The Family That Couldn't Sleep: A Medical Mystery

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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of throwing up his hands, as Introduction xxi generations of medical practitioners had done before him, he embraced the mystery, digging through records of the spectacular and confusing symptoms to get to the neurological alterations that were bringing about the deaths in his wife’s family. Meanwhile, Lisi, herself trained as a nurse, was on the telephone with her relatives, extracting information that they had left unexamined during two centuries of fear and humiliation. What did Rita die of

dried up when the wave retreated. But the mysterious disease was slowly being understood. In the 1930s, two French veterinarians injected healthy sheep with tissue from scrapie-affected Monkey Business 87 sheep. They had been trying to figure out whether scrapie was infectious or inherited, an inquiry that ended inconclusively many times before. This time, because they had noticed that sheep on farms rarely got scrapie before they were two years old, they gave the disease time to manifest

slowly when he speaks about disease, curbing his intense intelligence, always aware that the mind resists the idea of sickness in the body. While he has seen the misery FFI brings, as a doctor Ignazio can also admire its work—the extraordinary way Stanley Prusiner’s Nobelhonored prions hollow out the thalamus, the part of the brain that is destroyed by FFI, and how unusual this pathology is. Having also helped define a new disease, Ignazio has a sense of ownership (alongside horror at what FFI has

Silvano was a handsome man with the red hair of his great-grandmother Marianna, a strong brow, and a trim frame. With his graceful movements and elegant appearance, a pocket square always folded in his jacket, he could easily have been mistaken for a movie star. He loved movies, too, especially the poise its stars showed. Attractive to women, he never told his girlfriends about his fears that he would die young. For Silvano, intimacy was what you reserved for your family. He was very devoted to

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

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