You Can Stop Humming Now

by Daniela Lamas

  The most important tip when it comes to getting an organ donor? That’s a lesson Eddie initially picked up while working in sales, and it has little to do with any of what I learned about organ donation back in medical school, years ago. “It’s about networking,” he told me earnestly, before he got up from our coffee to head to his doctor’s appointment. “You need to network, network, and network some more.”

  8

  An Unexpected Adulthood

  The teenager tapped her foot as she waited in the examining room. Meghan Kiley had been going to see her doctor every three months for as long as she could remember. When she was younger, the visits were actually enjoyable. Sure, he listened to the sounds of her lungs, pressed on her belly, and used a bright light to peer into her eyes and ears. But he also made her laugh, and he quizzed her on the history facts she’d learned in school and asked her questions about what she wanted to be when she grew up. The pediatrician told Meghan that she would be the boss of something someday, and he spoke about her future with such certainty that she believed him.

  Because it was her reality, and because her parents tried to shield her from their worry, she assumed that everyone her age went to the doctor as frequently as she did. She had to take pills with her meals, but so did her friend who was lactose intolerant. She didn’t feel fragile. She had no idea that her father stayed up during the night listening to her coughing jags, half worried that she would stop breathing. She played volleyball, took ballet lessons, went off to camp in the summer.

  By the time she turned fifteen, Meghan had long ago learned about cystic fibrosis, this invisible thing inside her that would mandate her daily attention for the rest of her life. She knew that her mother, who accompanied her to every single appointment, kept a binder full of her prescriptions and insurance paperwork—a responsibility that Meghan would one day have to take on for herself. She knew that in order to stay healthy, she would have to keep track of her meds and stay on top of her physical therapy, day after day and year after year. But in a moment of what she could only explain as an ill-conceived act of adolescent rebellion, Meghan had skipped the pills she was supposed to take with meals to replace her pancreatic enzymes.

  The pain in her stomach was excruciating; Meghan would not miss a pill again. But she still had to do penance. Her regular doctor was away, so she would see one of his colleagues to review what had happened.

  The door opened. Her pediatrician generally entered the room with a warm, easy smile and a ready joke no matter how busy his schedule. In contrast, this doctor’s air was serious, his manner businesslike and almost cold. He examined her—stethoscope on her chest, hands on her abdomen. She was used to that routine. Then, as the visit wound to a close, something unexpected happened. The doctor took out a piece of paper and a pen, and proceeded to draw a simple graph. Meghan watched, uncertain where this would lead. He labeled one axis of the graph as time and the other how he anticipated her lung function would decline. He showed her where she was on the graph that day at age fifteen. He then traced the line out fifteen more years, to Meghan’s thirtieth birthday. This, he said, was when her lungs would fail and she would likely “expire.” The doctor drew a dot there at thirty to mark her expiration date, and then he circled the dot. Her surprise quickly turned to fury. Meghan knew she had CF, but no one had ever talked to her about death before. Time, which had seemed infinite, shrank and collapsed into that dot. Meghan’s face reddened, her anger louder than her fear.

  That day, she promised that when she turned thirty, she would enjoy the sweetest victory her fifteen-year-old self could imagine—a huge party. She would invite all her doctors and nurses and friends, and she would show them that she had made it; she was still alive.

  Meghan Kiley was born one year before me, in 1980. Perhaps because we are around the same age, I find that as I re-create the story she told me about that years-ago appointment and fill in the details, I’m tempted to place my own fifteen-year-old self in her position. Yet as much as I try, it is so far from my reality that it’s almost foolish to imagine how I would have reacted. After all, I spent much of my adolescence studying, planning, and preparing for a future so distant that I couldn’t even make out its shape. That dot at age thirty was just about where I thought my life would truly begin—with money, security, maybe a family. Had someone told me that my genetics meant an early death, I think I still would have gone to college if I had been healthy enough. Though it’s impossible to know, really, I doubt I would have decided to spend years in a lecture hall postponing adulthood in the hope of becoming a doctor. But maybe I would have. Maybe I would have felt the exhilarating pace of medical progress and realized that when I was born, babies with cystic fibrosis weren’t expected to live through adolescence, but there I was—alive. Perhaps I would have been optimistic or foolish enough to bet on a future that I might or might not get to enjoy.

  Meghan knew she was going to college. There was no question. Though her father wanted her to go to school down the road so she could live at home, she craved the real campus experience. It felt important for her to get away, and it felt important for her not to mention her diagnosis anywhere in her application. But cystic fibrosis was there, too, traveling the forty minutes from her home to her Providence College dorm and lingering in the background of the college life she worked to build. A physical therapist showed up at her room five times a week without fail to clap on Meghan’s back while she held different positions, loosening the mucus that accumulated in her lungs. When friends asked whether the woman visiting Meghan so often was her mother, she nodded. A nervous mother’s frequent visits were easier to explain than illness.

  Time was a strange thing. Meghan wasn’t really sure how to think about approaching her twenties. If she were truly to believe the graph that doctor had created and the future it foretold, she was entering her final decade and time was short. But other than the infrequent hospital admission for pneumonia, and the intravenous lines and antibiotics that inevitably came with it, Meghan’s daily reality was one of a young woman at the start of her adulthood. Maybe it was because her parents had never treated her as though she would break, maybe it was because her pediatrician had always encouraged her to look toward the future, or maybe it was just because of her own stubborn personality, but Meghan moved forward. She didn’t see any other choice. She finished college, made plans for graduate school, and decided to move to New York, where she knew no one. She found roommates on Craigslist to share a place on Long Island, and with her family (and her doctors) no longer a short drive away, it felt as if she had traveled to a different world.

  And yet at the same time, Meghan found herself making subtle compromises. She strikes me as a planner—the type who would begin saving for a down payment on a house even in her twenties. But she didn’t save. Instead, she used her money to go on vacation. After all, she thought, what was the point of putting all this money aside for the future if she wouldn’t get to use it? She dated, but never anyone who was good to her and serious about a relationship. She had always loved being around children. But given her conviction that she wouldn’t live long enough to have her own family, she figured she would find ways to satiate her maternal impulse by working in college education and helping other people’s children. Still, the years passed, one into another. And fifteen years after she sat in that clinic watching a doctor draw a graph, she was planning a thirtieth birthday party.

  It was as she’d imagined, outdoors on a sparkling summer day under a huge tent at her family home on Cape Cod. Meghan’s mother had died of breast cancer a few years before, but her father was there, and her family and friends and even some of the doctors and nurses who’d taken care of her. There was a DJ, a dance floor, catered food, a hot-pink-and-lime-green color theme. After dinner, a party bus came to transport the group to a nearby bar, where they continued to dance into the early hours. To Meghan, who assumed she would never have the chance to get married, this party felt like her equivalent of a wedding. It was
perfect. But when she woke up the next day and the day after that still feeling healthy, she slowly felt herself facing a strange and surprising question: “Now what?”

  How long would this reprieve last? She surely could have become paralyzed by that uncertainty. One woman with cystic fibrosis, approaching her fortieth birthday, explained that she had framed her life around the belief that she would die in her teens. After all, that was the expectation for those with the disease when she’d been born. Growing up to focus on the “now,” she never considered what an adulthood might actually look like. She never pursued a job. She surely never imagined aging, or outliving her grandparents. With new drugs and advances in infection control, though, she was doing just this, and remarkably well. But she was lost.

  A man in his early thirties started a new drug that targets one of the genetic defects responsible for the symptoms of cystic fibrosis. He was lucky. He had the right mutation, and the drug was working for him. He married, even had children. But every day he took that pill he feared it was the day it would stop working. It was as though he were waiting for the moment when his doctor’s prophecies from decades ago came true and he would die.

  Another young woman described a turning point when she decided she should start wearing sunscreen. She was still unsure about how much she could plan for her future. But it looked like she just might be around long enough for accumulated sun damage to matter. When your life expectancy continues to shift within your lifetime, it’s hard to know what to envision. You can’t help but shuttle between anxiety and fear on the one hand, and cautious optimism and hope on the other.

  It was a Monday evening. I had traveled to North Attleboro, a town south of Boston, to meet Meghan and her husband, Myles, in the home they shared with their Chihuahua. When Meghan answered the door I took in her fair skin and delicate features, brown hair in a neat shoulder-length cut. I never would have guessed her diagnosis. I’d arrived a few minutes before her physical therapist, so we would have time for a brief tour of the house that she and Myles had recently bought. The living room struck me as something out of a magazine, cozy but not cluttered, color coordinated and decorated with a chalkboard on the wall bearing the legend “There are only two options: Make progress or make excuses” in big block letters. The bedroom, bathroom, living area, and kitchen are all on one level, she noted, which was a selling point when they were looking to buy. One day, when walking has become hard, Meghan will still be able to make her way through her house without having to struggle up stairs.

  She doesn’t know when that day will come, but she works tirelessly to stave it off as long as possible. As a child, she only had to take a few pills each day. But with each passing decade the medical burden had grown, and it sometimes seemed as though her entire life had become structured around keeping sickness at bay. It was exhausting just to think about. This month, the regimen involved thirty minutes of an inhaled antibiotic in the morning and at night. (Never one to waste time, Meghan took up knitting to pass the sixty minutes of imposed stillness and recently made a hat.) During alternating months her doctors switch her to a different inhaled antibiotic three times a day—easier in some ways because each of the treatments is shorter, but harder because her midday dose leaves her scrambling to find a place at work to give herself the treatment without anyone watching through her office window.

  There were other meds over the years. Her doctors stopped a nebulizer with a high-salt solution when she started coughing up blood, and she traded the vibrating vest for exercise as a more effective method to help clear her lungs. Then there are pills, of course, pills in the morning and with snacks and meals. Also paperwork, insurance claims, reimbursements. She spends a few hours each week on the phone with the mail-order pharmacies that supply her meds, and it seems as though she’s constantly dealing with calls for prior authorization, trying to temper her frustration as she navigates the maze of the medical system. She remembers the binders full of diligent notes her mother kept during her childhood, and as hard as it is, she stays organized. Even so, it’s like having another job, she told me. “In the weirdest way, it’s a normal life because it’s normal for me,” she explained as I struggled to write it all down. “But no one else in my life other than my husband has any idea just how much work it is.”

  Meghan brought out a few large throw pillows from a closet and lay on her couch while Jen, the physical therapist who had arrived while we were talking, took her spot on the floor. At first it looked a little like a massage. Jen cupped her hands and slowly, rhythmically, clapped Meghan’s back. While she worked, the two women chatted and laughed with the easy familiarity of friends catching up over drinks. Myles headed down to the basement to watch some television in quiet, the dog following eagerly after him. The scene was homey and comfortable, and I felt myself start to relax into the couch. But when Jen finished liberating mucus from one lobe of Meghan’s lungs and she sat up, gave a deep cough, and spat sputum into a cup, the world shifted and it was a medical procedure once again.

  This intrusion of the highly medical into everyday life is unavoidable, and it is something that Meghan knows will increase over time. She tried to keep that part of her life from Myles when they first started dating, on one occasion even concealing an intravenous catheter under her shirt and leaving his house at 2 a.m. to dose her IV antibiotics while driving back to her home. She had finally worked up the nerve to tell him about her cystic fibrosis over drinks at a bar, and when she broke the news he shrugged, or at least that’s how Meghan remembers it. Myles didn’t know much about cystic fibrosis back then, he told me, and so he wasn’t quite sure how to react. But he would learn.

  When Myles started talking about the future, some months after those first dates, Meghan told him to “pump the brakes.” Before she could begin thinking of any kind of commitment, she wanted him to see everything. She needed him to know that CF was more than a box of pills and nebulizers and physical therapy, which meant that he would need to see her in the hospital. The winter after they first met, Meghan got sick and was admitted, as she is most winters, for IV meds and inhaled treatments. She is usually assigned to one of the highest floors of the hospital, where the walls are wood paneled, the rooms large, and the nurses familiar. I visited during an admission like that. Meghan was receiving intravenous antibiotics, and I had to wear a yellow contact isolation gown and gloves to enter. But she was dressed in an oversized Ruth Bader Ginsburg–themed “Notorious RBG” T-shirt and leggings, bickering comfortably with her father about why he had thought she would want a tray of cupcakes in the room. (For her visitors, he replied.)

  During the admission the winter after she met Myles, though, for some reason she ended up being sent to a room on a lower floor. The nurse referred to her as a “CFer,” a term that has always rubbed her the wrong way. Perhaps slightly anxious about the stakes involved in introducing Myles to the realities of cystic fibrosis, Meghan decided she would defuse the stress of the situation and have some—admittedly goofy—fun. When he sat at the side of her bed, she shrieked in mock horror, pretending he’d pulled out her IV. On another occasion, she hid in the bathroom and surprised him. He recovered nicely after a moment’s panic. And he stayed.

  Myles had fallen hard for Meghan’s smile, infectious enthusiasm, and intense energy. By the time he proposed, cystic fibrosis had become a part of his life. He understood the serious nature of his wife’s diagnosis, he told me. In the back of his mind, he was conscious of the possibility that she could get sick at any time. Myles, a middle school phys ed teacher, has an air of unflappable, perpetual calm (in fact, one of Meghan’s doctors described him as “her rock”), but he admitted to me that he probably seems far more laid-back about the disease than he actually is.

  Meghan’s most recent hospital stay had scared him. It had scared her, too. She had gotten sick—really sick, a fever to 105, unable to get out of bed—due to an abscess, a walled-off infection, in her lung. Doctors gave her one antibiotic after another, each name and
potential side effect profile longer than the last. Usually, a few days into an admission, Meghan feels good enough to have friends come by, order in pizza, and act as if the hospital room is a spa. But this time was different. Her kidney function worsened. She needed supplemental oxygen. She could barely walk. She only allowed members of her immediate family to visit.

  When Meghan finally made it home, she was far weaker and thinner than when she’d left, and she was shaken by how sick she’d become. Though she had been out of the hospital for a few months by the time we met, she still hadn’t rid herself of the feeling that she was “living on a razor’s edge.” It seemed that something had shifted. There was no dot on a chart to tell her when, but she knew her lung function would decline. Her admissions would escalate in frequency and severity. This would all happen again and it could be worse, no matter how much she washed her hands and exercised, even if she never skipped a treatment. That was the future. One day she might have to think seriously about the possibility of a lung transplant. So Meghan had started to wonder what it would be like to cut down on work or even to retire completely. Work had been part of her identity since graduating from college, and I imagine that her job—as the associate director of student success and retention at Providence, where she’d gone to college—was a key aspect of not feeling defined by sickness. But the days on campus were long and tiring, packed with meetings and undergraduates who needed her attention. While these were all things Meghan loved, she wasn’t sure how long she would have enough energy to devote herself fully to work while staying on top of the increasingly arduous task of managing her disease.