Sunday, November 11, 2012

The Family That Couldn't Sleep: A Medical Mystery by D. T. Max

The Family That Couldn’t Sleep is a well written, fascinating, terrifying and depressing read, so only read it if you have a rather morbid curiosity about scary medical conditions and aren’t freaked out by doomsayers.
Author D. T. Max (pictured right) does a good job of explaining a complicated group of related disorders caused by radical proteins called prion disease, which scientists predict will come to overshadow bird flu, aids and other viruses within the next two decades. 

He begins with the story of an Italian family that for at least 200 years has been plagued by an extremely rare hereditary disorder called fatal familial insomnia that, after the onset at approximately 50 years of age, destroys the brain’s capacity to fall asleep. There are about 40 families around the world known to have this horrible disease, the symptoms of which are particularly ugly. Victims begin to hold the head stiffly to one side and sweat profusely. Then their pupils contract to pinpoints, their heart rate increases dramatically, and sleep becomes impossible. Dementia is followed by a coma, and then death from exhaustion in about a year or two. So far doctors have not found anything to stop the progress of the illness, which is passed to one half of each succeeding generation.

The author then takes us to New Guinea where a related neurological condition, kuru, is discovered in the Fore tribe, who, after eating the brains of their dead relatives as a show of respect, develop symptoms similar to those of fatal familial insomnia. This brain-eating thing reminded me of Jean Aurel’s book, Clan of the Cave Bear, a fictional account of a pre-historic family who ate the brains of their deceased family members to gain their knowledge. The major difference being that kuru was discovered in 1976!

And then there’s bovine spongiform encephalopathy, commonly known as mad cow disease, and chronic wasting disease in deer, both of which are fatal neurodegenerative disease that causes a spongy degeneration in the brain and spinal cord; and sheep scrapies, a disease that makes sheep so itchy they scratch themselves bald and bloody in their search for relief. These three diseases apparently started when man got the bright idea to turn animals into cannibals to make them grow bigger and therefore more profitable for their owners. Yep, they were grinding up dead cows, sheep and deer to make feed for them, including the ones who died of the above prion diseases, passing along the disease to others cows, sheep and deer, and ultimately to us eaters of cows, sheep and deer. Max says, “Prions sit at the intersection of humans' ambition and nature's unpredictability and it is hard to say which is more dangerous.” Lovely.

And finally, Creutzfeldt-Jakob, a rare, fatal neurological disorder of humans that at least in some cases is thought to be the result of eating beef from cattle infected with mad cow disease. Creutzfeldt-Jakob symptoms include dementia, personality changes, impaired memory, judgment, and thinking, insomnia, depression, involuntary muscle jerks, and blindness. On the upside the deterioration is much more rapid than with Alzheimer’s or Huntington’s, so you die quicker.

The book also includes a good deal of information about an ongoing spat between two scientists (both of whom received Nobel Prizes for their prion research) over “ownership of the disease,” but those parts aren’t nearly as interesting.

The Family That Couldn’t Sleep will cause you to lose sleep, not just because you won’t want to stop reading it, but also because it predicts a future outbreak of prion disease, and that is pretty scary.

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