“I want to be able to travel, to see something other than this bubble that I’ve grown up in. I want my career to take me into sustainable development of third-world countries. I want to do research in the rain forests of Brazil, to work on an organic coffee farm in Nicaragua, to scuba dive in Australia. I want to be able to hike to the tops of really tall mountains and see what the view’s like up there, and to know what it feels like to finish a triathlon, and to be able to spend some time helping the world instead of letting the world help me.”
I know he related to those desires, and it was hard for him to deliver what he said next. “Because of your hemoptysis, it’s not safe for you to be far from a major medical center for the next few years. But after that, I don’t see why you shouldn’t be able to do some of these things! Proximity to electricity may be a limiting factor, but I don’t think your lung function has to be. I know CF patients out there doing the most amazing things, things they weren’t always sure would be possible. Don’t give up on those dreams.”
Even as he said those words, I knew to take them with a grain of salt. His job is to inspire confidence, and making the impossible seem attainable is a great way to motivate patients to stay happy and continue working hard. His words didn’t necessarily convince me that I could live elbow-deep in the soils of Central America for the rest of my life, but they showed that he cared about my future and was going to work with me to make it hold as much promise as possible.
* * *
—
On the fifth morning of that hospitalization, I woke up feeling much better and made up my mind that I would take a significant walk. Outside, the air was crisp and clear, typical of a beautiful Northern California fall day. As my mom and I walked out the door of the front entrance, the sky was clear and the sun hurt my eyes. I squinted and looked down. At the fountain in front of the hospital, ducks paddled around in an incomprehensible social dance. I dipped one foot in the chilly water, wondering whether it ever got cleaned, and then chose to ignore this concern. My second foot followed the first and I sat down on the ledge, ankle-deep in the fountain, goosebumps running up and down my legs. Craning around to look behind me, I saw my mom point at a girl who was walking a ways away with her dad.
“That girl is in the room next door to you. I’ve spoken to her dad in the hallway. Her name’s Kari. She’s not doing well. She has B. cepacia.”
C3, the unit I was staying in, is often called the CF ghetto because it gets a steady flow of us. Sometimes, in the winter months, we flood C3 in waves, but even in summer there are always at least a few of us. We’re hard to miss, especially if you know what to look for: skinny arms and legs that seem too small for the rest of the body, rattling cough, white mesh sleeve covering a PICC line, four daily visits from RTs, an IV pole with many lines coming from it. Even though we recognize each other, there isn’t a protocol for the first approach. Patient confidentiality trumps the need for social connection.
I watched Kari and her dad as they walked along the perimeter of the hospital, focusing on the eerily skinny shadow cast by her legs. When I’m well enough my mom and I sneak off the hospital grounds to go for longer walks to the Stanford Shopping Center, and I suspected that’s what they were doing. I found myself curious: What was her situation? How sick was she? Was she able to go to college? Was she happy?
The next morning, I learned that Kari’s dad and my mom had seen each other in the hallway and had bonded over our shared life experience. They arranged for us to meet, so the next day we met at the fountain.
One of the great tragedies of CF is that in addition to isolating patients from healthy peers, it also isolates patients from each other. Cross-infection, or transmission of a bacterial infection from one patient to another, is one of the most serious concerns in the CF community. Physical interaction with other patients can be extremely dangerous, so it’s highly discouraged. Patients are segregated from each other as a matter of course; we are not allowed to touch, stand within fifteen feet of each other, or be in each other’s vicinity without a mask on. For those of us with B. cepacia—only about 3 percent of patients—patient contact is expressly forbidden; in the small print at the bottom of any invitation, website, or brochure regarding any CF event read the words, “No patient with a positive culture for B. cepacia complex may attend.”
I’d never actually met anyone with CF in person before coming to Stanford and was happy to meet up with Kari the next day as she walked out of the hospital holding her dad for support. We exchanged small talk for a few minutes, both struggling to know what to say before talking about the things we so desperately wanted to discuss.
“Where are you from?” I asked.
“I live in Oregon but I’ve been coming to Stanford for the CF clinic since I had my liver transplant at fourteen,” she began. She spoke more quietly than I did and was much smaller; even though she’s two years older, she looks at least five years younger than me. “I used to go to OSU, but now I take classes online so I can live at home. My brother’s just starting there as a freshman. We’re both so excited…him to start college, and me because I get to retain my link to OSU.”
I told her that I go to Stanford, that I’m a junior, that I live in and staff a freshman dorm. Then I didn’t know how to proceed. I had been so excited to meet her but when the moment came, my true questions evaporated and my brain emptied. We were silent for a bit, but I wasn’t uncomfortable, and she didn’t appear to be, either. She struck me as stoic and thoughtful. A strong fighter, but very much at peace. Radiant smile lighting up a beautiful face atop a frail body. Finally, we started talking about what was going on with her health in this current hospitalization.
“This week has been challenging,” she admitted. “Because my lung function is so low now, I was evaluated for a double-lung transplant. But I was denied.”
My heart sank. When CF patients get to a certain point in the progression of the disease, the last remaining option for saving their lives is a double-lung transplant. New lungs often mean a second chance at life for people who would otherwise soon die. Any patient near end-stage disease hopes they will be strong enough to be eligible for transplant.
“Because of my B. cepacia, my previous liver transplant, and my weight, they said I wouldn’t survive the transplant,” she announced evenly. “So they won’t do it.”
“But…but…is that it?” I sputtered. “They won’t change their minds? What’s going to happen?”
“The doctor told me he wished he could give me some hope, but there was nothing they could do. ‘We’re going to help you live your life as long as these lungs will allow, but eventually you will be looking at hospice,’ he said to me. ‘Is that a term you’re familiar with?’ It was so abrupt. I was reactionless.” My eyes were fixed on her as she told me this, but she was looking down at the water. She paused for a moment, opened her mouth to continue, but then hesitated.
“Are…are you okay?” I asked. It wasn’t the right thing to say, but nothing was. I wanted to give her a hug so badly, not just for her but for myself as well.
She looked up and this time looked me right in the eye. “All I could think about was how my best friend asked me recently what I was most looking forward to after my transplant. I said I was so excited to be able to laugh again. I can’t now; my lungs are too weak and it hurts. I was really looking forward to that, to getting my laugh back.”
My body stiffened. I was heartbroken for her and stunned that she was so calm after hearing the worst piece of news a CF patient could hear. But I didn’t want to pry.
“When did this happen?” I finally allowed.
“Yesterday.”
We both fell silent again for a while. I curled my toes in the water and tried to focus on how the sun felt on the nape of my neck, hoping to shut out the questions I was dying to ask.
“Don’t you just wish sometimes your life could be like a Ta
ylor Swift song?” she asked, smiling a little bitterly. I nodded in grim agreement.
A few days later, I was discharged from the hospital and moved back into my dorm room. Kari had to stay a while longer.
As I got to know Kari, I learned more about her situation. The way I see it, all CF patients live on a continuum somewhere between the health of birth and the impending death from end-stage disease. How fast one moves along that continuum differs from person to person, and how much control we have over that is debated. Kari’s a bit farther along than I am, and so is another CF-er I’ve met, Caleigh.
A year ago, during an inpatient stay at Stanford Hospital, a rattling cough echoing through the wall between my room and the one next door signaled to me that the patient was one of my people. On daily walks through the halls, I slowed down in front of her doorway to peek inside, wanting to put a face to the cough. When she walked by my room, I saw her do the same.
On my fourth day in-house, during CPT, I asked my RT if the girl next door had CF. He said yes but would not tell me her name. “I’d get fired for breaking every patient privacy policy in existence.” But an hour after he left my room, he came back holding a slip of paper. “Don’t tell anyone,” he whispered nervously, “but she wanted to meet you, too.” An untidy scrawl on the paper read: “Caleigh Haber. Add me on Facebook. I’d love to talk!” Five minutes later, we were chatting online. Like prison inmates who communicate by knocking on connecting walls, we spent our days twenty feet apart, getting acquainted through the computer without hearing each other’s voices.
About a week later, my dad and I were roaming the halls to get exercise. Rounding a corner, I saw a little girl walking ahead with her back to me. She looked no more than eighty pounds and pulled an IV pole alongside her. A small dog walked beside her and there was a woman, too. We kept our distance, not wanting to intrude. When they reached the end of the hall, they turned around and started to walk back, and that’s when I realized that this wasn’t a child. It was my neighbor.
“Caleigh!” I called out, loud enough for her to hear but quiet enough not to disturb patients nearby. “It’s Mallory!” We sat down in the hall, separated by masks and fifteen feet of space to avoid cross-infection, and finally talked face-to-face. She was very frank. She told me how her life had diverged completely from the lives of her friends and how, in her opinion, peer alienation was the worst part of CF.
“It’s a constant struggle to get out of bed in the morning,” she told me. “To eat the calories I need to gain weight despite constant stomach pain, to spend four or more hours a day doing my breathing treatments, to fight fatigue, to sterilize, cook, clean, exercise, and sleep, but most of all, to be in such close proximity to the life I want but can never have.”
Throughout our conversation, we marveled at the parallels between our lives. Caleigh was twenty-one. I was nineteen. Caleigh had been a cheerleader in high school. I played volleyball and swam. We were both from Southern California and relocated to NorCal for college, both had been active and outgoing in high school, both had enjoyed relatively stable lung function throughout high school, which had allowed us to feel somewhat normal when we weren’t in the hospital. At the time of our meeting, I was a sophomore in college; she had just finished culinary school and was working as a chef.
In her four years since high school, Caleigh’s lung function had declined from 70 percent to less than 30 percent. In my two years since high school, my lung function had declined from 70 percent to 50 percent. Her story made my heart sink; although it was comforting to see the parallels between our lives and to be able to talk about them, it was also a reminder of how much scarier my disease could become and how quickly that could happen. We had been pursuing our dreams with the same tenacity, suffering similar accelerating disease progression as a result.
Caleigh’s story of rapid lung function decline seemed like a manifest warning, a road sign reminding me to slow down to avoid careening off a cliff. Two years ago, Caleigh was at that fateful curve; now, in free fall, her lung function was at 18 percent, and her lungs had been colonized by MRSA (methicillin-resistant Staphylococcus aureus, a species of bacteria almost as virulent as B. cepacia). Because of her low body weight, she, too, had been denied a transplant. I’m not sure she had the benefit of warning signs, the signs in my life that were Kari and Caleigh. Their stories forced me to question the way I live.
Kari, Caleigh, and I have a deep unspoken connection, forged in the halls and courtyards of Stanford Hospital. After meeting both of them, I connected them online; they now provide each other with a lot of support. We’re an unusual “trio,” the two of them so small that a slight breeze could knock them over, and me standing at six feet. Kari has an unwavering faith in God and radiates compassion with quiet courage and resilience. Caleigh has boundless energy kept in check only by her diminished lung function. Caleigh’s spirited irreverence fuels a fearless commitment to surpassing expectations. And then there’s me, the one who never actually looks sick. A few years behind the other two in progression and age, closer to Caleigh in beliefs but to Kari in disposition, seemingly so lucky by comparison, even though their reality will ultimately be mine. I’m at a loss for how to comfort them.
Their lives leave me with profound questions about my own future. If we’re all straddling that line between the real world and the war world of chronic illness, they spend much more time on the battlefront and get to feel normalcy much less frequently. Maybe where I am is actually the training camp, and they’ve already been deployed. When I think about how many years they have left, it’s hard not to think of them as future versions of myself, and fear that my prognosis will soon be as bleak as theirs. With a deep breath, I remind myself that they are not actually warning messengers. Each patient has her own progression. My disease has proven itself to be a cunning and unpredictable enemy, attacking from the inside like a Trojan horse. But even as this battle rages, even as every decision becomes more significant and every complication more ominous, the real world continues, providing one day after the next, each good day unfolding as a fleeting, beautiful gift.
I have the feeling that there’s nothing after this, that this life is all we’re ever going to get, and it’s fragile and brief and easier to throw away than we think. This understanding drives the way I carry myself from the time I open my eyes in the morning to when I close them in sleep. When I lie in bed at night, I think about mortality, about what I want. I hope that what I want is realistic…but fear that it’s not.
A few weeks ago, I went back to C3 to stop by Caleigh’s room. My few weeks out of the hospital had been a refreshing break, but I felt a strange sense of relief and belonging when I walked back into clinic and up to C3. I’d spoken to Caleigh many times in the past couple of months, since she’d been in the hospital for a very long time.
I’d gone to the market and picked up chocolate, cheese, crackers, berries, and magazines, all the while wearing gloves so I wouldn’t touch any of it. I asked her nurse to give Caleigh my care package: Caleigh was delighted by the gift but she couldn’t eat any of it because she was having surgery the next day. I chatted with her for a few minutes from the doorway. She was less animated than usual. Her mom told me in private that a release date was nowhere in sight. Putting on my best smile, I filled her in on my life but kept it very brief; I know how hard it is to hear the details of others’ “normal lives” out in the real world when you’re entrenched as an inpatient. When I told her a funny story about my first volleyball practice, she laughed and immediately started coughing, cringing in pain; this made me think of Kari, and I wondered how she was doing.
It was time for me to go, but saying goodbye was hard. I waved and said, “See you soon,” then slowly walked down the long hallway. I was grateful to be able to leave, but my gratitude was tempered by the awareness that they could not. And I, with the freedom of a soaring bird—if not forever, then at least in that moment—e
scaped to the real world, while they, like birds with broken wings, lay bedridden.
2014
1/10/14
I’m still pissed that Stanford won’t let me go back on ataluren, so I sent this:
To the Research Team:
I started ataluren the summer before my freshman year of college, sometime between June and August 2010. In the spring of 2010, before the trial, I lost 13 pounds, was in the hospital for 4 weeks receiving 3 antibiotics and IV lipids, and suffered an episode of life-threatening sepsis. I continued IVs for a total of 12 weeks. Despite receiving 2,000 calories a day of IV lipids I could not gain weight, and after 12 weeks on IVs my lung function did not improve.
When I started taking ataluren, I thought the best I could hope for would be to stay stable. It didn’t cross my mind that my lung function would get better, as that’s not typically how my disease progression has worked in the past. Once my baseline drops, it tends to stay down and not rise back up. But within just a few months, I was gaining weight and my lung function was increasing despite living a less healthy lifestyle than I ever had in the past (exercising less, skipping more treatments, sleeping less, college drinking, etc.). Every other year of college since then, one or two skipped treatments, one night of bad sleep, or a few days without exercise could cause an exacerbation and land me in the hospital. But in that year and the summer following (when I was on ataluren), it seemed that nothing I did could really make me sick.
Yes, I do know that I got sick in the fall of my sophomore year, when I was still on ataluren. However, there were significant changes that occurred that I believe are responsible for that first exacerbation in 16 months. I became a vegetarian that summer, and being a vegetarian with other dietary restrictions and eating in a dining hall meant I started to lose weight. I lived in a triple room with roommates who did not understand how important cleanliness was to my well-being; the room was deemed by the school to be a bio-safety hazard because it was moldy and filled with germs. I spent three weeks before school started in an intensive arts program where I did not have adequate time to do treatment, sleep, or exercise. I had no help moving into my room, didn’t have time to unpack for weeks, and couldn’t find some of my medications. I only slept about five or six hours a night those first few months because of my increased academic load and class schedule. My mental state was not ideal because I was extremely stressed about school and the living/eating situation.
Salt in My Soul Page 9