Towards criteria and symptoms of constipation in people with severe or profound intellectual and multiple disabilities: A Delphi study.

Background: Chronic constipation is common in people with intellectual disabilities, and seems to be highly prevalent in people with severe or profound intellectual and multiple disabilities (SPIMD). However, there is no current widely accepted definition for the constipation experienced by these individuals. Aim: This Delphi study aims to compile a list of operationalized criteria and symptoms of constipation in people with SPIMD based on practical experiences of and consensus between experts supporting them. Methods: A two-round Delphi study with an intermediate evaluation and analyses was conducted. Parents and relatives of persons with SPIMD and support professionals were included. The panel answered statements and open questions about symptoms and criteria of constipation. They were also requested to provide their opinion about classifying criteria and symptoms into domains. Answers to statements were analysed separately after both rounds with regard to consensus rate and displayed qualitatively; answers to open questions were analysed deductively. Results: In the first Delphi round (n = 47), consensus was achieved on criteria within the domains 'Defecation' and 'Physical features', that were assigned to broader categories. Symptoms retrieved within the domain 'Behavioural/Emotional' were brought back to the panel as statements. After the second Delphi round (n = 38), consensus was reached on questions about domains, and for eight criteria (domain 'Defecation' n = 5; domain 'Physical features n = 3). Within the domain 'Behavioural/Emotional', consensus was achieved for five symptoms. Criteria and symptoms with consensus >70% were considered 'generic' and <70% as 'personal'. Symptoms mentioned in the text boxes were used to operationalize categories. Discussion and conclusion: It was possible to compile a list of generic criteria related to the domains 'Defecation' (n = 5) and 'Physical features' (n = 3) supplemented with generic symptoms related to the domain 'Behavioural/Emotional' (n = 5). We propose using both generic as well as personal criteria and symptoms resulting in a personal profile for an individual with SPIMD. Based on the current results, we recommend follow-up research to develop a screening tool to be used by relatives and professional caregivers, and a definition of constipation. This may support reciprocal collaboration and lead to timely identification of constipation in people with SPIMD.

Implementation of a program to support direct support professionals to promote a healthy lifestyle for people with moderate to profound intellectual disabilities.

BACKGROUND: There is a lack of theory-based interventions for direct support professionals (DSPs) to support a healthy lifestyle for people with moderate to profound intellectual disabilities (ID) despite their major role in this. This study aims to evaluate the preparation, implementation, and preliminary outcomes of a theory-based training and education program for DSPs to learn how to support these individuals. METHODS: The program consisting of e-learning, three in-person sessions, and three assignments was implemented. The implementation process was evaluated with a mixed method design with the following components: preparation phase, implementation phase, and the outcomes. These components were measured with project notes, questionnaires, interviews, reflections, assignments, food diaries, Actigraph/Actiwatch, and an inventory of daily activities. RESULTS: Regarding the preparation phase, enough potential participants met the inclusion criteria and the time to recruit the participants was 9 months. The program was implemented in four (residential) facilities and involved individuals with moderate to profound ID (n = 24) and DSPs (n = 32). The e-learning was completed by 81% of the DSPs, 72-88% attended the in-person sessions, and 34-47% completed the assignments. Overall, the fidelity of the program was good. DSPs would recommend the program, although they were either negative or positive about the time investment. Mutual agreement on expectations were important for the acceptability and suitability of the program. For the outcomes, the goals of the program were achieved, and the attitudes of DSPs towards a healthy lifestyle were improved after 3 months of the program (nutrition: p = < 0.01; physical activity: p = 0.04). A statistically significant improvement was found for food intake of people with ID (p = 0.047); for physical activity, no statistically significant differences were determined. CONCLUSIONS: The theory-based program consisting of a training and education section for DSPs to support a healthy lifestyle for people with moderate to profound ID was feasible to implement and, despite some barriers regarding time capacity and mutual expectations, it delivered positive changes in both persons with moderate to profound ID and DSPs. Thus, the program is a promising intervention to support DSPs.

Variables related to the quality of life of families that have a child with severe to profound intellectual disabilities: A systematic review.

BACKGROUND: Family quality of life (FQoL) of families that have a child with severe to profound intellectual disabilities (SPID) is an important and emerging concept, however, related variables are inconclusive. AIM: To gain a better understanding of variables related to the FQoL of families that have a child with SPID, variables related to the FQoL of families that have a child with intellectual disabilities (ID) were systematically reviewed. METHODS AND PROCEDURES: A search strategy was performed in five databases. Critical appraisal tools were employed to evaluate the quality of both quantitative and qualitative studies. Data extraction and synthesis occurred to establish general study characteristics, variables, and theoretical concepts. Variables were categorised into four key concepts of the FQoL: systemic concepts, performance concepts, family-unit concepts and individual-member concepts. OUTCOMES AND RESULTS: A total of 40 studies were retrieved with 98 variables. Quality scores ranged from 7 to 13 (quantitative) and 5 to 13 (qualitative) out of 13 and 14 points, respectively. Five out of the 40 studies (13%) focused on individuals with SPID. Variables related positively or negatively to the FQoL, and were categorised within systemic concepts (n = 3); performance concepts (n = 11); family-unit concepts (n = 26); and individual-member concepts (n = 58). CONCLUSIONS AND IMPLICATIONS: Several variables were found to be (inter)related to the FQoL of families that have a child with ID. A contrasting picture emerged regarding the impact of a disability in relation to transitional phases. However, studies which include families of children with SPID were minimal, therefore, it remained ambiguous to what extent the identified variables apply to these families.

Patterns of multimorbidity in people with severe or profound intellectual and motor disabilities.

BACKGROUND: People with severe or profound intellectual and motor disabilities (SPIMD) experience multimorbidity and have complex health needs. Multimorbidity increases mortality, decreases functioning, and negatively influences quality of life. Information regarding patterns of multimorbidity in people with SPIMD may lead to proactive prevention, specifically detection and treatment of physical health problems at an early stage and prevention of secondary complications. AIM: The aim of this study was to explore patterns of multimorbidity in individuals with SPIMD. METHODS AND PROCEDURES: Data from medical records and care plans on reported physical health problems of 99 adults with SPIMD were analysed. To explore the co-occurrence of physical health problems, cross tabulations and a 5-set Venn Diagram were used. OUTCOMES AND RESULTS: The most common combination of two physical health problems comprise the most prevalent physical health problems, which included visual impairment, constipation, epilepsy, spasticity, and scoliosis. These five issues occurred as a multimorbidity combination in 37% of the participants. In 56% of the participants a multimorbidity combination of four health problems emerged, namely constipation, visual impairment, epilepsy, and spasticity. CONCLUSIONS AND IMPLICATIONS: People experiencing SPIMD have interrelated health problems. As a consequence, a broad variety of potential interactions between physical health problems and their treatments may occur. Identifying multimorbidity patterns can provide guidance for accurate monitoring of persistent health problems and, early detection of secondary complications. However, the results require confirmation with larger samples in further studies.

Gross motor function in children with spastic Cerebral Palsy and Cerebral Visual Impairment: A comparison between outcomes of the original and the Cerebral Visual Impairment adapted Gross Motor Function Measure-88 (GMFM-88-CVI).

PURPOSE: To investigate whether the adapted version of the Gross Motor Function Measure-88 (GMFM-88) for children with Cerebral Palsy (CP) and Cerebral Visual Impairment (CVI) results in higher scores. This is most likely to be a reflection of their gross motor function, however it may be the result of a better comprehension of the instruction of the adapted version. METHOD: The scores of the original and adapted GMFM-88 were compared in the same group of children (n=21 boys and n=16 girls), mean (SD) age 113 (30) months with CP and CVI, within a time span of two weeks. A paediatric physical therapist familiar with the child assessed both tests in random order. The GMFCS level, mental development and age at testing were also collected. The Wilcoxon signed-rank test was used to compare two different measurements (the original and adapted GMFM-88) on a single sample, (the same child with CP and CVI; p<0.05). RESULTS: The comparison between scores on the original and adapted GMFM-88 in all children with CP and CVI showed a positive difference in percentage score on at least one of the five dimensions and positive percentage scores for the two versions differed on all five dimensions for fourteen children. For six children a difference was seen in four dimensions and in 10 children difference was present in three dimensions (GMFM dimension A, B& C or C, D & E) (p<0.001). CONCLUSION: The adapted GMFM-88 provides a better estimate of gross motor function per se in children with CP and CVI that is not adversely impacted bytheir visual problems. On the basis of these findings, we recommend using the adapted GMFM-88 to measure gross motor functioning in children with CP and CVI.

Physical health issues in adults with severe or profound intellectual and motor disabilities: a systematic review of cross-sectional studies.

BACKGROUND: People with severe or profound intellectual and motor disabilities (SPIMD) encounter several risk factors associated with higher mortality rates. They are also likely to experience a cluster of health problems related to the severe brain damage/dysfunction. In order to earlier detect physical health problems in people with SPIMD, first of all, knowledge regarding the prevalence of physical health problems is necessary. The aim of this systematic review was to methodically review cross-sectional studies on the prevalence of various types of physical health problems in adults with SPIMD. METHOD: MedLine/PubMed, CINAHL, Embase, PsycINFO and Web of Science were searched for studies published between 2004 and 2015. The quality of the incorporated studies was assessed utilising an adjusted 'risk of bias tool' for cross-sectional studies. To estimate the prevalence of the health problems, the proportion and corresponding confidence interval were calculated. A random effect meta-analysis was performed when at least three studies on a specific health problem were available. RESULTS: In total, 20 studies were included and analysed. In the meta-analysis, a homogeneous prevalence rate of 70% (CI 65-75%) was determined for epilepsy. Heterogeneous results were ascertained in the meta-analysis for pulmonary/respiratory problems, hearing problems, dysphagia, reflux disease and visual problems. For the health problems identified in two studies or in a single study, the degree of evidence was low. As expected, higher prevalence rates were found in the current review compared with people with ID for visual problems, epilepsy and spasticity. CONCLUSION: This review provides an overview of the current state of the art research on the prevalence of health problems in adults with SPIMD. There is a substantial need for comprehensive epidemiological data in order to find clusters of health problems specific for people with SPIMD. This would provide insight into the excess morbidity associated with SPIMD.

Development and face validity of a cerebral visual impairment motor questionnaire for children with cerebral palsy.

AIM: The objectives of this study were (i) to develop two cerebral visual impairment motor questionnaires (CVI-MQ's) for children with cerebral palsy (CP): one for children with Gross Motor Function Classification System (GMFCS) levels I, II and III and one for children with GMFCS levels IV and V; (ii) to describe their face validity and usability; and (iii) to determine their sensitivity and specificity. BACKGROUNDS: The initial versions of the two CVI-MQ's were developed based on literature. Subsequently, the Delphi method was used in two groups of experts, one familiar with CVI and one not familiar with CVI, in order to gain consensus about face validity and usability. The sensitivity and specificity of the CVI-MQ's were subsequently assessed in 82 children with CP with (n = 39) and without CVI (n = 43). With the receiver operating curve the cut-off scores were determined to detect possible presence or absence of CVI in children with CP. RESULTS: Both questionnaires showed very good face validity (percentage agreement above 96%) and good usability (percentage agreement 95%) for practical use. The CVI-MQ version for GMFCS levels I, II and III had a sensitivity of 1.00 and specificity of 0.96, with a cut-off score of 12 points or higher, and the version for GMFCS levels IV and V had a sensitivity of 0.97 and a specificity of 0.98, with a cut-off score of eight points or higher. CONCLUSION: The CVI-MQ is able to identify at-risk children with CP for the probability of having CVI.

Prevalence of reported physical health problems in people with severe or profound intellectual and motor disabilities: a cross-sectional study of medical records and care plans.

BACKGROUND: People with severe or profound intellectual and motor disabilities (SPIMD) experience numerous serious physical health problems and comorbidities. Knowledge regarding the prevalence of these problems is needed in order to detect and treat them at an early stage. Data concerning these problems in individuals with SPIMD are limited. Therefore, the aim of this study was to determine the prevalence of reported physical health problems in adults with SPIMD through a review of medical records and care plans. METHOD: We conducted a cross-sectional study employing data obtained from medical and support records. A sample of adults with SPIMD was recruited in eight residential care settings. Physical health problems that had occurred during the previous 12 months or were chronic were recorded. RESULTS: The records of 99 participants were included. A wide range of physical health problems were found with a mean of 12 problems per person. Very high prevalence rates (>50%) were found for constipation, visual impairment, epilepsy, spasticity, deformations, incontinence and reflux. CONCLUSIONS: The results suggest that people with SPIMD simultaneously experience numerous, serious physical health problems. The reliance on reported problems may cause an underestimation of the prevalence of health problems with less visible signs and symptoms such as osteoporosis and thyroid dysfunction.

Feasibility of bioelectrical impedance analysis in persons with severe intellectual and visual disabilities.

BACKGROUND: Body composition measurements provide important information about physical fitness and nutritional status. People with severe intellectual and visual disabilities (SIVD) have an increased risk for altered body composition. Bioelectrical impedance analysis (BIA) has been evidenced as a reliable and non-invasive method to asses body composition in healthy persons and various patient populations; however, currently, there is no feasible method available to determine body composition in people with SIVD. In this study, therefore, we aimed to assess the feasibility of BIA measurements in persons with SIVD. METHODS: In 33 participants with SIVD and Gross Motor Functioning Classification System (GMFCS) Scale I, II, III, or IV, two BIA measurements were sequentially performed employing Resistance and Reactance in Ohm and fat-free mass (FFM) in kg as outcome variables, utilizing the Bodystat(®) QuadScan 4000. Feasibility was considered sufficient if ≥ 80% of the first measurement was performed successfully. Agreement between two repeated measurements was determined by using the paired t-test and Intraclass Correlation Coefficient (ICC; two way random, absolute agreement). Bland-Altman analyses were utilized to determine limits of agreement (LOAs) and systematic error. Agreement was considered acceptable if LOAs were <10% of the mean of the first measurement. RESULTS: The first BIA measurements were completed successfully in 88% of the participants. The paired t-test demonstrated no significant differences in Resistance, Reactance, and FFM between BIA Measurements 1 and 2 (P=0.140, 0.091, and 0.866). ICC was 0.965 (95% CI: 0.922-0.984) for Resistance; 0.858 (95% CI: 0.705-0.934) for Reactance; and 0.992 (95% CI: 0.982-0.996) for FFM. LOAs expressed as a percentage of the mean of Measurement 1 were 6.1% for Resistance, 17.6% for Reactance, and 3.8% for FFM. CONCLUSIONS: The results of this study suggest that BIA measurements seem to be feasible in persons with SIVD. Although these results require confirmation in a more extensive sample of persons with SIVD, the findings of this study are an important first step in the assessment of applicability of BIA measurements for non-invasive monitoring of physical fitness and nutritional status of persons with SIVD.

Reliability of the modified Paediatric Evaluation of Disability Inventory, Dutch version (PEDI-NL) for children with cerebral palsy and cerebral visual impairment.

PURPOSE: The aims of this study were to adapt the Paediatric Evaluation of Disability Inventory, Dutch version (PEDI-NL) for children with cerebral visual impairment (CVI) and cerebral palsy (CP) and determine test-retest and inter-respondent reliability. METHOD: The Delphi method was used to gain consensus among twenty-one health experts familiar with CVI. Test-retest and inter-respondent reliability were assessed for parents and caregivers of 75 children (aged 50-144 months) with CP and CVI. The percentage identical scores of item scores were computed, as well as the interclass coefficients (ICC) and Cronbach's alphas of scale scores over the domains self-care, mobility, and social function. RESULTS: All experts agreed on the adaptation of the PEDI-NL for children with CVI. On item score, for the Functional Skills scale, mean percentage identical scores variations for test-retest reliability were 73-79 with Caregiver Assistance scale 73-81, and for inter-respondent reliability 21-76 with Caregiver Assistance scale 40-43. For all scales over all domains ICCs exceeded 0.87. For the domains self-care, mobility, and social function, the Functional Skills scale and the Caregiver Assistance scale have Cronbach's alpha above 0.88. CONCLUSION: The adapted PEDI-NL for children with CP and CVI is reliable and comparable to the original PEDI-NL.

Feasibility, test-retest reliability, and interrater reliability of the Modified Ashworth Scale and Modified Tardieu Scale in persons with profound intellectual and multiple disabilities.

Caregivers of persons with profound intellectual and multiple disabilities (PIMD) often describe the quality of the daily movements of these persons in terms of flexibility or stiffness. Objective outcome measures for flexibility and stiffness are muscle tone or level of spasticity. Two instruments used to grade muscle tone and spasticity are the Modified Ashworth Scale (MAS) and the Modified Tardieu Scale (MTS). To date, however, no research has been performed to determine the psychometric properties of the MAS and MTS in persons with PIMD. Therefore, the purpose of this study was to determine the feasibility, test-retest reliability, and interrater reliability of the MAS and MTS in persons with PIMD. We assessed 35 participants on the MAS and MTS twice, first for the test and second a week later for the retest. Two observers performed the measurements. Feasibility was assessed based on the percentage of successful measurements. Test-retest and interrater reliability were determined by using the Wilcoxon signed rank test, intraclass correlation coefficients (ICC), Spearman's correlation, and either limits of agreement (LOA) or quadratically weighted kappa. The feasibility of the measurements was good, because an acceptable percentage of successful measurements were performed. MAS measurements had substantial to almost perfect quadratically weighted kappa (>0.8) and an acceptable ICC (>0.8) for both inter- and intrarater reliability. However, MTS measurements had insufficient ICCs, Spearman's correlations, and LOAs for both inter- and interrater reliability. Our data indicated that the feasibility of the MAS and MTS for measuring muscle tone in persons with PIMD was good. The MAS had sufficient test-retest and interrater reliability; however, the MTS had an insufficient test-retest and interrater reliability in persons with PIMD. Thus, the MAS may be a good method for evaluating the quality of daily movements in persons with PIMD. Providing test administrators with training and clear instructions will improve test reliability.

Feasibility and reliability of the modified Berg Balance Scale in persons with severe intellectual and visual disabilities.

BACKGROUND: The purpose of this study was to determine the feasibility and reliability of the modified Berg Balance Scale (mBBS) in persons with severe intellectual and visual disabilities (severe multiple disabilities, SMD) assigned Gross Motor Function Classification System (GMFCS) grades I and II. METHOD: Thirty-nine participants with SMD and GMFCS grades I and II performed the mBBS twice with 1-week interval. Feasibility was assessed by the percentage of successful measurements per task and of the total score. First, test-retest reliability was determined by intraclass correlation coefficients (ICC) for each task and for the total score of all tasks combined. Second, level of agreement between test-retest scores was assessed with the proportion of equal scores for each task. Finally, internal consistency of the distinct tasks was assessed by Cronbach's alpha. RESULTS: The results indicated that 92% of the measurements by the mBBS for all selected tasks were successful, indicating that the mBBS is a feasible instrument for the tested target group. ICC for the test-retest of the total score was 0.95. The proportion of equal scores for test-retest of the tasks was 0.80 or more, except for tasks 9 and 10. Cronbach's alpha of distinct tasks was 0.84. Test-retest reliability of tasks 9 and 10 was not acceptable. CONCLUSIONS: Feasibility of all tasks and test-retest reliability of 10 out of 12 mBBS tasks is acceptable. The mBBS is a both feasible and reliable test for evaluating the functional balance of persons with SMD and GMFCS grades I and II.

Measuring waist circumference in disabled adults.

To date, it is unknown whether waist circumference can be measured validly and reliably when a subject is in a supine position. This issue is relevant when international standards for healthy participants are applied to persons with severe intellectual, sensory, and motor disabilities. Thus, the aims of our study were (1) to determine the validity of waist circumference measurements obtained in a supine position, (2) to formulate an equation that predicts standing waist circumference from measurements obtained in a supine position, and (3) to determine the reliability of measuring waist circumference in persons with severe intellectual, sensory, and motor disabilities. First, we performed a validity study in 160 healthy participants, in which we compared waist circumference obtained in standing and supine positions. We also conducted a test-retest study in 43 participants with severe intellectual, sensory, and motor disabilities, in which we measured the waist circumference with participants in the supine position. Validity was assessed with paired t-test and Wilcoxon signed rank test. A prediction equation was estimated with multiple regression analysis. Reliability was assessed by Wilcoxon signed rank test, limits of agreement (LOA), and intraclass correlation coefficients (ICC). Paired t-test and Wilcoxon signed rank test revealed significant differences between standing and supine waist circumference measurements. We formulated an equation to predict waist circumference (R(2)=0.964, p<0.001). There were no significant differences between test and retest waist circumference values in disabled participants (p=0.208; Wilcoxon signed rank test). The LOA was 6.36 cm, indicating a considerable natural variation at the individual level. ICC was .98 (p<0.001). We found that the validity of supine waist circumference is biased towards higher values (1.5 cm) of standing waist circumference. However, standing waist circumference can be predicted from supine measurements using a simple prediction equation. This equation allows the comparison of supine measurements of disabled persons with the international standards. Supine waist circumference can be reliably measured in participants with severe intellectual, sensory, and motor disabilities.

Feasibility and reliability of body composition measurements in adults with severe intellectual and sensory disabilities.

BACKGROUND: Anthropometric measurements are widely used to reliably quantify body composition and to estimate risks of overweight in healthy subjects and in patients. However, information about the reliability of anthropometric measurements in subjects with severe intellectual and sensory disabilities is lacking. OBJECTIVE: The purpose of this study was to determine the feasibility and the test-retest reliability of body composition measures in subjects with severe intellectual and sensory disabilities. METHOD: The study population consisted of 45 subjects with severe intellectual and sensory disabilities. Body mass index, waist circumference, skin folds and tibia length were measured. Reliability was assessed by Wilcoxon signed rank test, limits of agreement (LOA) and intraclass correlation coefficients. The outcomes were compared with values provided by the World Health Organization. RESULTS: There were no significant differences between test and retest (P < 0.05). For the skinfold measurements, however, the LOA was insufficient. Intraclass correlation coefficients for all variables, except skinfold measurements, were 0.90 or above. CONCLUSION: Test-retest reliability and feasibility for all measurements are acceptable in subjects with severe intellectual and sensory disabilities. Skinfold measurements, however, could not be reliably performed in these subjects. Measuring tibia length and using the determined formula to calculate body height from tibia length is a reliable alternative for measuring body height. Although measuring the body height of subjects with severe disabilities was feasible, measuring tibia length was more feasible.

Vocalization and physiological response of pigs during castration with or without a local anesthetic.

The objectives of this project were to see whether heart rate, respiration rate, blood pressure, and vocalization could be used to evaluate stress of castration in pigs. Six groups of pigs 1, 2, 4, 8, 16, and 24 d of age were used in the study, a total of 172 pigs. Half of each group of pigs received lidocaine before castration, injected subcutaneously over the testicle and infiltrated around the cord; the other half were left as controls. Pigs castrated without lidocaine had a higher heart rate (P < .02) and higher frequency of highest energy (HEF) measurements of vocalization (P < .05). Incising the scrotum and severing the spermatic cord elicited the greater heart rate response (P < .05) to castration without anesthetic, whereas HEF was lower during cutting of the cord. Both the heart rate and HEF data suggest that castration without anesthetic is of greater stress for pigs 8 d of age or older. Respiration rate was not a viable measure of stress associated with castration.