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.

Assessing the reliability and validity of a health-related quality of life instrument, CPADULT, in a Dutch sample of adults with severe disabilities who are non-ambulatory.

BACKGROUND: A measure to provide insight regarding health-related quality of life of adults with severe motor and intellectual disabilities was lacking. For this reason, the CPADULT was developed. This measure includes domains relating to an individual's physical, mental, and social functioning. The purpose of this study was to assess the psychometric characteristics of the CPADULT. METHOD: Caregivers (n = 47; 77% female, 23% male) of individuals with severe disabilities who are non-ambulatory completed the questionnaire. Internal consistency, test-retest reliability and construct validity were analysed. RESULTS: Internal consistency was adequate with Cronbach's alpha values from 0.75 to 0.95. Test-retest reliability was good, as intraclass correlation coefficient of the total score was 0.84 (domains: 0.61-0.89). Construct validity was confirmed with significant differences between subgroups of motor or intellectual abilities. CONCLUSION: The CPADULT has sufficient reliability and validity as a proxy measure of health-related quality of life for adults with severe disabilities who are non-ambulatory.

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.

Changes in the early communicative behaviors of young children with significant cognitive and motor developmental delays in a two-year span.

INTRODUCTION: This study examines longitudinal changes in communicative behavior of young children with significant cognitive and motor developmental delays (SDD) and determines their individual communicative trajectories. A second focus of this study is the relation of changes in communicative behavior with motor skills. METHODS: Data consists of codes resulting from a self-developed coding scheme used on observations of 23 children in three different settings and responses on a questionnaire. First, group trends were determined to find out whether communication-related variables tend to significantly change over the course of two years. Furthermore, these findings were contrasted with the individual trajectories of the children. Next, the association of initial communicative skills and (the acquisition of) specific motor skills with the change in their communicative functioning was studied. Wilcoxon Signed Ranks and correlational analyses were used to answer the research questions. RESULTS: Out of sixteen different variables related to communicative behavior, ten changed significantly over the course of two years. Children with more focus on prompt on the first datapoint showed a significantly larger increase of signs of functionality. Still, all children showed highly individual trajectories. Children with better motor skills on the first datapoint showed a significantly larger increase in communication rate. CONCLUSIONS: Results show that if a myriad of detailed variables are taken into account children with significant cognitive and motor developmental disabilities do change regarding their communicative functioning, but that they tend to all show unique developmental trajectories. Children with stronger skills in some aspects of communication and motor functioning, can be considered advantaged regarding their communicative development.

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.

Describing the communicative profiles of young children with a significant cognitive and motor developmental delay.

The communicative behavior of young children with significant cognitive and motor developmental delays is generally considered to be limited, idiosyncratic and non-intentional. At present, changes between and within children over time regarding their communicative behavior are hard to detect. This article describes an exploratory observational study that draws on data from the first data point of 38 children who are participating in a longitudinal project on the developmental trajectories of children with significant cognitive and motor developmental delays. The aims of this study were to (a) describe the participants' communicative behavior in detail with communication-related variables that reflect differences across individuals, (b) create summarizing variables and (c) explore whether subgroups of children can be detected. A self-developed coding scheme and descriptive statistics combined with correlational analyses were used, followed by a principal component analysis and visual inspection of the outcome of this analysis. The within-group differences related to communicative behavior was characterized using 16 variables. Based on these variables, three overarching components were formulated: communication proficiency, Expressions of Discomfort and Rejection and Differentiation According to Focus. All participating children were found to be unique in terms of their component scores and the relationship among their component scores.

Snoezelen in people with intellectual disability or dementia: A systematic review.

Background: Snoezelen focuses on multisensory stimulation in an adapted environment and was originally developed for people with severe and profound intellectual (and multiple) disabilities. Snoezelen has been used for many years with various target groups and for different purposes. Variation in its application has resulted in a lack of understanding of snoezelen's application characteristics and of how they may relate to effects. Objective: The aim of this review was to provide an overview of the application and effects of snoezelen in people with intellectual disability or dementia in order to analyse the relationship between application characteristics and effects. Design: A systematic review. Methods: Five databases were searched for snoezelen studies that took place in a specially adapted environment. The methodological quality of the included studies was assessed using the Mixed Methods Appraisal Tool. The application characteristics (that is, the stimuli used, environment, and support given) and the effects were extracted. Reported effects were categorized into different human functioning dimensions using the model of intellectual disabilities of the American Association on Intellectual and Developmental Disabilities. Results: In total, 62 studies involving people with intellectual disability (n = 30) or dementia (n = 32) were included. An overview of snoezelen used in other target groups (n = 24) is provided as supplementary material. Details on the application of snoezelen were often lacking. A total of 10 application characteristics (for example, frequency, role of the support person) were extracted. All studies reported the presence of a support person (n = 62; 100%). Effects were found in all five human functioning dimensions. The most-reported effects (61.3% overall) related to mental health, such as a reduction in challenging behaviour and improved mood. In a minority of studies (n = 10, 16.1%), effects on the support person were also reported. Due to limited details about the application of snoezelen and the large variation in measured effects, analysing the relationship between these was impossible. Conclusions: The majority of studies lacked details on application characteristics during snoezelen. Reported effects varied, although most related to mental health. Future research should analyse in detail the relationship between application and effects.

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.

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.

Lessons learned: A critical reflection on child- and contextual variables related to the development of children with a significant cognitive and motor developmental delay.

BACKGROUND: The scientific study of young children with a significant cognitive and motor developmental delay is challenging due to the children's complex disabilities and high demands on family life and professional support. We aim to critically reflect on the measurement and analysis of child- and contextual variables within an ongoing research project on these children's developmental trajectories. METHOD: The OJKO-project tracked the development of a sample of children (n = 45) between the age of 6 months and 4 years with a significant cognitive and motor developmental delay, in Belgium and the Netherlands. RESULTS: The complexity of the children's disabilities and daily life context, and subsequent challenges in measurement and analysis of variables, were confirmed and reflected upon. CONCLUSIONS: Due to the uniqueness and complexity of this target group, research should be characterized by creativity, perseverance and substantial modesty in the immediate generalization of results.

In search of a novel way to analyze early communicative behavior.

The aim of this study was to develop a coding scheme that enables researchers and practitioners to conduct a detailed analysis of the communicative behavior of young children with significant cognitive and motor developmental delays. Currently, there is a paucity of methods to do conduct such an analysis. For the study, video observations of three different scenarios from 38 children with significant cognitive and motor developmental delays aged between 12 and 54 months, were used. Findings from the video observations served as the primary means for development of the coding scheme, which comprises three main categories - context, partner behavior, and individual behavior - and several subcategories. The coding scheme was used to document the early expressive communicative behavior of persons with significant cognitive and motor developmental delays in a detailed manner. This fine-grained information is necessary to differentiate children based on their communicative abilities, to monitor their communicative development longitudinally, and to inform person-centered communicative interventions.

Inventory of assessment practices in people with profound intellectual and multiple disabilities in three European countries.

BACKGROUND: Knowledge about the quality of assessment methods used in the support of people with profound intellectual and multiple disabilities (PIMD) is scarce. This study aimed to provide an overview of the assessment methods used in practice and to examine whether these instruments were studied for their psychometric properties for people with PIMD. METHOD: Professionals (N = 148) from three European countries completed a survey on assessment practices. We performed a literature search to find information about the psychometric properties of the instruments that were identified in the survey. RESULTS: Of the participants, 78.1% used assessments that were not developed for people with PIMD. Documentation on psychometric properties was found for 8 out of 116 instruments. CONCLUSIONS: Most of the instruments in use were not designed for people with PIMD, and information about their quality is lacking. Guidelines are needed regarding the use and development of assessment methods for people with PIMD.

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 .

Development and sensibility assessment of a health-related quality of life instrument for adults with severe disabilities who are non-ambulatory.

BACKGROUND: Insight in health-related quality of life (HRQoL) of adults with severe disabilities who are non-ambulatory is important, but a measure is lacking. The aim was to develop a HRQoL measure for this group. METHOD: The developmental process consisted of the adaptation process of a proxy HRQoL measure for children with severe disabilities who are non-ambulatory and the assessment of the sensibility of the developed instrument. A three-step process was used: focus groups, e-survey and interviews. RESULTS: In total, 72% of the items remained unchanged. Three new items and one element to an existing item were added. In ten items, the formulation of the items was adapted to the target group. Concerning the sensibility, respondents suggested minor changes to the instruction and the output scales. CONCLUSIONS: This study has yielded a proxy HRQoL measure for adults with severe disabilities who are non-ambulatory, the CPADULT, with good sensibility.

Looking back, looking forward: Methodological challenges and future directions in research on persons with profound intellectual and multiple disabilities.

BACKGROUND: Within the context of the Special Interest Research Group (SIRG) on Persons with Profound Intellectual and Multiple Disabilities (PIMD), researchers often discuss the methodological problems and challenges they are confronted with. The aim of the current article was to give an overview of these challenges. METHODS: The challenges are centred on six topics. These reflect the main components of a study's design: (a) participant demarcation, (b) participant recruitment, (c) data collection and instruments, (d) data analysis, (e) ethics/including the "voice" of persons with PIMD and (f) theoretical models. RESULTS: Next, to describing the specific challenges, possible solutions and pathways to address them are discussed. These are illustrated by recent studies by the authors and other researchers in the field. CONCLUSIONS: The current contribution wants to stimulate further discussion and exchange of ideas, and the development of creative research techniques.

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.