Background The Build, Educate, Advance, Transition, in Sickle cell disease (BEATS) music therapy program was developed to address health challenges faced by adolescents/young adults (AYA) with sickle cell disease (SCD) during the transition to adult medical care. Objective The purpose of this study was to investigate the effects of BEATS on self-efficacy, trust, knowledge about SCD, and adherence in adolescents/young adults (AYA) with SCD. Subjects Thirty AYA with SCD, 18–23 years old, recruited from an adult SCD clinic agreed to participate in four BEATS sessions over 1 year. Methods Self-efficacy, trust and SCD knowledge were measured prospectively at baseline and months 3, 6, 9, and 12. Adherence to clinic appointments and healthcare utilization were measured retrospectively from medical records. A repeated measures linear mixed-effect model with compound symmetry covariance structure was used to fit the data. Results BEATS participants demonstrated a significant improvement in SCD knowledge (p = 0.0002) compared to baseline, an increase in acute care clinic, but not emergency department, utilization (p = 0.0056), and a non-significant improvement in clinic attendance (p = 0.1933). Participants’ subjective evaluations revealed a positive response to BEATS. There were no significant changes in self-efficacy, trust, hospital admissions, or blood transfusion adherence. Conclusion Culturally tailored, developmentally appropriate music therapy transition interventions can concretely improve SCD knowledge and may improve transition for AYA with SCD.