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Health and Social Care Delivery Research Jun 2024People with learning disabilities are living longer. Despite government policy to encourage people to lead supported lives in their community, family carers often...
BACKGROUND
People with learning disabilities are living longer. Despite government policy to encourage people to lead supported lives in their community, family carers often maintain support due to dissatisfaction with services. This can lead to people moving from the family home in a crisis.
OBJECTIVES
(1) Find out what is known about health needs and resources for older people with learning disabilities (aged ≥ 40 years); (2) identify exemplars of good services for older people with learning disabilities; (3) explore service exemplars through ethnographic case studies; (4) evaluate support for older people with learning disabilities and their families through co-producing and testing future planning tools and (5) co-produce recommendations and resources.
DESIGN AND METHODS
Work package 1 rapid scoping reviews - three reviews focused on the health and social care needs of older people with learning disabilities and 'behaviours that challenge others', and family carers, and the co-ordination of support for this group. Work package 2 scoping and mapping exemplars of good practice - analysis of published service standards to assess excellence criteria, by mapping services, interviews ( = 30), survey ( = 9) and informal discussion with commissioners. Work package 3 ethnography of case studies of exemplar provision; independent supported living ( = 4); residential/nursing home ( = 2); day activities ( = 1), Shared Lives ( = 2). Fieldwork (20 days per model), interviews ( = 77) with older people with learning disabilities, family carers, support staff and commissioners. Work package 4 - co-producing and testing resources for older people with learning disabilities and their families involved interviews and focus groups with 36 people with learning disabilities, parents, and siblings, and experience-based co-design with 11 participants. Eight families evaluated the resources. Work package 5 - three stakeholder workshops co-produced service recommendations.
FINDINGS
The reviews confirmed an inadequate evidence base concerning the experiences and support of family carers and older people with learning disabilities and 'behaviours that challenge others'. Criteria of excellence were produced, and a shortlist of 15 services was identified for consideration in work package 3. The ethnographic work found that environmental, organisational and social factors were important, including supporting independence and choice about who people live with, matching staff to people, consistent relationships and adapting to ageing. Practices of institutionalisation were observed. In work package 4, we found that families were worried about the future and unsupported to explore options. 'Planning Ahead' cards and a booklet to record discussions were produced, and the evaluation was positively rated. Finally, formative discussion informed recommendations. Outputs include training packages, a carers' forum, a film, a podcast and academic papers.
CONCLUSIONS
There is little focus on older people with learning disabilities and family carers. Services vary in their approach to planning for older-age support. Families are unsupported to plan, leaving people without choice. 'Behaviours that challenge others' was found to be unhelpful terminology. Recommendations: A new strategy is recommended for older people with learning disabilities and family carers that encompasses commissioning practices, professional input and peer learning, proactive support in ageing well and excellent service design.
LIMITATIONS
The COVID-19 pandemic created recruitment challenges. Reliance on providers for recruitment resulted in a lack of diversity in work package 3. Families' plans, and therefore change, may be frustrated by insufficient service resources.
FUTURE WORK
Given the lack of focus in this area, there is a range of future work to consider: experiences of older people with learning disabilities from diverse ethnic backgrounds; supporting people to age and die 'in place'; best practice regarding designing/commissioning services, including housing; the role of social workers; access to nature; accessing mainstream support; and evaluation of the 'Planning Ahead' cards.
TRIAL REGISTRATION
This trial is registered as ISRCTN74264887.
FUNDING
This award was funded by the National Institute for Health and Care Research (NIHR) Health and Social Care Delivery Research programme (NIHR award ref: NIHR129491) and is published in full in ; Vol. 12, No. 16. See the NIHR Funding and Awards website for further award information.
Topics: Humans; Caregivers; Aged; Learning Disabilities; Female; Male; Middle Aged; Adult; Social Support; Qualitative Research; Aged, 80 and over; Anthropology, Cultural; Health Services Needs and Demand
PubMed: 38940476
DOI: 10.3310/MTHW2644 -
Journal of Clinical Medicine Jun 2024Floating-Harbor syndrome (FHS) is an extremely rare genetic disorder connected with a distinctive facial appearance, various skeletal malformations, delayed bone age,...
Floating-Harbor syndrome (FHS) is an extremely rare genetic disorder connected with a distinctive facial appearance, various skeletal malformations, delayed bone age, and expressive language delays. It is caused by heterozygous mutations in the Snf2-related CREBBP activator protein (SRCAP) gene. The aim of this paper is to describe the case of a 14-year-old male with FHS, referring to a review of the literature, and to collect all reported symptoms. In addition, the orthodontic treatment of the patient is described. For this, the electronic databases PubMed and Scopus were searched using the keyword "Floating-Harbor syndrome". Similar to previous cases in the literature, the patient presented with short stature; a triangular face with a large bulbous nose; deep-set eyes and narrow eyelid gaps; a wide mouth with a thin vermilion border of the upper lip; and dorsally rotated, small ears. They also presented some less-described symptoms, such as macrodontia and micrognathia. Moreover, mild mental retardation, microcephaly, and delayed psychomotor development were found. On the basis of an extraoral, intraoral examination, X-rays, and CBCT, he was diagnosed with overbite, canine class I and angle class III, on both sides. To the best of our knowledge, orthodontic treatment of this disease has not been assessed in detail so far, so this is the first case.
PubMed: 38929963
DOI: 10.3390/jcm13123435 -
Open Access Emergency Medicine : OAEM 2024The objective of this review was to explore parents' experiences and information needs regarding management of their child with an intellectual and/or developmental... (Review)
Review
Seeking Care for Children with Intellectual and/or Developmental Disabilities in the Emergency Department: A Mixed Methods Systematic Review of Parents' Experiences and Information Needs.
The objective of this review was to explore parents' experiences and information needs regarding management of their child with an intellectual and/or developmental disability (IDD) in the emergency department (ED). We searched six electronic databases and grey literature to identify primary studies in English published since 2000. We synthesized quantitative and qualitative outcome data simultaneously using a convergent integrated approach and used a Mixed Methods Appraisal Tool (MMAT) to assess methodological quality of the included studies. Nine articles derived from seven studies were included (3 qualitative, 3 quantitative, 1 mixed method). Four main themes related to parents' self-reported experiences were identified: 1) appropriateness of the ED to manage and support their child; 2) acknowledgement/recognition of their child's IDD and incorporation of those considerations into overall care and management; 3) managing and navigating the ED environment; and 4) decision to disclose their child's condition when visiting the ED. Two articles provided data relevant to information needs, highlighting parents' desire to have resources supporting ED orientation and access to services within and outside of the ED setting. From the limited number of studies, it was evident that parents wanted better communication with healthcare providers and a greater understanding by ED staff around physical space settings needed to support their child. Resources supporting ED staff and parents to communicate effectively and work together can ensure that children with IDDs care needs are met. Further research into understanding parents' experiences and information needs related to managing a child with an IDD in the ED is needed to guide the development of effective resources.
PubMed: 38912093
DOI: 10.2147/OAEM.S450191 -
Journal of Bodywork and Movement... Jul 2024Loss of hand function causes severe limitations in activity in daily living. The hand-soft robot is one of the methods that has recently been growing to increase the... (Review)
Review
INTRODUCTION
Loss of hand function causes severe limitations in activity in daily living. The hand-soft robot is one of the methods that has recently been growing to increase the patient's independence. The purpose of the present systematic review was to provide a classification, a comparison, and a design overview of mechanisms and the efficacy of the soft hand robots to help researchers approach this field.
METHODS
The literature research regarding such tools was conducted in PubMed, Google Scholar, Science Direct, and Cochrane Central Register for Controlled Trials. We included peer-reviewed studies that considered a soft robot glove as an assistive device to provide function. The two investigators screened the titles and abstracts, then independently reviewed the full-text articles. Disagreements about inclusion were resolved by consensus or a third reviewer.
RESULTS
A total of 15 articles were identified, describing 210 participants (23 healthy subjects). The tools were in three categories according to their actuation type (pneumatic system, cable-driven, another design). The most critical outcomes in studies included functional tasks (fourteen studies), grip strength (four studies), range of motion (ROM) (five studies), and user satisfaction (five studies).
DISCUSSION
Function and grip parameters are the most common critical parameters for tests of hand robots. Cable-driven transmission and soft pneumatic actuators are the most common choices for the actuation unit. Radder et al. study had the highest grade from other studies. That was the only RCT among studies.
CONCLUSION
Although few soft robotic gloves can be considered ready to reach the market, it seems these tools have the potential to be practical for people with a disability. But, we lack consistent evidence of comparing two or more soft robot gloves on the hand functions. Future research needs to assess the effect of soft robotic gloves on people with hand disorders with more populations.
Topics: Humans; Robotics; Hand Strength; Hand; Self-Help Devices; Range of Motion, Articular; Activities of Daily Living; Equipment Design
PubMed: 38876658
DOI: 10.1016/j.jbmt.2024.02.025 -
Journal of Applied Research in... Jul 2024Providing menstrual education and guidance for menstrual management for girls and young women with intellectual disabilities is recommended to ensure smooth pubertal...
BACKGROUND
Providing menstrual education and guidance for menstrual management for girls and young women with intellectual disabilities is recommended to ensure smooth pubertal transitions and to support menstrual self-agency.
METHOD
The purpose of this systematic review is to explore menstrual education interventions for girls and young women with intellectual disabilities.
RESULTS
Nine studies were included. Interventions were provided in small groups (n = 4) and individually (n = 5). Most studies used dolls (n = 7) and task analysis (n = 7) to teach pad-replacement skills. All reported significant improvements in participant skills and/or knowledge following the intervention. Only one study addressed self-agency and self-esteem as an outcome of the intervention. Menstrual education for girls and young women with intellectual disabilities is largely focused on pad-replacement skills.
CONCLUSION
Further research is needed to understand the impact of menstrual health and hygiene education on variables apart from skill improvement such as self-agency and long-term health outcomes related to menstrual health.
Topics: Adolescent; Adult; Child; Female; Humans; Young Adult; Developmental Disabilities; Health Education; Health Knowledge, Attitudes, Practice; Intellectual Disability; Menstruation; Sex Education
PubMed: 38863161
DOI: 10.1111/jar.13264 -
Epilepsy & Behavior : E&B May 2024The post-surgical outcome for Hypothalamic Hamartoma (HH) related epilepsy in terms of seizure freedom (SF) has been extensively studied, while cognitive and psychiatric...
The post-surgical outcome for Hypothalamic Hamartoma (HH) related epilepsy in terms of seizure freedom (SF) has been extensively studied, while cognitive and psychiatric outcome has been less frequently reported and defined. This is a systematic review of English language papers, analyzing the post-surgical outcome in series of patients with HH-related epilepsy (≥5 patients, at least 6 months follow-up), published within January 2002-December 2022. SF was measured using Engel scale/equivalent scales. We looked at the outcome related to different surgical techniques, and HH types according to Delalande classification. We evaluated the neuropsychological and neuropsychiatric status after surgery, and the occurrence of post-surgical complications. Forty-six articles reporting 1318 patients were included, of which ten pediatric series. SF was reported in 686/1222 patients (56,1%). Delalande classification was reported in 663 patients from 24 studies, of which 70 were type I HH (10%), 320 were type II HH (48%), 189 were type III HH (29%) and 84 were type IV HH (13%). The outcome in term of SF was reported in 243 out of 663 patients. SF was reported in 12 of 24 type I HH (50%), 80 of 132 type II HH (60,6%), 32 of 59 type III HH (54,2%) and 12 of 28 type IV HH (42,9%). SF was reached in 129/262 (49,2%) after microsurgery, 102/199 (51,3%) after endoscopic surgery, 46/114 (40,6%) after gamma knife surgery, 245/353 (69,4%) after radiofrequency thermocoagulation, and 107/152 (70,4%) after MRI-guided laser interstitial thermal therapy. Hyperphagia/weight gain were the most reported surgical complications. Others were electrolyte alterations, diabetes insipidus, hypotiroidism, transient hyperthermia/poikilothermia. The highest percentage of memory deficits was reported after microsurgery, while hemiparesis and cranial nerves palsy were reported after microsurgery or endoscopic surgery. Thirty studies reported developmental delay/intellectual disability in 424/819 (51,7%) patients. 248/346 patients obtained a global improvement (72%), 70/346 were stable (20%), 28/346 got worse (8%). 22 studies reported psychiatric disorders in 257/465 patients (55,3%). 78/98 patients improved (80%), 13/98 remained stable (13%), 7/98 got worse (7%). Most of the patients had non-structured cognitive/psychiatric assessments. Based on the available data, the surgical management in patients with HH related epilepsy should be individualized, aiming to reach not only the best epilepsy result, but also the optimal cognitive and psychiatric outcome.
PubMed: 38820683
DOI: 10.1016/j.yebeh.2024.109846 -
BMC Psychology May 2024Previous research has suggested that the core features of autism spectrum disorders (ASD) may contribute to offending behaviours and increased vulnerability within the...
Previous research has suggested that the core features of autism spectrum disorders (ASD) may contribute to offending behaviours and increased vulnerability within the Criminal Justice System. To date, there is a paucity of evidence assessing the effectiveness of interventions for offending behaviour in adults with ASD but without co-occurring intellectual disability (ID) across a broad range of forensic settings. The lack of robust evidence is concerning, as limited effectiveness may contribute to an increased likelihood of prolonged incarceration, particularly in the most restrictive settings. A PRISMA systematic review was conducted with a narrative synthesis to: (a) evaluate the evidence of the effectiveness of interventions aimed at reducing recidivism, (b) assess whether the core features of ASD impact the effectiveness of these interventions, and (c) identify additional factors that may affect the effectiveness of interventions within this population. Seven studies involving ten male participants were identified. The findings suggest that interventions for offending behaviours in adults with ASD without intellectual disability (ID) are largely inadequate, and that core ASD features need to be considered. Additionally, a complex interplay of risk factors potentially impacting intervention effectiveness was suggested. Limitations include heterogeneity across intervention types, measures of effectiveness, and what constitutes effectiveness. Despite the limited number of studies and data quality, the review aligns with a growing body of literature highlighting vulnerability and a need for evidence-based interventions for people with ASD. The review also discusses the broader implications of ineffective interventions.
Topics: Humans; Autism Spectrum Disorder; Criminals; Adult; Male; Recidivism
PubMed: 38816780
DOI: 10.1186/s40359-024-01770-1 -
Journal of Nursing Scholarship : An... May 2024Women with disabilities are more exposed to violence. The health sector has a key role in all three levels of prevention of violence against women. The objective of this...
PURPOSE
Women with disabilities are more exposed to violence. The health sector has a key role in all three levels of prevention of violence against women. The objective of this paper was to review the interventions for preventing gender-based violence and reducing its impact on the mental health of women with any form of disability.
METHOD
Relevant studies were identified through conducting searches in PubMed, Scopus, CINAHL, PsyInfo, Social Services Abstracts, and PILOTS. Two reviewers analyzed and selected studies. A qualitative synthesis was made.
RESULTS
3149 references were obtained, among which eight articles describing nine interventions from the USA and the UK. Most were intended for women with mental/intellectual disability and assessed intimate partner or sexual violence. Only one study showed high methodological quality. They were found to be particularly effective as regards improvement of the skills acquired by participants, but the results as regards improved mental health are not consistent.
CONCLUSION
Our review shows very little evidence of effective interventions. Further studies are required with higher internal validity and female sample groups with diverse disabilities.
CLINICAL RELEVANCE
Gender-based violence is a highly prevalent problem for women with disabilities, and in addition to being a public health challenge is a violation of human rights. Health care systems and policymakers should take a key role in all three levels of prevention of violence against women with disabilities. Interventions with longer follow-up times are required. It is also important for interventions to be designed in consultation with people with disabilities.
PubMed: 38804594
DOI: 10.1111/jnu.12985 -
Journal of Applied Research in... Jul 2024Self-determination is associated with lifelong positive outcomes. Students with intellectual disabilities typically have lower self-determination than their peers.... (Review)
Review
BACKGROUND
Self-determination is associated with lifelong positive outcomes. Students with intellectual disabilities typically have lower self-determination than their peers. Universal basic education access offers schools the opportunity to rectify this disparity. This is the first systematic review investigating the school-based practices that target self-determination development for students with intellectual disabilities.
METHOD
The review follows the PRISMA guidelines, spanning five databases (ProQuest databases, EMBASE, Scopus, Sage Journals, Taylor and Francis Online) from 2006 to 2021.
RESULTS
Across the 18 studies, the most used practice is the SDLMI. Research focuses on United States-based transition-aged students with mild to moderate intellectual disabilities. Social validity tends to be assessed in summative and informal ways. Students are not generally involved in decision-making about practices and individualisation of support.
CONCLUSIONS
Self-determination development for this population can begin before puberty. Future research should critically investigate social validity and holistic integration of student self-determination learning opportunities throughout the pedagogical cycle.
Topics: Humans; Intellectual Disability; Child; Personal Autonomy; Adolescent; Schools; Students
PubMed: 38797718
DOI: 10.1111/jar.13247 -
International Journal of Molecular... May 2024The gene encodes an orphan transcription factor of the steroid-thyroid hormone-retinoid receptor superfamily. This review focuses on the clinical findings associated... (Review)
Review
The gene encodes an orphan transcription factor of the steroid-thyroid hormone-retinoid receptor superfamily. This review focuses on the clinical findings associated with the pathogenic variants so far reported, including three unreported cases. Also, its role in neurodegenerative diseases, such as Parkinson's or Alzheimer's disease, is examined, as well as a brief exploration on recent proposals to develop novel therapies for these neurological diseases based on small molecules that could modulate transcriptional activity. The main characteristic shared by all patients is mild to severe developmental delay/intellectual disability. Moderate to severe disorder of the expressive and receptive language is present in at least 42%, while neuro-psychiatric issues were reported in 53% of patients. Movement disorders, including dystonia, chorea or ataxia, are described in 37% patients, although probably underestimated because of its frequent onset in late adolescence-young adulthood. Finally, epilepsy was surprisingly present in 42% of patients, being drug-resistant in three of them. The age at onset varied widely, from five months to twenty-six years, as did the classification of epilepsy, which ranged from focal epilepsy to infantile spasms or Lennox-Gastaut syndrome. Accordingly, we propose that should be considered as a first-tier target gene for the genetic diagnosis of developmental and epileptic encephalopathy.
Topics: Humans; Epilepsy; Nuclear Receptor Subfamily 4, Group A, Member 2; Developmental Disabilities; Intellectual Disability
PubMed: 38791237
DOI: 10.3390/ijms25105198