You know I love a good medical mystery. The Family That Couldn’t Sleep by D.T. Max was a such a remarkable book. It begins by introducing an Italian family in which many members die early because they can no longer sleep. The cause? A prion disease, Fatal Familial Insomnia. Prions are infectious malformed proteins. From here Max explores the history of prion discovery and research, other prion disease such as Kuru, Chronic Wasting Disease (in deer), and the most famous – Mad Cow Disease. He explores the scary way each of us could have been exposed to Mad Cow, and how still today nothing can be done to help those suffering from these disorders.
Max’s novel is fascinating and frightening. I was interested in prions in school, but I believe with a little effort someone who’s never heard of prions could understand and enjoy this book. This is the best non-fiction book I’ve read in quite a while.
I’m going to provide some interesting excerpts to hopefully clarify prions a little, and give an example of the author’s subjects.
“Prion…the infectious agent responsible for bovine spongiform encephalopathy, or mad cow disease, Creutzfeldt-Jakob disease, and a disease in sheep called scrapie, was not a virus or a bacterium but a protein, a nonliving thing.”
“But this family is cursed. For at least two centuries, its members have suffered from an inherited prion disease called fatal familial insomnia that strikes them…killing them by depriving them of sleep… In the general population the chance of having FFI is one in 30 million; within the affected branches of this Italian family, it is one in two.”
“…roughly eight years passed after that discovery [of mad cow disease] before an effective barrier was set up to protect humans from mad cow. It’s as if John Snow had successfully traced cholera to the Broad Street pump only to have the government tell Londoners to go on drawing their water from it while committee after committee studied the problem.”
“Fortunately, prions aren’t as infectious as, say, the flu. If they were, only long-time vegetarians would be alive in England today.”