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.     

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.     

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.     

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.     

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.     

Age and Body Satisfaction Predict Diet Adherence in Adolescents with Inflammatory Bowel Disease

The aim of the current study was to determine whether age and body satisfaction predict dietary adherence in adolescents with Inflammatory Bowel Disease (IBD), and whether older females are less adherent than younger males and females. Forty-four participants aged 10–21 with IBD were recruited. Participants provided informed consent and demographics. Body satisfaction was measured by a questionnaire and a task in which participants selected their current and ideal body image out of silhouettes depicting bodies ranging from underweight to obese. Adherence was measured by marking a 100 mm visual analog scale, the 1-week completion of a dietary log, and a questionnaire evaluating coping strategies used for overcoming obstacles to dietary adherence. Age was related to dietary adherence, with younger children being more likely to adhere. Participants more satisfied with their body reported better dietary adherence. Findings remained consistent across multiple measures of body satisfaction and adherence. Healthcare providers and parents should be informed of these findings in order to improve adherence.     

养老机构冠心病患者服药依从性现状及影响因素

采用Morisky推荐的MAQ对锦州市养老机构中153名冠心痛患者的服药依从性进行测评,探讨冠心病患者的服药依从性现状及影响因素.结果显示,养老机构冠心病患者服药依从性差的比率为81.7％,影响服药依从性的因素为文化程度、服药种类、医疗背景、入住年限.养老机构的护理人员应对上述因素给予更多的关注,从而提高其服药依从性,进而提升其临床治疗效果.     

Evaluation of a Disease-Specific Self-Efficacy Instrument in Adolescents with Sickle Cell Disease and its Relationship to Adjustment

The psychometric properties of a disease-specific instrument used to assess self-efficacy in adolescents with sickle cell disease, the Sickle Cell Self-Efficacy Scale, were evaluated in a sample of 131 adolescents ranging from 11 to 19 years of age. This nine-item instrument was associated with a one-item, general self-efficacy question and an item of self-control. After controlling for age, gender, highest grade of education completed, and the number of individuals in the household, high levels of self-efficacy were related to fewer physical, psychological, and total symptoms. Using the stress process framework to examine the relationship between self-efficacy and self-reported symptoms in adolescents may lead to the initiation of effective intervention programs capable of increasing levels of self-efficacy in adolescents. These interventions could lead to better outcomes for adolescents with sickle cell disease. Additional longitudinal investigations are needed to evaluate the ability of self-efficacy to predict adolescent adjustment over time.     

Being, Doing, and Knowing: Developing Ethical Competence in Health Care

There is a growing interest in ethical competence-building within nursing and health care practising. This tendency is accompanied by a remarkable growth of ethical guidelines. Ethical demands have also been laid down in laws. Present-day practitioners and researchers in health care are thereby left in a virtual cross-fire of various legislations, codes, and recommendations, all intended to guide behaviour. The aim of this paper was to investigate the role of ethical guidelines in the process of ethical competence-building within health care practice and medical research. A conceptual and critical philosophical analysis of some paragraphs of the Helsinki Declaration and of relevant literature was performed. Three major problems related to ethical guidelines were identified, namely, the interpretation problem (there is always a gap between the rule and the practice, which implies that ethical competence is needed for those who are to implement the guidelines); the multiplicity problem (the great number of codes, declarations, and laws might pull in different directions, which may confuse the health care providers who are to follow them); and the legalisation problem (ethics concerns may take on a legal form, where ethical reflection is replaced by a procedure of legal interpretations). Virtue ethics might be an alternative to a rule based approach. This position, however, can turn ethics into a tacit knowledge, leading to poorly reflected and inconsistent ethical decisions. Ethical competence must consist of both being (virtues) and doing (rules and principles), but also of knowing (critical reflection), and therefore a communicative based model is suggested.     

Serial Mediators of Memory Support Strategies Used With Cognitive Therapy for Depression: Improving Outcomes Through Patient Adherence and Treatment Skills

Patient memory for treatment is poor. Memory support strategies can be integrated within evidence-based psychological treatments to improve patient memory for treatment, and thereby enhance patient outcomes. The present study evaluated possible mechanisms of these memory support strategies. Specifically, we tested whether therapist use of memory support strategies indirectly predicts improved patient outcomes via serial improvements in (a) patient adherence throughout treatment and (b) patient utilization and competency of treatment skills. Adults with major depressive disorder (N = 178, mean age = 37.93, 63% female, 17% Hispanic or Latino) were randomized to Cognitive Therapy plus a Memory Support Intervention or Cognitive Therapy-as-usual. Because therapists from both treatment groups used memory support strategies, data from conditions were combined. Blind assessments of depression severity and overall impairment were conducted before treatment, immediately posttreatment (POST), at 6-month follow-up (6FU), and at 12-month follow-up (12FU). Patient adherence to treatment was rated by therapists and averaged across treatment sessions. Patients completed measures of treatment mechanisms—namely, utilization and competency in cognitive therapy skills—at POST, 6FU, and 12FU. Results of serial mediation models indicated that more therapist use of memory support predicted lower depression severity at POST, 6FU, and 12FU indirectly and sequentially through (a) increased patient adherence during treatment and (b) more utilization and competency of Cognitive Therapy skills at POST, 6FU, and 12FU. The same patterns were found for serial mediation models predicting lower overall impairment at POST, 6FU, and 12FU. Together, boosting memory for treatment may represent a promising means to enhance pantreatment mechanisms (i.e., adherence and treatment skills) as well as patient outcomes.