Noninvasive ventilation in neuromuscular disease: equipment and application

Noninvasive support of ventilation is commonly needed in patients with neuromuscular disease. Body ventilators, which are used rarely, function by applying intermittent negative pressure to the thorax or abdomen. More commonly, noninvasive positive-pressure ventilation (NPPV) is used. This therapy can be applied with a variety of interfaces, ventilators, and ventilator settings. The patient interface has a major impact on comfort during NPPV. The most commonly used interfaces are nasal masks and oronasal masks. Other interfaces include nasal pillows, total face masks, helmets, and mouthpieces. Theoretically, any ventilator can be attached to a mask rather than an artificial airway. Portable pressure ventilators (bi-level positive airway pressure) are available specifically to provide NPPV and are commonly used to provide this therapy. Selection of NPPV settings in patients with neuromuscular disease is often done empirically and is symptom-based. Selection of settings can also be based on the results of physiologic studies or sleep studies. The use of NPPV in this patient population is likely to expand, particularly with increasing evidence that it is life-prolonging in patients with diseases such as amyotrophic lateral sclerosis. Appropriate selection of equipment and settings for NPPV is paramount to the success of this therapy.