{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T16:38:20Z","timestamp":1790699900521,"version":"4.1.0"},"reference-count":25,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2024,4,19]],"date-time":"2024-04-19T00:00:00Z","timestamp":1713484800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Rehabil. Sci."],"abstract":"<jats:sec><jats:title>Background<\/jats:title><jats:p>The Ehlers-Danlos syndromes (EDS) are a group of inherited connective tissue disorders characterized by disruptions in collagen synthesis and processing. These disorders lead to various symptoms, including hypermobility, musculoskeletal conditions, and chronic pain that can significantly limit patients' daily living. In the absence of a curative treatment, an EDS specific disability index that tracks changes in patient-reported outcomes can facilitate the investigation of new treatment options and enhance the quality of life for EDS patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>An EDS-specific disability index was created using survey data and input from clinicians. A total of 222 EDS patients in a multidisciplinary clinical program completed the index during their initial visit. Exploratory and confirmatory factor analyses were conducted to determine the index's factor solution and assess its goodness-of-fit. Paired <jats:italic>t<\/jats:italic>-tests were performed with follow-up visit data collected over the course of one year.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The exploratory and confirmatory factor analyses indicated a two-factor solution, accounting for 42.40% of the variance. The index demonstrated adequate fit to the data, supported by Tucker and Lewis's index (0.85) and root mean square error of approximation (0.1). Data from follow-up visits showed significant improvement in three symptom related variables and one function related variable in addition to the total score and the symptom subscale score when compared to the initial visit.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>The development of an EDS-specific disability index is a crucial step in creating a clinical tool that enables healthcare professionals to gain a deeper understanding of the impact EDS has on patients\u2019 lives and potentially identify new therapeutic interventions.<\/jats:p><\/jats:sec>","DOI":"10.3389\/fresc.2024.1280582","type":"journal-article","created":{"date-parts":[[2024,4,19]],"date-time":"2024-04-19T04:37:05Z","timestamp":1713501425000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":1,"title":["Assessment of a novel Ehlers-Danlos syndromes disability index"],"prefix":"10.3389","volume":"5","author":[{"given":"Stephen","family":"Chai","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patricia","family":"Roney","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John","family":"Fagan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emily Rose","family":"Rosario","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2024,4,19]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"329","DOI":"10.1136\/ard.2004.022137","article-title":"Combating the burden of musculoskeletal conditions","volume":"63","author":"Smolen","year":"2004","journal-title":"Ann Rheum Dis"},{"key":"B2","first-page":"6","article-title":"The bone and joint decade. Strategies to reduce the burden of disease: the bone and joint monitor project","volume":"67","author":"Woolf","year":"2003","journal-title":"J Rheumatol Suppl"},{"key":"B3","doi-asserted-by":"publisher","first-page":"8","DOI":"10.1002\/ajmg.c.31552","article-title":"The 2017 international classification of the ehlers-danlos syndromes","volume":"175","author":"Malfait","year":"2017","journal-title":"Am J Med Genet"},{"key":"B4","doi-asserted-by":"publisher","first-page":"1657","DOI":"10.1111\/nmo.12665","article-title":"Ehlers Danlos syndrome and gastrointestinal manifestations: a 20-year experience at mayo clinic","volume":"27","author":"Nelson","year":"2015","journal-title":"Neurogastroenterol Motil"},{"key":"B5","doi-asserted-by":"publisher","first-page":"556","DOI":"10.1002\/ajmg.a.33231","article-title":"Natural history and manifestations of the hypermobility type Ehlers-Danlos syndrome: a pilot study on 21 patients","author":"Castori","year":"2010","journal-title":"Am J Med Genet A"},{"key":"B6","doi-asserted-by":"publisher","first-page":"1194","DOI":"10.1093\/rheumatology\/keh279","article-title":"Non-musculoskeletal symptoms in joint hypermobility syndrome. Indirect evidence for autonomic dysfunction?","volume":"43","author":"Hakim","year":"2004","journal-title":"Rheumatology (Oxford)"},{"key":"B7","doi-asserted-by":"publisher","first-page":"1","DOI":"10.3109\/09638280903178407","article-title":"Dignity not fully upheld when seeking health care: experiences expressed by individuals suffering from Ehlers\u2013Danlos syndrome","volume":"32","author":"Berglund","year":"2010","journal-title":"Disabil Rehabil"},{"key":"B8","doi-asserted-by":"publisher","first-page":"354","DOI":"10.1093\/fampra\/cmv026","article-title":"Living with joint hypermobility syndrome: patient experiences of diagnosis, referral and self-care","volume":"32","author":"Terry","year":"2015","journal-title":"Fam Pract"},{"key":"B9","doi-asserted-by":"publisher","first-page":"429","DOI":"10.36076\/ppj.2020\/23\/429","article-title":"Ehlers-Danlos syndrome: an analysis of the current treatment options","volume":"23","author":"Song","year":"2020","journal-title":"Pain Phys"},{"key":"B10","doi-asserted-by":"publisher","first-page":"2986","DOI":"10.1002\/ajmg.a.62393","article-title":"Physical therapy treatment of hypermobile Ehlers-Danlos syndrome: a systematic review","volume":"185","author":"Reychler","year":"2021","journal-title":"Am J Med Genet A"},{"key":"B11","doi-asserted-by":"publisher","first-page":"838","DOI":"10.1016\/j.jval.2017.05.015","article-title":"Patient-reported outcome and observer-reported outcome assessment in rare disease clinical trials: an ISPOR COA emerging good practices task force report","volume":"20","author":"Benjamin","year":"2017","journal-title":"Value Health"},{"key":"B12","author":"Sabino","year":""},{"key":"B13","doi-asserted-by":"publisher","first-page":"1309","DOI":"10.1056\/NEJMp1707537","article-title":"Making patients and doctors happier \u2014 the potential of patient-reported outcomes","volume":"377","author":"Rotenstein","year":"2017","journal-title":"N Engl J Med"},{"key":"B14","year":""},{"key":"B15","doi-asserted-by":"publisher","first-page":"186","DOI":"10.1016\/j.physio.2016.04.002","article-title":"Development and initial validation of the Bristol impact of hypermobility questionnaire","volume":"103","author":"Palmer","year":"2017","journal-title":"Physiotherapy"},{"key":"B16","doi-asserted-by":"publisher","first-page":"64","DOI":"10.1016\/j.msksp.2017.08.007","article-title":"Test-retest reliability and smallest detectable change of the Bristol impact of hypermobility (BIoH) questionnaire","volume":"32","author":"Palmer","year":"2017","journal-title":"Musculoskelet Sci Pract"},{"key":"B17","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1080\/10705519909540118","article-title":"Cutoff criteria for fit indexes in covariance structure analysis: conventional criteria versus new alternatives","volume":"6","author":"Hu","year":"1999","journal-title":"Struct Equ Modeling"},{"key":"B18","doi-asserted-by":"publisher","first-page":"265","DOI":"10.1037\/0033-2909.103.2.265","article-title":"Relation of sample size to the stability of component patterns","volume":"103","author":"Guadagnoli","year":"1988","journal-title":"Psychol Bull"},{"key":"B19","doi-asserted-by":"publisher","first-page":"803898","DOI":"10.3389\/fpsyt.2021.803898","article-title":"Ehlers-Danlos syndrome in the field of psychiatry: a review","volume":"12","author":"Ishiguro","year":"2022","journal-title":"Front Psychiatry"},{"key":"B20","doi-asserted-by":"publisher","first-page":"189","DOI":"10.1080\/09638288.2020.1761890","article-title":"The most common comorbidities in patients with Ehlers-Danlos syndrome: a 15-year nationwide population-based cohort study","volume":"44","author":"Leganger","year":"2022","journal-title":"Disabil Rehabil"},{"key":"B21","doi-asserted-by":"publisher","first-page":"273","DOI":"10.1007\/s12016-019-08755-8","article-title":"The relationship between hypermobile Ehlers-Danlos syndrome (hEDS), postural orthostatic tachycardia syndrome (POTS), and mast cell activation syndrome (MCAS)","volume":"58","author":"Kohn","year":"2020","journal-title":"Clinic Rev Allerg Immunol"},{"key":"B22","doi-asserted-by":"publisher","first-page":"158","DOI":"10.1002\/ajmg.c.31545","article-title":"The evidence-based rationale for physical therapy treatment of children, adolescents, and adults diagnosed with joint hypermobility syndrome\/hypermobile Ehlers Danlos syndrome","volume":"175","author":"Engelbert","year":"2017","journal-title":"Am J Med Genet"},{"key":"B23","doi-asserted-by":"publisher","first-page":"1123","DOI":"10.1080\/09638288.2020.1788177","article-title":"Assessing reliability and validity of a functional outcome measure for adolescents with hypermobility spectrum disorder","volume":"44","author":"Shotwell","year":"2022","journal-title":"Disabil Rehabil"},{"key":"B24","doi-asserted-by":"publisher","first-page":"630","DOI":"10.1080\/01942638.2022.2163601","article-title":"Pain characteristics and symptom management in children with hypermobile Ehlers-Danlos syndrome and hypermobility spectrum disorder","volume":"43","author":"Jeong","year":"2023","journal-title":"Phys Occup Ther Pediatr"},{"key":"B25","doi-asserted-by":"publisher","first-page":"1713","DOI":"10.1007\/s11136-014-0632-9","article-title":"Getting serious about test-retest reliability: a critique of retest research and some recommendations","volume":"23","author":"Polit","year":"2014","journal-title":"Qual Life Res"}],"container-title":["Frontiers in Rehabilitation Sciences"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fresc.2024.1280582\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,5,16]],"date-time":"2024-05-16T14:11:15Z","timestamp":1715868675000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fresc.2024.1280582\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,4,19]]},"references-count":25,"alternative-id":["10.3389\/fresc.2024.1280582"],"URL":"https:\/\/doi.org\/10.3389\/fresc.2024.1280582","relation":{},"ISSN":["2673-6861"],"issn-type":[{"value":"2673-6861","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,4,19]]},"article-number":"1280582"}}