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.
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