Quality of life and exercise capacity in patients with primary ciliary dyskinesia

Abstract

Introduction: Primary ciliary dyskinesia (PCD) negatively affects quality of life because of various symptoms. However, no study evaluated quality of life, exercise capacity, pulmonary functions, respiratory muscle strength and endurance in patients with PCD. Aims and objectives: To compare aforementioned outcomes in patients with PCD and healthy controls. Methods: Twenty-seven patients (10.74±4.01years,16F,11M) and 28 controls (12.07±3.64years,15F,13M) were included. Quality of life (6-12years child/parents’, 13-17years adolescent (QOL-PCD)), pulmonary functions (spirometer), exercise capacity (6-minute walk test (6MWT)), respiratory muscle strength (MIP, MEP) (mouth pressure device) and endurance (incremental threshold loading test) were evaluated. Results: Children with PCD (6-12years) and parents’ physical functioning, upper and lower respiratory symptoms, hearing functioning, parents’ treatment burden scores, 6MWT distance, FEV1(L), FVC(L), FEV1/FVC, PEF(L), FEF%25-75(L), FEF%25-75(%), MIP, respiratory muscle endurance were significantly lower (p 0.05). Conclusions: The quality of life is adversely effected in PCD patients during childhood. Pulmonary function, functional exercise capacity, respiratory muscle strength and endurance are impaired in patients with PCD. Patients should be included in appropriate pulmonary rehabilitation program at an early age.