Fewer than half of children aged 2-16 years with sickle cell anemia are receiving recommended annual screening for stroke, a common complication of the disease, according to a new Vital Signs report from the Centers for Disease Control and Prevention.
Many of these children also are not receiving the recommended medication, hydroxyurea, which can reduce pain and acute chest syndrome and improve anemia and quality of life, according to the report released Sept. 20.
Sickle cell anemia (SCA) is the most severe form of sickle cell disease (SCD), which is a red blood cell disorder that primarily affects Black and African American people in the United States. It is associated with severe complications such as stroke, vison damage, frequent infections, and delayed growth, and a reduction in lifespan of more than 20 years.
SCD affects approximately 100,000 Americans and SCA accounts for about 75% of those cases.
Physician Remembers Her Patients’ Pain
In a briefing to reporters in advance of the report’s release, Debra Houry, MD, MPH, the CDC’s acting principal deputy director, recalled “long, tough nights with these young sickle cell warriors” in her career as an emergency department physician.
“[S]eeing children and teens suffering from the severe pain that often accompanies sickle cell anemia was heartbreaking,” she said.
She asked health care providers to confront racism as they build better systems for ensuring optimal treatment for children and adolescents with SCA.
“Health care providers can educate themselves, their colleagues, and their institutions about the specialized needs of people with sickle cell anemia, including how racism inhibits optimal care,” Houry said.
She said people with SCA report difficulty accessing care and when they do, they often report feeling stigmatized.
Lead author of the report, Laura Schieve, PhD, an epidemiologist with CDC’s National Center on Birth Defects and Developmental Disabilities, and colleagues looked at data from more than 3,300 children with SCA who were continuously enrolled in Medicaid during 2019. The data came from the IBM MarketScan Multi-State Medicaid Database.
Key Recommendations Issued in 2014
In 2014, the National Heart, Lung, and Blood Institute (NHLBI) issued two key recommendations to prevent or reduce complications in children and adolescents with SCA.
One was annual screening of children and adolescents aged 2-16 years with transcranial Doppler (TCD) ultrasound to identify those at risk for stroke. The second was offering hydroxyurea therapy, which keeps red blood cells from sickling and blocking small blood vessels, to children and adolescents who were at least 9 months old to reduce pain and the risk for several life-threatening complications.
The researchers, however, found that in 2019, only 47% and 38% of children and adolescents aged 2-9 and 10-16 years, respectively, had TCD screening and 38% and 53% of children and adolescents aged 2-9 years and 10-16 years, respectively, used hydroxyurea.
“These complications are preventable – not inevitable. We must do more to help lessen the pain and complications associated with this disease by increasing the number of children who are screened for stroke and using the medication that can help reduce painful episodes,” said Karen Remley, MD, MPH, director of CDC’s National Center on Birth Defects and Developmental Disabilities, said in a press release.
Bridging the Gap
Providers, parents, health systems, and governmental agencies all have roles in bringing evidence-based recommended care to young SCA patients, Houry noted.
Community organizations can also help connect families with resources and tools to increase understanding.
Schieve pointed to access barriers in that families may have trouble traveling to specialized centers where the TCD screening is given. In addition, appointments for the screening may be limited.
Children taking hydroxyurea must be monitored for the proper dosage of medication, she explained, and that can be logistically challenging as well.
Providers report they often don’t get timely information back from TCD screening programs to keep up with which children need their annual screening.
Overall, the nation lacks providers with expertise in SCD and that can lead to symptoms being dismissed, Schieve said.
Hematologists and others have a role in advocating for patients with governmental entities to raise awareness of this issue, she added.
It’s also important that electronic health records give prompts and provide information so that all providers who care for a child can track screening and medication for the condition, Schieve and Houry said.
New Funding for Sickle Cell Data Collection
Recent funding to the CDC Sickle Cell Data Collection Program may help more people get appropriate care, Houry said.
The program is currently active in 11 states and collects data from people all over the United States with SCD to study trends and treatment access for those with the disease.
The data help drive decisions such as where new sickle cell clinics are needed.
“We will expand to more states serving more people affected by this disease,” Houry said.
The authors declared no relevant financial relationships.
This article originally appeared on MDedge.com, part of the Medscape Professional Network.