Caring for Young Adults with Sickle Cell Disease

Dr. Cece Calhoun (Yale School of Medicine) joins Heme Talks to discuss one of the most vulnerable moments in sickle cell care: the transition from pediatric to adult management. Using a real case of a 24-year-old with hemoglobin SC disease re-establishing care after a lapse, Dr. Calhoun walks through how to build trust with adolescent and young adult patients, why sickle cell disease is a systemic vascular and inflammatory condition rather than "just pain," and how to explain complex pathophysiology in patient-friendly language. The conversation covers essential screening guidelines for new adult patients, strategies for community providers managing sickle cell without specialist access, the evolving role of hydroxyurea (including the PIVOT trial in hemoglobin SC disease), and a practical, patient-centered approach to outpatient pain management.Clinical Pearls:The first visit is about trust, not just data.  Understanding a patient's life context, not just their labs is foundational, especially for AYA patients re-entering care after a gap; overwhelming a new patient with testing can undermine that trust. Sickle cell disease is systemic, not just a localized vaso-occlusive crisis. Even "milder" genotypes like hemoglobin SC cause damage through chronic inflammation, hemolysis, and vascular interaction. The historical framing of SC as mild disease is outdated, and end-organ damage can appear early in young adults. Structured screening and collaboration close care gaps. New adult patients need a full baseline workup (CBC/retic, electrophoresis, renal and liver labs, brain MRI, ophthalmology referral), and providers without sickle cell specialization should identify a partner institution and use published NHLBI/ASH guidelines rather than navigating complex cases alone. Hydroxyurea remains foundational, even without perfect evidence in SC disease. It's the only FDA-approved therapy shown to prolong survival in sickle cell disease; while the PIVOT trial's data in hemoglobin SC disease is still limited, empiric use is reasonable in symptomatic patients showing signs of pain or end-organ damage. For additional ASH resources related to Sickle Cell Disease, please consider visiting our ASH Clinical Practice Guidelines on Sickle Cell Disease web page.This podcast episode is supported by Sanofi.