{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,22]],"date-time":"2026-07-22T23:34:17Z","timestamp":1784763257646,"version":"3.55.0"},"reference-count":21,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2023,12,8]],"date-time":"2023-12-08T00:00:00Z","timestamp":1701993600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Internal Medicine Journal"],"published-print":{"date-parts":[[2024,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Sickle cell disease (SCD) is the most common monogenic disorder worldwide. In deoxygenated conditions, the altered beta chain (haemoglobin S [HbS]) polymerises and distorts the erythrocyte, resulting in pain crises, vasculopathy and end\u2010organ damage. Clinical complications of SCD cause substantial morbidity, and therapy demands expertise and resources. Optimising care for patients and planning resource allocation for the future requires an understanding of the disease in the Australian population. The Australian Haemoglobinopathy Registry (HbR) is a collaborative initiative of specialist centres collating and analysing data on patients with haemoglobin disorders.<\/jats:p><\/jats:sec><jats:sec><jats:title>Aims<\/jats:title><jats:p>To provide a snapshot of SCD in Australia over a 12\u2010month period based on data from the HbR.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Patients with a clinically significant sickling disorder across 12 clinical sites were included for analysis. Data include demographic and diagnostic details, as well as details of the clinical management of the condition over a 12\u2010month period.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Data on 359 SCD patients demonstrate a shift in the demographic of patients in Australia, with a growing proportion of sub\u2010Saharan African ethnicities associated with the HbSS genotype. Acute and chronic complications are common, and patients require significant outpatient and inpatient support. Prevalence of disease complications and therapeutic trends are in keeping with other high\u2010income countries.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>This study provides the first national picture of SCD in Australia, describing the characteristics and needs of SCD patients, elucidating demand for current and novel therapy and facilitating the planning of services for this vulnerable population.<\/jats:p><\/jats:sec>","DOI":"10.1111\/imj.16297","type":"journal-article","created":{"date-parts":[[2023,12,8]],"date-time":"2023-12-08T18:48:35Z","timestamp":1702061315000},"page":"764-772","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Sickle cell disease in Australia: a snapshot from the Australian Haemoglobinopathy Registry"],"prefix":"10.1111","volume":"54","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7589-5564","authenticated-orcid":false,"given":"Anna","family":"Nelson","sequence":"first","affiliation":[{"name":"Transfusion Research Unit, School of Public Health and Preventive Medicine Monash University  Melbourne Victoria Australia"},{"name":"Institute of Haematology Royal Prince Alfred Hospital  Sydney New South Wales Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P. Joy","family":"Ho","sequence":"additional","affiliation":[{"name":"Institute of Haematology Royal Prince Alfred Hospital  Sydney New South Wales Australia"},{"name":"Faculty of Medicine and Health University of Sydney  Sydney New South Wales Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Helen","family":"Haysom","sequence":"additional","affiliation":[{"name":"Transfusion Research Unit, School of Public Health and Preventive Medicine Monash University  Melbourne Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Neil","family":"Waters","sequence":"additional","affiliation":[{"name":"Transfusion Research Unit, School of Public Health and Preventive Medicine Monash University  Melbourne Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cameron","family":"Wellard","sequence":"additional","affiliation":[{"name":"Transfusion Research Unit, School of Public Health and Preventive Medicine Monash University  Melbourne Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Melissa","family":"Chee","sequence":"additional","affiliation":[{"name":"Transfusion Research Unit, School of Public Health and Preventive Medicine Monash University  Melbourne Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juliana","family":"Teo","sequence":"additional","affiliation":[{"name":"Department of Haematology Children's Hospital Westmead  Sydney New South Wales Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anthea","family":"Greenway","sequence":"additional","affiliation":[{"name":"Department of Haematology Royal Children's Hospital  Melbourne Victoria Australia"},{"name":"Department of Clinical Haematology Monash Health  Melbourne Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kylie","family":"Mason","sequence":"additional","affiliation":[{"name":"Department of Haematology The Royal Melbourne Hospital  Melbourne Victoria Australia"},{"name":"Department of Clinical Haematology Peter MacCallum Cancer Centre  Melbourne Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giselle","family":"Kidson\u2010Gerber","sequence":"additional","affiliation":[{"name":"Department of Haematology Prince of Wales Hospital  Sydney New South Wales Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zane","family":"Kaplan","sequence":"additional","affiliation":[{"name":"Department of Clinical Haematology Monash Health  Melbourne Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tina","family":"Carter","sequence":"additional","affiliation":[{"name":"Department of Haematology Perth Children's Hospital  Perth Western Australia Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Merrole F","family":"Cole\u2010Sinclair","sequence":"additional","affiliation":[{"name":"Department of Haematology St Vincent's Hospital  Melbourne Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pasquale","family":"Barbaro","sequence":"additional","affiliation":[{"name":"Department of Haematology Queensland Children's Hospital  Brisbane Queensland Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7527-2340","authenticated-orcid":false,"given":"Erica M.","family":"Wood","sequence":"additional","affiliation":[{"name":"Transfusion Research Unit, School of Public Health and Preventive Medicine Monash University  Melbourne Victoria Australia"},{"name":"Department of Clinical Haematology Monash Health  Melbourne Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"the Haemoglobinopathy Registry investigators","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2023,12,8]]},"reference":[{"key":"e_1_2_7_2_1","unstructured":"Sickle Cell Disease. 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