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Please click on titles for further information:
Cognitive and psychological profile of males with Becker muscular dystrophy (Completed 2006)
Literacy and laughter, numeracy and novelty: Strategies to overcome executive functioning difficulties in children with spina bifida – hydrocephalus (Completed 2005)
Medical expertise in the management of Attention Deficit Hyperactivity Disorder: An exploratory study (Completed 2005)
Working parents of children with chronic illness/disability: Caring responsibilities, workplace concerns and personal survival – An exploratory study (Completed 2005)
Social-cognitive aspects of children and adolescents with Velocardiofacial Syndrome (Completed 2004)
Social-cognitive profile of children with Neurofibromatosis Type 1 (Completed 2004)
Attentional functioning in children with Attention Deficit Hyperactivity Disorder (ADHD): An analysis of components (Completed 2003)
Parents’ and Teachers’ Perceptions of Educational Services for Students with Chronic Illness (Completed 2003)
Cognitive and psychological profile of males with Becker muscular dystrophy (Completed 2006)
Investigators: Dr Helen Young, Research Fellow, Institute for Neuromuscular Research, CHW; Dr Belinda Barton, Head, CHERI, CHW; Dr Richard Webster, Research Neurologist, CHERI, CHW: Professor Kathryn North, Head, Neurogenetics Research Unit, CHW.
Duchenne (DMD) and Becker muscular dystrophy (BMD) are allelic X-linked disorders causing progressive muscle weakness in males. DMD is caused by absence of dystrophin in the muscle and brain; boys with DMD have a static cognitive impairment with mean full scale IQ approximately one standard deviation below the mean. Less is known of the cognitive profile of males with BMD, which is associated with variable alterations in the amount or size of the dystrophin protein. The aim of this study was to describe the cognitive and psychological profile of males with BMD. This was a prospective cohort study. Clinical data collected included: Age at diagnosis and assessment, socioeconomic status, serum creatine kinase level and site of gene deletion/mutation (by exon number). Twenty-four males were enrolled.
Results indicated that mean IQ scores were normally distributed with a mean of 95.6 (SD 23.3), which did not differ significantly from the population mean. The frequency of learning difficulties for reading was 21%, for spelling was 32% and for arithmetic was 26%, significantly higher than the frequency in the general population. The frequency of total behavioural problems in the clinical range was 67% and the frequency of autism was 8.3%. Patients with BMD demonstrated a less homogeneous cognitive phenotype than that seen in DMD. Males with BMD have a high incidence of learning difficulties. Autism, behavioural and attention problems are also more common in BMD than in the general population.
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Literacy and laughter, numeracy and novelty: Strategies to overcome executive functioning difficulties in children with spina bifida – hydrocephalus (Completed 2005)
Investigators: Dr Angela Wilson, Senior Research Fellow, CHERI, CHW; Dr Carolyn West, Head, Spina Bifida Unit, CHW.
Students with spina bifida-hydrocephalus (SB-H) typically experience difficulties in executive functioning, for example in organisation, memory, planning and concentration.
These functions are critical in enabling an individual to interact and adjust to his or her environment, especially in the classroom situation during the school years. The aim of this project was to examine the learning capabilities of students with SB-H in the areas of literacy and numeracy. This project has resulted in the writing and publication of over 40 Strategy and Information Sheets that outline accommodations and adjustments that parents, family members and teachers can implement to assist students with SB-H to learn more effectively in their local schools.
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Medical expertise in the management of Attention Deficit Hyperactivity Disorder: An exploratory study (Completed 2005)
Investigators: Dr Linette Gomes, CHERI, Doctorate of Philosophy, University of Sydney (USyd): Supervised by Dr David Dossetor, Head, Department of Psychological Medicine, CHW and Professor Jeff Bailey, Director, CHERI, CHW.
Attention Deficit Hyperactivity Disorder (ADHD) is the most common, serious and disabling mental health problem in our society today. Although previous studies have examined management practices in this disorder, no research has addressed the nature or quality of medical expertise among doctors treating the disorder. As the understanding and conceptualisation of ADHD has changed, so too have attitudes to medicating children. Amidst this controversy and debate, it is important that evidence-based best practice occurs, and it is essential to obtain an understanding of the nature of medical expertise in this disorder, its strengths and weaknesses, and the factors which influence it.
This study examined, by questionnaire survey, the constructs of medical expertise in ADHD: knowledge, experience, diagnostic accuracy, and metacognitive skills, and possible influencing factors, such as self-confidence, training, area of specialisation and time in consultant practice, among a saturation sample of Australian paediatrics and psychiatrists. A spectrum of medical expertise was created and examined in relation to adherence to recommend best practice. The interaction of variables associated with expertise was examined. The important question of whether medical expertise matters, in terms of patient outcome and satisfaction, were addressed in an independent questionnaire survey of patients and parents.
Findings indicate that there is a spectrum of medical expertise amongst the doctors treating ADHD. Those higher on the spectrum practice more in accordance with recommended guidelines, but, reassuringly, it appears that the majority of doctors treating this condition adhere to most of the essential recommendations of best practice. The parameters of expertise interact in accordance with the literature, and important influence factors are current experience, training and area of specialisation. Although patient outcomes and satisfaction were not significantly related to the spectrum of medical expertise, there were individual aspects of expertise which influenced these variables favourably. These findings increase our understanding of medical expertise and provide a valuable insight into the execution of best practice in the management of ADHD.
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Working parents of children with chronic illness/disability: Caring responsibilities, workplace concerns and personal survival –
An exploratory study (Completed 2005)
Investigators: Dr Margaret H Vickers, School of Management, College of Law & Business, UWS; Professor Jeff Bailey, Director, CHERI, CHW.
It is increasingly the trend that, for both children and adults, health problems are chronic, that is long-lasting, rather than acute and immediately life-threatening. There has been much research into the problems of adults and children with chronic illness, as well as the role of carers. However, there have been no comprehensive studies examining the experiences and support needs of full time workers who care for children with chronic illness. This study examined the impact on the life and work of parents who have a child with a chronic illness/disability. Qualitative in-depth interviews, culminating in a group seminar with women who worked full time while caring for a child with chronic illness was conducted.
The results of this study indicated that these women have a very difficult time balancing their work and caring responsibilities. Their caring responsibilities impacted upon them in many different ways: professional and personal relationships; career advancement and enjoyment; a sense of disconnection and alienation from both personal and professional others; feelings of being overwhelmed and unsupported; and concerns about the negative impact that it may have on other family members. Our work in this area has continued and we are currently conducting a national survey to identify the support needs of full time workers who care for a child with a chronic illness in metropolitan areas in Australia (see Current Research).
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Social-cognitive aspects of children and adolescents with Velocardiofacial Syndrome (Completed 2004)
Investigators: Dr Belinda Barton, Head, CHERI, CHW; Dr David Dossetor, Head, Department of Psychological Medicine, CHW; Dr Meredith Wilson, Head, Clinical Genetics, CHW.
Velocardiofacial syndrome (VCFS) is a congenital autosomal dominant condition that is caused by deletion in chromosome 22q11.2. VCFS occurs as often as 1 in every 2000 to 4500 births, affecting almost every human system and organ. Over 180 clinical features are associated with VCFS, including congenital heart abnormalities, cleft palate, serious feeding and breathing problems, and language impairment.
Cognitive and other features include below average intelligence, learning disabilities, behavioural and attentional problems, and an increased rate of psychiatric illness. Social withdrawal and problems with social relationships, which are possibly related to impaired communication skills, are reported across the age range from early childhood to adolescence. The role of executive functions in learning and social adjustment is less well reported, but is an emerging area of importance. No studies have investigated the executive functioning of children with VCFS, or its relationship to other cognitive and social variables. Therefore, the aim of this study was to determine the cognitive and social profile of children and adolescents with VCFS.
Thirty-one children with VCFS were assessed using measures of intellectual functioning, academic achievement, and response inhibition. Parents and school teachers rated children’s executive functioning skills and social responsiveness. Results indicated that mean IQ scores was consistent with mild intellectual disabilities.Children with VCFS had significantly poorer maths, reading and oral language skills when compared to normative mean values. Parents and teachers rated children with VCFS as having significant executive function impairments, particularly in those areas involving metacognition. Social responsiveness scores for children with VCFS were consistent with scores reported for children with mood disorders or ADHD, and not autism.
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Social-cognitive profile of children with Neurofibromatosis Type 1 (Completed 2004)
Investigators: Belinda Barton, Doctorate of Philosophy, Faculty of Medicine, USyd and Honorary Associate Psychologist, CHERI, CHW and Neurogenetics Unit, CHW; Supervised by Professor Kathryn North, Head, Neurogenetics Research Unit, CHW.
Neurofibromatosis type 1 (NF1) is a genetic disorder that affects about 1 in 3000 people and involves the skin and nervous system. The severity of physical symptoms is variable, unpredictable, and can cause cosmetic disfigurement. Other features associated with NF1 include lowering of IQ, learning difficulties, attentional and organisational problems, and social problems. Despite the high prevalence of the disorder and the significant impact on physical appearance, overall wellbeing, and academic performance, very little research has addressed the psychosocial development of children with NF1. This study aimed to identify the cognitive and psychosocial profile of children with NF1. A total of 79 children with NF1, including 46 children with NF1 who were matched to their unaffected siblings were recruited. All children were aged between 8 to 16 years and underwent a psychometric assessment to measure their intelligence, academic achievement, attention, self-concept, and social skills.
The results of this study indicated that children with NF1 have significantly lower IQ, poorer academic achievement, and are more inattentive when compared to their unaffected siblings. The majority of children with NF1 demonstrated a positive self-concept. This may be due to the young age and hence milder disease severity of NF1 in this cohort. Alternatively, children with NF1 may lack the ability to make comparative judgements or experience a delay in the development of their self. Parental reports indicated that approximately 40% of children with NF1 had social and/or attentional problems in the clinical range. Children with NF1 and ADHD had the poorest social skills, social competence, and the most social problems when compared to children with NF1 and learning disabilities or children with NF1 alone. These findings dispel the previous assumption that NF1 alone is associated with poor social functioning, which has major implications for the development of effective interventions.
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Attentional functioning in children with Attention Deficit Hyperactivity Disorder (ADHD): An analysis of components (Completed 2003)
Investigators: Cheryl Soo, Doctorate of Philosophy, UWS, Nepean; Supervised by Professor Jeff Bailey, Director, CHERI, CHW.
This study investigated the assessment and characteristics of four components of attention (sustained, selective, divided and switching) in children with Attention Deficit Hyperactivity Disorder (ADHD) and children with learning disabilities. Five groups of boys and girls between the ages of 7 and 12 years were compared: ADHD Combined subtype with and without LD, ADHD Inattentive subtype, LD, and normal controls. Attentional measures included two Continuous Performance Tests (CPTs) – the Test of Variables of Attention (TOVA) and the Conners’ CPT (CCPT), and the newly developed non-CPT, Test of Everyday Attention for Children (TEA-Ch).
Results indicated that children with ADHD Combined subtype performed significantly worse on sustained and divided attention but not on selective attention and attentional switching compared to all other groups; also, few differences were found among the four clinical groups on the four components of attention. Attentional measures, such as the CPT, were also found to have adequate specificity and positive predictive ability but limited sensitivity and negative predictive ability. These findings extend our understanding of the componential characteristics of attention in four important clinical groups and provide evidence that these measures may not be useful as the sole criteria for diagnostically classifying children with ADHD.
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Parents’ and Teachers’ Perceptions of Educational Services for Students with Chronic Illness (Completed 2003)
Investigators: Shiona Shiu, Doctorate of Education, CHERI; Supervised by Professor Jeff Bailey, Director, CHERI, CHW.
This project investigated the concerns of parents and educators with regards to the provision of educational services for students with chronic illness. A survey instrument was developed to elicit parent and teacher responses to a range of issues and contained both qualitative and quantitative responses. The survey was distributed to 500 NSW Department of Education and Training Schools. Teachers were asked to complete the survey and also forward a copy of the survey to parents of a child with chronic illness. A total of 121 parents and 112 teachers completed the survey. An additional 61 schools indicated a nil response (ie. no children in their school with a chronic illness).
Results indicated that parent and teacher concerns varied according to the child’s illness and severity. Parents and teachers of children with mild and moderate illness had few concerns in general, the most commonly reported concerns being with regard to absenteeism and missed schooling, and teaching knowledge and skills.
However, for parents of children with severe conditions, the picture was dramatically different. While they represented 58 percent of respondents in the survey, they accounted for 87 per cent of the concerns reported. Children with high levels of absenteeism are missing out on learning and social activities, special events, and access to intrinsic and extrinsic reward systems that highlight school in the early years. They are also missing out on crucial building blocks that form the foundations for future learning, and are failing to access important opportunities to develop social skills and healthy peer relationships that will aid in their development of a positive self-concept and help them to cope with the many challenges of their medical condition.
Research findings from this study have been used by as a basis for writing a Training Module for Teachers Aides, NSW Department of Education and Training. The course is aimed at training teachers aides in the support of students with chronic illness.
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