Management of respiratory symptoms in ALS.
Hardiman, Orla
Hardiman, Orla
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Date
2012-02-01T10:02:08Z
Date Submitted
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Subject Mesh
Amyotrophic Lateral Sclerosis/*complications/physiopathology/*therapy
Animals
Disease Management
Humans
Palliative Care/methods
Respiration, Artificial/methods
Respiratory Insufficiency/etiology/*physiopathology/*therapy
Respiratory Mechanics/physiology
Respiratory Muscles/physiopathology
Animals
Disease Management
Humans
Palliative Care/methods
Respiration, Artificial/methods
Respiratory Insufficiency/etiology/*physiopathology/*therapy
Respiratory Mechanics/physiology
Respiratory Muscles/physiopathology
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Abstract
Respiratory insufficiency is a frequent feature of ALS and is present in almost all cases at some stage of the illness. It is the commonest cause of death in ALS. FVC is used as important endpoint in many clinical trials, and in decision-making events for patients with ALS, although there are limitations to its predictive utility. There are multiple causes of respiratory muscle failure, all of which act to produce a progressive decline in pulmonary function. Diaphragmatic fatigue and weakness, coupled with respiratory muscle weakness, lead to reduced lung compliance and atelectasis. Increased secretions increase the risk of aspiration pneumonia, which further compromises respiratory function. Bulbar dysfunction can lead to nutritional deficiency, which in turn increases the fatigue of respiratory muscles. Early recognition of respiratory decline and symptomatic intervention, including non-invasive ventilation can significantly enhance both quality of life and life expectancy in ALS. Patients with respiratory failure should be advised to consider an advance directive to avoid emergency mechanical ventilation.
Language
eng
ISSN
1432-1459 (Electronic)
0340-5354 (Linking)
0340-5354 (Linking)
eISSN
ISBN
DOI
10.1007/s00415-010-5830-y
PMID
21082322