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The Family That Couldn't Sleep: A Medical Mystery by D.T. Max
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The Family That Couldn't Sleep: A Medical Mystery

by D.T. Max

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Despite its sad subject this book was very good. I found it difficult to put down. Mr. Max looks at prion diseases which are as varied as scrapie in sheep, mad cow disease, Creutzfeldt-Jacob disease and Fatal Familial Insomnia - hence the title. The book opens with the title family in Italy which has been plagued with FFI for at least two hundred years. Max presents lots of science and the entire history of the discovery of prions and how they cause disease. There is., as yet, no cure for any of them. Scrapie and mad cow can be controlled and, I suppose, FFI could be bred out of existence but we are talking of humans. The remains of the Italian family have now all been tested and could know which of them will likely get the disease and/or pass it on. To a man/woman they have refused to know these results. If there were a cure they would likely feel differently.

The science is very simply and concisely explained and the history of discovery is full of characters. A very enjoyable book. ( )
CandySchultz | Feb 18, 2009 |  
A fascinating look at prions - rogue proteins that fold, creating chain reactions of folded proteins in human cells. Prions are not "germs" - neither viruses nor bacteria. They are not living entities, and yet they cause diseases in humans - terrifying illnesses like "mad cow disease." Max makes complicated medical science a compelling, and comprehensible, read. ( )
Vidalia | Dec 1, 2007 |  
A very well-written book on the latest research on prions and prion caused diseases (kuru, scrapie, mad cow disease etc), interspersed with a leit-motif of the history of a Venetian family suffering from the fatal familial insomnia- also a prion caused disease- robbing the victims first of sleep and then leading quickly to his or her death amidst a lot of suffering.
A prion is a protein that is neither bacterial, fungal nor viral and contains no genetic material. It occurs normally in a harmless form, but by folding into an aberrant shape, the normal prion turns into a rogue agent. It then makes other normal prions to become aberrant prions and wreaks havoc in the brain of the victim leaving it with holes and plaques resembling those of Alzheimer’s patients.
( )
Niecierpek | Aug 22, 2007 | 1 vote
Fascinating linkage betwen prion and misfolded protein diseases, very easy and interesting read - interspersing prion science with thehistory of one Italian family's Fatal familial insomnia. ( )
abuannie | Jul 17, 2007 |  
In sharing the story of an Italian family beset by a strange medical illness that causes insomnia and eventually death, the author also explains the history of that rare class of illness known as prion diseases. Much is still unknown about them, but he goes through the history of how what we do know was discovered. Prion diseases, like scrapie in sheep and mad cow disease in humans and cattle, are caused by a malformed protein. The author explains things pretty well this book does get dry in places. If you are interested in medical stuff or have a personal interest in prion diseases then this book will interest you. Otherwise it might not, but I did find the stuff about mad cow disease a bit frightening, as still so much is unknown about how it is spread, who might be exposed, and there is no cure. ( )
debs4jc | Jul 16, 2007 |  
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Amazon.com Product Description (ISBN 081297252X, Paperback)

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.

(retrieved from Amazon Fri, 24 Apr 2009 07:58:12 -0400)

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