Sickle Cell Disease and Cognitive Functioning: United States

Sickle cell disease is an inherited blood disorder in which recurrent pain, chronic anemia, and reduced oxygen delivery to the brain place children at risk for cognitive difficulty and delayed physical growth. Before the lab’s global work in sickle cell disease, Eve Puffer studied those cognitive effects in the laboratory of Dr. Jeffrey Schatz at the University of South Carolina.

Palmetto Health Richland Hospital, Columbia, South Carolina, one of the largest sickle cell clinics in the state. Hematologists and psychologists based here and at the University of South Carolina traveled to clinics across the state to provide services and engage children in the research.

Studied with 64 children with sickle cell disease and 81 demographically similar peers, ages 4 to 8

Setting University of South Carolina, United States

What it showed Physical growth partly accounts for cognitive differences, and hydroxyurea is associated with stronger cognitive functioning

Growth and cognition

Dr. Puffer and colleagues conducted a study with 64 children with sickle cell disease and 81 demographically similar peers ages 4 to 8, and found that height-for-age partially accounted for the cognitive differences associated with the disease, and that higher body mass index predicted stronger visual-motor and academic performance among the children with sickle cell disease. A second study followed growth velocity from age 2 in 46 children and found body mass index progression over time associated with global cognitive and visual-motor ability, identifying routinely collected growth data as a possible clinical marker of neurocognitive risk.

Hydroxyurea and pain

Earlier work compared children receiving oral hydroxyurea with children who were not, holding demographic factors and hematocrit constant, and found higher verbal comprehension, fluid reasoning, and general cognitive ability among those on the therapy, preliminary evidence that the treatment carries cognitive benefit. Dr. Puffer also assisted with studies on early digital interventions for pain, moving coping skills onto handheld devices young people could use at home.

Key Publications

Puffer, E. S., Schatz, J. C., & Roberts, C. W. (2016). Association between somatic growth trajectory and cognitive functioning in young children with sickle cell disease. Journal of Health Psychology, 21(8), 1620–1629.

Schatz, J., Schlenz, A., McClellan, C. B., Puffer, E. S., Hardy, S., Pfeiffer, M., & Roberts, C. W. (2015). Changes in coping, pain and activity following cognitive-behavioral training: A randomized clinical trial for pediatric sickle cell disease using smartphones. The Clinical Journal of Pain, 31(6), 536–547.

Puffer, E. S., Schatz, J. C., & Roberts, C. W. (2010). Relationships between somatic growth and cognitive functioning in children with sickle cell disease. Journal of Pediatric Psychology, 35(8), 892–904.

Puffer, E. S., Schatz, J., & Roberts, C. W. (2007). The association of oral hydroxyurea therapy with improved cognitive functioning in sickle cell disease. Child Neuropsychology, 13(2), 142–154.