Sickle-cell disease contributes to cognitive impairment in children

An examination of how sickle-cell disease contributes to cognitive impairment in children. The definition, classification, and pathophysiology of sickle-cell disease is discussed to support the cognitive impairment seen within children with sickle-cell disease. The quality of life that children with sickle cell experience is also discussed as it plays a role in how children with sickle cell experience the disease. Therapeutic measures are also examined to discuss the possible interventions that can be taken to aide children with sickle cell manage the disease. After careful research, it is concluded that four factors directly cause compromised neurological function in children with sickle-cell disease; (1) recurrent micro infarction of the central nervous system; (2) chronic hypoxic damage to the brain or diminished pulmonary function; (3) sub-acute brain damage that occurred during bouts of hypoxia associated with events such as aplastic crisis, acute chest syndrome, and obstructive sleep apnea; and (4) chronic nutritional deficiency associated with increased metabolic demands. The therapeutic interventions that are discussed to aid in the management of sickle-cell disease are inhibition of hemoglobin S polymerization and reduction of the intracellular hemoglobin concentration

Spirituality And Religiosity In Adolescents With Sickle Cell Disease: A Descriptive Qualitative Study

Sickle cell disease (SCD) is a serious debilitating chronic illness and global health problem. Spirituality and religiosity have been shown to have positive correlations with their health outcomes. Research addressing the spiritual and religious needs of adolescents living with SCD is limited. The aim of this descriptive qualitative study was to examine how adolescents (Mage = 16.2 years) with SCD describe and experience spirituality and religiosity. Nine adolescents completed two semi-structured interviews. Sickle Cell Disease Interview Guides were developed using the Spiritual Development Framework developed by Benson & Roehlkepartain (2008) as a guide. The framework provided a foundation for conceptualizing the spiritual element of human development. Sickle Cell Disease Interview Guides were used to elicit information on adolescents\u27 beliefs, spirituality, and religiosity. Participant Demographic Forms were completed by each adolescent and used to collect information regarding their demographics, SCD histories, and religiosities. Parent Demographic Forms were completed by each parent and used to collect information regarding parents\u27 demographics and religiosities. Data were analyzed using a template analysis style and a concurrent analysis process of data reduction, data display, and conclusion drawing/verification. The Spiritual Development Framework was used as a guide in constructing components of the analysis template. Adolescents verbalized their thoughts regarding their spirituality and religiosity. The adolescents believed that religiosity is personal, meaningful, and should be respected. Four main themes emerged to include: spirituality and religiosity as coping mechanisms, shaping of identity, the influence of beliefs on health and illness, and the expectations for health care providers. The theme spirituality and religiosity as coping mechanisms included six threads: interconnecting with God, interconnecting with others, interconnecting with creative arts, scriptural metanarratives, transcendent experiences, and acceptance and finding meaning. The theme expectations for health providers included two threads to include: religiosity is private/personal/communal and sharing spiritual and religious beliefs is risky. The current study examined spirituality and religiosity as described and experienced by adolescents with SCD. The current study highlights the role of spirituality and religiosity in an age-specific population living with sickle cell disease. Exploring spirituality and religiosity may lead to innovative interventions improving quality of life

Systematic review of transition models for young people with long-term conditions: A report for NHS Diabetes.

Aims For many young people with Type 1 diabetes, transition from paediatric to adult care can result in a marked deterioration in glycaemic control. A systematic review assessed the effectiveness of transition models, or components of models, for managing the transition process in young people with long-term conditions, including Type 1 diabetes. This involved identifying (i) the main barriers and facilitators in implementing a successful transition programme, and (ii) the key issues for young people with long-term conditions and professionals involved in the transition process. Methods The following databases were searched from inception to August 2012: MEDLINE, EMBASE, PsychINFO, CINAHL, ASSIA, Social Services Abstracts, Academic Search Complete, Social Science Citation Index, Cochrane and Campbell Libraries. Selected studies included young people aged 11 to 25 diagnosed with long-term conditions who were in transition from paediatric to adult secondary health care services. Results 16 systematic reviews and 13 primary studies were included from 9992 records retrieved. No single transition model was uniquely effective. The most successful transitions centred around: young person-focused; age and developmentally appropriate content and delivery; self-management education; family participation; paediatric and adult collaboration; designated transition clinics; transition co-ordinator; young person’s portfolio; specific professionals training; multidisciplinary approach; structured process embedded in service delivery. There were no distinctive characteristics of condition-specific Type 1 diabetes services. Conclusion This important and timely review summarises the key factors that need to be considered for the development of transition programmes for young people with long-term conditions, including those with Type 1 diabetes

Adolescent coping with sickle cell disease: The role of parental understanding

The study aimed to investigate 1) the adjustment status of adolescents with sickle cell disease 2) whether pain coping strategies were significant predictors of adjustment outcomes and 3) the contribution of parental understanding to adjustment outcomes for adolescents. Fifty-one parent-child dyads participated. The study design was cross-sectional and questionnaire-based within a structured interview format. There was no evidence of increased psychological morbidity for adolescents with sickle cell disease when compared to population norms. Coping patterns of the sample were consistent with previous studies on adolescents with sickle cell disease. After controlling for age and frequency of pain, adolescents with high scores on Negative Thinking had more hospital admissions, more school absence, more adjustment difficulties and poorer quality of life. The construct of parental understanding was operationalised in the study from parental ratings of adolescent coping strategies. After controlling for age and frequency of pain, parental understanding significantly predicted adolescent outcomes across the four domains of hospital admissions, school absence, adjustment and quality of life. The findings highlight the importance of identifying parent-child patterns of communication in clinical work and strengthening the resources available within families that contribute toward successful adjustment of young people

The Impact of Pain on Executive Functioning via Anxiety in Youths with Sickle Cell Disease without a History of Stroke

Research indicates that youths with SCD experience increased levels of pain-related anxiety and executive functioning impairments, even in the absence of stroke. Research also indicates that pain and anxiety predict executive functioning and that anxiety might mediate the relation between pain and executive functioning difficulties. The current study sought to evaluate the direct associations among pain, anxiety, and executive functioning, and to examine whether anxiety mediates the relation between pain and specific executive functioning impairments in a sample of youths (age 10 to 19 years) with SCD with no history of stroke. Findings did not support the hypothesis that pain-crisis frequency and anxiety predict executive functioning. Further, they did not indicate that anxiety mediated the relation between pain-crisis frequency and executive functioning

Biopsychosocial Predictors of Quality of Life in Paediatric Patients With Sickle Cell Disease

Sickle cell disease (SCD) refers to a group of inherited blood disorders with considerable morbidity that causes severe pain, reduces life expectancy, and requires significant self-management. Acute painful episodes are the hallmark of SCD, but persistent daily pain is also highly prevalent in this population. Characterising the impact and experience of SCD-related morbidity (i.e., sleep disruption, frequent emergency department visits, cognitive dysfunction) on health-related quality of life (HRQOL) requires multiple assessment methods to best capture the underlying mechanisms. To gain a greater understanding of the effect of common symptom categories on HRQOL and to determine potential pain coping targets, the present study investigated whether demographic, socioeconomic, sleepiness, pain burden, frequency of emergency department (ED) visits, and cognition predicted HRQOL in a paediatric sample of patients with SCD. Our study was a secondary analysis of baseline assessment data of children with SCD aged 8-15 years (n = 30) in the Prevention of Morbidity in Sickle Cell Anaemia Phase 2b (POMSb2) randomised controlled clinical trial of auto-adjusting continuous positive airways pressure. Patients completed cognitive testing (IQ, Processing Speed Index, Delis-Kaplan Executive Function Scale (DKEFS) Tower, Conner's Continuous Performance Test), sleepiness (Epworth Sleepiness Scale), and HRQOL (PedsQL Sickle Cell Module) at baseline. Patients reported pain burden (Sickle Cell Pain Burden Inventory-Youth) each month over 8 visits. Caregivers provided demographic information and reported their child's executive function (Behavioural Rating Inventory of Executive Function) at baseline. Data from our analysis demonstrated that demographic factors (i.e., age, gender, level of neighbourhood deprivation) and treatment variables (i.e., hydroxyurea use) did not independently predict HRQOL, and laboratory values (i.e., haemoglobin, haematocrit, mean oxygen saturation) were not significantly correlated with HRQOL (ps > 0.05). However, sleepiness, pain burden, ED visits, and executive dysfunction independently predicted HRQOL (R 2 = 0.66) with large effects (η2 = 0.16 to 0.32). These findings identify specific, measurable symptom categories that may serve as targets to improve HRQOL that are responsive to change. This knowledge will be useful for multimodal interventions for paediatric patients with SCD that include sleep management, pain coping strategies, and executive function training

End points for sickle cell disease clinical trials: patient-reported outcomes, pain, and the brain

To address the global burden of sickle cell disease (SCD) and the need for novel therapies, the American Society of Hematology partnered with the US Food and Drug Administration to engage the work of 7 panels of clinicians, investigators, and patients to develop consensus recommendations for clinical trial end points. The panels conducted their work through literature reviews, assessment of available evidence, and expert judgment focusing on end points related to: patient-reported outcomes (PROs), pain (non-PROs), the brain, end-organ considerations, biomarkers, measurement of cure, and low-resource settings. This article presents the findings and recommendations of the PROs, pain, and brain panels, as well as relevant findings and recommendations from the biomarkers panel. The panels identify end points, where there were supporting data, to use in clinical trials of SCD. In addition, the panels discuss where further research is needed to support the development and validation of additional clinical trial end points