Children’s Hospital Los Angeles Study Finds States Lag in Preventive Care for Sickle Cell Anemia

Children with sickle cell anemia are particularly vulnerable to serious infection and strokes. But twice-daily doses of antibiotics such as penicillin can protect children against life-threatening bacterial infections, and annual brain ultrasounds can identify the children who are at high risk of stroke.

To examine the quality of preventive care received by kids with sickle cell anemia, a research team led by investigators at Children’s Hospital Los Angeles studied how well states met two nationally endorsed standards for quality of care for children with sickle cell anemia. “What we found, unfortunately, was not what we’d hoped for,” says Ashaunta T. Anderson, MD, MPH, MSHS, Pediatrician at Children’s Hospital Los Angeles and lead author of the study, published in the journal Pediatrics.

When the CHLA research team and their collaborators compared Medicaid claims data between 2010 and 2019 from two states, California and Georgia, they found that only about 20% of children between 3 months to 5 years old with sickle cell anemia—in a patient population that was 90% African-American—received daily antibiotics against infection. And about half of children and adolescents between ages 2 and 15 received a yearly transcranial Doppler ultrasound to identify their stroke risk. Children with abnormal ultrasounds have a higher-than-normal risk of stroke, which can be significantly reduced by receiving blood transfusions.

An inherited, chronic condition

Sickle cell anemia warps the oxygen-carrying red blood cells—which normally resemble doughnuts—into crescent shapes. In acutely painful episodes, the misshapen red blood cells clump together in vessels to block blood-borne from reaching organs. Complications can involve joint pain, infections leading to sepsis, organ damage and stroke. An estimated 100,000 people in the U.S. suffer from this genetic disease—about 1 out of every 400 African-Americans and about 1 of every 19,000 Latinos.

“We have a long history of treating patients in the Sickle Cell Disease Program at CHLA,” says Thomas Coates, MD, Section Head, Hematology and study 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.”

Preventive care has big benefits

Children with sickle cell anemia who have private insurance generally receive the preventative care to keep them healthier, but the quality of care that children from low-income families insured by Medicaid receive varies by state and location—dependent on whether they live in cities or rural areas. “Patients may not have access to hospitals and clinics that can perform and interpret this kind of ultrasound,” says Dr. Anderson. “And refilling the penicillin prescription every two weeks can be challenging if a family lacks transportation or money.”

The study unexpectedly found that children with sickle cell anemia were more likely to receive antibiotics if they lived in rural Georgia than if they lived in a city. Trends in the data also suggested that patients had a greater chance of receiving preventative antibiotics if their provider was a specialist—a pediatric hematologist—instead of a general pediatrician. As patients became older, they were less were less likely to receive their annual scan for stroke risk. Encouragingly, the percentage of children getting ultrasounds increased over the years of the study, which could be due to more states doing data collection.

What parents can do

“One reason why these evidence-based quality standards are not being met could be lack of information,” says Dr. Anderson. “Patients and families may not know the importance of daily antibiotics and annual scans for stroke risk up to age 16, and providers may be unfamiliar with these guidelines.” Dr. Anderson recommends that parents ask their children’s doctors if there is anything else that they can do to get their children better care.

The team is hoping to turn these findings into action by investigating preventative care in more states. “We are encouraged that these kinds of measurements can be tracked in every state in the country,” Dr. Anderson says. “Policymakers could put these quality indicators for preventative antibiotics and transcranial ultrasounds onto the core set of benchmarks that insurance companies follow. We can use these two measures to start to do quality improvement programs in every clinic and hospital and deliver better care for children with sickle cell anemia to keep them healthier for longer.”