{"type":"rich","version":"1.0","provider_name":"Transistor","provider_url":"https://transistor.fm","author_name":"HemeTalks: Conversations in Hematology Education","title":"Beyond Vaso-Occlusion: The Hidden Pathways Driving Sickle Cell Disease","html":"","width":"100%","height":180,"duration":1615,"description":" In this episode of HemeTalks: Conversations in Hematology, hosts Dr. Ronak Mistry and Dr. Vivek Patel are joined by Dr. Enrico Novelli, Professor of Medicine at the University of Maryland and leader of its sickle cell disease program, for a deep dive into the biology and management of sickle cell disease. Dr. Novelli unpacks molecular pathways beyond vaso-occlusion, including hyper-adhesion, oxidative stress, and hemolysis, that drive disease complications and are opening the door to new therapeutic targets. The conversation covers why hydroxyurea remains the treatment backbone despite being underutilized, the challenges of designing clinical trials with meaningful endpoints, and when to reach for exchange transfusion in both acute and chronic settings. A must-listen for anyone caring for patients with sickle cell disease.\nClinical Pearls:\nHydroxyurea is an underutilized but critical backbone of therapy for treating patients with sickle cell disease.\nClinical trials for sickle cell disease need better, more diverse clinical endpoints.\nLimited options are currently available on the market for the management of sickle cell disease. Options include crizanlizumab and L-glutamine. Mitapivat is currently pending approval. Curative options, including stem cell transplant and gene therapy, are also available, but selecting the appropriate therapy is now the challenge.\nExchange transfusions remain a powerful tool. Consider them in the acute setting for patients needing a higher level of care and in the outpatient setting for patients with complications of disease despite maximal doses of agents, such as hydroxyurea.\nTrust-based, longitudinal care is central to high-quality sickle cell disease care.\nThis episode was supported by Sanofi.","thumbnail_url":"https://img.transistorcdn.com/ybjl9FP0yIIoAR9OW4-QMCeW4qWmBKc92wL1zHiuRRM/rs:fill:0:0:1/w:400/h:400/q:60/mb:500000/aHR0cHM6Ly9pbWct/dXBsb2FkLXByb2R1/Y3Rpb24udHJhbnNp/c3Rvci5mbS9mYzM1/NDgwODVmNTJhYTU0/M2VjOGNiMjVlNzlk/NWI3MS5qcGc.webp","thumbnail_width":300,"thumbnail_height":300}