PT  - JOURNAL ARTICLE
AU  - Steindor, Mathis
AU  - Wagner, Carolin E
AU  - Bock, Claudia
AU  - Eckerland, Maximilian
AU  - Heitschmidt, Laura
AU  - Pichlmaier, Leopold
AU  - Olivier, Margerete
AU  - Bouikidis, Anastasios
AU  - Grosse-Onnebrink, Joerg
AU  - Mellies, Uwe
AU  - Stehling, Florian
TI  - Home Noninvasive Ventilation in Pediatric Subjects With Neuromuscular Diseases: One Size Fits All
AID  - 10.4187/respcare.08079
DP  - 2021 Mar 01
TA  - Respiratory Care
PG  - 410--415
VI  - 66
IP  - 3
4099  - http://rc.rcjournal.com/content/66/3/410.short
4100  - http://rc.rcjournal.com/content/66/3/410.full
AB  - 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.