Does the effect of comprehensive respiratory physiotherapy home-program differ in children with cystic fibrosis and non-cystic fibrosis bronchiectasis?

Bronchiectasis is a form of airway damage as a consequence of endobronchial infection and inflammation and may be present in different diseases. The underlying aetiologies include both cystic fibrosis (CF) and a group of non-cystic fibrosis diseases (NCFB) such as immunodeficiency, primary ciliary dyskinesia, or severe pulmonary infection. Although children with CF and non-cystic fibrosis bronchiectasis (NCFB) have many similar clinical features, their responses to exercise may be different. The aim of this study was to compare the efficacy of a comprehensive respiratory physiotherapy (CRP) home-program in children with CF and NCFB. Thirty children with CF and thirty children with NCFB were included in the study. Both groups performed the CRP home-program twice daily for 8 weeks. Pulmonary function, exercise capacity, and respiratory and peripheral muscle strength were assessed at baseline and after 8 weeks of training. Both groups experienced significant improvements in pulmonary function, exercise capacity, and respiratory and peripheral muscle strength (p < 0.001). Maximum expiratory pressure, exercise capacity, and peripheral muscle strength were further improved in NCFB group compared to CF (p < 0.05); however, there was a great variability in the improvements for each variable.

Conclusion: CRP is beneficial both for children with CF and NCFB and adherence to the program was high in both groups.