The Progression of CF through the Years
Last updated: April 2021
The average life expectancy of someone with cystic fibrosis when I was born was 31 years old. Although I haven’t reached it, in a way, I have beaten the odds.
I recall birthdays growing up. My family would celebrate with a chocolate cake with raspberry filling, and my name written across the top in pink cursive frosting and the same number of small candles as the age I was turning. I can remember my dad’s voice quivering slightly by the end of the “happy birthday” song.
I think for each year I get older, metaphorically, he sheds an extra tear of joy – being happier than the year before, that with each passing year, I am one closer to living the long life they hope and pray their little girl would have. I know my parents have always had a little seed of heartache that they may outlive their youngest daughter. It’s a feeling I can’t comprehend, I just imagine.
Cystic fibrosis disease progression over the years
When I think about the progression of my disease, I realize, there is a gradual decline in the intensity of physical activities that I can accomplish with each passing year. My level of exertion ability decreases. My birthdays are marked by one less thing I can do and they are a reminder of what I’ve lost over time.
In the last year or two of high school, up until I was about 18, my brother was my fitness guru, always encouraging and sometimes nagging me to sprint from our mailbox to the corner stop sign and frog hop up and down the driveway. When I was extra motivated, I also was running about ½-1 mile in the neighborhood. One spring day, at the end of the loop finishing at the stop sign on the corner, I felt the urge to cough. Immediately, I was spewing globs of pure blood and bracing myself against the sign. This was one of the last times I ran any distance.
The year I turned 21, I started to require supplemental oxygen while exercising. Because of this, I got a treadmill and weights to use at home and I no longer went to a gym. Without using the extra oxygen, my blood oxygen levels began dipping into a range that could damage my heart. It was defeating to know that my lungs began failing at the job they are required to do - supplying oxygen to my body.
Carrying around a machine that puffs loudly with each breath and a tube connected to my nose, I wouldn’t allow myself to be seen as different or abnormal. Even today, I have a hard time being seen in public with it, and although I don’t require using it constantly yet, it is the way that my illness becomes visible from typically the invisible - and in turn, a mental obstacle.
At 25, I realized that bringing just 2 bags of groceries inside the door of my house from my car 50 feet away was equivalent to carrying a 50-pound bag up a steep cliff. Especially during exacerbations of the infections in my lungs, I am unable to carry anything or even walk at a normal pace, due to the lack of oxygen and my airways feeling like they are the diameter of a spaghetti noodle. During some of these times, I know my body needs the extra oxygen yet I’ve deprived it because of my unwillingness to show the outward signs of the progression of my disease.
Next month I turn 29 years old – the last year of my 20s! A few weeks ago, I found the first gray hair on my head. This was such a poignant moment. Growing up, I didn’t imagine experiencing life long enough to age at all. Granted, I am still young, but this gray hair was the first sign that I may reach an age closer to the lifespan of the average person.
Celebrating every year of life
Just a year ago, I didn’t think I would still be in this health state – having my native lungs. Last year I was referred for lung transplant after a terrible lung infection that dropped my lung function to only 18%, but thanks to starting Trikafta around the same time, my health has stabilized and hasn’t significantly progressed since that time.
Despite my best efforts to maintain my health and with the help of Trikafta, I know when I blow out my 30 candles next year, there will be some diminishment in my physical abilities, but I’ll think of all of the new things I did, places I went, and people I met. Despite my declines, birthdays always mark a celebration of my life.
How has disease progression impacted your cystic fibrosis journey? Share and connect with others in our forums.
Have you participated in clinical or market research before?