Care trajectories and adherence to respiratory management recommendations in persons living with amyotrophic lateral sclerosis: a ten-year cohort study in a French tertiary university centre

<i>Objective</i>: This study determined real-life care trajectories before and after initiation of noninvasive ventilation (NIV) in patients with amyotrophic lateral sclerosis (ALS). Caregiver adherence to respiratory management recommendations and the associated survival rate of people with ALS were also assessed. <i>Methods</i>: Data were obtained from a tertiary center prospective ALS database that included 10 years of follow-up data for people with ALS. Results are presented numerically and with graphical time sequence analysis through K clustering (TAK) representation. Kaplan Meier and Cox models were used to determine survival and associated prognostic factors. <i>Results:</i> 109 patients with ALS patients were included; median [interquartile range] follow-up was 25.0 months [15.3–43.3]. During study timeframe patients had a median of 4.0 [2.0–6.0] clinical visits; death occurred in 54.1%. Median time between clinical visits was 3.9 [2.8–6.5] months, between arterial blood gases was 4.3 months [3.0–6.6], between spirometry testing was 5.8 months [4.1–8.2], and between nocturnal oximetry was 4.4 months [3.0–7.8]. Visualization of care trajectories TAK show marked heterogeneity in survival, time to NIV initiation, and time from NIV initiation to death. Mortality was correlated with NIV initiation and arterial carbon dioxide pressure increase. <i>Conclusions</i>: The current framework in ALS guidelines should be adapted to the ALS disease stage and individual patient characteristics. Understanding how subgroups of patients with ALS use healthcare services over time could help to highlight fragility areas and priorities in the allocation of care resources and implementation of best practices.