The Forgetting Read online

  “The clinical interpretation of this Alzheimer’s disease is still confused.”

  Alzheimer’s disease was born.

  Every disease needs a name. As a matter of social reality, no disease exists until it has one.

  Acquired Immunodeficiency Syndrome • Acrocephalosyndactylia • Adams-Stokes Disease • Bell’s Palsy • Beriberi • Bloom Syndrome • Blue Rubber Bleb Nevus Syndrome • Bronchitis • Bronchopulmonary Dysplasia • Canavan Disease • Candidiasis • Cherubism • Chicken Pox • Cholangitis • Depression • Dermatofibroma • Dyslexia • Epilepsy • Erb’s Palsy • Esotropia • Evans Syndrome • Fibromyalgia • Fuchs’ Endothelial Dystrophy • Furunculosis • Giardiasis • Gilbert Disease • Glycogen Storage Disease • Gonorrhea • Goodpasture Syndrome • Hashimoto’s Disease • Hematospermia • Hemophilia A • Herpes Simplex • Influenza • Irritable Bowel Syndrome • Isaac’s Syndrome • Job’s Syndrome • Keratosis Follicularis • Klinefelter’s Syndrome • Klippel-Feil Syndrome • Kuru • Labyrinth Diseases • Laurence-Moon Syndrome • Lemierre’s Syndrome • Lentigo • Leukemia • Lyme Disease • Malaria • Malignant Hyperthermia • Measles • Moyamoya Disease • Multiple Myeloma • Multiple Sclerosis • Noonan Syndrome • Nystagmus • Obesity • Oculomotor Nerve Diseases • Osteoarthritis • Pellagra • Pertussis • Pleurisy • Pneumonia • Poland Syndrome • Proctitis • Q Fever • De Quervain’s Tendinitis • Reye’s Syndrome • Rhabdoid Tumor • Rhinitis • Rocky Mountain Spotted Fever • Romano-Ward Syndrome • Rubella • Sarcoma • Scabies • Scarlet Fever • Scheie Syndrome • Sezary Syndrome • Smallpox • Sprengel’s Deformity • Supraglottitis • Syringomyelia • Takayasu’s Arteritis • Tay-Sachs Disease • Tinea • Toxic Shock Syndrome • Trichinosis • Typhoid • Undulant Fever • Vasculitis • Volvulus • West Syndrome • Von Willebrand Disease • Wilms’ Tumor • Xerostomia • Yaws • Yellow Fever • Zoonoses • Zygomycosis

  The disease name is public recognition of a shared affliction. The name says, THIS is what you are suffering from. You are not alone. Others are suffering from the same thing.

  The name also says. We’re going to fight this thing. “Choosing to call a set of phenomena a disease,” writes medical philosopher H. Tristram Engelhardt, Jr., “involves a commitment to medical intervention, the assignment of the sick-role, and the enlistment in action of health professionals.” Naming a disease is tantamount to launching an assault against that disease.

  Finally, the name is also a necessary tag for an otherwise intangible phenomenon. A disease is not a thing but a process; it is neither the cause of the problem nor its visible effects—neither the virus infecting the tissue nor the damaged tissue itself—but the interaction between the two. Because of the elusive nature of disease, the name is often the only available emblem. Once accepted, specific names quickly come to dominate social reality. The flavor of the name can make a real difference in how the disease is perceived and acted on.

  Any casual observer can easily see that the history of disease-naming is a haphazard one, four thousand years of jigs and jags that have left us with appellations ranging from the vaguely mythological (“influenza” originally referred to the vast influence of the gods) to the icily clinical (acquired immunodeficiency syndrome). A disease might be named after the major symptom (smallpox), a side effect (yellow fever), a known or suspected cause (schistosomiasis, tuberculosis), or might instead be a vague reference to a social consequence (the plague).

  The sporadic tradition of naming a disease after the identifying physician seems to have started in the mid-nineteenth century, when French neurologist Jean-Martin Charcot coined the name “la maladie de Parkinson” after London physician James Parkinson’s 1817 “Essay on the Shaking Palsy.” Thomas Addison earned the honor of “Addison’s disease” in 1855 by characterizing a complex disorder involving the destruction of the adrenal glands. Huntington’s disease is named after George Huntington, who in 1872 detailed the hereditary chorea that begins with muscle spasms and ends in dementia.

  Kraepelin followed in this nascent tradition by naming what seemed to be a new discovery after the discoverer. It was also a political maneuver with two obvious payoffs for the namer. By bringing attention to this new disease, he ensured the maximum possible exposure for the emerging evidence of organic brain disease. And in fashioning a formal new disease after a staff member, Kraepelin also gained additional glory for his own institute.

  But what was the disease, exactly? Kraepelin wrote:

  The clinical Interpretation of this Alzheimer’s disease is still confused. While the anatomical findings suggest that we are dealing with a particularly serious form of senile dementia, the fact that this disease sometimes starts already around the age of fifty does not allow this supposition. In such cases we should at least presume a “senium praecox” [premature aging] if not perhaps a more or less age-Independent unique disease process.

  The words dropped onto the medical community like a giant crop circle—it was a powerful, resonant event, but what exactly did it mean? Even as the term “Alzheimer’s disease” quickly gained currency throughout the world, largely on the strength of Kraepelin’s formidable reputation (he would eventually come to be known as the “Linnaeus of psychiatry” for his importance in mental disease classification), it confused many.

  On the one hand, it seemed that he intended Alzheimer’s disease to refer only to a rare form of so-called presenile dementia that affected a tiny number of people in their forties and fifties. On the other hand, he included his description in the “senile dementia” section of his book, not the presenile section. Also, his suggestion that this was “perhaps a more or less age-independent unique disease process” seemed to imply that the disease could affect anyone, irrespective of age.

  Kraepelin’s suggestion was strangely jumbled for a man whose life’s work had been orderly classification. In one short sentence, he somehow managed both to brazenly introduce a new disease and to undermine it.

  On the surface, it seemed like sloppiness. But it could not have been. Kraepelin wasn’t blind to the ricochet effect of his words. Far from it: he was as much a political animal as a medical man, running the highest-profile psychiatric clinic in Germany (and arguably in all of Europe). Nor was he known for carelessness. Medical scholars looking back over the span of a century to particulars of his work are continually impressed with his precision and intelligence.

  He did it on purpose, as a way of recognizing Alois Alzheimer’s genuine discovery but sidestepping the question of how it challenged thousands of years of thinking about old age. For whatever reason, Kraepelin did not want to be the one to suddenly insist that senility was a disease to be fought. “Accepting Alzheimer’s disease as a separate disease prevented the coming about of another, more complicated question, i.e., the question whether senile dementia is a disease entity to be distinguished from aging,” suggests Dutch physician and historian Rob Dillman.

  So Kraepelin left it as “perhaps.”

  He left it fuzzy, proposing Alzheimer’s as a middle-age dementia—and “perhaps” a senile dementia.

  Still, it was the first strong hint that senility was not a reasonable part of aging. For all of human history, senile dementia had been tacitly accepted as merely a lamentable stage of life. “But if I am to live on,” the Greek historian Xenophon wrote in Memorabilia (fourth century B.C.), “haply [by chance] I may be forced to pay the old man’s forfeit—to become sand-blind and deaf and dull of wit, slower to learn, quicker to forget, outstripped now by those who were behind me.”

  In Ecclesiasticus 3.12–13 (second century B.C.) we find these words: “O son, help your father in his old age, and do not grieve him as long as he lives/even if he is lacking in understanding, show forbearance.”

  Most prominently of all, Shakespeare defined senility as one of life’s natural stages. In As You Like It, Jaques declares:

  … one man in his time plays many parts.…

  Last scene of al
l,

  That ends this strange eventful history,

  Is second childishness and mere oblivion.…

  Even as recently as Ralph Waldo Emerson’s steady sinking into what a biographer called his “soft oblivion,” through the 1870s and up to his death in 1882, his condition was lamented but accepted with an unwavering fatalism. This isn’t particularly surprising, given that in his own notebook Emerson had noted his admiration for a remark by his friend Bronson Alcott on the subject of senility: “That as the child loses, as he comes into the world, his angelic memory, so the man, as he grows old, loses his memory of this world.”

  The voluminous records of Emerson’s life suggest that he saw a doctor about his failing memory only once; apparently, nothing noteworthy came from it. And Edward Emerson, a physician, made no medical observations of his father’s condition. Though the disorder attacked Emerson’s single greatest asset, his mind, there was no public or private suggestion of treating his memory loss, fighting it, or in any way considering it a disease. Indeed, when a friend inquired about Emerson’s health late in his life, he replied, “Quite well; I have lost my mental faculties but am perfectly well.” It is striking to see, through the prism of modern medicine, the stark separation of Emerson’s senility from his physical health.

  Now, less than three decades after Emerson’s ordeal, Kraepelin was suggesting that perhaps senile dementia was not just a matter of aging or accelerated aging. Perhaps it was a disease; perhaps doctors now had a moral obligation to do something about it.

  Years passed and the world forgot about Kraepelin’s “perhaps.” Alzheimer’s disease congealed in medical circles as a rare, middle-aged disorder. It came to Meta Neumann and Robert Cohn in 1953 to challenge that thinking with new and convincing evidence. “There is no difference in the clinical or pathological picture in the various age groups,” they wrote in their article, published that year in the Archives of Neurology and Psychiatry.

  The data were meticulous and compelling, but not nearly enough to force a change in convention. Proof or no proof, people weren’t ready to call senility a disease. “They didn’t believe it,” Cohn recalled of colleagues’ reactions to the paper they published. “They felt that Meta was talking nonsense.”

  In the eyes of the psychiatric community, “Alzheimer’s” remained a designation for a very rare presenile disease. Senility remained a natural part of aging.

  The confusion endured.

  A.P.: On the kitchen door, I have little yellow Post-it Notes. So when I leave the house I know I have to do certain things. I can’t go out until I see that note on the door.

  M.W.: I do the same sort of thing, except that I have a big calendar in my kitchen.

  T.R.: I have a hook I write in every day.

  J.J.: Well, I make a list in the kitchen and then one in the bedroom. They are the same. Sometimes in the bathroom I put a different list

  H.K.: I never thought of that. That sounds so good to have two lists—one on one end of the house and another on the other end—because by the time I get back to the other end I think, “Why have I come?”

  —Houston, Texas

  Chapter 6

  A MOST LOVING BROTHER

  In 75 A.D., Plutarch chronicled the exuberant life and gloomy decline of the great Roman warrior-diplomat Lucius Licinius Lucullus, who fought in Italy and Asia under the emperor Sulla and subsequently governed Africa. After retiring to a life of extravagance, he slid into full-blown dementia. Lucullus’s intellect, said Plutarch, “failed him by degrees … so disabled and unsettled his mind, that while he was yet alive, his brother took charge of his affairs.”

  Of the ordeal, Plutarch seemed most impressed by the burden cast on Lucullus’s brother Marcus. It was Marcus who had to manage Lucullus’s slow decline, as well as his estate. And when Lucullus died (at age fifty-three), it was Marcus who stood up to popular pressure to bury the body in the Field of Mars alongside Sulla. Marcus instead honored Lucullus’s specific request that he be buried on his own family grounds. A short time later, Marcus himself was buried in the same spot, no doubt worn down by his tireless duties in service to his ailing brother. Admiring Marcus’s sacrifice, Plutarch closed his long essay with a benediction not for the famous victim but his caregiver. “In all respects,” he wrote, “a most loving brother.”

  The unique curse of Alzheimer’s is that it ravages several victims for every brain it infects. Since it shuts down the brain very slowly, beginning with higher functions, close friends and loved ones are forced not only to witness an excruciating fade but also increasingly to step in and compensate for lost abilities. We all rely on the assistance of other people in order to live full, rich lives. A person with dementia relies increasingly—and, in the fullness of time, completely—on the care of others. Lucullus had his brother. Reagan has his wife and Secret Service agents. Greta, Arnie, and Doris have their doctors, nurses, spouses, siblings, children, and friends.

  The caregiver must preside over the degeneration of someone he or she loves very much; must do this for years and years with the news always getting worse, not better; must every few months learn to compensate for new shortcomings with makeshift remedies; must negotiate impossible requests and fantastic observations; must put up sometimes with deranged but at the same time very personal insults; and must somehow learn to smile through it all. The work shift in this literally thankless job lasts for twenty-four hours a day, seven days a week. On-the-job training includes basic neurology, an introduction to nursing, and mind reading. Caregivers must be able to diagnose a wide variety of ordinary ailments—toothache, nausea, urinary tract infection, and so on—under extraordinary circumstances. Imagine a patient suddenly upset about something but completely unable to communicate the problem, or even to understand it himself. Is he hungry? Exhausted? Sore? Does he have a bad headache or did he break his toe? Is his back in spasms or is his appendix inflamed? Can he point to the problem? No, he cannot.

  The stress facing caregivers is so extraordinary that it commonly leads to very serious problems on its own. “Caregiver’s dementia” is widely used to describe the overpowering symptoms of fatigue and forgetfulness that often come with the role of Alzheimer’s caregiver—staying up all hours, going days or weeks without a break, and so on. The term is half tongue in cheek, not intended to refer to a biological dementia. Still, this stress-induced psychological condition can be very, very serious. One estimate has roughly half of all Alzheimer’s caregivers struggling with clinical depression.

  In the late 1990s, some 10–15 million Americans were called to duty. More often than not they were women, often in their forties or early fifties, often recently retired from the more conventional parenting role after nearly two decades. The kids had just gone off to work or started college; life was supposed to begin all over again—and then a call came in: Mom or Dad had been acting a little strange lately.

  There are no wages for this grueling job, of course, and depending on the patient’s health insurance and the size of her estate, the illness can actually cost the caregiver tens of thousands of dollars every year. Neither Medicare nor private health insurance covers the type of long-term care most patients need. The average out-of-pocket costs for Alzheimer’s patients are $12,500 per year. Nursing home care averages more than $40,000 annually.

  If estranged family members don’t happen to be sensitive to the burdens of Alzheimer’s disease, there might also be substantial legal bills. The very slow fade of a parent often tends to knock pegs out from under already shaky families. In Shakespeare’s rendition of King Lear, written in 1605–1606, Lear’s two elder daughters take cruel advantage of their father’s weakening state of mind while the third daughter, Cordelia, suffers for her loyalty and lack of guile. The play is about a family’s dissolution through misunderstanding and distrust. Senility is the playwright’s device.

  It was a striking plot choice. There had been at least fifty versions of the Lear (or “Leir”) story prior to Shakespeare’s, b
ut his was the first to put the king in a deep senile fog. Throughout Shakespeare’s play, Lear hallucinates, doesn’t recognize old friends, and cannot remember who he is. “My wits begin to turn,” he remarks in one scene; in another, “I am cut to the brains.” With a few exceptions (most notably his plot-driven mental recovery in the last act), his complaints are perfectly in synch with the Alzheimer’s experience:

  I fear I am not in my perfect mind.

  Methinks I should know you, and know this man;

  Yet I am doubtful; for I am mainly ignorant

  What place this is; and all the skill I have

  Remembers not these garments; nor I know not

  Where I did lodge last night.…

  —KING LEAR, ACT IV, SCENE 3

  Shakespeare’s decision to incorporate dementia may have been inspired by the real-life case of Bryan Annesley, a wealthy palace attendant to Shakespeare’s patron. Queen Elizabeth. In 1603—two years before Shakespeare wrote King Lear—Annesley’s senility became a public spectacle in the English court. Annesley was in the late stages of progressive dementia: “Fallen into such imperfection and distemperature of mind and memory,” reported an observer, “[and] altogether unfit to govern himself.” His youngest daughter (of three), named Cordell, the only one not yet married, remained at home to care for him. By contrast, the eldest daughter, Grace, kept her attentions focused on the sizable estate, suing to have her father declared a lunatic so she could take immediate custody of his possessions.

  Under the law at that time, a lunatic was deprived of all civil and human rights and was subject to the whims of the family property owner. Cordell’s successful defense of her father’s rights—she convinced the royal minister Lord Cecil to place the estate into the custody of a loyal family friend—very likely insured that he would have the most comfortable and dignified descent possible. Annesley died the next year, still under Cordell’s care.