Sleep-Disordered Breathing In Cystic Fibrosis

Abstract Introduction Cystic fibrosis (CF) is a life-shortening, genetic disease that affects approximately 30,000 Americans. Although patients frequently report snoring, mouth breathing, and insomnia, the extent to which sleep-disordered breathing (SDB) may underlie these complaints remains unknown. Methods Single-center retrospective review of polysomnography results from referred patients with and without CF individually-matched (1:2) for age, gender, race, and body mass index (BMI). Results Mean ages were 8.0±5.2(sd) and 35.9±12.9 years, among 29 children and 23 adults with CF respectively. The CF and non-CF groups were well-matched in age and BMI. Subjects with vs. without CF had 3 times greater odds of moderate-severe SDB (apnea-hypopnea index (AHI) ≥ 5 in children, ≥ 15 in adults) (p=0.01). Nocturnal oxygen saturation nadir (Minimum SpO2) was lower among CF vs. non-CF groups (p=0.002). For every 1-unit increase in AHI, the decline in Minimum SpO2 was larger for subjects with vs. without CF (p=0.05). In subjects with CF, forced expiratory volume in 1 second percent predicted (FEV1 PPD) was associated with Minimum SpO2 (Pearson r=0.68, p Conclusion Severity of SDB may be worse among referred patients with vs. without CF. The SDB may modify the relationship between CF lung disease and nocturnal hypoxemia. Markers of lung disease severity including lung function do not predict SDB severity, suggesting the need for routine polysomnography to screen for this sleep disorder. Study Rationale Little is known about the frequency or severity of sleep-disordered breathing (SDB) in patients with cystic fibrosis (CF) despite recurrent complaints of insomnia, snoring, and mouth breathing. The adverse health impact of SDB could create particular concern for individuals with CF, who already confront a life-limiting illness. Impact Patients with vs. without CF may have more severe SDB. Furthermore, SDB could contribute to some of the differences in sleep quantity and quality between CF and non-CF groups. The relationship between CF lung disease and nocturnal hypoxemia may be modified by the presence of SDB. Testing for SDB may help identify a modifiable risk factor for significant morbidity and mortality among patients with CF.