by D. T. Max
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.
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.
Reviews-
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January 29, 2007
An engaging nonfiction "medical mystery" starts with the strange case of an Italian family whose members, upon reaching a certain age, succumb to a sort of sleeping disorder that causes not only insomnia but certain death. The cause of this disease is determined to be prions—infectious agents derived from proteins, not viruses—so Max explores other prion diseases, such as mad cow disease and kuru, and delves into the history of prion research as a way of unraveling the mysteries behind the disease that's been plaguing the titular family for generations. Gardner lets the material do most of the heavy lifting by narrating in a plain, unadorned style that keeps his own contributions to the narrative minimal, the auditory equivalent of transparent prose. The pacing and fascinating subject matter keep the listener fully engaged throughout, resulting in an audiobook that will certainly be no cure for insomnia. In fact, it might even warrant an advisory warning: side effects may include sleepless nights, caused by a strong desire to get to the next chapter. Simultaneous release with the Random House hardcover (Reviews, July 31). -
July 31, 2006
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. -
September 1, 2006
Just when you thought you were familiar with most of the really unusual medical ailments, along comes fatal familial insomnia (FFI), which has afflicted one Italian family for two centuries. The disease strikes in middle age. Symptoms include profuse sweating, constipation, impotence, and sudden onset of menopause. Eventually the body, deprived of sleep, begins to shut down. Fifteen months after the first symptoms appear, the patient dies. The brutal thing about FFI is that, while a lot is known about its effects, very little is known about its cause or its treatment (if there is a treatment). The book is like a nonfiction version of a Michael Crichton novel: an enigmatic illness, a family in jeopardy, a race against the clock to find a cure. Max, a science journalist, uses FFI as a springboard to tell a broader story about the history of fatal diseases for which cures are desperately needed, such as Parkinson's and mad cow. It would be a mistake to call this book "entertaining"--it's too unsettling and scary for that--but it's certainly very timely and compellingly written.(Reprinted with permission of Booklist, copyright 2006, American Library Association.) - Michael Pollan, author of The Omnivore's Dilemma and The Botany of Desire "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."
- Laurie Garrett, author of Betrayal of Trust and The Coming Plague "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."
- Katrina Firlik, MD, author of Another Day in the Frontal Lobe "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."
- Philip Weiss, author of American Taboo "A great book.D.T. Max has drawn the curtain on a cabinet of folly and malady that will stagger your imagination."
- David Plotz, author of The Genius Factory "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."
