Product Description
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 FactoryFrom the Hardcover edition.

CUSTOMER REVIEWS (Average Customer Rating: 5.0 based on 26 reviews)

DT Max writes the back story on prion disease by Brian E. Moore (Central Illinois)

5 Stars
June 01, 2009
Most neuropathologists accept the party line that there are three basic forms of Creutzfeldt-Jakob disease (CJD): acquired, inherited, and sporadic. The general neurology and neuropathology textbooks do not acknowledge that the very existence of sporadic CJD is controversial. But D.T. Max, in his book The Family That Couldn't Sleep: A Medical Mystery (2006), does not skirt this controversy. Some patient advocacy groups claim that CJD cases now classified as sporadic are in fact infectious. "A surprising number of mainstream scientists also doubt the existence of sporadic CJD -- among them protein experts, epidemiologists, and neurologists," Max writes. "Their objection is that sporadic CJD is an unnecessary idea. If a disease is known to spread by infection, why assume that some people also get it by chance? Why not find the infectious source in their cases as well? They see theoretical gaps in the idea of sporadic CJD theory too. For one thing, it is strange, if sporadic CJD comes about as a result of the body's declining ability as it ages to manufacture proteins correctly, that the chance of getting sporadic CJD goes down at around seventy years of age." Among the sporadic CJD doubters is none other than D. Carleton Gajdusek, who in 1976 shared a Nobel prize for his work in tracking the cause of kuru (a disease later classified among the prion disorders) as resulting from the practice of funerary cannibalism among the Fore tribes in New Guinea. Of course, in 1976, the word prion had not yet been invented. It took Stanley B. Prusiner, who himself won the Nobel in 1997 for his discovery of prions as a new biologic principle of infection, to come up with that catchy term. But Gajdusek (who died this past December) never took to the term "prion", preferring to call the infectious proteins "nucleating amyloids" -- in large part because of his rabid animosity toward Prusiner. Gajdusek addresses Prusiner in this spicy journal entry made at the time of the announcement of Prusiner's award: "I never heard a word of original thought from you nor read such ideas in anything you authored for which I did not recognize immediately its source, which you always went out of your way to obscure. You a heretic? You a martyr? You a defender of unacceptable ideas? Bull___! You shrewdly jumped onto a bandwagon of creative ideas and experimental work and shrewdly got on to the winning cart, proclaiming outrageously in press and media it was yours! I respect you less and less as your despicable game succeeds and you bask in your coveted fame." There is no doubt that the prejudice, jealousy, and ambition played a large role in both the development and elucidation of the prion diseases. D. T. Max has written a wonderful book about the fascinating history of this strange class of diseases -- including CJD, kuru, fatal familial insomnia, bovine spongiform encephalopathy, and scrapie. As the mysteries surrounding the prion diseases are further unraveled, the secrets behind the more common neurodegenerative diseases (which feature their own kinds of aberrant, aggregating proteins) may also begin to be revealed. [...]

Frightening and thought-provoking by Cassandra E. Kling (Greenwood, IN)

5 Stars
February 05, 2009
Informative, riveting and one of the most compelling books I've read in years. Max starts with the story of an Italian family with a timebomb lurking in their genes and segues into the history and etiology of prion disease. Mankind is truly the author of its own destruction as we seek to bend nature to our will. May make you into a vegetarian after you finish reading.

Great read by Sara White (Salt Lake City, UT USA)

5 Stars
June 16, 2008
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 awake by Teresa J. Molinaro (Pennsylvania)

5 Stars
March 06, 2008
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 one by ubat (Sao Paulo, Brazil)

5 Stars
January 13, 2008
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.

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