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 Factory

Product Details

• Amazon Sales Rank: #421653 in Books
• Published on: 2006-09-05
• Released on: 2006-09-05
• Original language: English
• Number of items: 1
• Binding: Hardcover
• 336 pages

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

The Scourge of Prions
In 1765 a doctor in Venice died of what was labeled "an organic defect of the heart's sack", but he many have been the first recorded victim of a strange disorder passed down to his many descendants into the twenty-first century. It had so many weird symptoms and was so rare that the victims were frequently misdiagnosed, often being dismissed as alcoholics in withdrawal, or as having meningitis, depression, encephalitis, and many other incorrect labels. The symptoms are appalling. The illness strikes adults who have no previous significant medical problems and may have started families of their own. A victim begins to hold up the head stiffly, and then sweats profusely; family members are terrified when these initial symptoms appear, as the others follow inexorably. The pupils contract to pinpoints, the heart goes mad with increased pulse and blood pressure, and sleep becomes impossible, no matter what drugs are used to bring it on. The victim knows what is happening until dementia takes over, followed by a coma and then death in about a year or two after the symptoms began. Nothing at all can be done to stop the progress of the illness, which is passed to one half of each succeeding generation. It is, however, becoming more comprehensible as we learn more about prions, those bad proteins. In _The Family That Couldn't Sleep: A Medical Mystery_ (Random House), D. T. Max has not only told the story of this particular illness, but also of other illnesses that are (or might be) caused by prions. It is a tale full of undeserving victims and flawed heroes, and it tells just how far we are from solving some basic biological riddles.

Proteins are what DNA codes for; because prions aren't alive, they cannot be killed; radiation, formaldehyde, and all ordinary sterilization procedures do nothing to them. You might get prions by having your DNA code for them; that's what happens in the Italian family that has Fatal Family Insomnia (FFI). That's pretty rare, but you might also get prions by eating them, as in eating cows with Mad Cow Disease. There are prion diseases of sheep and deer as well. A strange neurological disease in New Guinea called kuru unlocked some of the mysteries of prions (in this case, passed by cannibalism), and Nobel prizes have stemmed from this work. One of the frustrating parts of this story is that prion afflictions have often been brought about by people. No one intended to get any animal or human sick, but human intervention made it happen. Scrapie started afflicting sheep two centuries ago as a direct result of intensive breeding to make bigger animals. Mad Cow Disease was caused by the unnatural feeding of sheep cadaver protein to cows. Chronic Wasting Disease in deer seems to have been passed to them when sheep were held in pens used for sheep with scrapie, but also may come about when deer farms, pressed to produce bucks with bigger racks for hunters, fed the deer the same sort of sheep cadaver protein that the cows got. It also got spread when humans transported these deer to different regions of the country, a migration they could not have done on their own. We attempt to control nature, and in response, nature presents us with problems no one could have foreseen.

Max has given a clear history of prion diseases and our attempts to understand them. A surprising part of the prion story is that it gives evidence that our hominid forebears practiced cannibalism, and therefore the genes of most people show a protective trait that helps keep prions from causing disease. The positive part of the story is that we are not as ignorant about prions as we were four decades ago, and even that prion research may open up answers to possibly related neuromuscular and neurodegenerative diseases like Parkinson's and Alzheimer's. But what we know is overbalanced by what we don't. Max writes, "Not since Pasteur's time have researchers attempted to counter an infection knowing so little about what they are fighting." Not only that, but prion diseases are all brand new diseases, as diseases go, and some have been manufactured in the past few decades; there is no telling what completely new one will be in tomorrow's headlines.

A Real-life Mystery with All the Elements of a Fictional Blockbuster
Spanning two centuries, this book traces the origins of prions (and the terrible diseases they cause) to our current state of understanding.

The author's treatment makes this story stand out. What might have been a dry recital of discovery becomes of tale of greed, discovery, ego, opportunities both missed and taken, and the rigidity of belief. Along the way, we meet a family cursed with a genetic heritage that destroys lives with a disease that leaves the sufferer unable to sleep and fully conscious of a horrible fate.

I was particularly interested in this book because prion disease in humans is sometimes misdiagnosed as Alzheimer's. I came away with a clearer understanding of the types of prion disease and how they differ from each other and from Alzheimer's. I only wish the book could have ended with a clear answer to prevention and cure, but perhaps when that comes, Max will favor us with another tale.

Highly recommended!

Phyllis Staff, Ph.D.
author:
"How to Find Great Senior Housing," and
"128 Ways to Prevent Alzheimer's and Other Dementias"

The Family That Couldn't Sleep
This book was so fascinating, I was compelled to email the author and congratulate him on his work. The Family That Couldn't Sleep tells the thought provoking story of an emerging medical mystery that is likely to affect all of us in our lifetime. Woven into the story are fascinating details regarding the history, politics and evolving research of a potential health related epidemic in our country. Max is able to communicate a complex disease phenomena in a gripping fashion that is also accessible to lay people such as myself...medical degree not required!