A primary care provider's guide to preventive and acute care management of adults and children with sickle cell disease.

PURPOSE: To familiarize primary care providers (PCPs) with the pathophysiological processes, diagnostic evaluation, and medical management of sickle hemoglobinopathies and their complications. Current standards of care, clinical research advances, and new treatment options will also be addressed to assist PCPs in the management of sickle cell disease (SCD). DATA SOURCES: A selective search and review of the current literature on SCD and the authors' experience. CONCLUSIONS: Management of individuals with SCD is very complex, requiring a multidisciplinary approach that includes the patient or parent, PCP, specialist, nurse, and social worker. More patients living with SCD are relying on PCPs in nonspecialty practices for comprehensive disease management. IMPLICATIONS FOR PRACTICE: Newborn screening detects new cases of SCD annually. The median life expectancy has more than doubled for individuals with sickle cell anemia. Healthcare providers are now in an era of increased routine screening, assessment, and management of chronic complications from this illness not previously seen in the care of adults with SCD.