Sickle Cell Disease in Children and Adolescents: Aligning Practice with Guidelines
CDC report points to areas for improvement.
The Morbidity and Mortality Weekly Report (MMWR) released by the Centers for Disease Control and Prevention on September 20, 2022, highlights an important opportunity for nurses to provide evidence-based care to children with sickle cell disease (SCD). The report highlights poor compliance with guidelines recommending that children ages 2 to 16 with sickle cell anemia (SCA)—the most common and often most clinically severe form of the disease—should receive transcranial Doppler (TCD) ultrasound screening and that hydroxyurea (HU) should be offered for all children with SCA ages 9 months and older.
In the past, children with SCD usually did not survive past age 20 because of infections/sepsis and stroke. Children with SCD now can live much longer, into their 40s, 50s, and 60s, as a result of the use of penicillin to prevent sepsis and screening for children at high risk for stroke. According to current guidelines for children and adolescents with SCA, when children are screened for stroke by TCD, those at high risk can be identified and referred for monthly transfusions, a practice that has been found to prevent stroke. In addition, there is much evidence supporting the use of HU, including prevention of severe pain episodes, acute chest syndrome, and hospitalization.
