When you read about CF in most places, after explaining how it affects the lungs, the next sentence is usually “There is no cure.” This is a tough reality for people with CF, since for our whole lives, it has been almost certain that our disease will not get better… it will only get worse. CF doesn’t go into remission, we cannot take a vacation from it, and its course cannot be predicted.
The only certainty about our futures is that they will be short.
When I was born, the median age of survival for a female with CF was 25. Soon, by the time I was 15, the age jumped up to 35. Now that I’m 25, life expectancy for people born in 1990 has been estimated to be around 40. With every year that goes by, people with CF are living longer and longer.
And yet.
These numbers never seemed big enough. Seeing a great difference in the life expectancy for CF compared to the time I was born is indeed progress. Each statistic means one more day that I get the opportunity to live. Each new drug will give us life. But a life expectancy of 40 meant I would never be a grandmother, I would never have a retirement, I would never sit on my doorstep in rocking chairs reflecting on a beautiful long life. It meant that every single day today is precious and fleeting, but the uncertainty scared me.
So when Vertex announced, back when I was in high school, that it was researching a drug that would treat the cause of CF… suddenly there was hope. There was some horizon in my twenties where CF would be treatable. When I talked to new parents of children with CF, I could tell them there is so much more out there for their children; there is so much hope. The day that I learned about Vertex and everything they were doing, tears welled up in my eyes. It was the beginning of hope. It was looking into my future and seeing possibility, rather than the endless progression of this illness.
When I talk to my doctor about this drug, he always reminds me that though it might not reverse my current symptoms, if I can stay as healthy as possible, perhaps we could stop the disease in its tracks. The hope of a future drug has given me a drive to maintain my health. It meant undertaking a new routine of running that has resulted in the healthiest 5 years within my memory. It meant taking no shortcuts when I came to taking care of myself. And it meant having hope, hope for a future where one pill would change everything.
It wasn’t all positive in these past 5 years, though. I saw my baseline lung function decline about 10%… and even though I was doing everything I was supposed to, I was reminded that CF doesn’t stop. Unexpectedly, I got really sick with pneumonia and I was reminded that CF doesn’t stop. Woven into these days of good health, I had days of fatigue, high fevers, days when I just wanted to be done. In these past 5 years, the CF community lost many people to CF, and we were all reminded that there is no time to wait for these treatments, because CF doesn’t stop.
Each bad day made me impatient. A new therapy was needed, and it was needed yesterday.
News about kalydeco/lumacaftor, now called Orkambi, caused my hope take a roller coaster ride. When studies were first starting, I told the researchers at my hospital to SIGN ME UP. But I ran into roadblocks because of the bacteria I culture. They couldn’t accommodate the special isolation requirements of patients like me. So I would have to wait, and rely on the wonderful CF patients who would participate in this study.
Soon, critics were arguing that it wasn’t as effective as kalydeco was in treating the 4% of people with the “correct” mutation. I read stories of stocks falling as results were not as miraculous. Lung function changes were thrown around… 3%… 2%… 5%…. “Discouraging,” they said.
But somewhere, deep down, I still had hope. I opened a retirement account, went back to school, and talked realistically, for once without fear, about marriage, and kids, and buying a house some day. You cannot live your life just waiting for something to happen. Hope makes uncertainty okay.
I heard stories of people who were doing so well on this drug in trials. Suddenly, the focus was on how it reduced exacerbations, though it did little to raise lung function. Progression is what ultimately kills people with CF… so anything that could slow the progression is HUGE. So I went back to what my doctor said… maybe this will stop it in its tracks. I held onto hope.
Life may be extended for me with this new drug, and perhaps even more with the 15 or so drugs currently being researched– so there is no longer a deadline.
Living a life so certain of uncertainty has become my reason for breathing in and appreciating every experience.
And when they find a drug to extend my life –maybe it will be Orkambi in a few months, or maybe it will be something similar/better in a few years– I will never take this life for granted. I will be beyond grateful for the chance at a long, beautiful, uncertain future.
