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

Res Involv Engagem. 2022 Feb 18;8(1):5. doi: 10.1186/s40900-022-00336-y.

Abstract

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.

Keywords: Arthrogryposis; Clinical guidance; Consensus; Integrated knowledge translation; Modified Delphi; Pediatrics; Rehabilitation.

Plain language summary

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.