20 Use of Noninvasive Positive Pressure Ventilation to Facilitate Extubation in Pediatric Patients with Neuromuscular Diseases.

Abstract

Introduction: Patients with neuromuscular diseases (NMDs) who require intubation are at risk of extubation failure due to respiratory muscle weakness and they may require tracheostomy. Purpose: To evaluate the use of noninvasive positive pressure ventilation (NPPV) with the BiPAP® (Respironics) device to facilitate extubation of children with NMDs and poor baseline pulmonary function who have been intubated for acute respiratory failure or surgery. Methods: Retrospective chart review, IRB approved. Results: During the time period Jan 2001–Dec 2004, NPPV was used to facilitate extubation for 22 episodes of intubation in 14 patients. Diagnoses: Duchenne muscular dystrophy (Duchenne MD) (7 patients); type 1 spinal muscular atrophy (2 patients); Becker MD (1 patient); congenital MD (1 patient); nemaline myopathy (1 patient); polymyositis (1 patient); acid maltase deficiency (1 patient). Mean age at time of extubation (± SD): 10.7+ 5.9 years (range: 1–20 years); 11 of 14 patients were male. Four of 22 episodes of extubation were after spinal surgery. In these patients, mean baseline vital capacity was 47.3+ 11.4% predicted. In 18 episodes, extubation was after acute respiratory illness with respiratory failure. Mean baseline vital capacity for these patients: 24.5+ 9.1% predicted (range: 11–38%) (n=10, excludes spinal muscular atrophy infants). NPPV was used successfully to facilitate extubation in all 4 episodes of post-surgical extubaton. NPPV was successful in 17 of 18 episodes of extubation during acute respiratory illnesses (94% success rate). At follow-up (mean 27.5+ 15.2 months after extubation), 2 patients have died (1 patient had Duchenne MD and refused chronic NPPV, 1 patient had acid maltase deficiency). The remaining 12 patients are alive, 9 of whom have no tracheostomy and are using chronic home NPPV. The mean vital capacity at follow-up in these patients is 27.5+ 14.2% predicted. Two patients are now ventilated with tracheostomy tubes. Conclusion: Patients with severe neuromuscular diseases who have been intubated are at risk for extubation failure and the need for tracheostomy. Our study suggests that, despite highly impaired pulmonary function, NPPV can be used to extubate many of these patients successfully. On long-term follow-up, most of our patients have avoided tracheostomy. Further study is needed to optimize the use of NPPV for patients with NMDs.