The Patient Talks Back

1.

Sarah Manguso, a poet, and the author of The Two Kinds of Decay, says that her memoir “is a usual book about illness. Someone gets sick, someone gets well.” People feel a need to talk or write about their illnesses, and most of us read these accounts with close attention. They are written to inform and encourage others, to bear witness to our common fate, and to give advice about how to avert it; and the more inspiring, cautionary, and tutelary the book, the more eagerly we pick it up—among these under review, Jill Bolte Taylor’s My Stroke of Insight has been on recent best-seller lists.

There’s a roster of distinguished preceding examples, from Helen Keller’s The Story of My Life to the moving book The Diving Bell and the Butterfly by Jean-Dominique Bauby, victim of the “locked-in syndrome” from a massive stroke at age forty-three, which left him imprisoned in his paralyzed body but with his mind intact¹; from Montaigne’s description of what it’s like to pass a kidney stone, to Alphonse Daudet’s In the Land of Pain, as he lay dying of syphilis, to Betty MacDonald on her tuberculosis in The Plague and I. There are books on just about any medical condition written from the point of view of the sufferer, the doctor, or a family member, valued testimonials to an apparently inevitable part of the human lot, often treated in painting too, though seldom in fiction except as convenient denouement.

Asking Google about “chronic idiopathic demyelinating polyradiculoneuropathy,” especially the eMedicine pages on the disease, or about stroke, especially the material furnished by the American Stroke Association and the American Heart Association, will provide a lot more information about these conditions than the books under review, but not about the subjective experience of them. Manguso’s illness, which goes by the acronym CIDP, was anything but usual. Between its onset in 1995 and presumed end in 2004, she suffered one relapse after another; her father, she writes, aged ten years during the first year she became ill, and her mother, wondering “why this had to happen,” reflected the spiritual or metaphysical or religious disquietude that affliction provokes.

Manguso vividly describes the morning she awoke at home with numb feet and tingling in her hands; how during the next few days at college she began to walk stiff-legged and then to stagger; how finally, after she fell down in the courtyard, she was taken to the hospital by her parents, and promptly admitted to the intensive care unit. Initially, she was diagnosed as having Guillain-Barré syndrome, an acute inflammatory disease of the peripheral nervous system, and was immediately treated with plasmapheresis, the removal of her blood plasma and its replacement by plasma from healthy persons, to rinse out the “rogue antibodies” that her body was indiscriminately producing, which were destroying the myelin sheaths that surround and protect peripheral nerves.

Though her diagnosis was later changed, the prompt plasma exchange may well have helped prevent her from having permanent nerve damage. Typically, Guillain-Barré syndrome evolves to reach a plateau within four weeks, and then is followed by a prolonged recovery, which can be hastened by painstaking physical therapy. People learned about Guillain-Barré from Joseph Heller’s No Laughing Matter, a book that was valuable for Manguso. Heller, the author of Catch-22, had the disease and wrote about it with his friend Speed Vogel.²

Manguso’s illness was different from Heller’s; it kept recurring, each time requiring hospitalization and plasmapheresis, leaving her progressively more neurologically impaired. Desperate, she switched from her old neurologist, whom she “already hated,” to a new one, whom she “loved immediately,” and who made the diagnosis of CIDP and outlined an aggressive program of treatment. (CIDP, commonly regarded as the chronic equivalent of Guillain-Barré syndrome, cannot be diagnosed until its course has declared itself over a period of at least eight weeks.) Manguso’s new neurologist stopped the plasmapheresis and switched her treatment to prednisone—a synthetic corticosteroid drug—the then-experimental drug interferon, and, later, intravenous gamma globulin (IVIg), which is used in several different immunologically mediated disorders. IVIg is believed to contain antibodies that neutralize the aberrant antibodies and other immunological factors that are attacking the peripheral nervous system in CIDP. There is no doubt now that it works.

But chronic relapsing diseases like CIDP that require repeated hospitalizations, and multiple therapeutic and procedural interventions, bring with them a high risk of side effects and clinical misadventures. Manguso had plenty of both. In brief, almost stanza-like paragraphs, she describes doctors’ inattention or disregard of troublesome symptoms, unwanted intrusions by medical students, supportive as well as disagreeable nurses, businesslike orderlies, the welcome arrival of a more efficient plasmapheresis machine, and the chronic fear of death—the sort of details sufferers wish to share and readers read such accounts to learn. She deals with mundane matters such as wiping your bottom when you are nearly completely paralyzed (you don’t, someone else does it for you and seldom to your satisfaction) and, especially, what goes on in the head of a young victim, including the social realities and status anxieties. At one period of remission, she agreed to take part in a college video, but was

less concerned that a handsome rich boy [the filmmaker] was going to see part of my right breast than I was ashamed he would see the hump of fat on my pimply shoulders and think I was a girl who not only had gone to public school but who had acne and was fat.

Her public school education was a source of special shame for her, but she also brooded about sex, and attributes her eventual cure to “the mystical power of intercourse”—conducted despite cannulas dangling from her corticosteroid-fattened and acne-riddled body. The prednisone and IVIg had brought her disease under control, and a powerful emotional uplift, which she craved and counted on, was bound to help further. In spite of this potentially transgressive behavior, the rebellious sufferer escapes with a strangely literary displacement of the punishment—it’s her partner, who “had selflessly had intercourse with an ugly version of a girl he’d once had a crush on,” who dies an early death sometime later from a ruptured aneurysm. Punishment is another lurking theme of her and others’ illness books, vestiges of religious legacies that are nowhere explicit.

The mystical power of sex didn’t put an end to her distress. After four years of prednisone therapy, the extra medication led to a steroid-induced psychosis, whose manifestations finally caused her to be rehospitalized, this time in a locked psychiatric ward, for severe depression, which waxed and waned for years. She recovered from her last serious depression in March 2004, but her understandable fear of a return to calvary remains: “My disease has been in complete remission for seven years, but I still act as if I expect it to come back tomorrow.” It hasn’t, and she has since written two books of poetry and a collection of short stories, and in 2007 won the Rome Prize of the American Academy of Arts and Letters. Yet illness has changed her life and future as it does that of every chronic sufferer.

2.

CIDP is a distinctly uncommon disease, about which little is known, a fact that Manguso valued “for its irrefutable proof of [her] specialness.” It is interesting that, just as many distinguished people have had a long childhood illness that conferred this feeling, Jill Taylor Bolte too feels what she describes as “joy” at her illness. Taylor, a Harvard University–trained neuroanatomist, had a disabling stroke, which temporarily robbed her of all cognitive function and of the ability to talk, understand speech, or move her body purposefully. As the stroke was unfolding, she understood what was happening to her sufficiently well to claim—in retrospect—“Wow, how many scientists have the opportunity to study their own brain function and mental deterioration from the inside out?”

Taylor, unlike Manguso, is not a nuanced writer, but her account is absorbing. To set the stage for what happened, in a chapter called “Jill’s Pre-Stroke Life,” she sketches her undergraduate and Ph.D. training at Indiana State University, and her postdoctoral work at Harvard Medical School in the department of neuroscience. After finishing her postdoc, to further her growing interest in the neuroanatomic basis of psychiatric disturbances, she moved to the department of psychiatry. Readers are then prepared for the onset of her stroke and its aftermath with two chapters that provide some basic information about how our brains work: “Simple Science,” a primer on cerebral function, and “Hemispheric Asymmetries,” which provides the neurological background for the main message of the book.

Taylor’s “Simple Science” section is indeed fairly simple, but in the process of simplification, she also omits some important information, both about the plasticity of the human brain, of which she is a profound admirer, and about the main cause of strokes. Part of her aim is to educate readers about these disasters, so she uses the American Stroke Association’s standard subdivision of strokes into “ischemic,” arising from the blockage of a blood vessel that supplies vital oxygen and nutrients to the brain, and “hemorrhagic,” from rupture of a blood vessel with bleeding directly into the brain substance. But for some reason she describes only a single kind of ischemic stroke, “embolic”: “a blood clot travels into the artery until the tapered diameter of the artery becomes too small for the clot to pass any farther,” thus depriving part of the brain of its neuron-preserving blood flow. She leaves out the most common form of ischemic and of all strokes, “thrombotic.” Thrombotic strokes are important not only because of their frequency, but because they are to a major extent preventable and thus have become the focus of public health educational campaigns.

Most ischemic strokes, like most heart attacks that damage the heart muscle (“myocardial infarctions”), are linked to high cholesterol and atherosclerosis. Such strokes and heart attacks are caused by the sudden formation of a thrombus, a blood clot that completely obstructs an artery already partly narrowed by an atherosclerotic plaque, a buildup of cholesterol, calcium, and other debris in the wall of a vessel supplying the brain or heart. The risk factors for the underlying atherosclerosis that provokes both forms are exactly the same—high blood pressure, smoking, elevated blood cholesterol and other plaque-generating lipids, lack of exercise, obesity, and diabetes.

Therefore, preventive measures are also exactly the same, though Taylor, more concerned with the emotional support of victims than with prevention, doesn’t mention any of the well-known recommendations to lose weight, stop smoking, etc., instead giving a list of forty do’s and don’ts for dealing with stroke victims: “I am not stupid, I am wounded. Please respect me,” “Come close, speak slowly, and enunciate clearly,” and so on. She also does not mention new therapeutic and preventative approaches to stroke, such as thrombus-dissolving enzymes, cholesterol-lowering drugs, and antiplatelet medications, the same agents that have already greatly lowered the incidence of myocardial infarction and improved its outcome when it occurs.

There is yet another omission. Taylor sides with the standard neuroanatomical belief that

neurons, the primary cell of the nervous system, do not multiply (for the most part) after we are born. That means that the majority of the neurons in your brain today are as old as you are.

While it is true that neurons don’t multiply, she leaves out recent view-changing discoveries that the brains of adult mammals—including humans—are not doomed to steady attrition from ongoing death of neurons and other key cells, whether from natural causes, disease, or injury. New brain cells can be formed and old ones replaced, thanks to the presence in parts of the brain of stem cells, astonishingly versatile progenitor cells that can differentiate into nerves or their neighboring supporting cells. There’s now no doubt that neurons deriving from stem cells, once given a start, mature into normal nerve cells that integrate and develop connections (synapses) with other neurons.³

This discovery represents a critical scientific breakthrough that has opened a new and promising avenue for the treatment of currently incurable nervous system disorders such as Alzheimer’s and Parkinson’s diseases. Such treatment might even promote the emergence of new neurons in areas of the brain impaired by a stroke such as Taylor had, either by stimulating new brain cell formation from resident stem cells or by transplanting harvested ones directly into damaged regions of the brain.

By far the most interesting and probably controversial sections of Taylor’s book concern the gigantic and amazing functional differences between the two cerebral hemispheres: the bulky upper parts of the brain, which are easily recognized by their wrinkly corrugated external surfaces. Although the two hemispheres look identical and they are wired together by a vast network of nerve fibers, they regulate competing neurological functions and they “process information in uniquely different ways.” Taylor defines dominance within the brain as “which hemisphere houses the ability to create and understand verbal language.” The 85 percent of Americans who are right-handed are virtually all left hemisphere dominant. Left-handed people (like both of the authors of this review) have always believed that they were the opposite—therefore special—but it turns out that “over 60 percent of left handed people are also classified as left hemisphere dominant.” Which hemisphere controls what is an important part of Taylor’s argument.

A pop-psych view that has been around for a long time has been more or less validated by research: located within their respective hemispheres are our left minds and right minds. Of the two, according to Taylor, the usually dominant left mind is thought to be the more hard-nosed, verbally oriented, preoccupied with down-to-earth task-dependent details, and unsentimental. By contrast, our right minds don’t think in words but in pictures (at least that is what hers did), are concerned with the abstract glory of the moment (not how it relates to the past, present, and future), are rich with pleasant sensations, such as joy and inner tranquillity, and are free of inhibitions and judgment.

Ordinarily, our two minds work in concert depending on the sensory input from our immediate environment and what we are engaged in at the time, as well as on our past experiences and on our present expectations with, usually, the more practical left mind predominating. But Taylor’s hemorrhage disabled her mundane left mind, thus liberating her right mind, which rejoiced in its newly found freedom. Taylor made a slow (seven years) but nearly complete recovery, and as her left brain began to regain its power, she became convinced that she could select which of its old programs she wanted to retain—the pleasant helpful ones—and which she wanted to reject—the “stubborn, arrogant, sarcastic and/or jealous” egocentric functions. She ended up with “the power to consciously choose which emotional and physiological loops” she wanted “to hook into,” and suggests—not entirely persuasively—that we can all do the same:

One of the greatest blessings I received as a result of this hemorrhage is that I had the chance to rejuvenate and strengthen my neurocircuits of innocence and inner joy. Thanks to this stroke, I have become free to explore the world again with childlike curiosity. In the absence of obvious and immediate danger, I feel safe in the world and walk the earth as though it is my backyard. In the consciousness of my right mind, we are laced together as the universal tapestry of human potential, and life is good and we are all beautiful—just the way we are.

Some say that months or even years of meditation may lead to the same level of inner consciousness that Taylor enjoys, but we should not hope for strokes to get us there. One wonders whether, without the useful computer-derived metaphors of “program” and “circuit,” she would describe her improved character with old-fashioned philosophical terms like “wisdom” or “maturity.” Strokes are the third most common cause of death in the United States, and high on the list of chronic disablers (right up there with arthritis and depression): Taylor’s must be the only one that was ever welcomed with enthusiasm.

3.

Both Manguso’s and Taylor’s books send strong positive messages that by combining deep personal resolve and courage with excellent medical care, people can bounce back from life- destroying illnesses, and that a will to survive is innate and, ordinarily, admired. David Shields, however, wonders whether or not that makes sense; he is in a long tradition of pessimism when he reminds us that after all The Thing About Life Is That One Day You’ll Be Dead. In the course of expressing this attitude, Shields has occasion to tell us a great deal about his father, Milton, a vigorous senior citizen of dubious likability, who has made it into his mid-nineties by regular jogging and tennis, maintaining his youthful weight, and as much lovemaking as he could find partners for.

Now, at ninety-seven, Milton has lost much of his energy and has become terminally bored with life, but still wants desperately to live to be one hundred. The text alternates between his determination to stay in shape, watch his diet and weight, and live forever, and David Shields’s rather sadistically wielded statistics that underscore why this is not going to happen. The reader earnestly hopes Mr. Shields will make it, if only to refute his son’s sour realism, with its unpleasant implications for us all.

We don’t learn why there are so few centenarians—only one in ten thousand people live that long—until well into the book, when Shields makes the key point that nature’s biological imperatives are only focused on perpetuating the species and are afterward indifferent to our fate. In America, for instance, more than 90 percent of people now live to be at least fifty, the crucial age that marks the onset of both menopause (the end of reproductive capacity) in women and the waning powers of procreation in men; but, unlike some insects and salmon, humans don’t die when they finish reproducing: they are likely to live another twenty-five or thirty years. (In 2005, life expectancy for American women was 80.4 years and 75.2 years for men.)

On the one hand, all this post- reproduction survival time can be viewed as evolutionary largesse, but on the other hand, it is also a long preparation for death, characterized by relentless physical and mental attrition. Beginning in the twenties and thirties, but accelerating in the forties, fifties, and sixties, is a steady age-related waning of physical performance as the lungs and heart reduce their capacities to deliver oxygen into the bloodstream and pump it around the body; blood vessels harden and become less elastic; muscles shrink and weaken; reflexes slow and coordination worsens; bone density decreases and fragility increases; and the usually vigilant immune system no longer protects against the onset of infection and the transformation of healthy cells into malignant ones. These are regular phenomena, to be sure, but with huge individual variation—and the possibility of, to some extent, remediable measures, such as physical exercise.

Shields seems to delight in documenting this attrition with one dire statistic after another, but he never reminds readers that these kinds of epidemiologically based data are derived from large numbers of subjects, often carefully selected and unrepresentative, for instance nurses or military recruits, and can’t say what is in store for a particular person, for instance Milton Shields. David Shields clearly buys into the theory that aging is under genetic control. After all, genes control our physical appearance, our internal cellular machinery—such as how we respond to stress, whether we react to various medications, whether we tolerate milk and cheese—as well as our susceptibility to and the age of onset of many of the numerous diseases that complicate aging and can bring life to an end.

In addition, several groups of investigators have found ways of greatly extending the life expectancy of various species of laboratory animals by genetic manipulation. Of these experimental tricks, the most robust and reliable is calorie restriction, which extends longevity in rats, fruit flies, worms, fish, mice, and hamsters, and is now being tested (and seems to be succeeding) in monkeys; the mechanisms at play are unknown, but probably involve activation of survival genes that shift the body’s metabolism into a slower, live-longer mode. The idea is interesting, and Shields is attracted to it, but we can only wonder how many humans are going to stick to the draconian restriction in their daily caloric intake, the technique customarily used to extend life expectancy in experimental animals; it would mean, basically, that people would have to practically starve themselves for most of their lives.

Genes unquestionably affect how well and how long we live, but many scientists believe that aging happens spontaneously, without a genetic program. According to the “no genetic blueprint” theory, aging represents the accumulation of biochemical and physical wear and tear, from both internal and external assaults, of vital cellular metabolic machinery. These theories are not mutually exclusive and it seems likely that both wear and tear and genetic influences determine how long we live. But for centuries, the most important determinant of longevity has been wealth, and this remains true today even in industrialized countries like France, where good health care systems are available to everyone. Compared with poor people, the rich have better nutrition and less obesity, they exercise more, smoke fewer cigarettes, and seek medical help sooner. Shields does not mention something that is rarely discussed about American health: the fact that despite our wealth, we are only thirtieth in average life expectancy, tied with Costa Rica, Kuwait, and Slovenia, and far below Japan, Australia, Canada, and all the Western European countries; the same is true for other measures of public health efficacy.

Shields’s father has practiced what many now preach concerning how to extend life. He’s a strong advocate of “nutritional discipline”; he retained his trim youthful weight throughout adulthood; and, he told his son, “the secret of a long, healthy life is to exercise every day even if it’s only for thirty minutes, and don’t let anything deter you from it”—recommendations identical to those that have been both endorsed and widely promoted by numerous public health authorities and professional medical and sports associations. We never really learn why Shields has obliquely chosen to denigrate these eminently sensible guidelines, but we can guess it has something to do with his feelings about his father. At the end of his book, he recounts a nightmare in which he’s in a foot race with the elder Shields: “He wins. He wins again. He always wins—except in the sense that in the end he’ll lose, as we all do.”

Do we really need reminding of this?