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Amyotrophic lateral sclerosis is a progressive neuromuscular disease characterized by both lower motor neuron and upper motor neuron dysfunction. While clinical presentations can vary, there is no cure for ALS and the disease is universally terminal, with most patients dying from respiratory complications. Patients die on average within 3-5 years of diagnosis, unless they choose to undergo tracheostomy, in which case, they may live on average 2 more years. Up to 95% with ALS in the United States choose not to undergo tracheostomy, so management of respiratory failure is aimed at both prolonging survival as well as improving quality of life. Standard of care for ALS patients includes treatment from multi-disciplinary teams, but many patients do not have regular access to a pulmonary physician who regularly sees patients with this disease. This review aims to serve as an overview of respiratory considerations in the management of ALS. This includes non-invasive ventilation in the management of respiratory muscle weakness, mechanical insufflation/exsufflation devices for airway clearance, treatment of aspiration including timing of PEG tube, as well as secretion management. In addition, it is important for physicians to consider end of life issues including advanced directives, hospice referral, and ventilator withdrawal.
PMID: 29990478 [PubMed - as supplied by publisher]