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The Family That Couldn't Sleep: A Medical Mystery [Paperback]

D.T. Max
4.6 out of 5 stars  See all reviews (47 customer reviews)

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Book Description

September 11, 2007
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.

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Editorial Reviews

From Publishers Weekly

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.
Copyright © Reed Business Information, a division of Reed Elsevier Inc. All rights reserved. --This text refers to an out of print or unavailable edition of this title.

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 --This text refers to an out of print or unavailable edition of this title.

Product Details

  • Paperback: 336 pages
  • Publisher: Random House Trade Paperbacks; 1 Reprint edition (September 11, 2007)
  • Language: English
  • ISBN-10: 081297252X
  • ISBN-13: 978-0812972528
  • Product Dimensions: 5.2 x 0.7 x 8 inches
  • Shipping Weight: 9.6 ounces (View shipping rates and policies)
  • Average Customer Review: 4.6 out of 5 stars  See all reviews (47 customer reviews)
  • Amazon Best Sellers Rank: #467,512 in Books (See Top 100 in Books)

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Customer Reviews

Most Helpful Customer Reviews
67 of 70 people found the following review helpful
5.0 out of 5 stars The Scourge of Prions December 27, 2006
Format:Hardcover
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.
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32 of 33 people found the following review helpful
Format:Hardcover
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"
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24 of 25 people found the following review helpful
5.0 out of 5 stars The Family That Couldn't Sleep October 26, 2006
Format:Hardcover
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!
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Most Recent Customer Reviews
5.0 out of 5 stars Interesting
Very well written book. If you are interested in an introduction to prion diseases, this is a great way to be introduced to it. Read more
Published 1 month ago by S. Devine
2.0 out of 5 stars Interesting but a little bland
This was an interesting book and you can tell that a lot of research went into the work that was done. However overall I found myself fairly bored when reading it. Read more
Published 2 months ago by Ambrosia Jefferson
4.0 out of 5 stars It is still legal to feed cows chicken feces...
Prions, cows eating chicken turds, and an Italian family locked into a diaphoretic demise--D.T. Max, a fellow sufferer of unfolded proteins himself, presents the obvious that has... Read more
Published 3 months ago by Peter Filak
5.0 out of 5 stars Fascinating delve in to the blossoming world of prion diseases
Couldn't put it down. I also cannot imagine the unfortunate families who suffer from FFI and hope that continued research opens up new understanding into this strange disease. Read more
Published 4 months ago by danno
4.0 out of 5 stars Interesting Medical Mystery
I really enjoyed this book. D.T Max manages to hit the right balance between heavier scientific and historical information against the personal narrative of people who've... Read more
Published 4 months ago by Amy Thibodeau
5.0 out of 5 stars great book!
I am aspiring medical scientist, and I devoured your book about FFI over a weekend. I think you're a brilliant writer, would like to thank you for your work. Keep it going!
Published 7 months ago by Paulius
4.0 out of 5 stars So good, I couldn't sleep...
I love reading about interesting medical cases and diseases, so a book about prions is right up my alley. Read more
Published 7 months ago by Vivos Mortuus
4.0 out of 5 stars Interesting and informative
This book tells the story of the family that can't sleep but also other prion diseases. I learned a lot and got a little frightened.
Published 12 months ago by MartyM
5.0 out of 5 stars Sleep without dreams
"The Family that couldn't sleep" is a powerful, tragic story about a Venetian family afflicted for centuries with a disease that renders them incapable of sleep. Read more
Published 16 months ago by Amaranth
5.0 out of 5 stars Engrossing
This book is a well written mix of scientific detail/development alternating with anecdotal effects of the prion diseases. I couldn't put it down.
Published 18 months ago by Janis M. Fitzgerald
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