National Alliance of Sickle Cell Centers Consensus Recommendations on Sickle Cell Disease Health Maintenance: A Consensus Statement

Importance: The most common inherited blood disorder in the world, sickle cell disease (SCD) is a known global health concern. While survival of children into adulthood has improved significantly over the last few decades in high-resource countries, substantial gaps in care standards and limited practice harmonization persist. Objective: To provide up-to-date recommendations for preventive care and treatment of all people living with SCD and its related comorbidities. Evidence Review: As part of the National Alliance of Sickle Cell Centers (NASCC), 41 pediatric and adult sickle cell disease experts developed consensus standards and recommendations based on available evidence and expert consensus garnered during an in-depth review of the literature. For these recommendations, there were 3 separate consensus panels (0-2 years, 2-18 years, and >18 years]) held in 2022. The initial expert panels included representation from 41 NASCC-recognized SCD centers (during the first 2 votes) and the third and final vote in 2023 included representatives from 81 NASCC-recognized centers. The primary focus was on: (1) preventative care including screening recommendations, (2) management of acute complications, and (3) treatment options. Findings: In this modified Delphi consensus statement, the experts developed consensus standards and recommendations for the clinical care of people living with SCD throughout the lifespan, as well as identified gaps in current evidence that need additional research. Standards and recommendations included routine screening and education, management of acute complications, as well as disease-modifying treatments such as hydroxyurea. Conclusions and Relevance: People with SCD require coordinated and consistent care starting soon after birth in an SCD center and throughout their lives; SCD is a chronic condition with a highly heterogenous phenotype necessitating lifelong care through an SCD center in collaboration with clinicians in multiple areas of medicine including primary care. Future work should prioritize the management of timing of initiation and precision use of disease-modifying therapies, infection management and prevention, and discussion on transformative therapies.