There has been a lot of progress in treating CF lately with the introduction of ivacaftor (Kalydeco®) and the combination therapy, ivacaftor and lumacaftor (Orkambi™). For some, these CFTR modulator drugs attack the root cause of CF, and the results have been amazing. I think this has led many in the CF community to count on a quick fix for their CF, focusing on their mutation and waiting for a blockbuster drug to give them good health.
Don't get me wrong -- knowing your CF mutation is important. But it doesn't have to be the sole focus of life. In fact, it absolutely should not be. If you're not one of the fortunate ones who benefit from the CFTR modulators available, you have to put the work in every day to maintain your health. In that way, CF is a marathon and not a sprint.
Much like training for a marathon, life with CF includes continual planning, work and discipline, coupled with twists, turns and opportunities to re-invent yourself.
I was diagnosed with CF in 1967 at a young age. At the time, not much was known about the disease. My doctors told my parents I wouldn't live past my 16th birthday. Based on that, my parents believed that the quality of my life was just as important as the quantity of years. We followed the medical protocol of that era, but my parents also wanted me to “be a kid,” which meant roughhousing with my three older brothers and playing on their sports teams. This combination of following my treatments, while doing what I loved, has served me well.
As I grew up, I realized that there was no quick fix that would result in good health. I followed daily goals to keep my body, mind and spirit healthy, including paying attention to nutrition and exercise and making smart choices in my work and social circles.
For me, being active and participating in sports have helped shape me into the person I am today: competitive, disciplined and determined. Some people may view exercise as a luxury, but, when you have CF, exercise isn't optional. When I started running, I couldn't jog even once around the track, but with dedication and training, I ran competitively in college. I have even run marathons as an adult. You don't need to run competitively, but I can't stress enough the importance of going outside and opening up your lungs. Compete with yourself to breathe!
It hasn't always been smooth sailing. Most days are wrought with obstacles, but I haven't let them deter me. Instead, I view them as catalysts to live life to the fullest. And yes, there are times when I struggle to stay medically compliant. I am human, and I can promise you that exhaustion and fears have visited me.
When I face challenges -- and, trust me, I've had plenty -- I approach them as if I took a spill while running a marathon. They're just temporary setbacks. I look for the solutions and adjust accordingly.
I would never turn down a cure for CF. But for many of us with CF, our reality isn't the sprint of a blockbuster drug. It's the marathon of relentlessly striving for the most awesome life we can live. The best is yet to come ...