31 Days of CF: Let the Long-term Planning Begin

Photo courtesy of Jenny Humphries

Day 9 of 31

This is Jenny Humphries’ story:

I’ve always been a planner. I don’t make a purchase without researching every possible option. If I’m going on a trip, you better believe I have a detailed itinerary with pictures and multiple backup plans. When I go out to dinner, I read the menu and choose my meal before I ever set foot in the restaurant.

Growing up, I understood the plan for my life: college, hopefully motherhood, and after that it got a bit nebulous. But it was generally understood that I had an early expiration date. It didn’t seem like a huge deal when I was young, but in my 20s, that “median life expectancy” started to loom large.

As any good planner would do, I began watching videos and reading stories about people getting lung transplants as I tried to wrap my brain around the next stage of my life.

Then, around 2011, I heard some rumblings of new medicines that were in development: CFTR modulators. These meds were rumored to change cystic fibrosis forever, and suddenly growing old became more of a reality than it had ever been before. Was I overwhelmingly excited, hopeful, and jubilant at this possibility? Absolutely! But I was shocked to find that I was also feeling scared and overwhelmed. This was not the plan! I have no 401(k)! Growing old was not on my life itinerary.

Turns out I wouldn’t start my first modulator until 2015, and that one didn’t actually do much for me. I was still getting sicker, and my lung function still declined. But in 2019, right after I started meeting with the lung transplant team, I started Trikafta, a treatment that combines three CFTR modulators.

Now, 2.5 years later, I’ve been dismissed from the transplant team, my lung function is up, I’m working for the first time in years, and most importantly, I feel good more days than I don’t.

A drug of this magnitude wasn’t in my plans. And it’s still a little scary to think about growing older. But you know what? Now I’ve got time to figure it out. Let the planning begin!

Cystic Fibrosis News Today’s 31 Days of CF campaign will publish one story per day for Cystic Fibrosis Awareness Month in May. Follow us on Facebook and Instagram for more stories like this, using the hashtag #31DaysofCF, or read the full series.