Children With Sickle Cell Anemia Using Medicaid Less Likely to Get Stroke Screening, Pain Treatment

In 2019, less than half of children aged 2 to 16 years with sickle cell anemia received the recommended screening for stroke, a common complication of the disease.

Few children with sickle cell anemia whose health care is covered by Medicaid are not getting the recommended screening they need to prevent stroke or receiving a recommended medication for pain, the CDC said Tuesday.

Sickle cell anemia is the most severe and common subtype of sickle cell disease (SCD), and in 2014, an expert panel convened by the National Heart, Lung, and Blood Institute issued recommendations to prevent or reduce complications in children and adolescents with sickle cell anemia.

The recommendations included annual screening of children and adolescents aged 2 to 16 years with transcranial Doppler (TCD) ultrasound to identify those at risk for stroke and offering hydroxyurea therapy to children and adolescents 9 months and older to reduce pain and acute chest syndrome. Hydroxyurea can also improve anemia and quality of life.

The red blood cell disorder affects approximately 100,000 Americans, primarily Black and African American individuals. It is already known that the 2 recommendations are underutilized due to various barriers.

In this study, published in Morbidity and Mortality Weekly Report (MMWR), researchers analyzed the IBM MarketScan Multi-State Medicaid Database, from January 1, 2010, to December 13, 2019, which includes medical claims data from approximately 24 million Medicaid enrollees from 5 to 15 states (the number of states varies by year). TCD screening and hydroxyurea use were defined based on procedure and pharmacy codes.

TCD screening and hydroxyurea use were examined for 3352 children and adolescents with SCA aged 2 to 16 years and continuously enrolled in Medicaid during 2019. Percentage change during 2014 to 2019 and variation by health subgroups were assessed, and analyses were stratified by age.

In 2019, less than half of children aged 2 to 16 years with sickle cell anemia received the recommended screening for stroke, a common complication of the disease.

Overall, screening TCD rose from 30% to 38% from 2014 to 2019 among children aged 2 to 9 years. Among children aged 10 to 16 years, there was an increase from 43% to 53%.

For hydroxyurea use over the same time period for the younger age group, the increase was similar (from 30% to 38%). For the older age group, hydroxyurea use rose from 43% to 53%.

Both stroke screening and hydroxyurea use were highest among children with high levels of health care use, including those who already had prior disease complications.

Hydroxyurea use exceeded 60% among children and adolescents aged 10 to 16 years if they had:

  • A recent hospitalization
  • 11 to 30 recent ambulatory care visits
  • 3 or more recent emergency department (ED) visits
  • A recent or previous acute chest syndrome hospitalization
  • 2 or more pain crises requiring hospitalization in the current year or a previous year

Hydroxyurea use was highest (47%-58%) in the same subgroups in the younger age group, with the exception of there being little variation in hydroxyurea use by number of ED visits.

Many patients with sickle cell anemia have cited barriers to receiving the recommended screening and treatment, barriers due to structural racism. Patients have reported feeling stigmatized and having their symptoms dismissed when they do receive care, or they do not have access to sickle cell specialists. Other barriers include concerns among parents and providers about potential adverse effects and the effectiveness of hydroxyurea.

“We must take action to ensure that children with sickle cell anemia are receiving potentially lifesaving treatment,” CDC Acting Principal Deputy Director Debra Houry, MD, MPH, said in a statement. “The pain and complications these children often experience can be excruciating and debilitating and can last for hours, days, or even weeks. Preventive care and medicines, such as hydroxyurea, can help ease the pain and suffering these children go through, and may extend their lives.”

The researchers recommended that health care systems implement quality care strategies and build accountability into electronic health records. They said it is also important that "strategies include proactively addressing both interpersonal and structural racism." In addition, expansion of surveillance coverage, now currently limited to a few states, would allow the CDC to better understand patient needs and disease outcomes.

Reference

Schieve LA, Simmons GM, Payne AB, et al. Use of recommended health care measures to prevent selected complications of sickle cell anemia in children and adolescents — selected U.S. states, 2019. MMWR Morb Mortal Wkly Rep. Published online September 20, 2022. doi:10.15585/mmwr.mm7139e1