The Family That Couldn't Sleep: A Medical Mystery

By D.T. Max

Book Overview

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

• Format: Paperback
• Language: English
• ISBN: 081297252X
• ISBN13: 9780812972528
• Release Date: September 2007
• Publisher: Random House Trade
• Length: 336 Pages
• Weight: 0.60 lbs.
• Dimensions: 0.6" x 5.2" x 8.0"

Customer Reviews

An excellent overview of Prion diseases

This book covers a wide range of Prion diseases and explains them quite well. I don't recommend it if you're one who's paranoid about what's in your food to begin with though.

barely focused on the family

its great if you want the history of prion disease but i was more interested in their story not mad cow and the history of it. title is misleading.

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

A fascinating and terrifying read

A prion disease is an illness caused by a protein that has become deformed. A family in Italy suffers from an inherited prion disease called fatal familial insomnia (FFI). The disease usually strikes its victims when they reach their 50s. As one might guess from the name, FFI victims lose the ability to sleep. When the disease first strikes, they perspire, the pupils of their eyes shrink, and they hold their head in a stiff, awkward manner. Eventually, they can no longer walk. In a prolonged exhausted state until death, the patients are completely aware of what is happening to them. The Italian family was once the only known group with fatal familial insomnia. Now, however, there are around 40 families around the world known to have the disease. The family has shunned publicity as much as possible. However, when they learned that author D. T. Max suffers from a neuromuscular disease that is also related to protein misinformation, family members began to hope that publicity would speed work toward a cure. Research uncovering information about FFI hopefully could help other diseases in which proteins become deformed. Prion diseases fascinate scientists. They seem to be the only ones that attack in three forms: inherited, infectious and by random chance. Researchers think prions are unique because, although they are proteins, they can infect like viruses and bacteria. Because it is so difficult to disinfect a prion, experts go to great lengths to avoid contamination. Radiation, boiling and heat won't kill prions. Scientists once opened a human brain afflicted with prion disease after the victim had been dead 20 years, and injected the brain tissue into lab animals. They all died of the disease. The author carries us from the Italian family to other prion diseases, such as the sheep disease known as scrapie. In the 1820s, the mysterious illness threatened England's robust sheep business. Sheep afflicted with scrapie suffer such an itch that they scrape themselves raw against walls and fences. Eventually the sheep die. The many scientists who tried to discover the cause of scrapie failed. In New Guinea in the 1950s, doctors discovered locals suffering another bizarre and puzzling illness, known as kuru. Victims, mostly women and girls, shivered, became glassy-eyed and cross-eyed, lost their balance, and died. Eventually scientists stumbled upon a connection to scrapie. Yet no one could prove exactly what the infectious agent was that caused these diseases until a chemist named Stanley Prusiner decided it was a "proteinaceaous infectious particle," which he dubbed a "prion." Prusiner created quite a stir in the scientific community, but mad cow was on the horizon --- and the experts' work had barely begun. THE FAMILY THAT COULDN'T SLEEP reads like a combination anthropological and medical history, horror tale and sympathetic unveiling of a cursed family. At heart it is a well-told medical mystery. The colorful characters (including cannibals and

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!

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"