Authors: Sherwin B Nuland
How We Die (31 page)
Every variety of microbial invader attacking the shattered defenses of the immune-compromised person with AIDS is equipped with its own unique assault weapons and directs its onslaughts against specific objectives. With little remaining CD4 cell resistance to bar their way, the individual divisions and regiments of opportunistic killers devastate the territory that comprises the patient’s tissues. Sometimes by exhausting a person’s energy and small supply of reserve firepower, and sometimes by knocking out a central structure like the brain, the heart, or the lungs, the swarming bits of infection will have their way. Though the pestilential offensives may be slowed or halted for a while by one or another of the newer pharmaceutical agents, they will always in time resume, if not in one form, then in another. A skirmish may be won here and there, or a battle prevented by timely use of prophylactic drugs, and some months of stability thereby achieved—but the eventual outcome of the struggle is preordained. The determined microbial aggressors will accept nothing less than the unconditional surrender that comes only with the death of their involuntary host.
Although AIDS patients may die of any of a number of pathological processes, a relatively small group of microbes is involved in the vast majority of deaths. Foremost among these is
Pneumocystis carinii
, the first one to be identified at the very outset of the worldwide pestilence. The figures are now declining because of prophylactic medication, but until fairly recently, more than 80 percent of patients had at least one experience of PCP, and many died during an episode, either from the respiratory insufficiency itself or from problems associated with it. Depending on the severity of the onslaught, an individual episode used to kill between 10 and 50 percent of its victims before effective means had been found to combat it. It remains a significant factor in the death of nearly half of all AIDS sufferers, but the percentage continues to decrease.
Pneumocystis carinii
, the first one to be identified at the very outset of the worldwide pestilence. The figures are now declining because of prophylactic medication, but until fairly recently, more than 80 percent of patients had at least one experience of PCP, and many died during an episode, either from the respiratory insufficiency itself or from problems associated with it. Depending on the severity of the onslaught, an individual episode used to kill between 10 and 50 percent of its victims before effective means had been found to combat it. It remains a significant factor in the death of nearly half of all AIDS sufferers, but the percentage continues to decrease.
The symptoms of PCP are essentially those experienced by Ishmael Garcia as his breathing became progressively compromised until he sought treatment. Occasionally, the organism may be found in other parts of the body than the lungs, and in autopsies of patients who die of this infection it is sometimes disseminated throughout virtually every major organ, most particularly the brain, heart, and kidneys.
Like patients with other types of pneumonia, those who die with PCP are asphyxiated by the infected lung’s inability to be aerated. As wider areas of tissue become involved, more and more alveoli are destroyed, and a point is reached where arterial oxygen levels cannot be raised in spite of every available means of forcing the gas into the soggy and plugged tissues. The lack of oxygen and the buildup of carbon dioxide damage the brain and finally stop the heart. Sometimes destruction of tissue has been so severe that cavities have been created in the areas of disintegration, very much as in tuberculosis.
The lung is the organ most commonly assaulted by AIDS. Virtually every one of the opportunists, as well as the tumors, looks to the lung as a target. On hospital rounds I have attended, tuberculosis, pus-forming bacteria, the herpeslike cytomegalic virus (CMV), and toxoplasmosis are among the most common problems discussed. Except for the last, they all seek out a home in the respiratory tissue. The incidence of tuberculosis in AIDS patients is some five hundred times what it is in the general population.
Toxoplasmosis is a disease that was at one time so rare that I had difficulty remembering just what it was when I first encountered it in an early AIDS patient. In little more than a decade, it has become a major belligerent in the HIV invasion, and I will never again have to search my memory about its details, so devastating are the things I have seen it do to defenseless people. The organism itself is a protozoan commonly found infecting birds, as well as cats and other small mammals. Most commonly, it is transmitted to humans in inadequately cooked meat or is ingested when food is contaminated with animal feces.
Toxoplasma
lives harmlessly in anywhere from 20 to 70 percent of Americans, its frequency depending on the social and economic group tested. In an immunodeficient patient, however, it manifests itself by fever, pneumonia, enlargement of the liver or spleen, rash, meningitis, encephalitis, and sometimes involvement of the heart or other muscles. Its most common focus of attack in AIDS is the central nervous system, where it can cause fever, headache, neurological deficits, seizures, and mental changes ranging from confusion to deep coma. On CT scans, the infected areas of the brain sometimes so much resemble the lesions of lymphoma that they are differentiated only with difficulty. This was the diagnostic dilemma that caused so much uncertainty in the care of Ishmael Garcia.
Toxoplasma
lives harmlessly in anywhere from 20 to 70 percent of Americans, its frequency depending on the social and economic group tested. In an immunodeficient patient, however, it manifests itself by fever, pneumonia, enlargement of the liver or spleen, rash, meningitis, encephalitis, and sometimes involvement of the heart or other muscles. Its most common focus of attack in AIDS is the central nervous system, where it can cause fever, headache, neurological deficits, seizures, and mental changes ranging from confusion to deep coma. On CT scans, the infected areas of the brain sometimes so much resemble the lesions of lymphoma that they are differentiated only with difficulty. This was the diagnostic dilemma that caused so much uncertainty in the care of Ishmael Garcia.
It is a rare AIDS patient whose nervous system escapes the pillaging of the disease. Even in the early period of HIV infection, some few people go through a transient period of neurological disabilities, which may sometimes appear even before AIDS itself has supervened; fortunately, this particularly distressing complication is far less common in early than in late stages of HIV disease, when it is more severe and called the AIDS dementia complex. Its eventual effects on cognition, motor function, and behavior can be devastating, but most frequently present initially as simple forgetfulness and loss of concentration. After a while, apathy and withdrawal become common symptoms, while some smaller number of patients complain of headaches or develop seizures. Should these findings not pass off when they occur early in HIV infection, they slowly worsen. In that case or in those far more common patients whose symptoms appear in the AIDS period, intellectual function often declines and difficulty with balance or muscular coordination appears. In the most advanced stages of the complex, patients are severely demented and show little response to their surroundings; they may be paraplegic and suffer tremors or occasional convulsions. These complications exist without any relationship to those processes caused by cerebral toxoplasmosis, lymphoma of the brain, or other opportunistic neurological disabilities such as meningitis caused by the yeastlike fungus cryptococcus. AIDS dementia complex is thought to be due to the virus itself, but its exact cause is unknown, and the cerebral atrophy seen on CT scan and biopsy is unrelated to any other factor. Of the many neurological problems associated with AIDS, this one and toxoplasmosis are the most common. Fortunately, the beneficial effects of AZT have resulted in some decline in its frequency.
Two cousins of the tuberculosis germ share the distinction of being the bacteria most frequently disseminated throughout the body of people with AIDS.
Mycobacterium avium
and
Mycobacterium intracellulare
(MAI), jointly called the
Mycobacterium avium
complex (MAC), are present in about half of AIDS patients when they die, having caused a wide variety of symptoms during life. MAI is now a more frequent cause of death than PCP. Fever, night sweats, weight loss, fatigue, diarrhea, anemia, pain, and jaundice are often attributable to these marauding twins. Although the complex rarely causes death on its own, its wasting effects are major contributors to the general debilitation and malnourishment that further weaken defenses against other invaders.
Mycobacterium avium
and
Mycobacterium intracellulare
(MAI), jointly called the
Mycobacterium avium
complex (MAC), are present in about half of AIDS patients when they die, having caused a wide variety of symptoms during life. MAI is now a more frequent cause of death than PCP. Fever, night sweats, weight loss, fatigue, diarrhea, anemia, pain, and jaundice are often attributable to these marauding twins. Although the complex rarely causes death on its own, its wasting effects are major contributors to the general debilitation and malnourishment that further weaken defenses against other invaders.
These are just a few of the manifestations of AIDS. Lengthening the list serves only to name some of the other common problems that beset patients, but it cannot even approach the complete inventory of suffering: the blindness of the retinitis resulting from CMV or
Toxoplasma
infection; the massive diarrhea that can have any of five or six causes, or sometimes none that are identifiable; the meningitis or occasional pneumonia of cryptococcosis; the thrush or swallowing difficulties of candidiasis, and perhaps the slimy wet ooze of its skin lesions; the discomfort of herpes around the anus; the fungal pneumonia or bloodstream seeding of histoplasma; the bacteria typical and atypical; the more than a score of creeping, crawling things with names like
Aspergillus, Strongyloides, Cryptosporidium, Coccidioides, Nocardia
—their time has come and they act like looters after a natural disaster, which is exactly what they are. Though they pose no danger to people with normal immunity, every one of them is a bane to those with a depleted store of CD4 lymphocytes.
Toxoplasma
infection; the massive diarrhea that can have any of five or six causes, or sometimes none that are identifiable; the meningitis or occasional pneumonia of cryptococcosis; the thrush or swallowing difficulties of candidiasis, and perhaps the slimy wet ooze of its skin lesions; the discomfort of herpes around the anus; the fungal pneumonia or bloodstream seeding of histoplasma; the bacteria typical and atypical; the more than a score of creeping, crawling things with names like
Aspergillus, Strongyloides, Cryptosporidium, Coccidioides, Nocardia
—their time has come and they act like looters after a natural disaster, which is exactly what they are. Though they pose no danger to people with normal immunity, every one of them is a bane to those with a depleted store of CD4 lymphocytes.
The heart, kidney, liver, pancreas, and gastrointestinal tract are affected in numerous ways by AIDS, as are the tissues less commonly thought of as specific organs, such as the skin, blood, and even the bones. Rashes, sinusitis, clotting abnormalities, pancreatitis, nausea, vomiting, draining sores and noxious discharges, visual disturbances, pain, gastrointestinal ulceration and bleeding, arthritis, vaginal infections, sore throat, osteomyelitis, infection of heart muscle and valves, kidney and liver abscesses—there are many others. Not enough that this disease depletes and dispirits, many patients feel humiliated by the details of their ordeal.
Kidney and liver function are often affected; there may be conduction or valvular abnormalities of the heart; the digestive tract betrays its owner in any number of ways; the adrenal and pituitary glands sometimes lose their power. When bacterial infection is no longer controllable, the familiar picture of sepsis supervenes. All the while, malnutrition and anemia are further weakening the body’s ability to slow down the processes of destruction. The malnutrition is often aggravated by huge protein losses through the damaged kidneys, resulting from a rapidly progressive condition of uncertain cause, called HIV-associated nephropathy (kidney disease). The nephropathy may go on to terminal uremia within three to four months of onset.
Even without direct involvement by infection, the heart in AIDS patients occasionally becomes enlarged and may fail, or it may develop a rhythm irregularity leading to sudden death. The liver, too, is susceptible to attack, not only because of AIDS itself but because so many patients are concomitantly infected with the hepatitis B virus. CMV, MAI, tuberculosis, and several of the fungi have a predilection for the liver. The hapless organ is not only battered by the disease but by attempts to treat it, as drug toxicity affects its performance in many ways. The liver is found to be abnormal in some way or other in 85 percent of autopsied patients.
The entire length of the gastrointestinal tract is a vast twisting tunnel of opportunity for the various predators of AIDS. From the herpes and that wide assortment of ulcerations and infections around and in the mouth to the running sores and problems of continence at the anus, the torment of the final months may be magnified by the involvement of so many structures that it inhibits eating, interferes with digestion, and produces uncontrollable watery diarrhea that not only is a source of constantly recurring distress but also makes it difficult to maintain proper hygiene of the raw areas around the anus and rectum. To imagine extracting a scrap of dignity from this kind of death is beyond the comprehension of most of us. And yet the indignity itself sometimes brings out moments of nobility that overcome for a while the reality of anguish—arising from sources so deep, they can only be marveled at, for they surpass understanding.
An intact immune system is needed not only to resist infection but to inhibit growth of tumors as well. In the absence of an effective defense, certain kinds of malignant processes find a favorable environment in which to make their appearance. HIV has been particularly conducive to one form of cancer previously so rare that I had seen exactly one case, in an elderly Russian immigrant, since my graduation from medical school almost forty years ago. The incidence of this malignancy, Kaposi’s sarcoma, has been magnified by a factor of well over a thousand—from 0.2 percent of the general population to more than 20 percent of Americans with AIDS. It is by far the most common tumor seen with this disease, and for as-yet-uncertain reasons, it afflicts a greater percentage of homosexual men (40 to 45 percent) than IV drug abusers (2 to 3 percent) or hemophiliacs (1 percent). These figures reflect only the people in whom the diagnosis is made during life. When autopsies are done, the frequency of KS triples or quadruples, making its presence somewhere in the bodies of gay men even more common.
In 1879, Moritz Kaposi, a professor of dermatology at the University of Vienna Medical School, described an entity he called “multiple pigment sarcoma,” consisting of a group of reddish brown or bluish red nodules that originates on the hands and feet and advances along the extremities until reaching the trunk and head. In time, stated his report, the lesions enlarge, ulcerate, and spread to the internal organs. “Fever, bloody diarrhea, haemoptysis [coughing of blood] and marasmus set in at this stage, and are followed by death. At the autopsy, similar nodules are found in large numbers in the lungs, liver, spleen, heart and intestinal tract.”
Sarcoma
is derived from the Greek
sark
, meaning “flesh,” and
oma
, meaning “tumor.” These growths originate in the same kinds of cells that give rise to connective tissue, muscle, and bone. In spite of Kaposi’s admonition about his disease that “the prognosis is unfavorable . . . and fatal termination could not be prevented by extirpation, local or general, or the administration of arsenic [a favored treatment for cancer at the time],” physicians for a century underestimated the danger of this unusual malignancy.
is derived from the Greek
sark
, meaning “flesh,” and
oma
, meaning “tumor.” These growths originate in the same kinds of cells that give rise to connective tissue, muscle, and bone. In spite of Kaposi’s admonition about his disease that “the prognosis is unfavorable . . . and fatal termination could not be prevented by extirpation, local or general, or the administration of arsenic [a favored treatment for cancer at the time],” physicians for a century underestimated the danger of this unusual malignancy.
Because the progression of KS was known to be slow, requiring “three to eight years or more,” subsequent textbooks most commonly employed the word
indolent
to describe its course. Thus was conveyed an erroneous message about the basically lethal nature of the malignancy, even though some authorities continued to write of its deadly manifestations, such as massive intestinal bleeding. The word
indolent
, in fact, appears in the original 1981 reports in British and American medical journals of outbreaks of Kaposi’s sarcoma among gay men. So alarmed, however, were the authors of those reports by the sudden raging aggressiveness of a disease traditionally regarded as lethargic that the American article saw fit to remind its readers that the course had sometimes been known to be “fulminant, with extensive visceral involvement”; the paper published in England made the same case and gave it immediacy by pointing out that “half our patients were dead within 20 months of diagnosis.” Clearly, this was a new form of KS, suddenly far more worrisome than even Kaposi had warned.
indolent
to describe its course. Thus was conveyed an erroneous message about the basically lethal nature of the malignancy, even though some authorities continued to write of its deadly manifestations, such as massive intestinal bleeding. The word
indolent
, in fact, appears in the original 1981 reports in British and American medical journals of outbreaks of Kaposi’s sarcoma among gay men. So alarmed, however, were the authors of those reports by the sudden raging aggressiveness of a disease traditionally regarded as lethargic that the American article saw fit to remind its readers that the course had sometimes been known to be “fulminant, with extensive visceral involvement”; the paper published in England made the same case and gave it immediacy by pointing out that “half our patients were dead within 20 months of diagnosis.” Clearly, this was a new form of KS, suddenly far more worrisome than even Kaposi had warned.
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