Pulmonary complications of chronic neuromuscular diseases and their management

Chronic neuromuscular diseases may affect all major respiratory muscles groups including inspiratory, expiratory, and bulbar, and respiratory complications are the major cause of morbidity and mortality. Untreated, many of these diseases lead inexorably to hypercapnic respiratory failure, precipitated in some cases by chronic aspiration and secretion retention or pneumonia, related to impairment of cough and swallowing mechanisms. Many measures are helpful including inhibition of salivation, cough-assist techniques, devices to enhance communication, and physical therapy. In addition, ventilatory assistance is an important part of disease management for patients with advanced neuromuscular disease. Because of its comfort, convenience, and portability advantages, noninvasive positive pressure ventilation (NPPV) has become the modality of first choice for most patients. Patients to receive NPPV should be selected using consensus guidelines, and initiation should be gradual to maximize the chances for success. Attention should be paid to individual preferences for interfaces and early identification of cough impairment that necessitates the use of cough-assist devices. For patients considered unsuitable for noninvasive ventilation, invasive mechanical ventilation should be considered, but only after a frank but compassionate discussion between the patient, family, physician, and other caregivers.