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The Family That Couldn't Sleep: A Medical Mystery

The Family That Couldn't Sleep: A Medical Mystery
By D.T. Max

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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.

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.


Product Details

  • Amazon Sales Rank: #109395 in Books
  • Published on: 2007-09-11
  • Released on: 2007-09-11
  • Original language: English
  • Number of items: 1
  • Binding: Paperback
  • 336 pages

Editorial Reviews

From Publishers Weekly
In 1765, Venetian doctors were stumped by the death of a man who had suffered from insomnia for more than a year and spent his final months paralyzed by exhaustion. Over the next two centuries, many of his descendants would develop similarly fatal symptoms, with a range of misdiagnoses, from encephalitis to alcohol withdrawal. Finally, in the early 1990s, their disease was recognized as a rare genetic form of prion disease. The family reluctantly shared their history with Max, who has written about science and literature for the New York Times Magazine and other publications. Max (inspired in part by his own neuromuscular disorder) has crafted a powerfully empathetic account of their efforts to make sense of their suffering and find a cure. But this is only half the story. Looking at prion disease in general, Max doubles back to the English mad-cow epidemic of the 1990s, retracing established backstories among New Guinea aboriginals and European sheep herds. There's enough fascinating material—in particular, a theory suggesting that early humans were nearly wiped out by a plague spread by cannibalism—to keep readers engaged, but they're likely to want still more about the genuinely captivating family drama. (Oct. 3)
Copyright © Reed Business Information, a division of Reed Elsevier Inc. All rights reserved.

From The New Yorker
Beginning with the story of an Italian clan whose members die of a mysterious inability to sleep, Max traces science's tortuous path toward understanding prion diseases—a category that includes scrapie in sheep, B.S.E. in cows, and kuru, a disease spread by cannibalism which decimated one New Guinean tribe. Victims of fatal familial insomnia lose control of neuromuscular function, existing in a merciless limbo between sleep and wakefulness until they die of exhaustion. For a half century, prion diseases have baffled scientists, because the transmission of illness by proteins, which are non-living, was considered impossible. Max, who suffers from a distantly related neuromuscular disease, narrates recent advances in prion science with engaging clarity. But, as he reflects ruefully, "the neurologist can diagnose you but he can't cure you."
Copyright © 2006 Click here to subscribe to The New Yorker

From Bookmarks Magazine
Framed by the story of one genetically cursed Italian family, The Family That Couldn't Sleep is a true medical mystery. D. T. Max, who conducted investigative research and interviews, has written a gripping, spooky, educational, and readable book with vast historical and geographic sweep, from 18th-century Italy and England to 1950s New Guinea. Its strong point is the dual focus on the Italian family's genetic legacy and the scientific discovery of prion diseases. The book wanders when it strays from these subjects, and Max milks too much sympathy for his Italians. Yet if Max stresses that scientists still have a long way to go to alleviate the threat of prions to humans, he also gives reason for hope.

Copyright © 2004 Phillips & Nelson Media, Inc.


Customer Reviews

Great read5
This book is a great book on the history of prions. Max easily illustrates how prions are connected to other important diseases such as alzheimers and diabetes. He flawlessly goes from past to present, connecting the two times with the venetian family who has a defective prion gene. It is really amazing that prions don't affect more people. It is also a wake up call for the beef industry in America.

will keep you awake5
This is a fascinating medical 'thriller', only it's real! it was nearly impossible to stop listening to it and i think anyone who likes medical thrillers or anything related to the medical field, would love this.
The book focuses on prions and their role in disease, especially 'mad cow disease'.

A story well told -- and, unfortunately, it's a true one5
This book does a lot to clear up the story of prions, what they are, what they do, how their threat is real. The Italian family who gives the story its title is but one instance of prions affecting human and animal life. The research is impeccable, and particularly interesting is the process by which medical and veterinary sciences came together to begin unraveling the prion mystery. Because, to be accurate, documentation on how livestock has been affected by prion disease had been, until recently, far more complete and detailed than human prion disease.

The author tells the story unemotionally, which is good, but the reading is far from arid or too technical. The human factor -- how scientists competed for the credit, sometimes damaging other professionals' reputations and careers -- makes it even more interesting. All this makes "The Family That Couldn't Sleep" a fundamental work for anyone who wants to understand these proteins better, and also for people curious about the inner workings of scientific research.