Pediatric Performance-Based Outcome Measures for Upper Extremity Function: A Scoping Review and Linking to the International Classification of Functioning, Disability, and Health.

Background. Performance-based outcome measures (PBOMs) are objective measures that assess physical capacity or performance in specific tasks or movements. Purpose. 1) to identify which PBOMs are most frequently reported to evaluate upper extremity (UE) function in pediatric rehabilitation 2) to determine the link between constructs of the ICF and meaningful concepts extracted from each identified PBOM. Methods. Pediatric UE PBOMs were searched in four databases. The selection of outcome measures included an initial title and abstract screening, followed by full-text review of the articles to be included based on identified selection criteria. Two reviewers were appointed to link the meaningful concepts identified in the outcome measures independently and a third reviewer was consulted in case of ambiguity to make a final decision. Findings. After the initial screening, 1786 full-text articles were reviewed, 1191 met the inclusion criteria, in which 77 outcome measures were identified and 32 were included in the linking process. From the included 32 outcome measures, 538 items were extracted and linked to the ICF. The most commonly cited measures included Assisting Hand Assessment, Jebsen-Taylor Hand Function Test, Melbourne Assessment of Unilateral Upper Limb. The Activity and Participation domain represented 364 codes followed by the Body Functions domain domain which represented 174 codes. Implications. A majority of the outcome measures identified were linked with the Mobility, Fine Hand Use of the ICF. Therefore, when selecting a PBOM, careful considerations need to be made regarding which concept of health is to be assessed.

Perspectives from clinicians and managers: facilitators and barriers to the uptake of rehabilitation guidance for children with arthrogryposis.

PURPOSE: To identify the perceived facilitators and barriers among clinicians and managers about the uptake of expert guidance for rehabilitation of children with arthrogryposis multiplex congenita (AMC) in practice. METHODS: Qualitative study using individual interviews, guided by the Theoretical Domains Framework (TDF), to explore beliefs and to identify facilitators and barriers to guidance uptake. Interviews were conducted with a convenience sample of 15 clinicians working with children with AMC and four pediatric clinical managers using Microsoft Teams©. Interviews were then transcribed verbatim and analyzed by four independent reviewers using deductive and inductive coding. RESULTS: The TDF domains of Environmental Context and Resources, Behavioural Regulation, Reinforcement, Beliefs about Consequences, and Social Influences were shared amongst clinicians and clinical managers across North America and Europe as being relevant and influential on the target behaviour of using rehabilitation expert guidance to manage pediatric patients. Among clinicians only, the domain Memory, Attention, and Decision-Making Processes was also found relevant. Among managers only, the domain Social/Professional Role and Identity was found relevant. CONCLUSIONS: Coupling shared relevant domains amongst clinicians and managers with individual supports and barriers helps to map out what is needed to promote the uptake of rehabilitation guidance at multiple levels.

The uptake of expert guidance is helpful for rehabilitation professionals to align best practice with expert opinion based on clinicians and lived experience for a condition as rare as arthrogryposis multiplex congenita (AMC).In order to facilitate the uptake of guidance, rehabilitation professionals and clinical managers need to collaborate during the development and uptake in practice.Rehabilitation professionals require support for the uptake of guidance, such as training, regular team encounters, and a knowledge broker.Rehabilitation professionals and clinical managers should be aware of identified facilitators and barriers to the uptake of guidance, such as time, clinical resources, and accessibility.

Current rehabilitation practice for the evaluation and treatment of children with arthrogryposis: an international survey.

PURPOSE: To describe the current practices in rehabilitation for the evaluation and treatment of children with arthrogryposis multiplex congenita (AMC). MATERIALS AND METHODS: Rehabilitation practitioners worldwide with at least 2 years of experience with AMC were invited to complete an electronic survey on the evaluations and treatments used with children with AMC within five areas: muscle and joint function, self-care, mobility, pain, participation and psychosocial wellbeing. RESULTS: Sixty five participants from nine countries completed the survey. Participants completed the sections applicable to their practice. Number of participants within each area varied between 24 and 53. Over 80% of participants used non-standardized evaluations across areas while <50% used patient-reported or standardized measures. Stretching of upper and lower limbs was reported by >80% across ages and clinical presentation severity. Strengthening reported by >70% was mainly used among children >3 years old with less severe contractures. Other interventions used across areas included orthotics (>70%), positioning (>80%), activity-based training (>80%), assistive devices for self-care (>50%) and mobility (>80%), and energy conservation (>70%). Over 70% of participants were involved in perioperative rehabilitation. CONCLUSION: Knowledge of current pediatric rehabilitation practice in AMC, together with empirical evidence, may guide clinical decision-making and identify avenues for future research.

Arthrogryposis multiplex congenita (AMC) is a group of rare conditions and there is currently little empirical evidence on rehabilitation, therefore expert opinion is important to guide best practice.Rehabilitation practitioners should consider the areas of muscle and joint function, self-care and mobility, pain, participation, and psychosocial wellbeing when evaluating and developing a treatment plan for children with AMC.Considering the heterogeneity of AMC, different assessment tools may be selected depending on the clinical presentation of each child.In addition to stretching, orthotic use, and strengthening, the use of activity based training and assistive equipment is important to promote self-care, mobility and participation.

Expert guidance for the rehabilitation of children with arthrogryposis: protocol using an integrated knowledge translation approach.

BACKGROUND: Arthrogryposis multiplex congenita (AMC) is a group of rare congenital disorders characterized by multiple joint contractures present at birth. Contractures can affect different body areas and impact activities of daily living, mobility and participation. Although early rehabilitation is crucial to promote autonomy and participation in children with AMC, empirical evidence to inform best practice is scarce and clinical expertise hard to develop due to the rarity of AMC. Preliminary research involving stakeholders in AMC (youth with AMC, parents, and clinicians) identified priorities in pediatric rehabilitation. Scoping reviews on these priorities showed a lack of high quality evidence related to rehabilitation in AMC. The objective of this project is to provide rehabilitation expert guidance on the assessment and treatment of children with AMC in the areas of muscle and joint function, pain, mobility and self-care, participation and psychosocial wellbeing. METHODS: An integrated knowledge translation approach will be used throughout the project. Current rehabilitation practices in AMC will be identified using a clinician survey. Using the Grading of Recommendations, Assessment, Development and Evaluations framework (GRADE) approach, a panel of interdisciplinary expert clinicians, patient and family representatives, and researchers will develop expert guidance on the assessment and treatment for pediatric AMC rehabilitation based on findings from the scoping reviews and survey results. Consensus on the guidance statements will be sought using a modified Delphi process with a wider panel of international AMC experts, and statements appraised using the Appraisal of Guidelines for Research and Evaluation II (AGREE II) tool. Theoretical facilitators and barriers toward implementing clinical guidance into practice will be identified among rehabilitation clinicians and managers to inform the design of dissemination and implementation strategies. DISCUSSION: This multi-phase project will provide healthcare users and providers with research-based, expert guidance for the rehabilitation of children with AMC and will contribute to family-centered practice.

Arthrogryposis multiplex congenita (AMC) is a group of rare disorders where a child is born with stiff joints. Joint stiffness can be present in different parts of the body, making it difficult for the child to move, to walk and to participate in activities. Rehabilitation interventions starting early in life are very important to maximize autonomy. AMC being rare, it is difficult for clinicians to develop experience with this population and there is little research specific to rehabilitation interventions in AMC. In a previous research project, a group of youth with AMC and their families identified five areas of priority for rehabilitation: muscle and joint function, pain, mobility and self-care, participation and psychosocial wellbeing. The purpose of this project is to provide guidance for the evaluation and treatment of children with AMC, in these priority areas. The project involves multiple steps. First, we will perform an online survey with international rehabilitation practitioners to learn about current evaluation and treatment approaches used with children with AMC. We will then bring together expert clinicians, patient and family representatives, and researchers to develop the guidance statements. Then, we will perform an online survey with a larger group of international experts to validate and finalize the guidance statements. Finally, we will interview rehabilitation clinicians and managers to find out about what could help them or limit them in integrating this guidance into their practice. This project will provide healthcare users and providers with research and expert-based guidance for the rehabilitation of children with AMC.

Psychosocial wellbeing among children and adults with arthrogryposis: a scoping review.

BACKGROUND: Arthrogryposis multiplex congenita (AMC) is a group of congenital conditions characterized by joint contractures in two or more body areas. Management of AMC starts early in life and focuses on improving mobility and function through intensive rehabilitation and surgical interventions. Psychosocial wellbeing is an important determinant of health and the psychosocial experience of individuals with AMC should be considered in the management of this condition. The aim of this scoping review was to explore what is known about the psychosocial wellbeing of children and adults with AMC, to identify the outcome measures used and to explore the factors associated with psychosocial outcomes in this population. METHODS: A comprehensive search in four databases was conducted. Articles discussing psychosocial outcomes and outcome measures used with children or adults with AMC were included. Data on the measures used, psychosocial outcomes, and factors associated with psychosocial outcomes, were extracted and analyzed descriptively and synthesized narratively. RESULTS: Seventeen articles were included in this scoping review, ten including the pediatric population, six including adults and one article including both children and adults with AMC. The most commonly used outcome measures were the PODCI in the pediatric studies, and the SF-36 in studies on adults. In the pediatric studies, psychosocial outcomes were often secondary, compared to the studies on adults. Results showed that in both children and adults, psychosocial outcomes are comparable with the levels of the general population. Qualitative studies reflected the affective needs of this population and issues with emotional wellbeing. Factors such as fatigue and pain were associated with poorer psychosocial outcomes in adults with an impact on social relationships, intimacy and family planning. CONCLUSION: Validated outcome measures, qualitative approaches and longitudinal studies are needed to better understand the psychosocial outcomes in AMC over time. Psychosocial support should be part of the multidisciplinary management of AMC throughout the lifespan.

Participation among Children with Arthrogryposis Multiplex Congenita: A Scoping Review.

AIM: To explore what is currently known regarding participation among children and youth with arthrogryposis multiplex congenita (AMC) using empirical studies, gray literature, and YouTube videos. The secondary objectives included identifying activity types, outcome measures used, interventions provided, and barriers and facilitators to participation. METHOD: Empirical studies and gray literature were searched through electronic databases and videos were searched on YouTube. Articles and videos pertaining to participation and youth with AMC were included by two reviewers. Data regarding activity types, location, outcomes measures, interventions, and barriers and facilitators to participation was extracted. Data was critically appraised using specific evaluation criteria. RESULT: Eleven empirical studies, six gray literature articles and 71 videos met the inclusion criteria. The most common activity types reported in the empirical studies and YouTube videos were active-physical, social, and skill-based activities. Outcome measures included evaluations and questionnaires, none of which were designed to address participation. Interventions did not target participation although the environments could affect participation. CONCLUSION: The paucity of research indicates a need for future studies of participation in this population. Interventions should target participation and address environmental barriers. Videos provide insight for clinicians, youth, and families to help promote participation in the natural environment.

Arthrogryposis multiplex congenita definition: Update using an international consensus-based approach.

Arthrogryposis multiplex congenita (AMC) has been described and defined in thousands of articles, but the terminology used has been inconsistent. Some have described it as a diagnosis or syndrome, others as a term or clinical finding. This lack of common language can lead to confusion in clinical and research communities. The aim of this study was to develop a consensus-based definition for AMC using international expert opinion. A consensus-based definition will help harmonize research and clinical endeavors and will facilitate communication among families, clinicians, and researchers. This article describes the methodology used leading to a proposed definition of AMC. First, a literature review was conducted to identify AMC definitions used in included studies. The most commonly used words in the definitions were extracted. Second, a group of eight experts in AMC was selected to identify elements considered critically important to the definition of AMC. Third, based on these critical elements and the literature review, a definition was drafted by the research team. Fourth, a modified Delphi consensus process was conducted using electronic surveys with 25 experts in the field of AMC from eight countries. Survey results were analyzed quantitatively and qualitatively and drafts were modified accordingly. Three rounds of surveys were completed until consensus was reached on a definition of AMC. An annotation of this definition, developed by a panel of international experts, is provided in a separate manuscript in this special issue.

International multidisciplinary collaboration toward an annotated definition of arthrogryposis multiplex congenita.

Arthrogryposis multiplex congenita (AMC) has been described and defined in thousands of articles, but the terminology used has been inconsistent in clinical and research communities. A definition of AMC was recently developed using a modified Delphi consensus method involving 25 experts in the field of AMC from 8 countries. Participants included health care professionals, researchers, and individuals with AMC. An annotation of the definition provides more in-depth explanations of the different sentences of the AMC definition and is useful to complement the proposed definition. The aim of this study was to provide an annotation of the proposed consensus-based AMC definition. For the annotation process, 17 experts in AMC representing 10 disciplines across 7 countries participated. A paragraph was developed for each sentence of the definition using an iterative process involving multiple authors with varied and complementary expertise, ensuring all points of view were taken into consideration. The annotated definition provides an overview of the different topics related to AMC and is intended for all stakeholders, including youth and adults with AMC, their families, and clinicians and researchers, with the hopes of unifying the understanding of AMC in the international community.

Driving performance in a power wheelchair simulator.

PURPOSE: A power wheelchair simulator can allow users to safely experience various driving tasks. For such training to be efficient, it is important that driving performance be equivalent to that in a real wheelchair. This study aimed at comparing driving performance in a real and in a simulated environment. METHOD: Two groups of healthy young adults performed different driving tasks, either in a real power wheelchair or in a simulator. Smoothness of joystick control as well as the time necessary to complete each task were recorded and compared between the two groups. Driving strategies were analysed from video recordings. The sense of presence, of really being in the virtual environment, was assessed through a questionnaire. RESULTS: Smoothness of joystick control was the same in the real and virtual groups. Task completion time was higher in the simulator for the more difficult tasks. Both groups showed similar strategies and difficulties. The simulator generated a good sense of presence, which is important for motivation. CONCLUSIONS: Performance was very similar for power wheelchair driving in the simulator or in real life. Thus, the simulator could potentially be used to complement training of individuals who require a power wheelchair and use a regular joystick. [Box: see text].