Prognosis of amyotrophic lateral sclerosis patients undergoing tracheostomy invasive ventilation therapy in Japan

Abstract

ObjectiveThe aim of this study is to describe and clarify the factors affecting the prognosis of Japanese patients with amyotrophic lateral sclerosis (ALS) undergoing tracheostomy invasive ventilation (TIV) therapy.MethodsWe conducted a prospective longitudinal observational case-control study using a multicentre registry. ALS patients who started TIV therapy after registration (TIV group) and those who did not receive TIV (non-TIV group) were included. We compared the survival time between the TIV group and the non-TIV group using a propensity score matching analysis and evaluated the prognostic factors in the TIV group.ResultsFrom February 2006 to January 2018, 190 patients in the TIV group and 1093 patients in the non-TIV group were included in this study. The mean age of disease onset and usage rate of gastrostomy and non-invasive ventilation therapy differed between the groups. In the propensity score matching analysis using known prognostic factors, the median overall survival time of the TIV group was significantly greater than that of the non-TIV group (11.33 years vs 4.61 years; p<0.001). Analysis using the Cox proportional hazard model suggested that older age of onset and respiratory onset was an independent factor for poor prognosis after starting TIV therapy.ConclusionWe showed that there was a significant difference of approximately 7 years in life expectancy between Japanese ALS patients who did and did not receive TIV therapy.

Non‐invasive and tracheostomy invasive ventilation in amyotrophic lateral sclerosis: Utilization and survival rates in a cohort study over 12 years in Germany

“…The median survival after initiation of TIV was 25 months and comparable to reports from Italy and Denmark (19–22 months) [11,13,48], but longer than in one other Italian study, and studies from Spain and the UK (8–10 months) [7,12,42]. In contrast to our register and cohorts in other European countries, longer survival was only recently reported with TIV in Japan, with a mean survival of more than 11 years [38]. Although the reasons for differences in survival prognoses have not been analysed systematically, this finding may reflect economic and cultural differences among countries.…”

A multi-ethnic meta-analysis identifies novel genes, including ACSL5, associated with amyotrophic lateral sclerosis

“…The ethics committees of all participating institutions approved the study. Details of the JaCALS have been described elsewhere 32 . All 1,245 sporadic ALS patients were genotyped with Illumina (San Diego, CA, USA) HumanOmniExpressExome-8 BeadChips (version 1.0, 1.2, and 1.3).…”

“…In the discovery cohort, we performed a GWAS in Japanese sporadic ALS cases from the Japanese Consortium for ALS research (JaCALS) 32 and in normal controls from the Tohoku Medical Megabank Project (TMM) 33 , 34 . In the replication cohort, we obtained DNA from ALS patients registered by BioBank Japan 35 , 36 and normal controls registered in the Pharma SNP Consortium.…”

Respiratorbehandling ved amyotrofisk lateral sklerose

“…Flere studier viser at pasienter med amyotrofisk lateral sklerose opprettholder god livskvalitet gjennom denne typen avansert behandling, og at pasienter ofte bedømmer egen livskvalitet som høyere enn legen gjør (15,16,17). Psykologiske, sosiale og eksistensielle faktorer betyr mer for egenrapportert livskvalitet ved amyotrofisk lateral sklerose enn fysisk funksjonsnivå (18).…”