• By Denise Mann HealthDay Reporter
• Posted September 21, 2022

Too Few Kids With Sickle Cell Anemia Get Screened for Stroke Risk

Too few children with sickle cell anemia are getting the recommended screening tests for stroke, a common complication of this disease, a new government report finds.

What's more, many aren't receiving hydroxyurea, a medication that can reduce the complications associated with the blood disorder, researchers from the U.S. Centers for Disease Control and Prevention noted.

Sickle cell anemia is the most severe form of sickle cell disease, a genetic disorder of the red blood cells that primarily strikes Black people. Sickle cell anemia can cause severe pain and shave 20 years off a person's life.

"The disheartening fact is that many young people do not receive lifesaving treatment for this disease, yet severe pain and complications associated with sickle cell anemia are preventable,"study author Laura Schieve, an epidemiologist at the CDC's National Center on Birth Defects and Developmental Disabilities, said during a Tuesday media briefing on the findings.

For the study, researchers analyzed data on 3,300 children with sickle cell anemia enrolled in Medicaid in 2019. Just half of the children aged 2 through 9 and 38% of kids aged 10 through 16 had transcranial Doppler ultrasound screening to determine their risk for stroke. If results from this ultrasound are abnormal, kids may receive blood transfusions to help prevent strokes.

In addition, just two in five younger kids and about half of older children were taking hydroxyurea, the study showed.

With sickle cell anemia, red blood cells are crescent-shaped and can become rigid and get lodged in the small blood vessels, leading to serious complications including strokes, Schieve explained.

"Hydroxyurea can prevent this from happening,"she said. This drug makes red blood cells bigger, rounder and more flexible, so they are less likely to turn into a crescent shape and get stuck in small blood vessels.

In the study, stroke screening and hydroxyurea use were highest among kids who saw doctors more frequently and those who had a history of complications from their disease.

Many people with sickle cell anemia report barriers to receiving the recommended screening and treatment, including those imposed by structural racism, Schieve said. Structural or systemic racism refers to the ways that society fosters racial discrimination through the housing, education, employment, health care and criminal justice systems.

Other barriers included concerns about potential side effects of hydroxyurea. Side effects are mild most of the time, she said. Some potential side effects may include thinning hair and nausea.

Everyone has a role to play in breaking down those barriers, added Dr. Debra Houry, the CDC's acting principal deputy director. "Patients and families can learn about the importance of annual screening for stroke and the use of hydroxyurea," she said.

Some families may not live near a facility that offers screening, and hydroxyurea requires regular blood monitoring which may not always be convenient, Houry noted. To compensate, health care providers can try to integrate screening into a single visit for kids with sickle cell anemia.

Community-based organizations can also connect patients and families with resources and tools to schedule screenings, provide transportation and find financial assistance, she suggested.

"Hematologists and other health care leaders can work with policymakers and advocates to bring attention to structural racism and the impact this has had on sickle cell funding and policies,"Houry added.

The findings were published Sept. 20 as a Vital Signs report.

More information

The U.S. Centers for Disease Control and Prevention has more on sickle cell anemia.

SOURCES: Laura Schieve, PhD, epidemiologist, National Center on Birth Defects and Developmental Disabilities, U.S. Centers for Disease Control and Prevention, Atlanta; Debra Houry, MD, MPH, acting principal deputy director, U.S. Centers for Disease Control and Prevention, Atlanta; Vital Signs, Sept. 20, 2022