A 10-Year Experience of Assessment of Pulmonary Function in Patients with Duchenne Muscular Dystrophy


Clinical Management

Poster Number: 25


Robert Troxler, MD, University of Alabama at Birmingham, Shelley Coskery, MSN, CRNP, Children's of Alabama, Allyson Brown, DO, University of Alabama at Birmingham

Background: DMD leads to a restrictive lung disease, hypoventilation, and premature respiratory failure. Our center began collecting lung function data in a systematic fashion in 2012 to determine the impact of routine pulmonary care, assessment, and steroids on lung health.

Objective: To assess the changes in pulmonary function among patients with Duchenne muscular dystrophy (DMD) attending a single MDA Care Center from 2012-2022.

Methods: This is a single-center retrospective review of pulmonary function testing in patients with DMD who attend the MDA Care Center at UAB/Children’s of Alabama from January 2012 through October 2022. Lung function data were obtained at visits to the Care Center from patients with DMD. Information regarding patient’s anthropomorphics, therapeutic interventions, and ambulatory status was collected. Data were analyzed using GraphPad Prism 9.0 for statistical analysis.

Results: 140 patients with DMD were seen in the Care Center, 122 were able to provide spirometry data at least once, and 33 patients lost the ability to perform spirometry. 1,035 spirometry measures were obtained and included in this study. The median age of the first encounter was 130.1 months and there was a median of 12 visits per patient with a median of 8 measurements per patient. There were significant differences in the rate of lung function decline among patients on prednisone compared to those on deflazacort or on no steroids. The median time in months before FVC declined below 60% was 156 months without steroids compared to 200 months on prednisone and 227 months for patients on deflazacort (p=0.001).

Conclusions: Lung function declines for patients with DMD, but decreases less rapidly while on steroids. Both deflazacort and prednisone result in a delay in forced vital capacity below 60% compared to no steroid use.