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    Disability and Rehabilitation

    ISSN: 0963-8288 (Print) 1464-5165 (Online) Journal homepage: https://www.tandfonline.com/loi/idre20

    Rehabilitation needs of youth with arthrogryposismultiplex congenita: Perspectives from keystakeholders

    Caroline Elfassy, Vasiliki Betty Darsaklis, Laurie Snider, Cynthia Gagnon,Reggie Hamdy & Noemi Dahan-Oliel

    To cite this article: Caroline Elfassy, Vasiliki Betty Darsaklis, Laurie Snider, Cynthia Gagnon,Reggie Hamdy & Noemi Dahan-Oliel (2019): Rehabilitation needs of youth with arthrogryposismultiplex congenita: Perspectives from key stakeholders, Disability and Rehabilitation, DOI:10.1080/09638288.2018.1559364

    To link to this article: https://doi.org/10.1080/09638288.2018.1559364

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    Published online: 11 Feb 2019.

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  • ORIGINAL ARTICLE

    Rehabilitation needs of youth with arthrogryposis multiplex congenita:Perspectives from key stakeholders

    Caroline Elfassya,b,c , Vasiliki Betty Darsaklisa,b , Laurie Sniderc, Cynthia Gagnond,e, Reggie Hamdya,f andNoemi Dahan-Oliela,b,c

    aClinical Research Department, Shriners Hospital for Children, Canada, Montreal, Canada; bRehabilitation Department, Shriners Hospital forChildren Canada, Montreal, Canada; cSchool of Physical and Occupational Therapy, McGill University, Montreal, Canada; dCentre deRecherche Charles-Le-Moyne, Sherbrooke University, Longueuil, Canada; eGroupe de Recherche Interdisciplinaire sur les MaladiesNeuromusculaires (GRIMN), Jonquiere, Canada; fPediatric Orthopedics Department, McGill University, Montreal, Canada

    ABSTRACTBackground: Arthrogryposis multiplex congenita is a term used to describe congenital contractures in atleast two body parts with an overall prevalence of 1 in 3000 live births. It is often caused by lack of fetalmovement in utero and presents as contractures of varying severity, which may affect the upper andlower extremities, the spine and jaw. Currently, no practice recommendations exist to inform best clinicalpractice for arthrogryposis multiplex congenita.Purpose: To identify the needs surrounding rehabilitation as experienced by youth with arthrogryposismultiplex congenita, caregivers, and clinicians and to propose solutions to develop family- and client-centred rehabilitation recommendations.Materials and methods: A modified experience-based co-design methodology was used where qualita-tive interviews were conducted with key stakeholders.Results: Twenty-seven participants completed the interviews and demographic information was collectedwhere early-active rehabilitation began at birth in most cases and became less frequent through adoles-cence. Three overarching themes were determined for each stakeholder group.Conclusions: All participants reported that early-active rehabilitation is beneficial as it helps determinefuture treatments. Transition times and participation need to be at the center of interventions to ensurethat the needs of youth with arthrogryposis multiplex congenita are being met. The development a con-dition-specific outcome-measure and rehabilitation practice recommendations will assist clinicians inaddressing the needs of youth with arthrogryposis multiplex congenita.

    IMPLICATIONS FOR REHABILITATION Arthrogryposis multiplex congenita presents in at least two different areas of the body as multiple

    congenital contractures of varying severity which may affect the upper and lower extremities, spineand jaw.

    Youth with arthrogryposis multiplex congenita identified participation as an essential componentof their life, however caregivers and clinicians did not emphasize this need.

    Gathering information from different stakeholders is important to ensure varying needsare addressed.

    Rehabilitation was reported to be beneficial from early childhood to late adolescence by youth, care-givers, and clinicians.

    Frequency of rehabilitation diminished over time, emphasizing the need for continued follow-up intoadolescence.

    ARTICLE HISTORYReceived 20 September 2018Revised 10 December 2018Accepted 11 December 2018

    KEYWORDSarthrogryposis multiplexcongenita; qualitativemethods; rehabilitation;pediatric; client-centredresearch

    Introduction

    Arthrogryposis multiplex congenita (AMC) is a term used todescribe multiple contractures present at birth in at least twobody parts with an overall prevalence of 1 in 3000 live births[1,2]. It is most often caused by lack of fetal movement in utero,can be sporadic or inherited in an autosomal or recessive fashion.Individuals with AMC display variable clinical features, which mayinclude contractures of varying severity to the upper and lowerextremities, the spine and the jaw, as well as involvement of the

    gastro-intestinal, genito-urinary and central nervous systems [36].The contractures are non-progressive in nature but may impactand limit independence in mobility, self-care and activities of dailyliving [36]. For this reason, early intensive rehabilitation is war-ranted to provide splinting, range of motion exercises, strengthen-ing programs. Surgical interventions are carried out to correctdeformities and promote daily function [710].

    Despite early intervention and the non-progressive nature ofthe condition, recurrence of joint contracture is common,

    CONTACT Caroline Elfassy caroline.elfassy@mail.mcgill.ca Clinical Research Department, Shriners Hospital for Children-Canada, 1003 Decarie Boulevard,Montreal, Quebec, H4A 0A9, Canada

    Supplemental data for this article can be accessed here.

    2019 Informa UK Limited, trading as Taylor & Francis Group

    DISABILITY AND REHABILITATIONhttps://doi.org/10.1080/09638288.2018.1559364

    http://crossmark.crossref.org/dialog/?doi=10.1080/09638288.2018.1559364&domain=pdfhttp://orcid.org/0000-0003-0918-9316http://orcid.org/0000-0002-3469-6507http://orcid.org/0000-0001-8567-7173https://doi.org/10.1080/09638288.2018.1559364http://www.tandfonline.com
  • particularly in skeletally immature patients [7]. Although early andcontinued intensive rehabilitation is justified for children withAMC and advocated by many researchers, very few studies havedocumented the rehabilitation process and services required forchildren with AMC and their families [11,12]. Indeed, the SecondInternational Symposium on Arthrogryposis held in St-Petersburgin 2014 indicated an urgent need for guidelines to be developedregarding various therapies [12]. As such, no rehabilitation guide-lines or practice recommendations currently exist to inform bestclinical practice for this population.

    The current literature available on rehabilitation treatment forchildren with AMC, emphasizes improving range of motion andminimizing joint contractures in order to facilitate mobility andincrease autonomy in activities of daily living [13,14]. Multiplemodels, including the Canadian Model of OccupationalPerformance and Engagement (CMOP-E), have shown that healthdoes not solely rely on physical function but rather is influencedby a multitude of factors including psychosocial well-being, par-ticipation and the environment [15]. The lack of informationregarding interventions to address issues in areas other thanphysical function in AMC creates a large knowledge gap for clini-cians [8,14,1620]. Additionally, as AMC is heterogeneous, witheach individual and family experiencing different needs whichpermeate throughout all spheres of function, care must be holisticand take into account the needs of both youth andtheir caregivers.

    Materials and methods

    Aim

    The objective of this study was to identify the needs surroundingrehabilitation as experienced by youth with AMC, caregivers, andclinicians (i.e., occupational therapists and physical therapists) andto propose solutions to develop family- and client-centredrehabilitation recommendations.

    Study design

    A modified experience-based co-design methodology was used.It is an approach to improving healthcare services that uses aco-design process, involving key stakeholders to reflect on theirexperiences and work together to identify priorities, devise andimplement changes [21]. In this study, qualitative interviews wereconducted in person or by telephone with key stakeholders,

    consisting of youth with AMC, their caregivers, and clinicians. Allinterviews were digitally recorded and transcribed for further ana-lysis. The research team developed two interview guides, foryouth and caregivers, and clinicians. The CMOP-E was used as thetheoretical framework to develop these interview guides, whichincluded items on the physical (e.g., strength, range of motion,endurance), cognitive (e.g., perception, judgement, attention),affective (e.g., self-esteem, motivation, interpersonal relations),environmental (e.g., physical, social, institutional barriers), occupa-tional performance and activity (e.g., self-care, mobility),