{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,27]],"date-time":"2026-02-27T01:34:13Z","timestamp":1772156053880,"version":"3.50.1"},"reference-count":22,"publisher":"The Royal Society","issue":"11","license":[{"start":{"date-parts":[[2024,11,1]],"date-time":"2024-11-01T00:00:00Z","timestamp":1730419200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2024,11,1]],"date-time":"2024-11-01T00:00:00Z","timestamp":1730419200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100009076","name":"Capgemini","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100009076","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Huntington Disease Association"}],"content-domain":{"domain":["royalsocietypublishing.org"],"crossmark-restriction":true},"short-container-title":["R. Soc. Open Sci."],"published-print":{"date-parts":[[2024,11]]},"abstract":"<jats:p>We develop a Huntington\u2019s disease (HD) progression model and integrate this with a novel economic model, accounting for the major factors of the HD\u2019s societal cost. Data from the Enroll-HD observational study were used to fit a continuous-time hidden Markov disease progression model, which identified five distinct states. The number of disease states was determined using a cross-validated maximum likelihood approach. A novel data augmentation method was used to correct the biased life expectancy of the progression model. Multiple sources of cost data were then mapped to Enroll-HD variables using expert experience. A simulation of a synthetic patient population was used to show the feasibility of the approach in estimating population costs and the impact of hypothetical intervention scenarios. Our results confirm that early cognitive decline, which is not captured by the total functional capacity score currently used by clinicians but flagged up in HD integrated staging system, can be quantified from participants\u2019 visits. Finally, the results of the UK cost modelling show that indirect costs of HD such as state benefits and lost gross domestic product contribution could be the driving factors for the societal cost, over and above health and social care costs.<\/jats:p>","DOI":"10.1098\/rsos.240824","type":"journal-article","created":{"date-parts":[[2024,11,19]],"date-time":"2024-11-19T19:05:47Z","timestamp":1732043147000},"update-policy":"https:\/\/doi.org\/10.1098\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Data-driven Huntington\u2019s disease progression modelling and estimation of societal cost in the UK"],"prefix":"10.1098","volume":"11","author":[{"given":"Andrew","family":"Pollard","sequence":"first","affiliation":[{"name":"Hybrid Intelligence, Capgemini Engineering, Stevenage, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3822-7908","authenticated-orcid":true,"given":"Danica","family":"Greetham","sequence":"additional","affiliation":[{"name":"Hybrid Intelligence, Capgemini Engineering, Stevenage, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"James","family":"Myatt","sequence":"additional","affiliation":[{"name":"Hybrid Intelligence, Capgemini Engineering, Stevenage, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hugh","family":"Rickards","sequence":"additional","affiliation":[{"name":"Huntington\u2019s Disease Association (England and Wales), Liverpool, UK"},{"name":"University of Birmingham, Birmingham, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cath","family":"Stanley","sequence":"additional","affiliation":[{"name":"Huntington\u2019s Disease Association (England and Wales), Liverpool, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dave","family":"Dungate","sequence":"additional","affiliation":[{"name":"Hybrid Intelligence, Capgemini Engineering, Stevenage, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"175","published-online":{"date-parts":[[2024,11,20]]},"reference":[{"key":"e_1_3_9_2_2","first-page":"1520","article-title":"Huntington study group cohort investigators","volume":"70","author":"Dorsey ER","year":"2013","unstructured":"Dorsey ER, Beck CA, Darwin K, Nichols P, Brocht AF, Biglan KM, Shoulson I. 2013 Huntington study group cohort investigators. Nat. Hist. Huntington Dis. 70, 1520\u20131530. (doi:10.1001\/jamaneurol.2013.4408)","journal-title":"Nat. Hist. 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Conf. on Neural Information Processing Systems","volume":"1017","author":"Alaa A","year":"2019","unstructured":"Alaa A, Schaar M. 2019 Attentive state-space modeling of disease progression. In Proc. of the 33rd Int. Conf. on Neural Information Processing Systems, vol. 1017, pp. 11338\u201311348, Red Hook, NY, USA: Curran Associates Inc."},{"key":"e_1_3_9_14_2","first-page":"212","article-title":"Enroll-hd: a global clinical research platform for huntington\u2019s disease","volume":"86","author":"Mestre TA","year":"2016","unstructured":"Mestre TA, Fitzer-Attas C, Giuliano J, Landwehrmeyer B, Sampaio C. 2016 Enroll-hd: a global clinical research platform for huntington\u2019s disease. Mov. Disord. 86, 212\u2013224. (doi:10.1212\/WNL.86.16_supplement.S25.005)","journal-title":"Mov. Disord."},{"key":"e_1_3_9_15_2","unstructured":"Enroll-HD. 2023 Enroll-HD periodic dataset documents - PDS6 Overview. See https:\/\/enroll-hd.org\/enrollhd_documents\/2023-10-R2\/ENROLL-HD_PDS6R2overview_v1.0_v20230920.pdf."},{"key":"e_1_3_9_16_2","doi-asserted-by":"publisher","DOI":"10.3233\/JHD-170270"},{"key":"e_1_3_9_17_2","doi-asserted-by":"publisher","DOI":"10.18637\/jss.v038.i08"},{"key":"e_1_3_9_18_2","unstructured":"NHS. 2022 NHS prescription cost analysis \u2013 England 2022-2023. See https:\/\/www.nhsbsa.nhs.uk\/statistical-collections\/prescription-cost-analysis-england\/prescription-cost-analysis-england-2022-23."},{"key":"e_1_3_9_20_2","unstructured":"Office for National Statistics. 2022 Office for National Statistics \u2013 the effects of taxes and benefits on household income 2019\/2020. See https:\/\/www.ons.gov.uk\/peoplepopulationandcommunity\/personalandhouseholdfinances\/incomeandwealth\/bulletins\/theeffectsoftaxesandbenefitsonhouseholdincome\/financialyearending2022."},{"key":"e_1_3_9_21_2","doi-asserted-by":"publisher","DOI":"10.1001\/jamaneurol.2019.2368"},{"key":"e_1_3_9_22_2","unstructured":"Github. 2024 dvgreetham\/hda_modelling. See https:\/\/github.com\/dvgreetham\/hda_modelling."},{"key":"e_1_3_9_23_2","unstructured":"Greetham D. 2024 dvgreetham\/hda_modelling: Published_version. Zenodo. (doi:10.5281\/zenodo.13934437)"},{"key":"e_1_3_9_24_2","unstructured":"Pollard A Greetham DV Myatt J Rickards H Stanley C Dungate D. 2024 Data from: Data-driven Huntington\u2019s disease progression modelling and estimation of societal cost in the UK. Figshare. 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