Respiratory Failure or Impairment in Amyotrophic Lateral Sclerosis

Respiratory complications account for the majority of deaths occurring in patients suffering from amyotrophic lateral sclerosis (ALS). Patients normally succumb to their illness within an average of 3 to 5 years from the time of diagnosis from complications such as hypoventilation, hypoxemia, hypercarbia, aspiration, and other pneumonia and pulmonary emboli. Although invariably disabling, ALS need not be fatal if respiratory involvement is detected early, which will allow sufficient time to discuss and implement treatment options. The recently published American Academy of Neurology guidelines for the management of ALS recommends the following: Serial measures of pulmonary function to guide management and determine prognosis. Noninvasive ventilatory support--an effective initial therapy for symptomatic chronic hypoventilation and prolonged survival. Invasive ventilatory support when long-term survival is the goal and noninvasive support is no longer sufficient. Physicians respect the right of the patient to choose, refuse, or withdraw ventilatory support. Liberal use of opiates and anxiolytics to relieve dyspnea and anxiety when ventilatory support is refused or withdrawn.