You Can Stop Humming Now

by Daniela Lamas

  She found herself wondering how she would reply when someone asked her, “What do you do?” It had once seemed like such a simple question. She offered some potential answers that night as Jen worked her way down her back. Would she say, “Nothing”? Would she say she had retired? She paused for a moment. She would need more time to find the language that sounded right.

  As we sat in her living room, the clap of Jen’s hands on Meghan’s chest in the background, I couldn’t help but once again project my own self and ambitions onto her. That’s a foolish thing to do, of course. I can’t really understand her experience, as I grew up with the sense that life and time are essentially limitless, while Meghan had long been aware that the good part might be shorter than she hoped. Even so, she had been healthy long enough to go full force at a career, and she had found a field in which she excelled and work that felt perfect for her. In her midthirties she’d reached a point at which she should have been able to enjoy accolades and advancement. In a way, it all should have just been starting. Instead, when her boss asked her to sit on a national committee or teach courses, she said no, even though she wanted to jump at the opportunities. Reality intruded. She wasn’t sure what her lung function would be like in the year to come, or when she would next be admitted to the hospital and for how long. Cystic fibrosis had always been in her life, to be sure, mandating that she wash her hands regularly, that she not come into close contact with others who had the disease lest their germs pass on to her. But it had never so clearly limited her life’s course until now.

  Before the shock of her most recent hospitalization, she and Myles had been thinking, cautiously, about trying to have a child. She’d always assumed pregnancy to be off-limits. It is hard to plan to build a family if you expect to die not too long after your thirtieth birthday. But at a visit with a high-risk obstetrician, Meghan and Myles learned that she actually had no strict medical contraindication to carrying a baby, and slowly, it seemed, the couple had allowed optimism to creep in. They had started to imagine the possibility. Maybe they could have a family. Then she got sick, and though she made it home and was back at work and gaining weight, with her friends asking when she was going to start “popping out babies,” she felt uncertain. What would pregnancy do to her tenuous lung function? What if she got sicker the next time and was unable to rebound?

  When Meghan thought aloud about adoption, Myles, ever supportive, encouraged her to explore the possibilities, but she wasn’t sure adoption was the answer, either. Even without the physical stress of pregnancy, her lung function would worsen, possibly taking with it her ability to mother the way she would want. She and Myles had talked about this. He knew he might have to take on more and more parental responsibility if her health declined, and he knew, too, that he might be left to parent alone. After seeing him with his students, she trusted that he would be a great father, and it was an experience she wanted for him. Even so, the reality of it all was hard to fathom. When Meghan’s mother died, it had gutted her. How could she decide to bring children into a family, knowing that she might grow sick and leave them?

  Meghan had outlived the expiration date her doctor had given her. She had marked so many milestones that were far from certain when she was a child—she went away to college, lived alone, dated while on intravenous antibiotics, married. She had even started to think about her previously unimaginable fortieth birthday (no party this time, just a dinner at a nice restaurant). But maybe having a child was something she wouldn’t be able to do. Maybe going to work each day, coming home and eating dinner with her husband, maybe that was enough for now. She was grateful for these “average” things that she loved, she told me. Maybe there didn’t need to be anything more.

  She was quiet for a moment. I listened to the rhythmic sound of Jen’s hands against Meghan’s chest. I listened to her cough and heard the spit land in the cup. She would do it all again the next day and the day after that. “Right now, I feel good,” Meghan offered, smiling. She had managed a treadmill run earlier that day. And there were things to look forward to. In seven weeks, she and Myles were heading to Mexico for a vacation. Though she worried about leaving her comfort zone for the trip, uncertain what would happen if she got sick or needed her doctors, she planned to fill her Kindle with books and enjoy the time away. It would be sunny and the sand would be warm. “I like to travel, and I don’t know how much time anyone has,” she told me. “So what choice is there?”

  A few days after I visited Meghan, I worked an overnight shift in the ICU. The intern and I were sitting at our computers, scanning lab results, when one of the cystic fibrosis specialists walked into the unit. Surprised, I glanced at the time on the computer screen. It was just after 11 p.m.—she should have been home hours ago. It was clear she’d had a rough day. Her mascara was a little smudged, her skirt crumpled, and she walked with her shoulders slumped. She sat down next to us and exhaled loudly. “I have an admission for you,” she said. I gestured to the intern to start taking notes.

  The patient was a thirty-seven-year-old man with CF, she told us. That meant he was just about Meghan’s age—and just about my age, too. But he was dying. Perhaps due to his own genetics, perhaps due to bad luck and bacteria, his disease had taken a severe course. He had been in the hospital for over a month, battling infection after infection. Now his lungs, destroyed by nearly four decades of the disease, could barely work to exchange oxygen for carbon dioxide without a machine strapped to his face to blow air in and push it out. Even with the machine he was on the edge, and he might need to be intubated. I nodded slowly, taking in the story. “Probably there’s nothing we can fix,” the specialist told us. The patient wasn’t ever going to get a lung transplant—he was too weak to tolerate the surgery, and his other organs were failing, too—so there was no backup plan.

  Sitting in the quiet of the ICU workroom, I felt fairly sure that this patient was nearing the end of his life. But outside the room things were more complicated, and there was a young man who did not want to die. He would come to the ICU, and we would give him more and stronger antibiotics and hope there was some way we could make him better. “This sounds like a terrible situation,” I said. My words felt insufficient. “Thanks for that heads-up.” I imagined him dying under my watch that night. I took a deep breath. “You should go home!” I told her. “We’ll take care of him.”

  About twenty minutes later, our patient’s stretcher rolled through the doors, pushed by his nurses from the general medical floor. I took him in as he moved past. He was small and too thin, curled up on his side on the stretcher. I thought of how it must have been for him as a child, how no one expected him to live to be thirty or thirty-five, and how he had celebrated these birthdays that weren’t supposed to come, one after the other. As the years passed, I imagined, hope may have started to appear around the edges.

  I followed the stretcher to his room and stood at the side of his bed. His limbs were skinny and muscles wasted, skin so pale it was almost gray, eyes rimmed with bluish circles. He was too weak to walk. The machine he wore to help him breathe had been on his face so long that it had left red indentations in the thin skin on the bridge of his nose. He had made it to thirty-seven with cystic fibrosis, lived nearly a decade he wasn’t due to live, in a daily testament to the sparkling forward march of medical advance. But at the moment when I met him that night and pressed my stethoscope to his skeletal chest to hear his heart racing, none of that seemed to matter. I stood at the side of the bed and there was only a man who could not breathe without a machine strapped to his face. There was only the rapid beating of his heart, the smell of sweat and sickness, and the sadness that shook me, all of a sudden, because thirty-seven is still young, and he was dying.

  When I first decided to write about cystic fibrosis, I thought I would focus on the genetic therapies that promise to drastically change this disease, or perhaps on the new rules of infection control that prohibit one person with CF from being in the same room as another. I reached ou
t to a handful of experts, and about a week after I met Meghan and admitted the man with cystic fibrosis to the ICU, I sat down with Dr. Ahmet Uluer, who directs the Adult CF Program at Boston Children’s and Brigham and Women’s Hospitals. By then I had started to think about the disease in a context far broader than cutting-edge treatments and improved infection control. Still, I wanted to understand the changes that were on the horizon.

  Dr. Uluer and I met for coffee and then headed over to talk among the books and Legos in his small but comfortable Children’s Hospital office. On that day, Uluer was dressed slightly more formally than the everyday doctor’s garb of chinos, button-down shirt, and fleece jacket, and as we walked, he mentioned that he was going to a patient’s funeral that afternoon. He tries to make it to each of his patients’ funerals, he told me. His wife had gone to the first one, but she cried so much (more than the patient’s mother, he remembered) that she hadn’t been able to go with him again. He also writes an obituary with colorful details about the person’s life and sends it out to the cystic fibrosis team via e-mail. That way, his colleagues all know when a patient has died and will remember a little something about the person.

  The funeral he was going to that day was for my thirty-seven-year-old patient. I had last seen him the morning after my shift, when he was no better but no worse, either, and I didn’t know that he had died. When Dr. Uluer told me, I wasn’t sure how to react at first. I had come to talk with him about advances in CF therapies, but there I was, remembering that room and thinking about endings. I asked how it had happened, and I learned that the young man’s breathing grew more labored, and ultimately, in the last choice he was able to make, he opted to die without a breathing tube.

  I wanted to know more about him. Dr. Uluer told me that he’d had a great, often crude, sense of humor. I didn’t learn this during our brief interaction in the middle of the night, when he hadn’t shown me his personality and I hadn’t asked him to. But he’d been hospitalized so many times that everyone on the cystic fibrosis team knew him and genuinely enjoyed coming to see him on rounds. Even when his body was failing and he was sick and angry that he was dying, he was still himself, cracking jokes in his hospital room. The Godfather was his favorite movie, and he held court in the ICU like Don Corleone himself, motioning each visitor to sit by his side. He set up an espresso machine he’d brought into each of his hospital rooms over the years, and on what would become the last morning of his life, he had savored a sip.

  I could tell that Dr. Uluer would miss this patient. He misses them all. And even though our conversation did ultimately turn to advances in CF treatment and to the small-molecule therapies that might one day transform this disease, the tone had changed. Yes, there are more adults than children living with this disease, and one day there will be geriatric cystic fibrosis clinics, and the letters CF will stand for “cure found.” The energy is palpable, and it is exciting. But the reality of that day was about something different. On that day, Dr. Uluer was going to a funeral for a thirty-seven-year-old man. And as we talked, it occurred to me that all of this—the man who had died, Meghan and Myles, their hopes and expectations and disappointments—this is cystic fibrosis today. It’s discovery and game-changing new drugs, some of which have come and others that are just beyond our reach. It’s babies born with life expectancies that likely will extend during their own lifetimes, who will grow up healthier than anyone could have imagined decades ago. It’s throwing a party on the thirtieth birthday you were told you wouldn’t have and building a career and falling in love and buying a house before you turn thirty-five, but making sure that house has only one floor because there could—no, there will—be a day in the not-too-distant future when you are too sick to walk up the stairs. It is learning that you are healthy enough to carry a child but being scared to go through with it because you do not know if you are healthy enough to watch that child grow. It is a young man just about my age dying in the ICU, and a doctor preparing to go to his funeral. It is possibility and time, opening and extending longer than expected, but not long enough.

  9

  How It Begins

  In the world of the hospital, July 1 is all movement and nervous energy.

  It’s a beginning and an ending, as this is the day that medical students become doctors, interns become residents, and fellows start the first real jobs of their lives. On July 1 almost fourteen years ago, I walked hesitantly into an anatomy lab to meet a cadaver named Murray whose body I would disassemble and commit to memory. Fourteen years is long enough to have a child and to watch that child finish middle school. It’s long enough to get a job and buy a house and grow disenchanted and leave your job for something new. In that time, you can marry and divorce, start dating and marry again. But I did none of these things. I lived in two cities and six apartments. I finished medical school and officially became a doctor. I went on to train as a resident in internal medicine and then as a fellow in critical care. I stopped writing in order to devote myself wholly to memorization, and then, when I missed it too much, I started to write once again. I learned which supply closets on which hospital floors have the best crackers—Ritz, not saltines—and the freshest little tubs of peanut butter. I learned to go to work each day surrounded by sickness and death and to recognize the sadness, but not to let it paralyze me. I learned to diagnose and treat, to connect and communicate. And then it was over; one day I was a doctor in the midst of my infinite training, and the next I was an attending physician, that faraway goal finally a reality.

  The shift was almost imperceptible. I wasn’t changing hospitals. I had already started working as the physician-in-charge overnight in my hospital’s intensive care unit. I had to change my health insurance benefits, but I needed no new passwords or computer access to do so. I didn’t even get a new hospital identification card. And yet something real had happened. It had been a luxury to spend all these years just a step away from command, always a little bit the observer, enjoying the buzz of responsibility without its weight. Part of me wished that stage of my life could have lingered on indefinitely. But time moves, and I moved with it.

  Which is how, one afternoon in early July, I came to sit in the examining room of the transplant outpatient clinic. As a junior faculty member I’ve been assigned duties that take me out of the intensive care unit and to the margins, to the realms of after and before. I spend some weeks each year caring for ventilated patients at our affiliated long-term acute care hospital, and in my outpatient clinic I see people whose doctors have referred them for consideration of lung transplant. These outpatient visits are unique. We have the luxury of time. We can sit. I review the records. We talk. And based on a handful of absolute rules and a few more subjective ones, we determine whether a patient can proceed on the path toward becoming a candidate for transplant. I think of this visit as a key inflection point. Illness is real and present, yet I meet these men and women early, before they have to make the decisions that set them on a path toward uncertain outcomes.

  Often I have learned about decisions through hindsight, from what comes after. But this clinic is different. The after hasn’t yet come to pass. Whether a transplant ever becomes reality, this clinic marks the beginning—before the consent forms, the surgery, the prolonged recovery, before the possibility of tracheostomy tubes and long-term acute care hospitals, before the return home with prescriptions and pills and appointments and anxiety. This is how it all starts, in a clinic waiting room on an ordinary afternoon.

  It was a Wednesday. Six people sat in a semicircle in a small meeting room in the outpatient clinic space, watching an informational video about the transplant process. I pulled up a chair to the outside of their circle and sat, looking in. I would introduce myself as the doctor later, but at the moment I wanted just to be present. There were three pairs, each made up of a patient and a family member: two husbands and wives, one mother and daughter. The woman next to me sat in a wheelchair. Her oxygen tank hissed softly. Halfway through the video, th
e battery of her tank must have given off some warning, because she rummaged in her bag and pulled out the kind of cord you’d use on a computer. Her husband stood up and helped her unravel the cord, looking around for a wall socket. “There’s a plug over there,” one of the patients offered. “There’s one over here, too,” someone else said. All three patients might one day be on the same transplant list hoping to be the recipient of a scarce resource, but nonetheless, at least in those early moments, there was camaraderie in the room, a sense of shared destiny that linked these strangers as they peered into the future. The cord reached an outlet, and with the oxygen tank safely plugged into the wall, the group relaxed and turned back to the video.

  In each pair, the healthy one sat at attention, eyebrows furrowed with worry, pen in hand, scribbling in a notebook or on scrap paper. In transplant, the need for a loved one is actually codified—at my hospital’s lung transplant program, a support system of at least three people is required before you’ll be considered for an organ. I flashed to the future for a moment and saw how these roles could become calcified in the months and years that would follow. The husband or wife or daughter would be the one who clarified details for the doctors and recorded lab values and asked questions and kept folders and remembered appointments and picked up prescriptions and spent nights in the family room wrapped in that scratchy, too-thin hospital blanket, waiting. There would be so much waiting.