Recent advances in respiratory care for neuromuscular disease.
The 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.
Simonds
Anita K
AK
Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK. A.Simonds@rbht.nhs.uk
D000328:Adult; D002648:Child; D045422:Continuous Positive Airway Pressure; D018575:Home Care Services, Hospital-Based; D006801:Humans; D016472:Motor Neuron Disease / Q000401:mortality / Q000628:therapy; D020388:Muscular Dystrophy, Duchenne / Q000401:mortality / Q000628:therapy; D009468:Neuromuscular Diseases / Q000401:mortality / Q000628:therapy*; D010166:Palliative Care; D012131:Respiratory Insufficiency / Q000401:mortality / Q000628:therapy*; D016019:Survival Analysis; D016896:Treatment Outcome