scholarly journals Reducing Health Care Disparities in Sickle Cell Disease: A Review

2019 ◽  
Vol 134 (6) ◽  
pp. 599-607 ◽  
Author(s):  
LaTasha Lee ◽  
Kim Smith-Whitley ◽  
Sonja Banks ◽  
Gary Puckrein
Keyword(s):  
Health Care ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Health Care Disparities ◽  
Minority Groups ◽  
The United States ◽  
Control Treatment ◽  
Cell Disease ◽  
Blood Disorder ◽  
Poor Outcomes

Sickle cell disease (SCD) is an inherited blood disorder most common among African American and Hispanic American persons. The disease can cause substantial, long-term, and costly health problems, including infections, stroke, and kidney failure, many of which can reduce life expectancy. Disparities in receiving health care among African Americans and other racial/ethnic minority groups in the United States are well known and directly related to poor outcomes associated with SCD. As an orphan disease—one that affects <200 000 persons nationwide—SCD does not receive the research funding and pharmaceutical investment directed to other orphan diseases. For example, cystic fibrosis affects fewer than half the number of persons but receives 3.5 times the funding from the National Institutes of Health and 440 times the funding from national foundations. In this review, we discuss the health inequities affecting persons with SCD, describe programs intended to improve their care, and identify actions that could be taken to further reduce these inequities, improve care, control treatment costs, and ease the burden of disease.

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

2020 ◽  
Vol 4 (16) ◽  
pp. 3804-3813
Author(s):  
Julie Kanter ◽  
Wally R. Smith ◽  
Payal C. Desai ◽  
Marsha Treadwell ◽  
Biree Andemariam ◽  
...  
Keyword(s):  
United States ◽  
Sickle Cell Disease ◽  
Access To Care ◽  
Sickle Cell ◽  
The United States ◽  
Models Of Care ◽  
System Level ◽  
Cell Disease ◽  
Blood Disorder ◽  
Essential Components

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.

The Department of Health and Human Services (DHHS) Sickle Cell Disease (SCD) Initiative: Increasing Access and Improving Care

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 4834-4834
Author(s):  
Susan B. Shurin ◽  
Hani Atrash ◽  
Coleen Boyle ◽  
R. Lorraine Brown ◽  
Janet L. Collins ◽  
...  
Keyword(s):  
Health Care ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Health Care Providers ◽  
The United States ◽  
Evidence Based ◽  
Care Providers ◽  
Cell Disease ◽  
Evidence Based Guidelines ◽  
Support Research

Abstract Abstract 4834 Over the past half century, the course of sickle cell disease has been transformed in the United States through the conduct of rigorous biomedical research and broad application of the results. Universal newborn screening with comprehensive medical care has dramatically reduced death and disability in childhood, and increased the numbers of patients surviving into adulthood. However, access to health care has not kept up with the changing demographics of those affected by sickle cell disease. Health care often becomes fragmented when patients transition from pediatric to adult health care providers. Access to comprehensive care has impeded both conduct of clinical and implementation of research results. To address these needs in this changing environment, HHS Secretary Kathleen Sebelius has charged six agencies of HHS – NIH, CDC, HRSA, FDA, AHRQ and CMS – and the Offices of Minority Health and Planning and Evaluation, to improve the health of people with SCD. The agencies are coordinating their programs and collaborating with the Office of the Secretary, to achieve the following goals:create a comprehensive database of individuals with SCD to facilitate the monitoring of health outcomes and clinical research;improve the care of adults and children through development and dissemination of evidence-based guidelines, which are anticipated in Spring, 2012, with broad implementation plans;identify measures of quality of care for individuals with SCD and incorporate them into quality improvement programs at HHS;increase the availability of medical homes to improve patient access to quality primary and specialty care;provide State Medicaid officials, health care providers, patients, families and advocacy groups with information about resources related to SCD care and treatment;work with the pharmaceutical industry and academic investigators to increase the development of effective treatments for patients with SCD;support research to improve health care for people with SCD;support research to understand the clinical implications of SC trait;engage national and community-based SCD advocacy organizations and experts in ongoing discussions to ensure that issues of importance to persons affected are addressed. Organizational and strategic actions are being taken at each agency to enhance implementation of research advances; provide evidence-based guidelines to families, health care providers, and payers; facilitate new drug development; and provide public health data to impact both the health care delivery and research agendas. The enthusiastic support of the American Society of Hematology and its members is essential for long-term success of this endeavor. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Sickle cell disease promotes sex-dependent pathological bone loss through enhanced cathepsin proteolytic activity

2021 ◽  
Author(s):  
Jada M Selma ◽  
Hannah Song ◽  
Christian Rivera ◽  
Simone Andrea Douglas ◽  
Abhiramgopal Akella ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Bone Loss ◽  
Sickle Cell ◽  
Sickle Cell Trait ◽  
Bone Microarchitecture ◽  
The United States ◽  
Cell Disease ◽  
Female Mice ◽  
Blood Disorder ◽  
The Impact

Sickle cell disease (SCD) is the most common hereditary blood disorder in the United States. SCD is frequently associated with osteonecrosis, osteoporosis and osteopenia and other bone related complications such as vaso-occlusive pain, ischemic damage, osteomyelitis, and bone marrow hyperplasia known as sickle bone disease (SBD)1,2. Previous SBD models have failed to distinguish the age- and sex-specific characteristics of bone morphometry. In this study, we use the Townes mouse model of SCD to study the pathophysiological complications of SBD in both SCD and sickle cell trait. Changes in bone microarchitecture and bone development were assessed by high-resolution quantitative micro-computed tomography (microCT) and the 3D reconstruction of femurs from male and female mice. Our results indicate that SCD causes bone loss and sex-dependent anatomical changes in bone. Particularly, SCD female mice are prone to trabecular bone loss while cortical bone degradation occurs in both sexes. Additionally, we describe the impact of genetic knockdown of cathepsin K and E-64 mediated cathepsin inhibition on SBD.

Hemoglobin F or: How I Learned to Stop Wondering and Love the Flow Cytometer

2019 ◽  
Vol 152 (Supplement_1) ◽  
pp. S15-S16
Author(s):  
Roger Fecher ◽  
Jui Choudhuri ◽  
Mohammad Barouqa ◽  
Seda Tolu ◽  
Caterina Minniti ◽  
...  
Keyword(s):  
Flow Cytometry ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
The United States ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Hemoglobin F ◽  
Blood Disorder ◽  
Cellular Concentration ◽  
Hbf Level

Abstract Sickle cell disease (SCD) is the most common inherited blood disorder in the United States. It is a hemoglobinopathy that leads to red blood cell (RBC) sickling and a broad range of disease complications including vaso-occlusive crisis, acute chest syndrome, and retinopathy. Hydroxyurea, a drug used to treat SCD, is known to increase expression of hemoglobin F (HbF), a type of hemoglobin normally expressed in infancy; HbF levels between 10% and 20% are associated with decreased vaso-occlusive episodes and improved survival. Hereditary persistent hemoglobin F (HPHF), a typically asymptomatic hemoglobinopathy associated with sustained hemoglobin F (HbF) expression into adulthood (HbF >10%), in combination with SCD is associated with decreased complications. Laboratories typically determine the HbF level via high-performance liquid chromatography (HPLC). HbF levels approaching 30% on HPLC are thought to be protective against SCD complications. However, HbF may be found within a majority or minority of RBCs, pancellular (deletional HPHF) or heterocellular distribution (nondeletional HPHF), respectively. Additionally, the quantity of HbF within cells can range from low (<10 picograms/cell) to high (>35 picograms). We sought to determine the quantity and distribution of HbF required to protect against sickle cell disease symptoms both via traditional HPLC as well as flow cytometry. This retrospective study was conducted at a large academic medical center over a period of 2 months (January-February 2019). We collected blood from sickle cell patients that had a detectable HbF level on hemoglobin electrophoresis. We then stained RBCs from 16 of the patients for HbF and performed flow cytometry to examine the HbF distribution. We calculated the cellular concentration of HbF within each HbF+ cell using the formula (MHC × %HbF)/%F-cells. We performed a chart review to determine the native hemoglobin type, exposure to hydroxyurea, and clinical symptoms of sickle cell disease. We identified four patients over the age of 20 with HbS/HPHP and no exposure to hydroxyurea. Two of these patients experienced no sickle cell disease complications; the protected patients had heterocellular distribution of HbF, but had a high concentration of HbF per HbF+ cell (>35 picograms/cell). Notably, these asymptomatic patients both had HbF level by HPLC less than 30. One of the symptomatic HbS/HPHF patients had heterocellular expression of HbF with low cellular concentration (28 picograms/cell) while the other patient had pancellular HbF expression with very low cellular concentration (6.4 picograms/cell). Our study demonstrates that HPHF alone does not prevent sickle cell disease complications. Our study highlights the importance of quantifying the cellular concentration of HbF, which can provide useful information beyond that of HPLC. In addition, our study raises the potential of the clinical use of hydroxyurea in patients with sickle cell disease even in the presence of HPHF.

Prevalence of Vaso-Occlusive Crises in Patients with Sickle Cell Disease: A Retrospective US Claims Database Analysis

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 8-9
Author(s):  
Abiola Oladapo ◽  
Elyse Swallow ◽  
Allison Briggs ◽  
Miriam L. Zichlin ◽  
Bjorn L Mellgard
Keyword(s):  
Health Care ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Clinical Characteristics ◽  
Cell Disease ◽  
Publicly Traded ◽  
Database Analysis ◽  
Claims Database ◽  
Blood Disorder ◽  
The Mean

Introduction: Sickle cell disease (SCD) is an inherited blood disorder affecting ~100,000 individuals in the US. SCD is considered a chronic, lifelong condition that requires comprehensive management. Vaso-occlusive crises (VOCs) are the most common complications of SCD, resulting in intense pain and potential irreversible organ damage. The objective of this study was to characterize the demographic and clinical characteristics of patients with SCD. Methods: A retrospective database analysis was conducted using data from the IBM MarketScan Commercial Claims and Medicare-Supplemental Claims database (July 1, 2013 to June 30, 2018). Patients were included if they met the following criteria: ≥2 diagnoses of SCD on different claims between July 1, 2013 and January 1, 2017, ≥6 years of age on January 1, 2017, and continuous enrollment throughout the 1-year study period (January 1, 2017 to December 31, 2017). Descriptive statistics were used to assess patient demographics (age and sex) and clinical characteristics (Charlson Comorbidity Index [CCI] and other selected comorbidities). In addition, the following outcomes were assessed: the proportion of patients who experienced ≥1 VOC, the frequency of VOCs by care setting, the duration of inpatient VOCs, the monthly VOC risk, and the time between subsequent VOCs. Results: A total of 8174 patients met the inclusion criteria. The mean (± standard deviation [SD]) age was 40.8 (±19.5) years and 63.5% of the patients were female. The mean (±SD) CCI was 0.6 (±1.3), with chronic pulmonary disease, diabetes, renal disease, cerebrovascular disease, and stroke identified as the most common comorbidities. Approximately 20% (n=1659) of patients experienced ≥1 VOC and the mean monthly VOC risk was 0.07 (±0.19). Among patients with ≥1 VOC, the mean (±SD) number of VOCs was 5.2 (±7.7) and the median (interquartile range) time from first to second VOC was 2.4 (0.5-8.2) months. Approximately 18% (n=1461) of patients experienced ≥1 VOC managed in an outpatient setting and 10% (n=844) of patients experienced ≥1 VOC managed in an inpatient setting. Approximately 8% (n=646) of patients experienced ≥1 inpatient and ≥1 outpatient VOC during the study period. Among patients with ≥1 outpatient VOC, the mean (±SD) number of outpatient VOCs was 4.6 (±6.9); among patients with ≥1 inpatient VOC, the mean (±SD) number of inpatient VOCs was 2.2 (±2.3) and the mean (±SD) inpatient VOC duration was 6.6 (±6.4) days. Conclusions: VOCs are common complications of SCD, affecting a subset of patients who often experience recurrent VOC episodes requiring professional health care. VOCs are associated with a significant disease burden on the patient and, potentially, the health care system. Disclosures Oladapo: Takeda:Current Employment, Current equity holder in publicly-traded company.Swallow:Analysis Group, Inc.:Current Employment.Briggs:Analysis Group, Inc.:Current Employment.Zichlin:BMS:Other: Employee of Analysis Group Inc., which received consulting fees.Mellgard:Baxalta US Inc., a Takeda company:Current Employment, Current equity holder in publicly-traded company.

Health care utilization and expenditures for privately and publicly insured children with sickle cell disease in the United States

2009 ◽  
Vol 53 (4) ◽  
pp. 642-646 ◽  
Author(s):  
Mercy Mvundura ◽  
Djesika Amendah ◽  
Patricia L. Kavanagh ◽  
Philippa G. Sprinz ◽  
Scott D. Grosse
Keyword(s):  
United States ◽  
Health Care ◽  
Sickle Cell Disease ◽  
Health Care Utilization ◽  
Sickle Cell ◽  
The United States ◽  
Care Utilization ◽  
Cell Disease ◽  
Publicly Insured
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