Respiratory management of adult patients with progressive neuromuscular disease: Non-invasive ventilation and the role of the Intensivist

Non-invasive positive pressure ventilation (NIV) is now widely used for a variety of chronic neuromuscular diseases, and available evidence demonstrates improved quality and quantity of life. Clinicians should be aware of the natural history of each neuromuscular disorder in order to make informed decisions about life-prolonging therapies in the intensive care setting. Attention to co-morbidities and reversible causes of exacerbations of respiratory failure are important given that these are often treatable. Nutritional factors play an important role in patients receiving NIV. End of life planning should be discussed with all patients requiring non-invasive ventilation.