Respiratory Care of the ALS Patient

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease marked by degeneration of motor neurons in the brainstem and spinal cord. This leads to spasticity and muscle atrophy of limb muscles, respiratory muscles, and muscles required for speech and swallowing. While there is a great deal of interpatient variability in disease progression, on average it causes death in 3–5 years from the time of diagnosis. Because patients all develop respiratory muscle weakness and bulbar muscle weakness, respiratory complications are common, and ultimately respiratory failure is the leading cause of death in people with ALS. This chapter provides an overview of the pathophysiology of ALS and describes the pulmonary evaluation of people with ALS and the various respiratory interventions that can benefit people with ALS. These include non-invasive ventilation, tracheostomy, and techniques to assist with clearance of respiratory secretions.