ObjectivesA significant proportion of adolescents with chronic musculoskeletal pain (CMP) experience difficulties in physical functioning, mood and social functioning, contributing to diminished quality of life. Generalized joint hypermobility (GJH) is a risk factor for developing CMP with a striking 35-48% of patients with CMP reporting GJH. In case GJH occurs with one or more musculoskeletal manifestations such as chronic pain, trauma, disturbed proprioception and joint instability, it is referred to as generalized hypermobility spectrum disorder (G-HSD). Similar characteristics have been reported in children and adolescents with the hypermobile Ehlers-Danlos Syndrome (hEDS). In the management of CMP, a biopsychosocial approach is recommended as several studies have confirmed the impact of psychosocial factors in the development and maintenance of CMP. The fear-avoidance model (FAM) is a cognitive-behavioural framework that describes the role of pain-related fear as a determinant of CMP-related disability.ContentPubmed was used to identify existing relevant literature focussing on chronic musculoskeletal pain, generalized joint hypermobility, pain-related fear and disability. Relevant articles were cross-referenced to identify articles possibly missed during the primary screening. In this paper the current state of scientific evidence is presented for each individual component of the FAM in hypermobile adolescents with and without CMP. Based on this overview, the FAM is proposed explaining a possible underlying mechanism in the relations between GJH, pain-related fear and disability.Summary and outlookIt is assumed that GJH seems to make you more vulnerable for injury and experiencing more frequent musculoskeletal pain. But in addition, a vulnerability for heightened pain-related fear is proposed as an underlying mechanism explaining the relationship between GJH and disability. Further scientific confirmation of this applied FAM is warranted to further unravel the underlying mechanism.In explaining disability in individuals with G-HSD/hEDS, it is important to focus on both the physical components related to joint hypermobility, in tandem with the psychological components such as pain-related fear, catastrophizing thoughts and generalized anxiety.

Background: To determine whether adolescents with generalized hypermobility spectrum disorder/ hypermobile Ehlers-Danlos syndrome (G-HSD/ hEDS) show changes in the level of disability, physical functioning, perceived harmfulness and pain intensity after completing multidisciplinary rehabilitation treatment. Methods: Pre-test post-test design. Fourteen adolescents with G-HSD/hEDS participated. The multidisciplinary rehabilitation treatment consisted of a combination of physical training and exposure in vivo. Physical training aims to improve aerobic capacity, muscle strength and propriocepsis for compensating hypermobility. Exposure in vivo aims to decrease disability and pain-related fear. Pre-and post-treatment assessments were conducted to assess the level of disability, physical functioning (motor performance, muscle strength and physical activity level), perceived harmfulness and pain intensity. Results: After completing multidisciplinary rehabilitation treatment, the adolescents showed a significant and clinically relevant improvement (improvement of 67%, p < 0.01) in functional disability. Furthermore, significant improvements were found in motor performance (p < 0.01), muscle strength (p < 0.05), perceived harmfulness (p < 0.01) and pain intensity (p < 0.01) after completing multidisciplinary rehabilitation treatment. Conclusion: Multidisciplinary rehabilitation treatment leads to a significantly and clinically relevant improvement in the level of disability for adolescents with G-HSD/hEDS. Positive effects were also found in physical functioning, perceived harmfulness and pain intensity. Although the results