title: Quality of life utility values for hereditary haemochromatosis in Australia.
abstract: BACKGROUND
  Hereditary hemochromatosis (HH) is a common autosomal recessive disorder amongst persons of northern European heritage. If untreated, iron accumulates in parenchymal tissues causing morbidity and mortality. As diagnosis often follows irreversible organ damage, screening programs have been suggested to increase early diagnosis. A lack of economic evidence has been cited as a barrier to establishing such a program. Previous analyses used poorly estimated utility values. This study sought to measure utilities directly from people with HH in Australia.
  
  
  METHODS
  Volunteers with HH were recruited to complete a web-based survey. Utility was assessed using the Assessment of Quality of Life 4D (AQOL-4D) instrument. Severity of HH was graded into four categories. Multivariable regression analysis was performed to identify parameters associated with HSUV.
  
  
  RESULTS
  Between November 2013 and November 2014, 221 people completed the survey. Increasing severity of HH was negatively associated with utility. Mean (standard deviation) utilities were 0.76 (0.21), 0.81 (0.18), 0.60 (0.27), and 0.50 (0.27) for categories 1-4 HH respectively. Lower mean utility was found for symptomatic participants (categories 3 and 4) compared with asymptomatic participants (0.583 v. 0.796). Self-reported HH-related symptoms were negatively associated with HSUV (r = -0.685).
  
  
  CONCLUSIONS
  Symptomatic stages of HH and presence of multiple self-reported symptoms were associated with decreasing utility. Previous economic analyses have used higher utilities which likely resulted in underestimates of the cost effectiveness of HH interventions. The utilities reported in this paper are the most robust available, and will contribute to improving the validity of future economic models for HH.
authors.affiliation: Menzies Institute for Medical Research, University of Tasmania, Medical Sciences Building 1, 17 Liverpool St, Private Bag 23, Hobart, TAS, 7000, Australia.
authors.forename: Barbara
authors.initials: B
authors.lastname: de Graaff
authors.affiliation: Menzies Institute for Medical Research, University of Tasmania, Medical Sciences Building 1, 17 Liverpool St, Private Bag 23, Hobart, TAS, 7000, Australia.
authors.forename: Amanda
authors.initials: A
authors.lastname: Neil
authors.affiliation: Menzies Institute for Medical Research, University of Tasmania, Medical Sciences Building 1, 17 Liverpool St, Private Bag 23, Hobart, TAS, 7000, Australia.
authors.forename: Kristy
authors.initials: K
authors.lastname: Sanderson
authors.affiliation: School of Medicine, University of Tasmania, Medical Sciences Building 2, 17 Liverpool St, Private Bag 68, Hobart, TAS, 7000, Australia.
authors.forename: Kwang Chien
authors.initials: KC
authors.lastname: Yee
authors.affiliation: Menzies Institute for Medical Research, University of Tasmania, Medical Sciences Building 1, 17 Liverpool St, Private Bag 23, Hobart, TAS, 7000, Australia. Andrew.Palmer@utas.edu.au.
authors.forename: Andrew J
authors.initials: AJ
authors.lastname: Palmer
mesh_terms: D000328:Adult; D001315:Australia; D005260:Female; D006304:Health Status; D006432:Hemochromatosis / Q000523:psychology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D011788:Quality of Life / Q000523:psychology*; D012720:Severity of Illness Index; D018434:Sickness Impact Profile