| Abstract [eng] |
Amyotrophic lateral sclerosis (hereinafter – ALS) is a rapidly progressive neurodegenerative disease that leads to full body paralysis. The primary consequence of the late stage of the disease is progressive respiratory muscle weakness, resulting in chronic respiratory insufficiency. In such cases, non-invasive ventilation (hereinafter – NIV) becomes one of the most important therapeutic interventions to compensate for impaired respiratory muscle function; however, in Lithuania it is still not used as a standard intervention. In this study, based on the PRO-ACT database, the effect of chronic respiratory insufficiency on the rate of ALS progression was evaluated. Repeated ALSFRS-R scores and hand grip strength measurements were used for the analysis, divided into two disease stages: the period when forced vital capacity (hereinafter – FVC) measurements remained ≥70%, and a later period when repeat-ed FVC measurements fell below 70%. A paired within-subject analysis was applied. In the NeuroBANK® database, disease progression was assessed before and after initiation of NIV, as well as patient survival. It was found that the development of chronic respiratory insufficiency (FVC <70%) is associated with a significantly increased rate of ALSFRS-R score decline (except in women with bulb-ar-onset disease). Analysis of hand grip strength in patients with upper-limb onset ALS showed similar trends; however, statistical significance was not reached. In addition, a group of patients who received NIV at the time recommended by pulmonologists (mean FVC 62 ± 6%) was evaluated. The rate of disease progression remained similar before and after the initiation of NIV – the acceleration of disease progression did not occur. In the male group receiving NIV, survival was prolonged by an average of approximately 7,8 months, and the difference was statistically significant, whereas in the female group a similar but not statistically significant trend was observed. These findings suggest that NIV helps maintain a more stable disease course and thereby contributes to prolonged patient survival. The primary goal of current ALS management is to preserve patient independence for as long as possible. In Lithuania, NIV is most often initiated only after the development of decompensated hypercapnic respiratory failure, by which time disease progression may already be significantly accelerated. This study highlights the need to reconsider current clinical practice in Lithuania in light of the latest evidence on the benefits of NIV. |
