In The News: Children With Sickle Cell Anemia Not Getting Treatments, Screening

In a CDC report detailed in Medscape, fewer than half of children with sickle cell anemia are receiving recommended annual stroke screening nor are many receiving the recommended medication for pain, acute chest syndrome and quality of life. Sickle cell anemia is the most severe form of sickle cell disease, which primarily affects Black and African American people in the United States. About 75% of the approximately 100,000 Americans with the disease have sickle cell anemia.

The acting CDC principal director asked at a press briefing on the report for health care providers to be wary of how racism inhibits ensuring optimal treatment for children and teens with sickle cell anemia. She replayed some of her own experience treating sickle cell from her career as an emergency department physician, as well as the difficulty some patients have in accessing care and feelings of stigmatization.

The reported screening and treatment shortcomings are measured against recommendations made by the National Heart, Lung, and Blood Institute in 2014. To prevent or reduce complications in those aged 2-16 years of age, the NHLBI advised annual transcranial Doppler ultrasound for risk of stroke. It also suggested offering hydroxyurea therapy to keep red blood cells from sickling and blocking blood vessels. Five years from the recommendation, the report found 47% of children aged 2-9 years had the screening and 38% used the medication. For adolescents aged 10-16, meanwhile, the numbers were 38% and 53%, respectively.

CDC experts involved in the report say providers, parents, health systems, and governmental agencies all have roles to play in bringing the appropriate care to young sickle cell anemia patients. Access to care, informative resources, adequate health records, a lack of providers with requisite expertise, and funding are among the biggest hurdles. It is hoped that new funding to the CDC Sickle Cell Data Collection Program will help navigate those barriers and help more people get the appropriate care they need.

PIDS member Rebecca Wallihan provided a comment for the story. “This report highlights some of the disparities in care of children with sickle cell disease, specifically transcranial Doppler ultrasound screening and use of hydroxyurea. While this alone is concerning, even more troubling is that this is likely only the tip of the iceberg, not only for children with sickle cell disease, but also for other conditions which primarily or disproportionately affect children from minoritized populations. Though on initial glance these screenings may not seem relevant to our work in pediatric infectious diseases, this article serves as a reminder for all of us to examine the systemic biases and structural racism which affect many of the children and families we care for.”