National Alliance of Sickle Cell Centers consensus standards for transition to adult care in sickle cell disease Open Access

Sickle cell disease (SCD) is an autosomal recessive hemoglobinopathy that causes anemia and affects ∼100 000 people in the United States according to the Centers for Disease Control and Prevention.¹ Individuals with SCD experience a spectrum of chronic complications, including retinopathy, cerebrovascular disease, nephropathy, and pulmonary hypertension, that progress across the life span. Although significant progress has been made in reducing mortality among pediatric patients with SCD, similar gains have not been seen in adolescent and young adult (AYA) patients aged 15 to 39 years.² Mortality rates improved between 1979 to 1998 and 1999 to 2009 for pediatric patients aged from birth to 14 years, but there was no improvement seen for patients with SCD aged 19 to 34 years.³ 

The process of transitioning from pediatric to adult health care systems can be disjointed, contributing to the high morbidity and mortality seen, especially in patient populations with chronic diseases such as type 1 diabetes.⁴ Previous research has shown that AYA patients with SCD have higher emergency department utilization and lower primary care use, highlighting the system failures that exacerbate the underlying chronic conditions common to SCD.^5,6 

AYA patients often access care at both pediatric and adult health care facilities, resulting in fractured and incomplete medical care. Many patients also have complicated social and mental health care needs that would benefit from additional supportive services not typically part of an adult primary care office. These additional services often include behavioral health, case management, social work, and pharmacy services. Although previous research has examined specific factors that might help ease the transition process, less is known about how to create a comprehensive care program for patients with SCD in the AYA age groups, especially one that spans multiple health systems.⁷ 

Significant research exists around pediatric sickle cell care and the way these patients access care. One notable study specifically examined relationships between acute care utilization and specific patient centered medical home components and found that comprehensive care was associated with fewer emergency department (ED) visits and hospitalizations.⁶ The study included pediatric patients only and relied on parent self-report, which may be a source of error.⁸ Similarly, AYA patients with SCD cared for in a coordinated health care system in United Kingdom experienced a mortality benefit.⁹ In contrast, a study examining 5 state Medicaid databases in the United States found that the rates of emergency department reliance increased during the transition from pediatric to adult care, with the highest rates among AYAs. They also found higher rates of hospitalization and higher costs of care in AYAs than pediatric patients.¹⁰ 

Of all life stages, AYA patients aged 18 to 30 years are the most vulnerable to adverse health outcomes and early mortality,^6,11-13 due to the cumulative effects of SCD,¹² poor transition of care to adult programs,^6,11 nonadherence to prescribed treatment regimens,¹⁴ and high rates of neurocognitive defects.^15,16 Mortality is 3 times higher in AYA patients with SCD than younger patients¹⁷ and occurs soon after transfer to adult care.¹⁷ Furthermore, ED and hospital utilization peak at 3.6 encounters per patient per year in AYA SCD compared to 1.5 to 2.0 encounters per patient per year in younger patients.¹³ Thirty-day readmission rates of 41.1%¹³ and increased ED reliance have been reported in this age group.⁶ Frequency of complications increases from 1.4 to 4.0 diagnoses per patient per quarter after age 16 years and is associated with decreasing use of preventive therapy.¹² Finally, >50% of young adults experience neurocognitive deficits affecting executive function.¹⁵ Lower intelligence quotient, verbal comprehension, and attention difficulties negatively affect transition outcomes.¹⁶ 

The American Academy of Pediatrics, American Academy of Family Physicians, and the American College of Physicians issued joint recommendations on effective transition in 2002,¹⁸ 2011, and 2018.^19,20 The Society for Adolescent Health in 2020 supported these recommendations.²¹ The National Alliance to Advance Adolescent Health’s Six Core Elements of Health Care Transition consensus recommendations also provide best practices and tools for successful transition.²² However, few practices follow the complete set of transition recommendations,^23-25 due to lack of pediatric and adult clinician skills, knowledge, or time, lack of a trained and dedicated staff member responsible for transition planning, unwillingness among families and pediatricians to end long-term relationships,^23,26 and difficulty locating an adult clinician with the right expertise in SCD.²⁷ Most importantly, the abovementioned recommendations are not specific to SCD. This research highlights the unmet needs of patients with SCD and provides a pathway to improve coordinated care for better outcomes.

National Alliance of Sickle Cell Centers (NASCC) uses a modified Rand/University of California Los Angeles Delphi Panel methodology, which allows for hybrid and large group participation to reach consensus, and is described in detail (Kanter et al, The Development of Consensus Recommendation to Improve Practice Harmonization for Sickle Cell Disease through the NASCC, accepted for publication, Journal of Sickle Cell Disease, March 2025). This process systematically and quantitatively combines expert opinion and published literature, is consistent with established processes used for expert practice harmonization in health care decision-making, and was used to develop standards and recommended elements of successful transition^28,29 among NASCC member centers. Standards were defined as being that which “clinicians must do to ensure safe and adequate care or where omission could lead to poor clinical outcomes” (these include key requirements of any service). Recommendations were defined as “those that would be beneficial and that clinicians should try to follow, but for which there is less evidence.” For statements, respondents anonymously rated definitions on a 9-point Likert scale from 1 (“completely disagree”) to 9 (“completely agree”). For data elements, respondents were given instructions to check “yes” next to each data element they believed was “essential to ensure safe and adequate transition of care where omission could lead to poor clinical outcomes” and to further endorse the level of specificity required for that element. Nonessential elements were left unchecked. Consensus was predefined as ≥70% of respondents positively endorsing each element's categorization and inclusion (Likert score ≥5 or “yes,” respectively). This threshold is commensurate with and within the range in use of the Delphi techniques among providers in health care settings.³⁰ Data were collected using REDCap Survey between June and August 2023. At the time of data collection, NASCC had a member count of 78 individual SCD centers (37 pediatric, 24 adult, and 17 life span). Presently, NASCC has a membership of 115 centers (Figure 1).

As with each consensus recommendation, the first round of assessment includes a survey of current practices used by SCD member centers, including those clinical evaluations that are or should be considered standard of care. For this panel, the topics included definitions of successful transfer of care, integration into adult care, responsibilities of pediatric and adult sickle cell centers, and elements that should be included in a transition of care document. Areas of consensus and divergence were identified before the in-person consensus meeting and highlighted for discussion.

All NASCC member centers were invited to attend a transition consensus session meeting at NASCC third Annual Conference (June 2023) to discuss the premeeting survey voting results and the developed statements for transition of care, transfer of care, and the transition summary data elements. As discussed, NASCC continues to grow with more SCD centers formed to enhance care and improve quality for individuals with SCD. Thus, the number of centers participating in each round of assessment is contingent on the number of centers approved at that time. For this hybrid meeting, >40 representatives from 25 centers convened to discuss these measures.

The final vote is completed by survey. Of the 78 approved NASCC member centers at that time, 69 returned completed surveys (overall response rate, 88.5%). Center voting representation among respondents included 33% adult (23/69), 39% pediatric (27/69), and 28% life span centers (19/69). Results of the survey can be found in supplemental Table 1.

Standards are those that clinicians must do to ensure safe and adequate care or the omission of which could lead to poor clinical outcomes. These include key requirements of any service.

Per standard, a successful transfer of care is defined as 2 visits with a comprehensive adult sickle cell program in the first year. Visits can be in person or via telemedicine.

Per standard, 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 scheduled outpatient visits in the 5-year period after transfer of care, along with patient identification of the adult center as their sickle cell medical home.

Recommendations are those that would be beneficial and that clinicians should try to follow, but for which there is less evidence.

Results and final vote tallies are presented in Table 1 and Table 2. Although final consensus decisions were made based on overall agreement rates, there were some differences between pediatric, adult, and life span centers. Life span centers tended to favor the inclusion of more information than adult and pediatric centers. Pediatric centers tended to favor a >50% rate of completed outpatient visits to define successful integration in adult care, whereas adult centers felt that a 50% rate was realistic. Adult and life span centers emphasized the importance of including results of neuropsychological testing and details on social determinants of health. Additionally, most centers did not favor including details of specific vaccination series, preferring instead to indicate only whether they were completed. Overall, there was agreement across center types that the details and specific dates of most SCD complications were not necessary.

The iterative process highlights the balance between necessary information and data that may be extraneous, burdensome, or need to be updated via direct history. Elements with a high level of consensus reflect the importance of these data for a successful transfer of care. When combined into a transition summary, these elements reflect a complete SCD summary to ensure continuous care across the transition period.

This initiative defines 2 key elements of successful pediatric to adult transition, which will allow for the study of interventions to improve outcomes: successful transfer and integration in adult care. Importantly, these consensus guidelines now provide the needed framework and quantifiable metrics for clinicians to evaluate their transition programs for quality improvement. Additionally, standard definitions for outcomes will also improve comparisons across intervention studies, improving knowledge gained. The study also highlights 2 additional critical elements deemed as standards: having a transition policy and tracking transition in both pediatrics and adult care. These are part of existing best practice recommendations and received ∼95% of the votes. The initiative emphasized the importance of the gap between the last pediatric and first adult visit being <3 months, to decrease adverse impacts on health outcomes. Resources that could be effective in supporting these measures were identified, such as having a transition coordinator and a formal system to identify AYA who have not had an initial adult visit within 6 months of their last pediatric hematology visit. As gaps in programs are identified, appropriate resources can be allocated to ensure that AYA patients with SCD have access to complete, high-quality care across the life span. Future research will be needed to further evaluate the efficacy of specific transition program components, especially as they are adapted to individual sites.

Most importantly, pediatric and adult SCD providers agreed on their specific roles in a successful transition and recognized the importance of a “push up” by pediatric centers and a “pull up” by adult centers. They also harmonized desired elements of medical history necessary for the complete transfer of knowledge about AYA patients with SCD (Table 2). Next steps will include programming these elements into the most widely used electronic health record templates to further support transition work. These tools can then be used as population health management tools, identifying care gaps and coordinating with national SCD registries. Future work is needed to understand the implementation of these definitions in quality improvement initiatives, to detail resources needed for transition of care, and to identify reimbursement models that support this valuable work.

The authors extend special thanks to all the members of the National Alliance of Sickle Cell Centers (NASCC) who took part in this initiative and for their commitment to providing excellent care to patients with sickle cell disease.

Part of this work was funded by a grant from Pfizer. NASCC received funding from the Formabridge Grant Program for dissemination of transition education material.

Contribution: J.K., D.M., S.L., and S.G. conceptualized the study; M.M. conducted data collection through surveys and performed study analysis with support from P.C.D., K.R., and A.J.; S.G. and M.M. drafted the initial manuscript; and all authors revised the manuscript for key intellectual content and read and approved the final manuscript.

Conflict-of-interest disclosure: D.M., S.L., and J.K. serve on the executive board of the National Alliance of Sickle Cell Centers (uncompensated). The remaining authors declare no competing financial interests.

Correspondence: Stephanie Howe Guarino, Center for Special Health Care Needs, ChristianaCare, Wilmington Hospital, 501 West 14th St, Wilmington, DE 19801; email: stephanie.guarino@christianacare.org.