by Azra Raza
Tanha gayay kyoun, ab raho tanha koi din aur
—GHALIB: EPISTEMOLOGIES OF ELEGANCE
Our paths had to cross again some other day
Going alone, now stay alone, until some other day
THE FIRST THING Andrew said after his diagnosis, his mother, Alena, told me when I met her at the hospital, was, “Call Azra, Mom. She is on the cutting edge of cancer. I want her involved in my care. She’ll make sure I’m okay.” The words cut deeply and reminded me why I had quit my fellowship in pediatric oncology forty years before.
Two of my older siblings were already doing their residencies in Buffalo, New York, so I landed there on January 2, 1977. Three weeks later, Buffalo was hit by the Blizzard of ’77. One hundred inches of snow fell within three days, and high winds blew the snow into drifts of thirty to forty feet. My brother and sister and their spouses got stranded in their respective hospitals. The two families lived in a duplex. Suddenly, I was the only adult, with five children between the two homes. We huddled in one living room, ate lots of bread and cheese, and watched Roots and Welcome Back, Kotter, which my thirteen-year-old brother, Abbas, was obsessed with.
When life returned to normal, I began making inquiries about finding work, as I had a six-month gap until the start of my internship in July. My sister Atiya, a third-year resident in pediatrics at The Children’s Hospital of Buffalo, had done a rotation at Roswell Park Memorial Institute. When she told Arnie Freeman, the chief of pediatric oncology at the institute, that I wanted to be an oncologist, he offered me a fellowship for six months so long as I was at least half as good as my sister. So I began a pediatric oncology fellowship. Within a couple of weeks, it was obvious that I would not last, but not because I was incompetent. I could not handle dying children.
Judy Ochs, my attending physician and Atiya’s close friend, had a serious talk with me one afternoon when she found me sobbing yet again in one of the back rooms. Frustrated, she marched me into the fourth-floor, windowless corner office of one Harvey David Preisler, chief of the adult leukemia program, and handed me over to him, saying, “Please give her a chance on your service. She might survive it. She has potential if she can face the pain.” Harvey tried to conduct some kind of an interview, but I was too heartbroken over losing a four-year-old girl to leukemia that day. I did, however, report to work on his floor early the next morning, thus beginning my lifelong association with the man who would become my husband eight years later.
Shortly afterward, the famous Elisabeth Kübler-Ross, the author of the pioneering book On Death and Dying, came to give grand rounds at Roswell Park. She was the first to describe five distinct reactions to death, both in patients and their loved ones: denial, anger, bargaining, depression, and acceptance. The important point she made was that acceptance, though hard to achieve, brought a modicum of relief, a sense of tranquility, and may even lead to a sharper appreciation of the larger issues of life and death, a much-needed inner peace.
Kübler-Ross spoke thoughtfully and calmly and had such a compassionate manner that I mustered up enough courage to ask her a question at the end of her talk. “If you could give me one piece of advice about informing terminally ill patients how much time they have left, what would it be?” I asked. She thought for only a few seconds before replying, “Don’t volunteer the information.”
Throughout Andrew’s sixteen months of illness, I agonized over the cruelty of the choices we oncologists offered. The issue became especially poignant when considering fundamental existential questions for Andrew. Is it less painful to let cancer kill him when experience and observation clearly indicate that the chance of recovery is practically nil, or should experimental drugs be offered with their attendant insufferable toxicities, prolonging survival by mere weeks at best? In the absence of experimental drugs, when the tumor began to invade organs, causing unbearable symptoms like the severe headaches and incessant, projectile vomiting, what was the right course of action? Palliation with pain medications and comfort care or aggressive attempts to produce remission with radiation and more chemotherapy, knowing that hope was nonexistent for any long-term remission?
Obviously, if there were even a remote possibility that a new experimental trial could help Andrew, the oncologists would have offered it themselves, but somehow, the patient and families have lost trust in their oncologists. His twenty-five-year-old sister searched desperately for any treatment strategy to help her brother, feeling, as many patients and their families do, that the burden of finding treatment rests with them. Why is this happening?
One reason relates to agency. On a daily basis, I have conversations with patients who are very, very sick. They want to have some control over what happens to them. Disease management, especially a chronic one, is truly a bilateral affair.
When treating low-risk MDS—a disease that requires complex long-term planning enacted on an evolving landscape of both illness and treatment—a patient’s trust and confidence in a physician is directly proportional to the sense of ownership and agency the patient feels. The following patient is a perfect example of this empowerment when it works.
4-23-2018
My name is Donna Meyers and I am 80 years old. I was diagnosed with MDS almost twenty-five years ago when anemia was detected. I was told of its seriousness and that I had to find a hematologic oncologist. Of course I was scared and started interviewing doctors. I met Dr. Azra Raza at Rush University Hospital in Chicago and knew immediately that she was the one I could trust with my life. She, from the beginning, made me a part of the process and I knew this would be a partnership. For me feeling that I had some control over my illness gave me a feeling of hope and agency.
My respect and admiration for Donna is directly proportional to the equanimity and poise with which she has handled, now for a quarter century, extremely challenging physical issues. A draining, sapping, wildly fluctuating profound anemia, weakness, uncertainty, the exhausting business of repeated recruitments into experimental trials with questionable benefits and unpredictable side effects. The burden of having to travel regularly to see me in Massachusetts and now in New York. Yet I have never heard her complain once about any of this. She quietly and calmly arranges everything in her life around the one thing she is certain of: the bimonthly blood transfusions. At the Northwestern University infusion center in Chicago, Donna has been a familiar face for decades; getting Procrit and Aranesp shots, receiving transfusions, undergoing added tests and treatments I prescribe long distance. Her wonderful local hematologist, Dr. Olga Frankfurt, and I remain connected via Donna’s super-diligent mediation. She maintains records of her hemoglobin and iron levels, number of transfusions, and medications she is taking, all on her cell phone, which she whips out with equal ease in airless hospital rooms and fancy restaurants to update me on the latest numbers. You might be surprised to hear about the quality of her life in all these years.
Despite the constant intrusion of her disease’s unpleasant reminders, Donna has been able to lead a very fulfilling life. She never stopped working in her profession and never stopped traveling. When in Chicago, she plays golf, has many hobbies, and has a ton of friends and family to socialize with. She enjoys her large family and travels extensively to see them. In short, she has more energy at eighty with her debilitating MDS than most healthy people have at forty. She refuses to let the disease dictate her daily activities, and she refuses to be pitied. Although her loving children and husband are always ready to accompany her, most of her trips to see me in New York are made by herself.
During all these years, every decision was jointly made by Azra and I; for 25 years we have managed my MDS together. There is a deep bond, a personal relationship, and love, we have developed over the years. I feel that this has been our journey. I am still alive at 80. I get up every day and say YES! I’m alive and I will do what I want and go where I want. Thank you to my family, my husband and to my wonderful friend, Azra, my doctor. I love you all.
Donna’s story highlights the importance of
agency in a chronic disease. One of my younger patients, Betty, suffering from a profound aplastic anemia, required multiple blood and platelet transfusions weekly. She looked exceptionally frustrated in clinic one day because of the long commute and inordinately protracted waiting times in the infusion center. She was exhausted to the point of crying. I asked her to inquire if we could arrange some of the transfusions in a facility five minutes away from where she lived. According to Tim, her doting husband, Betty was a different person within twenty-four hours. Because she had control of something finally—calling clinics, arranging appointments, making her wishes known, bargaining schedules—she was empowered. She was energized.
The concern with this bilateralism is the asymmetry between patient and physician. The patient’s experience with cancer is singular. Their treatment from their own oncologist is supplemented by frantic literature searches, Dr. Google, and curbside consults with anyone and everyone remotely connected to the medical field. The problem is that even when patients are well informed, the one thing they lack is experience. Their knowledge is half-baked and creates false hope. When patients are not offered some therapy apparently successful in another type of cancer to treat their own, they feel cheated by their oncologists and start looking every which way on their own. Oncologists, on the other hand, have the benefit of having treated hundreds of similar cases in addition to years of rigorous training. This has earned them the right to make treatment suggestions. The responsibility of the oncologist when offering a choice between multiple courses of action to the patient is inversely proportional to the patient’s experience and knowledge of the disease.
Another reason why patients and their families search frantically for treatments other than the ones offered by the primary treating oncologist is the way baby steps toward a new cancer treatment strategy are prematurely blown out of proportion by the media. Harvey and I experienced that firsthand in 1998 with an anti-angiogenic drug that showed efficacy against a variety of cancers in mice but zero response in human trials. More recently, this has happened for immune therapies. The success of one immune-based strategy called CAR-T therapy, in curing an extremely rare kind of childhood leukemia, is proclaimed from the rooftops as though it were the cure for all cancers. Bombarded with the massive coverage of these rare success stories in the media—contrasted with no mention of these therapies by the oncologist—patients begin to question the knowledge and intent of their doctors, start independent explorations of their own.
In addition to the sensationalized media reports of voguish therapies, there is yet another reason for this behavior. Cancer is a family affair, and not just for emotional reasons. Today, the traditional, paternalistic paradigm of medical treatment where doctors unilaterally made all treatment decisions is replaced with a more democratic system promoting autonomy and self-determination. Participation in treatment choice is a right of patients. This requires access to information, and online resources play a seminal role in this journey, especially for younger patients. Families are no longer the helpless bystanders, particularly as the end approaches. In their anxiety to assure that all is done for their loved ones, they question the expertise of oncologists, worry that they might not have received all the information to self-advocate. They search furiously, trying to find a reason to be hopeful in a desperate situation, knowing that nothing they choose is likely to work, yet unable to stop themselves from ceaselessly trolling the web.
IS DYING A FAILURE?
In March 2004, I was in New Canaan, Connecticut, for a lecture. I happened to pick up a copy of the Fairfield Weekly and read a brilliant piece by Lorraine Gengo on the English artist Barbara Griffith, whose New Canaan Observed: A Field Study explored “how two individuals relate to the group and how we fit into society and what that takes out of us.” In the article, Ms. Gengo discussed a painting by Ms. Griffith titled, The Role of Synchronized Clothing and Movement in Evading Death. This is how Ms. Gengo described it. The painting bears newspaper headlines: TEA DANCE FIGHTS CANCER; TENNIS OUTING FIGHTS CANCER; POM-POM HATS FOR BATTERED WOMEN. The women in the painting appear as a “chain-gang of domesticity”; in their conformity, which Ms. Gengo describes as camouflage, she sees a “religious procession,” the goal of which is the defiance of death. “As a marching body,” she writes, the women “become a powerful organism which rejects any non-conforming cell to affirm its own health and virtue. The fallen woman (or non-conforming cell) is the statistical sacrifice, the one in five who must die that they may live. It is a poignant primitive rite to avert evil.” It is a symbolic defiance of mortality through obsessive exercise by toned and tanned women jogging in groups.
Ultimately, the painting plays with themes that recur throughout our personal conversations and cultural discourse about cancer. Cancer is called a battle, a war, a fight; patients are the warriors, the foot soldiers, guided by the oncologists acting as their captains. The war is waged by groups comprising individuals, families, advocacy groups, industry, academia, and institutions, all joined in an effort to resist a malicious, evil adversary. The weapons used are surgery, chemotherapy, radiation therapy, and the occasional magic bullet. The individual patient is called upon to join the battle armed with a fighting spirit. This language is used by patients, doctors, families, the public, in formal meetings and informal discussions. It can serve as a positive reinforcement. Many patients take comfort in such militant metaphors; they put up a good fight, at least earlier on in the course of their illness. As the battle picks up and intensifies, however, the metaphor loses its power. The punishing, grueling, exhausting experience of dying is quite one-sided. No one feels heroic when they are throwing up or suffering from wearing, draining, unstoppable, persistent pain.
So it was for Harvey. In the second year of my husband’s diagnosis, his condition worsened rather dramatically, and he required repeated admissions for unpredictable, unexpected complications. Within the space of some eighteen months, the man went through a series of bewildering neoplastic manifestations and paraneoplastic syndromes; a new onset of severe asthma; drenching night sweats; exceedingly painful migratory polyarthritis; disfiguring facial edema; DVT; shingles; facial paralysis; tuberculous meningitis; and multiple episodes of fevers of unknown origin.
Once, his three grown children, Sarah, Mark, and Vanessa, arrived during such an emergency admission. Harvey was extremely close to all three and spoke to each of them on a daily basis. When he would get really sick, they would come in from the East and West Coasts at short notice and do everything possible to help out, including taking charge of Sheherzad with utmost love, responsibility, and concern. One day, they cornered me. “Az, Dad never talks to us about his illness. The first we hear something is seriously wrong is when you call and tell us to come urgently. Can you please encourage him to talk to us so we have a better idea of what is going on?” That night, I broached the subject with Harvey.
He looked wistful. “What can I tell you? So much of talking to others is about asking for help. No one can help me. So why bother them?”
I persisted, arguing it would help them deal with the situation better. A scientist as he was, his response was quintessential Harvey. He rejected outright the added burden of trying to put others at ease. “Az, I cannot begin to describe to you the psychic energy I have to invest just to carry on with business as usual given the dizzying turn of events I am facing on a daily basis. I don’t have energy left over to be concerned about how others are handling my illness. Even my own children. I want to, but cancer is draining in more ways than I imagined. You can talk to them if you want, but I honestly can’t.”
For a cancer patient, the only war is a war with one’s own organs, where the self serves as a battleground. This battleground is unlike any other in that the body is both the theater of war and the combatant forces themselves. The fight begins as an inside job, a civil war. Cancer starts by attacking one organ and then expanding its reach. To fight this one enemy would be bad enough; unfortunately, the very weapons u
sed to subdue the enemy and contain the civil war—chemo and radiation therapies—cause collateral damage, hurting the body indiscriminately, injuring organs, diseased or not. So how do we define this war when the body has to shield itself from internal and external aggressions simultaneously? It is a war for the body, on the body, by the body. The patient, held hostage by inside and outside forces, becomes aware of parts they never knew existed until unbearable pain and inflammation or a popping tumor or damage by chemotherapy unceremoniously bring them into the conscious realm. Caught in an incessant struggle between life and death, the body reluctantly yields a portion to cancer one day and to radiation and chemotherapy the next. Eventually, there is total confusion, and it becomes unclear whether organs need to be shielded from cancer or from the treatment. Total anarchy is the only endgame for such amorphous perversity. We say at this point that cancer is “winning” the war. But what is killing the body is as much the treatment as the cancer. So who is winning the war and who is losing? Cancer, chemo, oncologists, the cancer enterprise?
The very terms meant to empower end up detracting from the profound human experience of an individual facing mortality head-on in all its chaotic savagery, the physical suffering, anxiety, the grief. The patient, clinging obstinately to life, can only win this war by reconciling the body with death. They could achieve a more peaceful triumph if the mind were prepared to accept the need for such a reconciliation before the two sides—cancer and the insalubrious, noxious, vitriolic, awful treatments—locked horns in their violent, bloody struggle. Such thinking is entirely missing from the science of cancer, a tradition requiring urgent reexamination.
The terminology of positive thinking also stigmatizes by indirectly blaming the victim. When Miriam Hansen, one of my dearest, smartest, most brilliant friends, died, several friends and colleagues at her memorial service at the University of Chicago spoke about how she had fought the battle of cancer and survived, with a good quality of life for a number of years, because of her positive attitude, her willpower. Because of all the people praying for her. Her husband, Michael, rejected this. He categorically stated that his wife, Miriam, was able to live for twelve years with various cancers not because of willpower or positive thinking or prayers but because of her oncologists and the medical staff caring for her. To think otherwise is to say that those who died had no willpower, no positive thinking, and no one praying for them.