Grief Connects Us

by Joseph D. Stern

  At City of Hope, I was a brother trying to support his sister, attempting to comfort her and to interpret the medical facts for our family. If I felt this as a trained physician, how were people without an intimate understanding of the world of medicine able to cope with these issues? Or was my knowledge and training actually getting in my way? Was it perhaps better to take my sister’s approach and not ask hard questions?

  Soon after Victoria’s diagnosis, I saw William E. Williams (interviewed in Chapter Nine), a thirty-six-year-old African American man with a newly diagnosed brain tumor, in my clinic. William was a delightful person with whom I developed an immediate rapport. A musician and gospel singer who played in his local church ensemble and traveled frequently with his pastor, he had been perfectly healthy until he had a seizure and lost consciousness while on the road. His friend rushed him to the emergency room at Moses Cone Hospital in Greensboro, where an MRI scan showed a brain tumor, most likely a low-grade glioma, or intrinsic brain tumor, which takes origin from the glial cells, the supporting framework that feeds and provides structure for the neurons of the brain. The emergency room physician started William on Levetiracetam, a drug to prevent seizures, and he had no further seizures for a week. He came to my office to discuss his situation. He was calm and trusting, but clearly terrified. William’s father accompanied him. The elder Mr. Williams peppered me with questions.

  I explained that William would need a craniotomy, or surgical opening of the skull, to remove the tumor as completely as possible. This would be his best chance of long-term survival. Depending on how aggressive the tumor appeared to the pathologist, who would determine its grade or severity, further treatment might include follow-up imaging with MRI scans at regular intervals and possibly chemotherapy or radiation therapy, or both. After more discussion, William agreed to have the surgery. We planned to go ahead with it the following week.

  Prior to surgery, we obtained an additional planning MRI, which allowed us to create a three-dimensional model of William’s brain using our navigation computer in order to reference our precise location during the operation. This is important in most brain surgeries, but particularly so with low-grade gliomas, since the tumor is often indistinguishable from the normal surrounding brain. Without this tool, it would be impossible to define adequate borders of a complete surgical resection.

  The marriage of advanced technology and neurosurgery has allowed greater precision in removing tumors, also making possible smaller openings in the skull. While I was having my discussion with William, I was picturing Victoria: newly diagnosed, scared to death, putting on a brave face, and trusting her doctors with her life. Just as William was afraid for his own life, she was terrified that she might die. I became aware of a certain audacity—an audacity bordering on hubris, in fact—in my suggestion to William that he needed to have the top of his skull opened up and that I was the man to do it.

  Detachment is an essential component of my job. As a doctor, I would not have been able to treat my sister. My judgment would be affected and I would make poor decisions, based more on emotion and less on professional opinion. While I cared about William, he was my patient and not a beloved member of my family. And I was glad for that separation. I had always walked comfortably inside the rarefied world shared by physician and patient, shielded by what radiation oncologist Matt Manning (interviewed in Chapter Eight) refers to as “emotional armor.” This armor allows doctors to continue working despite the many tragedies in the lives of those we encounter. If we are consumed by the fear and grief our patients and their families experience, we can no longer help them. Our challenge is to care, to empathize, to honestly appraise the effectiveness of our treatments (ideally with data, rather than anecdotally), and to learn from our mistakes. We must continually improve the way we care for our patients without becoming overwhelmed by this daunting task.

  My wife once asked me if being a surgeon felt like being a pilot. I see a crucial distinction: while both professions demand attention to safety and intimate knowledge of specific complex systems, in a plane crash the pilot dies, too. In surgery, if disaster strikes, the patient dies but the surgeon and the surgical team remain intact. There are clear limits to empathy. As Dr. Hoff said often, “We do not have a problem. It is the patient who has a problem.” At the end of the day, I walk away from a patient’s suffering and his disease. But when it’s a family member lying in the hospital bed, there is not as clear a separation. When we ourselves become patients, that safe distance ceases to exist. While we may not be flying the plane at that point, we are definitely on board.

  During the time I was treating William, everything about my work felt more difficult, and the weight of my responsibility became heavier. For the first time I was experiencing the acute awareness of surgery’s inherent dangers that comes as one reaches a high level of skill and technical accomplishment. Victoria’s illness was causing me to see more clearly the suffering and risks my patients were experiencing, matters I had previously blocked from my mind.

  William’s surgery went extremely well from a technical standpoint. I took out the entire tumor, confirmed the next day on an MRI. The tumor was long and cylindrical, extending below the motor cortex of the supplementary motor area, an area in front of the motor strip, into the motor control areas of his brain, which controlled his right leg movements. When William awoke from surgery, he had perfectly intact sensation but was unable to budge his leg at all. Later, the weakness began to improve and became confined to his foot. We took him for an emergency CT, which showed there was no evidence of stroke or bleeding at the surgical site. Over the course of months, William would completely recover his leg strength, but at the time I did not know what to expect. On top of this, the pathologist informed me that the tumor was not grade two, as we had hoped, but a higher grade of three. This carried a considerably worse prognosis and required both radiation therapy and chemotherapy, in addition to regular visits with a neuro-oncologist.

  Sitting in the small examining room in my office, I told William that his diagnosis wasn’t good. At the same time, I was thinking about my sister, about the pieces of bad news her doctors were compelled to dole out after each test result came back. I now understood what it must feel like to be on the other side. It was sobering and frightening. I fought back tears as I discussed what this tumor would likely mean to William, who was about to get married and had plans for a family of his own. I also told him what was going on with my sister. That afternoon I booked a flight to Los Angeles.

  At first, Pat was upset with me. He had carefully organized visitors to their home and to my sister at City of Hope. The timing of my visit would disrupt that schedule. Still, Victoria wanted me to come, and I was able to take the time off, so we went ahead with this new plan. The gravity of Victoria’s illness was clear; I felt a desperate need to see her. I had gone from daily communication with Dr. Fischer to nonresponsiveness from Victoria’s new doctors. Normally, physicians will speak with family members who are physicians to keep them updated on a patient’s prognosis, but the new doctors were not responding to my calls and messages.

  Our family was trying, without great success, to identify potential bone marrow donors. My older sister, Caroline, and I had arranged for DNA testing for HLA typing and were awaiting the results. In the meantime, Victoria’s sample was tested against the database of potential donors. The test had produced only one potential match out of twenty-two million.

  The process of learning about HLA testing and bone marrow registries opened entire new worlds to me. I learned about altruism in the Jewish world, through organizations such as Gift of Life. I learned that the Holocaust partly explains why it is hard for people of Jewish heritage to find matches. Because the Nazis eliminated six million Jews, many potential descendants who could have become donors simply do not exist. I had not realized how directly tied to ethnicity HLA typing is, although this makes sense. People are more likely to find potential donors from within their tribe or ethnic group
, just as they are more likely to find matches from among siblings and immediate family members.

  I also learned how timing is everything. Victoria became ill around Thanksgiving, which meant that the search for potential donors was interrupted by the Christmas and New Year’s holidays, a time when databases were difficult to access and charitable organizations were closed for up to two weeks. The prolonged waits added to the sense of anxiety and urgency surrounding Victoria’s illness.

  Leukemia was not Victoria’s first cancer diagnosis, but her third. She had been diagnosed with amelanotic melanoma about five years earlier after a mole on her neck appeared to grow and was removed surgically. Melanoma frequently looks black with the pigment of melanin, but a small subgroup of melanomas lose their pigment and are white. They can spread throughout the body but may appear discolored. This process of losing previous features can occur with cancer and is often why tracing the source of a malignancy can be difficult.

  While the margins of the original resection were not clear (melanoma tends to spread to the rest of the body after invading locally through the layers of skin to the blood supply, which is deep in the surface layer), Victoria underwent regular surveillance studies that showed no evidence of new melanoma tumors.

  A few years later, she was diagnosed with thyroid cancer. After discussions with an endocrinologist and a surgeon, she elected to have this mass removed. It was a difficult decision, as the general course of thyroid cancer can be slow, but in the face of a previous malignancy she became convinced it was the safe, appropriate choice. Both of these procedures were relatively straightforward, although the regulation of her thyroid function with replacement hormones proved imperfect and, at times, frustrating.

  Not surprisingly, these diagnoses created a heightened fear of cancer in my sister. Victoria became vigilant about her health and that of her family. She read about diet and wellness, ate wisely, exercised regularly, and practiced yoga. She believed that her lifestyle would safeguard her against future cancers.

  It is not uncommon for people to develop multiple, seemingly unrelated malignancies or for cancer to run in families. Understanding of the genetics of cancer is improving rapidly as we identify genetic predispositions. Our DNA has built-in mechanisms to correct mutations and prevent cells from multiplying out of control. Tumor suppressor genes such as p53 are part of the normal cellular machinery to halt the development of malignancies. Our cells are programmed to reproduce and to die in an orderly fashion. We have two copies of each gene as a safety mechanism, so if one is damaged the second will override this malfunction and continue to function properly. But if a person already has a mutation in one of the p53 genes, then another insult to the person’s DNA, such as a mutation like the loss of a chromosome, can lead to loss of normal cellular function and the subsequent growth of a tumor.

  Most of the time, these malignancies occur in patterns. Seemingly unrelated cancers are in fact related through damaged tumor suppressor genes. Some patients will develop multiple endocrine neoplasias, while some women will develop breast or ovarian cancer. There are also families with high rates of cancer in which another mutation, BRCA, associated with high rates of ovarian and breast cancers, is passed to female offspring.

  But Victoria was not a member of any of these cancer families. Other than our grandmothers, who developed malignancies at advanced ages, no one in our immediate family has had cancer. The three types Victoria had are not typically associated with each other; thyroid and pancreatic cancer go together, but melanoma, thyroid cancer, and leukemia do not. It was a perplexing trio of diseases that did not raise alarms because there was no known relationship between these separate cancers. A recent population study suggests that leukemia rates are consistent with random mutations, rather than the result of some inherited genetic propensity to developing leukemia. Victoria’s cancer history could be merely “bad luck.” Still, three malignancies in a relatively short period did not seem likely to be the product of random chance.

  Immediately after receiving the diagnosis, Victoria became obsessed with tumor suppressor genes and seeking an explanation for “why me.” I tried to explain p53 and some of the other things I knew about the genetics of cancer, but also—and most importantly—I told her about the limitations of our current state of knowledge about the disease. She was worried that if she had a genetic propensity to cancer, she might have passed it to her sons and that they would be at risk for developing malignancies as well. No conclusive tests currently exist to answer this question, but even if they did, it is unlikely that they could lead to prevention or early treatment. Later, as my sister adjusted to the new reality of leukemia, these questions seemed to recede in their urgency, although throughout her illness she was consumed by the desire to protect her children, both by preventing illness in them and by conquering her blood cancer and remaining available to them as their mother.

  It was the beginning of 2015. Victoria had been in the hospital continuously, first at Saint John’s and later at City of Hope, since Thanksgiving. She had initially received conditioning chemotherapy through Dr. Fischer at Saint John’s, but when it became clear that she would need to undergo a bone marrow transplant, he transferred her to the care of Dr. Margaret O’Donnell, a leukemia and bone marrow transplant specialist at City of Hope.

  Victoria and her family had gone from a close-to-home facility with a warm and accessible physician and staff to a specialty cancer hospital east of Los Angeles in the foothills of the San Bernardino Mountains. While everyone was helpful and efficient, this place felt much more like an institution.

  My sister was placed in a special HEPA-filtration room because her white blood count was dropping to dangerously low levels and she had to avoid exposure to other people and their germs. Everyone had to gown, glove, and put on a special filtration mask before entering. The isolating effects of her disease were hitting home. Some days, Victoria was allowed to walk in the halls, but only when her white blood count was at an acceptable level. When it was low, along with her ANC (absolute neutrophil count), she was restricted to her room and visitors were kept to a minimum. Her days were often long and lonely.

  I arrived in Los Angeles in early January. The plan was for me to stay with Victoria out near Duarte and give Pat a week of respite. Their boys needed to get back into the post-holiday school routine and adapt to having their mother away for long stretches. Pat had been driving to City of Hope from Santa Monica each morning, a trip that, depending on traffic, could take anywhere from one to three hours. He would spend the day at the hospital before driving home to have dinner with Nick and Will and get them to bed. Our immediate family and several of Pat’s siblings had rotated in and out of the Whelan household, while friends organized meals and carpools. The gravity of Victoria’s condition was sinking in. Pat began to recognize that this would be a long, arduous fight for her life.

  After several weeks of commuting, Pat rented a small house in Monrovia, a short drive from City of Hope. I spent the week with my sister in her hospital room, sleeping at the rental house each night. At the end of my visit, I drove back to their home in Santa Monica and stayed with my nephews while Pat returned to Victoria’s bedside. I had spent little time on LA freeways and found the experience incredibly stressful, connecting between six highways each way to get between Santa Monica and Duarte. I knew we were fortunate to have the resources to manage this arrangement. I also knew that Victoria was lucky to have been admitted to a hospital specializing in leukemia to which she did not have to travel (many people had to come from out of state to be treated at City of Hope). I was reminded of how many issues the majority of my patients juggle while they put their lives on hold to battle illness or care for a sick family member. Through it all, they must figure out how to manage bills, prescriptions, childcare, and missed work.

  Before I’d arrived, I’d called to speak with Dr. O’Donnell or her treating physician. I left a message but never received a call back. This went on for several days. My sis
ter did not understand the treatment plan and anxiously awaited a meeting with Dr. O’Donnell to discuss the team’s strategy.

  Meanwhile, Caroline and I submitted cheek swabs to see if we qualified as potential bone marrow donors and waited for the results. Victoria was receiving chemotherapy to kill the blast cells (immature white cells), but the drugs were also destroying other blood components, including red cells and platelets. The plan was to give her a round of chemotherapy followed by a bone marrow biopsy to see if the blasts had disappeared. They would allow her a week or so to recover (and possibly let her go home) and then repeat another round of chemotherapy, followed by a second bone marrow biopsy. In an attempt to kill off her bone marrow, the second round of chemotherapy would be even stronger, followed by four days of whole-body radiation therapy. The course of treatment was a brutal assault on Victoria’s body, but she had no choice. If she did nothing, the leukemic blasts would take over and she would probably not survive more than a few months.

  Once the bone marrow is destroyed, the patient, without an infusion of new stem cells, will die. The whole process is precarious and time sensitive; you bring the patient to brink of death and then try to revive him or her with the transplanted stem cells. Even with donor-matched cells, there is a period of high risk prior to “engraftment,” when the new cells take hold. This is a crucial time when the recipient is susceptible to potentially fatal infections or other problems, such as internal bleeding.