The Family That Couldn't Sleep

The Family That Couldn't Sleep: A Medical Mystery Paperback – September 11, 2007

Author: Visit Amazon's D.T. Max Page | Language: English | ISBN: 081297252X | Format: PDF, EPUB

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The Family That Couldn't Sleep: A Medical Mystery – September 11, 2007
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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."
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Books with free ebook downloads available The Family That Couldn't Sleep: A Medical Mystery – September 11, 2007

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: 8 x 5.2 x 0.6 inches
Shipping Weight: 9.6 ounces (View shipping rates and policies)
Amazon Best Sellers Rank: #96,987 in Books (See Top 100 in Books)
- #92 in Books > Medical Books > History

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.

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.