A New Lease on Life: What Will You Do With It?
During CF Awareness Month, Emily’s Entourage invites members of the cystic fibrosis (CF) community to share their stories. Today’s blog post is by Drew Dotson, an adult with CF.
“My nephew started on Kalydeco,” my friend Andy told me, beaming. “He’s gaining weight and his PFTs are up. It’s incredible!”
“That makes me so happy,” I exclaimed.
This was a big win for the cystic fibrosis (CF) community.
I’d been fundraising for CF since childhood, and the introduction of Kalydeco in 2012 was tangible proof of progress. I wasn’t among the 4 to 5% of the CF population that could benefit from the medication, but I didn’t much care.
I was just grateful to know we were getting somewhere.
***
Growing up in the 90s, the CF community was tight-knit — maybe even too close at times. Each summer from 1996 to 2002, I attended a weeklong camp for people with CF. Given what we’ve since learned about infection risk*, it’s hard to fathom that I slept in a log cabin surrounded by other girls my age with CF.
We coughed in unison, swallowed enzymes in harmony, and dealt with our disease together.
Although some of my favorite childhood memories were born at Camp Wak-N-Hak (what a name!), they were later the source of significant heartache. I watched helplessly as my camp friends’ diseases outpaced medical advancements. I mourned their deaths as though I’d lost a piece of myself because, in a way, I had.
I struggled to comprehend why I was still here when they were gone. I shouldered tremendous survivor guilt as I carried on, uncertain why I’d experienced a different outcome.
But, at the same time, I knew how fortunate I was to be alive, and I channeled my gratitude into fundraising. No amount of money could bring my camp friends back, but maybe, just maybe, I could influence future outcomes.
***
After Kalydeco came Orkambi in 2015. And after Orkambi came Symdeko in 2018. And with each new drug, the CF population became further segmented. Either you were on a miracle drug or you weren’t.
I remember sitting next to my cousin, who also has CF, at a wedding reception as she swallowed her magic pills, and I longed for the day I could share in that joy with her.
It wasn’t that I wished it were me instead. I just wanted it for both of us.
Then came Trikafta, the drug that sent waves throughout the CF community. I’d been fortunate to take Trikafta during a clinical trial, but I had to discontinue it due to liver-related complications. I cherished those weeks when I breathed without consciously thinking about it. I regularly asked myself, “Is this what life should be like?” But as quickly as life had changed for the better when I started Trikafta, my life of labored breathing resumed when I was forced to come off of it.
I was overjoyed and heartbroken when Trikafta gained FDA approval in October 2019. With about 90% of the CF population on a miracle drug, the “haves” outnumbered the “have nots.” I watched as so much of the CF community thrived, and I couldn’t help but feel left behind.
Again, it wasn’t that I hoped to trade places with the “haves.” I just wanted it for all of us.
I observed as those who were taking Trikafta struggled to process their changing identities, wondering what to do with this newfound freedom to live. And I’ll admit — it was disheartening at times. Their transformations were living proof of the miracle of modern medicine.
Yet, at times it seemed like the remaining 10% of the CF population had been left to figure it out on their own.
***
Together with my pulmonologist, we attempted Trikafta again in 2021. This time, I took a gradual approach, which involved biweekly visits to the lab for bloodwork — accompanied by anxiety as I waited for the other shoe to drop. But it never dropped, and I’ve been on Trikafta for almost two years!
My cough has all but disappeared and my sinus issues are few and far between. My energy level has increased and I’ve reached a healthier weight. I still take pancreatic enzymes and follow the same respiratory therapy regimen, but those feel like minor inconveniences in an otherwise “normal” life.
Not a day goes by that I don’t reflect on how grateful I am to have lived long enough to experience this breakthrough. And not a day goes by without me remembering those who aren’t here for it — and the 10% of the CF population that’s still waiting.
Growing up, I couldn’t have imagined the success story I’d find myself in today. But I could just as easily be among the 10% whose lives remain unchanged — who still face the brutal realities of CF every moment of every day.
So, to the CF patients who, like me, have been granted a new lease on life: what do you plan to do with it?
Are you going to let your success story end with you?
Or will you use your platform to advocate until we can all share in the success?
It’s an easy choice for me.
Learn more about EE’s CF Crew, a group of people directly impacted by CF, who come together to raise funds and speed lifesaving breakthroughs for those in the final 10% of the CF community.
*Individuals with CF have bacteria in their lungs that can be spread only to other people with CF, which is referred to as “cross-infection.” These bacteria can be very dangerous for people with CF but do not pose a risk for people that don’t have CF. To reduce the risk of cross-infection, individuals with CF are advised to stay at least 6 feet away from others with CF.
Thank you to our tier-one Cystic Fibrosis Awareness Month sponsor
Author
Drew Dotson
Drew is an Atlanta-based author who uses her experience with cystic fibrosis, widowhood, and improv to inspire and uplift others. She writes regularly at www.drewdotson.com and is working on a memoir.