Most babies with sickle cell disease face double disadvantage

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 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.

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 funded by the Centers for Disease Control and Prevention.

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. 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.

Reference: Most babies with sickle cell disease face double disadvantage; Blood, DOI:10.1182/blood-2023-190363