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Sickle cell anemia can lead to childhood stroke


Early awareness key to prevention

Children with sickle cell anemia are vulnerable to serious infections and stroke, but many do not receive the preventive care that could help them stay healthier longer, according to a new Children’s Hospital Los Angeles released this week.

To assess the quality of preventive care received by children with sickle cell anemia, a team of investigators led by CHLA researchers examined how many children met two nationally endorsed quality standards. The researchers measured how many young children with sickle cell anemia received adequate preventive antibiotics to prevent infection and if children and adolescents with sickle cell anemia received annual brain ultrasounds to assess their stroke risk.

“What we found, unfortunately, was not what we’d hoped for,” said Dr. Ashaunta Anderson, a CHLA pediatrician who led the study.

When CHLA researchers and their collaborators compared Medicaid claims data from California and Georgia from 2010 to 2019, they found that only about 20% of children from three months to five years old with sickle cell anemia received preventive antibiotics in a given year, while about half of children and adolescents between the ages of two to 15 received an annual transcranial Doppler ultrasound, the study found.

Twice-daily doses of antibiotics, given consistently, can protect young children with sickle cell anemia from developing serious infections, researchers said. Children and adolescents with abnormal ultrasounds have a higher-than-normal risk of stroke, but once identified, their risk can be significantly reduced by receiving regular blood transfusions.

The research was published in the journal Pediatrics.

About 100,000 people in the United States have sickle cell anemia, including roughly one out of every 400 Black people and about one in 19,000 Latinos. The chronic, genetic disease distorts the doughnut-shaped red blood cells that carry oxygen in blood into sickle shapes, leading them to clump in vessels and block blood flow to organs. These acutely painful episodes in children can lead to complications, including joint pain, infections, organ damage and stroke.

“We have a long history of treating patients in our Sickle Cell Disease Program at CHLA,” said Dr. Thomas Coates, CHLA section head, hematology, and the study’s co-author. “We provide specialist care and access to clinical trials. We focus on developing innovations in safer care, such as stroke prevention. We also provide practical support for patients’ families, such as help with transportation to appointments to make it easier for them to get their children consistent care.”

CHLA found that children with private insurance generally meet the standards for preventive care, but because of health care disparities, the quality of care received by children from low-income families insured by Medicaid varies by state and can depend on whether the child’s family lives in an urban or a rural area.

As patients got older, they were less likely to receive their annual scan for stroke risk. However, the percentage of children getting scanned for stroke overall increased during the years of the study, which CHLA experts credit to growing data collection efforts by states—leading to more awareness.