ABSTRACT
The purpose of this article is to describe noninvasive respiratory management for patients with neuromuscular respiratory muscle dysfunction [NMD] and spinal cord injury [SCI] and the role of electrophrenic pacing [EPP] and diaphragm pacing [DP] in this respect. Long term outcomes will be reviewed and the use of noninvasive intermittent positive pressure ventilation [NIV], MAC, and EPP/DP to prevent pneumonia and acute respiratory failure, to facilitate extubation, and to avoid tracheotomy will be evaluated. Although ventilator dependent patients with most NMDs and high level SCI can be indefinitely managed noninvasively, most ALS patients can be managed for a limited time by continuous NIV before tracheostomy is necessary for survival. Glossopharyngeal breathing [GPB] can be learned by patients without any autonomous breathing ability and used by them in the event of ventilator/EPP/DP failure or loss of interface access. EPP/DP can maintain alveolar ventilation for high level SCI patients when they cannot grab a mouth piece to use NIV