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Keywords:

  • amyotrophic lateral sclerosis;
  • mechanical insufflation-exsufflation;
  • noninvasive ventilation;
  • practical guide;
  • respiratory therapy

Abstract

Respiratory complications are a common cause of morbidity and mortality in amyotrophic lateral sclerosis (ALS). Treatment of respiratory insufficiency with noninvasive ventilation (NIV) improves ALS patients' quality of life and survival. Evidence-based practice guidelines for the management of ALS patients recommend treatment of respiratory insufficiency with NIV as well as consideration of insufflation/exsufflation to improve clearance of airway secretions. Despite these recommendations respiratory therapies remain underused. In this review we provide a practical guide for the clinician to prescribe and manage respiratory therapies for the patient with ALS. Muscle Nerve 46: 313–331, 2012