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.     

Health-Related Quality of Life and Adaptive Behaviors of Adolescents with Sickle Cell Disease: Stress Processing Moderators

The objective of this study was to examine resilience among adolescents with sickle cell disease (SCD), focusing on the interaction of health-related quality of life with stress processing to explain adaptive behavior. Forty-four adolescents with SCD completed paper-and-pencil measures of health-related quality of life, appraisals (hope), pain coping strategies (e.g. adherence), and adaptive behavior. Self-reported health-related quality of life was significantly associated with adaptive behavior, as was adherence. Findings for moderation were mixed. Pain coping strategies moderated the association of health-related quality of life with adaptive behavior such that at lower levels of Coping Strategies Questionnaire (CSQ) Adherence, better quality of life was associated with higher adaptive behavior. Similarly, at higher levels of hope, better quality of life was associated with higher adaptive behavior, and poorer quality of life was associated with lower adaptive behavior. Adolescents with SCD showed resilience, particularly in terms of personal adjustment, that may be explained by their appraisals and stress processing strategies. Interventions to support an optimistic or hopeful outlook and improve adherence to recommendations for medical management of sickle cell pain may result in improved resilience/adaptive behavior.     

Neuropsychological functioning and social functioning of survivors of pediatric brain tumors: evidence of nonverbal learning disability.

The purpose of the study was to examine if survivors of pediatric brain tumors exhibit a pattern of performance consistent with nonverbal learning disability (NVLD) and to explore the relationship between neuropsychological and social functioning in these children. A comprehensive neuropsychological battery and objective measures of psychosocial function designed to assess NVLD were administered to 15 survivors of brain tumors, ages 8-12 years. Despite the small sample size, a trend for better verbal skills compared to nonverbal skills was found using composite scores. Parents reported significant social deficits and a tendency for greater internalizing behavior problems as expected in NVLD. Additionally, there was a trend for a positive association between nonverbal scores and social function. Further research is needed to determine if the NVLD pattern observed is attributable to white matter damage of the right hemisphere. Routine neuropsychological and psychosocial assessment and intervention are indicated.     

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.     

Optimism,appraisals, and coping in the adjustment of mothers and their children with spina bifida

Families of children with physical disabilities show substantial differences in the levels of adjustment of both the children and their parents. These differences result, in part, from the complex interplay of family and child adaptation resources, such as coping and social support. In order to identify factors which may differentiate levels of adjustment among families with children with physical disabilities, this study examined relationships among optimism, primary and secondary appraisals of and coping with child-related stressors, maternal psychological adjustment, and child adjustment in 29 families with a child with spina bifida without mental retardation and 28 comparison families with a nondisabled child. For the spina bifida group, primary appraisals were related to the use of avoidant coping strategies, and these coping strategies were related to maternal psychological adjustment and child internalizing behavior problems. No significant differences between the groups in terms of the nature of the relationships or in the levels of the variables were found with the exception that mothers of children with spina bifida were less optimistic than comparison mothers. These data support the importance of coping in understanding the psychological adjustment of mothers faced with a chronic stressor in the family.     

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.     

Posttraumatic Stress Symptoms and Stressful Life Events Predict the Long-term Adjustment of Survivors of Childhood Cancer and Their Mothers

Objectives: To examine prospectively the impact of posttraumatic stress symptoms (PTSS) in response to childhood cancer and treatment on general adjustment while accounting for the role of other stressful life events and appraisal of life threat and treatment intensity. Methods: As part of a larger study assessing PTSS, 56 childhood cancer survivors, aged 8 to 18, and 65 mothers completed self-report measures of PTSS and appraisal of the intensity and life threat associated with cancer treatment at time 1 and self-report measures of stressful life events and general adjustment at time 2 (approximately 18 months after time 1). Results: For child survivors and their mothers, posttraumatic stress at time 1 significantly predicted general adjustment at time 2, over and above the significant contribution of lifetime stressful events. The life events variable did not function as a mediator in the association of PTSS and general adjustment. Conclusions: Trauma responses to childhood cancer and its treatment have implications for the long-term adaptation of children and their families. Early signs and symptoms of posttraumatic stress and stressful life experiences require early assessment and intervention.     

Surviving cancer competently intervention program (SCCIP): a cognitive-behavioral and family therapy intervention for adolescent survivors of childhood cancer and their families.

Psychological reactions to having had childhood cancer often continue after treatment ends, for survivors and their parents. Based on our previous research, we developed an intervention program for adolescent survivors of childhood cancer, their parents, and siblings. Surviving Cancer Competently: An Intervention Program--SCCIP--is a one-day family group intervention that combines cognitive-behavioral and family therapy approaches. The goals of SCCIP are to reduce symptoms of distress and to improve family functioning and development. SCCIP is described and data from a pilot study of 19 families are presented. Program evaluation data indicated that all family members found SCCIP helpful. Standardized measures administered before the intervention and again at 6 months after SCCIP showed that symptoms of posttraumatic stress and anxiety decreased. Changes in family functioning were more difficult to discern. Overall, the results were promising with regard to the feasibility of the program and its potential for reducing symptoms of distress for all family members.