Sleep disorders in patients with primary ciliary dyskinesia, cystic fibrosis with and without pancreatic insufficiency

Abstract

Background: Cystic fibrosis (CF) and primary ciliary dyskinesia (PCD) are characterized by impaired mucociliary clearance causing sinopulmonary infections and airway inflammation. However, they differ in many aspects including multisystemic involvement and disease severity.

Aim: To compare sleep disorders and their correlation with quality of life (QOL) in three distinct patient populations: CF and pancreatic insufficiency (CF-PI), pancreatic sufficiency (CF-PS) and PCD.

Methods: Patients completed age appropriate sleep quality questionnaires (SDSC, PSQI), quality of life questionnaires (PedQL, QOL-BE) and the Epworth sleepiness scale (ESS). Medical records were reviewed for clinical data.

Results: Eighty patients, 33 CF-PI, 27 CF-PS, and 20 PCD; 40 females; 41 adults and 39 children completed the study. In adults, 66% slept less than 7 h per night, and sleep quality was reduced in 26.5%, 53% reported night wakings and 47% nocturnal snoring or coughing. In children 78% slept more than 8 h, SDSC scores were mostly in the normal range. A significant correlation was found between sleep quality and QOL in both age groups. Global sleep scores were correlated with hemoglobin levels. Despite differences in disease severity and QOL, there were no significant differences in sleep quality between the three groups.

Conclusion: Sleep disorders are common and correlate with QOL in CF and PCD. Despite differences in disease characteristics and severity, there are no differences in terms of sleep disorders between CF-PI, CF-PS and PCD patients, suggesting that they may represent indirect outcomes of disease.

Keywords: Cystic fibrosis; Pancreatic insufficiency; Pancreatic sufficiency; Primary ciliary dyskinesia; Quality of life; Sleep.