Neuropsychological Functioning in Preschool-age Children with Sickle Cell Disease: The Role of Illness-related and Psychosocial Factors

Abstract

Cognitive and academic deficits have been identified in school-aged children with sickle cell disease (SCD). However, there have been very few identified studies that examine neuropsychological functioning in preschool-age children with SCD. It is important to understand effects of SCD from a developmental perspective and to consider the contribution of environmental factors in this at-risk population. Neuropsychological functioning of preschool-age children with SCD and no history of overt stroke (n = 26) was examined across several domains (language, immediate memory/brief attention, visuospatial/visuoconstructional, motor/visuomotor). The mean Full Scale IQ for the sample was 89.0. Performance on the Immediate Memory/ Brief Attention domain was significantly higher than the other domains, although the pattern of performance was relatively consistent, with mean standard scores ranging from 88.0 to 95.0. Disease severity was not significantly related to cognitive functioning in this group of young children with SCD. Socioeconomic status (SES) was significantly correlated with most domain scores and, based on regression analyses, accounted for 18% to 47% of the variance in functioning. Psychosocial factors, such as number of children living in the home and parental stress levels, were negatively associated with Motor/Visuomotor skills, and weekly hours in school/day care was positively associated with language-related skills. Results suggest that, at this young age, psychosocial risk factors appear to be appropriate targets for intervention, with the goal of improving long-term outcome in children with SCD. Further investigations should include comparison to a matched control group.

Event-Based Prospective Memory in Children with Sickle Cell Disease: Effect of Cue Distinctiveness

“…Although SES and family functioning/environment variables are known to affect outcomes of children with SCD (Barakat, Patterson, Daniel, & Dampier, 2008;Mitchell et al, 2007;Palermo, Riley, & Mitchell, 2008;Sharpe, Brown, Thompson, & Eckman, 1994;Tarazi, Grant, Ely, & Barakat, 2007;Thompson et al, 2003;Thompson, Gustafson, Bonner, & Ware, 2002), no significant effects of SES or family functioning on EB-PM performance were found for EB-PM response accuracy. Although it involved children with a different etiology of brain dysfunction, this is consistent with recent investigations of EB-PM in children with severe traumatic brain injury both in subacute (McCauley, Pedroza, et al, in press) and chronic samples (McCauley et al, 2009) that also failed to find a significant effect of SES.…”

The Role of Neuropsychological Evaluation in Pediatric Sickle Cell Disease

“…In the absence of stroke, there is some evidence that neuropsychological functioning may decline with age relative to peers (Schatz, Finke, Kellet, & Kramer, 2002;Wang et al, 2001), that deficits appear early in development (Noll et al, 2001;Tarazi, Grant et al, 2007;Thompson et al, 2002), and that functioning may be related to hemoglobin/hematocrit level (Bernaudin et al, 2000;Steen et al, 1999;Swift et al, 1989). These deficits are thought to reflect potential effects of chronic anemia and hypoxemia on brain development (Puffer, Schatz, & Roberts, 2007;Quinn, Variste, & Dowling, 2009), with clear contributions from psychosocial and demographic factors (Schatz et al, 2004;Tarazi, Grant et al, 2007). Academic difficulties are often present and may reflect direct effects of disease factors, consequences of deficits in attention and executive functioning, and/or indirect effects of school absences and other psychosocial factors (Schatz et al, 2004).…”

“…Preschool-age children with SCD present with poorer neuropsychological functioning compared to population norms (Tarazi, Grant et al, 2007), and the pace of cognitive development is slower in children with SCD than typically developing children within the first few years of life (Thompson et al, 2002). Although demographic and psychosocial factors appear to significantly contribute PEDIATRIC SICKLE CELL DISEASE 913 to early development in children with SCD (Tarazi, Grant et al, 2007;Thompson et al, 2002), there is evidence that silent infarcts can occur within the first year of life (Wang et al, 2008).…”

“…Stroke in pre-school and school-aged children with SCD is associated with broad and pervasive neuropsychological impairments, including deficits in general intelligence (IQ), attention and executive functions, memory (short-and long-term memory), language, visuomotor abilities, and academic achievement in reading, writing, mathematics, and spelling (for review see Berkelhammer et al, 2007;Daly, Kral, & Brown, 2008;Kral et al, 2001;Tarazi, Grant, Ely, & Barakat, 2007). Findings have consistently revealed that the neuropsychological deficits associated with overt stroke are the most pronounced relative to children who suffer silent infarcts or those children with normal magnetic resonance imaging (MRI; for review see Daly et al, 2008).…”

Neurological Complications and MRI

“…People with HbSS and HbSβ 0 -thalassaemia genotypes are at highest risk, although stroke has been documented in children with HbSS and HbSβ + -thalassaemia genotypes [6]. Stroke can occur as early as 6-12 months [14] when HbF decreases and HbS begins to be synthesised; the first decade of life, when the onset of strokes typically occurs, appears to constitute a 'critical period' for neurologic complications and subsequent neurocognitive morbidity [6,15].…”