The Family That Couldn't Sleep: A Medical Mystery Review

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(More customer reviews)In 1765 a doctor in Venice died of what was labeled "an organic defect of the heart's sack", but he many have been the first recorded victim of a strange disorder passed down to his many descendants into the twenty-first century. It had so many weird symptoms and was so rare that the victims were frequently misdiagnosed, often being dismissed as alcoholics in withdrawal, or as having meningitis, depression, encephalitis, and many other incorrect labels. The symptoms are appalling. The illness strikes adults who have no previous significant medical problems and may have started families of their own. A victim begins to hold up the head stiffly, and then sweats profusely; family members are terrified when these initial symptoms appear, as the others follow inexorably. The pupils contract to pinpoints, the heart goes mad with increased pulse and blood pressure, and sleep becomes impossible, no matter what drugs are used to bring it on. The victim knows what is happening until dementia takes over, followed by a coma and then death in about a year or two after the symptoms began. Nothing at all can be done to stop the progress of the illness, which is passed to one half of each succeeding generation. It is, however, becoming more comprehensible as we learn more about prions, those bad proteins. In _The Family That Couldn't Sleep: A Medical Mystery_ (Random House), D. T. Max has not only told the story of this particular illness, but also of other illnesses that are (or might be) caused by prions. It is a tale full of undeserving victims and flawed heroes, and it tells just how far we are from solving some basic biological riddles.
Proteins are what DNA codes for; because prions aren't alive, they cannot be killed; radiation, formaldehyde, and all ordinary sterilization procedures do nothing to them. You might get prions by having your DNA code for them; that's what happens in the Italian family that has Fatal Family Insomnia (FFI). That's pretty rare, but you might also get prions by eating them, as in eating cows with Mad Cow Disease. There are prion diseases of sheep and deer as well. A strange neurological disease in New Guinea called kuru unlocked some of the mysteries of prions (in this case, passed by cannibalism), and Nobel prizes have stemmed from this work. One of the frustrating parts of this story is that prion afflictions have often been brought about by people. No one intended to get any animal or human sick, but human intervention made it happen. Scrapie started afflicting sheep two centuries ago as a direct result of intensive breeding to make bigger animals. Mad Cow Disease was caused by the unnatural feeding of sheep cadaver protein to cows. Chronic Wasting Disease in deer seems to have been passed to them when sheep were held in pens used for sheep with scrapie, but also may come about when deer farms, pressed to produce bucks with bigger racks for hunters, fed the deer the same sort of sheep cadaver protein that the cows got. It also got spread when humans transported these deer to different regions of the country, a migration they could not have done on their own. We attempt to control nature, and in response, nature presents us with problems no one could have foreseen.
Max has given a clear history of prion diseases and our attempts to understand them. A surprising part of the prion story is that it gives evidence that our hominid forebears practiced cannibalism, and therefore the genes of most people show a protective trait that helps keep prions from causing disease. The positive part of the story is that we are not as ignorant about prions as we were four decades ago, and even that prion research may open up answers to possibly related neuromuscular and neurodegenerative diseases like Parkinson's and Alzheimer's. But what we know is overbalanced by what we don't. Max writes, "Not since Pasteur's time have researchers attempted to counter an infection knowing so little about what they are fighting." Not only that, but prion diseases are all brand new diseases, as diseases go, and some have been manufactured in the past few decades; there is no telling what completely new one will be in tomorrow's headlines.


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