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Published on: April 11, 2018
Obstructive sleep apnea in children with cystic fibrosis on highly effective modulator therapy
Aarti Shakkottai1, Stephany Kim2, Ron B Mitchell3
1Department of Pediatrics, Division of Pediatric Pulmonology and Sleep Medicine, University of Texas Southwestern Medical Center, Dallas, Texas, USA.
Insights
Obstructive sleep apnea (OSA) is common in children with cystic fibrosis (CF), even after highly effective modulator therapies (HEMT). Children on HEMT were older and more likely to have OSA, suggesting a continued need for screening.
Area of Science:
- Pediatric Pulmonology
- Sleep Medicine
- Cystic Fibrosis Research
Background:
- Obstructive sleep apnea (OSA) is a known complication in pediatric cystic fibrosis (CF) patients.
- Highly effective modulator therapies (HEMT) have improved CF outcomes, but their impact on OSA frequency is unclear.
Purpose of the Study:
- To determine the frequency of OSA in children with CF in the post-HEMT era.
- To assess the association between HEMT use and OSA in this population.
Main Methods:
- Retrospective review of polysomnography (PSG) data from 49 children (aged 0-18 years) with CF between 2012-2023.
- Patients were categorized based on HEMT status.
- Logistic regression analysis was employed to evaluate the association between HEMT and OSA.
Main Results:
- OSA was diagnosed in 57% of the children studied.
- Children on HEMT were older and had a significantly higher odds of OSA (OR=4.3, p=0.02).
- Tonsillar hypertrophy was also independently associated with increased odds of OSA (OR=6.6, p=0.03).
Conclusions:
- OSA remains a frequent diagnosis in children with CF, even after the introduction of HEMT.
- While HEMT use was associated with older age and higher OSA likelihood, key health metrics like lung function and nutritional status were similar between groups.
- Further prospective research is warranted to fully elucidate the relationship between HEMT and OSA in pediatric CF patients.
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.
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