Breathe in. Exhale. Repeat. I can’t focus on the music flowing through my earbuds, because my brain is occupied with obtaining every molecule of oxygen that is being provided to me through a tube. I inhale what is statistically measured as a paltry, small breath. But for me, it’s the deepest I’ve had in quite some time.
Sweat drips down my neck as I pedal on the stationary bike, my legs burning. I read the meter measuring my oxygenation level and feel a slight twinge of discouragement: I’ve been exercising consistently, but my oxygen needs have remained the same.
But then I take a moment to reflect on the past year, and I can’t help but feel grateful for the research advancements that have gotten me here — a new medication treats my disease in a way that no therapy ever has before. You may have seen the drug, Trikafta, in the news: It was recently approved by the Food and Drug Administration to treat the underlying cellular cause of cystic fibrosis (CF). It has been hailed as a “breakthrough therapy” by researchers, doctors and patients alike.
The day I was born, the median life expectancy of someone living with cystic fibrosis was 31. I was shielded from this knowledge until about 12, when I secretly read my mother’s diary. The words “inevitable” and “death” still stick in my memory.
Today, I’m 27. In scientific terms, I have a genetic, progressive lung disease that causes thick, sticky mucus to build up primarily in the lungs, but also in the digestive tract and sinuses. Over time, this mucus breeds infections from bacteria, which I then inhale into my lungs; the cycle of infections and inflammation leads to lung damage, then eventually respiratory failure, and death. Lung transplantation is an option to prolong the life of someone who has severe lung disease, but it comes with its own set of challenges.
Growing up, I did everything most other kids my age did — playing soccer and scootering. But I also did many things they didn’t. Every day before lunchtime, I would go to the nurse’s office and complete an inhaled breathing treatment, drink a weight gain supplement and take enzymes to digest my food properly. In the third grade, I had four stints of hospital stays, each two to three weeks long, during which I received intravenous antibiotics. In college, the infections became more severe; I had four lung collapses, each requiring a separate surgery to re-inflate the lung.
I graduated college with a degree in biology and was offered my dream job, but I didn’t take it. Spending hours a day on breathing treatments, attending frequent doctors’ appointments, taking antibiotics — not to mention maintaining a social life and community involvement — weren’t conducive to a full-time working schedule. Choosing not to advance in my career made me feel left behind, made my health the focus of my life. I made the most of it, though, pursuing flexible, part-time work as a technician in a university microbiology lab and developing my skills as a writer and a patient advocate — something I never would have done otherwise.
In some ways, I feel like I’ve one-upped this disease. Thanks in part to a daily routine consisting of four one-hour sessions of breathing treatments and airway clearance to move the mucus out of my lungs, someone meeting me for the first time probably wouldn’t recognize my condition.
But over the past few years, infections have become more frequent, so much so that I have required oral antibiotics almost constantly. What’s more, as my lung function has declined over the years, my physical limitations have increased. I won’t be able to run again with these lungs, having not been able to do so for nearly 10 years. Motherhood through carrying a child naturally won’t be a possibility. These days, taking a flight of stairs leaves me incredibly winded.
After becoming ill with a dangerous lung infection earlier this year, I’d been preparing for a double lung transplant. During the peak of my sickness, my body was a shell of an existence — at 18 percent lung capacity, supplemental oxygen was constantly filling my nostrils. But after trying an experimental therapy, my body started to clear that infection, and I knew it was time to begin the transplant process before another infection might tip the scale into lung failure — the lungs’ inability to provide me enough oxygen to sustain my life.
Then — after being granted compassionate use weeks before it was FDA-approved — I began taking Trikafta. It’s now the third approved drug to treat the most common genetic variation of the CF mutation, but this one boasts the best results and expands potential use to nearly 90 percent of the CF population — including me. The media has heightened the buzz around the drug, but it’s important to note that some patients still have no treatment options like this available to them. (Once a person receives a lung transplant, this treatment is no longer viable.)
My expectations of Trikafta, before starting it, were immense. But to friends and family, I maintained an air of nonchalance. It was an attempt to minimize my inner anxiety through outward display.
But once I shared the news that I’d been able to start the drug, the cheers and questions and elation from loved ones, fellow CF friends and acquaintances on social media came flooding in. Accordingly, I found myself over-inflating my response, trying to match my enthusiasm to theirs.
It’s not that I’m not excited about the drug’s possibilities, but I still feel the need to keep my expectations in check. It’s difficult to articulate the nuanced changes the medication has brought: My results have been gradual and moderate in comparison to other patients. Day to day, for example, mucus reduction has been slight, but over the course of a few weeks, I’ve found relief for a few more hours between breathing treatments. I am thin, but I’ve gained a few extra pounds. I stand just slightly straighter as my lungs aren’t heaving for each breath, which used to force my chest to collapse and my shoulders to roll forward. On the stationary bike, I still require using about the same amount of supplemental oxygen, but I can pedal just a few tenths of a mile further. The volume of my breaths has increased by 5 percent — as good as my highest lung function in the last three years, albeit a small overall change.
The bottom line is that my life will be prolonged to some extent by this medication. That may translate into one year or five before I will need a transplant, but any length of time is enough for me to be thankful to have lived long enough to benefit to some capacity.
I’m so grateful to all the researchers who dedicate their work to improving my life, and I plead that they continue searching for and fine-tuning treatments. I hope that for those in the future with the same defective gene as mine, their suffering is diminished — or perhaps nonexistent.
Many will continue to experience respiratory failure too early in life. I will probably be one of them. For some, this treatment has come too late in the course of their disease to make any difference at all.
For now, I’ll still struggle on that bike, needing every molecule of oxygen afforded to me, plus some. But I’m hopeful for further incremental positive change. These days, I usually skip music altogether when I’m riding the bike. Instead, I listen to the occasional clearer, fuller breaths filling my lungs.