Attention deficits in children with sickle cell disease

Previous research has suggested that children with sickle cell disease may exhibit cognitive deficits even in the absence of direct cerebrovascular involvement (stroke). This study was designed to assess specific attentional deficits in children with sickle cell disease. 12 children with sickle cell disease (Hb SS) with a prior history of stroke, 14 children with sickle cell disease (Hb SS) without evidence of stroke, and 13 similar aged siblings (Hb AA or Hb AS) were compared on measures of attention, intellectual functioning, achievement, and adaptive functioning. Significant differences were found between children with sickle cell disease (with or without stroke) and healthy controls on a timed test of visual scanning, the Coding subtest of the Wechsler Intelligence Scale for Children-Revised, and subtests of Reading, Arithmetic, and Spelling from the Wide Range Achievement Test-Revised. The differences between children with sickle cell disease and their healthy siblings appear to be the result of strokes rather than sickle cell disease itself as children with sickle cell disease without strokes did not significantly differ from controls. Implications for the effects of sickle cell disease and stroke on academic performance are discussed.     

Resilience in siblings of children with sickle cell disease

This pilot study was conducted to identify factors responsible for promoting resilience in siblings of children with sickle cell disease. Twenty siblings (10–17 years of age) of children (5–13 years) with sickle cell disease were selected from the Pediatric Clinic of Howard University Center for Sickle Cell Disease. The siblings responded to questionnaires, and the data obtained was analyzed by chi-square for association. The results indicated that age, birth order, and gender had no effect on resilience in the siblings. However, family size, number of parents in the home, sibling's knowledge of the illness, degree of morbidity of the illness, socioeconomic status of the family, and parents' attitudes and childrearing practices were all found to affect resilience. These findings provide additional insight into the psychosocial aspects of, and genetic counseling for sickle cell disease, as well as for other chronic genetic disorders.     

Inhibitory control in children with frontal infarcts related to sickle cell disease.

Evidence from past studies indicates that children with traumatic brain injury experience difficulties with inhibitory control. Less is known about inhibitory control in children with frontal brain injury related to cerebral infarction. We compared the inhibitory performance of children with frontal infarcts related to sickle cell disease with that of a control group of children with sickle cell disease but no history of cerebral infarction. On a stimulus-response reversal task, children with frontal infarcts made significantly more accuracy errors in the inhibitory condition than controls. Findings from this study and from previous research suggest that impairments in inhibitory control are common following frontal injury in a range of pediatric populations.     

Central nervous system complications of sickle cell disease in children: an overview.

Complications involving the central nervous system are among the most devastating manifestations of sickle cell disease. Although overt stroke occurs in 1 in 10 children with Hemoglobin SS, "silent cerebral infarcts" are even more frequent. Both are associated with significant neuropsychological deficits. The end result of these effects on the CNS often is diminished school performance. The use of transcranial Doppler ultrasonography screening allows the identification of patients at high risk for clinical stroke as well as stroke prevention by chronic transfusion. However, definitive prophylaxis and treatment for most CNS complications of sickle cell disease have yet to be determined.     

Neuropsychological functioning in preschool-age children with sickle cell disease: the role of illness-related and psychosocial factors.

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.     

Association of social-environmental factors with cognitive function in children with sickle cell disease

The aim of this study was to examine the relationship between cognitive function in pediatric sickle cell disease (SCD) patients and mothers’ reports of social-environmental stress, depressive symptoms, and parenting. A total of 65 children with SCD completed comprehensive neuropsychological testing to assess several domains of cognitive functioning, including general intellectual ability, academic achievement, and executive function. Mothers reported on demographics, social-environmental stress, depressive symptoms, and parenting. As predicted, children with SCD significantly underperformed relative to normative data on measures of cognitive function. Associations between maternal social-environmental stress, maternal depressive symptoms, and parenting were mixed. The results show partial support for the hypothesis that greater stress and depressive symptoms and less positive parenting are associated with poorer cognitive function in children with SCD. Linear regression analyses showed that maternal financial stress was the strongest predictor across all domains of cognitive function. The findings replicate and extend past research, reaffirming that children with SCD are at risk for cognitive impairment across multiple domains. Additionally, social-environmental stress, particularly financial strain, is linked to mothers’ depressive symptoms and parenting behaviors as well as children’s cognitive function. Future studies using direct observations of parenting behaviors are needed. These findings, along with recent research on parenting interventions, may inform the development of concrete, teachable parenting and coping skills to improve cognitive functioning in children with SCD.     

Neurocognitive sequelae of pediatric sickle cell disease: a review of the literature.

This literature review summarizes all studies relating neuropsychological performance to neuroimaging findings in pediatric sickle cell disease (N=28; published 1991-2005). Although inconsistencies exist within and across domains, deficits in intelligence (IQ), attention and executive functions, memory, language, visuomotor abilities, and academic achievement have been identified. Overall neurocognitive compromise was revealed to be related to the level of neurological injury and the location of silent infarct. Attentional and executive dysfunction is prevalent and related to frontal lobe abnormalities.     

Physiological correlates of intellectual function in children with sickle cell disease: hypoxaemia, hyperaemia and brain infarction

Lowered intelligence relative to controls is evident by mid-childhood in children with sickle cell disease. There is consensus that brain infarct contributes to this deficit, but the subtle lowering of IQ in children with normal MRI scans might be accounted for by chronic systemic complications leading to insufficient oxygen delivery to the brain. We investigated the relationship between daytime oxyhaemoglobin saturation (SpO2), cerebral blood flow velocity (CBFV) and intellectual function (IQ) using path-analysis in 30 adolescents with sickle cell disease (mean age 17.4 years, SD 4.2). Initial analyses revealed that the association between SpO2 and Full Scale IQ (FSIQ) was fully mediated by increased CBFV, whereby SpO2 was negatively correlated with CBFV and CBFV was negatively correlated with FSIQ, i.e. decreases in oxygen saturation are associated with increases in velocity, and increased velocity is associated with lowered IQ scores. The mediated relationship suggests that lowered IQ may be a function of abnormal oxygen delivery to the brain. Further analyses showed that the association between CBFV and IQ was significant for verbal but not for performance IQ. The pathophysiology characteristic of SCD can interfere with brain function and constrain intellectual development, even in the absence of an infarct. This supports the hypothesis that lowered intellectual function is partly explained by chronic hypoxia, and has wider implications for our understanding of SCD pathophysiology.     

Increasing emotional competence improves psychological and physical well-being, social relationships, and employability

This study builds on earlier work showing that adult emotional competencies (EC) could be improved through a relatively brief training. In a set of 2 controlled experimental studies, the authors investigated whether developing EC could lead to improved emotional functioning; long-term personality changes; and important positive implications for physical, psychological, social, and work adjustment. Results of Study 1 showed that 18 hr of training with e-mail follow-up was sufficient to significantly improve emotion regulation, emotion understanding, and overall EC. These changes led in turn to long-term significant increases in extraversion and agreeableness as well as a decrease in neuroticism. Results of Study 2 showed that the development of EC brought about positive changes in psychological well-being, subjective health, quality of social relationships, and employability. The effect sizes were sufficiently large for the changes to be considered as meaningful in people's lives.     

Etiology of strokes in children with sickle cell anemia

The most devastating complication of sickle cell anemia is cerebral infarction, affecting approximately 30% of all individuals with sickle cell anemia. Despite being one of the most common causes of stroke in infants and children, the mechanism of cerebral infarction in this population has not been extensively studied and is poorly understood. Multiple, synergistic factors are important in the pathogenesis of stroke including the hemodynamic effects of cerebral arterial occlusive disease, viscosity, chronic and acute anemia and acute medical events. This review focuses on the relationship between these factors in order to provide a foundation for further study of the etiology of strokes in this high-risk population.     

Parental divorce and the well-being of children: a meta-analysis.

This meta-analysis involved 92 studies that compared children living in divorced single-parent families with children living in continuously intact families on measures of well-being. Children of divorce scored lower than children in intact families across a variety of outcomes, with the median effect size being .14 of a standard deviation. For some outcomes, methodologically sophisticated studies yielded weaker effect sizes than did other studies. In addition, for some outcomes, more recent studies yielded weaker effect sizes than did studies carried out during earlier decades. Some support was found for theoretical perspectives emphasizing parental absence and economic disadvantage, but the most consistent support was found for a family conflict perspective.     

The link between emotional competence and well-being: a longitudinal study

What aspects of emotional competence help protect people from stress, anxiety, and depression, and help to promote positive affect? A total of 163 university students completed a two wave study that involved measuring emotional competence and emotional well-being at 1-year intervals. As expected, Time 1 measures of emotional competence predicted Time 2 measures of well-being, after controlling for Time 1 measures of well-being. Specifically, ineffective problem orientation predicted increases in anxiety and stress, and decreases in positive affect. Difficulty identifying and describing emotions predicted increases in anxiety and decreases in positive affect. Finally, an aspect of difficulty managing emotions (i.e. rumination) predicted decreases in positive affect. Each emotional competence variable predicted unique variance after controlling for other significant variables. We discuss the implications of these findings for counselling practice.     

Repressive adaptation in children with cancer: a replication and extension.

Prior research has shown a higher incidence of a repressive adaptive style in children with cancer. To explore whether repressive adaptation in this population is premorbid or reactive, adaptive style was assessed longitudinally in children with cancer at the time of diagnosis and at 6 months and 1 year after diagnosis. Comparison groups included healthy children and children with other serious but nonmalignant chronic illnesses. At diagnosis, children with cancer showed a higher incidence of a repressive adaptive style than healthy children, and the incidence of repressive adaptation remained stable over time. Children with other chronic illnesses also showed levels of repressive adaptive style comparable to the cancer group. These findings suggest a shift toward repressiveness in response to the diagnosis of cancer that is then maintained over time and necessitate further examination of the health consequences of adaptive style in this population.     

Strength and vulnerability integration: a model of emotional well-being across adulthood

The following article presents the theoretical model of strength and vulnerability integration (SAVI) to explain factors that influence emotion regulation and emotional well-being across adulthood. The model posits that trajectories of adult development are marked by age-related enhancement in the use of strategies that serve to avoid or limit exposure to negative stimuli but by age-related vulnerabilities in situations that elicit high levels of sustained emotional arousal. When older adults avoid or reduce exposure to emotional distress, they often respond better than younger adults; when they experience high levels of sustained emotional arousal, however, age-related advantages in emotional well-being are attenuated, and older adults are hypothesized to have greater difficulties returning to homeostasis. SAVI provides a testable model to understand the literature on emotion and aging and to predict trajectories of emotional experience across the adult life span.     

Association between biological markers of sickle cell disease and cognitive functioning amongst Cameroonian children

Background: Some of the major complications of sickle cell disease (SCD) occur in the brain and apart from overt stroke, patients also present with cognitive impairments. We sought to evaluate the prevalence of cognitive deficits as well as their biological predicting factors in young SCD patients in Cameroon.

Methods: The cognitive performances of Cameroonian SCD young patients were evaluated using a neuropsychological test battery assessing four domains of cognitive functioning (executive functions, attention, memory, and sensory-motor skills) previously adapted and normalized on healthy subjects in Yaoundé.

Findings: Up to 37.5% of the 96 SCD patients aged 6 to 24 years (M?=?13.5, SD?=?4.9) had mild-to-severe cognitive deficits. The cognitive deficits tend to increase with age. There was a significant effect of SCD on executive functions and attention, whereas SCD patients performed as well as controls on memory and sensory-motor skills tests. Structural equation models showed a significant association between (a) severe anemia and lower executive functioning, (b) low fetal hemoglobin levels and lower executive functioning and attention, (c) history of cerebrovascular accidents and lower performances in executive functioning, sensory-motor skills, and memory, (d) pathological electroencephalogram and lower attention, and (e) abnormal Transcranial Doppler and lower memory.

Conclusion: SCD patients in Cameroon presented a very high prevalence of cognitive deficits, with a specific impairment of executive functions and attention. Routine neuropsychological evaluation for early detection of cognitive deficits in SCD patients could represent a cost-effective tool to implement in resource-limited contexts such as in sub-Saharan Africa.     

Executive function performance on the children's kitchen task assessment with children with sickle cell disease and matched controls

Objective: The aim of this study was to compare executive function abilities of 8- to 12-year-old children with sickle cell disease (SCD) with a matched control sample. The measures included the parent and teacher Behavior Rating Inventory of Executive Function (BRIEF); the Delis-Kaplan Executive Function System (D-KEFS), Free Sorting Test and Color Word Interference Test; and the Children's Kitchen Task Assessment (CKTA), a new performance measure. Methods: Twenty-two children with SCD were recruited from one hematology clinic and 22 community children, matched on characteristics of age, gender, and race, were selected from a larger sample of controls for comparison. Parents and teachers completed rating scales. Results:As hypothesized, children with SCD scored significantly lower than matched controls on Digit Span Forward; on 5 of the 9 D-KFES Color Word Interference and Sorting tasks; on CKTA organization, initiation, and task completion; and on the BRIEF's parent and teacher Metacognitive Index (MI) and Global Executive Composite (GEC) scores. Conclusion:Cognitive and performance evaluations indicate lower executive function among children with SCD. Results substantiate the need for evaluative triangulation for children with SCD: Neurocognitive testing supported by performance testing, and adult reflection of a child's daily performance compared to other children. These elements will provide rich data to create educational support for children with SCD who have frequent hospitalizations, school absences, and the potential presence of cerebral vascular accident symptomology.     

Written emotional expression and emotional well-being: the moderating role of fear of rejection

Empirical research shows that individuals high in fear of rejection typically report low levels of perceived social support and are more vulnerable to stressful experiences. At the same time, writing about stressful experiences in an emotional way seems to help people adapt to current stressors and not-yet-assimilated stressful experiences. Therefore, the authors suggest that written emotional expression may be a particularly effective strategy to manage negative emotions for individuals high in fear of rejection. Three studies were conducted to test these assumptions. Study 1 found that high fear of rejection is linked to a lack of perceived social support. Longitudinal Studies 2 and 3 supported our main hypothesis, demonstrating that written emotional expression is linked to lower levels of negative mood among individuals high (but not among individuals low) in fear of rejection.