(50 reviews) Author: ISBN : Product Detai New from Format: PDFWhat 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. They include 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.
- Audible Audio Edition
- Listening Length: 8 hours and 45 minutes
- Program Type: Audiobook
- Version: Unabridged
- Publisher: Tantor Audio
- Audible.com Release Date: October 3, 2006
- Language: English
- ASIN: B000JCE3F2
The Family That Couldn't Sleep: A Medical Mystery PDF
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.
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