French translation and validation of a fragile X syndrome screening checklist

Background: Fragile X syndrome (FXS) is the most common cause of inherited intellectual disability among men. This syndrome is frequently underdiagnosed in adults. The aim of this study was to develop and validate a French translation of the screening checklist Fragiele-X screeningslijst.

Method: The validation sample for the translated checklist included 22 pairs of men between the ages of 18 and 52, matched by age and degree of intellectual impairment.

Results: The translated checklist achieved coefficients of 0.92 for internal consistency, 0.90 for test-retest reliability and 0.65 for inter-rater reliability. These psychometric properties are commensurate with those of the original checklist.

Conclusion: Therefore, the translated checklist developed in this study can be considered a valid screening instrument for the detection of FXS in men with intellectual disabilities.

Abbreviations: FXS: fragile X syndrome; FMR1: fragile X mental retardation 1 (gene)     

Personal characteristics and school contextual variables associated with student self-determination in Spanish context

Background: Most theoretical models of self-determination suggest that both environmental and personal factors influence the development of self-determination. The design and implementation of interventions must be conducted with foreknowledge of such mediating and moderating factors if the intervention is to be successful.

Methods: The purpose of this study was to examine the degree to which several personal factors and school characteristics affect and explain students’ self-determination. A total of 232 students with intellectual disability from Spain participated. Their self-determination level was assessed by the ARC-INICO Scale.

Results: Students with moderate levels of intellectual disability obtained significantly lower scores on self-determination than their peers with mild intellectual disability. There were significant differences in relation to the level of support needs and their experience with transition programs. The level of support needs was a significant predictor.

Conclusion: These findings contribute to current research in this field and practical implications were discussed.     

An international survey of obesity and underweight in youth and adults with intellectual disabilities

Background: Increased levels of obesity have been reported for people with intellectual disability based on data mostly gathered in high income countries. Likewise few studies internationally have focussed on underweight in this population.

Method: Data on obesity and underweight were analysed from nearly 86,500 youth and adults with intellectual disabilities drawn from 178 countries attending Special Olympic events.

Results: Binary logistic regressions identified higher levels of obesity for athletes in high income countries – especially in North America – and for females and for adults aged 40 years and over; and for youth aged 8 to 10 years. Rates of underweight were higher for younger adults in low income countries of Asia-Pacific, and highest for males aged 11-13 from lower-middle income countries of Africa.

Conclusions: Contrasting intervention strategies are needed globally to enable people with intellectual disabilities to achieve healthy weight.     

“They need to be able to have walked in our shoes”: What people with intellectual disability say about National Disability Insurance Scheme planning

Background: Planning is a key mechanism by which the Australian National Disability Insurance Scheme (NDIS) ensures individuals with disability have choice and control over supports. People with intellectual disability will comprise the largest NDIS participant group and many will need assistance to engage in planning. In order to respond effectively, NDIS planners must understand the decision-making support required by individuals.

Method: Focus groups were conducted with 9 adults with intellectual disability living in an NDIS trial site to explore their experiences of NDIS planning.

Results: Thematic analysis identified 6 themes related to good planning experiences for people with intellectual disability: preparation, learning from mistakes, personal growth, and having a credible, consistent, and disability-aware planner.

Conclusions: Participants who developed a trusting relationship with a planner used planning to increase independence and social participation. Planner skills, particularly communication and sector knowledge, and attributes such as warmth and openness created trust.     

Oral health status and reported oral health problems in people with intellectual disability: A literature review

Background: People with intellectual disability (ID) experience poor oral health and are at greater risk of dental decay and periodontal diseases. This impacts on their general health and wellbeing. This review summarises the research literature about oral health status and contributing factors to poor oral health

Method: We conducted a literature review using “intellectual disability” and “oral health” as our two core areas of focus.

Results: People with ID had poorer oral health, greater numbers of tooth extractions, more caries, fewer fillings, greater gingival inflammation, greater rates of endentulism, and had less preventative dentistry and poorer access to services when compared to the general population. Anxiety during dental procedures was a key issue for females with ID.

Conclusions: Further research is needed to identify, pilot and test appropriate and effective interventions that can reduce this preventable health disparity. The design of an ID-specific dental anxiety scale is another priority.     

Perspectives of carers of people with intellectual disability accessing general practice: “I’d travel to the ends of the earth for the right person”⁣

Background: Informal carers often play an integral role in the lives of people with intellectual disability (ID) residing in the community. In this study, we explored the extent to which carers of people with ID believe that the health care needs of the person they care for are being accommodated by general practice.

Method: Semi-structured interviews were conducted with 25 informal carers to people with ID living in Australia.

Results: Carers of people with ID report that they experience considerable barriers to accessing general practice care on behalf of the people they care for.

Conclusions: Given the ever-increasing number of people with ID now living in the community and their vulnerability to health problems, it is imperative that future research focuses on the development of strategies to overcome the barriers identified in this study.     

Effect of activity-based mirror therapy on lower limb motor-recovery and gait in stroke: A randomised controlled trial

Objective: To determine the effect of activity-based mirror therapy (MT) on motor recovery and gait in chronic poststroke hemiparetic subjects.

Design: A randomised, controlled, assessor-blinded trial.

Setting: Rehabilitation institute.

Participants: Thirty-six chronic poststroke (15.89?±?9.01 months) hemiparetic subjects (age: 46.44?±?7.89 years, 30 men and functional ambulation classification of median level 3).

Interventions: Activity-based MT comprised movements such as ball-rolling, rocker-board, and pedalling. The activities were provided on the less-affected side in front of the mirror while hiding the affected limb. The movement of the less-affected lower limb was projected as over the affected limb. Conventional motor therapy based on neurophysiological approaches was also provided to the experimental group. The control group received only conventional management.

Main outcome measures: Brunnstrom recovery stages (BRS), Fugl-Meyer assessment lower extremity (FMA-LE), Rivermead visual gait assessment (RVGA), and 10-metre walk test (10-MWT).

Results: Postintervention, the experimental group exhibited significant and favourable changes for FMA-LE (mean difference?=?3.29, 95% CI?=?1.23–5.35, p?=?.003) and RVGA (mean difference?=?5.41, 95% CI?=?1.12–9.71, p?=?.015) in comparison to the control group. No considerable changes were observed on 10-MWT.

Conclusions: Activity-based MT facilitates motor recovery of the lower limb as well as reduces gait deviations among chronic poststroke hemiparetic subjects.     

Implementation of active support over time in Australia

Background: Research indicates the value of active support in achieving good outcomes across a number of quality of life domains for people with intellectual disabilities. However, implementation is not easy, and little research has explored why. We aimed to identify some of the factors that impact on implementation of active support in supported accommodation services.

Methods: Data on the quality of active support, staff training and practice leadership were collected through staff questionnaires, observations and manager interviews, for between two and four years across six organisations.

Results: Active support improved over time for more able people with intellectual disability, but not for people with higher support needs. There was a weak positive correlation between active support and (1) practice leadership scores, and (2) the percentage of staff reporting active support training.

Conclusions: It is important to recognise the influence of practice leadership and staff training on the quality of support and ensure provision for these in funding schemes.     

Intellectual,educational, and situation-based social outcome in adult survivors of childhood medulloblastoma

Purpose: To investigate intellectual and situation-based social outcome and educational achievement in adult survivors of childhood medulloblastoma and analyse factors influencing outcome

Methods: We collected demographic, medical and cognitive data, and social and educational outcome at a mean time since the end of treatments of 14.9 years in 58 adults, aged 19–35 years, consecutively treated in a single cancer center between 1989 and 2005.

Results: Ten survivors had severe intellectual disability, 12 were still studying, 23 had a regular employment and 13 were unemployed. Full Scale Intellectual Quotient, assessed 6.6 years after the end of treatments, ranged from 46 to 131. It was strongly associated with educational achievement and significantly lower in patients who experienced postoperative cerebellar mutism, and when parental education level was low.

Conclusion: These factors should be systematically considered at diagnosis in order to offer adequate and timely assessments and interventions.     

Scoping review of social participation of individuals with profound intellectual disability in adulthood: What can I do once I finish school?

Background: After 21 years of age, adults with a profound intellectual disability (PID) have limited access to specialised services. An important concern that emerges is the potential decrease in their social participation. Knowing the benefits of social participation for adults with PID, it is relevant to address this issue.

Method: This scoping review examined the literature on the social participation of people with PID in young adulthood and its influential factors.

Results: Results suggest a significant lack of information concerning social participation of these adults. During adulthood, people with PID have currently two main choices to occupy their days: activities in daily activities centre and leisure in community organisation. Few options to maintain their capabilities are available, and this situation has significant impacts on young adults and their families.

Conclusion: Future studies focusing specifically on adults with PID and their caregivers are needed to understand their reality when entering adulthood.     

Intellectual disability in Australian nursing education: Experiences in NSW and Tasmania*

Background: Persons with intellectual disability (ID) face significant health challenges. Nurses provide health care to this population in many settings, including general practice, community care, acute care, reproductive health, and palliative care. However, since the demise of the specialist training model in the 1990s, the extent to which general nurses in Australia are educated about the health needs of people with ID is unclear.

Methods: A systematic literature mapping and documentary analysis of educational resources available to nurses in Australia was undertaken, with particular reference to New South Wales and Tasmania.

Results: Minimal education content relating to the health needs of people with ID was found in the academic programs included in this study. Where it existed, it was inconsistently applied, being made available as an aspect of professional development rather than a clearly identified educational pathway.

Conclusions: This study adds to previous findings indicating the need for change to nurse education in this area, and for cross-country comparisons with other models of nurse education in the area.     

Can Motivational Interviewing follow-up calls improve the implementation and retention of a specific communication support (Key Word Sign) by staff supporting people with an intellectual disability? A pilot project

Background: This study examined whether Motivational Interviewing (MI) follow-up calls improved the extent to which a specific therapeutic technique (Key Word Sign) presented in training was retained and implemented by staff supporting people with an intellectual disability.

Method: Thirty-eight residential support workers who attended Key Word Sign (KWS) training were divided into three groups. One group received post training MI follow-up calls, the second received non-MI “check-in control” (CIC) follow-up calls and the third received no follow-up calls.

Results: Both follow-up conditions outperformed the no follow-up condition on KWS knowledge retention and use. No significant differences were noted between the MI and CIC condition in this study.

Conclusion: The results highlight the value of post-training follow-up in promoting knowledge retention and implementation of skills. Methodological challenges (including treatment fidelity issues across groups) prevented firm conclusions about the impact of MI from being drawn.     

Perceptions of psychiatry residents and non-residents on training needs and care related to patients with intellectual disability and mental health problems: A study from Singapore

Background: People with intellectual disability (ID) have higher risk of mental morbidity and specialised training may be required in their management. We explored the training needs and knowledge as well as perceptions of care of such patients among residents and non-residents working in psychiatry.

Method: The study was conducted as an anonymous survey. Questionnaires were sent to all residents and non-residents at a large psychiatric hospital in Singapore.

Results: Forty-eight out of the 76 questionnaires sent out were returned. All participants responded that postgraduate training was required in the area of ID and mental health and according to the majority, available training was inadequate. Over 90% believed that people with ID were vulnerable to exploitation and they should be under a specialist team.

Conclusion: Efforts should be made to include specialist training in psychiatry of ID in the Singapore psychiatry curriculum to enhance the knowledge and expertise of psychiatrists in this field.     

Epilepsy and challenging behaviour in adults with intellectual disability: A systematic review

Background: Epilepsy and challenging behaviour are both highly prevalent in the intellectual disability (ID) population and it is thus crucial to understand any possible associations between the two.

Method: PsycINFO, MEDLINE, Embase, CINAHL and Web of Science were searched for quantitative data about epilepsy and any forms of challenging behaviour in adults with ID.

Results: A total of 25 articles were included in the review. Overall the evidence suggests that while epilepsy is not a good explanatory variable for the presence of challenging behaviour; the relationship is complex. However, for some people with epilepsy and for certain types of challenging behaviour a link may exist.

Conclusions: Given the possible link between epilepsy-related factors such as seizure type and specific subgroups of challenging behaviour, investigating these relationships further and particularly exploring how to best measure challenging behaviour in people with epilepsy could be of great clinical benefit.     

Exploring dimensions of social support and resilience when providing care at the end of life: a qualitative study

Background: Research shows that formal and informal social support can facilitate resilience in carers. There is a paucity of research exploring social support and resilience amongst recently bereaved informal carers.

Aim: To examine how the presence or absence of distinct dimensions of social support facilitate or hinder resilience in recently bereaved informal carers.

Participants: 44 bereaved carers, who had been identified by GP as ‘main carer’ of someone recently deceased (3–12 months), aged between 38 and 87 years old (mean= 67).

Methods: Thematic analysis then the Ecological Framework of Resilience as an organisational tool to develop overarching themes in the data. We used the Sherbourne and Stewart model to identify social support that was lacking as well as social support that was present.

Results: A range of social support types were identified. There was an emphasis on the importance of relationships with both health professionals and family members, including the care recipient. However, social support was not necessary for resilience if the participant had other resources.

Conclusions: Social support for carers providing end of life care is almost exclusively based around end of life care ‘work’. In comparison to other research our study suggests that relationships with family and health professionals are paramount. Multidimensional support is needed for carers to enhance their resilience.     

Oral health of patients with special needs requiring treatment under general anaesthesia

Background: General anaesthesia (GA) remains a vital modality to facilitate dental treatment for patients with special needs but there is limited literature to describe the types of patients requiring this form of care and the treatment they receive.

Method: A cross-sectional clinical audit was conducted of patients treated under GA at the Day Surgery Unit of the Royal Dental Hospital of Melbourne during August 2015.

Results: The majority of patients had an intellectual disability and were referred due to their inability to tolerate conventional dental treatment. These patients demonstrated high levels of treatment need requiring an average of two fillings and six extractions.

Conclusions: Although these results demonstrate the value of GA in treating patients with intellectual disabilities, the level of treatment need identified raises concerns about current approaches to oral health for this cohort. Greater efforts are required towards preventive oral care for patients with special needs.     

Human papillomavirus vaccine uptake in adolescents with developmental disabilities

Background: The Human Papillomavirus (HPV) vaccine is offered in Australia to all males and females at 12 to 13 years. In 2015 the national HPV coverage was 77.4% for females and 66.4% for males. There is no Australian coverage data for the subgroup of adolescents with disabilities.

Method: We reviewed the HPV vaccine status of all 14 year-old adolescents who attended a developmental medicine clinic in a tertiary hospital in Melbourne during 2014. Motor function and intellectual impairment were also recorded.

Results: Of the 72 adolescents in the audit, only 39.5% of males and 44.1% of females were fully immunised against HPV. Those with intellectual impairment had particularly low levels of HPV immunisation (16/47 (34%)).

Conclusion: In this study HPV vaccine coverage in adolescents with developmental disabilities was found to be well below national levels. Larger studies of HPV uptake and exploration of the barriers to immunisation in this population are urgently needed.     

AAC technologies with visual scene displays and “just in time” programming and symbolic communication turns expressed by students with severe disability

Background: The current study investigated the impact of an intervention that included aided augmentative and alternative communication (AAC) technologies on the frequency of symbolic communication turns of school-age children, adolescents, and young adults with severe disability.

Method: Nine students ages 8–20 were engaged in interactive activities using an aided AAC system with visual scene displays (VSDs; concepts embedded within a photograph of a naturally occurring event), and “just-in-time” (JIT) programming (the capability to add new contexts “on the fly”). Effectiveness was evaluated using a single subject multiple probe across participants design.

Results: All participants demonstrated increases in symbolic communication turns upon introduction of the AAC technologies with VSDs using JIT technology.

Conclusions: AAC with VSDs and JIT programming may be effective in increasing symbolic communication for students with severe developmental disability. The fast and easy creation of VSDs and hotspots to provide communication may be a valuable tool for interventionists.