At the Volunteer Leadership Conference in March, Dr. Mike Boyle shared that people with CF now have a predicted median survival of 56 years. This number means that half of people with CF born between 2018 and 2022 with CF will live to be 56 or older. I’m 57, born in 1965, a year before the start of the CF Foundation Patient Registry, which collects patient data that is the basis for the median survival chart. I was diagnosed with CF in the late-1970s when the median survival was around 15, which was my age at that time. Fortunately, I chose to not let that number limit what I could do with my life. Instead, I focused on staying healthy, achieving in school, having a career, and living as normal a life as possible.
The increase in survival is an important milestone for the CF community and worth celebrating. The steady advance in survival demonstrates that tremendous progress is being made. The improving survival curve offers hope to patients and families that those with CF can have the opportunity to live longer, more fulfilling lives, to possibly have families, careers, and to live with fewer CF challenges. However, there are many factors that affect the disease course of each patient including their specific CFTR mutations, other factors in their genetic background, eligibility for or ability to tolerate new medications, adherence to treatments, and access to care, to name a few. As people with CF, we are well aware that everyone’s CF journey is different and even people with the same CF mutations can have very different experiences, complications, and outcomes.
As a research scientist and an adult living with CF, I tend to focus on the medical advances that have occurred over my lifetime, including new classes of antibiotics, inhaled antibiotics, inhaled treatments such as Pulmozyme and hypertonic saline, new anti-inflammatory drugs, vest physiotherapy, and CFTR modulators. Other factors including advances in transplant medicine, nutrition, and improved CF clinical care coordination have also positively affected our survival. The development of modulator drugs specifically targeting the CFTR protein has been an amazing leap forward for the CF community.
I followed the development of modulators closely and was finally able to start Trikafta® in December 2019. I wanted to be part of the early clinical trials, but was not eligible because I culture Burkholderia cepacia in my lungs, so I had to wait until it was approved by the U.S. Food and Drug Administration. Once I started Trikafta, I immediately felt a change in my lung function and overall health. I have continued with my previous treatment regimen and have maintained my improved lung function over the past three years.
The advances in CF survival highlight that what it means to live with cystic fibrosis is evolving. Currently, 60% of CF patients in the US are older than 18 years old. The fact that many patients are living longer into adulthood, while exciting for people with CF and their families, does offer some unique challenges in CF care.
In my lifetime I have seen continued improvements in CF care beyond just the new medicines. When I was growing up, my CF care focus was on lung infections, lung function, and digestive issues. Now as adults with CF, some of us face challenges with cystic fibrosis-related diabetes, osteoporosis, CF liver disease, and other manifestations of CF that hadn’t been in the forefront previously.
Particularly interesting is that adults with CF living longer now start to face the “normal issues” that come with aging including hypertension, heart disease, arthritis, cancer, mental health issues, and other health challenges that most people face as they age. Now our health care needs have expanded beyond our core CF care team and involve primary care doctors, endocrinologists, internal medicine doctors, oncologists, and other specialists — not all of whom are part of our CF care team and may have much less knowledge of CF. I have needed to be more proactive in my overall health care to make sure my CF care team and other doctors I need to see all have access to my medical records so that there can be coordination of my medical care, but ultimately, I need to be my own health care coordinator.
I am excited for a new era of CF care where we can celebrate the milestone advances that are leading to longer, healthier, and more productive lives but also focus more attention on new health concerns and coordination of care for the aging population. I hope additional advances including earlier introduction of modulators and genetic therapies can lead to entire lives being free of many of the effects of CF and the entire CF population can experience the luxury of longevity.
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