Most infants with sickle cell disease experience a double disadvantage

LOS ANGELES: As if having a hereditary blood illness that might be crippling at birth wasn't enough, research indicates that almost two-thirds of infants with sickle cell disease are born to moms who reside in underprivileged neighbourhoods.

However, the analysis reveals significant heterogeneity between states in the percentage of sickle cell kids born to locals who live in densely populated regions with few transit alternatives and other features.

According to the researchers, public health officials may be able to better target their efforts to meet the various requirements of sickle cell disease patients and their families by using their data.

A group of researchers from 11 states that are involved in the Centres for Disease Control and Prevention-funded Sickle Cell Data Collection Programme recently presented the findings at the annual conference of the American Society of Haematology.

This entails making more use of empirically supported therapies like hydroxyurea and antibiotics, which can significantly lessen the symptoms of the illness but are frequently underutilised.

The US Food and Drug Administration recently approved two gene-editing treatments for sickle cell disease; these treatments may cost USD 2 million or more per child and require months of hospitalisation at highly specialised medical centres, but under-prescribing of such common and affordable drugs has received far less attention.

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

In addition, 57 per cent of children born with sickle cell disease have sickle cell anaemia, 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 are Sarah Reeves, PhD, a University of Michigan epidemiologist based in the Department of Paediatrics 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."