Conference Coverage

Type of Sickle Cell Disease May Affect Risk of Neurodevelopmental Disorders



COLUMBUS, OHIO—Among children with sickle cell disease, type of disease and presence of comorbidities may increase the risk for attentional or behavioral problems, according to research presented at the 43rd Annual Meeting of the Child Neurology Society. Demographics and disease complications also may influence the risk of neurodevelopmental disorders among these children.

“Earlier identification of pediatric patients with sickle cell disease and attention deficit hyperactivity disorder (ADHD), intellectual disability, and specific learning disabilities will allow faster treatment of these disorders and may improve academic performance and quality of life,” said Eboni I. Lance, MD, Co-Medical Director of the Sickle Cell Neurodevelopmental Clinic at Kennedy Krieger Institute in Baltimore.

ADHD Was Common Among Participants
From May 2012 to March 2014, Dr. Lance and colleagues conducted a retrospective chart review of children with sickle cell disease who presented to Kennedy Krieger Institute or Johns Hopkins Hospital. The investigators reviewed the charts for documentation of neurodevelopmental diagnoses such as ADHD; attentional problems; behavioral problems; executive dysfunction; learning disabilities in math, reading, and reading comprehension; intellectual disabilities; developmental delay; fine motor disorders; language disorders; and autism spectrum disorders. The researchers also extracted from the charts data about age, genotype of sickle cell disease, disease complication history, treatments, and school services.

A total of 59 children met inclusion criteria, including 18 who presented to Kennedy Krieger Institute and 41 who presented to Johns Hopkins Hospital. Patients’ average age was 17, and 58% of participants were male. Nearly all (97%) of the children were African American. About 63% of the children had hemoglobin SS type sickle cell disease, 20% had hemoglobin SC, and 10% had hemoglobin S-Beta thalassemia.

When the researchers reviewed participants’ neurodevelopmental diagnoses, they found that 19% of patients had ADHD, 19% had developmental delay, 12% had attention problems, 12% had learning disabilities in math, and 12% had learning disabilities in reading comprehension. Also, 10% of participants had a language disorder, 8% had anxiety, and 8% had behavioral problems.

Associations and Risks for Neurodevelopmental Disorders
Children with hemoglobin S-Beta thalassemia plus or null had significantly higher odds of attention problems than children with the hemoglobin SS type of sickle cell disease. Children with sickle cell disease and a history of asthma had significantly greater odds of behavioral problems than children with sickle cell disease without a history of asthma, even after adjustment for gender and sickle cell disease type. The investigators found no other significant relationships between other neurodevelopmental disorders and demographic characteristics or disease-related complications. They noted that stroke was not associated with significantly increased risk of a specific neurodevelopmental diagnosis, in comparison with other neurodevelopmental disorders.

“There may be differences in the disease phenotype, demographics, and prevalence of certain neurodevelopmental disorders within the pediatric sickle cell disease population,” said Dr. Lance. “Children with sickle cell disease should be screened for neurodevelopmental disorders, with emphasis on specific disease-related characteristics and complications as potential risk factors,” added Dr. Lance. “Specifically, evaluations should include a detailed sickle cell disease history of disease characteristics and complications, as well the typical history of neurologic complications and neurodevelopmental symptoms.”

Erik Greb

Next Article:

Epileptologists more cautious when stopping kids’ AEDs a second time

Related Articles