All of these things preceded that first exacerbation. It makes no sense to me to attribute the exacerbation to ataluren. I was going to need a hospitalization eventually, and with all of those issues that fall of 2011, not even ataluren could protect me. But I got much worse after I stopped taking ataluren that year. Once I stopped the drug I developed a pulmonary embolism, elevated liver enzymes, gallstones, a fungal cavitation, etc., and lost all the weight I had gained the previous year. I do not think I would have declined so much or had so many complications my sophomore year or after that had I been on ataluren.
I am aware of the placebo effect and the fact that regular doctor visits as part of a clinical trial can have a beneficial effect whether you’re on the study drug or not. But I have always had regular doctor visits, both before and after enrolling at Stanford. This is something my parents insisted on, and when I was treated at Cedars-Sinai, I was free to just “pop in” whenever I felt the slightest symptom. As the patient, the person who experienced both the benefit of the first 16 months and the harm that ensued, I believe with 100% certainty that the drug made a huge difference in my quality of life and I want that back. I’ve lost so much lung function in the past three years that my life is barely recognizable, and I see the possibilities in my future shrinking. I don’t think it’s fair to exclude a patient from a trial this life-changing because of one pulmonary exacerbation that was BOUND to happen with or without the drug.
On a more philosophical level, this flat-out rejection dashes my hopes of ever getting a lung transplant. I know that my case is complicated on paper. But every doctor has said my clinical presentation doesn’t match what they see in the chart. If trials and transplant teams and doctors never took on complicated patients, it would do a great disservice to the CF community.
Let’s talk about the specific yellow and red flags you mentioned.
1. Aminoglycoside usage—I would be happy to commit to not taking TOBI*10 in any form. I never really thought TOBI worked for me. Ataluren is much more important to me.
2. Stability—how long do I have to be out of the hospital to qualify? All CF patients have exacerbations, and the one I had in December was extremely minor. I was in the hospital only 5 days. I don’t see why this is grounds for exclusion.
3. Recent anticoagulation—one doctor called my pulmonary embolism “subclinical,” and said it was discovered only by accident through a CT scan. He thought it didn’t warrant any treatment at all. Another doctor did think it warranted anticoagulants, which I did for 3 months. The risk factor I had for a clotting disorder was birth control pills. I have since discontinued oral birth control…so there’s no reason why I should need anticoagulants again.
4. GI issues—doesn’t every CF patient have GI issues?
5. B. cepacia—I had B. cepacia when I started the trial last time and it wasn’t grounds for exclusion then.
6. Hemoptysis—same as answer for “B. cepacia.”
7. Fungal lung infection—are you referring to the fungal infection I had in 2011? Or current cultures for fungi? As far as I know, I have 7 colonies of fungus, which the clinic assured me are minor and don’t warrant treatment.
I hope you understand why I think being on ataluren would change the trajectory of my life. Please let me know what you think.
The answer was nonresponsive.
2/9/14
I’ve been working out every day and feeling pretty strong—and my lungs feel pretty good! Feels like a miracle.
I’ve started reading this book called How to Be Sick, a Buddhist-inspired guide to living well with chronic illness and being happy. Which sounds super cheesy and stupid, but I feel like the author shares a lot of similarities with me. A lot of what the author describes, I’m like yes, yes, yes and find myself wanting to underline it and highlight stuff. I think part of it is coming to accept your fate and accept that discomfort, disease, struggle, and hardship are part of life. To not fight against your situation or try to change it. To accept that what you have is not going to go away and to learn how to live well anyway.
Part of me bristles against this because it feels like it’s like giving up. The author explains it’s not giving up, but rather giving in to the possibility that your life will constantly evolve and change in ways you never predicted, and it’s important to be able to live happily with disease instead of waiting to be healthy before being satisfied and happy with life. I do agree with that.
Sometimes I’m at peace with my reality…but sometimes I feel as if there’s something inside me that’s shaking and wobbly and anxious and scared. Something I can’t describe that makes it feel like things are not right. But I’m hoping that as I read this book and try to incorporate that mentality into my life, I will feel at peace and truly grateful for what I have, and optimistic, not necessarily about the length of my life or my health status, but about what I can do even if my health isn’t great.
I want to live in a state of serenity and happiness that’s stable, not ephemeral and dependent on outside circumstances. That’s the goal. Hopefully How to Be Sick will help, and I think it will because I’m in chapter 2 and it’s already spurred this reflection.
The coed bathroom situation just doesn’t work for me, and it’s weird because it’s not like I’m into any of the guys in this dorm, so I don’t know why it matters, but this is just always an issue for me. Public bathrooms are fine but only when they’re single sex. My stomach seems to be the thing that puts me in the worst mood…lung problems just don’t.
Another stress—the pitch I have to write for my CF/environment podcast that Julie Snyder of This American Life will read is due at 6:00 p.m. tomorrow. And Jonah*11 is SO strict on deadlines, and I haven’t started it and I’m probably not going to get back until 4:30 and when I do get back I will have only slept like four to five hours probably. Ugh. But I’m not going to let it get to me. I’ll finish it and it will turn out fine. It isn’t every day you get the chance to get your work read by NPR. Stanford provides amazing opportunities!
4/1/14
Gotta love class assignments cuz they force me to remember and to write:
The sea turtle (the honu) is a sacred animal, an ancient Hawaiian symbol for longevity, safety, emotional strength, and wisdom. Hawaiian legend has it that the honu guided Polynesians to the islands, so many Hawaiians revere the honu as a guardian spirit, an aumakua, which protects them. They evolved long before dinosaurs went extinct and long before the Hawaiian Islands were even formed.
It was 2012 in Maui, my sophomore year of college. My lung function was 50 percent. For the past year, I’d been slammed with complication after complication, and I’d been in the hospital five times. It seemed like every organ system of my body had suffered abuse. I was in a really dark place, and for the first time, I was diagnosed with depression.
One day, in the middle of the trip, I woke up and everything felt heavy—my body, my head, my heart. Driving to the beach, I had a hard time looking at the sun—the day was too bright, the sky too blue. When I got there, I went straight for the ocean and dove in, trying to hold my breath as long as I could so I could stay there, because it was the only place where the pieces of myself came close to coming back together again. I was thinking about mortality and disease progression and acceleration. And I felt helpless and vulnerable and broken.
I started to cry underwater and the salty tears were mingling with the salt water of the ocean. And I was completely alone. But then I wasn’t. I had exhaled all of my breath, so I could sink down below the surface a bit and I was lying faceup, looking at the sky through the water, and all of a sudden, something appeared in my right field of vision. This honu, this green sea turtle, swam up, and it got really close to me and just sort of stopped and hung there and stayed close. And I was probably imagining this, but it felt like it was looking at me, not just looking at me but really seeing me. And I looked b
ack at this creature, with its brown shell and cracked skin and large dark eyes, and thought to myself that it had come along at that moment for a reason. I’m not a spiritual person, but I had an epiphany that that one sea turtle helped set me on the path to feeling whole again.
I still struggled for a long time after that, but I will never forget that moment, looking at that turtle and imagining that it was looking back with compassion. My soul felt endangered at the time by the threats to my body, and that turtle made me feel as if I did have the strength to ride through the turbulence of my disease with grace. Eventually, I had a resurgence of hope, and I realized that my future would hold a lot more happiness and promise than I allowed myself to believe.
My depression was a critical turning point in my battle with cystic fibrosis. I really thought my future would be one of continual decline and then death from respiratory failure or organ rejection of transplanted lungs.
Even though I descended into a deep depression for parts of my sophomore year of college, even though I live today in objectively worse health standing than ever with a lung function of about 40 percent, I’ve never felt more hopeful, more resilient, more empowered. I’m fighting for the future health of my body.
I didn’t ask for illness but I own it, because if I don’t, no one else will. And taking ownership has empowered me to believe that things can change.
4/4/14
I’m insanely worried about my friend Melissa. She has CF, took a deep dive, and went technology-MIA for weeks because she was so sick. She’s on 20 liters of O2. I’m on TWO. She finally got in touch and sent me a video tonight. She’s having severe anxiety and PTSD about being back in the hospital, understandably. I can’t even think about it without crying. She seemed so dejected in the video, maybe sedated, and so weary. Just about how I feel, but she has a better excuse.
5/10/14
When I was nine, I decided to stop doing my breathing treatments. They took too long. None of my friends had to do them. I was sick of being different when I felt and looked just like other kids. My mother tried pleading, bribing, punishing, and yelling, but without success. My dad came home early from work to talk to me. I avoided his gaze as we sat facing each other across the kitchen table.
“Mallory,” he said, “your mother and I do not ask you to do your treatments so you’ll feel better. We do not ask you to do them so you won’t get sick.” He paused. “We ask you to do them so you won’t die. If you don’t do your treatments, you will die.”
I burst into tears, leapt up from the table, and ran to my room, slamming the door behind me. I hated them. I hated that keeping myself alive was such an inconvenience, I hated that my dad forced me to face my own mortality, and I hated that they were right. For three days, I didn’t speak to my parents. But since that day, I’ve never refused treatment, because I know what’s at stake. It’s not a matter of preference or convenience; it’s a matter of life or death.
I asked my dad recently about that day. Did he have to resort to such extreme measures to get me to comply? “Sometimes,” he said, “tough love and brutal honesty are the only arrows left in our quiver.”
That conversation twelve years ago made me realize that we cannot possess true emotional maturity without a deep awareness of the stakes of our actions. At age nine, the consequences of my choices were too far away for me to consider; someone had to shove reality in my face for me to realize what I was risking.
Humanity is living like seven billion nine-year-olds. We don’t accept that what we do now will hurt us later. The disparity between my seriousness and others’ flippancy regarding the planet drives me to use my experience facing illness and mortality to force people’s eyes open, so they have no choice but to understand what’s at stake.
Ever since my parents threw me into the water at age three, the ocean has been my escape, my passion, and a powerful healing agent. I’ve always faced complications of cystic fibrosis, from malnutrition to frequent and aggressive pneumonias. For years, I’ve had the unshakable sense that being in nature (and specifically, the ocean) somehow heals me. Clears my lungs. Prolongs my life. Maintains my sanity. Restores my soul.
My intuition was right. While I was swimming, surfing, and fighting CF, researchers in Australia were discovering what I already knew: CF patients who frequent the ocean live longer—ten years longer.
The concepts of sustainability and human health are intertwined. The sustainability of the planet is the cornerstone of human health. Every person, whether they accept it or not, depends on this planet for survival: air, water, and food. But we have adopted a paradigm that separates humans from the rest of nature. Though we owe our existence to the planet, most of us are passive bystanders, watching idly as development and destruction demolish our temple. We assume Earth will keep on giving, no matter what.
My health fluctuates more than a healthy person’s, so I’m acutely aware how my health declines in sick environments. In a polluted city, I cough, wheeze, and breathe with pain. Ocean swimming, the most effective natural therapy, harms me when the water is dirty. Simultaneously learning about environmental destruction and coping with the rapid progression of my disease, I was struck by parallels between my own struggling body and the planet itself.
As CF progresses, mucus builds up in my lungs, harboring bacteria and inviting an immune response that erodes the gas-exchange tissue. I push my body to function, but my destiny is dictated by my DNA.
The planet is diseased, too, and like any other organism would, it’s crying out for help. The visible signs of environmental destruction are outnumbered by the invisible ones, just like the damage inflicted by cystic fibrosis. But while I can talk about my disease, garnering sympathy and support, the environment can’t.
Human sickness and environmental destruction are one and the same. The bacteria in my lungs are to me what the human species is to the planet. It’s just a matter of scale and perspective. My experiences make me want to shake people and scream, “This is what it feels like! If we don’t do something, this is what will happen.”
6/15/14
Today I graduated college (well…I walked). It was a beautiful day. Beautiful ceremony. For Wacky Walk, a Stanford tradition, I was a tree with Maya, while Gia and Danny were tree huggers. I thought I would be too exhausted to enjoy the festivities, but it was too big of a day with too much adrenaline not to. The ceremony was NOT boring; Bill and Melinda Gates gave a great speech.
The day started in the Stanford stadium with the entire class (before we split off for our department graduations). I feel lucky to be in such a beautiful place with amazing intellectual and social vitality. And to be healthy enough to be part of the celebration. It’s a miracle that I wasn’t hospitalized my entire last quarter at Stanford! I’m so incredibly grateful!
One thing that dampened the joy was news that a senior was found dead in his dorm room—they think by suicide. Xavier and I lived in the same dorm sophomore year—Toyon. It’s unfathomable to think about. Devastating. On graduation day. The worst part is that he went to Wacky Walk this morning and then back to his room to kill himself. His sister went to find him when he didn’t show up to his department ceremony. My heart breaks for him and his family.
6/27/14
Home! At first glance, I saw that everything was as it had always been. The olive tree in the front yard hung over neglected grass, making an obnoxious show of its vitality. The warmth of the house—in both color and temperature—enveloped me, setting off powerful pangs of nostalgia. Even the smell of coffee, extra dark roast, brought me back to adolescence, to early mornings and big breakfasts before school.
In my room, I sat on the bed. Here, things were different—I couldn’t decide if my quarters, with their newly white walls, white dresser, and dark wood floors, looked elegant or sterile. Part of me missed the messy oranges, pinks, and greens of my childhood bedroom. One photo of me with fri
ends, propped up on the dresser in a cheap, plastic frame, had survived the great “clutter purge” that my mom had undertaken. It was from an old disposable camera, the kind no one used anymore. It was grainy, but the happiness on the faces of my best friends was unmistakable.
Other things were different, too, I realized. No white-coated lanky Labrador had bounded up to the window by the front door to see my face before greeting me. Since Dewey died, the house felt a little bit emptier. The fridge was emptier, too, the pantry barren in support of my dad’s low-fat, low-calorie, low-sodium diet. My mom offered to whip up a protein smoothie and a chicken stir-fry for me in support of the high-fat, high-calorie, high-sodium diet my doctors wanted. I declined, feeling more overwhelmed than underfed.
At the entrance to my room, I stood and looked down the long hallway to the front door. Our house has a terribly closed-off floor plan, not good for the entertaining my mom so loved to do; but she always made it work anyway. I loved this house that I’d grown up in. But on this day, the day I came home to L.A. after graduating, that narrow hallway was a stifling physical reminder of my narrowing prospects. I shouldn’t have been home—not to stay, at least. My pride, nostalgia, ambition, confusion, fear, and fatigue all mingled together as if gurgling in a cauldron.
Salt in My Soul Page 10