Abstract
Introduction: Amyotrophic lateral sclerosis (ALS) is a degenerative disease of motor neurons resulting in progressive weakness of striated muscles. Respiratory failure is the most common cause of death.
Aims and objectives: To present outcomes and perspectives of long term mechanical ventilation (LTMV) in ALS patients.
Methods: Data are based on a national register for the period 2002-2014. All units starting treatment were included. Prevalence and survival of ALS with LTMV was assessed using log rank test and cox regression analysis. Steady-state of patient population was estimated based on current incidence and survival rates.
Results: A total of 316 ALS patients were treated with LTMV, 75% with non-invasive ventilation (NIV) and 25% with tracheostomy, representing a 5 fold increase since 2002. More men (70%) than women (30%) with ALS used LTMV (p<0.05). Median (25/75 percentiles) survival was 15.4 months (5.9/30.2) months with NIV and 74.8 months (44.1/112.8) months with tracheostomy or NIV/tracheostomy. Survival time decreased with age, whereas gender had no significant impact on survival. Based on measured incidence and survival rates, the number of ALS cases with LTMV in Norway is expected to remain at current levels.
Conclusions: Median life expectancy of ALS patients on LTMV, in particular with tracheostomy, was higher than shown in previous studies and without significant gender differences. The number of ALS patients with LTMV will probably stabilize close to the 2014 level.
- Copyright ©ERS 2015
