Bounteous nature has cooked up countless unpleasant ways for us to meet our maker, from the sudden horror of a ruptured aneurysm to the boxed-in terror of A.L.S. to the odoriferous humiliation of cholera. Death by Ebola virus has always seemed an unreasonably cruel way to go, and only a sadist could have come up with cancer.
But even in comparison with those grim diseases, the strange, tossing-and-turning death suffered by generations of one old Venetian family seems particularly dreadful. For more than 200 years, as many as one out of every two members of this family has succumbed to an illness whose tell-tale symptom might have been ripped from the pages of a Gothic tale: relentless, agitated insomnia. One day, you’re happy and healthy; the next day—a day that often arrives in complacent middle age—you can no longer sleep. Days pass, weeks pass, months pass, during which you writhe toward a feverish, dementia-addled demise, your brain eaten away by infection. Until recently, no one knew the cause of this illness. To this day, no one has discovered a cure.
The story of this strange disease, known bluntly as Fatal Familial Insomnia, is one of the central, mind-bending riddles of The Family That Couldn’t Sleep, D.T. Max’s rich scientific history—or “medical mystery,” as he calls it. Like all good whodunits, this one whisks us through time and space, from the country house of an 18th-century Venetian doctor to the jungles of Papua New Guinea to a modern-day New Jersey racetrack, all in an effort to pin down the culprit behind Fatal Familial Insomnia. Only in this case the culprit isn’t a person, but a protein. A very deadly protein. Scientists call it a prion, and they have linked it to a rash of horrifying neurodegenerative ailments, including mad-cow disease, Creutzfeldt-Jakob Disease and a ghoulish 1950’s epidemic that nearly obliterated a tribe in Papua New Guinea. Two scientists have won Nobel Prizes in the quest to understand it.
In the mystery of prion disease, Mr. Max—who himself suffers from a neurological ailment—has hit on a scientific and journalistic gem, and he’s polished it to a lovely, angled sheen. Prions, as he explains, are among the great paradoxes of modern medicine, biological oddities that technically shouldn’t exist. In their most basic form, they’re just proteins, inert clusters of molecules that serve as the body’s “building blocks.” But unlike most proteins, they also behave in very odd ways: They act “like viruses or bacteria” and become infectious.
Proteins aren’t supposed to be infectious. They’re not alive; they don’t have DNA or nucleic acid, which are generally assumed to be among the essential ingredients of infectious agents. But for reasons no one can yet explain, prions have the ability (and in some poor people, the tendency) to shift shape, performing sudden “Jekyll-to-Hyde conversions” that cause them to do serious kamikaze-style damage to the cells in victims’ brains. And when this happens, people get sick, animals go nutty, and the very frame of nature seems out of joint.
“For biologists the prion theory is a heresy,” writes Mr. Max, alluding to a split within the scientific community between those who subscribe to prion theory and those who don’t (among the latter, the venerable James Watson). “To them, the word ‘infection’ means something specific—a disease process brought about by living things, more exactly by things with nucleic acids or genetic material in them …. The theory of prions thus threatens to unmake the defining moment in biology.”
If all this sounds heady, well, it is. But in Mr. Max’s hands, prion theory—and the equally tangled story of its discovery—is as clear as it is thrilling, a brain-teaser wrapped in more than 250 pages of fascinating facts and quirky characters. Like Carleton Gajdusek, the self-described “pedagogic pedophiliac pediatrician” who won the Nobel Prize in 1976 for his theory of “slow-viruses” (an important precursor to prion theory). Or Stanley Prusiner, the controversial king of prion research (he coined the term) whose aggressive, credit-hungry ways have won him labfuls of enemies as well as a Nobel Prize. And how can one forget the Fore, the New Guinea tribe whose brief experiment with cannibalism unleashed a prion disease called kuru that all but decimated the population?
But Mr. Max also has a message for us woven alongside these wild personalities and odd events. It’s a message not just about the scary workings of nature, but about the way in which nature can become exponentially scarier once humans tamper with it.
Over the course of the book, Mr. Max builds an argument that centuries of meddling with the natural world—trying to make it more productive by in-breeding sheep or turning cows into unwitting cannibals by feeding them animal protein—have given rise to new and deadly forms of prion disease. It brought mad-cow disease to England, infecting at least 800,000 cattle and 160 people, and it could well give rise to a similar epidemic in the United States. (Certainly Mr. Max’s description of the USDA’s generally lax and complacent response to the threat of mad-cow disease doesn’t breed faith in this country’s will to prevent it.)
“For symbolic as much as environmental reasons, we are in the era of the prion and can be in no other,” warns Mr. Max. “Prions sit at the intersection of humans’ ambition and nature’s unpredictability, and it is hard to say which is more dangerous.”
A statement like that sticks with you long after you’ve put the book down for the night; it pops up in your dreams, making you sweat with visions of mad cows, zombie proteins and sleepless Venetians. Particularly the tormented Venetians. But in the end, you’re also grateful for these nightmares: If you were dreaming, you must have been asleep.
Lizzy Ratner is a reporter at The Observer.