ABSTRACT
Many progressive neuromuscular disorders involve the muscles of the respiratory system,
and respiratory insufficiency is a major cause of morbidity and mortality. Thus ventilatory
assistance becomes an important part of disease management for patients with advanced
neuromuscular disease. The following discusses the evaluation of patients with neuromuscular
disease to characterize the nature and severity of respiratory muscle involvement,
indications and contraindications for the use of noninvasive ventilation, and techniques
for initiation and monitoring. Indications for invasive ventilation are also discussed,
and major principles of management and monitoring of invasive mechanical ventilation
are outlined. For optimal ventilator management of patients with neuromuscular disease,
clinicians must be familiar with the principles discussed in this article so that
treatment can be tailored to the individual's goals and desires. With proper implementation,
ventilatory support of patients with neuromuscular disease can bring about improvements
in quality of life and, for those who desire, potential prolongation of survival.
KEYWORDS
Noninvasive ventilation - neuromuscular disease - tracheostomy - mechanical ventilation
