Somatic symptoms, pain, catastrophizing and the association with disability among children with heritable connective tissue disorders

Lisanne E. de Koning, Jessica Warnink-Kavelaars, Marion A. van Rossum, Diederik Bosman, Leonie A. Menke, Fransiska Malfait, Rosa de Boer, Jaap Oosterlaan, Raoul H.H. Engelbert, Lies Rombaut

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Abstract

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.
Original languageEnglish
Pages (from-to)1792-1803
JournalAmerican Journal of Medical Genetics, Part A
Volume191
Issue number7
DOIs
Publication statusPublished - Jul 2023

Funding

We thank the parents, children, and adolescents who participated in this study. We are grateful to SIA RAAK‐PRO, part of the Dutch Organization for Scientific Research, for funding this project (NWO; SVB. RAAK>PRO02.007), which is part of a 5‐year research grant of the project “Follow You—a follow‐up program on physical, psychosocial functioning and participation in children and adolescents with (Heritable) Connective Tissue Disorders.” We also acknowledge the members of the Pediatric Heritable Connective Tissue Disorders study group: Marieke J.H. Baars, Eelco Dulfer, Yvonne Hilhorst‐Hofstee, Marlies J.E. Kempers, Ingrid P.C. Krapels, Bart L. Loeys, Ruth van der Looven, Laura Muiño Mosquera, Annelies van der Hulst, Femke Stoelinga as well as the Dutch Network Marfan and related disorders and both the Marfan and Ehlers–Danlos patient associations and the European Reference Network Skin—Mendelian Connective Tissue Disorders for the productive discussions.

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