Noninvasive Ventilation in Amyotrophic Lateral Sclerosis

• Published in: Sleep medicine clinics Vol. 15; no. 4; p. 527
• Main Authors: Cooksey, Jessica A, Sergew, Amen
• Format: Journal Article
• Language: English
• Published: United States 01.12.2020
• Subjects: Amyotrophic Lateral Sclerosis - complications; Amyotrophic Lateral Sclerosis - therapy; Humans; Noninvasive Ventilation - standards; Respiratory Insufficiency - etiology; Respiratory Insufficiency - therapy; Bulbar ALS; Amyotrophic lateral sclerosis; Noninvasive ventilation; Noninvasive positive pressure ventilation
• ISSN: 1556-4088
• DOI: 10.1016/j.jsmc.2020.08.004

Amyotrophic lateral sclerosis is a progressive neurodegenerative disease involving upper and lower motor neurons and has limited treatment options. The weakness progresses to involve the diaphragms, resulting in respiratory failure and death. Home noninvasive ventilation has been shown to improve survival and quality of life, especially in those with intact bulbar function. Once initiated, close monitoring with nocturnal oximetry, remote downloads from the home noninvasive ventilation machine, and measurement of serum bicarbonate should be conducted. Additionally, transcutaneous CO2 monitoring can be considered if available. This article discusses the indications, timing, initiation, and management of noninvasive ventilation in amyotrophic lateral sclerosis.