitamins supplements and regular exercise, Daniel began taking diazepam which would initially allow him to nap for about 15 minutes. When this no longer proved effective, he purchased his own isolation tank, dosed himself up on ketamine and nitrous oxide and was then able to sleep for approximately 4 ½ hours.


Unfortunately for Daniel, this chemically induced slumber would cause him to suffer from unbelievably vivid hallucinations and these would leave him unsure as to whether he was alive or dead.  He would continue with this treatment until it too became ineffective and would then begin experimenting with the use of electroconvulsive therapy. Although this extreme treatment would render him unconscious, it would still not allow his brain to achieve genuine sleep. Also, the repeated electric shocks caused him to develop terrible amnesia. In spite of these extraordinary measures, Daniel would eventually succumb to FFI, but he was able to live several years longer than his doctors had predicted.

Daniel’s case led researchers to believe that perhaps there may be alternative treatments that could extend the lives of the people suffering from this terrifying disease. Dr Cortelli, one of the many experts currently searching for a cure, said of Daniel’s case: “It at least opens the possibility to say that there is something we can do,”.

A Possible Cure

So, just how far along are the medical and scientific communities when it comes to finding a cure? Although it is impossible to answer this question with any true degree of certainty, there is at least one team of researchers whose work seems to have yielded a potentially promising result.

A team led by Gianluigi Forloni at the Mario Negri Institute of Pharmacological Research in Milan announced a clinical trial for a new drug. It is believed that this drug can prevent prions clumping together in the brains of patients who have been diagnosed with FFI. According to Gianluigi, “It might delay or completely disrupt the development of the disease”.

However, there is a major problem with this study, as it requires the genetic testing of the family members of people that have already passed away due to FFI and, perhaps understandably, the majority of these people are electing not to find out if they are carriers so that they do not have to live the rest of their lives in fear of the inevitable. Dr Cortelli has raised concerns about the trial, saying that “The drug may have side effects on some of the subjects, actually giving away their diagnosis and causing them unnecessary distress. And even if there are survivors at the end of the trial period, it wouldn’t necessarily indicate that the drug was successful. Those few individuals might just have been lucky, given that the gene is known to remain dormant in some people until they are in their 80s”.

Despite these reservations, the trial is currently underway, and it is just possible that it may reveal a cure for the hundreds of people around the world who live in constant fear that they may one day wake up and never be able to properly fall asleep again.

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