Outcome measures for assessing change over time in studies of symptomatic children with hypermobility: a systematic review.

BACKGROUND: Generalised joint hypermobility (GJH) is highly prevalent among children and associated with symptoms in a fifth with the condition. This study aimed to synthesise outcome measures in interventional or prospective longitudinal studies of children with GJH and associated lower limb symptoms. METHODS: Electronic searches of Medline, CINAHL and Embase databases from inception to 16th March 2020 were performed for studies of children with GJH and symptoms between 5 and 18 years reporting repeated outcome measures collected at least 4 weeks apart. Methodological quality of eligible studies were described using the Downs and Black checklist. RESULTS: Six studies comprising of five interventional, and one prospective observational study (total of 388 children) met the inclusion criteria. Interventional study durations were between 2 and 3 months, with up to 10 months post-intervention follow-up, while the observational study spanned 3 years. Three main constructs of pain, function and quality of life were reported as primary outcome measures using 20 different instruments. All but one measure was validated in paediatric populations, but not specifically for children with GJH and symptoms. One study assessed fatigue, reporting disabling fatigue to be associated with higher pain intensity. CONCLUSIONS: There were no agreed sets of outcome measures used for children with GJH and symptoms. The standardisation of assessment tools across paediatric clinical trials is needed. Four constructs of pain, function, quality of life and fatigue are recommended to be included with agreed upon, validated, objective tools.

The Impact of Podiatric Intervention on the Quality of Life and Pain in Children and Adolescents with Hypermobility.

The purpose of this study was to evaluate the effect of custom-made orthotics on pain, health-related quality of life (HRQoL), function and fatigue in children and adolescents with generalised joint hypermobility (GJH) and lower limb pain. Fifty-three children aged 5-18 years were fitted with custom-made polypropylene orthotics. Visual analogue scale (VAS) assessed lower limb pain severity, Paediatric Quality of Life Inventory assessed HRQoL and fatigue and six-minute walk test (6 MWT) measured functional endurance at baseline, at 1 month and 3 months post-intervention. A mixed model including a random intercept for participant and a fixed effect for time was used to assess differences in outcomes over time. Fifty-two children completed the study (mean age 10.6-years). Children reported significantly reduced pain (mean VAS reduction -27/100, 95%CI: -33, -21), improved HRQoL (mean total improvement 11/100, 95%CI: 7, -15), functional capacity (mean 6MWT improvement 27 m, 95%CI: 18, -36) and fatigue (mean total improvement 13/100, 95%CI: 9, -17) after 1 month of wearing the custom-made orthotics. From 1 month to 3 months there was further statistically but not clinically significant reduction in pain while benefit on other outcomes was maintained. In this study, children with GJH reported reduced lower limb pain, improved HRQoL, functional endurance and fatigue after a month post-fitting of custom-made orthotics which was maintained over a 3 month period. Orthotics were well-tolerated with no serious adverse events reported.

Validation of an Electronic Visual Analog Scale App for Pain Evaluation in Children and Adolescents With Symptomatic Hypermobility: Cross-sectional Study.

BACKGROUND: Rapid advances in mobile apps for clinical data collection for pain evaluation have resulted in more efficient data handling and analysis than traditional paper-based approaches. As paper-based visual analogue scale (p-VAS) scores are commonly used to assess pain levels, new emerging apps need to be validated prior to clinical application with symptomatic children and adolescents. OBJECTIVE: This study aimed to assess the validity and reliability of an electronic visual analogue scale (e-VAS) method via a mobile health (mHealth) App in children and adolescents diagnosed with hypermobility spectrum disorder/hypermobile Ehlers-Danlos syndrome (HSD/HEDS) in comparison with the traditional p-VAS. METHODS: Children diagnosed with HSD/HEDS aged 5-18 years were recruited from a sports medicine center in Sydney (New South Wales, Australia). Consenting participants assigned in random order to the e-VAS and p-VAS platforms were asked to indicate their current lower limb pain level and completed pain assessment e-VAS or p-VAS at one time point. Instrument agreement between the 2 methods was determined from the intraclass correlation coefficient (ICC) and through Bland-Altman analysis. RESULTS: In total, 43 children with HSD/HEDS aged 11 (SD 3.8) years were recruited and completed this study. The difference between the 2 VAS platforms of median values was 0.20. Bland-Altman analysis revealed a difference of 0.19 (SD 0.95) with limits of agreement ranging -1.67 to 2.04. An ICC of 0.87 (95% CI 0.78-0.93) indicated good reliability. CONCLUSIONS: These findings suggest that the e-VAS mHealth App is a validated tool and a feasible method of collecting pain recording scores when compared with the traditional paper format in children and adolescents with HSD/HEDS. The e-VAS App can be reliably used for pediatric pain evaluation, and it could potentially be introduced into daily clinical practice to improve real-time symptom monitoring. Further research is warranted to investigate the usage of the app for remote support in real clinical settings.