May an early integrated care program with NIV adaptation delay NIV failure in patients with ALS?

The most recent European Guidelines propose the start of non invasive ventilation (NIV) when the amyotrophic lateral sclerosis (ALS) patient’s sitting Forced Vital Capacity (FVC% prd) is less than the value of 80%. The aim of our study was to assess whether an early integrated care program with adaptation to NIV (patient with FVC%> 80) can lengthen free interval of NIV failure (tracheostomy and/or death). This retrospective study was conducted in 3 italian facilities on a cohort of 213 subjects with ALS with at least 36 months of follow-up from the NIV start. The subjects were then divided into two groups according to the sitting FVC% value prd (Late group = LG with FVC <80% and Early group = EG with FVC> 80%) at the time of NIV prescription. For each group we analyzed clinical and respiratory functional data, time free from “failure of NIV " (tracheotomy need/death) starting from the first symptoms, the ALS diagnosis and the NIV prescription. 167 patients failed NIV after 36 months of follow-up (in LG 125 and 42 in the EG). After one year from the NIV prescription, the % of failure rate was 50% in LG and about 10% in the EG while after three years by NIV prescription % of failure in the LG was 86% compared to 62% in the EG. When compared with the LG, the EG showed a lower probability of NIV failure starting from a) the time of NIV prescription (p = 0.0000) b) first symptoms (p = 0.001) (figure 1) c) diagnosis (p = 0.0003). An early integrated care with NIV prescription seems to prolong free interval to the some NIV failure (tracheotomy and/or death). Only robust randomized controlled trials will confirm our working hypothesis.