Book: The Family That Couldn’t Sleep: A Medical Mystery
Author: D.T. Max
Type of Book: Non-fiction, diseases
Why Did I Read This Book: As a chronic insomniac, the title caught my eye. In a quick read of the synopsis, I realized it really was far beyond my little, “I can’t sleep,” problem, and it seemed extremely interesting. I had never heard of prion diseases before buying this book.
Availability: Published by Random House in 2006, you can get a copy here:
Comments: I will be the first to admit that while I find disease fascinating, I am not strong in science. So elements of this book required me to reread sections to understand fully how it is that prion diseases destroy the brain, what sort of substance prions really are, etc. However, as a person with an imperfect understanding of some science, I can say that D.T. Max does a very good job of making science accessible to a person like me.
The family that could not sleep is a family in Italy that suffers from a disease called Fatal Familial Insomnia. There are several other families in the world affected by the condition, so it is extremely rare. It is a condition that strikes family members generally in late middle age and causes them to begin to lose physical control of their bodies as they stop sleeping. They sweat, they develop a very distinct pinprick appearance to the pupils in their eyes, they stop sleeping, and in end stages, have virtually no control over their bodies. In many cases, those who suffered from it were assumed to be either crazy or chronic alcoholics, and there is not a thing that can be done to help them. So few people suffer from the condition, and a cure would be so expensive to find that there is little incentive for drug companies even to research the condition.
FFI is a genetically dominant, hereditary disease. As a person with the gene that causes the condition ages, something triggers prions, a mostly protein infectious agent, to cause the proteins in the brain to mis-fold. Proteins in the brain fold in a particular pattern – when that pattern is altered, it causes neurological damage, resulting in death. Suffers are generally dead within two years of showing symptoms and their ends are terrifying. Since the brain no longer works correctly, the pain and mental anguish sufferers experience cannot be controlled pharmaceutically. Some painkillers and sedatives actually make the symptoms worse. While FFI is a prion disease that is genetic, there are many other prion diseases out there that are contagious and this book explores how those disease became zootrophic – meaning jumping from animals to humans. It discusses in very accessible language how prions were discovered, the scientists who discovered the prion diseases, and the lives of those who became infected with prion diseases.
Max discusses in detail how one of the first recorded prion diseases, scrapie, evidently became the scourge of England when sheep began to be bred for meat yield. In the process of breeding for size, the sheep became bred to develop scrapie, a communicable prion disease that caused the animals to lose neurological function and die. Autopsied sheep show brains filled with holes where the mis-folding proteins destroy the tissue. Land where scrapie infected sheep lived and grazed has been left fallow for a decade or more, only to find the prion disease is still there. Once land is infected with scrapie, it seems unlikely it will ever disappear entirely, but luckily there are sheep that are still genetically immune to the condition and are not infected.
Prions are a tricky substance. They are not really alive – they are a protein but a protein is not a living substance. A thing that is not alive cannot really be killed, so as of now, there is no way to combat prions. Nothing can get rid of them. Some studies show that using quantities of bleach may be effective in controlling them but even those studies were largely inconclusive. Prion disease has been found when stainless steel surgical instruments were used on an infected patient, then completely sterilized afterward.