Sickle Cell Disease at Birth: 1 in 350 Black Newborns in 11 States, 2016–2020

Sickle cell disease (SCD) is an inherited blood disorder caused by mutations in the hemoglobin subunit beta (HBB) gene. It brings early death and serious illness — vaso-occlusive pain, stroke and damage to many organs — along with lifelong disabilities and high costs of care.

HBB variants protect against severe Plasmodium falciparum malaria, so they are more common where malaria is widespread. That protection, combined with the history of the trans-Atlantic slave trade, means SCD in the United States mainly affects Black or African American people, adding to the burden on groups affected by structural racism and health inequities. Social conditions further worsen outcomes.

Every U.S. newborn has been screened for SCD and related conditions since 2006, but birth prevalence figures are scarce: the most recent race-specific estimate, about 1 in 365 Black newborns, used 2007 data, and 2015–2017 data showed 1 in 2,024 of all newborns.

Children with sickle cell disease have complex health needs. From CDC's MMWR.

The study

The CDC-funded Sickle Cell Data Collection (SCDC) program then covered 11 states: Alabama, California, Colorado, Georgia, Indiana, Michigan, Minnesota, North Carolina, Tennessee, Virginia and Wisconsin. Researchers used 2016–2020 newborn screening records, birth certificates and confirmation tests to count babies with confirmed SCD, and matched each mother's county to the Social Vulnerability Index (SVI), which ranks counties within a state on 16 social factors in four themes: socioeconomic status, household characteristics, racial and ethnic minority status, and housing type and transportation.

Key findings

• 3,305 newborns had confirmed SCD.
• That is 4.83 per 10,000 live births — about 1 in 2,070 — and 28.54 per 10,000 non-Hispanic Black births, about 1 in 350.
• 89.9% of the babies were Black and 4.1% Hispanic or Latino.
• 56.9% had HbSS or HbSβ0; HbSS is usually the most severe type.
• About two thirds (67%) of their mothers lived in counties with high or very high social vulnerability — 89% on the racial and ethnic minority theme and 64% on housing type and transportation.

Types of sickle cell disease

By state

The Black-birth rates may be overestimated, because the count of Black babies with SCD includes all ethnicities while the count of births includes only non-Hispanic Black babies. In Alabama, Colorado and Indiana, race was unknown for 9%–14% of babies with SCD, so those states' figures could be underestimated.

Mothers of newborns with sickle cell disease by county social vulnerability, 11 states, 2016–2020. From CDC's MMWR.

What could help

With most families in counties with high housing and transportation vulnerability, the authors point to ways of making comprehensive SCD care easier to reach:

• more flexible scheduling of medical transportation;
• reimbursement for existing transportation such as rideshares;
• medical transport through local faith- and community-based organizations;
• new care models, and using where affected babies live to plan specialty and primary care.

Medicaid is the main payer for SCD care, so the data can help Medicaid programs and state agencies weigh housing and transportation needs, and support partnerships with communities to decide where resources go. Overall, the authors call for tailored efforts in high-vulnerability areas to improve transportation and housing and advance equity for children with SCD.

Limits: newborn screening data lag about 3 years (though yearly counts barely changed); missing data prevented finer race and ethnicity groups; and county-level vulnerability can hide differences within counties and doesn't measure individual families' needs.

Sources

Based on Kayle M, Blewer AL, Pan W, et al., "Birth Prevalence of Sickle Cell Disease and County-Level Social Vulnerability — Sickle Cell Data Collection Program, 11 States, 2016–2020," Morbidity and Mortality Weekly Report 73(12), CDC (as corrected); a work of the United States government in the public domain.