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.     

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.     

Neuropsychological Aspects of Pediatric Sickle Cell Disease

Sickle cell disease (SCD), a class of genetic disorders characterized by abnormal, sickled red blood cells, is a chronic illness that results in progressive cerebrovascular disease. Neurocognitive sequelae of clinically apparent cerebrovascular accidents in children with SCD are characterized by pervasive impairments, including decrements in general intellectual functioning, language and verbal abilities, visual-motor and visual-spatial processing, memory, academic achievement, and processing of subtle prosodic information. In contrast, subtle neurocognitive deficits in the areas of attention and concentration, executive function, and visual-motor speed and coordination appear to be associated with silent infarcts that are not necessarily detected on physical examination. Investigation of the disease course and associated neurocognitive sequelae suggest a disease-specific model of neuropsychological impairment. Recommendations are made for clinical and research efforts in the field of pediatric neuropsychology.     

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.     

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.     

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.     

Psychosocial Considerations in Sickle Cell Disease (SCD): The Transition from Adolescence to Young Adulthood

Sickle cell disease (SCD) occurs in 1 of every 400 to 500 African American births. Despite its prevalence, there has been a relative dearth of attention paid to SCD in the psychological literature. African American SCD adolescents and young adults, individuals at an already vulnerable developmental stage, are at increased risk for psychological distress and psychosocial impairments (e.g., depression and low self-esteem, poor social and academic/vocational functioning). Such difficulties often result in problematic adherence to medical regimens, and thus represent a potentially serious clinical and public health problem that merits the attention of psychologists working in medical settings. This article (1) reviews the psychosocial needs peculiar to SCD patients transitioning from adolescence to young adulthood; (2) applies a culturally informed stress-coping-adjustment framework to assess and address the psychological status, psychosocial functioning, and disease outcomes of these individuals; and (3) describes a culturally and developmentally sensitive, psychoeducational group intervention.     

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.     

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.     

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.     

Applying the Transactional Stress and Coping Model to Sickle Cell Disorder and Insulin-Dependent Diabetes Mellitus: Identifying Psychosocial Variables Related to Adjustment and Intervention

This review paper examines the literature on psychosocial factors associated with adjustment to sickle cell disease and insulin-dependent diabetes mellitus in children through the framework of the transactional stress and coping (TSC) model. The transactional stress and coping model views adaptation to a childhood chronic illness as mediated by several psychosocial factors. This review examines the utility of the model in explaining adjustment in two different childhood diseases, identifies needed research and intervention targets, as well as highlights potential changes to the model. The major conclusions of this review suggest that, in addition to child-specific factors, family functioning is an area that interventions should address in sickle cell disease and insulin-dependent diabetes mellitus.     

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.     

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.     

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.     

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.     

Neuropsychologic Deficits in Children with Sickle Cell Disease and Cerebral Infarction: Role of Lesion Site and Volume

Little is known about the correlation between the location and size of cerebral infarction in children and neuropsychologic deficits. We related lesion location and volume on magnetic resonance exams to neuropsychologic performance in 28 children with cerebral infarcts due to sickle cell disease. Seventeen healthy siblings served as a comparison group. Children with anterior cerebral infarcts (n = 7) showed deficits in attention and executive skills, whereas children with more widespread cerebral infarcts (n = 18) showed additional deficits in spatial skills. The volume of cerebral infarction was associated with spatial and language performance, but minimally related to performance in other cognitive domains. The location and volume of cerebral infarction are both important for defining the type and magnitude of cognitive sequelae in childhood stroke.     

Everything is What it is,and Not Another Thing: Knowledge and Freedom in Isaiah Berlin's Political Thought

Although Isaiah Berlin's critique of positive liberty has achieved canonical status, its place within his wider political philosophy remains obscure. However, the re-publication of one of his most important philosophical essays, From Hope and Fear Set Free, as part of a new edition of Four Essays on Liberty, simply entitled Liberty, has opened the door to a re-evaluation of Berlin's political project.

At the heart of Berlin's argument, which gains its fullest expression in From Hope and Fear Set Free, stands his contention that positive liberty, in its incarnation as self-realisation, conflates liberty with knowledge, which leads to the distortion of our phenomenologically inescapable structure of experience. This transgression against the conceptual framework in terms of which we think, Berlin argues, played its desolate part in linking the ‘totalitarian temptation’ latent in liberty as self-realisation with the totalitarian politics of the mid-twentieth century.     

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.