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.