There are significant age differences in adherence. Younger patients have better overall adherence likely secondary to increased parental supervision. Having better nutritional status is associated with improved adherence.

Cystic fibrosis is a chronic, life-shortening illness that affects multiple systems and results in frequent respiratory infections, chronic cough, fat malabsorption and malnutrition. Poor sleep is often reported by patients with cystic fibrosis. Although objective data to explain these complaints have been limited, they do show poor sleep efficiency and frequent arousals. Abnormalities in gas exchange are also observed during sleep in patients with cystic fibrosis. The potential impact of these abnormalities in sleep on health and quality of life remains largely unstudied. This review summarizes what is known about sleep in children with cystic fibrosis, and implications for clinical practice. This report also highlights new evidence on the impact of sleep problems on disease-specific outcomes such as lung function, and identifies areas that need further exploration.

Frequent home PFT monitoring is feasible in CF adolescents and could successfully improve medication adherence without significantly impacting treatment burden.

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 SpO 2 ) was lower among CF vs. non-CF groups (p=0.002). For every 1-unit increase in AHI, the decline in Minimum SpO 2 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 SpO 2 (Pearson r=0.68, p<0.0001) but not AHI (r=−0.19, p=0.27).

Medication adherence is poor among pediatric cystic fibrosis (CF) patients, with adolescents having one of the lowest adherence rates. We wanted to identify an adherence intervention that would be acceptable to CF adolescents and assess its feasibility. We surveyed 40 adolescents with CF and asked about barriers to and motivators for their own adherence and to generate ideas for potential adherence interventions. Since most of the respondents chose frequent spirometry at home and medication reminders for interventions, we selected 5 subjects, 10 to 14 years of age, with CF to test the feasibility of home spirometry and medication reminders in pediatric CF patients. This article summarizes the results of both the survey and the feasibility pilot study.

Introduction: Despite emerging data that suggest a high frequency and severity of obstructive sleep apnea (OSA) among patients with cystic fibrosis (CF), few of them are referred for polysomnography. Little is known about which patients with CF are at increased risk for OSA and which sleep symptoms merit investigation. Methods: A single-center retrospective analysis of clinical and polysomnographic data from 2009, January 1 to October 31, 2020 in referred children and adults with CF.Results: Among 74 patients (42 children, 32 adults) with CF, 39 (53%) had OSA. No age or sex differences emerged in OSA frequency. Mean apnea-hypopnea index (AHI) was higher among overweight/obese adults (n = 16) as compared with adults of normal weight or underweight (11.4 vs. 6.2; p = 0.005). Adults with (n = 10) versus without a crowded oropharynx had 13.0 times greater odds of OSA (95% confidence interval (CI): 1.4, 121.4; p = 0.02). Children with (n = 24) versus without tonsillar hypertrophy had a higher risk for OSA (OR = 5.2; 95% CI: 1.4, 19.8; p = 0.02), as did children with (n = 10) versus without symptomatic chronic sinusitis (OR = 5.8; 95% CI: 1.1, 32.1; p = 0.04). Neither snoring, excessive daytime sleepiness, nor lung disease severity were associated with OSA. Conclusion:Key risk factors for OSA may differ between children and adults with CF: upper airway pathology appears important in children and overweight/obesity or a crowded oropharynx in adults. Given the lack of sensitivity of snoring, daytime sleepiness, and lung disease severity, detection of OSA may require a low threshold for polysomnographic assessment in this vulnerable population.

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Introduction The frequency of obstructive sleep apnea (OSA) may be high among patients with cystic fibrosis (CF), a life-shortening, genetic respiratory disease that affects approximately 30,000 Americans. Yet, the potential relationship between OSA and lung function has not been thoroughly explored. Methods Single-center retrospective review of polysomnography (PSG) results from 2009-2017 in referred patients with CF and available pulmonary function data (PFTs) obtained at time of PSG and at 3, 6, 9, and 12-months prior. Results Mean ages were 11.1±3.9 (sd) and 37.1±14.1 years, among 18 children and 16 adults, respectively. Mean body mass index (BMI) was normal in both groups (62.5±26.6% in children; 25.1±6.4 kg/m2 in adults). Twenty-six subjects (76%) had OSA (apnea-hypopnea index &gt;1 in children, ≥5 in adults). Mean forced expiratory volume in 1 second percent predicted (FEV1 PPD) was higher among subjects with vs. without OSA at PSG and at each time-point in the year prior, independent of age and BMI at PSG (longitudinal mixed effects model, β=19.0, SE=8.1, p=0.028). While FEV1 PPD remained unchanged in the non-OSA group, FEV1 PPD at PSG was lower, in comparison to the year prior in subjects with OSA, with the greatest difference observed at 9-months prior to PSG (2-sample t-test, difference of -6.6% vs 0.6% in OSA vs. non-OSA groups respectively, p=0.078). Conclusion The PFTs, as daytime markers of CF lung disease severity, do not seem to reliably predict risk for OSA. In our sample, CF patients with vs. without OSA had better PFTs at baseline but they also showed a greater tendency for decline in PFTs over the year prior to OSA diagnosis. Larger sample size and longer duration of assessment may help, going forward, to assess any potential adverse impact of OSA on lung function decline. Support NIH Training Grant (T32NS007222, F32HL145915)