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.
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September 05, 2006
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Excerpt from The Family That Couldn't Sleep by D. T. Max
THE DOCTORS' DILEMMA VENICE, 1765
Who is it that says there is a great difference between a good physician and a bad one; yet very little between a good one and none at all
' ARTHUR YOUNG, Travels in Italy and France
In November 1765 a respected doctor from a good Venetian family died in the Campo Santi Apostoli, near the Jewish ghetto in Venice. The cause of his death was "an organic defect of the heart's sack" ' or so contemporary parish records state. In truth no one would have known for sure what he suffered from and we can't know either. But priests ordinarily only wrote detailed descriptions of diseases when what they saw was noteworthy, and it is intriguing that the description of the doctor's illness in the parish book is one of the longest of the year.
The entry records that the deceased suffered for more than a year from "intermittent difficulty in breathing" and adds that he was bedridden and "totally paralyzed for two months" before his death. Many of the doctor's descendants would experience similar symptoms in the course of dying from fatal familial insomnia, suggesting that the Venetian doctor may have been the earliest recorded case of a disease that has gone on to torment his relatives for more than two centuries.
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Mid-eighteenth-century Venice was a place of gaiety and vice. The city always had a fairy-tale aspect, but until the seventeenth century its irreality was checked by an appetite for business. Venice stood at the crossroads of east and west, Asia and Europe, and it avidly cashed in on its location. But with the colonization of the Americas, trade turned the other way ' across the Atlantic ' and Venice began spending down its inheritance. Goethe, visiting the city in 1786, noted that the Venetians' lagoon was silting up, their trade "declining, their political power dwindling ' Venice, like everything else that has a phenomenal existence, is subject to Time." The end was near, and everyone there knew it.
Venice's fall, though, was the time of its greatest opulence. This was the era of Casanova's wanton memoirs and the splendid Venetian regattas and processions painted by Canaletto and Francesco Guardi. One story may help to convey the moment: in 1709, there was a ball at the home of a Venetian noble in honor of the king of Denmark, Frederick IV. As the king danced with one of the guests, a newly married noblewoman named Caterina Quirini, his buckle caught a string of pearls that adorned the belt of her dress, scattering them on the floor. The lady paid them no mind. The king was about to bend down to retrieve the pearls, whereupon her husband stood up, walked across the dance floor, and crushed them under his feet, while his wife danced on.