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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.
The author carries us from the Italian family to other prion diseases, such as the sheep disease known as scrapie. In the 1820s, the mysterious illness threatened England's robust sheep business. Sheep afflicted with scrapie suffer such an itch that they scrape themselves raw against walls and fences. Eventually the sheep die. The many scientists who tried to discover the cause of scrapie failed.
In New Guinea in the 1950s, doctors discovered locals suffering another bizarre and puzzling illness, known as kuru. Victims, mostly women and girls, shivered, became glassy-eyed and cross-eyed, lost their balance, and died. Eventually scientists stumbled upon a connection to scrapie. Yet no one could prove exactly what the infectious agent was that caused these diseases until a chemist named Stanley Prusiner decided it was a "proteinaceaous infectious particle," which he dubbed a "prion." Prusiner created quite a stir in the scientific community, but mad cow was on the horizon --- and the experts' work had barely begun.
THE FAMILY THAT COULDN'T SLEEP reads like a combination anthropological and medical history, horror tale and sympathetic unveiling of a cursed family. At heart it is a well-told medical mystery. The colorful characters (including cannibals and quirky scientists) are detailed in a manner that makes them live on the page. The reader views the interlocking medical mysteries through the empathetic lens of the author. I highly recommend this book as a fascinating and terrifying read.
--- Reviewed by Terry Miller Shannon (terryms2001@yahoo.com)
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