Three years ago I saw a book listed on Goodreads that completed intrigued me. Published in 2006 and entitled The Family That Couldn’t Sleep: A Medical Mystery, D.T. Max’s book tells the tragic story of an Italian family cursed with a rare genetic disorder that renders its victims completely sleepless. Labeled Fatal Familial Insomnia or FFI and affecting roughly 1 out of 30 million people, victims of the incredibly rare inherited disorder exhibit no symptoms until the onset of early middle age. Then, one day out of the blue suffers begin experiencing fever, pinpoint pupils, anxiety, rapid sexual decline (menopause in women, impotence in men) and insomnia. Unable to sleep for months or even several years, victims gradually descend into madness, thrashing about in a weird twilight state neither fully conscious nor awake with death their only release.
When I saw my public library had an available copy of The Family That Couldn’t Sleep I immediately grabbed it. Max’s book made for interesting reading, because it’s not just a book about FFI, but also about prion-causing diseases in general. Discovered by scientists only in the last decade or so, prions are rogue proteins that for reasons little understood, cause bodily proteins to misshape. These nonliving infectious agents are what cause the dreaded animal diseases bovine spongiform encephalopathy (BSE or more commonly mad cow disease) and scrapie in sheep. In humans, it’s the causative agent in kuru, a transmissible and eventually fatal brain disease found among the Fore community on the island of New Guinea. It’s also what causes rare disorders like FFI and Creutzfeldt-Jakob disease. When compared to our understanding of other disease-causing agent like viruses, bacteria and parasites, our knowledge of prions is considerably limited. But we’re learning more each day. So another cool thing about The Family That Couldn’t Sleep is you can see how our knowledge has increased over the last few years. Considering this book was published a decade ago, I’ll be keeping my eyes open for a more recently published book on the subject. It should make for some fascinating reading,