Pulmonary evaluation and prevalence of non-invasive ventilation in-patients with amyotrophic lateral sclerosis: A multicenter survey

Purpose: Despite the relationship between respiratory status and the probability of survival in Amyotropnic Lateral Sclerosis (ALS) patients, there is a great variability in the approach to monitor pulmonary function among centers. This study describes the different methods used to monitor and evaluate pulmonary function and the prevalence of non-invasive ventilation (NIV) in ALS patients. Methods: All Muscular Dystrophy Association and ALS association multidisciplinary ALS Centers were invited to participate. Each participating center completed a questionnaire regarding their ALS patients. Results: Twenty centers reported information on 2357 ALS patients. The mean interval time for routine follow-up was 3 months (range 2-6). In five centers, patients were routinely evaluated by a pulmonologist; and two never consulted one. PFTs were performed in 17/20 institutions. Inspiratory and expiratory pressures, arterial blood gases and serum chloride were followed in 3/20 centers. The criterion to initiate NIV in most of the centers was a FVC between 20-40% of predicted. 360 patients (15%) were on NIV (BIPAP 13%, CPAP/IPPB 2%) and 66 (2.8%) had tracheostomy. 1155 (49%) of the patients had a "do not resuscitate" status. Conclusions: There is a great variability in the approach to monitor pulmonary function among ALS centers. NIV is used only in a small number of patients with ALS. Clinical Implications: Given the modest effects of current treatment, it becomes imperative to optimize the respiratory management of ALS patients. The impact on quality of life and mortality of a protocol aimed to detect and treat early deterioration in pulmonary function needs to be prospectively studied.