Hydroxyurea is Recommended for Children with Sickle Cell Anemia. So Why Does It Remain Underused?

In 2014, the National Heart, Lung, and Blood Institute released updated clinical guidelines in reference to the care of children with sickle cell anemia. The updated guidelines recommended the use of hydroxyurea in children who are nine months old or older—whether their sickle cell anemia is mild or severe. However, shares Matthew Shinkle in Healio, uptake has been slow and the treatment remains underused in the pediatric sickle cell anemia population. 

In a study published in JAMA Network Open, a research team performed a cross-sectional study that sourced data from 4,302 children (ages 1-17) with this disorder. Data was sourced from an 8-year period and focused specifically on children with Medicaid in two areas of the country: Michigan and New York. A slight majority of the children included in the study were Black. 

The research team found that children were not widely treated with hydroxyurea, with a mean annual days’ supply of 47.2 days and 97.4 days per child in Michigan and New York, respectively. They did find that usage was trending upwards in Michigan. While this is a positive sign, the study researchers note that more work must be done—not just to encourage patient adherence, but to address issues in health equity facing this community. 

A Primer on Sickle Cell Anemia

Even though sickle cell anemia is rare, it is also considered one of the more common, well-known, and severe forms of sickle cell disease. In sickle cell anemia, gene mutations prevent the formation of normal, healthy hemoglobin. Instead, red blood cells become stiff and sickle-shaped. These get caught along the walls of blood vessels, preventing oxygen flow and creating blockages. In addition, sickle-shaped cells deteriorate faster and the body can’t produce enough healthy cells to make up for this. Sickle cell anemia is especially prevalent within the Black community. 

Symptoms relating to this condition typically appear around 4-6 months old and may include:

  • Frequent or recurrent infections
  • Fatigue
  • Anemia (low red blood cell count)
  • Pulmonary hypertension
  • Fussiness (in infants)
  • Painful swelling of the hands and feet
  • Delayed puberty
  • Jaundice (yellowing of the skin, eyes, and mucous membranes)
  • Episodes of sharp, throbbing pain (pain crises) 
  • Organ damage
Jessica Lynn

Jessica Lynn

Jessica Lynn has an educational background in writing and marketing. She firmly believes in the power of writing in amplifying voices, and looks forward to doing so for the rare disease community.

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