Ask the Advocates: Notable Changes in Your CF Journey
Last updated: March 2022
Cystic fibrosis (CF) has had many advances over the years in treatment, research and awareness. Each CF patient has a unique journey to travel, with notable changes along the way. We asked our advocates to share the most notable change they have experienced in their CF journey.
Response from Ella
Beginning Trikafta over 2 years ago has been the most notable change in my health journey. Although the changes in my health overall have been moderate in comparison to other patients, I know it has changed the trajectory of my disease and prolonged the time until lung transplant. When starting Trikafta, I was weeks away from being listed for a transplant. Day to day, for example, mucus reduction has been slight, but over the course of a few months, I found relief for a few more hours between breathing treatments.
I have gained a few extra pounds, something I’ve struggled with my whole life despite always having a calorie rich diet. During exercise, I still require using about the same amount of supplemental oxygen, but I can push my body further and I started lifting weights. The volume of my breaths is now 30% FEV1, increased by 6 percent — as good as my highest lung function in the last three years, albeit a small overall change.
My life will be prolonged to some extent by this medication. This may translate into one more year or five years before I will need a transplant, but with other new gene therapy treatment options in the pipeline, I am optimistic for the possibilities in accessing some of these new therapies in the coming years.
Response from Nicole D
Growing up I never realized how CF may impact my adult life; my lung function was always better than 100% and I never really got sick. As I reached my senior year of high school, I remember a time in the spring when I realized I wasn’t feeling great (and wasn’t getting better); I had my first appointment where my lung function wasn’t over 100%. This decline, albeit it was only the first time, scared me and instilled anxiety that I would not be able to make it up. The next few visits demonstrated the decline wasn’t a fluke and it was then I realized (and personally experienced) the progression of CF.
I think I was naïve to think I was an anomaly, and that CF didn’t really impact my health negatively as long as I kept up with my treatment regimen and followed my doctor’s orders. When things started to change, I realized I truly had no control over CF and despite being dedicated and diligent with my treatments, I could not control the disease. This scared me (and still does) but I started focusing on what I could control and actively try not to go down a rabbit hole of fear.
Response from Meagan
For us as a family, the most notable change was anything that granted Margo more independence in her care. Two things that come to mind are teaching her to swallow pills at a young age and getting the vest. Swallowing her pills helps her save time and helps her count how many pills she needs to take for a snack or meal. It’s helped ease the process in that my husband and I don’t have to prep the applesauce, open the pills, and help her eat the applesauce, making sure she swallows all the enzymes.
The vest has allowed us to give Margo a choice everyday: does she want to use that for her treatment or manual PT. She knows how to plug in the machine and put on the vest, plugging in the tubes. She gets to use the tablet for games or to watch a show. It’s offered the freedom of choice for her and that’s a big step in her CF journey.
Response from Marieliz
How much my body has changed was the biggest change. I can’t breathe as well as I used to, I can’t physically do much as I used to, and I have significantly more pain and respiratory issues than I used to. That was a big change because I have declined and mentally accepting that and finding a positive way to cope was challenging for me.
Response from Janeil
I think the most notable change in my CF journey has been a change in the outlook of my life after starting Trikafta. Prior to Trikafta, I was experiencing a very tough decline with low lung function, low weight, infection after infection, and chronic and debilitating pain all while having a very small child. It was incredibly difficult and I felt like I was on the trajectory of having to make very tough and painful decisions and squash a lot of dreams at a very young age. It almost felt like I was just going to get sicker and sicker with no way to stop it. I felt powerless, overwhelmed, and was in survival mode 24/7.
Thankfully Trikafta changed that trajectory for me in all ways. I stopped declining and stabilized, my quality of life improved immensely, I was able to open the doors to dreams I thought I was forced to let go of. Best of all, I could see a future again that was filled with hope and opportunity. What a wonderful change it’s been!
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