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aALS Research Center, Dipartimento Universitario di Neuroscienze Cliniche, Università di Palermo, Palermo, Italy bPulmonary and Respiratory Intensive Care Unit, Ospedale Civico, Palermo, Italy
danielelococo{at}yahoo.com
Abstract
ObjectiveTo determine whether the Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS), a validated instrument that assesses the functional status and the disease progression in ALS, predicts hospital length of stay and survival time in ALS patients treated with tracheostomy-intermittent positive-pressure ventilation (TIPPV).
MethodsThirty-three consecutive ALS patients with acute respiratory failure who underwent TIPPV were prospectively followed from their admission to the hospital until death. The association of ALSFRS score at hospital admission with length of hospital stay, and survival after TIPPV were examined using Cox proportional hazard models, adjusting for age at baseline, sex, and symptom duration.
ResultsThe median ALSFRS score of the ALS patients at hospital admission was 11 (range: 4 to 22). The median length of hospital stay was 55 days (range: 7 to 124 days), with a hospital mortality rate of 9%. For the 30 patients (91%) discharged from hospital, median survival time was 37 months (range: 2 to 64 months). The total ALSFRS score (above or below the median score) was a significant predictor of length of hospital stay (hazard ratio [HR]: 2.86, 95% CI: 1.2 to 6.5, p = 0.003) and survival after TIPPV (HR: 3.76, 95% CI: 1.4 to 9.7, p = 0.002). The total ALSFRS score at hospital admission was also associated with length of hospital stay (HR: 2.1, 95% CI: 1.1 to 5.1, p = 0.005), and survival (HR: 0.52, 95% CI: 0.1 to 0.8, p = 0.002) when included in a Cox multivariable model together with the other demographic and clinical variables.
ConclusionIn ALS patients with acute respiratory failure treated with TIPPV, the total ALSFRS score may predict length of hospital stay and long-term survival after invasive mechanical ventilation.
Key Words: Amyotrophic lateral sclerosis respiratory failure tracheostomy mechanical ventilation ALSFRS survival
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