Sleep Disturbances in Cystic Fibrosis and Primary Ciliary Dyskinesia Over Time, Before and after Modulator Therapy
2Faculty of Medicine, Hebrew University of Jerusalem, Israel
3Medical Student, 5th Year, Faculty of Medicine, Hebrew University of Jerusalem, Israel
4Shaare Zedek Medical Center, The Hebrew University of Jerusalem, Israel
Background: Prior studies have shown that cystic fibrosis (CF) and primary ciliary dyskinesia (PCD) are associated with sleep disturbances affecting quality of life (QOL).
Aim: To analyze changes in sleep complaints over time in patients with CF and pancreatic insufficiency (CF-PI), pancreatic sufficiency (CF-PS) and PCD, and explore the effect of CFTR modulators.
Methods: Children ≤18 years old on recruitment completed the Pediatric QOL Inventory (PedsQL), Sleep Disturbance Scale For Children (SDSC) and modified Epworth Sleepiness Scale (mESS), and repeated them 3 years later. Medical records were reviewed for clinical data.
Results: Of a total of 50 patients, 27 were children: 16 CF-PI, 6 CF-PS, and 5 PCD completed the study. On the 3 year SDSC follow up the score was pathological, ≥55, in 5 children (18.5%), suggestive of a high rate of disturbed sleep. A significant correlation was found between sleep quality and quality of life (QOL). The mean SDSC score was unchanged after 3 years, 41.1±11.5 and 42.5±12.35, at baseline and follow up respectively (p= 0.778). There were no significant changes on the global mean SDSC score between children on and off modulators. However, the daytime somnolence SDSC domain score was unchanged in patients on modulators but decreased in the remaining (P=0.055).
Conclusions: Sleep disorders are common and correlate with QOL in CF and PCD pediatric patients. They appear unchanged with time; however, modulator therapy may cause worsening somnolence in CF patients while improving other aspects of patients` sleep.