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Airway clearance after highly effective CFTR modulators: Normalizing life and reducing treatment burden
Danielle M Goetz1, Carla K Frederick2, Geovanny Perez1
1Department of Pediatrics, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, Buffalo, New York, USA.
Pediatric Pulmonology
|May 26, 2023
Summary
Many people with cystic fibrosis (CF) are reducing or stopping airway clearance therapy (ACT) after starting highly effective CFTR modulator therapy (HEMT). This shift impacts treatment decisions for CF care teams.
Area of Science:
- Pulmonology
- Medical Therapeutics
- Patient-Reported Outcomes
Background:
- Airway clearance therapy (ACT) is a cornerstone of cystic fibrosis (CF) management, yet it presents a significant treatment burden.
- Highly effective CFTR modulator therapy (HEMT) has revolutionized CF care, improving pulmonary function for many individuals with CF (pwCF).
Purpose of the Study:
- To investigate the evolving attitudes and practices surrounding ACT in the post-HEMT era among pwCF and CF care providers.
- To understand how HEMT influences decisions regarding ACT and exercise regimens.
Main Methods:
- Cross-sectional surveys were administered to CF community members (including pwCF and parents of children with CF) and CF care providers.
- Data collection occurred between July 20 and August 3, 2021, utilizing CF Foundation platforms.
Main Results:
- A substantial proportion of pwCF and parents reported reducing or ceasing ACT after initiating HEMT (up to 51% of adults and 36% of parents reduced ACT; 13% of adults stopped ACT).
- A majority of both community members (59%) and providers (68%) believed exercise could partially substitute for ACT.
- Half of CF care providers adjusted their ACT recommendations for patients on HEMT, and over half of all respondents discussed ACT modifications with their care team.
Conclusions:
- CF care providers must recognize that pwCF may be altering their ACT regimens due to the benefits of HEMT.
- Treatment burden associated with ACT should be a key consideration in shared decision-making regarding ACT and exercise for pwCF on HEMT.
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