Living with Hereditary Haemorrhagic Telangiectasia: stigma,coping with unpredictable symptoms,and self-advocacy

Abstract

Objective: Hereditary Haemorrhagic Telangiectasia (HHT) is a genetic condition causing frequent nose bleeds, skin lesions (telangiectasia) and arteriovenous malformations. Approximately, 50% of people experience life-threatening HHT symptoms including haemorrhages in the brain, lungs and liver. This study aimed to gain a qualitative understanding of the psychosocial impact of HHT over time. Design: Using a phenomenological framework, a rigorous narrative analysis was performed on 20 semi-structured interviews with individuals with HHT aged 20s–60s. Main outcome measures: Qualitative themes explaining life experiences prior to and following a clinical diagnosis of HHT. Results: Narratives highlighted four psychosocial themes: (i) the psychological impact of visible symptoms was significant and related to experiences of social stigma, (ii) individuals struggled to identify triggers of symptoms in order to reduce unpredictability, (iii) an illness identity was rejected by minimising HHT when talking about the present self, and by positive reframing as ‘lucky’ and (iv) self-advocacy was necessitated due to lack of expert coordinated care. Conclusion: HHT has a demanding impact on social, physical and psychological well-being. These findings have significant implications for health care, as narratives about interactions with health professionals often used the terms ‘frustrating’ and ‘not being heard’.     

The role of positive thinking in social perceptions of cancer outcomes

Pressure for ‘positive thinking’ (PT; i.e. focusing on positive thoughts/suppressing negative thoughts to ‘fight’ cancer) burdens cancer patients facing health deterioration. It was determined whether PT exposure enhanced effort, control and responsibility attributions assigned to an individual for his/her cancer trajectory. Within an online blog a hypothetical same-gender person describes a personal cancer experience. 482 participants were assigned to one of six experimental conditions in which we manipulated PT exposure (blogger learns about ‘power of PT’ but does not try it, blogger tries PT, control/no PT) and cancer outcome (successful/unsuccessful treatment). A 3?×?2?×?2 multivariate analysis of covariance (with personal cancer experience covariates) tested PT exposure?×?cancer outcome?×?gender effects on attributions for the blogger's cancer outcome. Results indicate that PT exposure enhanced effort and responsibility attributions assigned to individuals for their cancer outcomes and that responsibility attributions differed as a function of gender. Findings suggest that exposure to the idea of PT may lead to cancer patients being perceived as culpable if they do not recover from the disease.     

Emotion processing and regulation in women with morbid obesity who apply for bariatric surgery

Emotional eating, the tendency to eat when experiencing negative affect, is prevalent in morbid obesity and may indicate that ways to deal with emotions are disturbed. Our aim was to compare emotion processing and regulation between 102 women with morbid obesity who apply for bariatric surgery and 102 women from the general population (control group) and to examine in the group with morbid obesity the association of emotion processing and regulation with emotional eating. The group with morbid obesity reported higher scores on difficulty identifying feelings (alexithymia, p?=?0.002) and suppression of emotions (p?=?0.003) than the control group. In the women with morbid obesity, more negative affect and a higher difficulty identifying feelings were correlated with more emotional eating (r?=?0.36 and r?=?0.35, p?<?0.001). Our study suggests that negative emotions and unhealthy emotion processing may play a role in emotional eating, and it indicates the possible relevance of emotion processing and emotional regulation as initiating or perpetuating mechanisms in morbid obesity.     

Neuropsychological Functioning in Children with Medium Chain Acyl Coenzyme A Dehydrogenase Deficiency (MCADD): The Impact of Early Diagnosis and Screening on Outcome

Children with medium chain acyl coenzyme A dehydrogenase deficiency (MCADD) have been reported to be at high risk for neurocognitive deficits. However this has not been systematically studied and little is known about the exact nature of neuropsychological sequelae or of the impact of early diagnosis and screening on outcome. We examined cognitive and adaptive outcome in children with MCADD (N?=?38, age range: 2 years, 2 months – 10 years, 3 months) diagnosed either through a newborn screening program (tandem mass spectrometry/MSMS) or upon clinical presentation. There was no evidence of overall intellectual impairment in either groups but there was some suggestion of poorer verbal and specific executive functioning (i.e., planning) abilities in the unscreened cohorts. Adaptive functioning was relatively intact with the exception of reduced Daily Living Skills in both our screened and unscreened groups. Early diagnosis and greater number of hospitalizations were related to higher verbal, communication, and socialization skills. Overall, our results highlight the importance of early diagnosis and management for children with MCADD.     

Predictors of quality of life enjoyment and satisfaction in individuals living with HIV and AIDS in a resource-constrained setting in South Africa

The burden of HIV disease is concentrated in sub-Saharan Africa, particularly in South Africa (SA). Whilst there have been many studies conducted on the biomedical and socio-psychological aspects of HIV and AIDS, insufficient attention has been paid to the quality of life of those infected with the virus. The primary purpose of the study was to determine the predictors of quality of life enjoyment and satisfaction (Q-LES) among individuals infected with HIV. A battery of questionnaires, which included the Hospital Anxiety and Depression Scale (HADS) and the Quality of Life Enjoyment and Satisfaction Questionnaire (Q-LES-Q), were administered to 121 participants. Data was analysed using SPSS. Of the total sample (n = 121), 74% were females. The study found that a large proportion (49.5%) of the sample within the age group 25–49 years old experienced psychological distress. Those who were not on antiretroviral treatment (ART) were significantly affected (66%). Psychological distress was significantly prevalent among HIV positive individuals and also the strongest predictor of Q-LES among these individuals.     

Validating Measures of Psychological Well-being by Contrasting Samples Employed in Hazardous and Less Hazardous Work

The study investigated whether differences between contrasting groups reflect significant differences in psychological well-being scores. Membership of hazardous vs. less hazardous occupations in the same profession served as proxy measures of well-being. Antonovsky's Sense of Coherence (SOC), Block & Kremen's Ego-resiliency, and Goldberg's Emotional Stability scales were validated. Study 1 compared mine rescue team members and ordinary miners (n = 34, 41) and Study 2 police special task force members and regular officers (n = 43, 41). In Study 1, SOC and job satisfaction predicted group membership. In Study 2, satisfaction with life and extraversion scales predicted group membership, as did SOC in a secondary analysis. The SOC scale could predict psychological well-being in hazardous work settings.     

Psychological strengths and subjective well-being in South African white students

This study investigated the role of individual resources, notably self-efficacy, gratitude, and hope, in subjective well-being of white dormitory students at a historically white institution of higher learning. Using a convenience sample of white students (N?=?227), we tested the role of generalised self-efficacy, gratitude, and hope as indicators of a latent factor, labelled personal resources, in a structural equation model with subjective well-being as the latent output variable, measured by self-esteem and satisfaction with life. Path analyses indicated a reasonable fit between the data and our hypothesised theoretical model which proposed positive relations between levels of generalised self-efficacy, gratitude, dispositional hope, self-esteem and satisfaction with life. White students were psychologically doing well, considering above-midpoint levels obtained for levels of generalised self-efficacy, gratitude, dispositional hope, and satisfaction with life However, scores obtained for self-esteem and adult dispositional hope were below the mid-point and neutral respectively, indicating that White students do not agree that they experience high levels of these two psychological strengths. White female students experienced higher levels of hope, gratitude, and life satisfaction, while no significant gender differences were found for generalised self-efficacy and self-esteem. The results of this study highlight the potential for using psychological strengths to promote well-being in racially diverse students.     

Pathways to flourishing among pharmacy students: The role of study demands and lecturer support

This study investigated possible pathways to flourishing among pharmacy students attending a South African university (N = 799; females = 81.6%; Afrikaans-speakers = 84.7%, mean age = 20.42 years, SD = 2.25 years). The students completed the Mental Health Continuum – Short Form (MHC-SF) and an Overload and Lecturer Support Survey. Of the 779 pharmacy students, 40.3% self-reported as flourishing, with high mean scores on workload and lecturer support. The level of emotional well-being of year group 2 was the highest. Following structural equation modelling, overload and lecturer support explained between 18% and 31% of the variance in the flourishing of the different year groups. From the second to the third year, the predictive value of overload and lecturer support for flourishing decreased by 8.8%. Lecturer support and low overload appear to play a positive role in the flourishing of higher education students.     

Interactive insight problem solving

Insight problem solving was investigated with the matchstick algebra problems developed by Knoblich, Ohlsson, Haider, and Rhenius (1999 Knoblich, G., Ohlsson, S., Haider, H. and Rhenius, D. 1999. Constraint relaxation and chunk decomposition in insight problem solving. Journal of Experimental Psychology: Learning, Memory and Cognition, 25: 1534–1555. [Crossref], [Web of Science ®] , [Google Scholar]). These problems are false equations expressed with Roman numerals that can be made true bymoving one matchstick. In a first group participants examined a static two-dimensional representation of the false algebraic expression and told the experimenter which matchstick should be moved. In a second group, participants interacted with a three-dimensional representation of the false equation. Success rates in the static group for different problem types replicated the pattern of data reported in Knoblich et al. (1999 Knoblich, G., Ohlsson, S., Haider, H. and Rhenius, D. 1999. Constraint relaxation and chunk decomposition in insight problem solving. Journal of Experimental Psychology: Learning, Memory and Cognition, 25: 1534–1555. [Crossref], [Web of Science ®] , [Google Scholar]). However, participants in the interactive group were significantly more likely to achieve insight. Problem-solving success in the static group was best predicted by performance on a test of numeracy, whereas in the interactive group it was best predicted by performance on a test of visuo-spatial reasoning. Implications for process models of problem solving are discussed.