Recent Advances in Respiratory Care for Neuromuscular Disease

Learning Objectives1. Noninvasive ventilation (NIV) allows patients with nonprogressive neuromuscular disease to live a near-normal life expectancy, extends survival by many years in other conditions, and can palliate symptoms in patients with rapidly progressive diseases2. NIV works in part by enhancing chemosensitivity, and in many neuromuscular conditions the most effective time to introduce NIV is when symptomatic sleep-disordered breathing developsThe impact of ventilatory support on the natural history of neuromuscular disease (NMD) has become clearer over the last 2 decades as techniques have been more widely applied. Noninvasive ventilation (NIV) allows some patients with nonprogressive pathology to live to nearly normal life expectancy, extends survival by many years in patients with other conditions (eg, Duchenne muscular dystrophy), and in those patients with rapidly deteriorating disease (eg, amyotrophic lateral sclerosis) survival may be increased, but symptoms can be palliated even if mortality is not reduced. A growing number of children with NMD are surviving to adulthood with the aid of ventilatory support. The combination of NIV with cough-assist techniques decreases pulmonary morbidity and hospital admissions. Trials have confirmed that NIV works in part by enhancing chemosensitivity, and in patients with many different neuromuscular conditions the most effective time to introduce NIV is when symptomatic sleep-disordered breathing develops.