Reem A. Tarazi

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.

The Role of Neuropsychological Evaluation in Pediatric Sickle Cell Disease

Neuropsychological impairment occurs in children and adolescents with sickle cell disease. While the neuropsychological sequelae associated with cerebrovascular disease in these children have been identified, a well-informed clinical approach to neuropsychological evaluation for these vulnerable children has not been delineated. Moreover, issues related to standard of care for children and adolescents with sickle cell disease and transition to adulthood remain understudied despite the relevance to long-term medical, psychosocial, and neuropsychological outcomes. We provide recommendations for an informed approach to neuropsychological evaluation of youth with sickle cell disease that is determined by pertinent illness-related factors, consideration of general developmental level and timing of evaluation (i.e., baseline vs follow-up evaluation), relationship to medical treatment, and transition to adulthood. Although the neuropsychological methods presented in this manuscript are specific to sickle cell disease, this empirically informed approach may serve as a model for other pediatric populations.

Predictors of Academic Achievement for School-Age Children with Sickle Cell Disease.

Children with sickle cell disease (SCD) are at risk for neurocognitive impairment and poor academic achievement, although there is limited research on factors predicting academic achievement in this population. This study explores the relative contribution to academic achievement of a comprehensive set of factors, such as environmental (socioeconomic status), disease-related (stroke, transfusion therapy, adherence) and psychosocial variables [child behaviour, child quality of life (QoL)], controlling for intellectual functioning (IQ). Eighty-two children with SCD completed measures assessing IQ and academic achievement, while parents completed questionnaires assessing adherence, child behaviour and child QoL. Medical chart reviews were conducted to determine disease-related factors. Hierarchical regression analyses indicated that 55% of the variance in academic skills were accounted for by IQ, parent education, chronic transfusion status and QoL [R 2 = 0.55, F (5,77) = 18.34, p < 0.01]. Follow-up analyses for broad reading [R 2 = 0.52, F (5,77) = 16.37, p < 0.01] and math calculation [R 2 = 0.44, F (5,77) = 12.14, p < 0.01] were also significant. The findings suggest a significant contribution of factors beyond IQ to academic achievement. Findings allow for identification of children with SCD at risk for academic difficulties for whom psychoeducational interventions may enhance academic achievement.

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.

Psychosocial and Pharmacological Management of Pain in Pediatric Sickle Cell Disease

Abstract For children with sickle cell disease (SCD), pain is associated with significant current and future morbidity and mortality. Unfortunately, few evidence–based guidelines exist for the management of pain episodes in children with SCD. To inform empirically based treatment strategies for pain management in pediatric SCD, this review integrates and evaluates the extant literature on psychosocial and pharmacological approaches to the management of pain. Findings reveal a paucity of rigorous investigations of psychosocial and pharmacological pain management interventions in children with SCD. Psychosocial interventions included were primarily cognitive–behavioral in nature, whereas pharmacological approaches targeted non–opioid analgesics (ie, nonsteroidal anti–inflammatory drugs and corticosteroids) and opioid medications (ie, morphine and oxycodone). However, to date there is not a “gold standard“ for pain management among children with SCD. Because psychosocial and physiological processes each play a role in the etiology and experience of pain, effective pain management requires multidimensional, comprehensive treatment approaches. Considering the significant impact of pain on functional outcomes and quality of life among children with SCD, additional clinical trials are warranted to ensure that interventions are safe and efficacious.

The Kennedy Krieger Independence Scales-Spina Bifida Version: a measure of executive components of self-management

PURPOSE/OBJECTIVE Successful implementation of functional self-care skills depends upon adequate executive functioning; however, many scales assessing adaptive skills do not address the inherent executive burden of these tasks. This omission is especially relevant for individuals with spina bifida, for whom medical self-care tasks impose a significant burden requiring initiation and prospective memory. The Kennedy Krieger Independence Scales-Spina Bifida Version (KKIS-SB) is a caregiver-reported measure designed to address this gap; it assesses skills for managing both typical and spina bifida-related daily self-care demands, with a focus on the timely and independent initiation of adaptive skills. RESEARCH METHOD/DESIGN Parents of 100 youth and young adults with spina bifida completed the KKIS-SB. Exploratory factor analysis and Pearsons correlations were used to assess the factor structure, reliability, and construct validity of the KKIS-SB. RESULTS The scale demonstrates excellent internal consistency (Cronbachs alpha = .891). Exploratory factor analysis yielded four factors, explaining 65.1% of the total variance. Two primary subscales were created, initiation of routines and prospective memory, which provide meaningful clinical information regarding management of a variety of typical (e.g., get up on time, complete daily hygiene routines on time) and spina bifida-specific self-care tasks (e.g., begin self-catheterization on time, perform self-examination for pressure sores). CONCLUSIONS/IMPLICATIONS Based upon internal consistency estimates and correlations with measures of similar constructs, initial data suggest good preliminary reliability and validity of the KKIS-SB.

Family Functioning, Medical Self-Management, and Health Outcomes Among School-Aged Children With Sickle Cell Disease: A Mediation Model

Background Informed by the Pediatric Self-Management Model, the present study tested relationships between parent and family functioning, sickle cell disease (SCD) self-management, and health outcomes for children with SCD. Method 83 children with SCD and a parent completed baseline data as part of a larger investigation of a family-based, problem-solving intervention for children with SCD (M age = 8.47). Youth and parents completed a measure of child health-related quality of life (HRQOL), and parents completed measures of family efficacy, parenting stress, and SCD self-management. SCD pain episodes and urgent health utilization information over the past year were obtained via medical chart review. Results SCD self-management mediated the relationship between parent-reported family efficacy and parent proxy HRQOL, as well as the relationship between parenting stress and child and parent proxy HRQOL. Mediation models were nonsignificant for outcomes beyond HRQOL, including SCD pain episodes and urgent health utilization. Conclusion Fostering family efficacy and reducing parenting stress may be meaningful intervention targets for improving SCD self-management and child HRQOL among school-aged children. Although findings were consistent with the Pediatric Self-Management Model in terms of HRQOL, the model was not supported for pain episodes or urgent health utilization, highlighting the need for multi-method, longitudinal research on the SCD self-management behaviors that are linked to preventable health outcomes.

The kennedy krieger independence scales-sickle cell disease: Executive components of transition readiness

Purpose/Objective: Youth with sickle cell disease (SCD) are at an increased risk for executive dysfunction and simultaneously have increased self-management needs compared to typical adolescents. This unique combination may contribute, in part, to difficulties during transition to young adulthood. Current measures assessing adaptive skills do not assess the executive components (e.g., initiation, prospective memory) of SCD-related self-care tasks. Modeled on the KKIS-Spina Bifida (Jacobson et al., 2013), the Kennedy Krieger Independence Scales-Sickle Cell Disease (KKIS-SCD) is a new caregiver-report measure that assesses independence with self-management of SCD-specific demands as well as routine daily activities in adolescents with SCD. Research Method/Design: Thirty-three youth with SCD and their caregivers participated in this preliminary validation study examining the construct validity of the KKIS-SCD total and composite scores (Initiation of Routines, Prospective Memory) and exploring relationships of this measure with intellectual functioning, demographic factors, illness severity, and age. Results: The KKIS-SCD exhibited generally good internal consistency (Cronbach’s alpha = .733 to .803), and demonstrated evidence for construct and discriminant validity when compared to an existing measure of adaptive function. The KKIS-SCD was significantly associated with caregiver-report of executive behaviors but not with intellectual functioning, demographic factors, illness severity, or age. Conclusions/Implications: Results provide preliminary support for the KKIS-SCD as a reliable and valid tool for the assessment of executive components of self-care management skills for youth with SCD. Identifying specific weaknesses in executive function related to self-care management skills might assist in guiding intervention and individualizing transition planning in these at-risk youth.

Executive Functioning Among College Students With and Without History of Childhood Maltreatment

ABSTRACT This study utilized performance-based and self-report measures to examine differences in executive functions in college students with and without histories of childhood maltreatment. On the performance-based measures of executive function, all scores fell within the normal range for age. However, participants in the childhood maltreatment group reported more problems with metacognition than those without history of maltreatment. Severity of childhood maltreatment was associated with poorer cognitive inhibition/switching and phonetic fluency. Although significant group differences were found on a laboratory-based task of executive function, performance across the other tasks was generally similar. However, those with a history of maltreatment reported more problems with metacognition, suggesting a potential avenue for brief interventions focused on improving metacognitive skills important for success in the college environment. Implications and suggestions for future research are discussed.