Home mechanical ventilation for patients with Amyotrophic Lateral Sclerosis: A Canadian Thoracic Society clinical practice guideline

Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative disorder involving both upper and lower motor neurons that results in progressive weakness of skeletal muscles. Regardless of site of first onset, death usually occurs as a result of progressive respiratory muscle involvement, with 50% of patients dying within three years of symptom onset. Mechanical ventilation is becoming increasingly accepted in ALS. In Canada, noninvasive ventilation is the most common form of ventilation applied, with tracheostomy ventilation being very uncommon. The current guideline addresses respiratory muscle testing, the benefits of mechanical ventilation in ALS, timing of initiation of ventilation and modes, settings and place of initiation. It also reviews diaphragm pacing and respiratory muscle training. Finally, given the challenges involved with tracheostomy ventilation in ALS, the question of tracheostomy ventilation is addressed.