The aim of the present study was to investigate the nature and prevalence of nonspecific somatic symptoms, pain and catastrophizing in children with Heritable Connective Tissue Disorders (HCTD), and to determine their association with disability. This observational, multicenter study included 127 children, aged 4–18 years, with Marfan syndrome (MFS) (59%), Loeys-Dietz syndrome (LDS) (8%), Ehlers-Danlos syndromes (EDS) (12%) and hypermobile Ehlers-Danlos syndrome (hEDS) (23%). The assessments included the Children's Somatization Inventory or parent proxy (CSI, PCSI), pain visual-analogue scale (VAS), SUPERKIDZ body diagram, Pain Catastrophizing Scale Child or parent proxy (PCS-C, PCS-P) and Childhood Health Assessment Questionnaire (CHAQ-30). Data from children aged ≥8 years were compared to normative data. In children ≥ 8 years (n = 90), pain was present in 59%, with a median of 4 (IQR = 3–9) pain areas. Compared to normative data, the HCTD group reported significantly higher on the CSI (p ≤ 0.001, d = 0.85), VAS pain intensity (p ≤ 0.001, d = 1.22) and CHAQ-30 (p ≤ 0.001, d = 1.16) and lower on the PCS-C (p = 0.017, d = −0.82) and PCS-P (p ≤ 0.001, d = −0.49). The intensity of nonspecific somatic symptoms and pain explained 45% of the variance in disability (r2 = 0.45 F(2,48) = 19.70, p ≤ 0.001). In children ≤ 7 years (n = 37), pain was present in 35% with a median of 5(IQR = 1–13) pain areas. The mean(SD) VAS scores for pain intensity was 1.5(2.9). Functional disability was moderately correlated to the number of pain areas (r = 0.56, p ≤ 0.001), intensity of nonspecific somatic symptoms (r = 0.63, p ≤ 0.001) and pain (r = 0.83, p ≤ 0.001). In conclusion, this study supports the need for comprehensive assessment of nonspecific somatic symptoms, pain, and disability in children with HCTD to allow tailored treatment.
OBJECTIVE:To develop a blended physiotherapeutic intervention for patients with non-specific low back pain (e-Exercise LBP) and evaluate its proof of concept.DESIGN:Focus groups with patients, physiotherapists, and eHealth and LBP experts were conducted to investigate values according to the development of e-Exercise LBP. Proof of concept was evaluated in a multicentre study.SETTING:Dutch primary care physiotherapy practices (n=21 therapists).PARTICIPANTS:Adults with non-specific LBP (n=41).INTERVENTION:e-Exercise LBP was developed based on clinical LBP guidelines and the focus groups, using the Center for eHealth Research Roadmap. Face-to-face physiotherapy sessions were integrated with a web application consisting of 12 information lessons, video-supported exercises and a physical activity module with the option to gradually increase individuals' level of physical activity. The intervention could be tailored to patients' risk of persistent disabling LBP, according to the STarT Back Screening Tool.MAIN OUTCOME MEASURES:Functional disability, pain, physical activity, sedentary behaviour and fear-avoidance beliefs, measured at baseline and 12 weeks.RESULTS:After 12 weeks, improvements were found in functional disability [Quebec Back Pain Disability Scale: mean difference (MD) -12.2/100; 95% confidence interval (CI) 8.3 to 16.1], pain (Numeric Pain Rating Scale: MD -2.8/10; 95% CI 2.1 to 3.6), subjective physical activity (Short Questionnaire to Assess Health Enhancing Physical Activity: MD 11.5minutes/day; 95% CI -47.8 to 24.8) and objective sedentary behaviour (ActiGraph: MD -23.0minutes/day; 95% CI -8.9 to 55.0). Small improvements were found in objective physical activity and fear-avoidance beliefs. The option to gradually increase physical activity was activated for six patients (15%). On average, patients received seven face-to-face sessions alongside the web application.CONCLUSIONS:The results of this study provide the first indication of the effectiveness of e-Exercise LBP, particularly for disability and pain among patients with LBP. Future studies will focus on end-user experiences and (cost-) effectiveness.KEYWORDS:Low back pain; Physiotherapy; Telemedicine; e-Health
Heritable Connective Tissue Disorders (HCTD) show an overlap in the physical features that can evolve in childhood. It is unclear to what extent children with HCTD experience burden of disease. This study aims to quantify fatigue, pain, disability and general health with standardized validated questionnaires.METHODS: This observational, multicenter study included 107 children, aged 4-18 years, with Marfan syndrome (MFS), 58%; Loeys-Dietz syndrome (LDS), 7%; Ehlers-Danlos syndromes (EDS), 8%; and hypermobile Ehlers-Danlos syndrome (hEDS), 27%. The assessments included PROMIS Fatigue Parent-Proxy and Pediatric self-report, pain and general health Visual-Analogue-Scales (VAS) and a Childhood Health Assessment Questionnaire (CHAQ).RESULTS: Compared to normative data, the total HCTD-group showed significantly higher parent-rated fatigue T-scores (M = 53 (SD = 12), p = 0.004, d = 0.3), pain VAS scores (M = 2.8 (SD = 3.1), p < 0.001, d = 1.27), general health VAS scores (M = 2.5 (SD = 1.8), p < 0.001, d = 2.04) and CHAQ disability index scores (M = 0.9 (SD = 0.7), p < 0.001, d = 1.23). HCTD-subgroups showed similar results. The most adverse sequels were reported in children with hEDS, whereas the least were reported in those with MFS. Disability showed significant relationships with fatigue (p < 0.001, rs = 0.68), pain (p < 0.001, rs = 0.64) and general health (p < 0.001, rs = 0.59).CONCLUSIONS: Compared to normative data, children and adolescents with HCTD reported increased fatigue, pain, disability and decreased general health, with most differences translating into very large-sized effects. This new knowledge calls for systematic monitoring with standardized validated questionnaires, physical assessments and tailored interventions in clinical care.
