Ventilatory Management Issues in Acute Respiratory Failure Among Patients with Amyotrophic Lateral Sclerosis

Introduction: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease leading to impairment of the respiratory muscles, which causes respiratory failure, the primary cause of death in these patients. Management of acute respiratory distress in ALS patients is particularly challenging due to the absence of a curative treatment. Invasive ventilation may prolong survival but does not alter the inevitable course of the disease. Case Reports: We report two cases of patients with ALS admitted to the emergency department for acute respiratory failure. The first patient, a 36-year-old male, developed acute respiratory distress secondary to aspiration. Upon admission, his SpO₂ was below 70%, blood pressure 110/70 mmHg, heart rate 110 bpm, respiratory rate 30 breaths/min, with diffuse bronchial crackles on pulmonary auscultation. He underwent intubation followed by invasive mechanical ventilation and ultimately required tracheostomy due to ventilator dependency. His condition deteriorated, and he died three months after hospitalization. The second patient, aged 42, developed Legionella pneumonia complicated by acute respiratory failure. He presented with SpO₂ below 80%, blood pressure 110/60 mmHg, heart rate 100 bpm, respiratory rate 26 breaths/min, and bronchial congestion. He also required intubation followed by tracheostomy. Unlike the first case, he remains alive under assisted ventilation but is in a state of complete dependency. Conclusion: These cases illustrate the complexity of emergency care in ALS patients with acute respiratory failure. Although intubation and invasive ventilation are sometimes unavoidable, they often result in prolonged dependency, raising questions about their benefit-risk ratio. In countries where guidelines exist, decisions are made in accordance with the patient's advance directives and through multidisciplinary deliberation. However, in our context, these principles are rarely followed, leading to often improvised emergency therapeutic decisions. Close collaboration between emergency physicians and neurologists is essential to optimize management and avoid prolonged invasive interventions that may not align with the patient’s prognosis. Keywords: Amyotrophic lateral sclerosis, respiratory distress, emergency care, invasive ventilation, medical ethics.