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.

The hand of an infant with dactylitis—a
painful swelling of the fingers or toes. Photo courtesy of Tom D. Thacher, MD.

The report from the CDC analyzed data from over 3,000 children with SCA. While the number of children receiving TCD and HU increased over the years, in 2019 only 47% and 38% among children ages 2 to 9 and 10 to 16, respectively, received TCD screening. As children aged, they were less likely to be screened.

In 2019, HU use was higher in older children ages 10 to 15 (53%) when compared to its use in younger children ages 2 to 9 (38%).

Important knowledge for nurses in multiple settings.

This report is important for nurses, whether RNs or NPs. RNs work in clinics, home, or a hospital settings. Every encounter with a person with SCD offers an opportunity for assessment and education. When working with individuals living with SCD, nurses should ask about whether or not children ages 2 to 16 have received a TCD screening, and if not, why not. Nurses can provide education about why it is so important.

Lack of screening results in lack of treatment, stroke, and early death. HU has countless benefits. Patients often have myths and misunderstandings, particularly about HU use. Nurses can correct these myths and refer them back to the SCD provider.

The role of NPs.

Nurse practitioners have the same opportunities to provide assessments and education, but can go one step further to order the TCD and prescribe HU. NPs are a critical part of the SCD team. NPs also may be providing care in a community or primary care setting. This is another opportunity to screen and refer patients back to the SCD clinic, which would want to be aware of the NP’s concern and order the TCD. NPs in a primary care setting could easily co-manage HU with the SCD specialist.

In summary, nurses have a unique opportunity to work with SCD patients, and parents, to make sure they understand how important TCD screening and HU use is to prevent complications and early death, and promote a good quality of life. For an in-depth review of SCD complications for nurses, read our 2019 CE article in AJN, “Understanding the Complications of Sickle Cell Disease.”

Regena Spratling, PhD, RN, APRN, CPNP-PC, FAANP, FAAN, is a professor in the School of Nursing at Georgia State University.

Paula Tanabe, PhD, RN, FAEN, FAAN, is the Laurel B. Chadwick Distinguished Professor in the School of Nursing at Duke University, and a professor in the School of Medicine.