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.
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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.
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.
Venice was a hereditary oligarchy. Its ruling class—its doges, procurators, and ambassadors—were drawn from two hundred families whose names, like that of the Quirini, had been inscribed in a “book of nobility” in the early fourteenth century. The Venetian doctor descended from one of these patrician families, great merchants and secretaries of state, whose surname adorned one of the central squares of the city. Though he was not himself entitled to wear the red toga that indicated patrician status, he enjoyed many of the other privileges of high birth in the republic.
The doctor had a three-story palazzo on a canal and a country home in the Veneto (both still standing today). The country house was in a town near the Piave River, a trip the doctor could make in three days, by crossing the lagoon by gondola to Portegrandi and then continuing in a carriage. In the country, on terra firma, a doctor of good family could play cards and chess with well-born friends, and supervise his gardens and the collection of rents from his tenant farmers, all the while staying clear of the infections summer brought to Venice.
If the doctor did have fatal familial insomnia—the idea has been suggested by his descendants in their search for the origin of their mutation—he likely would have noticed his first symptoms there in the summer of 1764. His servants, seeing his glassy eyes and sweaty forehead, might have gossiped that a witch had hexed him, but the doctor would not have entertained that idea for a moment. He was a graduate of the School of Medicine at Padua, the best in Europe. For him, sickness was not a magical but a natural process. The scientific method was blossoming in the Venetian state, of which Padua was a part. Its secular saint, Galileo, had set out the goal for all to see. “Science,” he had written, could be found “in a huge book that stands always open before our eyes—I mean the universe.” To read it, though, you needed to understand the language it was written in: “the language is mathematics.” A physician’s job, then, was to substitute exact observation for speculation, to look with vision unclouded by metaphysics or theology.
The sleepless doctor could trace his medical pedigree directly to Galileo. Galileo had taught the monk Castell, who had taught Borelli, who had taught the anatomist Malpighi, who had taught Valsalva, who had taught Morgagni, who had taught him. They had many of them learned their craft in the Acquapendente in Padua, the most beautiful autopsy theater in Europe. Its wooden balconies rose up in a narrowing series of concentric ovals—a “high funnel,” as Goethe described it—from which the audience could look down on the human body lying in Euclidean splendor. Italian doctors were brilliant with their scalpels; seen from above, their work would have seemed to promise to the eager medical student the doctor had once been that if you studied nature hard enough, it would give up its secrets.
That promise wasn’t working for him now, though. He was no longer sleeping well and he did not know why. In the beginning, the feeling might not have been unpleasant—he could stay up all night playing cards or maybe read Morgagni’s famous comparisons of the body to a machine, published just a few years before. Just as any machine needed rest to prepare for the next day, so did the human. Yet the doctor’s machinery seemed to be running nonstop. He was sweating more and more and his servants would by now be bringing him fresh shirts several times a day.
If the doctor had turned to contemporary authorities on insomnia, he would have found little of use. Sleep, even now a mystery in many ways to science and medicine, was utterly obscure to the doctors of his time, who were still struggling to understand what went on in plain sight in the day. Indeed, the unsleeping doctor would have had to go all the way back to Galen, the ancient Roman physician whose teachings had dominated medicine until Galileo had challenged him a century before. Galen had learned most of what he knew about sleep from Aristotle, who had pronounced confidently on the question (as he had on most scientific issues). “All things that have a natural function,” he wrote authoritatively in On Sleep and Dreams, “must, whenever they exceed the time for which they can do a certain thing, lose their capacity and cease from doing it, e.g., the eyes from seeing. Necessarily, everything that wakes must be capable of sleeping. For it is unable to be active at all times.” Aristotle proposed that sleep was a byproduct of eating. After a person had a big meal the warm fumes from the process of digestion entered the veins, thence to the brain, and thither to the heart, the seat of consciousness, where their cooling brought about sleep. Galen, writing five centuries after him, corrected Aristotle— the warm meal went directly to the brain, where it plugged the pores by which sensations entered and exited.
It is unlikely the Venetian doctor took either Galen or Aristotle seriously. Both would have seemed speculative to him when compared to the certainties of the Acquapendente. Still, back in Venice now, to be on the safe side, he might have ordered his cook to produce bigger meals—roast fowls and hams and sausages and fish from the Adriatic doused in the heavy French sauces that were newly in style. If so, the results would have confirmed his skepticism about the learnedness of anyone who had not gone to Padua. He would have sat awake in a chair, his stomach distended, waiting for the meal to warm his head. To help the process along he would have had plenty of wine, as well—wine was particularly recommended for insomnia—and then sat up through the night until the sun rose over the Lido.
Or maybe he got up and went out, frustrated by the noise—the hawkers and prostitutes and gondoliers singing to one another filled the nighttime air of a city in which, as the fussy Goethe noted, “the people…appreciate volume more than anything else.” If it was Lent, the doctor could have gone to one of the huge carnevale masked balls in the palaces on the Grand Canal, where, as a man of noble birth, he was always welcome. There, dressed as the medico della peste, he could have kept his vigil. It was the sort of wit Venetians admired—a doctor in the black cape, the long white nose, and the face mask of the plague fighter. Dressed this way, the only evidence of his problem would have been the twin pinpricks of his eyes, peering from behind his mask. He’d have come home to the sound of the Marangona, the great bell in Piazza San Marco, tolling the beginning of the work day, sleep still well beyond his grasp.
The doctor would have asked himself whether he had caught something. Infection was on the mind of mid-eighteenth-century Venice. The city had a public health department well versed in the subject. They burned the bedding of disease victims and left their clothes out in the sun and air for a week to help neutralize the contagion. But what were they neutralizing? Here there was less agreement. The predominant view was that infection was an invisible substance carried in the air as smell. Thus the plague doctor costume of carnevale had a sponge at the tip of the long white nose, and workers who disinfected clothing for the Venetian authorities were also required to perfume the room and the personal effects of the deceased.
But in the lexicon of disease there was no infection that corresponded to what the doctor was feeling. He was not just hot but extraordinarily anxious, like a horse at full gallop, sweaty and prone to a shaking that seemed to come from deep within. He was exhausted, falling in and out of a light, dream-wracked sleep. His servants might have heard him knocking on his own window, thinking it was a door, or spied him preparing the leeches for use, swishing around imaginary water in the dark glass jar where he kept them. The servants would have gone into his room to waken him, followed by his distraught wife. He would not remember sleeping, nor would he feel at all rested. “I’m tired”—“Mi so straco,” he would say in Venetian. And when the servants had left the room, to his wife alone he would express his deepest horror: “Mi divento mato.” Was he indeed going crazy? Was he destined for the ships of fools, those boats in the farthest parts of the lagoon where the Serenissima housed the poor souls from whom God had taken the power of reason?