Nasal ventilation in progressive neuromuscular disease: experience in adults and adolescents.

A nihilistic approach to ventilatory support in progressive neuromuscular disorders is no longer acceptable. Noninvasive ventilation may improve survival and quality of life in some patients. In other situations, worthwhile palliation of symptoms of nocturnal hypoventilation and dyspnoea may be achieved. Noninvasive ventilation combined with cough assistance may obviate the need for tracheostomy ventilation, or can be employed as part of a staged approach. Individual titration of ventilatory support to meet the needs of the individual and family is vital. The participation of the patient, family and carers in decision-making is crucial and advanced directives are likely to be used increasingly.