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

Abstract

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.

The range of publications on arthrogryposis multiplex congenita from 1995 to 2022—A scoping review

“…Among all HRQoL studies, we found a wide range of questionnaires. Our scoping review aligns with the findings from Cachecho and colleagues, who found that the most commonly used outcome measures were the PODCI for children and the SF‐36 for adults, but also with varied outcome measures, especially for adults (Cachecho et al, 2021).…”

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

“…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.…”

“…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.…”

“…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.…”