The Family That Couldn't Sleep: A Medical Mystery

The Family That Couldn't Sleep: A Medical Mystery

4.4 9
by D.T. Max
     
 

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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,

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.

Editorial Reviews

The stories read like Outer Limits plots: A Stone Age tribe in Papua New Guinea is decimated by a mysterious illness that afflicts its victims with laughter and then death. A noble Venetian family dynasty is cursed by a disease that turns them into insomniacs. Thousands of wild deer perish of starvation in high fields of grass. Unfortunately, these Ripley's Believe or Not-worthy episodes possess more than passing interest; they signal the advance guard of diseases that may lurk as time bombs around the globe. D. T. Max's The Family That Couldn't Sleep can be read as a fascinating study of brain proteins and neurological diseases or as a full-blast medical wake-up call.
Natalie Angier
In this gripping, cleanly written, cannily plotted and elegantly educational book, D. T. Max shows us what happens when the insomnia isn't ordinary and doesn't end, no matter how aggressive the medical intervention or generous the sedative prescription…the book brims with great tales, some tragic, others cautionary.
—The New York Times
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 2006 Reed Business Information.

Product Details

ISBN-13:
9781588365583
Publisher:
Random House Publishing Group
Publication date:
09/05/2006
Sold by:
Random House
Format:
NOOK Book
Pages:
336
Sales rank:
531,170
File size:
462 KB

Meet the Author

D. T. Max was born and raised in New York City and graduated from Harvard in 1984. He has been an editor at Washington Square Press, Houghton Mifflin, and The New York Observer. For the past eight years, he has reported mostly for The New York Times Magazine. His work has also appeared in the Los Angeles Times, The Wall Street Journal, San Francisco Chronicle, and Chicago Tribune. He lives outside Washington, D.C., with his wife, their two young children, and a rescued beagle named Max.


From the Hardcover edition.

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Family That Couldn't Sleep: A Medical Mystery 4.5 out of 5 based on 0 ratings. 10 reviews.
AardvarkGal More than 1 year ago
Engrossing, well-written accounts of prion disorders in various species. Those with even the most basic biological understanding will have no trouble grasping the science behind the afflictions described. Absolutely fascinating - I couldn't put it down.
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