Hospitalizations due to respiratory failure in patients with Amyotrophic Lateral Sclerosis and their impact on survival: A population-based cohort study

Background: Respiratory failure, infections and aspiration pneumonia, are the main causes of morbidity and mortality in Amyotrophic Lateral Sclerosis (ALS). In a population-based cohort, we assessed (a) hospital utilization and (b) impact of hospitalization for respiratory failure on survival. Methods: All patients with incident ALS in Friuli Venezia Giulia region, Italy, from 2002 to 2009, were identified through multiple sources. Diagnosis was validated through clinical documentation review. For each patient, we extracted the records of all hospitalizations after ALS diagnosis from the regional hospitalization database. Cox proportional hazards model survival Hazard Ratio (HR), with 95 % Confidence Interval (95 % CI), was calculated. Results: Out of 262 patients, 98.1 % had at least 1 and 58.0 % ≥3 hospitalizations. Emergency admissions occurred in 77.5 % of patients and a diagnosis of respiratory failure in 55.0 %. Patients underwent a total of 885 hospitalizations. The leading diagnosis was respiratory failure (31.6 % of hospitalizations). This diagnosis occurred most frequently in emergency (45.6 %) than in elective admissions (26.4 %). The second leading diagnosis was pneumonia (14.2 %), 24.9 and 6.3 % respectively. The leading procedure was mechanical ventilation (18.4 %), performed in 29.9 % of emergency and in 12.4 % of elective admissions. After adjustment for site of onset, age and diagnostic delay, a first hospitalization for respiratory failure had a strong adverse effect on survival (HR 4.00; 95 % CI 3.00; 5.34). Conclusions: Respiratory failure, pneumonia and aspiration pneumonia were major determinants of hospitalizations and emergency admissions and often dealt with in emergency admissions. A first hospitalization for respiratory failure had a strong adverse effect on survival. Strategies to improve home management of respiratory conditions in patients with ALS and to optimize hospital care utilization are needed.