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Home Noninvasive Ventilation in Pediatric Subjects With Neuromuscular Diseases: One Size Fits All

Mathis Steindor¹, Carolin E Wagner², Claudia Bock²

¹Pediatric Pulmonology and Sleep Medicine, Children's Hospital, University of Duisburg-Essen, Essen, Germany. mathis.steindor@uk-essen.de.

Respiratory Care

|October 7, 2020

Insights

Noninvasive ventilation (NIV) settings for pediatric patients with neuromuscular disease can be similar across age groups. Recommended settings include expiratory positive airway pressure of 4-5 cm H2O and inspiratory pressure delta of 8-10 cm H2O.

Area of Science:

Pediatric Pulmonology
Respiratory Medicine
Neuromuscular Diseases

Background:

Home noninvasive ventilation (NIV) is crucial for managing respiratory insufficiency in pediatric patients with neuromuscular diseases.
Optimal ventilator settings for this population are not well-established.

Purpose of the Study:

To determine appropriate ventilator settings for pediatric patients with neuromuscular disease receiving home NIV.
To compare ventilator settings across different pediatric age groups.

Main Methods:

Retrospective analysis of ventilator settings in 128 pediatric patients (0-17 years) with neuromuscular disease using NIV.
Comparison of settings across four age groups: <1, 0-5, 6-11, and 12-17 years.
Correlation analysis of ventilator settings with patient age and forced vital capacity (FVC).

Main Results:

Ventilator backup rate significantly decreased with age.
Expiratory positive airway pressure (EPAP) and inspiratory positive airway pressure (IPAP) showed no significant differences across age groups.
IPAP increased with age and decreasing FVC; backup rates increased with decreasing FVC.

Conclusions:

A standardized approach to NIV settings is feasible for pediatric patients with neuromuscular disease.
Proposed starting NIV settings: EPAP 4-5 cm H2O, IPAP delta 8-10 cm H2O, and age-adjusted backup rate.
Higher IPAP and backup rates may be needed for patients with advanced disease stages.

Read full analysis

Abstract

Background:

Home noninvasive ventilation (NIV) improves disease courses of patients with respiratory insufficiency due to neuromuscular diseases. Data about appropriate ventilator settings for pediatric patients are missing.

Methods:

In this retrospective study, ventilator settings of 128 subjects with neuromuscular disease aged 0-17 y with NIV were compared between 4 age groups (< 1 y, 0-5 y, 6-11 y, and 12-17 y). Additionally, correlations of ventilator settings with age and vital capacity were investigated in an ungrouped approach.

Results:

Ventilator backup rate decreased significantly with age, leading to significant backup rate differences between all groups except the oldest two. Median (interquartile range) backup rates were 36 (11.5), 24 (4), 20 (4), and 20 (3) breaths/min in groups 1-4, respectively. Median [IQR] expiratory positive airway pressures (4 [0.5], 4 [0], 4 [0], 4 [1] cm H₂O, respectively) and median [IQR] inspiratory positive airway pressures (12 [1.5], 12 [5], 12 [2.3], and 14 [4] cm H₂O, respectively) showed no significant differences. However, correlation analyses indicated an increase of inspiratory positive airway pressure with age and decreasing FVC, as well as an increase of backup rates with decreasing FVC.

Conclusions:

Similar NIV settings fit all age groups of pediatric subjects with neuromuscular disease. Thus, we propose an expiratory positive airway pressure of 4-5 cm H₂O, an inspiratory pressure delta of 8-10 cm H₂O, and an age-oriented backup rate as a starting point for NIV titration. Patients with advanced disease stages might require slightly higher inspiratory positive airway pressures and backup rates.

Keywords:

Duchenne muscular dystrophy chronic respiratory insufficiency home ventilation neuromuscular disease spinal muscular atrophy

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