Comprehensive multidisciplinary care for adult Duchenne muscular dystrophy in South Korea

Abstract

Background: With advancements in cardiorespiratory care, the lifespan of patients with Duchenne Muscular Dystrophy (DMD) has significantly increased, shifting the focus from merely extending life to enhancing quality of life and managing complications effectively. However, there is a lack of interest in the management of adult DMD, highlighting a gap in holistic care approaches for this aging population.

Objective: This study aims to assess the current state of care for adult DMD patients in South Korea, focusing on multidisciplinary management and identifying key areas for improvement.

Methods: We retrospectively reviewed the medical records of 168 adult DMD patients who visited the Pulmonary Rehabilitation Center at [blinded for review] from 2022 to 2023. Demographic data, ventilatory support status, cardiac function, gastrointestinal, renal, and skeletal health were analyzed.

Results: Of the patients, 93.8% required non-invasive ventilatory support, while 6.2% used invasive ventilation. Cardiac medications were prescribed to 82.1% of patients, with beta-blockers being the most common. Renal dysfunction was found in 10.1% of patients, with one patient requiring dialysis. A significant portion (54.8%) were on anti-constipation medications due to gastrointestinal issues, and 41.7% of patients had undergone scoliosis correction surgery. Bone mineral density was significantly reduced in most patients, with an average Z-score of -3.3.

Conclusions: This study emphasizes the need for improved care protocols that enhance lifespan and quality of life and manage complications for patients with DMD. This study advocates a shift in healthcare strategies toward a more integrated and holistic care model for adult patients with DMD.