Preliminary observations on vamorolone in Duchenne muscular dystrophy: a short-term, single-center experience


Topic:

Clinical Management

Poster Number: P69

Author(s):

Michelle Lee, MD, Stanford, Jessica Guzman, RN, Stanford, Rachel Estep, RN, Stanford, Richard Gee, PT, Stanford, Karolina Watson, PNP, Stanford, Cara Piccoli, MD, Stanford, Jenna Klotz, MD, Stanford, Carolina Tesi Rocha, MD, Stanford University

Introduction: Vamorolone is a novel dissociative steroid recently approved by the FDA in October 2023 for treatment of patients with Duchenne muscular dystrophy (DMD) age 2 years and older, regardless of genetic variants in dystrophin or ambulatory status. Early clinical trials were suggestive of efficacy and a more favorable safety profile with fewer side effects related to linear bone growth and bone turnover as compared to those treated with traditional glucocorticoids. _x000D_
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Methods: Clinical features and treatment response were reviewed retrospectively for 31 patients diagnosed with DMD who were transitioned to vamorolone from alternative glucocorticoid therapy since March 2024. _x000D_
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Results: Patients were ages 3 to 19 years old at time of initiation of vamorolone therapy. All patients were previously on prednisone or deflazacort therapy. 10 out of 31 patients were receiving exon skipping therapy and 4 out of 31 patients had received gene therapy with delandistrogene moxeparvovec at time of initiation of vamorolone therapy. 1 patient undergoing exon skipping therapy reported rapid weight gain trajectory on vamorolone for which he was transitioned back to prior deflazacort therapy. 4 other patients also reported significant weight gain within 3 months of vamorolone initiation for which the dose has been decreased to 4 to 5 mg/kg/day. Most notable, 4 patients in this cohort reported notable progression of weakness within 1 month of vamorolone initiation with treatment at 6 mg/kg/day. These patients did not have clear evidence of adrenal insufficiency. These 4 patients were transitioned back to prednisone or deflazacort therapy. _x000D_
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Conclusion: Data on optimal and safe transition to and from vamorolone for treatment in DMD in combination with other novel therapies is lacking at this time. Long-term efficacy and safety in this cohort remain to be determined.