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

Reasonably interesting, but no mystery

Daniel T. Max
The Family That Couldn't Sleep: A Medical Mystery
Random House, 2006
ISBN: 1-4000-6245-4
256 pages (main text)

Because the third season of House, M. D. continues to languish at the bottom of my Netflix queue with an availability date of "unknown" I've been without a good medical mystery lately. So I was glad when D. T. Max's recent book The Family That Couldn't Sleep: A Medical Mystery arrived.

The book is good in many ways but, alas, it's not a medical mystery. The family of the title is from the Italian Veneto (the area near Venice) and they suffer from an inheritable disease that has come to be known as fatal familial insomnia. The reality of the disease is worse the the name makes it sound; it's a degenerative disease of the brain. It's just that one of its first symptoms is insomnia. Mr Max tells us the only really mysterious thing about the disease early in the book: it's a prion disease.

Prion diseases used to be mysterious. What characterizes them is that they seem depend only on proteins (very large molecules of which about a zillion kinds are essential to any living thing). It seems that some of the proteins in humans and animals can be folded into more than one shape. One shape is normally found in the body and the other isn't. It appears to be the case with prions that, if the version that's folded wrong comes into contact with the version that's folded right, it can influence the correctly-folded version to fold the wrong way. That causes a problem, presumably either because eventually there's not enough of the right kind around to do some important job or because the wrong kind actively causes a problem. Either way, something goes badly wrong. Prion diseases are also possibly a little scary since prions amount to potentially infectious agents that are very hard to get rid of. Killing a bacterium or rendering a virus unable to infect a cell are much easier than destroying a protein molecule. Various prion diseases have been identified: scrapie in sheep, chronic wasting disease in deer, bovine spongiform encephalopathy (BSE or mad cow disease) in cows with some transmission to humans, kuru in humans mostly in New Guinea, Creutzfeldt-Jakob disease in humans, and Gerstmann-Sträussler-Scheinker disease in humans.

Not much is known about fatal familial insomnia other than that it's a prion disease, so Mr Max can't tell us all that much about it. We do learn something about how it was identified and how the some members of the family are dealing with their possibly-fatal inheritance. But that doesn't come close to filling this short book. Most of the book is about how prions came to be understood and how particular diseases have been identified as being caused by them. That's an interesting enough story and it could be told as a mystery, but Mr Max doesn't. He tells the story as straightforward history. So, unlike reading Berton Roueche's articles and books or even watching House, M. D., his audience doesn't get to join in the frustration of following blind alleys or the excitement of discovery. Still, there are interesting twists in the history of the discoveries. One of the scientists who laid some of the groundwork for understanding prion diseases plead guilty to child molestation. And the politics that affected the British inquiry into BSE is depressing but worth knowing about.

The chapter "Coming to America?" about the possibility of a large outbreak of BSE in the United States is mostly well reasoned though of course highly speculative. But toward the end of the chapter, in discussing what people think, Mr Max goes so far as to quote anonymous internet message-board postings. Postings of that sort probably do indicate that someone thinks what's said in them, but they're not generally the place to find good sense.

The Family That Couldn't Sleep is worth reading by anyone who's interested in the history of this part of medicine, but it's not exciting.

I suspect that, like me, Mr Max is a fan of P. G. Wodehouse since he uses "bruited" and the Latin abbreviation "viz." as Mr Wodehouse does in many Bertie Wooster novels. Though it must be said that Mr Max uses "viz." (from Latin "videlicet" and meaning "specifically" or "namely") in places where "e.g." ("exempli gratia", meaning "for example") would be more apt (p. 201).

Also, if I had been Mr Max's editor, I'd have suggested that the explain the "(caet par.)" that he quotes on page 28. It's short for "ceateris paribus", Latin for "all else being equal".

The word's meaning is clear enough from context, but I'd also have suggested explaining "epizootic" (p. 175). And I personally have a dislike of uses such as "five long years" (p. 177). I think it's a bad metaphor since years don't vary much in length. There's also "farmer's" where "farmers'" is wanted on page 180.

Posted: Tue - January 23, 2007 at 06:40 PM   Main   Category: