Dealing with CF's Changes
CF impacts every patient’s life uniquely. Even though we may share the same mutations, our symptoms can be totally different from each other. This can make a life with CF especially isolating, as shared experiences are rare to come by. One thing we all have in common with each other, though, is change. Our health is in a near-constant state of change, and with the idea of change on everyone’s mind now that we’ve entered a new decade, I thought it might be useful to discuss a few aspects of change and how it plays into a life with cystic fibrosis.
Many CFers have care teams to help manage their illness, but no caregiver can truly be present 24-7. As more and more of us reach and learn to navigate independence and adulthood, we all need to be vigilant in identifying cystic fibrosis changes or abnormalities in our symptoms. No one knows your body better than you. That simple idea makes each patient the first and most important line of defense in the battle against CF. The faster we can identify something different (be it a more productive cough, fatigue, digestive issues, or any other number of symptoms), the faster we can work with our care teams to address it.
Staying vigilant also means keeping up with recommended tests and screenings for complications that we can’t detect ourselves. CF is a sneaky disease, with many different ways to affect us. Fortunately, detection of new complications and progressing issues has improved, allowing for earlier identification and intervention. It all starts with us though, staying aware of changes and what to look out and be tested for.
You can’t always stop cystic fibrosis changes
When it comes to life with cystic fibrosis, change is inevitable, and unfortunately often for the worse. Personally, I grew up very lucky to have PFT scores of over 100% throughout a largely carefree childhood. I always felt rewarded for my diligence in keeping up with treatments, exercise, and medications. As time progressed and I grew older, I have watched as my PFTs decrease each year, to the point where I am excited to achieve a 77% if possible. I was unprepared for the effect of the decrease, even though it was gradual.
This change was unstoppable. It required me to adjust to it, rather than the opposite. Physically, this meant I had to increase treatments and exercise just to maintain a baseline. This adjustment decreased the time and energy I had for other more enjoyable endeavors. More significantly, though, I needed to mentally comprehend that my health was going to continue to degrade. That’s no easy concept to grasp, especially when there is so much else happening in one’s late teens. Like so many other patients, I had to learn the reality of what CF's progression meant to my body.
Change can be sudden
Change can also come on without warning. My first bout of hemoptysis (coughing up blood) is still a vivid memory. One moment, I'm 17 years old on an extended big family vacation, enjoying it as much as any 17 year-old. The next moment, I'm experiencing something new and terrified of what was happening to me. I ended up staying up all night, waiting for it to cease while sitting in the family van watching The Godfather with my dad.
It doesn’t matter how many times I’ve had it happen (hundreds) in the years since, I am still a scared little kid every time I cough up red. There is something about it that I will never get used to; a very visceral reminder of how fragile I am and how dangerous CF is.
Over time, after that initial fear with each occurrence, I learned to act; I record what I did to seemingly trigger it (bending over, laying down, eating too much, etc) and become more conscious of that action going forward. I’ve adjusted my diet, changed how I do certain activities, and have been sleeping upright for over 3 years. Even though I wasn’t prepared for it and I haven’t been able to prevent hemoptysis, I’ve found ways to reduce the risk of it and be prepared going forward.
Change can be widespread
Another change I’ve personally experienced with cystic fibrosis has been the development of CF-related diabetes. That’s right, we even have a special blend of diabetes to call our own! This is one that may become more prevalent as CF life expectancy continues to grow, as I understand that it’s the result of pancreatic scarring over time and the decreased ability to produce insulin. I was diagnosed pre-diabetic in high school, which required frequent blood sugar monitoring. I had to learn to work to avoid blood sugar spikes that would lead to further CF complications.
Fortunately, I was able to alter my diet (aka stop eating a sleeve of Starburst candy every day) and eventually stop the blood sugar testing. I remained borderline diabetic in the years since, continuing to learn more about diet and making changes to reduce my reliance on insulin production. Despite that effort, I was still diagnosed with full CFRD at the end of 2019.
I’m continuing to learn how to adjust my diet, water intake, and eating times for what my body can handle, and what kind and intensity of exercise will help to keep organs functioning as well as they can. I’m adapting in ways that have permeated every facet of my life to accommodate my new diagnosis.
Accepting cystic fibrosis changes
CF can impose many problematic changes in our lives, far beyond the few that I've described above. I've learned to deal with those changes by rolling with the punches and doing my best to make things work. On the positive side of things, all the adversity CF puts us patients though renders us well-prepared for changes and better at adapting to new situations than average people. I’ve also learned plenty about diet and exercise, and to appreciate how lucky I still am (77% PFT at age 32 is pretty incredible). I now think of change as an opportunity to learn new skills and information. It’s like school, where even though the subject matter may be dry, I have a powerful incentive to succeed.
There’s a certain freedom in the changes that I’ve experienced in my cystic fibrosis journey. Many of them are things that I couldn’t have prevented despite my diligence in medications and treatments. Maybe I could have delayed things if I had really pushed myself, but these changes were always going to occur. I don’t have to feel guilty about not doing enough to prevent them, but I can feel proud that I was able to catch changes and act on them soon enough to prevent them from getting worse or leading to additional complications. After that first lesson about my health decreasing, I changed my outlook and become more effective at fighting CF. That's another thing that can unite us: striving to be better at battling our common illness.
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