How to manage exacerbation of respiratory failure in a patient diagnosed with amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis (ALS) is a debilitating neurodegenerative disease of unclear pathogenesis, which leads to the damage of motor neurons. The majority of patients survive two to five years after a diagnosis is made. Despite numerous clinical trials and some progress in understanding ALS pathology, no treatment that effectively inhibits the disease progression has been developed. The aim of this paper is to review a patient’s disease history from diagnosis, through the occurrence of respiratory failure features, until the start of non-invasive ventilation (NIV), including the assessment of the efficacy of this therapy during respiratory failure exacerbation. ALS is an incurable disease, but many of its symptoms may be relieved, and therapy should be aimed at quality of life improvement and maintaining the patient’s independence and comfort as long as possible. Better quality of life and longer survival are currently well-established advantages of NIV. Palliat Med Pract 2020; 14, 1: 44–50Amyotrophic lateral sclerosis (ALS) is a debilitating neurodegenerative disease of unclear pathogenesis, which leads to the damage of motor neurons. The majority of patients survive two to five years after a diagnosis is made. Despite numerous clinical trials and some progress in understanding ALS pathology, no treatment that effectively inhibits the disease progression has been developed. The aim of this paper is to review a patient’s disease history from diagnosis, through the occurrence of respiratory failure features, until the start of non-invasive ventilation (NIV), including the assessment of the efficacy of this therapy during respiratory failure exacerbation. ALS is an incurable disease, but many of its symptoms may be relieved, and therapy should be aimed at quality of life improvement and maintaining the patient’s independence and comfort as long as possible. Better quality of life and longer survival are currently well-established advantages of NIV. Palliat Med Pract 2020; 14, 1: 44–5