Establishing a Definition of Transition of Care in Sickle Cell Disease: National Alliance of Sickle Cell Disease Centers Consensus

Introduction

Sickle cell disease (SCD), an autosomal recessive hemoglobinopathy, affects approximately 100,000 people in the United States. Individuals with SCD experience chronic multi-system complications. Although significant progress has been made in improving morbidity and mortality for pediatric SCD patients over the past four decades, improvements have not been made for adolescent and young adult (AYA) patients.

The process of transitioning from pediatric to adult SCD health care systems can be disjointed, contributing to the high morbidity and mortality seen in this population. There is no universally accepted definition of a successful SCD care transition or existing standards/recommendations for SCD clinicians. Utilizing a modified Delphi process to reach consensus among its members, the National Alliance of Sickle Cell Centers (NASCC) defined standards and recommendations for transitioning care from pediatric to adult care health systems, including the definition of successful transfer and integration into adult care and the essential health data elements needed for a standardized EHR transition note to facilitate clinician communication.

Methods

A panel of 6 NASSC SCD experts (3 Adult and 3 Pediatric Hematologists) seeded this modified Delphi consensus initiative. The project consisted of three rounds; (1) Panel development of pre-formative statements around both standards and recommendations for successful transfer and transition of care as well as pediatric and adult transition program operating practices; (2) Discussion among all SCD stakeholders at NASCC’s 2^nd Annual Conference (June 2023) to refine and approve predeveloped statement definitions; and (3) Survey revision based on round two results and a final survey among NASCC Directors to vote on the final definitions, standards, and recommendations (July 2023). A standard is defined as being that which providers must do to ensure safe and adequate care or where omission could lead to poor clinical outcomes. A recommendation is being those that would be beneficial and that providers should try to follow, but for which there is less evidence. Consensus was defined as ≥ 70% of respondents positively endorsing (Likert score ≥5, scale 1-9) an item, commensurate with the use of the Delphi-technique in health care settings.

Results

The final 2^nd round consensus survey was sent electronically to NASCC Directors (N=78). Center representation by Directors, included: 33.3% (23/69) Adult, 39.1% (27/69) Pediatric, and 27.5% (19/69) Lifespan. Consensus was achieved for 2 Standards and 14 Recommendations for transitions of care across the lifespan in SCD in the areas of transition policies, documents, tracking, definitions of successful transfer and transition of care, and initial visit responsibilities.

A successful transfer of care is defined as 2 visits with a comprehensive adult sickle cell program in the first year, in person or via telemedicine. A successful integration into adult care, an essential component of transition of pediatric to adult-centered health care, is defined as ‘completion of at least 50% of annual comprehensive visits in the 5-year period after transfer of care and the patient identification of the adult center as their sickle cell medical home.’

Conclusions

This initiative defines a successful transition, which will allow for the study of interventions to improve outcomes. Importantly, these materials now provide the needed framework and quantifiable metrics for providers to evaluate their transition programs for quality improvement.