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Death, Morbidity in Pregnancy Continue to Plague Sickle Cell Disease Patients

— A 10- to 26-fold greater maternal mortality rate observed compared with the general population

Ƶ MedicalToday
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Sickle cell disease (SCD) patients in recent years continued to have a much greater risk of adverse pregnancy outcomes, including death, compared with the general population, researchers found.

The maternal mortality rate in deliveries involving SCD patients was 10 to 26 times greater when compared with deliveries among Black and non-Black people without SCD, respectively, reported Lydia Pecker, MD, MHS, and colleagues of Johns Hopkins University in Baltimore, in

Specifically, using data from the National Inpatient Sample (NIS) encompassing the years 2012-2018, Pecker and her colleagues showed that the maternal mortality rate was 13.3 per 10,000 SCD deliveries versus 1.2 and 0.5 per 10,000 deliveries among the Black and non-Black populations without SCD.

This compares with data from 2000-2003 showing a mortality rate of

"Nationally, measures of maternal mortality and severe maternal morbidity for people with sickle cell disease have not improved in nearly two decades," Pecker told Ƶ. While administrative datasets "paint a grim national picture of severe maternal morbidity for women with sickle cell disease, there are several opportunities for action," she said.

Pecker suggested that federal legislation should include funding for comprehensive, multidisciplinary care for pregnant people with SCD that would ideally include specialists in SCD and high-risk obstetrics. Additionally, she said action is needed by the federal government to address the limited availability of high-quality, comprehensive data on nationwide SCD care.

In this study, the researchers analyzed a sample of over 5.4 million deliveries, 3,091 of which were deliveries among pregnant persons with SCD (84% of whom were Black), and 742,164 deliveries among Black people.

When compared with the non-Black control group, persons with SCD and those with Black race were more likely to be younger, have public insurance, and live in areas with a higher proportion of households below the median income.

Pecker and colleagues showed that compared with the non-Black control group, SCD deliveries had several-fold higher odds of severe maternal morbidity (adjusted OR 7.22, 95% CI 6.25-8.34). The odds were particularly high for cerebrovascular events (adjusted OR 22.00, 95% CI 15.25-31.72), thromboembolism (adjusted OR 17.34, 95% CI 11.55-26.03), and acute respiratory distress syndrome (adjusted OR 15.99, 95% CI 12.35-20.72).

The authors also analyzed how racial disparities contributed to the risk of severe maternal morbidity and other adverse outcomes and determined that the median excess risk (ER) attributable to racial inequities was 28.9%, with racial disparities accounting for about half of the increased risk in SCD deliveries for acute kidney failure, intrauterine fetal demise, and eclampsia.

Pecker and her colleagues also observed that deliveries to Black persons and those with SCD had an elevated risk of cesarean delivery compared with the non-Black control sample. However, they reported only a small proportion of the increased risk for cesarean delivery risk among SCD deliveries was attributable to racial disparities (ER 8.8%, 95% CI 8.3-9.2).

"This study identifies that structural racism partly contributes to the poor outcomes. However, sickle cell disease itself, and the care structures for people with the disease, will also need to be addressed to reduce mortality and morbidity. There is a profound need for adequate, comprehensive multidisciplinary care and research for pregnant people with sickle cell disease," Pecker said.

Her group acknowledged that the NIS did not provide reliable information about parity, obesity, smoking, outpatient prenatal care, or the involvement of subspecialty consultants for adjustment in the study.

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    Mike Bassett is a staff writer focusing on oncology and hematology. He is based in Massachusetts.

Disclosures

Pecker reported receiving consulting fees from Global Blood Therapeutics and Novo Nordisk.

Primary Source

JAMA Network Open

Early ML, et al "Severe maternal morbidity and mortality in sickle cell disease in the National Inpatient Sample, 2012-2018" JAMA Netw Open 2023; DOI:10.1001/jamanetworkopen.2022.54552.