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

Pediatr Pulmonol. 2021 Jun;56(6):1704-1715. doi: 10.1002/ppul.25308. Epub 2021 Mar 17.

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.

MeSH terms

  • Child
  • Continuous Positive Airway Pressure
  • Down Syndrome* / complications
  • Down Syndrome* / therapy
  • Humans
  • Noninvasive Ventilation*
  • Retrospective Studies
  • Sleep Apnea, Obstructive* / therapy