The effectiveness of a multidisciplinary intervention strategy for the treatment of symptomatic joint hypermobility in childhood: a randomised, single Centre parallel group trial (The Bendy Study)

Joint hypermobility is common in childhood, with a high prevalence reported in the literature when adult criteria are applied [1‐7]. Hypermobile joints are not synonymous with morbidity; for example, gymnasts, musicians and athletes can use this flexibility to their advantage [8, 9]. However, pain has been reported to occur in as many as 55% of all children with hypermobility [10] and children with symptomatic hypermobility account for a large proportion of referrals to tertiary paediatric rheumatology services [11]. Symptomatic hypermobility (previously referred to as joint hypermobility syndrome (JHS)) is varied in its presentation and is challenging both to diagnose and manage, reflected by a wide variation in practice.

Given the frequency of this presentation there has been a need to develop consistent approaches to treatment. Management guidelines produced by The British Society for Paediatric and Adolescent Rheumatology based on clinical consensus advocate a multidisciplinary approach including physiotherapy (PT), occupational therapy (OT) and podiatry (Additional file 1), with an emphasis on self-management and delivered in clinical, home and school settings. The therapeutic interventions are often targeted at increasing muscle strength and proprioception in the affected joints and limbs [12‐15]. However, objective evidence of their effectiveness is almost completely lacking [15].

A total of 157 children were assessed for eligibility by the paediatric rheumatologist (KA). Of these 38 were not randomised: 11 did not meet the eligibility criteria and 27 declined consent.

As illustrated in Fig. 1, 119 children were randomised, and 59 (50%) received the intervention. Of these, 111 attended 3 month follow up and 105 attended 12 month follow up. Of the 59 randomised to the intervention, 56 (93.3%) completed the multidisciplinary, multisite programme. All 56 attended 3 month follow up and 54 (91.5%) attended 12 month follow up. Of the 60 randomised to receive standard care, 55 (91.7%) were assessed at 3 months and 51 (85%) at 12 months. Overall the drop out was 6% at 3 months and a further 6% at 12 months.

This randomised, single centre parallel group trial compared a structured multidisciplinary, multisite intervention programme with standard care in children with symptomatic hypermobility. No added benefit was seen from this intervention. Both treatment groups showed improvements in child and parent reported pain scores, coordination and grip strength after one year follow up. The self-reported HRQoL (as assessed by CHU9D) did not change in either group through the period of follow up. Our HRQoL scores for both groups remained within the normal reference range for healthy children [29, 30], suggesting that while children with symptomatic hypermobility experience a degree of pain and motor impairment, these symptoms tend not to limit their overall wellbeing compared to their healthy peers.

Clinicians faced with diagnosing and planning treatment for children with hypermobility and pain currently have very sparse evidence on which to base their decisions. A systematic review [27] identified only three robust studies on the management of hypermobility [14, 31, 32], only two of which were conducted in the paediatric population [14, 32] and just one was a randomised comparative trial [14]. The non-randomised studies conducted in children were limited in their scope and targeted single joint-specific areas associated with hypermobility (knee proprioception and handwriting) [31, 32]. The single randomised trial focused on physiotherapy and compared targeted with a general programme of treatment. As in our own study, children showed an improvement in pain through follow up with no evidence to favour any particular management strategy. However, interpretation is limited by the small size (57 participants) and consequent lack of power, and low retention rate (just 56%). The influence of a fuller range of interventions that might be delivered by the multidisciplinary team has not been addressed.

The present study was designed to address the potential value of a multidisciplinary, multisite intervention package. Our intervention was developed through systematic literature review and clinical consensus, and adheres to the current British Society for Paediatric and Adolescent Rheumatology guidance. The intervention was designed for efficient delivery in a general hospital and community setting in a limited time.

This study has important limitations. We adopted a pragmatic approach to diagnosis, utilising a ‘real world’ clinical setting, and included children that an experienced paediatric rheumatologist diagnosed with symptomatic hypermobility. In taking this approach, we feel that the participants included reflect the broad range of the population referred to paediatric secondary and tertiary care and subsequently diagnosed with symptomatic hypermobility. Diagnostic criteria for JHS or Ehlers-Danlos hypermobility type (now termed hypermobility spectrum disorders and hypermobile EDS) [33, 34] have not been validated in the paediatric population. We applied Bulbena and Beighton scoring systems to confirm generalised hypermobility, to add face validity and to facilitate comparison with other published studies. The cut off values used conform to published literature, however population data on joint hypermobility scores across all age groups is still lacking. Higher cut off scores have been suggested but not validated [1, 34, 35].

All participants received at least ‘standard care’ as an ethical requirement and could not be blinded to the interventions, which require active engagement. Participants gave fully informed consent and were therefore aware of the interventions in the treatment arm, which may have influenced behaviour. The trial coordinator recorded the number of therapy attendances in the preceding 3 months at each follow up appointment in all participants to ensure that the assessing physiotherapist (VE) remained fully blinded. We are aware that in the standard care arm 36 children (60%) received at least one additional session of physiotherapy or occupational therapy and five received orthotics. Inevitably those randomised to the standard care group, irrespective of additional sessions, received a greater degree of attention than might have occurred outside an interventional trial.

We did not include direct measurement of child or parental anxiety in our study protocol. A systematic review of the literature conducted by our group has shown an association between anxiety and pain in children with hypermoblity [36]. It is possible that a reduction in anxiety due to clarification of the condition and education could have resulted in the improvements in pain seen across both groups.

A further limitation is the lack of validated outcome measures for symptomatic hypermobility in childhood. The pain measure used in the study assessed the intensity at the most prominent site and did not reflect the variable nature of pain or the variation in sites over the period assessed. The CHAQ is an assessment tool for children with juvenile idiopathic arthritis. It has not been validated for use in symptomatic hypermobility but has been used in a previous study [14]. It is conceivable that the outcome measures used may lack adequate sensitivity to detect a difference between the interventions. It is also possible that the sample size may have limited our ability to detect differences between the two intervention groups.

This study does not address whether hypermobile children report more pain compared to their non-hypermobile peers. In the multilevel model, there was no direct relationship between pain and degree of hypermobility and the degree of hypermobility was not related to level of symptom improvement or response to treatment. The data suggest that the relationship between the degree of joint mobility and a child’s experience of pain is not direct.

We conclude that for children presenting with symptomatic hypermobility, standard care offered in routine practice in the UK is sufficient to lead to a sustained improvement in symptoms over a twelve-month period and that a more intensive multidisciplinary approach offers little further benefit. We recognise that symptomatic hypermobility is a heterogeneous condition and there may be subgroups that differ sufficiently to benefit from more intensive intervention [37, 38]. Nevertheless, our results suggest that for the majority the provision of education and positive interventions aimed at promoting healthy exercise and self-management leads to significant benefit without the need for more complex interventions. We believe our findings will help the development of prompt, simple and effective approaches to management, and will help diffuse beliefs about disability and negative consequences of this condition.