Building access to care in adult sickle cell disease: defining models of care, essential components, and economic aspects

Abstract

Sickle cell disease (SCD) is the most common inherited blood disorder in the United States. It is a medically and socially complex, multisystem illness that affects individuals throughout the lifespan. Given improvements in care, most children with SCD survive into adulthood. However, access to adult sickle cell care is poor in many parts of the United States, resulting in increased acute care utilization, disjointed care delivery, and early mortality for patients. A dearth of nonmalignant hematology providers, the lack of a national SCD registry, and the absence of a centralized infrastructure to facilitate comparative quality assessment compounds these issues. As part of a workshop designed to train health care professionals in the skills necessary to establish clinical centers focused on the management of adults living with SCD, we defined an SCD center, elucidated required elements of a comprehensive adult SCD center, and discussed different models of care. There are also important economic impacts of these centers at an institutional and health system level. As more clinicians are trained in providing adult-focused SCD care, center designation will enhance the ability to undertake quality improvement and compare outcomes between SCD centers. Activities will include an assessment of the clinical effectiveness of expanded access to care, the implementation of SCD guidelines, and the efficacy of newly approved targeted medications. Details of this effort are provided.

Low rates of transfusion‐transmitted infection screening in chronically transfused adults with sickle cell disease

“…This study highlights that complex SCD care requires a high level of clinical coordination and resources 26,27 . Adults with SCD require resource intensive care and are underserved, a consequence of improved pediatric survivorship and long‐standing inequities in funding for research and clinical care 27,28 .…”

Sobrevida de pacientes com doença falciforme diagnosticados durante a triagem neonatal: revisão sistemática

“…Embora tenha ocorrido melhoria na sobrevida dos pacientes com DF ao passar dos anos, a expectativa de vida ajustada pela qualidade de vida chega a ser a metade para indivíduos com DF se comparado com os sem DF (33 versus 67 anos) (Lubeck et al, 2019). Este fato ressalta a necessidade de maior acesso dos pacientes ao atendimento com modelos de tratamento e programas abrangentes, contínuos e personalizados, os quais podem impactar diretamente e positivamente na mortalidade (Sarat et al, 2019;Kanter et al, 2020) e proporcionar maior sobrevida.…”

Swaying sickle cell research forward in support of patient reported outcomes

“…The second important issue raised by this work is that over 60% of respondents were interested in mental health services, yet we know that the availability of these resources are highly limited 11 . This highlights that in order to meet the needs of people with SCD comprehensive sickle cell clinics should prioritize the availability of mental health services 12 and recognize the impact of mental health on overall health.…”