{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T04:45:45Z","timestamp":1787028345636,"version":"build-2736575974"},"reference-count":55,"publisher":"MDPI AG","issue":"11","license":[{"start":{"date-parts":[[2022,5,26]],"date-time":"2022-05-26T00:00:00Z","timestamp":1653523200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"University Grant Committee of Hong Kong, Research Grant Council","award":["14602620"],"award-info":[{"award-number":["14602620"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJERPH"],"abstract":"<jats:p>Background: This study aims to identify factors affecting health-related quality of life (HRQoL) in Chinese patients with hemophilia in Hong Kong, and to examine the association between treatment adherence and HRQoL outcomes. Methods: Patients with hemophilia A or B from a non-governmental organization reported their HRQoL and treatment adherence to prophylactic therapy using validated tools. Univariate tests and multivariable regression analysis were used to compare differences in outcomes across clinically relevant subgroups. Results: Fifty-six patients were recruited (mean age 30.4 [17.4] years; majority hemophilia A: 75%; moderate-to-severe severity: 88%). Patients who received prophylactic treatment reported fewer work\/school problems (25.8 [18.9] versus 51.5 [26.3]; p = 0.001) than those who received on-demand therapy. The multivariable model showed that older age (B = 0.42, 95% CI = 0.093\u20130.75) and living in public housing (B = 10.24, 95% CI = 0.70\u201319.77) were associated with worse HRQoL. Older age was associated with treatment non-adherence (r = 0.66, p &lt; 0.0001). Patients with poor adherence tended to report worse functioning in sports\/leisure (r = 0.31, p = 0.033). Conclusions: Our results suggest that patients who were older, had lower education attainment and received on-demand treatment had poorer perception of their health. Improving adherence may lead to better HRQoL. Future work includes evaluating the occupational needs prospectively in this population.<\/jats:p>","DOI":"10.3390\/ijerph19116496","type":"journal-article","created":{"date-parts":[[2022,5,27]],"date-time":"2022-05-27T07:05:07Z","timestamp":1653635107000},"page":"6496","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Treatment Adherence and Health-Related Quality of Life in Patients with Hemophilia in Hong Kong"],"prefix":"10.3390","volume":"19","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9874-8938","authenticated-orcid":false,"given":"Yin Ting","family":"Cheung","sequence":"first","affiliation":[{"name":"School of Pharmacy, Faculty of Medicine, The Chinese University of Hong Kong, Hong Kong, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pok Hong","family":"Lam","sequence":"additional","affiliation":[{"name":"Department of Paediatrics, Faculty of Medicine, The Chinese University of Hong Kong, Hong Kong, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Henry Hon Wai","family":"Lam","sequence":"additional","affiliation":[{"name":"Hong Kong Haemophilia Society, Hong Kong, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chung-Tin","family":"Ma","sequence":"additional","affiliation":[{"name":"School of Pharmacy, Faculty of Medicine, The Chinese University of Hong Kong, Hong Kong, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alex Wing Kwan","family":"Leung","sequence":"additional","affiliation":[{"name":"Department of Paediatrics, Faculty of Medicine, The Chinese University of Hong Kong, Hong Kong, China"},{"name":"Department of Paediatrics & Adolescent Medicine, Hong Kong Children\u2019s Hospital, Hong Kong, China"},{"name":"Hong Kong Hub of Paediatric Excellence, The Chinese University of Hong Kong, Hong Kong, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9011-4268","authenticated-orcid":false,"given":"Raymond Siu Ming","family":"Wong","sequence":"additional","affiliation":[{"name":"Department of Medicine and Therapeutics, Faculty of Medicine, The Chinese University of Hong Kong, Hong Kong, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2810-5758","authenticated-orcid":false,"given":"Chi Kong","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Paediatrics, Faculty of Medicine, The Chinese University of Hong Kong, Hong Kong, China"},{"name":"Department of Paediatrics & Adolescent Medicine, Hong Kong Children\u2019s Hospital, Hong Kong, China"},{"name":"Hong Kong Hub of Paediatric Excellence, The Chinese University of Hong Kong, Hong Kong, China"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,5,26]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1447","DOI":"10.1016\/S0140-6736(11)61139-2","article-title":"Modern haemophilia care","volume":"379","author":"Berntorp","year":"2012","journal-title":"Lancet"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"187","DOI":"10.1016\/S0140-6736(15)01123-X","article-title":"The past and future of haemophilia: Diagnosis, treatments, and its complications","volume":"388","author":"Peyvandi","year":"2016","journal-title":"Lancet"},{"key":"ref_3","unstructured":"Centers for Disease Control and Prevention. United States (2022, May 17). Data & Statistics on Hemophilia. National Center on Birth Defects and Developmental Disabilities, Available online: https:\/\/www.cdc.gov\/ncbddd\/hemophilia\/data.html."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"51","DOI":"10.15256\/joc.2011.1.2","article-title":"Haemophilia and Joint Disease: Pathophysiology, Evaluation, and Management","volume":"1","author":"Knobe","year":"2011","journal-title":"J. Comorbidity"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"7894","DOI":"10.4081\/hr.2019.7894","article-title":"Health Status and Quality of Life in Patients with Severe Hemophilia A: A Cross-Sectional Survey","volume":"11","author":"Davari","year":"2019","journal-title":"Hematol. Rep."},{"key":"ref_6","doi-asserted-by":"crossref","unstructured":"Baek, H.J., Park, Y.S., Yoo, K.Y., Cha, J.-H., Kim, Y.-J., and Lee, K.S. (2020). Health-related quality of life of moderate and severe haemophilia patients: Results of the haemophilia-specific quality of life index in Korea. PLoS ONE, 15.","DOI":"10.1371\/journal.pone.0238686"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"335","DOI":"10.1016\/j.htct.2019.03.010","article-title":"Evaluation of quality of life in hemophilia patients using the WHOQOL-bref and Haemo-A-Qol questionnaires","volume":"41","author":"Trindade","year":"2019","journal-title":"Hematol. Transfus. Cell Ther."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"845","DOI":"10.1097\/MBC.0000000000000571","article-title":"Pain and pain management in haemophilia","volume":"27","author":"Auerswald","year":"2016","journal-title":"Blood Coagul. Fibrinolysis"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"28915","DOI":"10.3402\/qhw.v10.28915","article-title":"Shared topics on the experience of people with haemophilia living in the UK and the USA and the influence of individual and contextual variables: Results from the HERO qualitative study","volume":"10","author":"Palareti","year":"2015","journal-title":"Int. J. Qual. Stud. Health Well-Being"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"e056668","DOI":"10.1136\/bmjopen-2021-056668","article-title":"Health-related quality of life of patients with haemophilia: A cross-sectional survey in the Northeast of China","volume":"12","author":"Niu","year":"2022","journal-title":"BMJ Open"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"243","DOI":"10.1111\/hae.13945","article-title":"Assessment of self-\/parent-reported quality of life in Japanese children with haemophilia using the Japanese version of KIDSCREEN-52","volume":"26","author":"Furuichi","year":"2020","journal-title":"Haemophilia"},{"key":"ref_12","first-page":"178","article-title":"The modern treatment of haemophilia: A narrative review","volume":"11","author":"Franchini","year":"2013","journal-title":"Blood Transfus."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"44","DOI":"10.1046\/j.1365-2516.2002.00581.x","article-title":"Quality-of-life differences between prophylactic and on-demand factor replacement therapy in European haemophilia patients","volume":"8","author":"Royal","year":"2002","journal-title":"Haemophilia"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1111\/hae.14046","article-title":"WFH Guidelines for the Management of Hemophilia, 3rd edition","volume":"26","author":"Srivastava","year":"2020","journal-title":"Haemophilia"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"1677","DOI":"10.2147\/PPA.S139851","article-title":"Treatment adherence in hemophilia","volume":"11","author":"Thornburg","year":"2017","journal-title":"Patient Prefer. Adherence"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"S346","DOI":"10.1016\/j.amepre.2011.09.003","article-title":"Self-Reported Barriers to Hemophilia Care in People with Factor VIII Deficiency","volume":"41","author":"Zhou","year":"2011","journal-title":"Am. J. Prev. Med."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"1500","DOI":"10.1007\/s11096-021-01270-x","article-title":"Association between reported medication adherence and health-related quality of life in adult patients with haemophilia","volume":"43","author":"Bago","year":"2021","journal-title":"Int. J. Clin. Pharm."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"493","DOI":"10.1111\/j.1365-2516.2007.01526.x","article-title":"Quantifying adherence to treatment and its relationship to quality of life in a well-characterized haemophilia population","volume":"13","author":"Rice","year":"2007","journal-title":"Haemophilia"},{"key":"ref_19","first-page":"189","article-title":"A synopsis of current haemophilia care in Hong Kong","volume":"17","author":"Au","year":"2011","journal-title":"Hong Kong Med. J."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"e51","DOI":"10.1111\/hae.14217","article-title":"An overview of patients with haemophilia A in China: Epidemiology, disease severity and treatment strategies","volume":"27","author":"Song","year":"2021","journal-title":"Haemophilia"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1111\/j.1365-2516.2009.02131.x","article-title":"A study of reported factor VIII use around the world","volume":"16","author":"Stonebraker","year":"2010","journal-title":"Haemophilia"},{"key":"ref_22","unstructured":"The Government of the Hong Kong SAR (2021, October 08). Press Releases. LCQ21: Rare Diseases, Available online: https:\/\/www.info.gov.hk\/gia\/general\/202102\/24\/P2021022400468.htm."},{"key":"ref_23","first-page":"A127","article-title":"Assessment of health-related quality of life in patients with haemophilia with the newly developed haemophilia-specific instrument Haem-A-QoL","volume":"8","author":"Scalone","year":"2005","journal-title":"Value Health"},{"key":"ref_24","first-page":"P0813","article-title":"Health-related Quality of Life in Adult Patients with Haemophilia\u2014Assessment with a New Disease-specific Questionnaire (Haem-A-QoL)","volume":"3","author":"Gringeri","year":"2005","journal-title":"J. Thromb. Haemost."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1111\/j.1355-0691.2004.00875.x","article-title":"Development and testing of an instrument to assess the Quality of Life of Children with Haemophilia in Europe (Haemo-QoL)","volume":"10","author":"Bullinger","year":"2004","journal-title":"Haemophilia"},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"47","DOI":"10.1046\/j.1351-8216.2001.114.doc.x","article-title":"Pilot testing of the \u2018Haemo-QoL\u2019 quality of life questionnaire for haemophiliac children in six European countries","volume":"8","author":"Bullinger","year":"2002","journal-title":"Haemophilia"},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"643","DOI":"10.1111\/j.1365-2516.2006.01346.x","article-title":"Comparing two measures of quality of life for children with haemophilia: The CHO-KLAT and the Haemo-QoL","volume":"12","author":"Bradley","year":"2006","journal-title":"Haemophilia"},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"383","DOI":"10.1111\/hae.13140","article-title":"Measurement properties of the Haem-A-QoL in haemophilia clinical trials","volume":"23","author":"Auguste","year":"2017","journal-title":"Haemophilia"},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"547","DOI":"10.1007\/s10198-021-01273-5","article-title":"Evaluating the psychometric properties of the EQ-5D-5L and SF-6D among patients with haemophilia","volume":"22","author":"Xu","year":"2021","journal-title":"Eur. J. Health Econ."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"247","DOI":"10.1111\/j.1365-2516.2009.02129.x","article-title":"VERITAS-Pro: A new measure of adherence to prophylactic regimens in haemophilia","volume":"16","author":"Duncan","year":"2010","journal-title":"Haemophilia"},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"64","DOI":"10.1111\/hae.12533","article-title":"Adherence to prophylaxis is associated with better outcomes in moderate and severe haemophilia: Results of a patient survey","volume":"21","author":"Krishnan","year":"2015","journal-title":"Haemophilia"},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"S53","DOI":"10.1016\/j.thromres.2013.10.012","article-title":"Relationship between haemophilia and social status","volume":"134","author":"Holstein","year":"2014","journal-title":"Thromb. Res."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"1273","DOI":"10.1007\/s11165-016-9602-2","article-title":"The Use of Cronbach\u2019s Alpha When Developing and Reporting Research Instruments in Science Education","volume":"48","author":"Taber","year":"2018","journal-title":"Res. Sci. Educ."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"26","DOI":"10.1111\/j.1355-0691.2004.00876.x","article-title":"Health status and health-related quality of life of children with haemophilia from six West European countries","volume":"10","author":"Gringeri","year":"2004","journal-title":"Haemophilia"},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"382","DOI":"10.1111\/hae.13731","article-title":"Health-related quality of life and health status in persons with haemophilia A with inhibitors: A prospective, multicentre, non-interventional study (NIS)","volume":"25","author":"Mahlangu","year":"2019","journal-title":"Haemophilia"},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"449","DOI":"10.3109\/08880018.2010.489933","article-title":"Hemophilia-Specific Quality of Life Index (Haemo-QoL and Haem-A-QoL questionnaires) of children and adults: Result of a single center from Turkey","volume":"27","author":"Mercan","year":"2010","journal-title":"Pediatr. Hematol. Oncol."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"S3","DOI":"10.1002\/ajh.24220","article-title":"Treatment outcomes, quality of life, and impact of hemophilia on young adults (aged 18-30 years) with hemophilia","volume":"90","author":"Witkop","year":"2015","journal-title":"Am. J. Hematol."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"314","DOI":"10.5581\/1516-8484.20130108","article-title":"Health-related quality of life in hemophilia: Results of the hemophilia-Specific quality of life index (Haem-a-Qol) at a Brazilian blood center","volume":"35","author":"Ferreira","year":"2013","journal-title":"Rev. Bras. Hematol. Hemoter."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"28","DOI":"10.1186\/s12955-019-1083-3","article-title":"Health-related quality of life in children with haemophilia in China: A 4-year follow-up prospective cohort study","volume":"17","author":"Zhang","year":"2019","journal-title":"Health Qual. Life Outcomes"},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"356","DOI":"10.1016\/j.htct.2019.08.001","article-title":"The existing scenario of haemophilia care in Canada and China\u2014A review","volume":"42","author":"Pratap","year":"2020","journal-title":"Hematol. Transfus. Cell Ther."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"147","DOI":"10.1002\/rth2.12060","article-title":"Balance, falls, and exercise: Beliefs and experiences in people with hemophilia: A qualitative study","volume":"2","author":"Flaherty","year":"2017","journal-title":"Res. Pract. Thromb. Haemost."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"461","DOI":"10.1080\/21642850.2020.1823226","article-title":"Illness perceptions and their relation to physical activity in children and adolescents with hemophilia","volume":"8","author":"Amesse","year":"2020","journal-title":"Health Psychol. Behav. Med."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"487","DOI":"10.1111\/hae.12118","article-title":"The benefits of exercise for patients with haemophilia and recommendations for safe and effective physical activity","volume":"19","author":"Negrier","year":"2013","journal-title":"Haemophilia"},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"85","DOI":"10.2147\/JBM.S84848","article-title":"Strategies to encourage physical activity in patients with hemophilia to improve quality of life","volume":"7","author":"Goto","year":"2016","journal-title":"J. Blood Med."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"558","DOI":"10.1007\/s00508-019-01548-1","article-title":"Establishing an online physical exercise program for people with hemophilia","volume":"131","author":"Wagner","year":"2019","journal-title":"Wien. Klin. Wochenschr."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"3671","DOI":"10.1182\/blood.V116.21.3671.3671","article-title":"Complications of Aging In Patients with Hemophilia","volume":"116","author":"Sandhu","year":"2010","journal-title":"Blood"},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1186\/s13023-020-01423-7","article-title":"Medical expenditure for patients with hemophilia in urban China: Data from medical insurance information system from 2013 to 2015","volume":"15","author":"Gong","year":"2020","journal-title":"Orphanet J. Rare Dis."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"862","DOI":"10.1111\/hae.13621","article-title":"Adherence to prophylactic haemophilic treatment in young patients transitioning to adult care: A qualitative review","volume":"24","author":"Mortensen","year":"2018","journal-title":"Haemophilia"},{"key":"ref_49","doi-asserted-by":"crossref","unstructured":"Huang, Z., Nicholas, S., Yang, Y., Chen, X., Maitland, E., Ma, Y., and Shi, X. (2022). Medical costs and hospital utilization for hemophilia A and B urban inpatients in China: A national cross-sectional study. BMC Health Serv. Res., 22.","DOI":"10.1186\/s12913-022-07626-x"},{"key":"ref_50","first-page":"141","article-title":"Extended Half-Life Coagulation Factors: A New Era in the Management of Haemophilia Patients","volume":"36","author":"Ar","year":"2019","journal-title":"Turk. J. Hematol."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1111\/hae.13364","article-title":"Use of telehealth in the delivery of comprehensive care for patients with haemophilia and other inherited bleeding disorders","volume":"24","author":"Kulkarni","year":"2018","journal-title":"Haemophilia"},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"e12340","DOI":"10.2196\/12340","article-title":"Telehealth Interventions for Improving Self-Management in Patients With Hemophilia: Scoping Review of Clinical Studies","volume":"21","author":"Qian","year":"2019","journal-title":"J. Med. Internet Res."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"e27985","DOI":"10.2196\/27985","article-title":"Technology Acceptance Among Patients With Hemophilia in Hong Kong and Their Expectations of a Mobile Health App to Promote Self-management: Survey Study","volume":"5","author":"Cheung","year":"2021","journal-title":"JMIR Form. Res."},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"102779","DOI":"10.1016\/j.transci.2020.102779","article-title":"Patient blood management: The best approach to transfusion medicine risk management","volume":"59","author":"Bolcato","year":"2020","journal-title":"Transfus. Apher. Sci."},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"821","DOI":"10.1111\/hae.13811","article-title":"Comparing objective and self-reported measures of adherence in haemophilia","volume":"25","author":"Guedes","year":"2019","journal-title":"Haemophilia"}],"container-title":["International Journal of Environmental Research and Public Health"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/1660-4601\/19\/11\/6496\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T23:19:21Z","timestamp":1760138361000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/1660-4601\/19\/11\/6496"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,5,26]]},"references-count":55,"journal-issue":{"issue":"11","published-online":{"date-parts":[[2022,6]]}},"alternative-id":["ijerph19116496"],"URL":"https:\/\/doi.org\/10.3390\/ijerph19116496","relation":{},"ISSN":["1660-4601"],"issn-type":[{"value":"1660-4601","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,5,26]]}}}