Blog Chronic Disease

Sickle cell disease and cognitive development

By Gavi Forman, BA candidate in Political Science at Amherst College; SPRIGHT Scholar in the 2020 Institute for Public Health Summer Research ProgramPublic and Global Health Abbreviated Track

I originally matched with Allison King, MD, MPH, PhD, a pediatrician and researcher at Washington University in St. Louis after being admitted to the Institute for Public Health Summer Research Program – Public and Global Health Track. Dr. King has been generous in informing me about her research on sickle cell anemia even though COVID-19 has prevented in-person research in her lab.

Sickle cell disease (SCD) is an inherited disorder in which red blood cells become C-shaped (sickle-shaped), harden, and stick together. This change in shape results in clogged blood vessels, and because sickle cells die prematurely, a shortage of red blood cells: anemia. This causes pain, damaged organs, and shortened life spans. SCD is a lifelong disease that requires treatment to address its effects. The sickle cell trait is, interestingly, also protective against malaria. SCD affects over 3 million people globally and about 100,000 Americans – 90 percent of whom are African-American. SCD is most common among people whose ancestors come from sub-Saharan Africa, India, South and Central America, and Mediterranean countries. These are places where malaria is (or has been) common.

Much of Dr. King’s current research focuses on addressing the cognitive delay in children with SCD. Past research in the field has looked more at biological risk factors for cognitive delay, but she has done a lot of work with the less-explored environmental risk factors.

In one multi-center study, Dr. King’s findings indicated that household income per capita was associated with grade retention of children with sickle cell anemia; however, a silent stroke (cerebral infarct) is not associated with grade retention. The study demonstrates that the biological risk factor of a silent stroke is not a significant indicator but environmental factors are. As published, “perhaps the effect of poverty on educational attainment is so strong that the influence of other factors is difficult to detect.” What steps can be taken to help improve the educational outcomes for these children? How can the challenges of poverty begin to be addressed?

Dr. King led a pilot program to improve the home environment for young children with SCD. The researchers educated the parents about SCD, did home visits, and encouraged positive parent-child relationships. They found that these interventions led to improved cognitive and early language development. Dr. King’s work looking outside of biological risk factors associated with SCD starts to fill in an important gap of factors associated with cognitive delay. Still, this issue of detrimental environmental risk factors is very large. With the basis established by Dr. King’s research, it is clear that continued interventions are needed in order to improve outcomes for children with SCD.

This post is part of the Summer Research Program blog series at the Institute for Public Health. Subscribe to email updates or follow us on Twitter or Facebook.