Obstructive sleep apnea in children with cystic fibrosis on highly effective modulator therapy

Introduction: Obstructive sleep apnea (OSA) is common in children with cystic fibrosis (CF). Highly effective modulator therapies (HEMT) have led to improved sinopulmonary disease, but whether this translates to a lower frequency of OSA is unknown. Methods: We conducted a single center retrospective review of polysomnographic (PSG) data from 2012 to 2023 in patients aged 0-18 years with CF to assess frequency of OSA. Participants were classified based on HEMT status. Logistic regression was used to quantify the association between HEMT and OSA with p < .05 considered significant. Results: Forty-nine children underwent PSG during the study period. Ten percent were of non-White race and 24% were of Hispanic ethnicity. Twenty-one children (43%) were on HEMT. These children were older than those not on modulators (11.6 vs. 6.4 years; p = .0001) but no different with respect to gender, race, nutritional status, or lung function. Twenty-eight (57%) children had OSA. Odds of having OSA were higher in the HEMT group (odds ratio [OR] = 4.3; 95% confidence interval [CI]: 1.2-14.9; p = .02). Tonsillar hypertrophy was associated with an increased odds of having OSA independent of modulator status (OR: 6.6; 95% CI: 1.2-37.9; p = .03). Conclusions: OSA is frequently diagnosed in the post-HEMT era in this large, racially diverse group of children with CF. Children on HEMT were older and more likely to have OSA as compared to those not on modulators but similar in nutritional status, lung function, and presence of upper airway pathology. Prospective studies are needed to further clarify the relationship between HEMT and OSA in children with CF.