The Family That Couldn't Sleep

It sounds like something from a fairy tale; a family cursed to die from sleeplessness. In Italy, a family has been afflicted for generations by a prion disease called Fatal Familial Insomnia. Prions destroy the area of the brain that allows them to sleep. The onset begins in middle age when they begin running high fevers, and the pupils of their eyes shrink; they exist in a half state between sleep and wakefulness. Their bodies eventually burn themselves out and they die from exhaustion. Their story is told in The Family That Couldn’t Sleep by D.T. Max. The author also discusses other prion diseases; Kuru with its links to cannibalism, Scrapie which decimated sheep flocks in Britain, and of course, the other infamous British prion disease- Mad Cow.

This is where the story becomes truly terrifying. Because the government agencies entrusted with overseeing the safety of the food chain are also responsible for promoting the health of the agricultural industry in Britain and in the U.S., many more of us are potentially afflicted Mad Cow than believed. “It can be estimated, based on a European Union Scientific committee’s work, that the English ate as many as 640 billion doses of BSE (mad cow disease) during the crisis as a whole”. One hundred Fifty Britons have died so far, tests indicate that as many as 4000 more are infected. Don’t think you are safe here in America, Mad Cow has already surfaced, and our elk and deer are infected with another prion disease called Chronic Wasting Disease.

This is an intriguing chronicle of the attempts to solve the mysteries of prions.