Respiratory Care Trajectory in Patients With Duchenne Muscular Dystrophy in the Advanced Stage: A Retrospective...

Data on respiratory status and care in older adults with Duchenne muscular dystrophy (DMD) remain limited. This study aimed to characterize respiratory status, respiratory physiotherapy, and associated clinical features in patients with DMD aged ≥ 30 years.

This single-center retrospective study analyzed medical records of 44 individuals with DMD aged ≥ 30 years who received physiotherapy at our center between March 2017 and March 2023. Data included respiratory function (vital capacity [VC], cough peak flow [CPF], and lung volume recruitment [LVR]-assisted measurements), respiratory complications, and clinical characteristics.

The median age was 37 years. Respiratory function was severely impaired, with a median VC of 275 mL and CPF of 0 L/min. LVR enabled assessment of assisted lung inflation, achieving a median insufflation volume of 1040 mL and increasing CPF to 150 L/min. Non-invasive ventilation (NIV) and mechanical insufflation-exsufflation (MI-E) were each used by 95.5% of patients, and 79.5% required continuous ventilatory support. A history of pneumonia was documented in 70.5% (31 patients), and a history of pneumothorax in 29.5% (13 patients). The median ejection fraction was 42%, and 18.2% of patients had died.

LVR enabled evaluation of lung insufflation capacity (LIC) even in patients with advanced-stage DMD. Although causal inference cannot be made, these findings suggest that LVR may serve as a component of respiratory physiotherapy for assessment in adults with DMD.