The Body, page 35
Among the most puzzling of headaches are migraines. Migraine (the word is a corruption of the French demi-craine, meaning “half the head”) affects 15 percent of people but is three times more common in women than in men. Migraines are almost wholly a mystery. They are highly individual. Oliver Sacks in a book on migraines described nearly one hundred varieties of migraine. Some people feel surprisingly wonderful before migraines. The novelist George Eliot said she always felt “dangerously well” just before a migraine started. Others are indisposed for days and left feeling starkly suicidal.
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Pain is curiously mutable. It can be increased, attenuated, or even ignored by the brain depending on the situation. In extreme circumstances, it may not register at all. A famous instance was at the Battle of Aspern-Essling during the Napoleonic Wars, when an Austrian colonel, directing operations from horseback, was informed by his aide-de-camp that his right leg had been shot away.
“Donnerwetter, so it has,” replied the colonel phlegmatically, and kept on fighting.
Being depressed or worried will almost always increase perceived levels of pain. But equally pain is decreased by pleasant aromas, soothing images, pleasurable music, good food, and sex. Just having a sympathetic and loving partner cuts the reported pain of angina by half, according to one study. Expectation is hugely important, too. In one experiment done by Tracey and her team, when subjects in pain received morphine without being told, its analgesic effects were greatly lessened. In many ways, we feel the pain we expect to feel.
For millions of people, pain is a nightmare that cannot be escaped. According to the U.S. Institute of Medicine, part of the National Academy of Sciences, about 40 percent of adult Americans—100 million people—are experiencing chronic pain at any given moment. One-fifth of them will suffer it for more than twenty years. Altogether chronic pain affects more people than cancer, heart disease, and diabetes combined. It can be hugely debilitating. As the French novelist Alphonse Daudet noted in his classic In the Land of Pain (La doulou in French) almost a century ago, the pain that racked him as he was slowly ravaged by the effects of syphilis left him “deaf and blind to other people, to life, to everything except my wretched body.”
Medical science offered very little in the way of safe, lasting relief back then. We are not much further along now. As Andrew Rice, a pain researcher at Imperial College London, told Nature in 2016, “The drugs we have relieve 50 percent of pain in somewhere between one in four and one in seven of the patients we treat. That’s for the best drugs.” In other words, some 75 percent to 85 percent of people get no benefit at all from even the best pain drugs, and those who do get benefit don’t usually get much. Pain relief, as Tracey puts it, has been “a pharmacological graveyard.” Pharmaceutical companies have poured billions and billions into drug development but have not come up with a drug that controls pain effectively and doesn’t cause addictions.
One unhappy result has been the infamous opioid crisis. Opioids, as surely everyone knows by now, are painkillers that act in much the same way as heroin and come from the same addictive source: opiates. For a long time, they were mostly used sparingly, primarily for short-term relief after surgery or in the treatment of cancer. But in the late 1990s, pharmaceutical companies began pushing them as a long-term solution to pain. A promotional video made by Purdue Pharma, the maker of the opioid OxyContin, featured a physician who specializes in pain treatment looking straight into the camera and claiming with evident sincerity that opioids were perfectly safe and hardly ever addictive. “We doctors were wrong in thinking that opioids can’t be used long term. They can be and they should be,” he added.
The reality was rather different. People across America were becoming rapidly addicted and often dying. Between 1999 and 2014, by one estimate, a quarter of a million Americans died from opioid overdoses. Opioid abuse remains for the most part a peculiarly American problem. The United States has 4 percent of the world’s population but consumes 80 percent of its opioids. About two million Americans are thought to be opioid addicts. Another ten million or so are users. The cost to the economy has been put at over $500 billion a year in lost earnings, medical treatments, and criminal proceedings. Opioid use has become such big business that we have now reached the surreal situation that pharmaceutical companies are producing drugs to alleviate the side effects of opioid overuse. Having helped to create millions of addicts, the industry is now profiting from medications designed to make their addiction a little more comfortable. So far the crisis doesn’t seem to be going away. Every year opioids (both legal and illegal) claim forty-five thousand or so American lives, far more than are killed in car crashes.
The one positive to come out of the experience is that opioid fatalities have led to a rise in organ donations. In 2000, according to The Washington Post, fewer than 150 organ donors were opioid addicts; today the number is over 3,500.
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In the absence of pharmaceutical perfection, Irene Tracey focuses on what she calls “free analgesia”—understanding how people can manage their pain through cognitive-behavioral therapies and exercise.
“It’s been really interesting to me,” she says, “how useful neuroimaging has been to persuade people to engage with the brain to recognize that it does seem to have a big role in making pain bearable. You can achieve a lot with that alone.”
One of the great advantages with pain management is that we are marvelously suggestible, which is of course why the well-known placebo effect works. The concept of the placebo effect has been around for a very long time. In the modern medical sense of something given for psychological benefit, it is first recorded in a British medical text as far back as 1811, but the word “placebo” itself has existed in English since the Middle Ages. For most of its history, it meant a flatterer or sycophant. (Chaucer used it in that sense in The Canterbury Tales.) It comes from a Latin term meaning “to please.”
Nobody knows quite why placebos work, but they do. In one experiment, people who had just had a wisdom tooth extracted had their faces massaged with an ultrasound device and overwhelmingly reported feeling better. What was interesting was that the treatment worked as well with the machine turned off as on. Other studies have shown that people given a colored tablet with corners will report feeling better than when given a plain white tablet. Red pills are deemed more fast acting than white pills. Green and blue pills have a more soothing effect. Patrick Wall, in his book on pain, reported how one doctor got good results from handing his patients pills held in a forceps, explaining that they were too potent to be held by bare fingers. Extraordinarily, placebos are even effective when people know they are placebos. Ted Kaptchuk of the Harvard Medical School gave people suffering from irritable bowel syndrome sugar pills and told them that that’s all they were. Even so, 59 percent of those tested reported relief of symptoms.
The one problem with placebos is that while they are often effective for matters over which our mind has some control, they can’t help with problems that lie below the conscious level. Placebos don’t shrink tumors or banish plaque from narrowed arteries. But then, come to that, neither do more aggressive painkillers, and placebos at least have never sent anyone to an early grave.
20 WHEN THINGS GO WRONG: DISEASES
I came to typhoid fever—read the symptoms—discovered that I had typhoid fever, must have had it for months without knowing it—wondered what else I had got; turned up St. Vitus’s Dance—found, as I expected, that I had that too,—began to get interested in my case, and determined to sift it to the bottom, and so started alphabetically—read up ague, and learnt that I was sickening for it, and that the acute stage would commence in about another fortnight. Bright’s disease, I was relieved to find, I had only in a modified form, and, so far as that was concerned, I might live for years.
—JEROME K. JEROME ON READING A MEDICAL TEXTBOOK
I
IN THE AUTUMN of 1948, people in the small city of Akureyri, on the north coast of Iceland, began to come down with an illness that was at first taken to be poliomyelitis, but then proved not to be. Between October 1948 and April 1949, almost five hundred people, out of a population of ninety-six hundred, grew ill. The symptoms were wondrously diverse—muscle aches, headaches, nervousness, restlessness, depression, constipation, disturbed sleep, loss of memory, and generally being out of sorts but in a pretty serious way. The illness didn’t kill anyone, but it did make nearly every victim feel wretched, sometimes for months. The cause of the outbreak was a mystery. All tests for pathogens came back negative. The disease was so peculiarly specific to the vicinity that it became known as the Akureyri disease.
For about a year, nothing more happened. Then outbreaks began to occur in other, curiously distant places—in Louisville, Kentucky; in Seward, Alaska; in Pittsfield and Williamstown, Massachusetts; in a little farming community in the far north of England called Dalston. Altogether through the 1950s ten outbreaks were recorded in the United States and three in Europe. The symptoms everywhere were broadly similar but often with local peculiarities. People in some places said they felt unusually depressed or sleepy or had very specific muscle tenderness. As the disease proliferated, it attracted other names: post-viral syndrome, atypical poliomyelitis, and epidemic neuromyasthenia, by which it is most commonly known now. Why outbreaks didn’t radiate outward to neighboring communities but rather leaped across great geographical expanses was just one of many puzzling aspects of the disease.*1
All the outbreaks attracted little more than local attention, but in 1970, after several years of quiescence, the epidemic reappeared at Lackland Air Force Base in Texas, and now at last medical investigators began to look at it closely—though not, it must be said, much more productively. The Lackland outbreak made 221 people sick, most for about a week but some for up to a year. Sometimes just one person in a department came down with it; sometimes nearly everyone did. Most victims recovered completely, but a few experienced relapses weeks or months later. As usual nothing about the outbreak fit into a logical pattern, and all tests for bacterial or viral agents came back negative. Many of the victims were children too small to be suggestible, ruling out hysteria—the most common explanation for otherwise unexplained mass outbreaks. The epidemic lasted for a little over two months, then ceased (apart from the relapses) and has never returned. A report in The Journal of the American Medical Association concluded that the victims had been suffering from a “subtle but nevertheless primarily organic illness whose effects may include exacerbation of underlying psychogenic illness.” Which is another way of saying, “We have no idea.”
Infectious diseases, as you will gather, are curious things. Some flit about like Akureyri disease, popping up seemingly at random, then going quiet for a time before popping up somewhere else. Others advance across landscapes like a conquering army. West Nile virus surfaced in New York in 1999 and within four years had covered the whole of America. Some diseases wreak havoc and then quietly withdraw, sometimes for years, occasionally forever. Between 1485 and 1551, Britain was repeatedly ravaged by a terrifying malady called the sweating sickness, which killed untold thousands. Then it abruptly stopped and was never seen there again. Two hundred years later, a very similar illness appeared in France, where it was called the Picardy sweats. Then it too vanished. We have no idea where and how it incubated, why it disappeared when it did, or where it might be now.
Baffling outbreaks, particularly small ones, are more common than you might think. Every year in the United States about six people, preponderantly in northern Minnesota, grow ill with Powassan virus. Some victims suffer only mild flu-like symptoms, but others are left with permanent neurological damage. About 10 percent die. There is no cure or treatment. In Wisconsin in the winter of 2015–16, fifty-four people, from twelve different counties, fell ill from a little-known bacterial infection called Elizabethkingia. Fifteen of the victims died. Elizabethkingia is a common soil microbe, but it only rarely infects people. Why it suddenly became rampant across a wide area of the state, and then stopped, is anyone’s guess. Tularemia, an infectious disease spread by ticks, kills 150 or so people a year in America, but with unaccountable variability. In the eleven years from 2006 to 2016, it killed 232 people in Arkansas, but only one person in neighboring Alabama despite abundant similarities in climate, ground cover, and tick populations. The list goes on and on.
Perhaps no case has been harder to explain than Bourbon virus, named for the county in Kansas where it first appeared in 2014. In the spring of that year, John Seested, a healthy, middle-aged man from Fort Scott, about ninety miles south of Kansas City, was working on his property when he noticed he had been bitten by a tick. After a while he began to grow achy and feverish. When his symptoms didn’t improve, he was admitted to a local hospital and given doxycycline, a drug for tick-bite infections, but it had no effect. Over the next day or two, Seested’s condition steadily worsened. Then his organs began to fail. On the eleventh day he died.
Bourbon virus, as it became known, represented a whole new class of virus. It came from a group called thogotoviruses, which are endemic to regions of Africa, Asia, and eastern Europe, but this particular strain was entirely novel. Why it appeared suddenly in the very middle of the United States is a mystery. No one else got the disease in Fort Scott or anywhere else in Kansas, but a year later a man 250 miles away in Oklahoma came down with it. At least five other cases have since been reported. The Centers for Disease Control is curiously reticent about numbers. It says only that “as of June 2018, a limited number of Bourbon virus disease cases have been identified in the Midwest and southern United States,” a somewhat odd way of putting it because there is clearly no limit on the number of infections any disease can cause. The most recent confirmed case, at the time of writing, was a fifty-eight-year-old woman who was bitten by a tick while working in Meramec State Park in eastern Missouri and died soon afterward.
It may be that all of these elusive diseases infect lots more people, but not seriously enough to be noticed. “Unless doctors are doing laboratory tests specifically for this infection, they’ll miss it,” a CDC scientist told a reporter for National Public Radio in 2015, in reference to Heartland virus, yet another mysterious pathogen. (There really are a lot of these.) As of late 2018, the Heartland virus had infected some twenty people and killed an unknown number since it first appeared near St. Joseph, Missouri, in 2009. But so far all that can be said for sure is that these diseases only infect a very unlucky few people far removed from each other with no known connections between them.
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Sometimes it turns out that what seems to be a new disease is not new at all. Such proved to be the case in 1976 when delegates to an American Legion convention at the Bellevue-Stratford Hotel in Philadelphia began to fall ill from a disease no authority could identify. Soon many of them were dying. Within a few days, 34 were dead and another 190 or so were ill, some gravely. An additional puzzle was that about one-fifth of the victims had not set foot in the hotel, but had only walked past it. Epidemiologists from the Centers for Disease Control took two years to identify the culprit, a novel bacterium from a genus they called Legionella. It had spread through the hotel’s air-conditioning ducts. The unlucky passersby had been infected by walking through exhaust fumes.
Only much later was it realized that Legionella was almost certainly responsible for similarly unexplained outbreaks in Washington, D.C., in 1965 and in Pontiac, Michigan, three years later. Indeed, it turned out that the Bellevue-Stratford Hotel had suffered a smaller, less lethal cluster of pneumonia cases two years earlier during a convention of the Independent Order of Odd Fellows, but that had attracted little attention because no one died. We now know that Legionella is widely distributed in soil and freshwater, and Legionnaires’ disease has become more common than most people suppose. A dozen or so outbreaks are reported each year in America, and about eighteen thousand people become sick enough to need hospitalization, but the CDC thinks that that number is probably underreported.
Much the same thing happened with Akureyri disease where further investigations showed that there had been similar outbreaks in Switzerland in 1937 and 1939 and probably in Los Angeles in 1934 (where it was taken to be a mild form of poliomyelitis). Where, if anywhere, it was before that is unknown.
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Whether or not a disease becomes epidemic is dependent on four factors: how lethal it is, how good it is at finding new victims, how easy or difficult it is to contain, and how susceptible it is to vaccines. Most really scary diseases are not actually very good at all four; in fact, the qualities that make them scary often render them ineffective at spreading. Ebola, for instance, is so terrifying that people in the area of infection flee before it, doing everything in their powers to escape exposure. In addition, it incapacitates its victims swiftly, so most are removed from circulation before they can spread the disease widely anyway. Ebola is almost ludicrously infectious—a single droplet of blood no bigger than this o may contain a hundred million Ebola particles, every one of them as lethal as a hand grenade—but it is held back by its clumsiness at spreading.
