MPS II: Adaptive Behavior of Patients and Impact on the Family System

Mucopolysaccharidosis type II (MPS II), also known as Hunter syndrome, is a chronic and progressive X-linked lysosomal disease that mainly affects males. It occurs in 1 in every 65,000 to 1 in 132,000 births. There are two distinct forms of the disease based on age of onset and clinical course: mild and severe. MPS II affects many organ systems including the nervous, cardiovascular, gastrointestinal and respiratory systems. Complications can include vision problems, progressive hearing loss, thickened and elastic skin, mental impairment, and enlarged liver and spleen. We herein focus on the adaptive behavior of individuals with MPS II, and the impact of MPS II on the family system. Outcomes from the Vineland-II Adaptive Behavior Scales showed that the MPS II patient sample experienced significantly lower functioning in communication, daily living skills, socialization, and motor skills compared to normative data. Patients with severe MPS II were found to have significantly lower adaptive functioning in all domains, as compared to those with mild MPS II. Length of time on ERT had no significant relationship to adaptive functioning. Results from the Peds QL Family Impact Module indicated that families of patients with MPS II experienced a lower overall health-related quality of life and overall lower family functioning (including lower emotional and cognitive functioning) than those with chronic illnesses residing in an inpatient setting.     

Feminine Gender Role Discrepancy Strain and Women’s Self-Esteem in Daily and Weekly Life: A Person x Context Perspective

Sex Roles - Social pressures to adhere to traditional feminine roles may place some women at risk of experiencing gender role discrepancy strain, when they behave, think, or feel in ways discrepant...     

‘Emotional turbulence’: the development of symbolic thinking in the psychotherapeutic treatment of an adolescent girl

Abstract

This paper is drawn from doctoral research which analysed clinical data from the once weekly psychotherapy of a young person who experienced both gender identity dysphoria and an eating disorder and whose depression had proved resistant to other interventions. Based on the psychoanalytic thinking of Klein, Segal, Bion and others regarding the role of symbolisation in development, the links between symbol formation, the depressive position and the negotiation of the Oedipus complex are outlined. Special attention is given to current thinking about the nature and genesis of gender identity dysphoria in the light of these concepts. The association with autistic features for a number of cases is briefly explored. In the light of these theoretical links, findings from the study are used to investigate and illustrate the movement this patient made within her therapy from an emotionally turbulent position where concrete thinking was manifest, to a more symbolic way of thinking that allowed emotional change, and less concrete acting out. Clinical narrative excerpts as well as findings from the study are used to discuss the progress made by the patient and to evaluate how her journey may inform future practice.     

Evaluating the Character of People Who Insult the Nation: Implications for Immigrant Integration

We examine immigrant integration by analyzing how natives evaluate immigrants' character. Most literature examines how natives distinguish between immigrants with different levels of assimilation, which is best suited to identifying integration boundaries between different types of immigrants. We shift the analysis and examine the boundary between immigrants and natives, which measures integration by the extent to which immigrant status is relevant for character evaluations. We compare how natives respond to national insults that come from immigrants as opposed to natives. We focus on insulting the nation because it highlights the salience of national identity and clarifies the importance of group boundaries. We measure responses to national insults with vignette experiments from three original surveys in the United States. Our results are consistent with situationist theories of interpersonal interactions because they suggest that character evaluations are more dependent on the situational distinction between people who do and do not insult the nation than the demographic distinction between whether the insult comes from a native or immigrant. These findings have multiple implications for our understanding of national identity, immigrant integration, and immigrant‐native boundaries.     

Knowledge of drug-related health problems among injecting drug users: Implications for the transmission of HIV infection

Abstract

In a project that investigated the HIV-related risk behaviour of injecting drug users, respondents were categorized along dimensions of expertness in HIV/AIDS knowledge and knowledge about drug-related health problems. Knowledge about drug-related health problems was found to be significantly associated with sharing. Better informed respondents were less likely to share injecting equipment and to predict they would share in future. They were more likely to adopt consistent injecting hygiene, seek information about AIDS, and perceive themselves as in control of their lives. No significant relationships were observed between sharing and HIV/AIDS knowledge. The data have relevance for AIDS educational strategies, since knowledge about AIDS and HIV transmission appears to be insufficient to induce behavioural change. Greater emphasis on the health problems that afflict most injectors may be a way of encouraging general harm minimization.     

Working with the complexities of adolescent mental health problems: applying time-limited adolescent psychodynamic psychotherapy (TAPP)

This article discusses the application in a CAMHS setting of a distinctive intervention for adolescent mental health difficulties, time-limited adolescent psychodynamic psychotherapy (TAPP). TAPP has been developed specifically for working with adolescents and the characteristic developmental and psychosocial complexities they present to mental health services. It is widely recognised that supporting the developmental process in adolescence is central to therapeutic interventions and the therapeutic aim of TAPP is to enable recovery of the capacity to meet developmental challenges. The key factors of TAPP are described, including the formulation and working with a developmental focus, the therapeutic stance, working with transference and counter-transference, working with time limits, and the emphasis is placed on engagement of adolescents in therapy in TAPP. The experiences of introducing and developing TAPP in the CAMHS service are discussed with two brief and one extended case examples and this leads to a discussion of the kinds of outcomes achieved. It is concluded that TAPP is a key and relevant intervention for adolescents in complex and vulnerable situations; further work will be undertaken to continue its application in these settings and to formally assess outcomes.     

Group therapy for school-aged children who stutter: a survey of current practices

Although group therapy is recommended for school-aged children who stutter (CWS), it is not widely researched. This study aimed to explore this provision, using a postal survey which investigated the current practices of Speech & Language Therapists (SLTs) in the UK. Seventy percent of SLT services provided some group therapy, but the level of provision was variable. There was a lack of consensus on what the main aims of group therapy should be. Important barriers to group therapy provision were identified, including a perceived lack of clients' interest in group therapy, and insufficient numbers of clients able to travel to group venues. This study enhances the profession's understanding of the provision of group therapy for CWS by identifying patterns of service delivery and highlighting areas of need. EDUCATIONAL OBJECTIVES: Readers should be able to: (1) Provide a rationale for the provision of group therapy for school-aged CWS; (2) Summarize the factors affecting group therapy provision for school-aged CWS; (3) Summarize the aims of therapy identified by the respondents to this survey.     

Personality and risk for Alzheimer's disease in adults 72 years of age and older: a 6-year follow-up

[Correction Notice: An erratum for this article was reported in Vol 26(2) of Psychology and Aging (see record 2011-05802-001). This article contains an error in the Discussion, under the Implications, Caveats, Future Directions heading. The third paragraph includes the sentences that should have been removed. The corrected paragraph appears in the correction.] We conducted secondary analyses to determine the relationship between longstanding personality traits and risk for Alzheimer's disease (AD) among 767 participants 72 years of age or older who were followed for more than 6 years. Personality was assessed with the NEO-FFI. We hypothesized that elevated Neuroticism, lower Openness, and lower Conscientiousness would be independently associated with risk of AD. Hypotheses were supported. The finding that AD risk is associated with elevated Neuroticism and lower Conscientiousness can be added to the accumulating literature documenting the pathogenic effects of these two traits. The link between lower Openness and AD risk is consistent with recent findings on cognitive activity and AD risk. Findings have implications for prevention research and for the conceptualization of the etiology of AD.