Purpose: Amyotrophic lateral sclerosis (ALS) entails a risk of acute respiratory failure (ARF). The decision to admit such patients to the intensive care unit (ICU) is difficult given the inexorable prognosis of ALS. To fuel this discussion, this study describes the ICU and post-ICU survival of ALS-related ARF.
Material and methods: Retrospective cohort analysis over 10 years (university hospital setting, ALS reference center).
Results: Of 90 patients (66 men, median age: 67 [IQR 59-71], median interval since ALS diagnosis: 26.5 months [14-53], ALSFRS-R: 19 [12-30], bulbar signs 73%), 48 were managed by noninvasive ventilation (NIV) only, 7 were already tracheotomized upon admission, 12 were tracheotomized during the ICU stay (advance care planning project), 18 were already intubated before admission, 5 received oxygen and physiotherapy only. Median ICU stay was 4 days [2-9] with 20% mortality. Median hospital stay was 10 days [5-22] with 33% mortality. The 3-month and one year mortality wer 46% and 71%. Hospital mortality was higher in patients with more severe respiratory acidosis and higher simplified acute physiology scores on admission.
Conclusions: The prognosis of ALS-related ARF requiring ICU admission resembles that of ARF complicating other conditions with high short-term mortality (e.g. lung cancer).
Keywords: Acute respiratory failure; Amyotrophic lateral sclerosis; Chronic respiratory failure; Intensive care unit.
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