Factors influencing communication partners of persons with severe/profound intellectual disability use of augmentative and alternative communication: an integrative review.

PURPOSE: To present a synthesis of evidence related to the factors influencing communication partners' use of augmentative and alternative communication with persons with severe/profound intellectual disability. MATERIALS AND METHODS: An integrative review guided by five steps; problem identification, literature search, data evaluation, data analysis and presentation was undertaken. In accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement, nine databases were searched, 1,342 studies were screened against the eligibility criteria, and 15 studies underwent thematic analysis. RESULTS: Two themes emerged; (1) Achieving Meaningful Communication and (2) Communication Partners' Preparedness to Use Augmentative and Alternative Communication. Achieving meaningful communication was central to communication partners' use of augmentative and alternative communication and was two-fold. It involved identifying the persons' communication methods and encouraging them to communicate. Communication partners' preparedness also influenced their use of augmentative and alternative communication. This preparedness was impacted by communication partners' preconceived thoughts about and knowledge of augmentative and alternative communication, nurturing their belief in augmentative and alternative communication, and the interpersonal dynamic between network members. CONCLUSION: Communication partners' use of augmentative and alternative communication is influenced by multiple and complex factors. The findings contribute to the knowledge of the potential factors to be considered to prepare communication partners to use augmentative and alternative communication.

Multiple, complex factors influence communication partners of persons with severe/profound intellectual disability use of augmentative and alternative communication (AAC), which include communication partners' beliefs, attitudes, expectations, knowledge and resources such as training, support and time.To offer individuals with severe/profound intellectual disability opportunities to communicate, communication partners need to recognise their attempts and thus, their ability. Continuously being sensitive to the individuals' communication methods, whilst being cognisant that these methods can change may enhance communication partners' awareness and understanding of the individuals' communication attempts.Communication partners' need to feel prepared to use AAC. To feel prepared, they need to be aware of the potential benefits that AAC can offer the interaction and the long-term outcomes, develop their knowledge, and be surrounded by a supportive team dynamic.

The roles of adult siblings of individuals with a profound intellectual disability.

BACKGROUND: Adult siblings are important in the lives of individuals with profound intellectual disabilities, especially as parents age. However, little is known about the roles they assume. METHOD: We examined these roles among 58 participants from the Netherlands, who completed an online questionnaire. RESULTS: Most participants (89.7%) assumed multiple roles, and the majority were content with their roles. Between 7% and 58% indicated that they were the only individuals providing a certain role. Shared roles primarily involved healthcare professionals, other siblings, and parents. Almost half the participants (48.2%) assigned higher scores for the amount of support provided than for the experienced burden. CONCLUSIONS: Adult siblings often assume multiple roles. The shared roles emphasise the importance of collaboration.

Scoping review on communication systems used by adults with severe/profound intellectual disability for functional communication.

BACKGROUND: Adults with severe/profound intellectual disability typically face poor communication outcomes as they are often nonverbal and need their supporters to provide for their communication needs. This review aimed to identify studies focused on the communication resources people with severe/profound intellectual disability use for functional communication, and the enablers and barriers to functional communication. METHODS: Nine databases were systematically reviewed with keywords pertaining to the functional communication of adults with severe/profound intellectual disability. Out of 3427 identified articles, 12 met the inclusion criteria. Hand searches and ancestral searches identified another 4 articles. Out of the 16 articles, two did not meet the quality assessment criteria and were excluded. Thus, 14 articles were included in this review. RESULTS: The findings revealed that picture exchange communication systems is the most common communication system used to support the development of functional communication. The most common functions enabled by the communication systems were choice-making and making requests. Several barriers (e.g., individual factors related to adults with severe/profound intellectual disability, others' attitudes, behaviour and knowledge) to and enablers (e.g., accessibility and availability of the communication system, training for those supporting adults with severe/profound intellectual disability) of functional communication were identified. CONCLUSIONS: Removing the barriers and enabling functional communication is essential to developing the functional communication of adults with severe/profound intellectual disability.

Advancing inclusive research with people with profound and multiple learning disabilities through a sensory-dialogical approach.

People with profound and multiple learning disabilities are often excluded from the processes of knowledge production and face barriers to inclusion in research due to cognitive and communicative challenges. Inclusive research-even when intending to be inclusive-tends to operate within criteria that exclude people with profound and multiple learning disabilities. The aim of this article is to provide a state-of-the-art review of the topic of inclusive research involving people with profound disabilities and thereby challenge traditional assumptions of inclusive research. The review presents themes that will inform a discussion on how to challenge the criteria in ways that make it possible to understand inclusive research for people who communicate in unconventional ways. We argue that a fruitful way of rethinking inclusive research is by applying a sensory-dialogical approach that privileges the dialogical and sensory foundations of the research. We suggest this might be a way to understand inclusive research that regards the person's communicative and cognitive distinctiveness.

Piloting attachment psychoeducation provided to parents of children with severe disabilities: Testing the feasibility of a digital micro-intervention.

BackgroundA digital micro-intervention offering attachment psychoeducational videos was explored regarding its feasibility in parents of children with severe disabilities. Method: A mixed-methods study (including daily diaries and one-time questionnaires) with 16 parents (75.0% female) of children with severe disabilities (up to 10 years of age) was done during a three-week intervention. Results: Parents were positive about the video series and almost no drop-out occurred. The videos stimulated their learning and thinking and offered parents some personal meaning. Preliminary efficacy tests showed no major changes in parents' parenting self-efficacy (PSE) or perceptions of statements on parent-child attachment. Conclusions: The study showed promising results regarding the micro-interventions' acceptability and implementation. The limited efficacy testing did not show major changes in parents' PSE. Further research is needed to investigate the differential relevance of the micro-intervention, based on parents' needs, as well as its optimal embeddedness in a broader intervention trajectory.

Communication partners experiences of communicating with adults with severe/profound intellectual disability through augmentative and alternative communication: A mixed methods systematic review.

People with severe/profound intellectual disability experience challenges in communicating and require their communication partners to adapt to their means of communication. Augmentative and Alternative Communication (AAC) is recognised as a potential means to meet their communication needs. Interventions need to be aimed at both the individual and their communication partners. We conducted a mixed methods systematic review of the literature to synthesise evidence on communication partners experience of communicating with adults with severe/profound intellectual disability through AAC. Eight publications met the inclusion criteria, they underwent thematic synthesis where four themes emerged. A shared commitment to communication partnership is fundamental for the effective and efficient use of AAC. However, there was a disconnect between communication partners perceptions of their roles and responsibilities. This review prompts further research to explore communication partners perceptions of their roles and responsibilities in the use of AAC with people with severe/profound intellectual disabilities.

Meaningful moments of interaction with people with profound intellectual disabilities: Reflections from direct support staff.

BACKGROUND: High-quality, affective relationships are built on meaningful moments of interaction, which are challenging for support staff to establish with people with profound intellectual disabilities. Therefore, we explored what makes a moment of interaction meaningful to support staff and what circumstances facilitate meaningful moments of interaction taking place. METHOD: Five direct support staff took part in unstructured, in-depth interviews. The interviews were analysed using interpretative phenomenological analysis (IPA). Member checks were also conducted. RESULTS: Support staff experienced moments of interaction as meaningful because they felt a connection with a person with profound intellectual disabilities and/or they had the feeling of being meaningful for this person. Staff-related and contextual circumstances facilitating meaningful moments of interaction to take place were described. CONCLUSIONS: Meaningful moments of interaction are highly valued by support staff, who believe these moments are valued by persons with profound intellectual disabilities as well. Implications for daily practice are described.

Exploring co-occurrence of sensory, motor and neurodevelopmental problems and epilepsy in children with severe-profound intellectual disability.

BACKGROUND: Severe to profound intellectual disability (SPID) is associated with multiple neurodevelopmental disorders and problems. In the most severe cases, the term profound intellectual and multiple disabilities (PIMD) is used. This study aimed to explore the co-occurring disorders and neurodevelopmental problems in a sample of twins where the proband had SPID. METHOD: Within a population-based sample of (30 312) twins, 20 individuals with a national patient register SPID diagnosis were identified. Parent telephone interview data (screening of neurodevelopmental disorders) and register data (APGAR, birth weight, intellectual disabilities, epilepsy, motor and sensory disorders) were gathered for probands and co-twins. RESULTS: The 20 individuals with SPID all had between one and five additional disorders or problems, with autistic traits, motor problems and epilepsy being the most common. Clear discordance was found for ID and all additional disorders and problems between probands with SPID and their non-SPID co-twins. CONCLUSION: Children with SPID almost never present without neurodevelopmental and/or sensory and/or motor comorbidities. This heterogeneity should be reflected in clinical routine and in research targeting individuals with SPID. The results support a previously suggested conceptualization of a S/PIMD "spectrum". Autism may be considered for inclusion in future elaborations of such a S/PIMD spectrum.

Assistive technology for persons with profound intellectual disability: a european survey on attitudes and beliefs.

BACKGROUND: Persons with profound intellectual disability (PID) are mostly not able to use assistive technology (AT) independently. Caregivers play an important mediating role in implementing AT in the daily life of persons with PID. Both first-order barriers, extrinsic to caregivers, and second-order barriers, intrinsic to caregivers, influence the attitudes and behaviors of caregivers with regard to AT-use. It could be asked if increased knowledge on and experience with AT may impact the effect of first- and second-order barriers. This study investigated how knowledge and experience influence the professional caregivers' beliefs about which factors may impact the AT use in persons with PID and their intentions to use AT for persons with PID. METHODS: A questionnaire on the experienced limitations and successes in using AT was developed. The questionnaire was send to professionals working with or responsible for persons with PID in various countries in Europe. In total the answers of 195 respondents were included in this study. RESULTS AND CONCLUSIONS: This study's results demonstrate that AT is used for various reasons in persons with PID, mostly to support communication and interaction or for fun or relaxation. Based on the answers of the respondents can be concluded that both experience and knowledge of caregivers seem to influence first- and second-order barriers. Besides, a possibility to overcome the second-order barriers is to provide professionals with possibilities to increase their knowledge and experience.IMPLICATIONS FOR REHABILITATIONAT for persons with PID is mostly used for communication and interaction or for fun and relaxation.Professional caregivers belief that AT-use may positively influence various aspects in the life of persons with PID, especially communication and interaction, active engagement and participation in activities, and self-esteem of the person.Caregivers need to have sufficient experience in order to rate the barriers of AT-use as less limited in the group of persons with PID.In order to overcome the barriers experienced in implementing AT in persons with PID, knowledge of caregivers is essential.

FBXO28 causes developmental and epileptic encephalopathy with profound intellectual disability.

Chromosome 1q41-q42 deletion syndrome is a rare cause of intellectual disability, seizures, dysmorphology, and multiple anomalies. Two genes in the 1q41-q42 microdeletion, WDR26 and FBXO28, have been implicated in monogenic disease. Patients with WDR26 encephalopathy overlap clinically with those with 1q41-q42 deletion syndrome, whereas only one patient with FBXO28 encephalopathy has been described. Seizures are a prominent feature of 1q41-q42 deletion syndrome; therefore, we hypothesized that pathogenic FBXO28 variants cause developmental and epileptic encephalopathies (DEEs). We describe nine new patients with FBXO28 pathogenic variants (four missense, including one recurrent, three nonsense, and one frameshift) and analyze all 10 known cases to delineate the phenotypic spectrum. All patients had epilepsy and 9 of 10 had DEE, including infantile spasms (3) and a progressive myoclonic epilepsy (1). Median age at seizure onset was 22.5 months (range 8 months to 5 years). Nine of 10 patients had intellectual disability, which was profound in six of nine and severe in three of nine. Movement disorders occurred in eight of 10 patients, six of 10 had hypotonia, four of 10 had acquired microcephaly, and five of 10 had dysmorphic features, albeit different to those typically seen in 1q41-q42 deletion syndrome and WDR26 encephalopathy. We distinguish FBXO28 encephalopathy from both of these disorders with more severe intellectual impairment, drug-resistant epilepsy, and hyperkinetic movement disorders.

Outcomes and costs of skilled support for people with severe or profound intellectual disability and complex needs.

BACKGROUND: With increasing reductions in funding for social care across many countries, the need to ensure that resources are used to best effect is becoming increasingly important, in particular for those with severe and complex needs. METHODS: In order to explore the outcomes and costs of skilled support for this group of people, quality of life was assessed for 110 people in 35 services in England. Information on costs was also collected. RESULTS: People who received consistently good active support experienced better outcomes in terms of several quality of life domains. Good support did not require significantly more staff time, and there was no evidence of higher total costs for those receiving good support. CONCLUSIONS: The inclusion of active support in government guidance and local commissioning practices related to people with severe intellectual disabilities is likely to improve user outcomes. Observation should be an important element in measuring service quality.

Comfort provided by parents versus strangers after eliciting stress in children with severe or profound intellectual disabilities: does it make a difference?

The relationships between children with severe or profound intellectual disabilities (ID) and their parents may fulfil attachment functions, such as regulating emotional responses to stress. This study examined the extent to which children with severe or profound ID differentiate between their parents and a stranger as a resource for stress-regulation. A home-based experimental paradigm was conducted and video-recorded in 38 families. Children (1-8 years) were exposed to four naturalistic stressors followed by comfort, randomly provided by the parents or the stranger. Emotional behaviour (arousal and valence) and the skin conductance level were simultaneously recorded. With regard to both emotional behaviour and skin conductance, children significantly differentiated between their parents as attachment figures and the stranger during stress and comfort, despite their impairments on various developmental domains. Behavioural observation and physiology show complementary manifestations of parent-child attachment in this population.

Can I be your safe haven and secure base? A parental perspective on parent-child attachment in young children with a severe or profound intellectual disability.

BACKGROUND: The general developmental as well as the disability specific literature has stressed the crucial influence of parents on their child's social-emotional development. Attachment theory provides a framework to describe parental roles within the parent-child attachment relationship. The current study explored parents' perspectives on their role as attachment figure and the preconditions they consider necessary to establish secure attachment in children with severe or profound intellectual disability (ID). METHODS: Semi-structured interviews with 54 parents on their child's social-emotional development, attachment behaviour and the parent-child attachment bond were analysed using the Framework Method. All children were between 15 months and seven years old and had a severe or profound ID. RESULTS: Parents reported their child's clear preference towards them and acknowledged the role they fulfil as stress regulator. Children differed in the extent to which they use their parent to explore new environments. Overall, parents described the attachment relationship with their child as positive but challenging. CONCLUSIONS: Parents acknowledged the roles they fulfil both as a safe haven for their child, and (to a lesser extent) as a secure base. Clinical practice could benefit from a parental perspective to identify particular challenges parents encounter in building a secure attachment relationship.

Young children with significant developmental delay differentiate home observed attachment behaviour towards their parents.

BACKGROUND: The hallmark of attachment is that contact, proximity and relief from stress are sought from specific individuals, laying important groundwork for healthy socioemotional functioning. This study investigated the extent to which differentiated attachment behaviour can be observed in young children with significant developmental delay (DD). METHOD: Video-taped observations of the parent-child and stranger-child interaction were conducted at home and complemented with questionnaires in 20 families with a child with significant DD (age 2-7 years with an average DD of 49 months). RESULTS: Children displayed more intense and persistent contact-seeking, contact-maintaining and resistant behaviour in the episodes with their parent compared to the episodes with the stranger. Parent-reported secure attachment behaviour was slightly more characteristic towards mother compared to father. CONCLUSIONS: Even children with significant DD develop differentiated attachment behaviour. Detailed observations may support parents in identifying the interactions that make the attachment relationship with their child special.

Transition from school to adult services for young people with severe or profound intellectual disability: A systematic review utilizing framework synthesis.

BACKGROUND: The transition to adulthood has been described as a difficult time in the lives of young people with intellectual disability. There has been little emphasis on young people with severe or profound intellectual disability specifically, even though their pathways may differ, due to greater support needs across the life course. METHODS: A systematic review was conducted utilising Bronfenbrenner's ecological model to inform framework analysis to synthesise qualitative findings. RESULTS: Taking an ecological perspective proved valuable. The transition process was described as stressful and barriers were identified across the ecological levels. Parents accounted for the majority of participants in studies, and the needs of young people and their parents emerged as highly interdependent. CONCLUSION: Themes reflect the complex nature of the question what adulthood should look like for individuals with severe or profound intellectual disability. There is a lack of involvement of multiple stakeholders and young people themselves within studies.

Rate and characteristics of urgent hospitalisation in persons with profound intellectual disabilities compared with general population.

BACKGROUND: Little is known about the hospitalisation rate of adults with severe/profound intellectual disability (PID) presenting at emergency services or about the appropriateness of hospital admissions in this population. Examining the possible differences in the patterns of hospitalisation between people with PID and those without intellectual disability (ID) may shed light on aspects of health and illness in these patients and may in turn make it possible to differentiate more clearly between mild-moderate ID and PID. METHODS: After an evaluation of the emergency visits made by adults with PID and by people without ID, patients in both groups requiring one or more hospitalisations were subsequently followed up for 18 months. The appropriateness of the decision to hospitalise was assessed using the ambulatory care-sensitive conditions index. RESULTS: There were no differences in the proportion of people with PID and controls admitted to hospital after their emergency visit. The median hospital stay was higher for PIDs: 7.5 vs. 4 days for controls. People with PID were admitted more than controls for respiratory reasons and somewhat less for other somatic causes unrelated to the nervous system. There were no admissions for psychiatric causes in the group with PID other than unspecified conduct disorders. There were no differences in other diagnostic groups. The rate of inappropriate admissions was similar in the two study groups. CONCLUSIONS: In contrast to previous results reported for the group with ID as a whole, patients with PID consulting the emergency service were not admitted to hospital more frequently than the general population nor did they present a higher rate of inappropriate admissions. These results support the utility of maintaining two distinct groups of people with ID: mild-moderate and severe-profound.

Staffs' documentation of participation for adults with profound intellectual disability or profound intellectual and multiple disabilities.

PURPOSE: This study investigated what areas of International Classification of Functioning, Disability and Health were documented in implementation plans for adults with profound intellectual disability or profound intellectual and multiple disabilities with focus on participation. METHODS: A document analysis of 17 implementation plans was performed and International Classification of Functioning, Disability and Health was used as an analytic tool. RESULTS: One hundred and sixty-three different codes were identified, especially in the components Activities and participation and Environmental factors. Participation was most frequently coded in the chapters Community, social and civic life and Self-care. Overall, the results showed that focus in the implementation plans concerned Self-care and Community, social and civic life. The other life areas in Activities and participation were seldom, or not at all, documented. CONCLUSIONS: A deeper focus on participation in the implementation plans and all life areas in the component Activities and participation is needed. It is important that the documentation clearly shows what the adult wants, wishes, and likes in everyday life. It is also important to ensure that the job description for staff contains both life areas and individual preferences so that staff have the possibility to work to fulfill social and individual participation for the target group. Implications for rehabilitation There is a need for functioning working models to increase participation significantly for adults with profound intellectual disability or profound intellectual and multiple disabilities. For these adults, participation is achieved through the assistance of others and support and services carried out must be documented in an implementation plan. The International Classification of Functioning, Disability and Health can be used to support staff and ensure that information about the most important factors in an individual's functioning in their environment is not omitted in documentation.

Supporting end of life decision making: Case studies of relational closeness in supported decision making for people with severe or profound intellectual disability.

BACKGROUND: The United Nations Convention on the Rights of Persons with Disabilities (UNCRPD) promotes the use of supported decision making in lieu of substitute decision making. To date, there has been a lack of focus on supported decision making for people with severe or profound intellectual disability, including for end of life decisions. METHODS: Five people with severe or profound intellectual disability's experiences of supported decision making were examined. This article is particularly focused on one participant's experiences at the end of his life. RESULTS: All five case studies identified that supporters were most effective in providing decision-making support for participants when they were relationally close to the person and had knowledge of the person's life story, particularly in relation to events that demonstrated preference. CONCLUSIONS: Findings from this study provide new understandings of supported decision making for people with severe or profound intellectual disability and have particular relevance for supporting decision making at the end of life.

A randomised controlled trial on evaluation of the clinical efficacy of massage therapy in a multisensory environment for residents with severe and profound intellectual disabilities: a pilot study.

BACKGROUND: Recent literature has suggested that relaxation activities can reduce the challenging behaviours of people with intellectual disabilities, particularly in severe and profound grades, due to the counteractive effect of muscle relaxation on emotional frustration or psychological distress. Despite having inconclusive evidence, multisensory environment (MSE) and massage therapy (MT) are the commonly used approaches to relaxation among these people. However, these two approaches have not yet practised or tested in combination for reducing these people's challenging behaviours. METHODS: A preliminary clinical efficacy trial was conducted to evaluate the effects of MT, MSE and their combined use for residents with intellectual disabilities in a long-term care facility on reducing their challenging behaviours. Eligible residents were recruited and randomly assigned to one of the four study groups (n = 11-12 per group), that is, MT in MSE, MSE alone, MT alone or usual care, for a 10-week intervention after a 1-month washout period. Outcome measures, including the Behaviour Problem Inventory, pulse and respiration rates, Behaviour Checklist and Alertness Observation Checklist, were assessed at recruitment and immediately following the interventions. RESULTS: A total of 42 participants (17 men and 25 women) completed the study. There were no significant differences in frequency and severity of challenging behaviours and most of the outcome measures between the four groups at post-test. Nevertheless, there were statistical significant differences on the active and inactive state (Alertness Observation Checklist) between the three treatment and control groups. Many participants in the three treatment groups changed from an active to inactive state (i.e. reduced activity levels) throughout the interventions, especially the MT in MSE. Such inactivity might suggest the participants' brief exhaustion followed by a period of alertness during the treatment activities. But their attention span and social contact to the immediate environment could still be maintained. CONCLUSIONS: Participants of MT in multisensory environment acquired more inactive state than the other study groups. This inactive state indicates a state of 'passive alertness', which is more likely in a relaxing manner.

Pain underreporting associated with profound intellectual disability in emergency departments.

BACKGROUND: Little information is available regarding the visits made by persons with profound intellectual disability (PID) to general hospital emergency departments (ED). This study aims to know whether persons with PID who attend ED are given the same type of diagnoses as people with no such disability. METHODS: Over a period of 18 months, we gathered data from all non-scheduled visits to an ED by persons with PID to identify the reason for consultation (according to the classification used by the Spanish Society for Emergency Nursing) and the final diagnosis upon discharge. The results were compared with data obtained from a control group of people with no ID who attended an ED for any reason during the same period. RESULTS: Somatic complaints were the main reason for ED attendance among persons with PID (90% of consultations). These complaints were more often related to the central nervous system than was the case among non-ID patients (16 vs. 4.7%), whereas other kinds of non-central nervous system somatic complaint were less common among persons with PID (74 vs. 91%). A diagnosis implying physical pain was given less often to people with PID than to controls (3 vs. 20%). CONCLUSIONS: The results suggest that persons with PID are less able to conceptualise and communicate information about their symptoms, especially as regards pain, and that this influences the diagnosis they are given when attending an ED. Professionals working in this environment need to be aware of this possibility so as not to underestimate or overlook such symptoms and the illnesses related to them.