Exploring the underlying mechanism of pain-related disability in hypermobile adolescents with chronic musculoskeletal pain

Abstract

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.

Generalized Joint Hypermobility and Anxiety in Adolescents and Young Adults, the Impact on Physical and Psychosocial Functioning

“…fear of failure, fear of injury) and psychological stress in these high performers can induce fear avoidance behavior, which may have influenced maximal performance of individuals with GJH and anxiety. Performance avoidance goals were also observed by Lench et al in participants with high trait anxiety [ 27 ], and a recent review on hypermobile adolescents and young adults proposed that vulnerability to anxiety might cause avoidance behavior as a coping strategy to avoid injury and complaints [ 9 ]. In addition, GJH is highly prevalent in the general child population and considered common during growth [ 28 ].…”

“…There have been minimal studies pertaining to disease progression in GJH, h-EDS, and HSD, with the available studies primarily addressing physical functioning [ 8 , 9 ]. In the disease progression of hEDS/HSD from childhood, adolescence, and adulthood roughly three phases are proposed based on cross-sectional observations by Castori et al [ 10 ].…”

“…However, this theory has not been verified yet. Current models in the GJH literature describe an association between GJH and anxiety but do not differentiate individuals with both GJH and anxiety from those with GJH alone [ 9 , 11 ]. The association between GJH and anxiety and the impact on physical functioning in healthy adolescents and young adults has not been studied in detail, whereas adolescents and young adults, in this specific lifespan, might be even more vulnerable for anxiety related disability, based on fear of negative evaluation or social phobias [ 12 ].…”

Sensing hand position in Ehlers-Danlos Syndrome

“…Unfortunately, there have been no EDS proprioceptive studies to date which have included a measure of physical activity in their protocol; we recommend including such a questionnaire in future studies on proprioception in EDS to explore the possibility that physical activity is also a partial predictor of proprioceptive imprecision. It is important to note that a few studies have already found various forms of exercise to be effective in relieving pain, and sometimes any initially observed proprioceptive deficits in those with joint hypermobility [1,6,19], which provides support for the fear-avoidance model proposed by van Meulenbroek et al [18]. It is our impression that each of these theories at least partially explains the proprioceptive deficit observed in our research, and the work of others, but further studies need to be done to fully understand the impact of each of these potential causes on proprioceptive sensitivity in EDS.…”

“…Unfortunately, no studies to date have directly linked peripheral nerve damage to deficits in proprioception in this population; we recommend that future studies on nerve damage in EDS incorporate some form of proprioceptive testing to better understand this possibility. Recently, van Meulenbroek et al [18] suggested that the proprioceptive differences commonly seen in adolescents with Hypermobile EDS could be due to physical deconditioning, because of kinesiophobia, since those with joint hypermobility are more prone to injuries and experience pain more intensely than the general population. Unfortunately, there have been no EDS proprioceptive studies to date which have included a measure of physical activity in their protocol; we recommend including such a questionnaire in future studies on proprioception in EDS to explore the possibility that physical activity is also a partial predictor of proprioceptive imprecision.…”