Fatal familial insomnia (FFI) is a very rare but always fatal prion disease that is passed on genetically from generation to generation. Only around 50 to 70 families worldwide are affected by the genetic mutation, and males and females are equally likely to inherit it.
FFI is caused by a mutation in a gene called PRNP. The mutation causes proteins to develop abnormally in that they become misfolded versions of normal proteins. The abnormal proteins are toxic to body cells, especially neurons in the brain. The thalamus in the brain is the region which regulates body functions including sleep, and it is vulnerable to FFI.
Children need to inherit only one copy of the mutated gene to develop the disease, and in rare cases, the condition can arise spontaneously with no family history of FFI. Yet, it can still be passed on to subsequent generations.
Patients at first can be diagnosed as having insomnia or difficulty in falling asleep. Over time, they can’t fall asleep at all. They commonly also experience memory loss, high blood pressure, hallucinations, jerking of their muscles, profuse sweating, and loss of coordination.
Eventually patients enter a coma-like state and die within 9 to 30 months after symptoms first emerge. These symptoms usually become noticeable around age 40 but could begin as early as age 20 or as late as age 70.
At present there is no known cure for FFI. At best, patients can be advised on how to manage their symptoms and live as comfortably as possible. For example, they can be given a drug to reduce muscle jerking. Efforts to induce sleep such as vigorous exercising and taking narcoleptic drugs may also extend life somewhat.
A clinical trial has begun to see if the antibiotic drug doxycycline can be used for FFI patients. Doxycycline has been shown to prevent misfolded proteins in Creutzfeldt-Jakob disease, another prion condition, helping patients to live twice as long as they otherwise would have been able to.
Comments: Of course, unless Jesus returns to us first, we all will fall into a sleep from which we cannot wake up for a while. But then eventually we will wake up into a permanent situation in which we will never need to sleep again. This will happen when Jesus returns to judge the world and take all believers with him to a wonderful and eternal home in heaven.
FFI is just another example of what sin has done to this world. Whether we are talking about common maladies such as cancer or the flu or rare illnesses such as FFI, sin causes all sorts of problems. We are grateful an eventual cure for all problems is available for all followers of Jesus Christ.
Busy people might think it would be great if they could spend 24 hours of every day attending to their pursuits without having to “waste time” sleeping. But sleep is a gift of God meant to keep us healthy. Harvard says that scientists “have discovered that sleep plays a critical role in immune function, metabolism, memory, learning, and other vital functions.”
But sleep is not a good thing when we are talking about our spiritual lives. May we always be wide awake to the condition of our sinful souls and the cure we find in our Savior.
“And do this since you understand the present time. It is already the hour for you to wake up from sleep, because our salvation is nearer now than when we first believed.” Romans 13:11
By Warren Krug
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QUESTION OF THE DAY
How big was the largest snowflake ever recorded?
The largest snowflake ever seen measured 15 inches across.
Source: Peter Schriemer, “In Good Shape,” Kids Answers [January-March, 2025], page 9.
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