Comparison of non-invasive ventilation use and outcomes in children with Down syndrome and other children using this technology
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Published on: May 26, 2023
Rafiaa Valji1, Maria L Castro-Codesal1, Melanie Lewis2,3
1Divisions of Respiratory Medicine, Department of Pediatrics, Faculty of Medicine and Dentistry, University of Alberta, Edmonton, AB, Canada.
Insights
Long-term non-invasive ventilation (LT-NIV) is effective for children with Down syndrome (DS), primarily for upper airway obstruction. Despite lower adherence, most children with DS successfully use LT-NIV.
Area of Science:
- Pediatric Pulmonology
- Sleep Medicine
- Genetics
Background:
- Children with Down syndrome (DS) are significant users of long-term non-invasive ventilation (LT-NIV).
- Unique physiological characteristics in DS may impact LT-NIV efficacy and usage patterns.
- Understanding these differences is crucial for optimizing respiratory support in this population.
Purpose of the Study:
- To compare the utilization and outcomes of LT-NIV in pediatric patients with DS versus a matched comparison group (MCG).
- To investigate the efficacy and adherence patterns of LT-NIV in children with DS.
- To identify specific indications and treatment modalities for LT-NIV in children with DS.
Main Methods:
- A retrospective review of 622 children initiated on LT-NIV in Alberta, Canada.
- 106 children with DS were matched 1:2 with MCG based on age and therapy start date.
- Data extracted from medical and sleep laboratory records.
Main Results:
- Upper airway disease was the primary indication for LT-NIV, significantly higher in the DS group (90% vs. 50%).
- Continuous positive airway pressure (CPAP) was more frequently used in children with DS (93.3% vs. 69.2%).
- Adherence was lower in the DS group, yet a majority used LT-NIV for over 4 hours nightly; discontinuation and mortality rates did not differ.
Conclusions:
- LT-NIV is an effective treatment for children with DS, particularly for upper airway obstruction.
- While adherence challenges exist, most children with DS achieve successful LT-NIV use.
- Further research may explore strategies to improve adherence in this population.
Rationale:
Children with Down syndrome (DS) make up a substantial portion of long-term non-invasive ventilation (LT-NIV) users though it is unclear if their unique features alter LT-NIV efficacy or use. The aim of this study is to compare the use and outcomes of LT-NIV for children with DS and a matched comparison (MCG).
Methods:
This is a sub-study of a 10-year retrospective review of children initiated on LT-NIV in Alberta, Canada (N = 622). Children with DS (n = 106) were matched in a 1:2 ratio with other children using LT-NIV based on age and therapy start date. Data was collected from medical and sleep laboratory records.
Results:
Upper airway disease was the most common indication for LT-NIV in both groups, though was higher in children with DS (DS: 90% vs. MCG: 50%, OR 8.64 [95% CI 4.38-17.04]). Sleep and respiratory parameters, at the baseline diagnostic sleep study and the change from baseline to treatment study, did not differ between groups. Nasal masks were the predominant mask type in both children with DS (55%) and the MCG (66%) with more children with DS, compared to the MCG, using full face masks (DS: 45 vs. MCG: 33%, p < 0.05). Continuous positive airway pressure was used more often in children with DS (93.3% vs. 69.2%, p < 0.001) while bilevel-positive airway pressure was more common in the MCG (DS: 6.7% vs. MCG 30.8%, p < 0.001). Children with DS were followed longer than children in the MCG (DS: 2.4 [IQR 2.8] vs. MCG: 1.8 [IQR 2.7] years, p < 0.05). Adherence was lower in children with DS at both 6-12 month follow-up and most recent visit with a similar decrease in adherence in both groups over the follow-up period (0.0 [IQR 1.4] vs. -0.3 [IQR 2.0]. Despite this, 66% and 49% of children with DS used LT-NIV for more than 4 h/night at the 6-12 month and most current visit, respectively. Discontinuation of LT-NIV and mortality did not differ between groups.
Conclusion:
LT-NIV is a common and efficacious treatment in children with DS used predominantly for upper airway obstruction. While adherence is lower, the majority of children with DS are successful at using LT-NIV.
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