Stable With Trikafta

On the brink of being listed for lung transplant, I started Trikafta®, which has helped stabilize my health and helped me avoid it for now, but a transplant could be in my future.

| 6 min read
Amber Howell
Amber Howell
Amber smiling with her husband.

March 17, 2016 — my very first “tune up” ‐ I was 30 years old.

I was diagnosed with cystic fibrosis at birth in 1985. This was a time when there was still a lot of unknowns about CF and many of us didn't live to school age. I had meconium ileus, which is a typical sign of the disease. After I underwent an ileostomy, at 36 hours old, my mom received the news that I had tested positive for CF. I had quite a few hospitalizations as an infant — which included the reversal of my ileostomy at 9 months old. I had around the clock chest percussions, 20 minutes on each lung lobe while I laid across my mom’s lap in various positions on a pillow; nebulizer treatments; pancreatic enzymes

Even after all of that, if you were to ask me now, I believe I led a normal childhood despite my health issues. I managed to keep up with my peers and loved playing soccer and riding bikes. My earliest memory of a hospitalization was in 1992 for pneumonia when I was 7. Twenty‐three years sailing through CF unscathed until 2016.

So there I was, having my very first tune up, and my doctor mentions lung transplant. I had just given birth to my son two months prior — the timing could not have been worse. My doctor softened the blow by saying I was only to meet the transplant team to get established as a patient so when the time came, we would have all our ducks in a row. 

I had two more tune ups between 2016 and 2018, which showed my lung function and weight were decreasing, and it was becoming more difficult to recover after each exacerbation. I had a 2-year-old toddling around while I transported an IV pole administering antibiotics to and from my full-time job, and then came home to my son who needed my full attention. I was burning the candle at both ends and realized the flame was close to burning out.

On August 30, 2018, I pulled into the parking garage of Duke University for a five-day transplant evaluation. This was a series of appointments and tests to determine if I was in my “transplant window” sick enough to require a transplant but well enough to withstand the rigors of the process. I remember reading the summary following my visit: “Will need a transplant six months to two years.” 

Following the initial evaluation, I continued to see my regular care team in Gainesville — what used to be three-month intervals turned into six or even four weeks. I was being closely monitored and now on nighttime oxygen with a BiPAP. I no longer had a slim petite figure, but now looked malnourished and had a skeleton‐like appearance. I felt like each holiday would be the last one I would spend with my family. 

After every follow-up visit at Duke, it became more apparent that I was getting closer to opening that transplant window. I was maintaining, but barely. As each day passed it became more and more difficult to walk across a room without feeling winded. Laughing was no longer a joy but came along with anxiety as I was struggling to catch my breath afterward. 

I managed to keep things afloat for the next year or so until I needed a third sinus surgery. This time, I had a mucocele that was causing my left brow bone to erode and it was only a matter of time before my vision would be affected. Prior to this appointment, I had met with my CF care team who had brought up the possibility of Trikafta®. I had previously tried both Orkambi® and Symdeko® with hopes of promising results, only to experience little to no change. So when Trikafta was mentioned, I didn’t have much of a reaction. At this point, I didn’t have much faith. I knew that at any moment I was just one exacerbation away from being placed on the transplant list. 

Two days after my sinus surgery — February 13, 2020 — I took my first dose of Trikafta. In only a couple of days, I woke up and didn’t find myself coughing before rolling out of bed. No cough, no catching breath, no recovery. I took a deep breath. I felt my lungs fill with air, I exhaled; I didn’t wheeze, I didn't get light‐headed. 

I quickly began tasks that I had to give up before — simple things, like vacuuming and taking out the trash. It soon progressed to riding bikes with my son, racing him up and down our block, lifting weights at the gym, and participating in two 5k mud runs. Before I knew it, was 126 pounds and could no longer see the skeleton‐like frame I had become so familiar and comfortable with. I had finished that story and started a new book. 

Today, I’m winning. “T” used to stand for “transplant.” Right now, “T” stands for “Trikafta.” There is still the inevitable, though. I still have CF. Are there exacerbations and tune ups in my future? Yes. Is Transplant in my future? Yes. But if you were to ask me three years ago, I would have told you I wouldn’t be here. 

Interested in sharing your story? The CF Community Blog wants to hear from you.

This site contains general information about cystic fibrosis, as well as personal insight from the CF community. Opinions and experiences shared by members of our community, including but not limited to people with CF and their families, belong solely to the blog post author and do not represent those of the Cystic Fibrosis Foundation, unless explicitly stated. In addition, the site is not intended as a substitute for treatment advice from a medical professional. Consult your doctor before making any changes to your treatment.

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Amber Howell

Amber is 37 years old and is mom to 6-year-old Logan. She works full-time for the Lions Eye Institute for Transplant and Research overseeing their tissue donation department. When she is not working, she enjoys exercising and spending time with her son, family, and her significant other, Adam.

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