Ongoing Treatment and Care Management

Many people with sickle cell anemia report barriers to receiving the recommended screening and treatment.

CDC, 2022

“Health care for children with (sickle cell anemia) remains suboptimal” (Pediatrics, 2022). Barriers to health care must be overcome to improve and save the lives of individuals with sickle cell disease (CDC, 2022). “Structural racism is one of those barriers” (Ibid.).

Since 2014, federal evidence-based guidelines recommend that children ages 2 through 16 years with sickle cell anemia have an annual transcranial Doppler (TCD) ultrasound screening to identify who is at increased risk for stroke. “Sickle cell anemia is a leading cause of childhood stroke” (CDC, 2022).

The federal guidelines also recommend that children ages 9 months and older with sickle cell anemia—even if they are asymptomatic—be offered a daily dose of hydroxyurea, which prevents red blood cell sickling, reduces pain and acute chest syndrome, and can improve anemia and boost quality of life.

However, fewer than half of the children ages 2 through 16 years who have sickle cell anemia received the recommended screening for stroke, the CDC reported in 2022. The study included 3,300 children enrolled in Medicaid in 2019. CDC researchers found that only 47 percent of the children ages 2 through 9 years received the screening, and only 38 percent of the children ages 10 through 16 years were screened properly.

In addition, hydroxyurea was used by only 2 in 5 children ages 2 through 9 years and by just over half of children ages 10 through 16 years.

“Despite their extensive health care needs, many people with sickle cell anemia do not have access to providers with expertise in treating the disease or report feeling stigmatized and having their symptoms dismissed when they do receive care” (CDC, 2022). “Other barriers include concerns among parents and providers about potential side effects and effectiveness of hydroxyurea.”

It is important for health-care providers to understand and overcome unconscious biases, receive continuing education about barriers to health care, and change office practices to reduce social challenges faced by their patients when trying to access health care.

“The pathway to quality care for people with SCD is not complicated nor torturous, but rather is achievable by utilizing things that we know, such as TCD screening and hydroxyurea utilization, and working together as a community” (Pediatrics, 2022.)