Use of Non-invasive Ventilation in Neuromuscular Disease

Purpose of Review: The purpose of this review is to add to the growing body of literature on non-invasive ventilation (NIV) for patients with neuromuscular disease (NMD). We synthesize information on screening for respiratory failure, describe protocols for initiating and troubleshooting NIV in NMD patients, discuss the use of advanced modes of ventilation such as volume-assured pressure support (VAPS), and provide recommendations for long-term follow-up and management. Recent Findings: Screening of respiratory insufficiency increases by testing supine FVC, MIP, nocturnal oximetry, and a higher threshold for FVC. PSG may not be needed in all patients starting NIV. VAPS mode can increase patient comfort and compliance. Sialorrhea management is essential to successful NIV, and myriad options exist. Summary: Utilization of NIV will increase as the NMD population increases. Clinicians must be comfortable with screening and diagnosing chronic respiratory failure, initiating NIV, and the long-term follow-up of these patients.