Columbia Around the World 

Preventing Brain Injury in Children with Sickle Cell Anemia

July 24, 2019

Before the implementation of standard sickle cell anemia care and stroke prevention in the US, children affected had reduced survival and high prevalence of neuro-cognitive impairment due to stroke. Through research, Columbia and other institutions throughout the country collectively developed and tested highly successful therapies that are now routinely implemented nationally. Worldwide, rates of childhood mortality and stroke remains high for children with sickle cell anemia who lack access to the necessary interventional resources. The susceptibility to stroke for children without access to screening and care is 200-fold higher than the general pediatric population. That’s why Columbia’s Nancy Green, MD and colleagues are bringing these resources and assessing risk of stroke and cognitive impairment beyond the US. Their work is supported by an NIH-funded research grant, “The Burden of Sickle Cell Disease on the Brains of Children in Uganda.”   

Over 300,000 children with sickle cell anemia are born each year, the vast majority in sub-Saharan Africa. In the East African country of Uganda, 15,000 to 20,000 affected infants are born annually with sickle cell anemia. Children with sickle cell anemia are highly susceptible to stroke, neurological and cognitive impairment as manifestations of pediatric “sickle cerebral vasculopathy.” Working closely with Makerere University School of Medicine, Dr. Green and her team examined 265 children with sickle cell anemia and assessed their neurological and cognitive abilities and performed stroke screening to determine the prevalence and types of pediatric brain injury in children with sickle cell anemia in Kampala, Uganda. A subset of these children also had magnetic resonance imaging (MRI) to evaluate for prior stroke and sickle cerebral vasculopathy.

They discovered a shockingly high prevalence of children with neurological and cognitive disabilities—one in five children. These findings expose the need for interventional treatment to reduce and prevent pediatric brain injury in children with sickle cell anemia in Uganda and throughout sub-Saharan Africa. With these findings, Dr. Green and her colleagues are now working on the next step in Uganda: to expand their research by testing the impact of a disease-modifying intervention to reducing the risk of stroke and other manifestations of pediatric cerebral vasculopathy in children with sickle cell anemia.