By Simmonds JV1, 2, 3, Ellen Ewer1, 4, De Wandele Inge5, Kazkaz Hanadi2, 6, Ninis Nelly7, Rowe Peter8, Tang Eudora1, 6
Often, people with symptomatic joint hypermobility report symptoms of pain, fatigue and unstable joints. However, we now understand that symptoms related to joint hypermobility extend further than this, sometimes affecting the heart, the bladder and bowel and a person’s mental health. The presence and severity of these symptoms vary from person to person; however, all can greatly impact on quality of life. With this in mind, an international research group are developing ‘The Spider’, a questionnaire which aims to provide an overview of an individual’s symptom profile.
The Spider has eight legs which each represent a symptom domain. An individual with symptomatic hypermobility will be asked to complete the 31-item questionnaire which assesses the impact that these eight symptom domains have on their life. The domains include pain, fatigue, neuromusculoskeletal, cardiac dysautonomia, gastrointestinal, urogenital, anxiety and depression symptoms. Once the questionnaire has been completed, a radar graph shaped like a Spider’s web is produced, giving a visual representation of the individuals symptom profile (Figure 1). When used in a clinical setting, this allows the health professionals (and children and their parents) to gain a clear picture on the impact of symptoms, and ensure care and management are correctly directed.
Before this tool can be published, the research team need to make sure it is valid – that each domain accurately measures what we intend it to measure. Initial face and content validity of the Spider has been established,1 and significant convergent and known group validity of the pain and fatigue domains were confirmed.2 This study aimed to establish convergent and known group validity of the anxiety and depression domains of the Spider. Convergent validity assesses the degree in which the concept of the measure being tested (the Spider) correlates with another measure. Known group validity assesses the ability of the tool to differentiate between the group of interest (people with symptomatic joint hypermobility) and the ‘known group’ (people without joint hypermobility).
The HMSA kindly supported recruitment for this project, alongside the Ehlers-Danlos Support UK and the Ehlers-Danlos Society. Two-hundred participants aged between 12 and 18 were recruited for this project, and we express our immense gratitude to the HMSA members who volunteered. The participants were asked to complete the Spider anxiety and depression domain questions, and the Revised
Children’s Anxiety and Depression Scale (RCADS-25), a validated outcome measure which assesses anxiety and depression. The average scores from these two measures were analysed using a correlational statistical test (Spearman’s rho). The anxiety domain had significant moderate convergent validity with the RCADS-25 anxiety questions (r=0.613, p=<0.001) and the depression domain had significant strong convergent validity with the RCASD-25 depression questions (r=0.794, p=<0.001).
In summary, this means that the pain, fatigue, anxiety and depression domains have been shown to be valid in measuring what they were designed to measure in adolescents with symptomatic joint hypermobility. Currently, the research team are validating the remaining four domains with adolescents who have symptomatic hypermobility to ensure the entire screening tool is valid. We also hope to validate this tool with adults and develop a scoring system to aid clinicians in monitoring symptoms over time. This tool will guide holistic care and management for adolescents with symptomatic hypermobility, ensuring each individual’s symptom profile is considered.
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References:
1. De Wandele, I. K., H. Tang, E. Ninis, N. Rowe, P. Simmonds, J (2020). Development and initial validation of The Spider, a multisystem symptom impact questionnaire for patients with joint hypermobility (Part One). EDS ECHO Virtual Summit. .
2. Tang I., De Wandele I., Kazkaz H, Ninis N, Rowe P, Simmonds JV (2020) Development and initial validation of The Spider, a multisystem symptom impact questionnaire for patients with joint hypermobility (Part Two). EDS ECHO Virtual Summit. September.
The research team:
Simmonds JV 1,2,3, Ellen Ewer 1,4, De Wandele Inge 5, Kazkaz Hanadi 2,6, Ninis Nelly 7, Rowe Peter 8, Tang Eudora 1,6
1.UCL Great Ormond Street Institute of Child Health
2. University College London Hospital NHS Trust, London, UK
3. London Hypermobility Unit, Central Health Physiotherapy, Wellington Hospital, London, UK
4. Hounslow and Richmond Community Healthcare NHS Trust, UK
5. Center for Medical Genetics, Ghent University Hospital, Ghent, Belgium
6. National University Hospital, Singapore
7. St Mary’s Hospital, Imperial Hospital NHS Trust, London, UK
8. Johns Hopkins University, Baltimore, USA
Roles and contributions:
Dr Jane Simmonds: Associate Professor at UCL and clinical specialist physiotherapist in Ehlers Danlos Syndrome (EDS) and hypermobility spectrum disorders. Principal Investigator for this research project. Dr Simmonds is part of the initial team who created the Spider questionnaire. Advising on research design and recruitment from the London Hypermobilty Unit. Also advising on comparator questionnaires for musculoskeletal, gastrointestinal and urogenital comparators based on previous research. Dr Simmonds has piloted the Spider questionnaire within the London Hypermobility Unit.
Ellen Ewer: Specialist Paediatric Physiotherapist and honorary research fellow at UCL. Ellen completed validation of the anxiety and depression domains of the Spider during her MSc at UCL and is continuing to validate the remaining four domains with the research team.
Dr Inge De Wandele: Postdoctoral researcher and specialist physiotherapist. Dr De Wandele is part of the initial team who created the Spider questionnaire. She has expertise in EDS and cardiac dysautonomia. Her PhD focused on cardiac dysautonomia in EDS. Inge will advise on the comparator questionnaires relating to musculoskeletal symptoms, cardiac dysautonomia and on statistical analysis.
Dr Hanadi Kazkaz: Consultant Rheumatologist. Dr Kazkaz is part of the initial team who created the Spider questionnaire with specialist input in relation to the gastrointestinal and urogenital questions. The questionnaire has been piloted in the NHS and within the London Hypermobility Unit.
Dr Nelly Ninis: Consultant Paediatrician with a special interest in dysautonomia and multisystem implications. Dr Ninis is part of the initial team who created the Spider questionnaire with multisystem input particularly related to the fatigue, gastrointestinal and urogenital questions. She has published an international consensus paper on dysautonomia in adolescents.
Professor Peter Rowe: Professor of Paediatrics: Professor Rowe is part of the initial team who created the Spider questionnaire. His expertise are in dysautonomia and fatigue and he is published widely in this field. Prof Rowe will advise on the comparator questionnaires for cardiac dysautonomia.
Eudora Tang: Specialist Paediatric physiotherapist who has successfully conducted validation of two domains of the Spider (Pain and Fatigue) as part of her MSc at UCL.