Introduction

chest physiotherapy, airway clearance techniques, and physical exercise are associated with better outcomes in cystic fibrosis, but adherence to these recommendations remains a challenge.

Objective

to assess adherence to chest physiotherapy, airway clearance techniques, and physical exercise by children and adolescents with cystic fibrosis and analyze the factors related to adherence.

Methods

retrospective study of children and adolescents with cystic fibrosis from a pediatric referral center in Brazil. Data were collected from electronic records of the multidisciplinary team responsible for caring for these patients at the referral center.

Results

the sample included all 83 patients registered at the referral center, with a mean age of 7.88 ± 4.88 years. Among them, 28.9% did not have weekly chest physiotherapy, 66.3% did not practice physical exercise regularly, and only 38.6% practiced airway clearance techniques from 6 to 7 times a week. Higher adherence to airway clearance techniques was associated with higher mean age (p<0.01) and correlated with FEV1 (r=-0.39; p=0.03), FEF25-75% (r =-0.36; p=0.02), FEV1/FVC (r =-0.43; p=0.01) and lower mean Shwachman-Kulczycki Score (r=-0.34; p<0.01). Higher physical exercise adherence was associated with a higher mean age (p<0.01). Individuals using public financing of physiotherapy services had a lower Shwachman-Kulczycki Score (p=0.02) than those using non-public services. The metropolitan region had a higher percentage of individuals using non-public services than other regions (p<0.01).

Conclusion

adherence to chest physiotherapy, airway clearance techniques and physical exercise was lower than recommendations. Airway clearance techniques and physical exercise adherence increased with age, and airway clearance techniques adherence was associated with the high severity of disease. These results suggest that patients only increase adherence when they get older and the disease worsens, highlighting the need for strategies to increase adherence early.

        · abstract in Portuguese     · text in English | Portuguese     · English ( pdf ) | Portuguese ( pdf )