Many children, adolescents with sickle cell anemia still do not receive recommended care
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Key takeaways:
- Rates of screening for stroke risk with transcranial Doppler have increased since 2014 but remain low.
- Treatment with hydroxyurea appears to be underused in the pediatric sickle cell anemia setting.
- In the Vital Signs briefing, experts urged greater awareness of screening and treatment at the patient, clinician and policy levels.
Despite the severe pain and mortality risk associated with sickle cell anemia, few children undergo the recommended screening and treatment for this condition and its complications, according to a CDC report.
The Vital Signs report, published in Morbidity and Mortality Weekly Report, showed that among pediatric patients with sickle cell anemia, almost half of those aged 2 to 9 years and over a third aged 10 to 16 years received screening for stroke risk with transcranial Doppler (TCD) ultrasound.
The report also noted that only about a third of children aged 2 to 9 years and half of children aged 10 to 16 years received recommended treatment with hydroxyurea.
“The bottom line is, the severe pain and complications associated with sickle cell anemia are preventable,” Laura A. Schieve, PhD, epidemiologist at CDC’s National Center on Birth Defects and Developmental Disabilities, said in a news briefing. “Much can be done to reduce pain and suffering for our youngest patients with this disease.”
‘Promising and disheartening’
Researchers analyzed data from 2010 to Dec. 13, 2019, in the IBM MarketScan Multi-State Medicaid Database, which includes medical claims data from roughly 24 million Medicaid enrollees in up to 15 states. They evaluated changes in hydroxyurea use and TCD screening, which is recommended to assess stroke risk, among 3,352 children and adolescents aged 2 to 16 years with a diagnosis of sickle cell anemia.
Results showed that from 2014 to 2019, screening with TCD increased by 27% among children and adolescents aged 10-16 years and by 9% among children aged 2 to 9 years. Despite the smaller increase among younger children, the younger group had higher TCD screening rates than their older counterparts in both years. By 2019, the proportion of children who received screening with TCD was 47% among children aged 2 to 9 years vs. 38% among those aged 10-16 years.
Use of hydroxyurea increased significantly between 2014 and 2019 both among children aged 2 to 9 years (27%) and children aged 10 to 16 years (23%). In 2019, rates of hydroxyurea use reached 53% among children and adolescents aged 10 to 16 years and 38% among children aged 2 to 9 years.
Use of hydroxyurea varied based on health indicators, according to the report. Among children and adolescents aged 10 to 16 years, hydroxyurea use exceeded 60% for those with a recent hospitalization, 11 to 30 recent ambulatory care visits, three or more ED visits, a recent or prior hospitalization for acute chest syndrome, or two or more pain events requiring hospitalization within the current or previous year. Among children aged 2 to 9 years, rates of hydroxyurea use appeared highest (47% to 58%) in the same subgroups, although researchers observed little difference in hydroxyurea use by number of ED visits.
“These data are both promising and disheartening,” Debra Houry, MD, MPH, acting principal deputy director of the CDC, said during the briefing. “We see that among pediatric patients who have had acute chest syndrome, almost half of the younger children and about 60% of the older patients received hydroxyurea. However, that means that almost half and almost 35% were not treated with hydroxyurea.”
Schieve emphasized the need to pay close attention to potential health care disparities in treating sickle cell anemia.
“We have to question why these gaps are there and what we can do to lessen them,” she said. “We have to look at these disparities so we don’t have children with these acute complications.”
Addressing barriers
Schieve cited barriers to appropriate sickle cell anemia screening and treatment at the provider and institutional levels.
“Care coordination is difficult — providers have reported that they often don’t get timely information back from TCD screening programs to remind them of children who are due for their annual TCD screening,” she said. “We also know that there’s just a general lack of health care providers with expertise in sickle cell disease. When patients do seek care, they are often seeking care in emergency departments, where there’s a particular lack of knowledge about their disease.”
Some strategies have been successful in educating patients and families about recommended screening and treatment, Schieve said.
“There have been quality care initiatives that have used electronic health care records to better implement tracking within clinics,” she said. “Patients and families can then learn about the importance of getting a TCD screen and the use of hydroxyurea. Community-based organizations can really be critical in developing patient and provider resources and connecting patients and their families with these resources.”
Houry added that disparities in sickle cell anemia care can also be taken up at the policy level.
“Hematologists and other health care leaders can work with policymakers and advocates to bring attention to racism and the impacts this has had on sickle cell funding,” Schieve said. “Health care systems can develop formal reporting systems to document and respond to racist behavior and empower people with sickle cell anemia to safely report their concerns about prejudice or inequality.”
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