The Emperor of All Maladies. Siddhartha Mukherjee

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The Emperor of All Maladies - Siddhartha  Mukherjee


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and like all stories, theirs have gaps and inconsistencies. The “cancers” described by them may have been true neoplasms, or perhaps they were hazily describing abscesses, ulcers, warts, or moles. The only incontrovertible cases of cancer in history are those in which the malignant tissue has somehow been preserved. And to encounter one such cancer face-to-face—to actually stare the ancient illness in its eye—one needs to journey to a thousand-year-old gravesite in a remote, sand-swept plain in the southern tip of Peru.

      The plain lies at the northern edge of the Atacama Desert, a parched, desolate six-hundred-mile strip caught in the leeward shadow of the giant furl of the Andes that stretches from southern Peru into Chile. Brushed continuously by a warm, desiccating wind, the terrain hasn’t seen rain in recorded history. It is hard to imagine that human life once flourished here, but it did. The plain is strewn with hundreds of graves—small, shallow pits dug out of the clay, then lined carefully with rock. Over the centuries, dogs, storms, and grave robbers have dug out these shallow graves, exhuming history.

      The graves contain the mummified remains of members of the Chiribaya tribe. The Chiribaya made no effort to preserve their dead, but the climate is almost providentially perfect for mummification. The clay leaches water and fluids out of the body from below, and the wind dries the tissues from above. The bodies, often placed seated, are thus swiftly frozen in time and space.

      In 1990, one such large desiccated gravesite containing about 140 bodies caught the attention of Arthur Aufderheide, a professor at the University of Minnesota in Duluth. Aufderheide is a pathologist by training but his specialty is paleopathology, a study of ancient specimens. His autopsies, unlike Farber’s, are not performed on recently living patients, but on the mummified remains found on archaeological sites. He stores these human specimens in small, sterile milk containers in a vaultlike chamber in Minnesota. There are nearly five thousand pieces of tissue, scores of biopsies, and hundreds of broken skeletons in his closet.

      At the Chiribaya site108, Aufderheide rigged up a makeshift dissecting table and performed 140 autopsies over several weeks. One body revealed an extraordinary finding. The mummy was of a young woman in her midthirties, found sitting, with her feet curled up, in a shallow clay grave. When Aufderheide examined her, his fingers found a hard “bulbous mass” in her left upper arm. The papery folds of skin, remarkably preserved, gave way to that mass, which was intact and studded with spicules of bone. This, without question, was a malignant bone tumor, an osteosarcoma, a thousand-year-old cancer preserved inside of a mummy. Aufderheide suspects that the tumor had broken through the skin while she was still alive. Even small osteosarcomas can be unimaginably painful. The woman’s pain, he suggests, must have been blindingly intense.

      Aufderheide isn’t the only paleopathologist to have found cancers in mummified specimens. (Bone tumors, because they form hardened and calcified tissue, are vastly more likely to survive over centuries and are best preserved.) “There are other cancers found in mummies where the malignant tissue has been preserved. The oldest of these is an abdominal cancer from Dakhleh in Egypt from about four hundred AD,” he said. In other cases, paleopathologists have not found the actual tumors, but rather signs left by the tumors in the body. Some skeletons were riddled with tiny holes created by cancer in the skull or the shoulder bones, all arising from metastatic skin or breast cancer. In 1914, a team109 of archaeologists found a two-thousand-year old Egyptian mummy in the Alexandrian catacombs with a tumor invading the pelvic bone. Louis Leakey,110 the anthropologist who dug up some of the earliest known human skeletons, also discovered a jawbone dating from two million years ago from a nearby site that carried the signs of a peculiar form of lymphoma found endemically in southeastern Africa (although the origin of that tumor was never confirmed pathologically). If that finding does represent an ancient mark of malignancy, then cancer, far from being a “modern” disease, is one of the oldest diseases ever seen in a human specimen—quite possibly the oldest.

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      The most striking finding, though, is not that cancer existed in the distant past, but that it was fleetingly rare. When I asked Aufderheide about this, he laughed. “The early history of cancer,”111 he said, “is that there is very little early history of cancer.” The Mesopotamians knew their migraines; the Egyptians had a word for seizures. A leprosy-like illness112, tsara’at, is mentioned in the book of Leviticus. The Hindu Vedas have a medical term for dropsy and a goddess specifically dedicated to smallpox. Tuberculosis was so omnipresent and familiar to the ancients that—as with ice and the Eskimos—distinct words exist for each incarnation of it. But even common cancers, such as breast, lung, and prostate, are conspicuously absent. With a few notable exceptions, in the vast stretch of medical history there is no book or god for cancer.

      There are several reasons behind this absence. Cancer is an age-related disease—sometimes exponentially so. The risk of breast cancer113, for instance, is about 1 in 400 for a thirty-year-old woman and increases to 1 in 9 for a seventy-year-old. In most ancient societies, people didn’t live long enough to get cancer. Men and women were long consumed by tuberculosis, dropsy, cholera, smallpox, leprosy, plague, or pneumonia. If cancer existed, it remained submerged under the sea of other illnesses. Indeed, cancer’s emergence in the world is the product of a double negative: it becomes common only when all other killers themselves have been killed. Nineteenth-century doctors often linked cancer to civilization: cancer, they imagined, was caused by the rush and whirl of modern life, which somehow incited pathological growth in the body. The link was correct, but the causality was not: civilization did not cause cancer, but by extending human life spans—civilization unveiled it.

      Longevity, although certainly the most important contributor to the prevalence of cancer in the early twentieth century, is probably not the only contributor. Our capacity to detect cancer earlier and earlier, and to attribute deaths accurately to it, has also dramatically increased in the last century. The death of a child with leukemia in the 1850s would have been attributed to an abscess or infection (or, as Bennett would have it, to a “suppuration of blood”). And surgery, biopsy, and autopsy techniques have further sharpened our ability to diagnose cancer. The introduction of mammography to detect breast cancer early in its course sharply increased its incidence—a seemingly paradoxical result that makes perfect sense when we realize that the X-rays allow earlier tumors to be diagnosed.

      Finally, changes in the structure of modern life have radically shifted the spectrum of cancers—increasing the incidence of some, decreasing the incidence of others. Stomach cancer, for instance, was highly prevalent in certain populations until the late nineteenth century, likely the result of several carcinogens found in pickling reagents and preservatives and exacerbated by endemic and contagious infection with a bacterium that causes stomach cancer. With the introduction of modern refrigeration (and possibly changes in public hygiene that have diminished the rate of endemic infection), the stomach cancer epidemic seems to have abated. In contrast, lung cancer incidence in men increased dramatically in the 1950s as a result of an increase in cigarette smoking during the early twentieth century. In women, a cohort that began to smoke in the 1950s, lung cancer incidence has yet to reach its peak.

      The consequence of these demographic and epidemiological shifts was, and is, enormous. In 1900, as Roswell Park noted, tuberculosis was by far the most common cause of death in America. Behind tuberculosis came pneumonia (William Osler, the famous physician from Johns Hopkins University, called it “captain of the men of death”114), diarrhea, and gastroenteritis. Cancer still lagged115 at a distant seventh. By the early 1940s, cancer116 had ratcheted its way to second on the list, immediately behind heart disease. In that same span, life expectancy among Americans117 had increased


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