University Of Tasmania
107821 Journal Article.pdf (224.05 kB)

Quality of life utility values for hereditary haemochromatosis in Australia

Download (224.05 kB)
journal contribution
posted on 2023-05-18, 18:20 authored by Barbara de GraaffBarbara de Graaff, Amanda NeilAmanda Neil, Kristy Sanderson, Kwang YeeKwang Yee, Andrew PalmerAndrew Palmer
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.


Publication title

Health and Quality of Life Outcomes



Article number









Menzies Institute for Medical Research


BioMed Central Ltd.

Place of publication

United Kingdom

Rights statement

Copyright 2016 de Graaff et al. Licensed under Creative Commons Attribution 4.0 International (CC BY 4.0)

Repository Status

  • Open

Socio-economic Objectives

Diagnosis of human diseases and conditions

Usage metrics

    University Of Tasmania