Clinical Implications of Routine Monitoring of Pulmonary Function and Ventilation in Patients with Duchenne Muscular Dystrophy

Abstract

Purpose: To investigate the effect of regular monitoring of pulmonary function and ventilatory status on the initiation of non-invasiveventilation (NIV) between patients who were routinely monitored before receiving NIV and those who were not. Materials and Methods: This retrospective cohort study included subjects with Duchenne muscular dystrophy (DMD) who first receivedNIV between 2010 and 2019. The subjects were assigned to either the regular-follow-up (REG) group or the non-REG group,according to their follow-up status, before initiating NIV. We compared the number of emergent cases, the results of nocturnal ventilatorymonitoring, and the pulmonary function of each group at initial ventilatory support. Results: In total, 73 subjects were enrolled in the REG group and 47 subjects in the non-REG group. There were significantly moreemergency cases due to respiratory insufficiency in the non-REG group (12/47, 25.5%) than in the REG group (3/73, 4.1%). At thetime of initial ventilatory support, hypoventilatory symptoms were more common and relatively severe in the non-REG group(37/47, 78.7%) than in the REG group (18/73, 24.7%). The average age at initial ventilatory support of the non-REG group was 2.15years older than that of the subjects in the REG group. Moreover, subjects who were not regularly monitored exhibited greater deteriorationin pulmonary function compared to those who were regularly followed up. Conclusion: Regular evaluation of pulmonary function and ventilatory status before the onset of ventilatory insufficiency is crucialto reduce the risk of patients with DMD requiring emergency care due to ventilatory insufficiency.