The Cystic Fibrosis Foundation recently announced that there are almost 40,000 people living with CF in the United States. Not only are there more of us with CF, but we are now living longer. Based on the 2021 CF Foundation Patient Registry data, the current life expectancy for CF patients born between 2017 and 2021 is 53 years — a substantial jump from a decade ago when the life expectancy was 38. Now, almost 60% of us are older than 18. These numbers mean something special for us and the way we live our lives.
I’m a 22-year-old with CF living in south Texas. Since starting Trikafta in 2019, I’ve experienced a huge improvement in my health. Trikafta has allowed me to graduate college, start a full-time job in a new city, and live a freer lifestyle overall. In high school, before Trikafta, these achievements felt almost impossible.
Now that I’ve seen the information about the CF population increase, I can’t imagine how patients’ and families’ lives will change even more in the next few years. It’s so exciting to think about how my treatment regimen has changed in the past two years, and based on this information, I can only imagine my daily routine — and that of other patients — continuing to change for the better. Seeing these increased numbers gives me hope for the future of my health.
I was able to ask Dr. Whitney Brown, senior director of clinical affairs at the CF Foundation, about what these numbers might mean for our care. In addition to leading the CF Foundation’s work on adapting care to meet the changing needs of people with CF, Dr. Brown is also a practicing adult CF doctor.
Does the rise in the CF population mean that care center resources will be stretched thinner?
Not necessarily. CF programs, particularly adult programs, will likely continue to experience increases in the number of patients they see, but patients may be healthier overall.
Certainly as adults age, their medical needs may become more complex, requiring greater coordination between CF care teams and specialists. However, this trend may be offset by those who benefit from CFTR modulators and experience improved health, like fewer pulmonary exacerbations. So, although adult CF programs may be caring for more people with CF overall, many patients will be healthier and require less intense medical care, like fewer antibiotics and hospital admissions. As a result, care teams may be focused more on outpatient care.
As a CF provider, what have you seen your patients do that previously seemed more difficult for them?
Many of my patients describe having more energy to live life more fully. My patients have marveled over being able to blow up a balloon for the first time, keep up with their kids on a soccer field, and not have to stop to rest when walking up a hill with friends. I think having improved health has been a little bittersweet during the pandemic, as many have felt well enough to go out and travel more but have been restricted by COVID-19. I have had patients express gratitude that they cough so much less on Trikafta — and we all know that cough is particularly stigmatizing during a pandemic.
With more energy and fewer respiratory symptoms, I have encouraged patients to challenge themselves by working out. Channeling energy into exercise is good for lung health, for mental health, and general well-being. Plus, it can take many forms (walking, hiking, biking, dancing) and can be done safely outside.
What is important for care teams to keep in mind as they care for more adults?
Along with a growing number of adults with CF, there is more diversity among adults with CF — diversity in backgrounds, interests, health status, lung function, CF complications, as well as goals and dreams. As adult CF care providers, we must meet patients where they are in their CF journey and tailor our approach to meet individual needs.
Even with so much growth and change, the need to create space to connect and get to know patients as individuals remains critically important. We need to have open discussions with patients about their treatment plan, one that considers all aspects of who they are and what they value most.
As people with CF are living longer, we may see different manifestations of the disease as well as other issues that come along with the general aging process, like high blood pressure and heart disease.
Partnering with other medical specialists to help people with CF navigate a spectrum of health needs will be key.
Lots of people have felt a sort of “imposter syndrome,” because they are living a healthier life and decreasing treatments. How can our community support patients as they live longer and experience these feelings?
It is critical to acknowledge and normalize these feelings. Although everyone is different, there are many common emotions and experiences for people with CF going through similar circumstances. Whether it is feeling a sense of lost identity after experiencing a dramatic change in health status or feeling left behind because of not benefitting from CFTR modulator therapy, telling one’s story can be therapeutic not only for the person sharing, but for others who can relate.
The CF community should look for opportunities to create a safe place for sharing and acknowledge that there are a wide range of emotions involved in any change. Living healthier and longer is the goal we have always worked for, but ironically, it often comes with unanticipated feelings and challenges.
Does the updated estimate change the Foundation’s priorities?
As the number of people living with CF increases, their health care needs will also change. Tracking these changes in the Registry helps inform how the CF Foundation collaborates with the CF community to prioritize investments in research, clinical training, programs, and policies that support people with CF and their families — to address needs they are experiencing now and what they’ll need in the future.
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