Preventing Sickle Cell Anemia Complications in Children — CDC Vital Signs

A CDC Vital Signs report updated September 20, 2022, with data from 2019.

At a glance

• Sickle cell anemia, which mainly affects Black people, can shorten life expectancy by more than 20 years.
• Fewer than half of children aged 2–16 got the recommended screening for stroke risk in 2019.
• Only 2 in 5 children aged 2–9 used recommended medicine that can prevent complications.

The disease

Sickle cell anemia is the most severe form of sickle cell disease, a group of inherited red-blood-cell disorders in which the cells become oddly shaped, hard and sticky. They clump together and block the blood carrying oxygen through the body. Sickle cell anemia mainly affects Black or African American people and is linked to a shorter life and to life-threatening complications anywhere in the body, which cause pain and suffering. It is a common cause of stroke in children.

Two measures that prevent complications

An expert panel recommended in 2014:

• Transcranial Doppler (TCD) ultrasound screening, which identifies children at higher risk of stroke;
• Hydroxyurea, a medicine that reduces several complications, including severe episodes of acute pain and acute chest syndrome, which can injure the lungs and make breathing hard.

Far too few children get them.

Despite recent improvement, many children are still going without one or both.

Barriers to care

Managing sickle cell anemia in children is complex, and these children face discrimination and many barriers to care. Racism and prejudice contribute to and worsen those barriers, causing great physical, emotional and mental distress for children and their families.

• Limited access — too few providers have expertise in sickle cell anemia, and many may not know the TCD and hydroxyurea guidelines.
• Fears — families and providers worry about hydroxyurea's side effects and are unsure it will work.
• Appointments — hydroxyurea needs regular monitoring and lab visits; TCD appointments may be scarce, and the radiology centres that do them may be far from a child's usual care.
• Poor coordination — without timely information from radiology centres, providers struggle to track who is due for screening.
• Racism and prejudice — bias against people with sickle cell anemia can mean poor, unequal care, and policies that limit opportunities in housing, jobs and health insurance widen the gaps.

Advancing health equity

Preventing complications starts with recognising where racism exists in health care and deciding to act against it. Providers can learn — and teach colleagues and their institutions — about the particular needs of people with sickle cell anemia, including how racism stands in the way of better health, and can advocate for and listen to their patients.

• Work together — hematologists and other health leaders can work with policymakers and advocates to reverse the effects of years of structural racism on sickle cell funding, research and policy.
• Formal reporting — health systems can set up ways to document and respond to racist behaviour and let patients safely report prejudice or inequity.
• Education and training — in using the treatment guidelines effectively among people from racial and ethnic minority groups.
• Use data — state and local governments can find geographic gaps in care and direct resources to them.

Preventing complications

Childhood stroke and other complications are preventable, not inevitable.

More TCD screening

• Providers: track screenings and follow-up in electronic health records; fold screening into a single comprehensive sickle cell visit where possible; teach patients and families about it.
• Patients and families: learn why a yearly TCD screening matters, and talk with your provider about the results and next steps if they show a higher stroke risk.
• Community organisations: create resources on the importance of yearly screening, and help families book appointments and pay for transport.

More hydroxyurea use

• Providers: learn the prescribing guidelines and the evidence on hydroxyurea's safety; tackle practice barriers such as too little staff or time for counselling; add reminders to the electronic health record.
• Patients and families: learn about hydroxyurea and take it as directed; talk with your provider about regular monitoring and how it compares with other treatments.
• Community organisations: share patient information on its benefits and safety (American Society of Hematology); build tools such as reminder apps; create provider resources on its safety and prescribing.

Sources

• Centers for Disease Control and Prevention, Vital Signs, Preventing Sickle Cell Anemia Complications in Children, and the MMWR report behind it, Use of Recommended Health Care Measures to Prevent Selected Complications of Sickle Cell Anemia in Children and Adolescents — Selected U.S. States, 2019. The page's stock photo collage is left out.