Selective difficulties in lexical retrieval and nonverbal executive functioning in children with HbSS Sickle cell disease

Language deficits in multilingual children with sickle cell disease (SCD) are poorly understood. We tested the hypothesis that selective language deficits in this population could relate to an impaired frontal lobe functioning often associated with high-risk homozygous HbS disease (HbSS). In all, 32 children from immigrant communities with HbSS SCD aged 6 to 12 years (mean age = 9.03, n = 9 with silent infarcts) and 35 demographically matched healthy controls (mean age = 9.14) were tested on their naming skills, phonological and semantic fluency, attention, and selected executive functions (response inhibition and planning skills). Analyses of variance showed significant differences between patients and controls in inhibition and planning (p = .001 and .001), and phonological fluency (p = .004). The poorer performance in phonological fluency of the children with SCD was not associated with any visible brain damage to language areas. Hierarchical regression analyses showed that, whereas the control children's vocabulary knowledge explained their performance in the phonological fluency tasks, only inhibition skills accounted for variance in the performance of the children with SCD. These results suggest a selective impairment of verbal and nonverbal executive functioning (i.e., planning, inhibition, and phonological fluency) in children with SCD, with deficits possibly owing to frontal area hypoxia.