Improving Outcomes for Patients with Sickle Cell Disease Amid Racial Disparities

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Among other associations, those in educational attainment, employment status, mental health, and pain episode frequency and severity highlight the need for comprehensive care that considers the psychological well-being of individuals with SCD.

Individuals with sickle cell disease (SCD) are not only affected by racial disparities when it comes to maternal morbidity but in many other aspects, including challenges they face as young adults when transitioning from pediatric to adult care for their condition.

To improve outcomes for young adults with SCD, it is crucial to develop antiracist, patient-centered care models allocated to dedicated resources in a systematic approach, according to an article authored by investigators including Alexander Boucher, MD, Division of Pediatric Hematology and Oncology, Division of Hematology, Oncology, and Transplantation, Departments of Pediatrics and Internal Medicine, University of Minnesota Medical School, and investigators.1

According to the article, systemic issues such as funding disparities and the limited availability of adult specialists need to be addressed. By prioritizing the care of young adults with SCD and implementing comprehensive transition programs, we can strive for more equitable and improved health outcomes for this vulnerable population.

Young SCD Patients Face More than Disease Burden

Additionally, individuals with SCD often experience pain, and factors such as employment status, sex, age, and depression are associated with pain frequency. Comprehensive care that considers the mental health and overall experiences of these patients is essential.2

Investigators led by Kelly M. Harris, PhD, Program in Occupational Therapy, Washington University in St Louis School of Medicine, said screening for depression is warranted especially in those with higher pain frequency and severity.

The study included a total of 2,264 participants aged 15 - 45 years with SCD. Among the participants, 56.2% were female, and the mean age was 27.9 years with a standard deviation of 7.9 years. The data revealed that nearly half of the participants reported taking daily pain medication (47.0%) and/or using hydroxyurea (49.2%).

Data also demonstrated 28% of participants received regular blood transfusions, and 20% had a confirmed diagnosis of depression based on medical record abstraction.

The majority of these participants (79.8%) reported experiencing severe pain, with pain crises rated as 7 or higher on a scale of 0 - 10, and 47.8% reported having more than four pain episodes in the previous 12 months. The mean pain frequency and severity scores for the sample were 48.6 and 50.3, respectively, with standard deviations of 11.4 and 10.1.

Educational attainment and income were not associated with increased pain frequency or severity, according to the study. However, unemployment and female sex were associated with increased pain frequency, with higher beta coefficients of 2.13 and 1.78, respectively.

Being younger than 18 years was inversely associated with pain frequency and pain severity, with beta coefficients of -5.72 and 5.10, respectively. Depression was associated with increased pain frequency (beta coefficient of 2.18) but not pain severity.

Maternal Mortality in Sickle Cell Disease

When looking within the context of pregnancy, SCD significantly contributed to racial disparities in severe maternal morbidity, especially among Black individuals. Among a multitude of investigations, a couple of studies highlighted this aspect of healthcare.

Tailoring care and comprehensive interventions are crucial to improving these outcomes for pregnant individuals with SCD, Nansi S. Boghossian, PhD, Department of Epidemiology and Biostatistics, Arnold School of Public Health, University of South Carolina, and a team of investigators, said in their study.3

The elevated risk of severe maternal mortality rate was significant among Black individuals with SCD as examined in a cohort of 956,951 Black individuals, including 3586 with SCD.4

Black individuals with SCD have been shown to be more likely to have Medicaid insurance, undergo cesarean deliveries, and reside in South Carolina. Comparing Black individuals with SCD to those without, the crude risk ratios for severe maternal mortality and non transfusion of the condition during the delivery hospitalization were significantly higher.

After adjusting for various factors, the risk ratios remained elevated, indicating a persistent association between SCD and severe maternal mortality. The study identified specific related indicators, such as air and thrombotic embolism, puerperal cerebrovascular disorders, and blood transfusion, with the highest adjusted risk ratios.

Among countless other investigations, these combined findings demonstrated the extensive impact of SCD on health outcomes. The association between educational attainment, employment status, mental health, and pain episode frequency and severity highlights the need for comprehensive care that considers the psychological well-being of individuals with SCD.


  1. Boucher AA, Lyons M, McGann PT. Rethinking Care Models for Young Adults With Sickle Cell Disease. JAMA Health Forum. 2023;4(5):e230877. doi:10.1001/jamahealthforum.2023.0877
  2. Harris KM, Preiss L, Varughese T, et al. Examining Mental Health, Education, Employment, and Pain in Sickle Cell Disease. JAMA Netw Open. 2023;6(5):e2314070. doi:10.1001/jamanetworkopen.2023.14070
  3. Boghossian NS, Greenberg LT, Saade GR, et al. Association of Sickle Cell Disease With Racial Disparities and Severe Maternal Morbidities in Black Individuals. JAMA Pediatr. Published online June 05, 2023. doi:10.1001/jamapediatrics.2023.1580
  4. Early ML, Eke AC, Gemmill A, Lanzkron S, Pecker LH. Severe Maternal Morbidity and Mortality in Sickle Cell Disease in the National Inpatient Sample, 2012-2018. JAMA Netw Open. 2023;6(2):e2254552. doi:10.1001/jamanetworkopen.2022.54552