Physical health conditions in young children with profound intellectual and multiple disabilities: The prevalence and associations between these conditions.

BACKGROUND: It is thought that physical health conditions start at a young age in people with profound intellectual and multiple disabilities (PIMD). Knowledge regarding the prevalence, associations and development of these physical health conditions could be used for purposes of prevention as well as appropriate care and support but is currently lacking. OBJECTIVE: The aim of this study is to gain insight into the prevalence of physical health conditions and associations between these conditions in young children with PIMD. METHODS: The study used cross-sectional data related to the physical health conditions of children with PIMD (n = 51, aged between 12 and 61 months). Data were collected in Belgium and in the Netherlands through a checklist filled in by primary caregiver(s). Physical health conditions were classified into categories by the 10th revision of the International Classification of Diseases and Related Health Problems (ICD-10) system. The number of physical health conditions and associations between them were analysed. The analysis focused on prevalence rates and associations represented by odds ratios (p < 0.05). A graphical model was estimated to represent dependencies and conditional dependencies between physical health conditions. RESULTS: We found a mean of 3.8 (range 1-8, SD 1.9) physical health conditions per child. Most of the physical health conditions were found in the ICD-10 chapter 'Nervous System', with hypotonia as the most frequent at 70.6%. Five significant large associations were found between spasticity-contractures (OR 9.54); circulatory system-contractures (OR 7.50); scoliosis-contractures (OR 10.25); hearing impairments-skin problems (OR 58.20) and obstipation-hypotonia (OR 19.98). CONCLUSION: This study shows that at a young age, multiple physical health conditions are present in children with PIMD. In addition, we found five associations between physical health conditions.

Family Resilience Affecting Well-Being of Families With a Child With Profound Intellectual and Multiple Disabilities.

Persons with profound intellectual and multiple disabilities (PIMD) have pervasive support needs, which are often managed by their families. By being resilient and positively adapting to this challenge, families may maintain a positive family quality of life (FQOL). We therefore aimed to understand how families with a child with PIMD experience their family resilience, and if and how it affects their FQOL. Participants were 64 parents of a person with PIMD from 44 families. Total family resilience, as well as most subscales received positive scores, and also had a significant positive effect on FQOL. These results provide more insight into the family dynamics of families with a child with PIMD, which should inform policies, and provided services for these families.

From assistive to inclusive? A systematic review of the uses and effects of technology to support people with pervasive support needs.

BACKGROUND: Although particular technologies can enhance the quality of life (QoL) of people with profound intellectual and multiple disabilities (we use the term pervasive support needs), their objectives and outcomes are understudied. A systematic literature review was therefore conducted to explore this topic. METHOD: A search of four databases yielded 64 studies. Data were extracted on their general characteristics, methods and sample characteristics as well as the technology types, QoL domains and application within ecological systems. A narrative synthesis was subsequently developed. RESULTS: Most of the studies applied assistive technology (AT) and focused on personal development and self-determination on an individual level. CONCLUSIONS: Technology can enhance the QoL of people with pervasive support needs. There are indications that although MT and UD-based technology are used in practice, few studies have examined these technologies. Therefore, there is a knowledge gap regarding the kinds of technology that are used in practice.

Transition to adulthood of adolescents with profound intellectual and multiple disabilities: Content validation of the SGU-PIMD to support families.

BACKGROUND: For families with adolescent children, the transition to adulthood is usually challenging. This period may be extra demanding for families with a child with profound intellectual and multiple disabilities due to the child's strong and persistent support needs. To support these families during this phase and to facilitate the transition process of these adolescents, we adapted the Canadian skills for growing up (SGU) into the skills for growing up-profound intellectual and multiple disabilities (SGU-PIMD). The aim of this study is to determine its content validity. METHOD: A Delphi study with family members and healthcare professionals was conducted. RESULTS: Results showed good content validity. However, the Delphi panel suggested minor adjustments to improve relevance, comprehensibility and comprehensiveness. CONCLUSIONS: The current SGU-PIMD can be used in practice for supporting adolescents with profound intellectual and multiple disabilities. However, there are also recommendations for research into the feasibility and acceptability of the instrument.

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.

Reliable assessment of pain behaviour in adults with profound intellectual and multiple disabilities: The development of an instruction protocol.

BACKGROUND: Persons with profound intellectual and multiple disabilities (PIMD) are vulnerable when it comes to experiencing pain. Reliable assessment of pain-related behaviour in these persons is difficult. Aim To determine how pain items can be reliably scored in adults with PIMD. METHODS: We developed an instruction protocol for the assessment of pain-related behaviour in four phases. We used videos of 57 adults with PIMD during potentially painful situations. The items were assessed for inter-rater reliability (Cohen's kappa or percentage of agreement). RESULTS: The developed instruction protocol appeared to be adequate. Twelve items had satisfactory inter-rater reliability (n = 9: .30-1.00; n = 3: 85%-100%). DISCUSSION: Calibrating and adjustments to the instructions and item set appeared to be crucial to reliably score 12 items in adults with PIMD. Further research should focus on creating an assessment instrument based on these reliably scored items.

Dementia in People with Severe/Profound Intellectual (and Multiple) Disabilities: Practice-Based Observations of Symptoms.

Introduction: Observable dementia symptoms are hardly studied in people with severe/profound intellectual (and multiple) disabilities (SPI(M)D). Insight in symptomatology is needed for timely signaling/diagnosis. This study aimed to identify practice-based observations of dementia symptoms in this population. Methods: Care professionals and family members were invited to complete a survey about symptoms. Quantitatively analyzed survey data were further deepened through semi-structured interviews with care professionals having vast experience in signaling/diagnosing dementia in this population. Symptoms were categorized using a symptom matrix. Results: Survey respondents and interviewees frequently observed a decline in activities of daily living (ADL) functioning and behavioral and psychological changes, like increased irritability, anxiety, apathy and decreased eating/drinking behavior. Cognitive symptoms were particularly recognized in persons with verbal communication and/or walking skills. To lesser extent motor changes and medical comorbidities were reported. Conclusion: Increased insight in dementia symptoms contributes to developing a dedicated screening instrument for dementia in people with SPI(M)D.

Definitions, signs, and symptoms of constipation in people with severe or profound intellectual disabilities: A systematic review.

Background: It is difficult to diagnose constipation for people with severe or profound intellectual disabilities. Definitions for this are ambiguous, and the symptoms and signs are often unnoticed. The aim of this study is to identify clear definitions of constipation for people with different levels of intellectual disabilities and to identify signs and symptoms. Method: Guided by the PRISMA statement, a systematic review of the literature was conducted within electronic databases MEDLINE, Embase, CINAHL, Cochrane, and PsycINFO. Definitions, signs, and symptoms were extracted and the quality of definitions was assessed. Results: In total, 24 studies were included. Quality of definitions ranged from poor to good quality. Standard and referenced definitions were used in ten studies, a self-composed definition was employed in eleven studies; and three studies did not refer to a source of the definition. The self-composed definitions had not been evaluated after being used for the target group, and no scientific substantiation was available. A broad range of signs and symptoms were described. Conclusions: No substantiated definition has been ascertained for constipation for people with severe or profound intellectual disabilities. Further research will be necessary to identify which signs and symptoms are important for defining constipation in this target group.

Behaviour change techniques used in lifestyle support of adults with mild intellectual disabilities.

BACKGROUND: Professional caregivers are important in the daily support of lifestyle change for adults with mild intellectual disabilities; however, little is known about which behaviour change techniques (BCTs) are actually used. This study aims to gain insight in their use for lifestyle behaviour change using video observations. METHODS: Professional caregivers (N = 14) were observed in daily work supporting adults with mild intellectual disabilities. Videos were analysed using the Coventry Aberdeen London Refined (CALO-RE-NL) taxonomy and BCTs utilised were coded. RESULTS: Twenty one out of 40 BCTs were used by professional caregivers. The BCTs 'Information about others' approval', 'Identification as role model', 'Rewards on successful behaviour', 'Review behavioural goals' and 'Instructions on how to perform the behaviour' were most employed. CONCLUSION: Professional caregivers used BCTs to support healthier lifestyle behaviour of adults with mild intellectual disabilities. However, most promising of them as defined previous by professionals were rarely used by professional caregivers.

Development of a Dutch Training/Education Program for a Healthy Lifestyle of People With Intellectual Disability.

Individuals with intellectual disability (ID) need support from direct support professionals (DSPs) to engage in a healthy lifestyle. However, literature shows DSPs feel insufficiently equipped to support a healthy lifestyle. Therefore, the aim of this study is to develop a theory-based program for DSPs to support physical activity and healthy nutrition for people with moderate to profound levels of ID, and to design its evaluation. The Intervention Mapping Protocol (IM) was followed to develop a theory-based program for DSPs. The program evaluation consists of process and feasibility evaluations. This study provided a theory-based program consisting of a training and education section with online and face-to-face components to support DSPs in promoting health for people with ID.

The neglected puzzle of dementia in people with severe/profound intellectual disabilities: A systematic literature review of observable symptoms.

BACKGROUND: Dementia is increasingly prevalent in people with severe/profound intellectual disabilities. However, early detection and diagnosis of dementia is complex in this population. This study aimed to identify observable dementia symptoms in adults with severe/profound intellectual disabilities in available literature. METHOD: A systematic literature search was conducted in PubMed, PsycINFO and Web of Science with an exhaustive search string using a combination of search terms for severe/profound intellectual disabilities and dementia/ageing. RESULTS: Eleven studies met inclusion criteria. Cognitive decline, behavioural and psychological alterations, decline in activities of daily living as well as neurological and physical changes were found. CONCLUSIONS: Only a very limited number of studies reported symptoms ascribed to dementia in adults with severe/profound intellectual disabilities. Given the complexity of signalling and diagnosing dementia, dedicated studies are required to unravel the natural history of dementia in this population.

Dementia in people with severe or profound intellectual (and multiple) disabilities: Focus group research into relevance, symptoms and training needs.

BACKGROUND: Differentiating dementia from baseline level of functioning is difficult among people with severe/profound intellectual (and multiple) disabilities. Moreover, studies on observable dementia symptoms are scarce. This study examined (a) the relevance of dementia diagnosis, (b) observable symptoms and (c) training/information needs. METHODS: Four explorative focus groups were held with care professionals and family members who have experience with people with severe/profound intellectual (and multiple) disabilities (≥40 years) and decline/dementia. RESULTS: Thematic analysis showed that participants wanted to know about a dementia diagnosis for a better understanding and to be able to make informed choices (question 1). Using a categorisation matrix, cognitive and behavioural changes were shown to be most prominent (question 2). Participants indicated that they needed enhanced training, more knowledge development and translation, and supportive organisational choices/policies (question 3). CONCLUSIONS: Timely identifying/diagnosing dementia allows for a timely response to changing needs. This requires a better understanding of symptoms.

Development and process evaluation of a motor activity program for people with profound intellectual and multiple disabilities.

BACKGROUND: The support of people with profound intellectual and multiple disabilities (PIMD) rarely focused on motor activity, which might have negative consequences for the quality of life of these people. Evidence-based motor activity programs that present individually tailored and structural motor activity for these people are, regretfully, lacking. This study developed such a program for these people and evaluated the implementation process. METHODS: The motor activity program is developed in accordance with the theoretical premises of the educational program and consists of four methodological steps in which the content is individually filled with: motor activity structurally embedded within the activities of daily living, and 3-5 motor activities aimed at a specific goal, which is evaluated. Program delivery consisted of a manual, explanation to the teams, and coaching of one contact person per participant (n = 9). Process evaluation included the delivered fidelity, dose, reach, and adaptations made during the program. In addition, mechanisms of impact and the influence of contextual factors were evaluated. Data collection included researcher logbooks, individual program content, and staff reports. RESULTS: The intended fidelity, dose, and reach were not obtained in most participants. Content has been made explicit for seven participants, but only in one participant all critical steps in implementation were performed as intended, though later in time. In three participants, previously offered motor activities were described within the weekly program, but without all activities having a clear link with the goal set. It is showed that the core elements of the program were affected with the conceived implementation plan. The time schedule, critical elements in implementation and program content were influenced by a lack of conditions such as professionals' motivation and responsibility, methodical working, interdisciplinarity and continuity in staff. CONCLUSIONS: The results suggest that the implementation might be improved in case more attention is paid to the organizational conditions and implementation structure. The findings led to substantial changes in the implementation strategy. This study underlines the importance of process evaluation prior to testing for effectiveness. TRIAL REGISTRATION: The (overarching) study was registered at the Netherlands Trial Register (number 6627) on February 10, 2017: https://www.trialregister.nl/trial/6449 .

Intervention effects on professionals' attitudes towards the participation of adults with visual and severe or profound intellectual disabilities.

BACKGROUND: We investigated the effects of the "Care for Participation+" (CFP+) intervention on direct support professionals' (DSPs') attitudes regarding the participation of adults with visual and severe or profound intellectual disabilities (VSPID). METHODS: We implemented a pilot non-randomized controlled trial with two control groups to compare DSPs' attitudes towards CFP+ using the Attitudes towards Participation Questionnaire (APQ) and DSPs' written profiles of adults with VSPID. RESULTS: CPP+ and the Participation Mind Map control group showed a positive trend for the "leisure/recreation," "social relations," and "ability to act" APQ domains compared to the usual care control group. The CFP+ group described significantly fewer disabilities at 6 months, reflecting a more positive attitude than controls. CONCLUSION: CFP+ had positive effects on DSPs' attitudes towards the participation of adults with VSPID. The small sample size, ceiling effects, measurement instruments used, and implementation difficulties may have hampered understanding the full potential of CFP+.

Construct validity of the Actiwatch-2 for assessing movement in people with profound intellectual and multiple disabilities.

BACKGROUND: Valid measures to assess either small or assisted performed movements of people with profound intellectual and multiple disabilities (PIMD) are required. We analysed the construct validity of the Actiwatch-2 to assess movement in people with PIMD. METHOD: Twenty-two persons with PIMD were video recorded while wearing an Actiwatch-2. We used 15s-partial-interval recording to record upper body movement, body position and activity situation. Multilevel analyses were used to evaluate if the Actiwatch-2, based on produced counts, could detect changes in these factors. RESULTS: The presence versus absence of upper body movement and an activity situation in which participants were involved versus not involved resulted in significantly higher counts, with a large variety in predicted counts between participants. No relationship between body position and counts was found. CONCLUSIONS: The Actiwatch-2 seems able to assess obvious upper body movement in people with PIMD, and whether there is involvement in an activity situation.

Use of behaviour change techniques by direct support professionals to support healthy lifestyle behaviour for people with moderate to profound intellectual disabilities.

BACKGROUND: Behaviour change techniques (BCTs) can be employed to support a healthy lifestyle for people with intellectual disabilities. The aim of this study is to determine whether and which BCTs are used by direct support professionals (DSPs) for supporting healthy lifestyle behaviour of people with moderate to profound intellectual disabilities. METHOD: Direct support professionals (n = 18) were observed in their daily work using audio-visual recordings. To code BCTs, the Coventry Aberdeen London Refined (CALO-RE-NL) taxonomy was employed. RESULTS: Direct support professionals used 33 BCTs out of 42. The most used BCTs were as follows: 'feedback on performance', 'instructions on how to perform the behaviour', 'doing together', 'rewards on successful behaviour', 'reward effort towards behaviour', 'DSP changes environment', 'graded tasks', 'prompt practice' and 'model/demonstrate behaviour'. CONCLUSIONS: Although a variety of BCTs is used by DSPs in their support of people with moderate to profound intellectual disabilities when facilitating healthy lifestyle behaviour, they rely on nine of them.

Improving the participation of adults with visual and severe or profound intellectual disabilities: a process evaluation of a new intervention.

BACKGROUND: While the participation of adults with visual and severe or profound intellectual disabilities (VSPID) in society and community life is important, evidence-based interventions to improve their participation are lacking. We conducted a process evaluation of the implementation of 'Care for Participation+' (CFP+), a new intervention targeting the attitudes of direct support professionals (DSPs) toward the participation of adults with VSPID, within a residential facility in the Netherlands. METHODS: CFP+ was inspired by the Boston Psychiatric Rehabilitation Approach and adapted by adopting a new definition and operationalization of the concept of participation for adults with VSPID. Following systematic training, 16 DSPs of adults with VSPID were able to apply key elements of CFP+ to explore diverse roles and activities for this population, facilitating their self-management, teaching them necessary skills for participation, and organizing support. Our process evaluation entailed an investigation of the delivered dose, reach, fidelity, and adaptation of CFP+ during and after the CFP+ intervention. We also evaluated the mechanisms of impact and context using questionnaires, assignments, documentation, interviews, and a logbook. RESULTS: The intended dose, reach, and fidelity relating to the implementation of CFP+ were not achieved. Despite this fact, an assessment of the mechanisms of impact indicated that assignments of CFP+ were well (75%) or reasonably well (17%) understood by DSPs. CFP+ was applied by DSPs to stimulate self-management (83% of DSPs), new activities (100%), enhanced involvement in existing activities (67%) and to explore new roles (50%) for adults with VSPID. A negative contextual factor mentioned by the trainer and manager was the DSPs' lack of commitment to the training program. Another negative contextual factor mentioned by DSPs was the lack of time for implementing CFP+. CONCLUSIONS: CFP+ provides new opportunities to improve the participation of adults with VSPID. Despite the non-optimal conditions for implementing CFP+ and the DSPs' general reluctance to apply the new intervention, some have actively used CFP+ within the residential facility. Future studies should focus on the outcomes of CFP+ regarding attitudinal changes among DSPs relating to the participation of adults with VSPID and their quality of life.

Progressive resistance training for persons with intellectual disabilities and visual impairment.

BACKGROUND: Knowledge concerning the feasibility and effects of progressive resistance training (PRT) for persons with intellectual disabilities and visual impairment who are categorized in Gross Motor Function Classification System (GMFCS) Level 1 is limited. The aim of our study was to evaluate feasibility and effect of PRT on participants' Quadriceps strength and personal goals. METHODS: Eight Participants followed a PRT program for 10 weeks. Feasibility was determined by percentage of attendance and compliance. The effect of PRT was analyzed with a linear mixed model (p < 0.05) and by normalized bootstrap (95% CI). RESULTS: Participants attended 87.8% of the sessions and trained according to the PRT program, indicating sufficient compliance. Quadriceps strength increased significantly by 69%, and participants' personal goals were achieved. CONCLUSION: PRT is a feasible and potentially effective method for increasing Quadriceps strength as well as achieving personal goals in persons with intellectual disabilities and visual impairment with GMFCS Level 1.

Examining determinants of lifestyle interventions targeting persons with intellectual disabilities supported by healthcare organizations: Usability of the Measurement Instrument for Determinants of Innovations.

BACKGROUND: Due to complex processes of implementation of innovations aimed at persons with intellectual disabilities in healthcare organizations, lifestyle interventions are not used as intended or not used at all. In order to provide insight into determinants influencing this implementation, this study aims to ascertain if the Measurement Instrument for Determinants of Innovations (MIDI) is useful for objectively evaluating implementation. METHOD: With semi-structured interviews, data concerning determinants of implementation of lifestyle interventions were aggregated. These data were compared to the determinants questioned in the MIDI. Adaptations to the MIDI were made in consultation with the author of the MIDI. RESULTS: All determinants of the MIDI, except for that concerning legislation and regulations, were represented in the interview data. Determinants not represented in the MIDI were the level of intellectual disabilities, suitability of materials and physical environment, multi-levelness of interventions and several persons who could be involved in the intervention, such as direct support persons (DSPs), a therapist or family, and the communication between these involved persons. CONCLUSION: The present authors suggested making adjustments to existing questions of the MIDI in order to improve usability for deployment in organizations that provide care to persons with intellectual disabilities. The adjustments need to be tested with other interventions.

The convergent validity of a Dutch Screening tool for Dysphagia (Signaleringslijst Verslikken) for people with severe or profound intellectual and multiple disabilities.

BACKGROUND: Dysphagia is potentially life-threatening and highly prevalent in people with severe/profound intellectual and multiple disabilities (SPIMD). The "Signaleringslijst Verslikken" (SV) is a frequently used Dutch screening tool to detect dysphagia. The aim was to examine the convergent validity of the SV for people with SPIMD. METHOD: Direct support staff completed the SV, with speech and language therapists scoring a validated tool, the Dysphagia Disorders Survey (DDS), for 41 persons with SPIMD, aged ≥50 years. The results were compared for agreement using the McNemar's Test. RESULTS: The proportion of agreement was 0.59 (95% CI 0.43-0.72). The SV did not detect dysphagia in 17 participants (44%) who were assessed as having dysphagia according to the DDS. The difference in proportion of detection of dysphagia between the two methods was significant (p < 0.0001). CONCLUSIONS: The results suggest that the convergent validity of the SV is insufficient: the SV is not sensitive for detecting dysphagia in people with SPIMD.