The Story of My Illness

I am a pediatric urologic surgeon who came to work one morning to perform a renal transplant only to realize that I was, myself, ill. It was a typical Tuesday morning, the day of the week I always reserved for urinary and pelvic reconstruction in children with renal insufficiency. On this day I was scheduled to perform a living related donor transplant in a child I had been following for years. Despite my commitment to this child and family, I arranged for another surgeon to take my place, as my fever and fatigue would prohibit me from operating safely.

I called my wife, Maureen, and together we went to see my primary physician. He ordered a chest X ray and blood work and indicated that he would call me with the results. We went home to await his call. That call was devastating. My hemoglobin level was half of what was normal, and my white blood cell count (neutrophil) was dangerously low. My wife called a dear friend, Cindy, who happened to be an oncology nurse. It was her day off, so she drove to our house to take us to the oncology unit where she worked. Before this day would end, I would receive a bone marrow biopsy and be given a preliminary diagnosis of leukemia. I was reassured that I was most likely suffering from chronic myelogenous leukemia, a relatively indolent form of leukemia that can often be managed without a major disruption of one’s life. I was given an appointment to return the next day to receive two units of blood and to confirm the biopsy result.

With a degree of cautious optimism, my wife and I returned home to gather our thoughts. When we returned for our appointment, I could sense an air of unease—that unease so often expressed by reluctance to fully engage the eyes upon greeting. As I was placed into an exam room, I was told that I would be seeing a different oncologist from the one that I had seen the day prior, the one who specialized in leukemia. “Not good,” I thought. I was informed that I would receive a blood transfusion and be admitted for induction chemotherapy—my diagnosis was now acute myelogenous leukemia (AML). In just 24 hours, my life was forever altered. The prior week I was at the peak of my surgical career, with national prominence in my field of practice, but that day I was . . . “Who am I? Who am I now?” I wondered.

An intravenous line was placed, and a blood transfusion begun. My wife was escorted into the hallway as she collapsed in despair. I asked those who remained to allow me a few minutes alone. I watched as the blood progressed from the bag to the line and then entered my body—the body that had been, until now, so reliable, so seemingly indestructible. It seemed so invasive, so violating, so desecrating. I cried, almost uncontrollably. “When was the last time I cried?” I asked myself. I couldn’t even remember. I was to be admitted upstairs to the oncology unit. So I had to gather my composure; I couldn’t let my family see me like this. As I was wheeled to my room, we passed other patients who were further along in their journey. Some were obviously weak, requiring assistance with ambulation. Others had those sparse, patchy remnants of hair. It occurred to me that they represented my future.

The first order of business was placement of a central line. No problem: I had placed hundreds of these myself and knew exactly what to expect. But then came the chemotherapy. I was mostly prepared for what was to come—the nausea and vomiting and the strict protective isolation with very restricted visitation: no flowers, no raw fruits, and no vegetables. Visitors would require a mask and gown. My wife was my lifeline. She brought in a mattress, moved the furniture in the room aside, and slept every night in my hospital room. She almost never left. Sometimes she would lie beside me in my hospital bed, and we would find comfort in each other’s arms. I had so very much to live for.

As I was dealing with the side effects of chemotherapy, the genetic markers of my malignancy began to return from the laboratory; slowly, progressively, one or two each day. Each one would portend a worse prognosis than the last. My oncologist was the kindest and most gentle physician I had ever experienced. I remember him sitting with my wife and me, detailing the significance of my tests. I learned that if I were to have any chance of survival I would require a bone marrow transplant. Because of the poor prognosis, it would be a complicated transplant, and I could not be treated locally—meaning that I would have to leave my family. As it turned out, transferring to another facility would not be as smooth as I had anticipated. In the days preceding the transfer, I developed sepsis with high fever, accompanied by what by then was decidedly less tolerable: nausea and vomiting. My endurance was exhausted. I became unstable and required an air ambulance transport to the chosen cancer center, an institution that was several states away.

As we left the hospital, one of my most vivid memories was being wheeled through the lobby of the hospital to the car that was to take me to the airport. A large crowd of friends and colleagues had assembled to send their love. As I passed through the crowd, these friends, surrounding me on each side, were wishing me their best and offering up their thoughts and prayers. By this time I was becoming disoriented, but I could recognize something, something in their eyes: “They don’t think I’m coming back!” I thought.

Accompanied by my wife, children, and Cindy, we flew cross-country toward our destination. The flight seemed so long and so cold. Little did I know that I was to feel cold, literally, for the next two years, due to profound loss of weight. Once the plane landed, I was immediately transported to the emergency department. There were no beds available; the hospital was running at 110 percent capacity. The first night I would spend tucked away in a small alcove of the emergency department, with Maureen and Cindy sleeping on a couple of very small and very uncomfortable chairs.

Finally, a room became available, and I could hear my nurse calling in a report to the nurse who was going to receive me in the new unit. “He’s a surgeon from Ohio,” she said, and added, after a pregnant pause: “No, this one seems to be quite nice.” I was sent to the X-ray suite, for a CT scan of the head and chest, on my way up to the room. In the confines of the scanner, I developed sudden and massive emesis. I struggled to get my mask off my face, but it was too late—I had aspirated. I was evacuated from the scanner, suctioned, and cleaned up. I seemed to be okay. Over the next several hours, however, my condition worsened, and I was transferred to the intensive care unit.

I was by then very hypotensive and was placed on vasopressors. I was developing multisystem organ failure from sepsis. I was essentially unable to converse, but I could make out some of the conversations that were going on around me. They were explaining to my wife that they couldn’t intubate me because patients with white counts as low as mine basically never survive on ventilation. Instead, they were able to support me with a BiPAP mask and 100 percent oxygen. The head of my bed was raised as high as it would go. “His chest X ray is a white out,” I heard. “What?” I thought. I was now sufficiently aroused that I could ask to see my X ray. They pulled a computer screen to my bedside and pulled up my chest film. I couldn’t see it; I needed my glasses. I remember trying to put them on my head, but there was no way that they would fit with the BiPAP mask in place. I folded my glasses so that I could hold one lens up to one eye in order to see the screen. Both of my lung fields were completely opacified, with the exception of a tiny crescent at the very top of each lung. “My God,” I thought. “Is that me?” Over the next several hours I would fade in and out of consciousness. “My bed is moving,” I thought. “I am being taken down endless corridors with strange lights and very odd, distorted walls. No, I’m hallucinating.”

Surprisingly, over the next week, I slowly began to recover from my multisystem organ failure. More surprisingly, my neutrophil count began to gradually improve. I would ultimately be found to be in a short-lived remission. This would buy me sufficient time to gain enough strength to undergo a bone marrow transplant. I received a matched unrelated donor bone marrow transplant that would prove to be successful. The transplant process required greater endurance than anything I had ever encountered. Once again I struggled with nausea and vomiting, much more intense than what I had experienced earlier. I also experienced intense and protracted pain, with the development of mucositis and viral cystitis. Unexpectedly, however, the greatest drain on my endurance was the emaciation associated with this entire process. I had severe anorexia and lost 40 pounds in weight. I was always cold, so cold that I would dread my daily shower, which was pure torture. Even walking would prove difficult, and for a time I required tremendous assistance with ambulation. I would be placed in a harness with handles in order to allow assistants to support my weight. The nights were long and lonely. There were screams from other patients who were in fear or in pain. The little sleep I was able to get was regularly interrupted by the piercing sound of alarms.

As I lay awake my thoughts would repeatedly—no, constantly—return to the same questions: Why? What about my family? Had I done enough to provide for them in my absence? Why me? Why now? How could this have happened at the very peak of my career? A few short weeks ago my career represented an important and unique contribution, both regionally and nationally. Would I ever contribute again? Would I ever be relevant again? Who am I? Who am I . . . now?

Edmund Pellegrino has so accurately described the fact of illness as severe vulnerability, a state of wounded humanity.^{Footnote 1} He defines humanity according to three concepts:

1. 1) Freedom of action: Of course, the body is the means of this action, and mine just wasn’t working.

2. 2) Freedom to make rational choices: Even with a very substantial amount of background medical knowledge, my illness involved concepts with which I was incompletely familiar. Further, my mind wasn’t as clear as I was used to it being. I had to rely on others, much more than I could have anticipated, for the choices that I made.

3. 3) Freedom from the power of others: I was, in all reality, completely at the mercy of the integrity, competence, and motivations of others.

As fate would have it, I survived. I returned home, and with great effort directed at exercise and attitude, I continued to progress until I had almost attained my baseline strength and stamina. Eight months after my release, a routine follow-up bone marrow biopsy revealed equivocal results. I would have to wait several days for the result of more sophisticated tests on the biopsy. When the call came, it was devastating—I had relapsed. I could barely remain standing. They offered me two choices: (1) a repeat bone marrow transplant or (2) forgoing treatment and letting the malignancy take its course. I was so conflicted. I wanted very much to live but could not imagine going through another transplant. I sought the advice of a close friend and colleague with whom I had worked essentially my entire career. The decision was impossible, even with all the help I could muster. Ultimately, I convened my entire family, and my wife and I presented the dilemma to our children. I indicated that, after a great deal of thought, I had decided to forgo a second transplant, as the first had been so disruptive to my family, and because the chance of survival was so low. Our children were outraged with this decision. My youngest son responded: “You won’t know what I become.” I was sufficiently moved that I immediately got up, packed my suitcase, and once again my wife and I made our way to the cancer center.

As it happened, a procedure that was in its infancy at the time of my first transplant had become more generally available. This was what was referred to as a haplotype-matched bone marrow transplant. This would originate from one of my children. They were all tested, and one, my youngest son, was found to have natural killer T cells that were mismatched for antigens on the cell surface of my malignant cells. Again, I received ablative chemotherapy. My son had his bone marrow harvested, and at the appropriate time his marrow was infused into my bloodstream. For a time, I received immunosuppression to prevent graft-versus-host disease. I was again cast into that intensive struggle of nausea, vomiting, pain, and emaciation. The graft took perfectly, and ultimately I recovered. I am now almost five years out from transplant with no evidence of disease. Although my strength and stamina have returned to near normal, I have been unable to return to my practice because of the risk of exposure to all of the viruses in the pediatric population that I had served. Further, I have painful peripheral neuropathy in my feet, eliminating my capacity to stand throughout the long surgeries that my practice entailed.

What I Have Learned

What have I learned in this life-altering process? I have learned about meaning, kindness, and the vast differences (greater than I could ever have imagined) in quality among providers and hospitals.

What I Hope For

Since my return, I have had the great opportunity to sit on the ethics committee as well as the physician practice evaluation committee of my institution. It is my distinct hope that I will learn enough in this process to understand how medicine might be changed for the good of the patient. Initially, I thought the answer was professionalism. Certainly, we all recognize that we are facing a crisis in professionalism on a national basis. Unfortunately, frustratingly, just as the policies and procedures we have established to eliminate medical error have been less productive than initially hoped, so have our efforts on professionalism. The fact is, with very rare exception, providers know how they should and should not behave. They know that their behavior can influence the outcome of care. They know that they should remain current from the perspective of knowledge and skill pertinent to their practice. They know that their inability to work respectfully and cooperatively with other providers is fundamental to the effacement of coordinated care and that this inability sets the stage for error. Further, they know that the manner in which they engage their patients is fundamental to the establishment of trust and hope so essential to a positive outcome.

How, then, can we possibly hope to change the character of providers and the culture of hospitals? I now believe that the answer will be more fundamental than an effort on professionalism alone. It will require revisiting the very philosophy of medicine: how we are to think about who we are and what we do. So much has changed since the wonderful work of Pellegrino and Thomasma, in which they described medicine as occurring precisely at the interface between the provider and the patient. The provider worked for the patient. The professional would make a promise, a “profess-ion,” to the patient as to his or her current competence as well as advocacy and ownership regarding the outcome of care. The medical action would be technically right and morally good. This implicit contract could only be based on the character and virtues of the provider—ultimately, there can be nothing else. The commodification of medicine has changed this dynamic, irreversibly. Physicians now, for the most part, work for hospitals. Documentation and billing activities erode the face time that providers should spend with their patients or in reflection as to whether or not the judgments they have made should be revisited. The moral angst is palpable. How do we change institutional culture and virtue in the face of all these adversities? It is to this end that I have now become a graduate student in philosophy. It is my hope that, somehow, I might turn my tragedy into a contribution.