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.     

Hypoxia and Inflammation in Children with Sickle Cell Disease: Implications for Hippocampal Functioning and Episodic Memory

Children with sickle cell disease (SCD) suffer from systemic processes (e.g., chronic anemia, recurrent hypoxic-ischemic events, chronic inflammation) that have been associated with neurocognitive impairment in a range of clinical populations, but which have been largely understudied in relation to specific domains of cognitive functioning in children with SCD. This review focuses on episodic memory, as the hippocampus may be especially vulnerable to the systemic processes associated with SCD. The first part of the paper outlines the pathophysiology of SCD and briefly reviews the extant literature on academic and cognitive functioning in children with SCD, emphasizing the dearth of research on episodic memory. Next, the complex systemic processes of hypoxia and inflammation associated with SCD are reviewed, along with research that has associated these processes with hippocampal damage and memory impairment. The paper concludes with suggestions for future research that are informed, in part, by the literature on developmental amnesia.     

Executive functioning and health-related quality of life in pediatric sickle cell disease

Research consistently indicates that children with sickle cell disease (SCD) face multiple risk factors for neurocognitive impairment. Despite this, no empirical research to date has examined the impact of neurocognitive functioning on quality of life for this pediatric group. Thus, the current study aims to examine the relationship between executive functioning and quality of life in a sample of children with SCD and further explore psychosocial and family/caregiver resources as moderators of this relationship. A total of 45 children with SCD aged 8 to 16 years and their caregivers completed measures of quality of life, behavioral ratings of executive functioning, and psychosocial functioning. Hierarchical linear regression models were utilized to determine the impact of executive functioning on quality of life and further test the interaction effects of proposed moderating variables. Controlling for age, pain, and socioeconomic status (SES), executive functioning was found to significantly predict child- and parent-reported quality of life among youth with SCD. Psychosocial resources of the primary caregiver or family was not found to moderate the relationship between executive functioning and quality of life. These results provide the first empirical evidence that lower executive skills negatively predict quality of life for children with SCD, supporting clinical and research efforts which aim to establish efficacious interventions that target cognitive decrements within this pediatric population.     

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.     

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.     

Sickle cell disease as a neurodevelopmental disorder

Sickle cell disease (SCD) is a blood disorder; however, the central nervous system (CNS) is one of the organs frequently affected by the disease. Brain disease can begin early in life and often leads to neurocognitive dysfunction. Approximately one-fourth to one-third of children with SCD have some form of CNS effects from the disease, which typically manifest as deficits in specific cognitive domains and academic difficulties. We discuss SCD as a neurodevelopmental disorder by reviewing the mechanisms of neurological morbidity in SCD, the timing of these mechanisms, the types of cognitive and behavioral morbidity that is typical, and the interaction of social-environmental context with disease processes. The impact of the disease on families shares many features similar to other neurodevelopmental disorders; however, social-environmental factors related to low socioeconomic status, worry and concerns about social stigma, and recurrent, unpredictable medical complications can be sources of relatively higher stress in SCD. Greater public awareness of the neurocognitive effects of SCD and their impact on child outcomes is a critical step toward improved treatment, adaptation to illness, and quality of life.     

Predictors of Psychosocial and Cognitive Adaptation in Children with Sickle Cell Syndromes

Examined the role of socioecological factors (family functioning and socioeconomic variables) in predicting behavioral and cognitive adaptation in children with sickle cell syndromes (SCD). Participants included 74 African American children and adolescents with SCD and their primary caretakers. Outcomes included internalizing and externalizing behavior symptoms, as rated by caretakers, as well as intellectual abilities and academic achievement, as assessed by individually administered standardized instruments. Family functioning consisted of both caretaker adjustment and family environment (i.e., family adaptability and cohesion). Hierarchical multiple regression analyses revealed that caretaker psychological adjustment predicted externalizing behaviors, while family environment (i.e., adaptability and cohesion) predicted neither behavioral nor cognitive functioning. Socioeconomic variables alone predicted intellectual abilities and academic achievement and also contributed to the prediction of child behavioral adaptation. Results are discussed in terms of relevant sociocultural issues and implications for family-level prevention and intervention.     

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.     

A Blueprint for Social Cognitive Development

ABSTRACT— The field of social cognitive development (SCD) has historically failed to emerge as a dominant approach in developmental psychology. We take this opportunity to articulate the assumptions, goals, and contributions of SCD with the aim of invigorating research from this perspective. We begin by describing the current landscape of social and cognitive development, suggesting what they have and have not given us. We then outline major goals of the social cognitive developmental approach and walk through examples of successful SCD research. Finally, we examine the unique potential of the social cognitive approach to cross-fertilize social and cognitive development (as well as related fields such as social psychology and neuroscience) and to answer new questions about development.     

Sickle cell disease: primary stroke prevention

Stroke is an important and common complication of sickle cell disease (SCD), affecting children as well as adults. Clinically evident stroke, usually brain infarction, is usually associated with stenosis or occlusion of the intracranial arteries of the Circle of Willis, sometimes with formation of moyamoya (a Japanese word for "hazy" or "like a puff of smoke" that describes the appearance of a abnormal microvasculature on angiography believed secondary to internal carotid artery stenosis or occlusion and the resultant extensive collateralization). Several types of intracranial hemorrhage are observed but usually in older children and adults. Cerebrovascular diseases restricted to small vessels may go unrecognized but is associated with cognitive and learning problems. Prevention of recurrent stroke has been accomplished with chronic blood transfusion. A primary prevention strategy for clinical stroke, based on the Stroke Prevention in Sickle Cell Anemia Trial, has been tested in a randomized clinical trial. Over 2,000 young children with SCD were screened with transcranial Doppler ultrasound (TCD) to detect elevated blood flow velocity indicative of vessel disease and high risk of future stroke. Those randomized to standard care (no transfusion) had a 10%/year risk of stroke, which was reduced >90% with chronic transfusion. This approach is the only primary stroke prevention strategy so far tested in SCD in a randomized controlled trial. Silent lesions on magnetic resonance imaging are associated with an approximately 1.5%/year risk of clinical stroke and a trial is now starting in children with these lesions who do not meet Stroke Prevention in Sickle Cell Anemia Trial criteria for transfusion based on TCD. A controlled trial, based on intervention for nocturnal hypoxemia, is also underway. Hydroxyurea, bone marrow transplantation, antiplatelet, and antithrombotic agents may work but have not been tested in primary prevention in a systematic way. If early and repeated, TCD screening of children, as recommended by National Heart Lung and Blood Institute and the American Stroke Association, were implemented broadly the incidence of new strokes could be greatly reduced in these children.     

Parental Problem-Solving Abilities and the Association of Sickle Cell Disease Complications with Health-Related Quality of Life for School-Age Children

Children with sickle cell disease (SCD) are at risk for poor health-related quality of life (HRQOL). The current analysis sought to explore parent problem-solving abilities/skills as a moderator between SCD complications and HRQOL to evaluate applicability to pediatric SCD. At baseline, 83 children ages 6–12 years and their primary caregiver completed measures of child HRQOL. Primary caregivers also completed a measure of social problem-solving. A SCD complications score was computed from medical record review. Parent problem-solving abilities significantly moderated the association of SCD complications with child self-report psychosocial HRQOL (p = .006). SCD complications had a direct effect on parent proxy physical and psychosocial child HRQOL. Enhancing parent problem-solving abilities may be one approach to improve HRQOL for children with high SCD complications; however, modification of parent perceptions of HRQOL may require direct intervention to improve knowledge and skills involved in disease management.     

Individual and Family Contributions to Depressive Symptoms in African American Children with Sickle Cell Disease

Depressive disorders in children with chronic illnesses have received little attention despite some evidence suggesting that these children experience increased psychological difficulties as a result of their medical condition. Most of the research on depressive disorders among children has been conducted primarily with Caucasian youth, limiting generalizability to ethnically diverse populations. Further, even less research exists on depressive disorders among African American children with Sickle Cell Disease (SCD) and for African American youth from lower socioeconomic status who are at greater risk for depressive disorders. This paper is an extensive review of the major studies conducted on depression and psychosocial aspects of SCD in African American children. The Transactional Stress and Coping model is provided as a conceptual framework. Following the review, clinical and research implications are provided, as well as an illustration of a psychoeducational family intervention with an African American child who has SCD and internalizing behaviors.     

Peer relationships and emotional well-being of children with sickle cell disease: a controlled replication.

We completed a replication study examining the social and emotional functioning of children with sickle cell disease (SCD) who have not had an overt stroke and a group of demographically similar comparison classmates based upon data from multiple informants. Relative to comparison peers, children with SCD were described by teachers as more prosocial and less aggressive. Peers described them as having fewer friends, less athletic, ill more often, and missing more school. No group differences were identified for emotional well-being. Effect size analyses indicated that this study replicated a number of findings from our previous study of children with SCD (Noll et al., 1996). Findings from both studies suggest relative psychological hardiness among children with SCD who have not had an overt stroke.     

Hypoxic adaptation during development: relation to pattern of neurological presentation and cognitive disability

Children with acute hypoxic-ischaemic events (e.g. stroke) and chronic neurological conditions associated with hypoxia frequently present to paediatric neurologists. Failure to adapt to hypoxia may be a common pathophysiological pathway linking a number of other conditions of childhood with cognitive deficit. There is evidence that congenital cardiac disease, asthma and sleep disordered breathing, for example, are associated with cognitive deficit, but little is known about the mechanism and whether there is any structural change. This review describes what is known about how the brain reacts and adapts to hypoxia, focusing on epilepsy and sickle cell disease (SCD). We prospectively recorded overnight oxyhaemoglobin saturation (SpO2) in 18 children with intractable epilepsy, six of whom were currently or recently in minor status (MS). Children with MS were more likely to have an abnormal sleep study defined as either mean baseline SpO2 <94% or >4 dips of >4% in SpO2/hour (p = .04). In our series of prospectively followed patients with SCD who subsequently developed acute neurological symptoms and signs, mean overnight SpO2 was lower in those with cerebrovascular disease on magnetic resonance angiography (Mann-Whitney, p = .01). Acute, intermittent and chronic hypoxia may have detrimental effects on the brain, the clinical manifestations perhaps depending on rapidity of presentation and prior exposure.     

Change in the Psychological Adjustment of Children with Cystic Fibrosis or Sickle Cell Disease and Their Mothers

Objectives: This study has three interrelated objectives: (1) to track the adjustment of children and adolescents with sickle cell disease (SCD) or cystic fibrosis (CF) and their mothers through a third assessment point 2 years after the initial assessment; (2) to determine whether the adaptational processes of the transactional stress and coping model associated with adjustment at the initial assessment continue to be associated with adjustment 2 years later; and (3) to determine whether the pattern of association of adaptational processes with adjustments differs by illness subgroup. Methods: The study samples included 59 children with CF and 50 children with SCD and their mothers. Measures were obtained on maternal adjustment and appraisals of daily and illness stress, coping methods, and family functioning. Child measures included child-reported and mother-reported child adjustment and child perceptions of self-worth and health locus of control and pain coping methods. Results: Consistency in adjustment classification was only 31–32% for child self-report, 66% for mother-reported child behavior problems, and 56–77% for mother self-reported adjustment for the CF and SCD groups, respectively. Support was provided for the association of adaptational processes with maternal adjustment and with the adjustment of children with SCD but not for children with CF. Conclusion: The stability of adjustment has implications for prevention and treatment intervention and subsequent research steps. Intervention efforts should be focused on the relatively small subgroups of children with chronic illnesses and their mothers with consistently poor adjustment.     

Bilingualism and cognitive development in relation to threshold theory

A study was conducted to examine the relationship between bilingualism and cognitive development, as predicted by threshold theory. This theory maintains that there may be levels of linguistic proficiency which bilingual children must attain in order to avoid cognitive deficits and to allow the cognitive benefits. On the whole, the results were consistent with the theory, in that an overall bilingual superiority was found only for those children who had attained a high degree of bilingualism. An overall bilingual superiority was not found for those children who had attained lower degrees of bilingualism. These results are discussed further in relation to Bialystok's model.The author is indebted to G. Tyson for valuable comments on an earlier version of this paper.     

Predictors of Social Anxiety in Children and Adolescents with Sickle Cell Disease

The aim of this study was to examine the potential moderating effect of age on the child-reported pain–social anxiety relationship in children and adolescents with sickle cell disease (SCD). Participants were children and adolescents (ages 8–17; 33 girls, 25 boys) diagnosed with SCD who completed measures of social anxiety and severity of usual pain. Caregivers provided demographic information, and mean hemoglobin levels were computed as a measure of objective disease severity. Ratings of more severe pain were associated with greater social anxiety, including fear of negative evaluation, for older children and adolescents only, revealing a moderating effect of age. Increased relevancy of peer relationships in adolescence, limited social contacts due to SCD complications, and misreading of social cues (e.g., maladaptive coping response to pain) may explain why older children and adolescents reported greater social anxiety in the presence of a stressor such as pain.     

Psychosocial Stressors of Sickle Cell Disease on Adult Patients in Cameroon

Sickle Cell Disease (SCD) is a debilitating illness that affects quality of life. Studies of the psychosocial burden of SCD on patients have been rarely reported in Africa. We used a quantitative method, with face-to-face administered questionnaires, to study indices of psychosocial stressors on adult SCD patients in Cameroon. The questionnaire included a 36-item stress factors scale evaluating general perceptions of stress and five main stressors’ domain: disease factors, hospital factors, financial factors, family factors and quality of personal-life factors. Items pertaining to psychosocial stressors involved four response options with increasing severity: 0, 1, 2 or 3. Non-parametric tests were used for analysis. The majority of the 83 participants were urban dwellers, female, 20–30 years old, single, unemployed, with at least a secondary or tertiary education. Median age at diagnosis was 100 months; 47.8 % had >3 painful vaso-oclusive crises annually. Only 4.8 % had been treated with hydroxyurea. The majority reported moderate to severe difficulty coping with SCD. The “degree of clinical severity” category displayed the highest median score (2.0), while familial stressors showed the lowest (0.8). Being female, married, with low education level, an additional affected sibling and low direct income were significantly associated with specific stressors’ categories. In Cameroon, there is an urgent need to implement policies that ensure affordable access to health-care and practices to reduce SCD morbidity and improve patients’ quality of life.     

Cognitive Behavioral Science behind the Value of Play: Leveraging Everyday Experiences to Promote Play,Learning, and Positive Interactions

ABSTRACT

Participating in play affords physical, social, and cognitive benefits. Here, we review the cognitive behavioral science literature highlighting the value of play and describe the different types of play along with the evidence linking play to positive outcomes for children in areas such as social-emotional, cognitive, academic, and social-emotional development. Several case studies demonstrate how educators, caregivers, and community members can integrate low-cost, evidenced-based playful learning interventions into community settings to impact children where they live.