E. Ewer1,2, I. De Wandele3, H. Kazkaz4,1, N. Ninis5, P. Rowe6, E. Tang7, R. De Pauw8, L. Rombaut3, C. Barrett1,9, J.V. Simmonds1,9
1University College London, Great Ormond Street Institute of Child Health, London, United Kingdom, 2Hounslow and Richmond Community Healthcare NHS Trust, London, United Kingdom, 3Ghent University Hospital, Center for Medical Genetics, Ghent, Belgium, 4University College London Hospital NHS Trust, London, United Kingdom, 5St Mary’s Hospital, Imperial Hospital NHS Trust, London, United Kingdom, 6John Hopkins University, Baltimore, United States, 7National University Hospital, Singapore, Singapore, 8Statting, Ghent, Belgium, 9Central Health Physiotherapy, London, United Kingdom
Background: Symptoms of Hypermobile Ehlers-Danlos Syndrome (hEDS) and Hypermobility Spectrum Disorders (HSD) often extend beyond the musculoskeletal system. These symptoms significantly impact quality of life, but are neglected when seeking diagnosis and treatment. Further insight into the profile of symptoms of hEDS/HSD during clinical assessment is required to offer holistic and targeted care.
Purpose: A comprehensive questionnaire, the Spider, has been developed to evaluate the impact of these symptoms and guide management. The eight domains assess neuromusculoskeletal, pain, fatigue, cardiac dysautonomia, urogenital, gastrointestinal, anxiety and depression symptoms. Once completed, a radar graph shaped as a spider web is produced, allowing identification of symptoms needing assessment and management. Content and face validity of the Spider were established (De Wandele et al., 2020). Three studies examined the convergent and known group validity of The Spider pain and fatigue domains (Tang et al., 2020), anxiety and depression domains (Ewer et al., 2021) and gastrointestinal, urogenital, cardiac dysautonomia and neuromusculoskeletal domains (Ewer et al., 2022) in adolescents.
Methods: Ethical approval was granted by the University College London Ethics Committee. 768 participants, aged 12 to 18, were recruited through 3 patient charities (EDS support UK, ED Society and HMSA) and Central Health hypermobility unit. 684 patients had HSD/hEDS and 84 were healthy controls. Participants anonymously answered Spider questionnaire domains and validated comparative questionnaires via REDCap (an online survey platform). Results were analysed using SPSSv27.
Results: Convergent validity was established through correlational analysis with significant results (p=<0.001). Strong correlations were found between the Spider pain domain and the CALI-9 (r=0.74), the Spider fatigue domain and the MFS (r=-0.70), the Spider depression domain and the RCADS-25 depression domain (r=0.79), the Spider cardiac domain and the COMPASS-31 (r=0.73), the Spider gastrointestinal domain and the PEDS-QL (r=0.77) and the Spider neuromusculoskeletal domain and the BIOH (r=0.74). Moderate correlations were shown between the Spider pain domain and the NPRS (r=0.54), the Spider anxiety domain and the RCADS-25 anxiety domain (r=0.6) and the Spider urogenital domain and Peds-DVSS (r=0.6). Known group validity assessment showed the Spider could differentiate between those with and without hEDS/HSD in 7 domains (p=<0.001). The urogenital domain did not show a significant difference, likely due to reduced numbers of hypermobile participants with bladder symptoms (p=2.36).
Conclusions: The results demonstrate acceptable convergent validity of the Spider questionnaire. The Spider provides a novel approach to assessing the multisystemic symptoms associated with HSD/hEDS to help direct care.
Implications: In clinics, the completed questionnaire will help direct care to meet individual needs. In research, the Spider will help track the natural history of the co-morbidities of people with hEDS/HSD, and may help discover determinants for more severe prognoses. The Spider scores may help identify subgroups of patients with similar symptom profiles, and help describe differences between patients with different heritable connective tissue disorders.
Future research includes validation in younger and older populations and factor analysis and internal reliability analysis of the Spider. An app is being designed so the Spider can be used by clinicians and researchers internationally. Translation studies will be completed to allow international access.
Future research includes validation in younger and older populations and factor analysis and internal reliability analysis of the Spider. An app is being designed so the Spider can be used by clinicians and researchers internationally. Translation studies will be completed to allow international access.
Funding acknowledgements: Research was funded by Ehlers Danlos Support UK and the Private Physiotherapy Education Fund (PPEF).
Keywords:
Hypermobility
Questionnaire
Multi-systemic symptoms
Hypermobility
Questionnaire
Multi-systemic symptoms
Topics:
Innovative technology: information management, big data and artificial intelligence
Paediatrics
Globalisation: health systems, policies & strategies
Innovative technology: information management, big data and artificial intelligence
Paediatrics
Globalisation: health systems, policies & strategies
Did this work require ethics approval? Yes
Institution: University College London
Committee: Office of the Vice-Provost (Research, Innovation & Global Engagement)
Ethics number: 19629/001
All authors, affiliations and abstracts have been published as submitted.
