{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,10]],"date-time":"2026-06-10T19:56:32Z","timestamp":1781121392808,"version":"3.54.1"},"reference-count":17,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2023,1,14]],"date-time":"2023-01-14T00:00:00Z","timestamp":1673654400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100004191","name":"Novo Nordisk","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100004191","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Haemophilia"],"published-print":{"date-parts":[[2023,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>In chronic diseases, disease\u2010related distress can impact disease outcomes. Distress and haemophilia\u2010related distress has been demonstrated in people with haemophilia (PwH). The association of haemophilia\u2010related distress on disease outcomes among PwH is unknown.<\/jats:p><\/jats:sec><jats:sec><jats:title>Aim<\/jats:title><jats:p>To study the association of haemophilia\u2010related distress with haemophilia specific outcomes, including adherence to prophylactic therapy, the presence of a target joint, healthcare utilization and work\u2010impairment.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>In a cross\u2010sectional study, adults with haemophilia A or B were enrolled in a study to validate the haemophilia\u2010related distress questionnaire (HRDq). In this planned analysis, univariate and multivariate associations between the HRDq total score and disease outcomes were explored.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The 114 participants in this analysis were male, mostly with haemophilia A (92%) and severe disease (52%) with a median age of 31.9 years. On univariate analysis, HRDq total score (5\u2010point change) was associated with the presence of a target joint (<jats:italic>P<\/jats:italic>\u00a0=\u00a0.002), high healthcare utilization (<jats:italic>P<\/jats:italic>\u00a0=\u00a0.011), poor adherence (<jats:italic>P<\/jats:italic>\u00a0=\u00a0.033) and work\u2010impairment (<jats:italic>P<\/jats:italic>\u00a0\u2264\u00a0.001). After adjustment for age, race, severity and other potential confounders, adherence (a\u03b2\u00a00.33, 95% CI .17, .49) and work\u2010impairment (a\u03b2\u00a04.69, 95% CI 3.27\u20106.1) remained statistically significantly associated with HRDq total score.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Haemophilia\u2010related distress is associated with poor adherence to factor prophylaxis and work\u2010impairment. The direction of the association (causation) is yet to be determined and requires future study.<\/jats:p><\/jats:sec>","DOI":"10.1111\/hae.14741","type":"journal-article","created":{"date-parts":[[2023,1,14]],"date-time":"2023-01-14T09:10:05Z","timestamp":1673687405000},"page":"505-512","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Cross\u2010sectional study evaluating the association of haemophilia\u2010related distress and clinically relevant outcomes"],"prefix":"10.1111","volume":"29","author":[{"given":"Sara","family":"Guasch","sequence":"first","affiliation":[{"name":"Department of Medicine Virginia Tech Carillion School of Medicine and Research Institute  Roanoke Virginia USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lia C.","family":"Scott","sequence":"additional","affiliation":[{"name":"Department of Hematology and Medical Oncology Emory University School of Medicine  Atlanta Georgia USA"},{"name":"Hemophilia of Georgia Center for Bleeding &amp; Clotting Disorders of Emory Emory University School of Medicine  Atlanta Georgia USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Janet","family":"Figueroa","sequence":"additional","affiliation":[{"name":"Pediatric Biostatistics Core, Department of Pediatrics Emory University School of Medicine  Atlanta Georgia USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1954-4385","authenticated-orcid":false,"given":"Tyler W.","family":"Buckner","sequence":"additional","affiliation":[{"name":"University of Colorado School of Medicine  Aurora Colorado USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5952-5512","authenticated-orcid":false,"given":"Shanna","family":"Mattis","sequence":"additional","affiliation":[{"name":"Department of Hematology and Medical Oncology Emory University School of Medicine  Atlanta Georgia USA"},{"name":"Hemophilia of Georgia Center for Bleeding &amp; Clotting Disorders of Emory Emory University School of Medicine  Atlanta Georgia USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"suffix":"Jr","given":"Duc Q.","family":"Tran","sequence":"additional","affiliation":[{"name":"Department of Hematology and Medical Oncology Emory University School of Medicine  Atlanta Georgia USA"},{"name":"Hemophilia of Georgia Center for Bleeding &amp; Clotting Disorders of Emory Emory University School of Medicine  Atlanta Georgia USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2391-2016","authenticated-orcid":false,"given":"Christine L.","family":"Kempton","sequence":"additional","affiliation":[{"name":"Department of Hematology and Medical Oncology Emory University School of Medicine  Atlanta Georgia USA"},{"name":"Hemophilia of Georgia Center for Bleeding &amp; Clotting Disorders of Emory Emory University School of Medicine  Atlanta Georgia USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2023,1,14]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.7249\/TL221"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1177\/1359105319842931"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1136\/jech-2013-203063"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10198-016-0785-2"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12748"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.13850"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.14187"},{"key":"e_1_2_8_9_1","unstructured":"AH.Department of Psychology Yale University;1975."},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1097\/00004583-200203000-00006"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2009.02129.x"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.2165\/00019053-199304050-00006"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.2147\/PPA.S175254"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12885-017-3260-2"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.14302"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1182\/bloodadvances.2018020552"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPH.0000000000001329"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.3111\/13696998.2015.1016228"}],"container-title":["Haemophilia"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/hae.14741","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/hae.14741","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/hae.14741","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,11,3]],"date-time":"2023-11-03T12:00:39Z","timestamp":1699012839000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/hae.14741"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,1,14]]},"references-count":17,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2023,3]]}},"alternative-id":["10.1111\/hae.14741"],"URL":"https:\/\/doi.org\/10.1111\/hae.14741","archive":["Portico"],"relation":{},"ISSN":["1351-8216","1365-2516"],"issn-type":[{"value":"1351-8216","type":"print"},{"value":"1365-2516","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,1,14]]},"assertion":[{"value":"2022-04-14","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-12-20","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-01-14","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}