Authors:
Victoria Busby | University College London, Buckinghamshire Healthcare NHS Trust | United Kingdom
Ellen Ewer | University College London | United Kingdom
Inge De Wandele | Ghent University and Ghent University Hospital | Belgium
Prof. Jane Simmonds | University College London, Central Health Physiotherapy | United Kingdom
Background
The symptoms of hypermobility vary widely and can change across the lifespan. Whilst pain, joint instability and soft tissue injury are most commonly reported, many individuals report manifestations extending beyond the musculoskeletal system. Without routine screening, these multisystemic symptoms may go undetected and, if suboptimally managed, can significantly impact quality of life. The Spider, a 31-item self-report questionnaire, was developed to identify the presence and impact of the symptoms of hypermobility across eight domains: pain, fatigue, neuromusculoskeletal, cardiac dysautonomia, gastrointestinal, urogenital, depression and anxiety. Face and content validity of this new screening tool have been tested, and construct validity of the individual Spider domains has been established in adolescents.
Purpose
The aim of this study was to determine if the total Spider score, aggregated across all domains, provides a valid measure of the magnitude of symptom impact in adolescents and to explore which symptoms have the strongest associations with perceived functional ability in daily life. Convergent validity of the Spider’s total score was examined using an established measure of daily functional ability, the Paediatric Quality of Life Inventory (PedsQL). Known group validity testing determined whether the total score could differentiate between those with and without hypermobility. Finally, the strength with which each multisystemic manifestation (Spider domains) were associated with poorer functional ability relative to one another were explored.
Methods
This was an observational, cross-sectional study. Ethical approval was granted by the UCL Research Ethics Committee. Participants aged 13 to 18 years with and without symptomatic hypermobility were invited to complete the Spider and PedsQL questionnaires online via advertisement through 3 hypermobility charities (EDS UK, The ED Society, HMSA) and a private physiotherapy hypermobility unit. Data was analysed using descriptive and inferential statistics in SPSS.
Results
441 adolescents completed the study. Convergent validity testing, using Pearson’s correlation coefficient, demonstrated a strong inverse correlation between the Spider and PedsQL scores. As Spider scores increased, indicative of greater symptom impact, PedsQL scores decreased accordingly (r=-0.750, p < 0.001). Known-groups validity was evidenced by the significant between-group differences in Spider scores for those without and without hypermobility (median difference 42.34, p < 0.001). Multiple linear regression analysis of the domain scores revealed that the fatigue, cardiac dysautonomia and depression domains were the mostly strongly associated with poorer functional ability in adolescents.
Conclusion(s)
Results support the aggregation of scores from the eight Spider domains as a valid measure of the overall impact of hypermobility on functional ability in daily life in adolescents. Prioritising the management of fatigue, cardiac dysautonomia and depression, where present, may alleviate more of the burden associated with this condition.
Implications
The total Spider score can be used by clinicians to indicate the magnitude of symptom burden of hypermobility in adolescents, whilst the domain scores detail how a person is impacted to facilitate more directed management. Where fatigue, cardiac dysautonomia and depression predominate, clinicians may anticipate a greater level of disability in daily life and prioritise interventions accordingly to prevent further decline.