The expression and assessment of emotions and internal states in individuals with severe or profound intellectual disabilities

The expression of emotions and internal states by individuals with severe or profound intellectual disabilities is a comparatively under-researched area. Comprehensive or standardised methods of assessing or understanding the emotions and internal states within this population, whose ability to communicate is significantly compromised, do not exist. The literature base will be discussed and compared to that within the general population. Methods of assessing broader internal states, notably depression, anxiety, and pain within severe or profound intellectual disabilities are also addressed. Finally, this review will examine methods of assessing internal states within genetic syndromes, including hunger, social anxiety and happiness within Prader-Willi, Fragile-X and Angelman syndrome. This will then allow for the identification of robust methodologies used in assessing the expression of these internal states, some of which may be useful when considering how to assess emotions within individuals with intellectual disabilities

A study to investigate the relationship between parental stress, social support and level of child difficulty in parents of children attending an assessment unit

Parental stress includes "subjective experiences of distress as emotional pain and anxiety" (Deater-Deckard, 2004). High levels of parental stress can have a cumulative effect over time and have a negative impact on family relationships (Quittner, Glueckay and Jackson, 1990).Parents of children with intellectual disabilities report relatively high levels of distress, with a wide range of child, parent, family and service support factors implicated in parental distress (Hatton and Emerson, 2003). Severity of a child's difficulty is often linked with the level of parental stress (Keller and Honig, 2004).The role of social support variables in protecting and maintaining physical and psychological health has been well established across a variety of studies (e.g. Koeske and Loeske, 1990). Generally it can be suggested that a lack of positive social relationships can lead to negative psychological states such as anxiety or depression.It was the aim of this study to investigate any association between levels of parental stress, social support and levels of child difficulty in a group of parents whose children had previously undergone a multi-professional assessment of their difficulties. A further aim was to investigate whether level of reported depressive symptomology related to the level of parental stressParticipants included forty-four parents whose children had been assessed six to twelve months previously. Parents completed questionnaires measuring parental stress, social support, level of child difficulty and a screening measure of depressive symptomology.The results of this study indicate that there was evidence of associations between level of parental stress, reported social support and perceived level of child difficulty. The results also suggest that there was evidence of an association between parental stress and level of reported depressive symptomology.The results of this study support the hypothesis that both social support and level of child difficulty are predicative of level of parental stress.The limitations of this study and implications for future research are discussed

Brief Report:A Longitudinal Study of Excessive Smiling and Laughing in Children with Angelman Syndrome

Elevated laughing and smiling is a key characteristic of the Angelman syndrome behavioral phenotype, with cross-sectional studies reporting changes with environment and age. This study compares levels of laughing and smiling in 12 participants across three experimental conditions [full social interaction (with eye contact), social interaction with no eye contact, proximity only] at two data points. No differences were noted in frequency of laughing and smiling over time in any condition. However, with age as a covariate, the frequency of laughing and smiling decreased over time in the full social interaction (with eye contact) condition only. As this is the first longitudinal study to explore these behaviors in Angelman syndrome, the results suggest a gene–environment–time interaction within the behavioral phenotype.Full Tex

Organizational communication and awareness: a novel solution for health informatics

As organizations grow larger and more distributed, the problems of maintaining corporate awareness and effective communication channels escalate. The clinical domain poses particular challenges to maintaining good corporate communications because users have limited time to access information and often have negative technology perceptions. This paper highlights how a screen saver application, initially designed to increase privacy and security, developed into a new communication medium improving corporate communication across the organization. An ethnographic study of the application within a hospital setting, analyzed using grounded theory methods, details the iterative and organic development of the design through ‘community of practice’ involvement. This application was found to not only increase awareness of resources, activities and hospital changes but also positively influence users’ perceptions of, involvement in and ownership of general IT developments. User involvement also raised the importance, for the designers, of application usability, quality and aesthetics. As well as strengths, application limitations are discussed; we also identify further research and developments required if this approach is to realize its full potential

The investigation of single nucleotide polymorphisms in genes of the cell cycle and related pathways as candidate modifiers of the age of disease onset in hereditary nonpolyposis colorectal cancer

Hereditary Non-Polyposis Colorectal Cancer (HNPCC) is the most common type of inherited colorectal cancer. Eighty to ninety percent of identified mutations in HNPCC families involve MSH2 or MLH1 genes. However, a great degree of variability has been observed within and between families carrying the same mutation. Therefore, other factors such as modifying genes may be involved in the presentation of this disease. The cell cycle, the mismatch repair pathway, and folate metabolism have been associated with cancer. Therefore, I studied 31 single nucleotide polymorphisms (SNPs) from genes in these pathways to determine if they had a modifying effect on the disease penetrance. Two MSH2 kindreds were used in this study, one from Newfoundland and one from the Lower North Shore of Quebec. They included 135 mutation carriers. I identified 3 SNPs CCND1¹⁷²²GC, CCNA2 GA, and CDKN1B ( p27KIP1 ) TG, which had significant effect on the age of disease onset

Coping Strategies in Mothers of Children with Intellectual Disabilities Showing Multiple Forms of Challenging Behaviour:Associations with Maternal Mental Health

Background: It is well documented that mothers of children with intellectual disabilities experience elevated mental health difficulties and that these are exacerbated by the presence of challenging behaviour. However, comparatively little is known about the effect of specific coping strategies for managing such behaviours. Aims: This paper aims to document coping strategies used by mothers of children showing multiple forms of challenging behaviour and to explore how these relate to positive and negative maternal mental health. Method: Eighty-nine mothers of children with intellectual disabilities completed questionnaires assessing maternal mental health (Hospital Anxiety and Depression Scale, Positive and Negative Affect Scale) and maternal coping strategies (Brief COPE). Results: Coping strategies were not associated with child age or ability, but were associated with maternal mental health. Higher levels of problem- and positive-coping strategies were associated with higher positive affect. Although active-avoidance coping was the least frequently reported, it was associated with higher levels of negative affect and increased anxiety and depression. Moderated mediation analyses identified that active-avoidance coping mediated the relationship between the number of forms of challenging behaviour and poor maternal mental health, but only in mothers with lower levels of problem-focused coping. Conclusions: Active-avoidance coping is associated with poorer negative mental health in mothers of children with intellectual disabilities who have average to low levels of problem-focused coping. This is reflective of that noted within a range of populations, highlighting it as a key area for intervention.Arts, Education & Law Group, School of Education and Professional StudiesFull Tex

The expression and assessment of emotions and internal states in individuals with severe or profound intellectual disabilities.

The expression of emotions and internal states by individuals with severe or profound intellectual disabilities is a comparatively under-researched area. Comprehensive or standardized methods of assessing or understanding the emotions and internal states within this population, whose ability to communicate is significantly compromised, do not exist. The literature base will be discussed and compared to that applicable to the general population. Methods of assessing broader internal states, notably depression, anxiety, and pain within severe or profound intellectual disabilities are also addressed. Finally, this review will examine methods of assessing internal states within genetic syndromes, including hunger, social anxiety, and happiness within Prader–Willi, Fragile-X and Angelman syndrome. This will allow for identification of robust methodologies used in assessing the expression of these internal states, some of which may be useful when considering how to assess emotions within individuals with intellectual disabilities.Full Tex

Ceremony at a Boundary fire: a story of Indigenist Knowledge

National Science Foundation (United States of America