Perception and participation in sport and exercise in cystic fibrosis: The impact of CFTR modulators
Related Experiment Video
Updated: Jun 9, 2025

Generation of Human Nasal Epithelial Cell Spheroids for Individualized Cystic Fibrosis Transmembrane Conductance Regulator Study
Published on: April 11, 2018
Akos Berthold1, Eileen Barr2, Ajay Kasi3
1Inova Health System, Falls Church, VA, USA.
Background:
People with cystic fibrosis (pwCF) have barriers to physical activity including exercise intolerance and fatigue. The advent of small molecule cystic fibrosis transmembrane conductance regulator (CFTR) modulators have shown great clinical improvements in pwCF; however, the effect of CFTR modulators on exercise perception and participation is unknown. The purpose of this study was to investigate whether the administration of CFTR modulators changed the perception and participation in sport and exercise in pwCF.
Methods:
A survey-based, retrospective cohort study was conducted including individuals aged 18 and above.
Results:
Eighty-three participants were recruited with a mean age of 30.0 ± 10.5 years. The majority (82 %) of participants were taking CFTR modulators. Participants in the modulator group rated enjoyment of exercise and importance of exercise higher than the non-modulator group and were more likely to exercise as a means to socialize. Participants in the modulator group reported lack of time as the most frequent barrier to sport and exercise whereas fatigue was most reported in the non-modulator group. Twenty-eight percent of the modulator group participated in team/structured sports, whereas no participant engaged in team and structured sports in the non-modulator group.
Conclusions:
Adults with CF who used CFTR modulators rated exercise more favorably and engaged in more team sport activities compared to pwCF who did not use CFTR modulators. The most frequent barrier to exercise in pwCF who used modulators might no longer be CF-related. More research is needed to confirm whether CFTR modulator administration resulted in increased overall physical activity.
More Related Videos
07:04Forskolin-induced Swelling in Intestinal Organoids: An In Vitro Assay for Assessing Drug Response in Cystic Fibrosis Patients
Published on: February 11, 2017
09:59Functional Reconstitution and Channel Activity Measurements of Purified Wildtype and Mutant CFTR Protein
Published on: March 9, 2015
Related Concept Videos
Cystic Fibrosis: Management
Sinus disease and chronic...
Cystic Fibrosis: Pathogenesis
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation,...
Chest Physiotherapy
Purpose
CPT is primarily used for patients with excessive bronchial secretions who have difficulty clearing...
Chronic Obstructive Pulmonary Disease-V: Management
Smoking Cessation
Exercise and Muscle Performance
Endurance exercises
Endurance exercises involve running, swimming, or cycling, which require repetitive movements with low force output. When a person engages in endurance exercise, a few noticeable changes occur in their skeletal muscles. For instance, the number of capillaries...
COPD: Management Using Bronchodilators and Corticosteroids