Cystic fibrosis care has changed over time, and it has contributed to major gains in health and survival. CFTR modulators have improved outcomes for many, and as people with CF live longer, healthier lives, the needs of people with CF are becoming more diverse. Care must continue to change to meet those needs — and to accommodate technological tools like telehealth. In a recent issue of the Journal of Cystic Fibrosis, the Cystic Fibrosis Foundation published two position papers regarding changes in cystic fibrosis care. It took more than a year to develop these papers, which were researched and written by a multidisciplinary committee of CF care team members, people with CF, and parents of children with CF.

As these community members were knee-deep in research and weekly discussions during the writing of these papers, they wanted to offer their individualized perspectives on how key elements of CF care may realistically be impacted by the suggested changes to the CF care model and care team structure.  

Linda Bowman: Coordination of Care

Living with CF involves navigating a complex web of symptoms and treatments. While advancements in CF care have significantly improved the quality of life for many, there remains a considerable gap between the current state and the ideal care model. This gap becomes even more evident when additional health issues arise, as I recently experienced.

After a long period of misdiagnoses and hospitalization, I needed a urologist. Reaching out to my CF care team, I was disheartened to learn they had no recommendations for a urologist familiar with CF. This situation is unfortunately common, as there are few specialists who truly understand the intricacies of CF, particularly in older patients dealing with multiple health challenges.

In an ideal world, my CF care team would have established relationships with a network of trusted specialists. This would ensure that, regardless of the health issue, I could be referred to a specialist who is not only skilled in their field but also knowledgeable about the unique challenges CF patients face. This "calibration of care" would involve close coordination between my CF care team and various specialists, ensuring seamless and informed care across all facets of my health.

The journey to improve the CF care model is ongoing, but the goal is clear: to create a care system where CF patients, especially those with additional health challenges, receive the comprehensive, coordinated care they deserve. By bridging the gap between the current state and the ideal state, we can enhance the quality of life for all CF patients, ensuring they receive the best possible care throughout their lives.

Rebecca Preslar: Frequency of In-Person Clinics

When I realized how much my two children with CF were thriving after having been on modulators for several years, I began to question how many treatments, medications, and doctor visits they really needed anymore.

I believed the CF care model needed a review, which is why I was thrilled for the opportunity to participate on the CF Foundation Care Model Committee as a community representative. What I didn’t expect was how much my eyes would open to the varying experiences of others with CF across the country.

The reality is every individual with CF has unique medical needs, personal goals, financial circumstances, and preferences. 

All these factors and more could impact someone’s reason to visit clinic more or less frequently. While there are stages in life where more visits will always be recommended for optimal monitoring, there are other moments when less frequency will be perfectly acceptable. There will never be one solution that fits the needs of every person with CF!

Olivia Surry: Integration of Telehealth

As a mom to an active 2-year-old, prioritizing my own health has certainly evolved. While I recognize the importance of ensuring my CF is stable so I can be healthy enough to care for my son, finding the balance to care for both of us has been a constant learning curve and uphill battle. If there is one thing I have come to realize undoubtedly, it is that time is precious. Enter telehealth — an approach that has come to be a game-changer for life as a busy, dedicated mom.

While I will always be an advocate of the benefits of seeing the care team in person, I very much appreciate the ability to hop on a quick touch-base with my provider when needed. Whether it be a brief, virtual appointment to let her know all is good, or a more urgent request to get an antibiotic called into my local pharmacy, telehealth visits have allowed me to find that difficult balance of staying on top of my own health needs, while also keeping up with my maternal demands. With the option of telehealth once in a while, I feel that I am able to reclaim the valuable time I may have spent driving down to my care center 20 minutes away and sitting in a waiting room for 45 minutes so I can have more time and energy to be the healthy mom my son deserves.

Jennifer Kyle: Expectation of Remote Monitoring 

As a CF patient living a far distance from my CF clinic, I’ve had to familiarize myself with remote monitoring. Finding a good primary care provider (PCP) who would work with my CF clinic was crucial. The PCP can help sign off on orders if my clinic does not operate under the purview of the facility where I will be getting tests done. Labs, radiology, IV-line care, sputum collection, and pulmonary function tests are just some examples. Also, the PCP can help communicate the results to my clinic if needed. Learning home spirometry was also important to help convey lung function information to my care team. Staying on top of test results through the patient portals also helps with my care. Often, results come quickly through the portal, and I can send them directly to my care team.  

Noah Singer: More Tools for the Road Ahead

All parents want the best for their children. Parents of children with CF are no different. What is different is the care team’s critical role and the unique way you have to approach your child’s health. It can be hard to understand care options if you're not medically trained. It really is a bit of “you don’t know what you don’t know.”

Being involved in conversations about how CF care is changing has opened my eyes to what’s possible. Hearing from cross-disciplined professionals has been incredibly educational. As a parent, I feel more empowered to do my own research, ask better questions, and think more broadly about what options exist for my child.

One of the biggest shifts for me has been recognizing the elevated role of the pharmacist on the CF team. Managing insurance across multiple pharmacies and navigating a long list of medications is not simple. The CF pharmacist plays a crucial role in advocating for your child’s health and helping you cut through the complexity. Strengthening my relationship with our CF pharmacist has genuinely made it easier to take care of my child.

Whether you are a person with CF advocating for yourself, or a parent or caregiver supporting a loved one, the evolution of CF care gives all of us more tools in our tool belt. We now have clearer language, better concepts, and more thoughtful approaches to help guide each of our unique CF journeys.

Interested in sharing your story? The CF Community Blog wants to hear from you.