Non-invasive Ventilation in NeuroMuscular Diseases

Neuromuscular diseases result in respiratory failure due to insufficient ventilation from respiratory muscle weakness. In patients without bulbar affection, non-invasive ventilation (NIV) can be used as an alternative to ventilation, usually via tracheostomy. When used in conjunction with cough assist methods and techniques to sustain and improve clearance of respiratory secretions, NIV can be used both day and night. Mouthpiece ventilation (MPV), also known as sips ventilation, first reported at a post-poliomyelitis respiratory equipment conference in 1953 has also enabled better quality of life. When initiated prior to development of hypercapnia in early myasthenia crisis, NIV decreases the rate of intubation and shortens the duration of hospitalization. Choice of NIV for chronic progressive NMDs must be based on the knowledge and assessment of the ventilatory abnormality for that specific diagnosis.