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.     

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.     

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

Event-based prospective memory (EB-PM) is the formation of an intention and remembering to perform it in response to a specific event. Currently, EB-PM performance in children with sickle cell disease (SCD) is unknown. In this study, we designed a computer-based task of EB-PM; No-Stroke, Silent-Infarct, and Overt-Stroke groups performed significantly below the demographically similar control group without SCD. Cue distinctiveness was varied to determine if EB-PM could be improved. All groups, with the exception of the Overt-Stroke group, performed significantly better with a perceptually distinctive cue. Overall, these results suggest that EB-PM can be improved significantly in many children with SCD.     

Treatment Adherence in Children with Sickle Cell Disease: Disease-Related Risk and Psychosocial Resistance Factors

To identify disease-related risk factors and psychosocial resistance factors that impact adherence to prescribed treatment in the context of admission to a Hematology Acute Care Unit (HACU) designed to provide acute care for children with sickle cell disease (SCD) presenting with pain or fever. A total of 73 primary caregivers and 24 children (if age 8 or older) completed standardized forms during the HACU admission. Treatment adherence variables (medical staff rating, SCD-related care activities, percentage of agreement between treatment recommendations made and care activities, and attendance at hematology clinic) indicated moderate-to-high adherence. Based on regression analyses, the risk variable of disease-related stress and the resistance variables of family flexibility and less reliance on passive coping accounted for significant portions of the variance in treatment adherence. Empirical evaluation of interventions designed to improve communication regarding expectations for the care of children with SCD and to support active family problem solving during times of SCD-related stress must be ongoing.     

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.     

Peer Relationships and Emotional Well-Being of Children with Sickle Cell Disease: A Controlled Replication

This study investigated the effects of stimulus presentation modality on working memory performance in elementary school-age children ages 7–13. The experimental paradigm implemented a multitrial learning task incorporating three presentation modalities: Auditory, Visual, and simultaneous Auditory plus Visual. The first experiment compared the learning and memory performance of older and younger elementary school children. The second experiment compared verbal learning and memory performance in elementary school children with major depressive disorder (MDD) to the performance of nondepressed children. All participants benefited from the pictorial presentation of information during learning and recall of information as compared to the auditory presentation alone. Both age and socioeconomic status affected working memory performance in typically developing children. Children with depression demonstrated a more passive learning style during the auditory list acquisition. The present study supports the pictorial superiority hypothesis in verbal learning tasks and the theory that working memory matures during elementary school years. Furthermore, current results indicate that complex working memory measures are not entirely independent of previous experience.     

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.     

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.     

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.     

Caregiver Knowledge and Adherence in Children With Sickle Cell Disease: Knowing is Not Doing

Though the impact of disease knowledge on adherence to medical recommendations has long been the focus of research, little of this research has focussed on sickle cell compliance in the child and adolescent population. The present study examined the relationship of caregiver knowledge of sickle cell disease (SCD) and children's adherence to prescribed behaviors. Though caregivers with high levels of knowledge of SCD were more likely to report higher levels of adherence, knowledge test score did not predict adherence for the majority of participants (72%). The effect of caregiver knowledge on reported adherence was greater among preadolescents (age ≤ 11) than adolescents (age ≥12). The relationship between knowledge and adherence may not be as strong or direct as is assumed in many theories of health behavior change.     

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.     

Parental Locus of Control and Family Functioning in the Quality of Life of Children with Sickle Cell Disease

The objective was to examine the association of parental locus of control (PLOC) with pediatric quality of life (QOL) for children with sickle cell disease (SCD), specifically addressing the potential mediating role of family functioning. Associations with disease severity were also investigated. Thirty-one primary caregivers completed standard measures of parental locus of control, family functioning, and quality of life during their child's inpatient admission for pain or fever. Results indicated that the PLOC total score was significantly associated with QOL in terms of self-competence and showed marginal significance with QOL social competence; it was significantly negatively correlated with family functioning. The association of the PLOC with QOL subscales was maintained when controlling for family functioning. Disease severity was not associated with PLOC scores. Primary caregivers endorsed more external parental locus of control beliefs. Further investigation of the association of caregiver appraisals and family functioning is warranted with the goal of addressing the stress that disease management with sickle cell may add to parent–child relationships.     

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.     

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.     

Biopsychosocial Factors Associated with Parenting Stress in Pediatric Sickle Cell Disease

Caregivers of children with sickle cell disease (SCD) experience significant physical and emotional hardship with their child’s disease management. Little is known about the potential contributors to parenting stress in pediatric SCD. The present study aimed to identify child and caregiver biopsychosocial factors associated with disease-related parenting stress in pediatric SCD. Participants included 74 caregiver-youth dyads. Parenting stress was associated with increased child pain frequency, more missed school days, and increased healthcare utilization, and inversely correlated with caregiver mental health and social-emotional functioning. Parenting stress also partially explained the relationship between child pain frequency and healthcare utilization after controlling for parent depression and anxiety. Parenting stress may play a unique and critical role in pediatric SCD and underscore the impact parenting stress may have on youth in medical and academic settings. Further research is warranted to determine risk factors and appropriate interventions for parenting stress to improve comprehensive patient care.

     

Longitudinal Assessment of Pain, Coping, and Daily Functioning in Children with Sickle Cell Disease Receiving Pain Management Skills Training

Objective. To conduct intensive pain management skills training (IST) in children with sickle cell disease (SCD) and their parents and to comprehensively evaluate pain, coping, and daily functioning in children pre, immediately post, and 3 months following treatment. Methods. Three children who received IST in nonpharmacological and pharmacological pain management strategies completed a Coping Strategies Questionnaire (CSQ) at pre, post, and follow-up assessments, and daily pain and activity diaries for 18 weeks, spanning from 1 week pretreatment to 11 weeks posttreatment. Results. From pre- to posttreatment, 1 child receiving IST indicated increased report of active coping attempts and all 3 children indicated decreased report of negative thinking on the CSQ. Participants in IST used coping skills on 90% of days with pain and reported the skills to be moderately helpful on the daily diaries. For daily activities such as eating dinner, playing with friends, and hours slept, children participated similarly on days with pain and days without pain during the posttreatment period. Given the small number of participants in this study, individual cases are discussed to highlight similarities and differences in how participants responded to the treatment and during the 3-month follow-up period. Conclusions. In this pilot study, each participant showed improvement in coping and daily functioning after completing the IST program. Individual differences in response to treatment indicate the need for more targeted intervention programs that incorporate pharmacological and nonpharmacological components. The results of this study highlight both the promise and the complications of conducting comprehensive pain intervention and functional outcome studies in children with SCD.     

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.     

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.     

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.