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.

Lower airway clinical outcome measures for use in primary ciliary dyskinesia research: a scoping review

“…Definitions of outcome measures varied considerably between studies ( supplementary table 2 ). Of 18 studies reporting on microbiology as study outcomes, 14 provided data on chronic colonisation by potentially pathogenic bacteria [ 29 , 31 , 34 , 38 , 59 , 64 , 65 , 70 , 75 , 82 , 96 , 100 ]. The terms chronic colonisation and chronic infection were sometimes used interchangeably, and the classification used to define them varied.…”

Association of sleep disorders with heart rate variability in children and adolescents with cystic fibrosis

“…These findings are in accordance with data in the literature that demonstrate reduced sleep efficiency and a positive correlation with disease severity. 2 Nocturnal hypoxemia was present in 26.7% of individuals; evidence suggests that frequent episodes can lead to inflammation of the pulmonary parenchyma, as well as the development of pulmonary hypertension and failure of the right ventricle, leading to associated changes in HRV, with increased sympathetic predominance. 24 …”

“…Among several complications, individuals with CF often present sleep disorders, including hypoxemia and obstructive sleep apnea syndrome (OSAS). 1 , 2 Episodes of nocturnal hypoxemia have been related to worsening pulmonary function and, consequently, increased mortality. 3 , 4 In addition, the decrease in the peripheral oxyhemoglobin saturation (SpO 2 ) during the night has been associated with pulmonary hypertension, reduced sleep efficiency, and neurocognitive dysfunction during exacerbations.…”

Topic Sessions

“…Patients with CF present often with depression, anxiety and other psychological symptoms affecting quality of life and adherence to treatment, which in turn affect morbidity and mortality 17 . S leep disorders are commonly observed in patients with CF, with a strong correlation between quality of sleep and quality of life throughout the patient's life span 18 . The occurrence of attention deficit hyperactivity disorder (ADHD) symptoms in patients with CF is substantially higher than in the general population and should be recognized as a co‐morbidity of CF 19 , possibly associated with low adherence to treatment and consequently, poor prognosis.…”