by University of Michigan

Normal blood cells next to a sickle-blood cell, colored scanning electron microscope image. Credit: Wikipedia/Illustration from Anatomy & Physiology

As if starting life with a potentially disabling genetic blood disease wasn't enough, a study shows that almost two-thirds of babies born with sickle cell disease are born to mothers who live in disadvantaged areas.

But the study shows wide variation between states in the rate of births of babies with sickle cell to residents of areas with crowded housing, limited transportation options and other characteristics.

The study was presented recently at the annual meeting of the American Society of Hematology by a consortium of researchers from 11 states who are part of the Sickle Cell Data Collection Program.

The researchers say their data could help public health authorities focus efforts to support the complex needs of children with sickle cell disease and their families.

That includes increasing use of evidence-based treatments such as antibiotics and hydroxyurea—drugs that can greatly reduce the condition's symptoms but are widely underused.

Under-prescribing of such inexpensive and common drugs has received much less attention than two gene-editing treatments for sickle cell disease that recently received approval from the United States Food and Drug Administration, even though the new treatments may cost $2 million or more per child and will involve months of hospitalization at highly specialized medical centers.

The study also finds that sickle cell disease affects 1 in every 350 babies who are Black and not Hispanic, and that 90% of children born with sickle cell disease are Black.

In addition, 57% of children born with sickle cell disease have sickle cell anemia, the subtype that causes the most debilitating symptoms, including pain crises and infections.

If not controlled with medication, these symptoms can lead to frequent emergency department visits, and can affect school attendance, employment, mental health and social relationships.

The findings are some of the first to look at the rates and variations in births of children with sickle cell disease and examine county-level social vulnerability that may affect outcomes since national newborn screening for the condition took effect in 2006.

Among the researchers is Sarah Reeves, Ph.D., a University of Michigan epidemiologist based in the Department of Pediatrics at Michigan Medicine and the Susan B. Meister Child Health Evaluation and Research Center or CHEAR.

Reeves notes that 315 of the 3,305 babies born with sickle cell disease in these states between 2016 and 2020 were born in Michigan.

"This work and other studies speak to why there is such a lack of access to high-quality care in this population," she said. "Sickle cell disease is complex to manage for anyone, but for people from less-advantaged backgrounds living in under-resourced areas, it's even harder."

More information: Mariam Kayle et al, Sickle Cell Disease Births and Social Vulnerability (2016-2020): A Report from the Sickle Cell Data Collection Program, Blood (2023). DOI: 10.1182/blood-2023-190363

Journal information: Blood 

Provided by University of Michigan