Response to British Society for Rheumatology “Guidance for Management of Symptomatic Hypermobility in Children and Young People – A Guide for Professionals managing Children and Young People with this condition” June 2019
In June 2019 the British Society for Rheumatology published an update of its 2013 document entitled “Guidance for Management of Symptomatic Hypermobility in Children and Young People – A Guide for Professionals managing Children and Young People with this condition.” The Ehlers-Danlos Society has considered this and gives its response below, concluding with some important actions and next steps to improve things.
The Ehlers-Danlos Society, with the hEDS-HSD Working Group and Pediatric Working Group of the International Consortium on Ehlers-Danlos Syndromes (EDS) and Hypermobility Spectrum Disorders (HSD), welcomes in general this guidance. We share the objectives of the British Society for Rheumatology guidelines in supporting medical and allied health professionals in assessing and treating all hypermobility-related health and social care needs, and supporting children and young people in managing their health and wellbeing.
We commend the guidance in raising awareness regarding the need to listen to young people and their families; understand their concerns; assess each individual case thoroughly and as objectively as possible; support and guide care in partnership with the person and their family; encourage a holistic “biopsychosocial” approach; and, encourage self-management and adaptation of a healthy lifestyle.
We welcome the focus of this article on comprehensive multidisciplinary management, as The Ehlers-Danlos Society continues to hear from people experiencing pain and disability being told by their doctors they are “just hypermobile” and not being referred on for management and rehabilitation.
Our first concern, however, relates to comments about joint hypermobility. We recognize that hypermobility can be an asset in some people, however this is clearly not the case for others. We feel such statements about it being an “asset” encourage the view that hypermobility-related disorders are benign, easily remedied, and of little significance. Although it is appropriate to reassure those who are likely to have a straightforward health journey and respond promptly to interventions, it does not help those with persistent, chronic, disabling, hypermobility-related symptoms. There is emerging evidence to assist clinicians in recognizing children with hypermobility who are likely to have persistent problems (Scheper et al, 2017).
Joint instability episodes, recurrent subluxation and joint dislocation, and concerns with proprioception, are often identifiable on clinical assessment. We feel this area is poorly addressed in the guidance. Clinicians should be alerted to consider assessment of joint instability. There are now tools available to support these assessments (e.g. Lower Limb Assessment Score [LLAS], Upper Limb Hypermobility Assessment Tool [ULHAT], and Foot Posture Index [FPI]).
Also, the guidance highlights some but not all of the conditions that add complexity to presentation of EDS and HSD. In addition, it states that these are rare; but in a recent cohort study of a similar population presenting for specialist care, these conditions were frequent (abdominal pain 25%, constipation 30%, diarrhoea 20%, stress incontinence 25%, dizziness 38% [Pacey et al. 2015]) . We recognize the need for, and are actively supporting research, to better understand the relationships between these conditions and EDS and HSD. We recommend that significant non-musculoskeletal symptoms, irrespective of an established relationship with EDS or HSD, are assessed by a medical practitioner.
We recognize that a proportion of children and young people make good recovery to their prior normal levels of function with appropriate intervention. We also recognize that others with more complex concerns can do very well with the correct treatment, though may not return to full function. Others, regardless of having received the correct management, continue to have varying levels of disability. We agree that a return to physical activity and sport participation should be a goal of rehabilitation, and as stated on page 9 “goal setting should be realistic.” For some people, however, high-quality multidisciplinary rehabilitation is either not available or they cannot participate (due to injury, systemic ill health, or severe depression for example). In some circumstances the use of mobility aids should also be considered to facilitate social, vocational, and community participation.
The guidance also raises the importance of avoiding over-medicalization. The Ehlers-Danlos Society and International Consortium fully support the principles that underlie this. However, a balanced approach is required. We raise this concern because in our experience too many young people and adults with EDS or HSD wait too many years for their medical conditions to be correctly diagnosed and validated, which delays appropriate and effective treatment.
The Ehlers-Danlos Society is committed to improving the diagnosis, validation, management, and care of children and young people living with EDS and HSD. We believe that one of the most important steps needed to improve things is to establish Diagnostic and Management Guidelines for Ehlers-Danlos Syndromes and Hypermobility Spectrum Disorders. We are delighted that we have European and global consensus that the International Consortium should lead with these efforts and we are now working hard with influential stakeholders to ensure such guidance is recognized and adopted worldwide.
In parallel to this, at the start of 2019 the hEDS-HSD Working Group in the International Consortium began review of the 2017 diagnostic criteria for hypermobile EDS, with a focus to also create criteria for children and young people. We expect the outcome of this review in the near future.
In addition, we will be holding a round-table discussion focused on these issues in the Spring of 2020 at the Royal Society of Medicine, with global experts from the EDS and HSD International Consortium and key stakeholders, to discuss ways to prioritize the research needed and the best ways to ensure that it gets done. We will also discuss the international growing concerns surrounding child protection, fabricated and induced illness, and over-medicalization.
We welcome the opportunity to work with all organizations seeking to improve care in EDS and HSD.
Lara Bloom, Dr Alan Hakim, Dr Louise Tofts
President and CEO, Chief Medical Officer and Chair hEDS-HSD Working Group, and Chair Paediatric Working Group
The Ehlers-Danlos Society and International Consortium on EDS and HSD
Mark C. Scheper, Lesley L Nicholson,Roger D. Adams, Louise Tofts and Verity Pacey(2017). The natural history of children with joint hypermobility syndrome and Ehlers Danlos Hypermobility type: a longitudinal cohort study. Rheumatology. Online early DOI 10.1093/rheumatology/kex148
Pacey, V, Tofts, L,Adams, R., Munns, C., Nicholson, L (2015). Quality of Life prediction in children with joint hypermobility syndrome.J. Paed Child Health.51(7), 689-95.
