Sleep-disordered breathing in patients with cystic fibrosis

Abstract

Objective: To test the hypothesis that disease severity in patients with cystic fibrosis (CF) is correlated with an increased risk of sleep apnea. Methods: A total of 34 CF patients underwent clinical and functional evaluation, as well as portable polysomnography, spirometry, and determination of IL-1β levels. Results: Mean apnea-hypopnea index (AHI), SpO2 on room air, and Epworth Sleepiness Scale score were 4.8 ± 2.6, 95.9 ± 1.9%, and 7.6 ± 3.8 points, respectively. Of the 34 patients, 19 were well-nourished, 6 were at nutritional risk, and 9 were malnourished. In the multivariate model to predict the AHI, the following variables remained significant: nutritional status (β = −0.386; p = 0.014); SpO2 (β = −0.453; p = 0.005), and the Epworth Sleepiness Scale score (β = 0.429; p = 0.006). The model explained 51% of the variation in the AHI. Conclusions: The major determinants of sleep apnea were nutritional status, SpO2, and daytime sleepiness. This knowledge not only provides an opportunity to define the clinical risk of having sleep apnea but also creates an avenue for the treatment and prevention of the disease.

Sleep disturbances and their impact in pediatric cystic fibrosis

“…Nocturnal oxygen saturations are lower among children with CF who have OSA as compared to those who do not (27, 36, 50, 61, 62). However, the severity of the SDB in these children does not appear linked to the degree of pulmonary impairment as measured by the FEV1 % predicted (27, 63). Furthermore, sleep efficiency and frequency of arousals also seem no different in childhood CF with and without OSA (61).…”

“…Obstructive sleep apnea (OSA), a common and consequential form of SDB, is characterized by repeated cessation or diminution of airflow during sleep, due to upper airway obstruction and despite adequate continued respiratory effort. The frequency of OSA is higher among young children with CF as compared to healthy controls (36, 61–63). Polysomnography in children with CF between 6 months and 11 years of age reveals moderate OSA (apnea-hypopnea index (AHI) ≥ 5) in approximately 46% (36).…”

“…This raises the possibility that, despite the high prevalence of SDB in children with CF, the OSA might not explain the increased burden of sleep complaints. However, in the two studies that have evaluated the potential relationship between lung function and SDB to date, all or most of the participants had a normal AHI (27, 63). The possibility also remains that children with SDB may have subtle inspiratory microarousals not readily detected by standard polysomnography but still impactful on sleep quality (64–66).…”

Sleep‐disordered breathing in cystic fibrosis

“…People with CF may be inherently at risk for circadian rhythm disorders due to defective cystic fibrosis transmembrane regulator (CFTR) function in the hypothalamus, however CFTR has not been implicated in SDB and a genetic predisposition to SDB has not yet been identified in CF 33,34 . SDB can potentially be suspected by daytime oxygen saturation, nutritional status, and daytime sleepiness 5,35 . In patients with moderate to severe lung disease, evening paO 2 can also raise suspicion nocturnal hypoxemia and hypoventilation 36 …”

Insomnia in adults with cystic fibrosis: strong association with anxiety/depression and impaired quality of life

“…In addition to lung function, other factors are likely to impair sleep quality in CF adult patients such as night-time symptoms (cough (14), pain (15)), restless legs syndrome (RLS) (16) (17), anxiety and depression (12) (18), sleep-related breathing disorders (19) (20) or unhealthy lifestyle.…”