Noninvasive ventilation in neuromuscular disease

The treatment of respiratory failure in patients who have NMD continues to be an evolving process. Negative-pressure ventilation, once prominent in the 1940s and 1950s, gave way to intermittent positive-pressure ventilation with tracheostomy or endotracheal tubes in the 1960s. Now there is a resurgence of noninvasive ventilation, brought about by innovative modes of positive pressure delivered through nasal and facial masks. Although frequently relegated to second-line choices, negative-pressure devices still offer a practical treatment alternative as patient preference still plays a role in selecting a proper mode of ventilation. Studies have shown that noninvasive ventilation can prevent or reverse respiratory failure and improve quality of life and longevity. Despite the seemingly widespread acceptance of noninvasive ventilation in the treatment of respiratory failure, physicians still appear reluctant to use ventilatory assistance in the neuromuscular arena. In 1985, a survey found that respiratory support systems were utilized routinely in only 33% of the 132 responding Muscular Dystrophy Association (MDA) clinics. Bach recently surveyed 273 MDA clinic directors and co-directors from 167 clinics, to evaluate their current use of mechanical ventilation. Ventilatory assistance was recommended and used electively in only 43 (26%) of the 167 clinics. Furthermore, it was the policy in 68 of the clinics to discourage the use of mechanical ventilation. Even more importantly, only 2 physicians who discouraged the use of mechanical ventilation were familiar with the newest noninvasive methods of ventilatory support. Sadly, although our methodologies in the treatment of respiratory failure continue to improve, physician practice has lagged behind. Physicians who treat patients who have NMD need to become cognizant of these new techniques and incorporate them into their present therapeutic armamentarium.