At the age of 15, I learned that it was unlikely I would live to 30. I read a school newspaper describing the decline of a young teacher with cystic fibrosis, and from that day onward, my own life expectancy was rarely far from my mind. Like a specter looming in the shadows, it influenced my choices around education, relationships, and retirement. While advances in CF care kept me alive beyond 30, my health was still clearly declining. Just before starting Trikafta, I had initial conversations with my doctors about the lung transplant process. My hope for the new “miracle” drug was so small, it sat on the floor of my room for two weeks because I wanted to finish my supply of Orkambi before starting the new medication.
On January 1, 2020, I took my first dose. Within 12 hours, I felt a little stronger and my breathing felt different as I jogged at the gym. I assumed I was just having a “good day” and didn’t consider that Trikafta was the reason. The next evening, I felt even stronger and jogged an additional minute. It hit me as I was riding home from the gym. Maybe it was the medication? Maybe it was making a difference?
Unlike many people who started Trikafta, I did not have the “purge.” I coughed more for the next few days and then slowly began to cough less. It was not until I was transcribing a research interview and heard someone constantly coughing that it hit me. I was the one on the interview coughing and now, I almost never coughed. I was amazed, holding back the tears that formed in my eyes.
Soon, the weight gain began. One morning before work, I couldn’t get my pants buttoned. I assume they had shrunk in the laundry and so I grabbed another pair. To my amazement, they didn’t fit either. “How did I get so fat?” I mused as I looked at my body, which for the first time, looked like a healthy weight. At first, I equated that weight with shame because I didn’t know how to process the change appropriately. My low expectations of Trikafta meant I never bothered to read about possible side effects.
So far, the changes I have described are physical. I could breathe more clearly, was far less tired at the end of the day, and quickly gained weight. The changes to the way I see the world and my place in it are much harder to explain and process.
Going from preparing to die at the age of 30 to possibly living to retirement changes everything about how I make decisions. When I try to explain this to people, especially those with CF who have not had the same transformative experience, it can appear as ingratitude. But I do not think it is a contradiction to say I am grateful … and confused at the same time. I have spent my whole life living like a person who was dying and now, I am trying to rewrite the basic code that has been embedded in the marrow of my decision making.
When trying to explain this to people, I tell them it is like training for a half marathon and just as you are getting to the finish line, you are told that it is now a full marathon. You would say, “I am not prepared for this. I have not trained for this. If I had known, I would have done things differently.”
I wrote a book entitled The Roses Speak, which collects stories from the CF community. Talking to other people with CF has taught me that these feelings are common for many members of the CF community across culture, religion, gender, geography, and race. For those taking Trikafta and having amazing results, it can be hard to move beyond the psychological lens of a shortened life.
It can also mean harboring survivor’s guilt for those who have died and those who are being left behind in the modulator revolution — such as those who have had a bad experience with them, those who live in places where they do not have access, and those who are part of the 10-15% who are not eligible for them. It is so important that each and every voice in the CF community is heard, each story is valued, and each circumstance is considered unique. The celebrations of Trikafta victories can’t drown out the cries for support from the margins.
As I reflect on my life, I sometimes wonder who I am and what I feel called to do with my bonus years. I have spent time working as a hospital chaplain, sitting with those who are dying. I am currently working as a high school religion teacher, inviting young people to ask bigger questions about the meaning of life.
I do not know why I have been gifted with more years in life, but I do know I want to spend the time I have helping others and encouraging people to continue to advocate for CF research. If I learned nothing else from writing The Roses Speak, it is that those of us with CF are all part of the same family. I celebrate the life that Trikafta has given me, a life I never expected. And I will celebrate just as much when every member of the CF community is able to know that feeling.
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