Authors: Sherwin B Nuland
How We Die (16 page)
Montaigne believed, in that uncertain and violent era, that death is easiest for those who during their lives have given it most thought, as though always to be prepared for its imminence. Only in this way, he wrote, is it possible to die resigned and reconciled, “patiently and tranquilly,” having experienced life more fully because of the constant awareness that it may soon come to an end. Out of this philosophy grew his admonition, “The utility of living consists not in the length of days, but in the use of time; a man may have lived long, and yet lived but a little.”
V
Alzheimer’s Disease
V
IRTUALLY EVERY DISEASE
can be described in terms of cause and effect. The symptoms a patient presents to his doctor, and the physical findings elicited on examination, are the direct results of very specific pathological changes within cells, tissues, and organs, or of disorders in biochemical processes. Once these underlying alterations have been identified, they can be shown to have led inevitably to the observed clinical manifestations. It is the purpose of the diagnostic workup to find the cause, using its effects as clues.
IRTUALLY EVERY DISEASE
can be described in terms of cause and effect. The symptoms a patient presents to his doctor, and the physical findings elicited on examination, are the direct results of very specific pathological changes within cells, tissues, and organs, or of disorders in biochemical processes. Once these underlying alterations have been identified, they can be shown to have led inevitably to the observed clinical manifestations. It is the purpose of the diagnostic workup to find the cause, using its effects as clues.
For example: Atherosclerotic obstruction in the artery that nourishes a segment of heart muscle will cause angina or infarction, with the resultant symptoms of those conditions; a tumor that produces an oversupply of insulin drastically reduces levels of glucose in the blood, preventing proper brain nutrition and leading to coma; a virus that attacks the motor cells in the spinal cord causes paralysis of the muscle to which those cells send messages; a loop of gut becomes twisted around a strand of internal postoperative scar tissue, and the consequent intestinal obstruction produces distension, vomiting, dehydration, and chemical imbalances in the blood, which in turn can lead to cardiac arrhythmia; a ruptured appendix fills the abdominal cavity with pus and the resultant peritonitis floods the bloodstream with bacteria that cause high fevers, sepsis, and shock. The list of examples is endless, and is the stuff of medical textbooks.
The patient comes to the doctor with one or more signs or symptoms—angina, or coma, or paralyzed legs, or persistent vomiting and a swollen belly, or fever accompanied by abdominal pain—and the detective work begins. It is to the series of events that has led to the observable set of symptoms and other clinical findings that the physician refers when he uses the term
pathophysiology
.
pathophysiology
.
Pathophysiology is the key to disease. To a physician, the word has connotations that convey both the philosophy and the aesthetic of poetry—not surprisingly, part of its Greek root,
physiologia
, has a philosophic and poetic meaning: “an inquiry into the nature of things.” When
pathos
—“suffering” or “disease”—is prefixed to it, we have a literal expression of the essence of the doctor’s quest, which is to make inquiry into the nature of suffering and disease.
physiologia
, has a philosophic and poetic meaning: “an inquiry into the nature of things.” When
pathos
—“suffering” or “disease”—is prefixed to it, we have a literal expression of the essence of the doctor’s quest, which is to make inquiry into the nature of suffering and disease.
It becomes the doctor’s job to identify the instigating cause of sickness by tracing back along the sequence until he has found the ultimate culprit—microbial or hormonal, chemical or mechanical, genetic or environmental, malignant or benign, congenital or newly acquired. The investigation is done by following the clues left in the identifiable damage done to the body by the perpetrator. The crime is thus reconstructed and a treatment plan devised that rids the patient of the influence of the instigator of the disease.
In a sense, then, every doctor is a pathophysiologist, an investigator who identifies the disease by tracing the origins of its symptoms. That having been done, appropriate therapy can be chosen. Whether the aim is to excise the pathology, destroy it with drugs or X ray, counteract it with antidotes, strengthen the organs it is attacking, kill its causative germs, or simply to hold it in check until the body’s own defenses can overwhelm it, a plan of action must be organized against each disease if the patient is to stand any chance of overcoming it. When a physician engages in combat to struggle against his patient’s mortality, his knowledge of cause and effect is the armory to which he turns to help him choose his weapons.
The result of this past century’s biomedical research is that the pathophysiology of the great majority of diseases is well known, or at least known well enough so that effective treatment is available. But there remain some diseases in which the relationship between cause and effect has been less clearly delineated than we might hope, and a few of these diseases are among the greatest scourges of our time. The malady which these days is called “senile dementia of the Alzheimer type” not only falls into this category but carries the additional vexation that its primary cause has continued to elude scientists since the problem was first brought to medical attention in 1907.
The fundamental pathology of Alzheimer’s disease is the progressive degeneration and loss of vast numbers of nerve cells in those portions of the brain’s cortex that are associated with the so-called higher functions, such as memory, learning, and judgment. The severity and nature of the patient’s dementia at any given time are proportional to the number and location of cells that have been affected. The decrease in nerve-cell population is in itself sufficient to explain the memory loss and other cognitive disabilities, but there is another factor that seems to play a role as well—namely, a marked decrease in acetylcholine, the chemical used by these cells to transmit messages.
These are the basic elements of what is known about Alzheimer’s disease, but they are far too few to provide a direct linkage between structural and chemical findings on the one hand and the specific manifestations presented at any given moment by the patient on the other. Many of the details of the pathophysiology of the disease still elude the most determined efforts of medical science to pin them down. The sequential features in the long lists of causes, effects, and treatments that appear in the foregoing paragraphs have no analogy to the present state of our knowledge (or ignorance) of Alzheimer’s. We know not a whit more about what might cure it than we do about what might cause it.
Consequently, in the course of describing how Alzheimer’s disease kills its victims, it will not be possible to stop here and there during the narration of a downhill course to correlate specific symptoms with the stages of pathophysiology of which they are manifestations. Such explanatory digressions would be unsatisfactory and confusing. But there are some very interesting things it
will
be possible to do, and I present them here in yet another list: It
will
be possible to describe the fundamental pathological changes that occur in the brain, and some of the areas of study by which attempts are being made to elucidate them; it
will
be possible to use the gradual historical development of our present knowledge of the illness in such a way that the often abstruse aspects of disordered brain function may be made comprehensible; it
will
be possible to chronicle the emotional carnage visited on the families of victims; it
will
be possible to tell what happens to an afflicted person—and how he or she dies.
will
be possible to do, and I present them here in yet another list: It
will
be possible to describe the fundamental pathological changes that occur in the brain, and some of the areas of study by which attempts are being made to elucidate them; it
will
be possible to use the gradual historical development of our present knowledge of the illness in such a way that the often abstruse aspects of disordered brain function may be made comprehensible; it
will
be possible to chronicle the emotional carnage visited on the families of victims; it
will
be possible to tell what happens to an afflicted person—and how he or she dies.
“Everything came to a head just ten days before our fiftieth wedding anniversary.” Janet Whiting was recalling the six tormented years of her husband’s agonizing decline into the final stages of Alzheimer’s disease. I have known Janet and her husband, Phil, since my boyhood. They were young and very attractive newlyweds the first time my family visited their apartment in the late 1930s; he was twenty-two and she was twenty. Compared with my immigrant parents, who were staidly ensconced in their forties, the Whitings appeared like a movie-star couple, a pair of juveniles not old enough to be doing anything in that recently furnished apartment but playing house.
Not that I doubted the reality of the excitement Janet and Phil very obviously felt about each other—what I doubted was the likelihood that a couple whose shared life was so joyous could really be married. I was sure they were just trying it out; I knew from personal observation that married people don’t behave like this. If the Whitings expected things to work out, they would simply have to stop acting as though they were crazy about each other.
To a great extent, they never did. There remained in that marriage a certain mutuality of gentle regard that I learned increasingly to value as I grew old enough to know something about how it is between a man and a woman. Even the overt unselfconscious expressions of affection never disappeared. As the years passed, Phil made a prosperous career in commercial real estate, and the Bronx apartment was in time succeeded by the beautiful house in Westport, Connecticut, where the three Whiting children were raised. After the kids were grown, Janet and Phil moved to a luxurious condominium in Stratford. When Phil retired from full-time work at sixty-four, the children were long since successfully on their own, there was plenty of money, and the future seemed secure.
After several decades of not seeing the Whitings between my early twenties and forties, my path crossed theirs again in 1978, when they were living in the condominium, not far from my home near New Haven. To spend an evening with those two great-hearted people was to admire the equanimity of their relationship and the tender respect that was implicit in even their slightest references to each other. Their union had more than fulfilled the promise of its first months. When Phil finally retired completely, and he and Janet made a permanent move to Delray Beach, Florida, my wife and I felt that we had been wrenched away from two valued friends. What we didn’t know was that small strange things had already begun to happen.
Even before the move, Phil, whose keen mind had always soaked up works of nonfiction in every spare moment, had stopped reading books. Only in retrospect did that seem strange to Janet, and only in retrospect she found herself years later understanding why he began to insist that she arrange her day so that he might never be out of her company. “I didn’t retire,” he would grumble when she was leaving to spend an afternoon in town, “to be alone.” In his earlier days, outbursts of anger had been rare; now they became more frequent, and turned into full-blown temper tantrums during those last few years in Stratford; increasingly, Phil seemed to find reasons to criticize his daughter Nancy—her visits to the condominium usually ended in tears before she got back on the train to return to her apartment in New York City. After the move to Florida, unexplainable episodes of confusion took place with mounting frequency, and Phil responded to them with disbelief and anger, as though someone else were always at fault. For example, more than once he went to the wrong shop for his regular haircut, then berated the blameless barber for supposedly neglecting the appointment he had really made elsewhere. On one occasion, he threatened to punch a startled motorist at a gas pump, just because the fellow was reaching for an adjacent fuel nozzle—this from a man who had never in his life raised a hand in anger.
Finally, there appeared the first major clue that these new failings were not merely the worsening idiosyncrasies of an aging executive restlessly unfulfilled in his retirement. One evening, Janet invited to dinner a couple whom she and Phil had not seen for several years, Ruth and Henry Warner. Phil had always been an affable host, proud of his wife’s table and his own extensive knowledge of wines. Having grown somewhat corpulent while still a young man, he had learned to wear his girth well, so that his ample belly and easy round-faced smile contributed to an air of cheerful prosperity that fairly exuded from some bountiful generosity of spirit within. He was an easy man to like, and he knew how to expand the atmosphere of comfortable bonhomie that his very presence suggested. In his own home or someone else’s—it made no difference—Phil was like a bighearted innkeeper whose only wish was the well-being of everyone around him.
And so it had been at that dinner. Janet’s food was delicious, Phil’s wines were expertly chosen, the table talk was by turns intense and lighthearted, and the evening was enveloped in the cozy mist of
gemütlich
pleasure that was typical of a visit to the Whiting home. The Warners said their good nights in the warm haze of that good feeling so well remembered from earlier years.
gemütlich
pleasure that was typical of a visit to the Whiting home. The Warners said their good nights in the warm haze of that good feeling so well remembered from earlier years.
On the following morning, Phil couldn’t remember any of it. He was unaware of having so much as seen the Warners, and no amount of explanation would convince him that they had visited. “And that frightened me,” recalled Janet, whose mind until that hour had been seeking rationalizations for the undeniable changes in Phil’s recent behavior. And yet, even at that morning’s point of seeming no return, she tried to explain away this most recent of the disquieting episodes she was so often observing. “I thought, Well, sometimes I forget things, too, and maybe he’ll talk about it later.” So desperate was she to look away from the glaring terror of thoughts that were mounting ever higher in her awareness that she almost convinced herself of the insignificance of her husband’s latest lapse.
But a few weeks later, Janet’s fragile structure of defenses was overwhelmed by an incontrovertible demonstration that forced itself in sharp focus into her direct line of sight, refusing to be dispersed or even blurred by her exhausted powers of justification. On returning home from a few hours away one afternoon, she found herself confronted by an outraged Phil, angrily accusing her of having gone to visit her lover. Even more upsetting than the accusation itself was the identity of the putative “lover”: Phil’s cousin Walter, who had been dead for many years. “At that time, I didn’t even know what Alzheimer’s was—I only knew that I was scared. Something terrible was happening to Phil, and I couldn’t ignore or explain it away anymore.”
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