In March 2020, while a global pandemic was hitting the world, I nervously took my first dose of Trikafta^®. I had read about this breakthrough medication for the most common mutation of cystic fibrosis (F508del) — my mutation — the year prior, but I was still hesitant to try it. I read testimonies from fellow people with CF on how it radically changed their lives for the better, but I had heard about negative side effects as well. 

Being the skeptic that I am, I was wary of taking any new drugs, especially one this new. My lung function was 30% at the time, leading me to become progressively more paranoid that it would keep dropping until I’d eventually hear my doctor mention a double-lung transplant. I’m already a post-liver transplant patient and did not want to add more to that title. 

My CF doctor finally convinced me to try it, and with the support of my liver transplant doctor, I tried half a dose. Over the first few days, I was constantly coughing up hard chunks of mucus. From what I had heard from others on Trikafta, I knew this was part of the “purging” stage. I experienced some headaches as well. However, after about a week, the coughing and headaches subsided. I began sleeping through the night without coughing for the first time in years; I was waking up feeling rested after seven hours of sleep! I started putting on weight rapidly, which I admit was a bit unnerving, but that eventually plateaued. I could breathe deeply, run, laugh, and sleep without issues, and my lung function was climbing! It felt odd and like my life just got turned on its head. I felt amazing and free. It was a new high. A relief. 

I was no longer hyper-focused on my health and anxiously awaiting each lung function test. My days are no longer consumed by breathing treatments and airway clearance. I no longer fight so hard to stay alive. Suddenly, I had no idea what to do with myself. What do I fill my time with? My mental and physical energy didn’t know where to go. I had been so used to having to sacrifice my time for my health, or my health for my time. Now, I could have both at once, which left me with space. Space I had never really felt. Uncomfortable space. 

I began to realize how much cystic fibrosis had defined my life, even when I told myself it didn't.

Growing up, I often felt I was on the outside of “normal” life. I was living through an alternate reality compared to others my age. I was forced to undergo life-saving surgeries, procedures, and therapies. None of it was a truly personal choice because I was too young. I felt alone in my experience. Kids at school seemed to whisper about me when I returned from extensive absences. I could tell they viewed me as the “sick girl” suffering from some mysterious disease, even though I looked fine. Not understanding, not knowing. The image of myself that the world seemed to mirror back at me was that of a fragile, reserved, quiet, strange, unknown girl — someone to pity or ignore. 

I think it’s an easy and comforting idea to believe something as big as a cystic fibrosis diagnosis doesn’t make up who you are. However, if you were raised with it being at the forefront of your life experience, like I was, then I think that notion is unrealistic. CF helped set the stage for my life at the very young age of 3. I barely had a life before it. CF became my identity over time because it had to be. It was the fire that forged me — the part that encompassed my life, my mental and physical focus, and my emotional experience. It was the grounds for how I perceive life, empathize with people, and believe in a deeper meaning for everything. You can’t be born with a genetic illness and think of it as random and meaningless. At least I can’t. 

I had long fantasized about my life without ever factoring in CF. I imagined all the things I would want to do and accomplish. But when I attempted to do those things, my body would always get in the way and say, “no.” Lung exacerbation after lung exacerbation, sinus infections, kidney stones, a broken foot, liver rejection, you name it, something always came up. I became an expert at riding out the unexpected waves that hit me over the years. The downside is that I also gained a fear of commitment and following through on things I wanted. 

I was in a constant uphill battle while blaming myself for not doing more, for not achieving “normal people things,” and sowing doubt in my capabilities. I was like Sisyphus, pushing that boulder up a hill only for it to tumble back down at me. And now, Trikafta has finally given me a well-deserved break. With Trikafta, while I am certainly not cured, the unwelcome health challenges that once felt like sudden tidal waves knocking me off my feet now feel like small waves coming up to splash me occasionally. But I remain standing. 

The pressure I had to stay as healthy as possible has diminished now that it’s not all on my shoulders, but a new pressure has arisen — a need to make my life into something separate from CF, something more. Like what I always wanted as a kid. But that fear of committing and following through? That is my new challenge. I’m attempting to learn how to override this trauma response that has been built into me after countless disappointments. A secret pessimism that even though I can envision the life I want — and tell myself that it’s possible — deep down in my body I doubt I could ever achieve anything. There’s still a voice within me saying, “Slow down, don’t push yourself, don’t strive too hard, you’re going to sabotage your health, it’s too risky!” Sometimes I wonder if I only survived by coasting. I never even pictured myself aging and growing old. Now I’m faced with learning how to make long-term plans when I had only ever focused on making the most out of my life in the present. 

If CF isn’t my battle anymore, isn’t my job, isn’t my constant focus, and motivator… then what is? What do I do? Who do I become? Who do I want to be? Who can I be?

I once thought I had sure answers to these questions. But now that I’m faced with the possibility of those answers becoming tangible, I suddenly don’t know anymore. My life feels open-ended, my energy scattered, my nervous system a wreck. 

How do you let go of a struggle that’s been built into you? That’s helped define you? What happens when the thing holding you loosens its grip, but you’re unsure how to loosen yours? 

I told myself cystic fibrosis was not my identity and did not define me. I now realize this thought was diminishing the impact it's had on me. It’s only when something changes or is removed that we realize how stuck we were with it.

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