The Family That Couldn't Sleep: A Medical Mystery
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For two hundred years a noble Venetian family has suffered from an inherited disease that strikes their members in middle age, stealing their sleep, eating holes in their brains, and ending their lives in a matter of months. In Papua New Guinea, a primitive tribe is nearly obliterated by a sickness whose chief symptom is uncontrollable laughter. Across Europe, millions of sheep rub their fleeces raw before collapsing. In England, cows attack their owners in the milking parlors, while in the American West, thousands of deer starve to death in fields full of grass.
What these strange conditions–including fatal familial insomnia, kuru, scrapie, and mad cow disease–share is their cause: prions. Prions are ordinary proteins that sometimes go wrong, resulting in neurological illnesses that are always fatal. Even more mysterious and frightening, prions are almost impossible to destroy because they are not alive and have no DNA–and the diseases they bring are now spreading around the world.
In The Family That Couldn’t Sleep, essayist and journalist D. T. Max tells the spellbinding story of the prion’s hidden past and deadly future. Through exclusive interviews and original archival research, Max explains this story’s connection to human greed and ambition–from the Prussian chemist Justus von Liebig, who made cattle meatier by feeding them the flesh of other cows, to New Guinean natives whose custom of eating the brains of the dead nearly wiped them out. The biologists who have investigated these afflictions are just as extraordinary–for example, Daniel Carleton Gajdusek, a self-described
“pedagogic pedophiliac pediatrician” who cracked kuru and won the Nobel Prize, and another Nobel winner, Stanley Prusiner, a driven, feared self-promoter who identified the key protein that revolutionized prion study.
With remarkable precision, grace, and sympathy, Max–who himself suffers from an inherited neurological illness–explores maladies that have tormented humanity for centuries and gives reason to hope that someday cures will be found. And he eloquently demonstrates that in our relationship to nature and these ailments, we have been our own worst enemy.
“The Family that Couldn’t Sleep is a riveting detective story that plumbs one of the deepest mysteries of biology. The story takes the reader from the torments of an Italian family cursed with sleeplessness to the mad cows of England (and, now, America), following an unlikely trail of misfolded proteins. D. T. Max unfolds his absorbing narrative with rare grace and makes the science sing.” –Michael Pollan, author of The Omnivore’s Dilemma and The Botany of Desire
“Much has been written about prions and Mad Cow Disease–nearly all of it is worthless. Thankfully, from the world of journalism comes D.T. Max to set things right. Throw all those other “Mad Cow” books in the trash: This is the book to read about prions–or whatever you want to call them. It’s a riveting tale, told by someone with a very special understanding, derived in part from his own strange ailment. Find a cozy spot, clear your schedule and dive in.”
– Laurie Garrett, author of Betrayal of Trust and The Coming Plague
“D. T. Max deftly unfolds the mysterious prion in all its villainous guises. Although scientists do not fully understand these proteins–how they replicate and wreak such havoc in their victims’ brains–The Family That Couldn’t Sleep reveals their historical, cultural, and scientific place in our world. Prepare to be enlightened, entertained, and frightened.”
–Katrina Firlik, MD, author of Another Day in the Frontal Lobe
“A great book. D.T. Max has drawn the curtain on a cabinet of folly and malady that will stagger your imagination.”
– Philip Weiss, author of American Taboo
“D.T. Max has combined the enthralling medical anthropology of Oliver Sacks with the gothic horror of Stephen King to produce a medical detective story that is as intelligent as it is spooky. The villain of The Family That Couldn’t Sleep is the prion, a tiny little protein that causes some of the most terrifying, brain-mangling, creepy diseases known to man. Always fascinating–how could it not be, given that its characters include cannibals, mad cows, madder sheep, a Nobel prize-winning pedophile, and, most poignantly, an Italian family cursed by fatal insomnia?–Max’s book is also a gripping account of scientific discovery, and a heartfelt meditation on what it means to be cursed with an incurable, and brutal, illness.” – David Plotz, author of The Genius Factory
From the Hardcover edition.
They had no idea they lived on an island, or even what an island was. The Fore had a subsistence economy. They lived on sweet potato and taro root, supplementing their garden vegetables with game the men hunted. The women and children captured rats and bugs by hand and ate them. The Fore kept pigs as well, whose foraging they tried to control: much of the men’s work consisted of fencing the gardens against animals. Pigs were enormously valuable and prestigious possessions, so they lived with the
disease and aspects of the novel nature of the organism behind it. Thanks to his energy, everyone in the scientiﬁc world heard about “slow viruses,” as he took to calling the agent behind kuru and such related diseases as Creutzfeldt-Jakob disease and scrapie, and scientists threw resources at slow viruses until they began to yield their secrets. His work would change our understanding of disease. Of course, from the Fore point of view, neither the Glasses nor Gajdusek nor the millions of dollars
fast enough for the molecules to separate. Then you take the separated-out molecules and inject them into mice or hamsters. If some get sick quicker than others, you know that they received a more concentrated agent: you keep repeating the process until you’ve come as close to purity as possible. Then you try to deﬁne the agent by its properties. Does it dissolve in water? What dye does it react with? And so on. Paradoxically, the puriﬁcation of something as small as a protein is a commitment to
virus hiding behind the protein, doing the dirty work of infection. But prions had a surprise in store for Prusiner. Once he and his lab puriﬁed the prion enough to determine part of its amino acid sequence, they were shocked to ﬁnd that prions were ordinary proteins manufactured by a healthy gene in the host’s own body. In other words, prions were not something that infected the victim from outside; they were something the victim himself produced. 124 T H E F A M I LY T H AT C O U L D N ’ T S
an e-mail list: “My husband seen his brain X-ray and said it looked like someone shot him with a .22 shotgun.” xxvi Introduction I sat next to Ignazio that day, the two of us behind a child’s school desk, and looked out across the terra-cotta ﬂoor at the family seated in a circle around us. I was there as a visitor, a guest, a journalist who had written about the family, and an American, from the land where technology ﬁxes everything. Ignazio welcomed the group, told them how important this