Preventing Sickle Cell Anemia Complications in Children | VitalSigns

Sickle cell anemia is the most severe form of sickle cell disease, a group of inherited red blood cell disorders causing unusually shaped, hard, and sticky red blood cells. These cells clump together, blocking blood flow carrying oxygen through the body. Sickle cell anemia, which primarily affects Black or African American people, is associated with a shorter life span and life-threatening complications that can affect all parts of the body. These complications cause pain and suffering. Sickle cell anemia is a common cause of childhood stroke.

There are screenings and treatments available to ease children’s suffering from sickle cell anemia. Two recommended healthcare measures to prevent complications in children with sickle cell anemia are:

• Transcranial doppler (TCD) ultrasound screening, which identifies children with increased risk for stroke.
• Hydroxyurea therapy, which reduces the occurrence of several complications, including severe acute pain episodes and acute chest syndrome, which can result in lung injury and trouble breathing.

Far too few patients are receiving these potentially lifesaving prevention measures, recommended by an expert panel in 2014. Fewer than half of children aged 2–9 years (47%) and 10–16 years (38%) received a TCD ultrasound screening in 2019. Similarly, less than half of children aged 2–9 years (38%) and slightly over half of children 10–16 years (53%) used hydroxyurea. Managing sickle cell anemia in children is complex. These children face discrimination and multiple barriers to care. Racism and prejudice contribute to and worsen these barriers to care, making it harder to receive quality care. This leads to immense physical, emotional, and mental distress for children and their families.

Everyone can help improve care for people with sickle cell anemia by taking steps to address racism and prejudice. The healthcare system can promote tailored strategies to reduce barriers and increase TCD screening and hydroxyurea use among children with sickle cell anemia.