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Efficacy and adherence of noninvasive ventilation treatment in children with Down syndrome

Lauren MacDonagh¹, Lisa Farrell², Ruth O'Reilly²

¹School of Medicine, Department of Health Sciences, University College Dublin, Belfield, Dublin, Ireland.

Pediatric Pulmonology

|March 17, 2021

Insights

Children with Down syndrome (DS) show good adherence to noninvasive ventilation (NIV) for obstructive sleep apnea (OSA). However, increased system leak may reduce treatment effectiveness in this population.

Area of Science:

Pediatric Pulmonology
Sleep Medicine
Genetics

Background:

Children with Down syndrome (DS) exhibit a higher incidence of obstructive sleep apnea (OSA).
Noninvasive ventilation (NIV) is a primary treatment for OSA in children with DS.
Understanding NIV adherence and efficacy in this population is crucial.

Purpose of the Study:

To evaluate the adherence and efficiency of NIV delivery in children with DS.
To compare NIV outcomes between children with DS and those without DS.
To identify factors influencing NIV effectiveness in children with DS.

Main Methods:

A retrospective cohort study of 106 children with confirmed OSA using home NIV.
Patients were categorized into DS (n=44) and non-DS (n=62) groups.
Data analyzed included adherence, apnea-hypopnoea index (AHI), positive airway pressure delivery, and system leakage.

Main Results:

The DS cohort demonstrated significantly higher NIV usage compared to the non-DS cohort (78.95% vs. 72.11%).
Despite greater usage, the DS cohort experienced poorer clinical outcomes with a higher AHI (p=.0493).
Excessive system leakage was significantly greater in the DS cohort (41.00 L/min vs. 36.52 L/min, p=.022).
Children with DS and prior cardiac surgery showed reduced compliance across all parameters.

Conclusions:

Satisfactory adherence to NIV is achievable in children with DS.
Excessive system leak at the machine-patient interface is a significant challenge.
Addressing system leak is essential to optimize NIV efficacy in children with DS.

Read full analysis

Abstract

Objective:

Children with Down syndrome (DS) have an increased prevalence of obstructive sleep apnea (OSA). Noninvasive ventilation (NIV) is a common modality of OSA treatment in this cohort. This study aimed to measure adherence and efficiency of NIV delivery in children with DS.

Study Design:

This was a retrospective cohort study involving 106 children with confirmed OSA and home NIV with downloadable data capacity. Children were divided into DS (n = 44) and non-DS cohorts (n = 62). Adherence, clinical outcomes apnea-hypopnoea index (AHI), positive airway pressure delivery, and leakage were recorded and compared between DS and non-DS cohorts and within the DS cohort based on past surgical history.

Results:

Significantly greater NIV usage was observed in the DS cohort, they showed more consistent use with an increased percentage of days used relative to their non-DS counterparts (78.95 ± 2.26 vs. 72.11 ± 2.14, p = .031). However, despite greater usage, poorer clinical outcomes in the form of increased AHI (p = .0493) was observed in the DS cohort, where significantly greater leakage was also shown 41.00 ± 1.61 L/min versus 36.52 ± 1.18 L/min (p = .022). Twenty children with DS had prior cardiac surgery; compliance across all parameters was significantly reduced relative to those without.

Conclusion:

These data confirm that satisfactory NIV adherence is achievable in children with DS. However, we have identified excessive system leak at the machine-patient interface as a factor, which could undermine NIV efficacy in children with DS.

Keywords:

Down syndrome adherence leakage noninvasive ventilation obstructive sleep apnea

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