The Family That Couldn't Sleep by DT Max

Sleep and I don't have the best relationship. I know we should spend more time together--in fact, we should be hanging out right now. Sometimes it's just so hard to make the schedules work. However, despite our problems, I don't know what I'd do without sleep.

That's what makes the subject of the nonfiction book The Family That Couldn't Sleep: A Medical Mystery so horrifying. The titular family is an Italian clan, dating back a single ancestor in the 1700s, whose members have a tendency to develop Fatal Familial Insomnia in middle age. FFI victims have a sudden onset of symptoms: since their brains can't rest, the body deteriorates as well. Organs begin to shut down. The person sweats and hallucinates uncontrollably. Even the ultimate coma is a nightmare of thrashing and agony. Finally, maybe months later, the person dies of total exhaustion. Five years later, his sister develops the signs. Twenty years later, it's his niece and one of his sons. It's the family plague.

While tracing the history of the family from that eighteenth-century Venetian doctor through a Mussolini-era local politician to the present day, science journalist D.T. Max takes a harder look at the science behind the disease. It turns out that FFI isn't caused by run-of-the-mill genetic defects, or an unlucky susceptibility to viruses. It's caused by "prions," or infectious particles derived from protein and almost impossible to eradicate. Nobody's really sure what to do about prion diseases. Since the 1950s, doctors and scientists have known that prions exist, and that they cause devastating (and almost always fatal) diseases like FFI and the similar Crutzfeldt-Jakob Disease (one strain of which is mad cow). But there's virtually no treatment available, and definitely no cure. Accurate diagnoses are rare as well; the Italian family had to fight against doctors who assumed nervous exhaustion and/or alcoholism were to blame for the strange tremors and rapid degeneration. Modern British and American people who have CJD aren't having much more luck--especially older ones, whose symptoms are generally assumed to be Alzheimer's.

The sections talking about the mad cow epizootic/epidemic are the most terrifying parts of the book. The fact that the disease is on an invisible timer ("Happy 45th birthday! Remember those burgers you ate all those years ago?"), and doctors can't predict which people will develop symptoms, is bad enough. But then add that countries like England, Canada, and the U.S. know that this disease is generally transmitted by feeding infected cow proteins to other cows, which are then fed to humans--yet they still can't get up the regulatory wherewithal to pull all the infected cows out of the beef population and ensure that farmers are using the proper precautions. Basically, we're all screwed.

(In completely related news, I don't think my birthday dinner will include the usual steak this year.)

if the subject matter is depressing, at least the storytelling is very good. The most disheartening part of the book (after you're done calculating all the beef you've eaten in your lifetime, and figuring out how much quality sleep you really got last night) may be that there's no real resolution. If the book has a drawback, that's it. A "happy" ending isn't feasible, but there's no ending at all. Just the politics of science, with slow drabbles of genuine scientific advance.

Now: who's up for some veggie burgers, followed by a long, luxuriant nap?