Research Article: Executive Function and Processing Speed in Children Living with Sickle Cell Anemia
Abstract:
Executive function and processing speed difficulties are observed in children living with sickle cell anemia (SCA). The influence of processing speed on executive function is not well understood. We recruited 59 children living with SCA and 24 matched controls aged 8–18 years between 2010 and 2016 from clinics in the UK. Children completed tests in processing speed and cognitive flexibility, subdomains of executive function. MRI scans were conducted within one year of testing; oxygen saturation was obtained on the day of testing. Hemoglobin levels were obtained from medical records. Caregivers completed the executive function questionnaire. Hierarchical linear regressions found that hemoglobin, oxygen saturation, age, infarct status, and processing speed were not independent predictors for any model. However, for all cognitive flexibility tests, there was a significant interaction between infarct status and processing speed; children without silent cerebral infarction (SCI) with faster processing speed had better cognitive flexibility. Our findings indicate that, when interpreting executive function difficulties, it is important to account for the relationship between SCI status and processing speed. More research is needed to elucidate the mechanisms, but clinically, including executive function testing as part of clinic visits by embedding psychologists within the healthcare team would appear to be a critical step.
Introduction:
Sickle cell disease (SCD) is a life-threatening inherited red blood cell disorder characterized by the production of abnormal hemoglobin in the red blood cells (RBCs) [1,2]. Sickle cell anemia (SCA) occurs when the homozygous allele is present (HbS) and is often associated with the most clinical severity [3,4]. Approximately 100,000 people are affected in the United States, most with African ancestry, along with 40–50,000 in Europe [5,6] as well as individuals in the Caribbean, South America, the Mediterranean,…
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