Pain among children and adults living with arthrogryposis multiplex congenita: A scoping review

Cirillo, Alexa; Collins, Jessica; Sawatzky, Bonita; Hamdy, Reggie C.; Dahan‐Oliel, Noémi

doi:10.1002/ajmg.c.31725

Cited by 12 publications

(16 citation statements)

References 57 publications

(148 reference statements)

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“…Individuals with AMC may present varying degrees of physical limitations and may undergo several surgeries throughout their life [ 18 ]. Pain has been identified as an important issue and limiting factor in adults with AMC [ 19 ]. Therefore, in order to tailor interventions and optimize health and wellbeing throughout the lifespan, it is important to understand the psychosocial experience of individuals with AMC and to identify influencing factors.…”

Section: Introductionmentioning

confidence: 99%

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

Cachecho

Boruff

Wong

et al. 2021

Health Qual Life Outcomes

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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.

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Section: Introductionmentioning

confidence: 99%

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

Cachecho

Boruff

Wong

et al. 2021

Health Qual Life Outcomes

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“…The survey will inquire about the different assessments and interventions that rehabilitation practitioners use with this population, in relation to the identified priority areas (i.e., muscle and joint function, pain, mobility and self-care, participation, and psychosocial wellbeing), for each age group (0–2 years, 3–6 years, 7–12 years, 13–18 years). The questionnaire will be based on the findings from the scoping reviews and from the experience of clinicians on the team, and include the assessment tools and treatment strategies reported in the literature [ 8 , 19 – 21 ]. We will gather demographic information on the respondents (profession, country, years of experience working with children with AMC), and their e-mail address should they wish to be contacted for the subsequent phases of the project.…”

Section: Methodsmentioning

confidence: 99%

“…Meetings will be led by an expert in the GRADE approach. The groups will consider findings from the scoping reviews [ 8 , 19 – 21 ], and the results from the clinician survey (Phase 1) to guide the discussions regarding important outcomes pertaining to each priority area. Important questions and outcomes will be identified, and the balance of desirable (e.g., improved muscle and joint function, mobility, autonomy) and undesirable outcomes (e.g., fatigue, pain) will be considered.…”

Section: Methodsmentioning

confidence: 99%

“…Preliminary work consisted of a series of scoping reviews on the five priority areas as a lack of empirical studies in rehabilitation precluded a systematic review [ 8 , 19 – 21 ]. These scoping reviews revealed a lack of high-quality studies (e.g., clinical trials) or consensus guidelines to guide clinicians toward the most clinically useful and suitable assessment tools and best evidence-based rehabilitation interventions for individuals with AMC.…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2022

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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.

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“…However, contractures can recur after intervention or worsen over time [5]. In addition to these contractures, individuals with AMC may present with decreased muscle mass and bone density, bone deformities, and pain, leading to activity limitations and participation restrictions in activities of daily living such as feeding, dressing, mobility, and sports [3,[6][7][8][9]. Early interventions such as stretching, splinting, bracing, casting, rehabilitation, and surgical procedures have been shown to augment and maintain range of motion and strength, and therefore, to promote independence in daily activities [10].…”

Section: Introductionmentioning

confidence: 99%

A Telerehabilitation Intervention for Youths With Arthrogryposis Multiplex Congenita: Protocol for a Pilot Study (Preprint)

Gagnon¹,

Collins²,

Elfassy³

et al. 2020

Preprint

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BACKGROUND Arthrogryposis multiplex congenita (AMC) is characterized by joint contractures present in at least two body areas. In addition to these contractures, individuals with AMC can have decreased muscle mass, leading to limitations in activities of daily living. Exercise has the potential to maintain or improve the range of motion and muscle strength. However, this type of intervention necessitates frequent follow ups that are currently difficult to provide for youths with AMC because they often live far from a specialized hospital. To overcome this distance challenge, telecommunication technologies can be used to deliver rehabilitation remotely, which is called telerehabilitation. The study protocol for one such type of rehabilitation will be presented in this paper. OBJECTIVE This pilot study aims to (1) evaluate the feasibility of using telerehabilitation to provide a home exercise program for youths with AMC, and (2) assess the effectiveness of a home exercise program. METHODS A total of 10 youths aged 8-21 years with AMC will be recruited. The intervention consists of a 12-week individualized home-based exercise program delivered remotely using telerehabilitation. At baseline, youths will complete the Physical Activity Questionnaire for Adolescents and the Pediatrics Outcomes Data Collection Instrument to assess pain, function, and level of physical activity. During the first telerehabilitation meeting, the rehabilitation therapists will measure range of motion using a virtual goniometer and assess the youth’s functional level. The therapists will then use the Goal Attainment Scale to set objectives and develop the individualized intervention. Follow ups will occur every 3 weeks to make sure exercises are performed safely and to progress the exercises when needed. At the end of the 12-week intervention, rehabilitation therapists will re-evaluate the youth using the same outcome measures as the initial evaluation. The youths will be asked to complete the same questionnaires, with the addition of questions about their satisfaction regarding the intervention. Nonparametric and descriptive statistics will be used to evaluate the feasibility and effectiveness. RESULTS Ethics approval was obtained in October 2018. Recruitment and data collection started in January 2019 and was completed in May 2020. CONCLUSIONS This pilot study will help us learn how a large-scale project may work in practice to improve outcomes in physical activity, pain, and function, and goal attainment among youths with AMC, thus informing a future clinical trial. INTERNATIONAL REGISTERED REPORT DERR1-10.2196/18688

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Pain among children and adults living with arthrogryposis multiplex congenita: A scoping review

Cited by 12 publications

References 57 publications

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

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

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

A Telerehabilitation Intervention for Youths With Arthrogryposis Multiplex Congenita: Protocol for a Pilot Study (Preprint)

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