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


Ontology type: schema:ScholarlyArticle      Open Access: True


Article Info

DATE

2016-09-22

AUTHORS

Caroline E Walker, Trinity Mahede, Geoff Davis, Laura J Miller, Jennifer Girschik, Kate Brameld, Wenxing Sun, Ana Rath, Ségolène Aymé, Stephen R Zubrick, Gareth S Baynam, Caron Molster, Hugh J S Dawkins, Tarun S Weeramanthri

ABSTRACT

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 2016. More... »

PAGES

546-552

Identifiers

URI

http://scigraph.springernature.com/pub.10.1038/gim.2016.143

DOI

http://dx.doi.org/10.1038/gim.2016.143

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1043619859

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/27657686


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30 schema: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 2016.
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42 admission costs
43 advanced data-linkage technologies
44 average length
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46 burden
47 code
48 cohort
49 cohort members
50 cohort study
51 collective health
52 collective impact
53 combined health-system costs
54 cost
55 data
56 data-linkage technologies
57 diagnostic codes
58 discharge
59 disease
60 disease cohort
61 disparities
62 economic impact
63 epidemiological data
64 estimates
65 evidence
66 future rare-disease studies
67 general population
68 government strategies
69 greater average length
70 health
71 health priority
72 health service use
73 health system costs
74 hospital
75 hospital administrative data
76 hospital admission costs
77 hospital costs
78 hospital discharge
79 hospital service use
80 impact
81 inpatient hospital costs
82 length
83 marked disparity
84 members
85 methodology
86 need
87 new evidence
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89 outcomes
90 people
91 population
92 population-based cohort
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94 priority
95 proportion
96 public health priority
97 rare disease
98 rare disease cohorts
99 rare disease studies
100 selection
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105 state inpatient hospital costs
106 stay
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