Gemma M. Griffith

‘I just don’t fit anywhere’: support experiences and future support needs of individuals with Asperger syndrome in middle adulthood

The experiences of individuals in middle adulthood with Asperger syndrome have been the subject of little previous research, especially in terms of their experience of support services. In the present research, 11 adults with Asperger syndrome were interviewed. Interpretative phenomenological analysis (IPA) was used to interpret the interviews. Four themes emerged from the analysis: living with Asperger syndrome; employment issues; experiences with mainstream support; and future steps towards supporting adults with Asperger syndrome. The findings highlighted the anxiety, depression, and communication difficulties that people with Asperger syndrome may experience. Much of the available support is perceived as unsuitable for individuals with Asperger syndrome. All participants wanted to remain as independent as possible, and believed an individualized approach to support would be greatly beneficial. Recommendations are made for future practice to help support adults with Asperger syndrome.

Psychological Well-Being in Parents of Children with Angelman, Cornelia de Lange and Cri du Chat Syndromes.

BACKGROUND The current study focuses on mothers and fathers of children with three rare genetic syndromes that are relatively unexplored in terms of family experience: Angelman syndrome, Cornelia de Lange syndrome and Cri du Chat syndrome. METHOD Parents of children with Angelman syndrome (n =15), Cornelia de Lange syndrome (n = 16) and Cri du Chat syndrome (n = 18), and a matched comparison group of parents of children with autism and intellectual disabilities (n = 20) completed questionnaires on both psychological distress (stress, anxiety, depression) and positive psychological functioning. RESULTS Parents of children with Angelman syndrome consistently reported the highest levels of psychological distress, and parents of children with Cornelia de Lange syndrome the lowest, with parents of children with Cri du Chat syndrome and autism scoring between these two. Positive psychological functioning was similar across the four aetiology groups. CONCLUSIONS Parents of children with rare genetic syndromes are at risk for high levels of stress and mental health problems. Methodological issues and the practical applications of these results are discussed.

Outcomes of a behavioral education model for children with autism in a mainstream school setting

The authors report 1-year outcomes for 11 children (3-7 years) with autism who attended an “Applied Behavior Analysis (ABA) classroom” educational intervention in a mainstream school setting. The children learned new skills by the end of 1 year and learned additional skills during a 2nd year. Group analysis of standardized test outcomes (IQ and adaptive behavior) showed moderate to large effect size changes over 1 year, with further changes during a 2nd year. Standardized test outcomes for nine children after 2 years were also analyzed against a comparison group (n = 18) of children with autism receiving “education as usual.” These controlled comparisons were associated with statistically significant large effects in favor of the ABA group for adaptive skills. Exploratory analysis also showed that increases in language and learning skills in the ABA class group were generally associated with positive changes in standardized test scores. A comprehensive behavioral intervention model can be successfully implemented in a mainstream school setting.

Brief Report: Fathers’ and Mothers’ Ratings of Behavioral and Emotional Problems in Siblings of Children with Autism Spectrum Disorder

Debate is ongoing about whether typically developing siblings of children with autism spectrum disorder (ASD) are at greater risk of behavioral or emotional problems than siblings of children without ASD. Most data on behavior is provided by mothers, and we do not know whether fathers’ reports differ. The strengths and difficulties questionnaire (Goodman in J Child Psychol Psychiatry 38(5):581–586, 1997) was completed by 168 mothers and 130 fathers. Parents were more likely to rate siblings as having ‘abnormal’ behavior when compared to a normative population. We found moderate correlations between mother–father ratings. More research may be needed to understand any clinical benefits of gathering data about sibling adjustment from more than one parent in the family. Implications for clinical practice and future research are discussed.

“You Have to Sit and Explain it All, and Explain Yourself.” Mothers’ Experiences of Support Services for Their Offspring with a Rare Genetic Intellectual Disability Syndrome

The experiences of mothers of adult offspring with Angelman, Cornelia de Lange, or Cri du Chat syndrome have not been previously explored in research. The current study focuses on experiences with social and medical services and the impact the rareness of an adult offspring’s syndrome has on the experiences of mothers. Eight mothers of adults with Angelman, Cornelia de Lange, or Cri du Chat syndrome were interviewed. Thematic Content Analysis was used to interpret the interviews. Four themes emerged from the analysis: (i) The rarity of their offspring’s syndrome, (ii) Uneven medical and social care service provision, (iii) The inertia of social care services, and (iv) Mothers as advocates. Mothers felt that the rareness of their offspring’s syndrome did not affect experiences with social care services, but did affect access to medical services and some aspects of day-to-day living. Accessing appropriate social care services was reported to be a lengthy and complex process. These data may help inform care service providers about how best to support adults with rare genetic syndromes and their families.

Social Behavior and Characteristics of Autism Spectrum Disorder in Angelman, Cornelia de Lange, and Cri du Chat Syndromes

We evaluated autism spectrum disorder (ASD) characteristics and social behavior in Angelman (AS; n  =  19; mean age  = 10.35 years), Cornelia de Lange (CdLS; n  =  15; mean age  = 12.40 years), and Cri du Chat (CdCS, also known as 5 p-syndrome; n  =  19; mean age  =  8.80 years) syndromes. The proportion of individuals meeting the ASD cutoff on the Social Communication Questionnaire was significantly higher in the AS and CdLS groups than in the CdCS group (p < .01). The groups demonstrated divergent social behavior profiles during social conditions in which adult availability, adult familiarity, and social demand were manipulated. Social enjoyment was significantly heightened in AS, whereas social approaches were heightened in individuals with CdCS. Social motivation, social communication, and enjoyment were significantly lower in CdLS. The findings highlight the importance of detailed observation when evaluating ASD and social behavior in genetic syndromes.

“We are all there silently coping.” The hidden experiences of parents of adults with Asperger syndrome

Abstract Background The experiences of older parents of adults with Asperger syndrome have not been explored in the research literature. Method Four families who had middle-aged offspring with Asperger syndrome were interviewed (3 mothers and 1 couple), and the interviews were analysed using interpretative phenomenological analysis (IPA). Results Six themes emerged from the analysis: (a) providers of “hidden” support, (b) role of advocate, (c) social isolation, (d) intrafamilial relationships, (e) support for parents, and (f) future concerns. Conclusions The findings of this study offer insight into the experience of parents of adult sons with Asperger syndrome. Implications for future support interventions and research are suggested.

Cornelia de Lange syndrome: Extending the physical and psychological phenotype†‡

Cornelia de Lange Syndrome: Extending the Physical and Psychological Phenotype Chris Oliver,* Maria Francesca Bedeschi, Natalie Blagowidow, Cheri S. Carrico, Anna Cereda, David R. FitzPatrick, Cristina Gervasini, Gemma M. Griffith, Antonie D. Kline, P. Marchisio, Joanna Moss, Feliciano J. Ramos, Angelo Selicorni, Penny Tunnicliffe, Jolanta Wierzba, and Raoul C.M. Hennekam School of Psychology, University of Birmingham, Birmingham, UK Department of Maternal and Pediatric Sciences, Milan, Italy Harvey Institute of Human Genetics, Greater Baltimore Medical Center, Baltimore, Maryland Speech-Language-Hearing Clinic, Elmhurst College, Elmhurst, Illinois Department of Pediatrics, Fondazione Ospedale Maggiore Policlinico, Mangiagalli e Regina Elena, Milan, Italy MRC Human Genetics Unit, Western General Hospital, Edinburgh, UK Medical Genetics, San Paolo School of Medicine, Milan, Italy School of Psychology, Bangor University, Bangor, UK Institute of Psychiatry, Kings College, London, UK Laboratorio de Gen etica Cl ınica y Gen omica Funcional, Facultad de Medicina, Zaragoza, Spain Department of General Nursery and Department of Pediatrics, Hematology, Oncology and Endocrinology, Medical University, Gdansk, Poland Department of Pediatrics, Academic Medical Centre, UVA, The Netherlands

Challenging behaviours in adults with an intellectual disability: A total population study and exploration of risk indices

OBJECTIVES Considerable variation has been reported in the prevalence and correlates of challenging behaviour (CB) in adults with intellectual disabilities (ID). To provide a robust estimate of prevalence, we identified the entire administrative population of adults with ID in a defined geographical area and used a behaviour assessment tool with good psychometric properties. METHODS Data from 265 adults who were known to services were collected using a demographic survey tool and the Behavior Problems Inventory - Short Form. The prevalence of self-injurious, aggressive/destructive, stereotyped, and overall CB was evaluated. We explored the potential of developing cumulative risk indices (CRI) to inform longitudinal research and clinical practice. RESULTS The prevalence of overall CB was 18.1% (95% CI: 13.94-23.19%). The prevalence of self-injurious behaviour was 7.5% (95% CI: 4.94-11.37%), aggressive-destructive behaviour 8.3% (95% CI: 5.54-12.25%), and stereotyped behaviour 10.9% (95% CI: 7.73-15.27%). Communication problems and severity of ID were consistently associated with higher risk of CBs. CRIs were significantly associated with CBs, and the five methods of CRI development produced similar results. CONCLUSIONS Findings suggest a multi-element response to CB is likely to be required that includes interventions for communication and daytime activity. Exploratory analyses of CRIs suggested these show promise as simple ways to capture cumulative risk in this population. Subject to longitudinal replication, such a tool may be especially useful in clinical practice to identify adults who are priority for interventions and predict future demand on services. PRACTITIONER POINTS The prevalence of challenging behaviour (CB) was 18.1% in this total population study. Stereotypy was the most frequent type of CB. Communication difficulties and severe-profound intellectual disabilities were most systematically related to the presence of CB. Establishing the effect of multiple risk factors is likely to identify people who are priority for interventions. Addressing multiple, rather than singular risks, is likely to be more efficacious. We tested five different methods of putting together a multiple risk index. All methods provided a reasonable association with CB. The most user-friendly method was the additive cumulative risk index (CRI). Limitations This is a cross-sectional design which enabled factors currently associated with CB to be identified for the whole cohort, but these variables may not be those conferring risk for the development or maintenance of CB over time. Future longitudinal research is required to replicate these CRI analyses before concluding about the CRI method with the highest predictive validity.

Mothers' expressed emotion towards children with autism spectrum disorder and their siblings

BACKGROUND Expressed emotion (EE) is a construct used to measure the emotional climate within families. EE is of interest to researchers in the field of autism spectrum disorder (ASD) because of its putative implications for child development. The aim was to explore whether maternal EE differs towards a child with ASD and a non-disabled sibling. METHODS We adopted a within-family design with 143 mothers of children with ASD and a non-disabled sibling. EE was measured using the Five-Minute Speech Sample. RESULTS Wilcoxon signed-rank tests were utilised. Mothers were coded as significantly more critical and less warm towards their child with ASD than towards the sibling. There were no significant differences in maternal emotional overinvolvement or overall EE towards the child with ASD and a sibling. CONCLUSIONS The data support the results of previous research suggesting that EE is linked to the relationship a mother has with individual children, rather than being evidence of the character disposition of mothers. More research is needed to understand the emotional dimensions of parent-child relationships in families with children with ASD.