TY - JOUR T1 - Home Noninvasive Ventilation in Pediatric Subjects With Neuromuscular Diseases: One Size Fits All JF - Respiratory Care SP - 410 LP - 415 DO - 10.4187/respcare.08079 VL - 66 IS - 3 AU - Mathis Steindor AU - Carolin E Wagner AU - Claudia Bock AU - Maximilian Eckerland AU - Laura Heitschmidt AU - Leopold Pichlmaier AU - Margerete Olivier AU - Anastasios Bouikidis AU - Joerg Grosse-Onnebrink AU - Uwe Mellies AU - Florian Stehling Y1 - 2021/03/01 UR - http://rc.rcjournal.com/content/66/3/410.abstract N2 - 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. ER -