The collective impact of rare diseases in Western Australia: an estimate using a population-based cohort.

PURPOSE: It has been argued that rare diseases should be recognized as a public health priority. However, there is a shortage of epidemiological data describing the true burden of rare diseases. This study investigated hospital service use to provide a better understanding of the collective health a...

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Main Authors: Walker, C., Mahede, T., Davis, G., Miller, L., Girschik, J., Brameld, Kate, Sun, W., Rath, A., Aymé, S., Zubrick, S., Baynam, G., Molster, C., Dawkins, H., Weeramanthri, T.
Format: Journal Article
Published: 2016
Online Access:http://hdl.handle.net/20.500.11937/5672
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author Walker, C.
Mahede, T.
Davis, G.
Miller, L.
Girschik, J.
Brameld, Kate
Sun, W.
Rath, A.
Aymé, S.
Zubrick, S.
Baynam, G.
Molster, C.
Dawkins, H.
Weeramanthri, T.
author_facet Walker, C.
Mahede, T.
Davis, G.
Miller, L.
Girschik, J.
Brameld, Kate
Sun, W.
Rath, A.
Aymé, S.
Zubrick, S.
Baynam, G.
Molster, C.
Dawkins, H.
Weeramanthri, T.
author_sort Walker, C.
building Curtin Institutional Repository
collection Online Access
description PURPOSE: It has been argued that rare diseases should be recognized as a public health priority. However, there is a shortage of epidemiological data describing the true burden of rare diseases. This study investigated hospital service use to provide a better understanding of the collective health and economic impacts of rare diseases. METHODS: Novel methodology was developed using a carefully constructed set of diagnostic codes, a selection of rare disease cohorts from hospital administrative data, and advanced data-linkage technologies. Outcomes included health-service use and hospital admission costs. RESULTS: In 2010, cohort members who were alive represented approximately 2.0% of the Western Australian population. The cohort accounted for 4.6% of people discharged from hospital and 9.9% of hospital discharges, and it had a greater average length of stay than the general population. The total cost of hospital discharges for the cohort represented 10.5% of 2010 state inpatient hospital costs. CONCLUSIONS: This population-based cohort study provides strong new evidence of a marked disparity between the proportion of the population with rare diseases and their combined health-system costs. The methodology will inform future rare-disease studies, and the evidence will guide government strategies for managing the service needs of people living with rare diseases.Genet Med advance online publication 22 September 2016Genetics in Medicine (2016); doi:10.1038/gim.2016.143.
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spelling curtin-20.500.11937-56722019-02-19T05:34:53Z The collective impact of rare diseases in Western Australia: an estimate using a population-based cohort. Walker, C. Mahede, T. Davis, G. Miller, L. Girschik, J. Brameld, Kate Sun, W. Rath, A. Aymé, S. Zubrick, S. Baynam, G. Molster, C. Dawkins, H. Weeramanthri, T. PURPOSE: It has been argued that rare diseases should be recognized as a public health priority. However, there is a shortage of epidemiological data describing the true burden of rare diseases. This study investigated hospital service use to provide a better understanding of the collective health and economic impacts of rare diseases. METHODS: Novel methodology was developed using a carefully constructed set of diagnostic codes, a selection of rare disease cohorts from hospital administrative data, and advanced data-linkage technologies. Outcomes included health-service use and hospital admission costs. RESULTS: In 2010, cohort members who were alive represented approximately 2.0% of the Western Australian population. The cohort accounted for 4.6% of people discharged from hospital and 9.9% of hospital discharges, and it had a greater average length of stay than the general population. The total cost of hospital discharges for the cohort represented 10.5% of 2010 state inpatient hospital costs. CONCLUSIONS: This population-based cohort study provides strong new evidence of a marked disparity between the proportion of the population with rare diseases and their combined health-system costs. The methodology will inform future rare-disease studies, and the evidence will guide government strategies for managing the service needs of people living with rare diseases.Genet Med advance online publication 22 September 2016Genetics in Medicine (2016); doi:10.1038/gim.2016.143. 2016 Journal Article http://hdl.handle.net/20.500.11937/5672 10.1038/gim.2016.143 http://creativecommons.org/licenses/by/4.0/ fulltext
spellingShingle Walker, C.
Mahede, T.
Davis, G.
Miller, L.
Girschik, J.
Brameld, Kate
Sun, W.
Rath, A.
Aymé, S.
Zubrick, S.
Baynam, G.
Molster, C.
Dawkins, H.
Weeramanthri, T.
The collective impact of rare diseases in Western Australia: an estimate using a population-based cohort.
title The collective impact of rare diseases in Western Australia: an estimate using a population-based cohort.
title_full The collective impact of rare diseases in Western Australia: an estimate using a population-based cohort.
title_fullStr The collective impact of rare diseases in Western Australia: an estimate using a population-based cohort.
title_full_unstemmed The collective impact of rare diseases in Western Australia: an estimate using a population-based cohort.
title_short The collective impact of rare diseases in Western Australia: an estimate using a population-based cohort.
title_sort collective impact of rare diseases in western australia: an estimate using a population-based cohort.
url http://hdl.handle.net/20.500.11937/5672