The Family That Couldn't Sleep: A Medical Mystery
D.T. Max
Language: English
Pages: 336
ISBN: 081297252X
Format: PDF / Kindle (mobi) / ePub
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.
Advance praise
“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.
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family was not from the Veneto. Or it may have been because she had red hair, and family members believed that those who had red hair were more likely to come down with the malady. But she was not the carrier: her husband, Vincenzo, was. Starting with their children, FFI followed an unfortunate pattern. Those branches that carried the mutation grew poorer, because they The Doctors’ Dilemma 17 kept losing able-bodied adults. In response, like most poor families, they tended to have extra
welcomed the breeder to St. James’ Palace. Young reported that they spoke for more than an hour. With the mark of His Majesty’s approval, Bakewell now became even better known—and 24 T H E F A M I LY T H AT C O U L D N ’ T S L E E P richer. One year he made £1,200 (£150,000 in contemporary money) from the stud fees paid him for a single ram. Bakewell tried to form combines with those who had bred using his Dishley Leicesters to keep prices high, but he couldn’t control the progress he had
both were just the product of molecules behaving according to the laws of 96 T H E F A M I LY T H AT C O U L D N ’ T S L E E P chemical bonds. Griffith’s idea would explain why you could stop scrapie only by denaturing the scrapie protein. It would also explain why there were no antibodies to the protein in sufferers from FFI, like Pietro and his family, or from sufferers of Creutzfeldt-Jakob disease; the lethal proteins came from the victims themselves. Griffith was not well placed to confute
initiative around the disease. Soon, other young members of the group were asking about a cure. Ignazio was cautious. “Work toward a cure is going on,” he said, “but the disease is difficult.” Publicity, he added, would speed things up. That was where I came in. Until recently the family members had all kept silent, even to one another. They saw isolation and denial as selfprotection. And some people at the reunion regarded me warily. “Why are you here?” one of the young men asked me. Yes, I was
years—a few cows in Surrey, a few in Hertfordshire. In the early 1980s it began to intensify, and the more interested vets began to notice it. One of them, Raymond Williams, was thirty-four years old when he saw a sick cow in the fall of 1983 on a farm in Wiltshire in southern England. The cow was anxious, kept to itself along the edges of the walk from the milking parlor to the examination pen, and was losing weight even though she ate normally. It was sometimes too aggressive to be milked. The
