ObjectivesThe present study aims to evaluate the efficacy and complications of combined noninvasive ventilation (NIV) and assisted coughing by mechanical in-exsufflator (MIE) for acute respiratory failure (ARF) in children with neuromuscular disease (NMD). MethodsA prospective study was conducted in the pediatric intensive care unit. Children with NMD and ARF treated by combined NIV and MIE were included. Treatment success was defined as freedom from tracheal intubation during the hospital stay. Physiologic indices including PaO2, PaCO2, pH, and PaO2/FiO(2) were recorded before and 12, 24hr after the use of NIV/MIE. ResultsCombined NIV/MIE was used in 15 NMD children (mean: 8.1 years, range: 3 months to 18 years) with 16 cases of ARF. There was no mortality in this cohort. Treatment success was achieved in 12 cases (75%), including six cases (38%) demanding Do Not Intubate. ARF was due to pneumonia, with a mean baseline PaCO2 of 73.219.0mmHg. In the success group, hypercarbia and acidosis improved after use of NIV/MIE for 24hr (PaCO2: 71.7 +/- 18.6mmHg vs. 55.8 +/- 11.6mmHg, P<0.01; pH: 7.29 +/- 0.07 vs. 7.38 +/- 0.05, P<0.01). All patients tolerated NIV/MIE well despite transient skin pressure sores in five cases. ConclusionsCombined NIV/MIE is a safe and effective approach to rapidly improve physiologic indices and decrease the need for intubation in NMD children with ARF. NIV/MIE provides a good alternative for those refusing intubation. Pediatr Pulmonol. 2014; 49:589-596. (c) 2013 Wiley Periodicals, Inc.