Statement of Principles concerning HEREDITARY HAEMOCHROMATOSIS (Balance of Probabilities) (No. 24 of 2021) The Repatriation Medical Authority determines the following Statement of Principles under subsection 196B(3) of the Veterans' Entitlements Act 1986. Dated 24 December 2020 The Common Seal of the Repatriation Medical Authority was affixed to this instrument at the direction of: Professor Nicholas Saunders AO Chairperson Contents 1 Name 2 Commencement 3 Authority 4 Repeal 5 Application 6 Definitions 7 Kind of injury, disease or death to which this Statement of Principles relates 8 Basis for determining the factors 9 Factors that must exist 10 Relationship to service 11 Factors referring to an injury or disease covered by another Statement of Principles Schedule 1 - Dictionary 1 Definitions 1 Name This is the Statement of Principles concerning hereditary haemochromatosis (Balance of Probabilities) (No. 24 of 2021). 2 Commencement This instrument commences on 25 January 2021. 3 Authority This instrument is made under subsection 196B(3) of the Veterans' Entitlements Act 1986. 4 Repeal The Statement of Principles concerning haemochromatosis No. 22 of 2012 (Federal Register of Legislation No. F2012L00451) made under subsection 196B(3) of the VEA is repealed. 5 Application This instrument applies to a claim to which section 120B of the VEA or section 339 of the Military Rehabilitation and Compensation Act 2004 applies. 6 Definitions The terms defined in the Schedule 1 - Dictionary have the meaning given when used in this instrument. 7 Kind of injury, disease or death to which this Statement of Principles relates (1) This Statement of Principles is about hereditary haemochromatosis and death from hereditary haemochromatosis. Meaning of hereditary haemochromatosis (2) For the purposes of this Statement of Principles, hereditary haemochromatosis: (a) means any genetic disorder of iron transport and metabolism which results in accumulation of excess iron, and with: (i) documented iron overload in parenchymatous organs; or (ii) clinical evidence of iron-related organ or tissue dysfunction; and (b) includes: (i) classical (type 1) hereditary haemochromatosis due to a mutation of the HFE gene; and (ii) non-classical hereditary haemochromatosis due to a mutation of a non-HFE related gene. Note: Hereditary haemochromatosis is characterised by the gradual increase in body iron stores in organs and tissues, which can lead to various clinical disorders, including lethargy, hepatic fibrosis or cirrhosis, hepatocellular carcinoma, heart failure, diabetes mellitus, hypogonadism, arthritis, hypopituitarism and skin pigmentation. (3) While hereditary haemochromatosis attracts ICD‑10‑AM code E83.1, in applying this Statement of Principles the meaning of hereditary haemochromatosis is that given in subsection (2). (4) For subsection (3), a reference to an ICD-10-AM code is a reference to the code assigned to a particular kind of injury or disease in The International Statistical Classification of Diseases and Related Health Problems, Tenth Revision, Australian Modification (ICD-10-AM), Tenth Edition, effective date of 1 July 2017, copyrighted by the Independent Hospital Pricing Authority, ISBN 978-1-76007-296-4. Death from hereditary haemochromatosis (5) For the purposes of this Statement of Principles, hereditary haemochromatosis, in relation to a person, includes death from a terminal event or condition that was contributed to by the person's hereditary haemochromatosis. Note: terminal event is defined in the Schedule 1 - Dictionary. 8 Basis for determining the factors On the sound medical‑scientific evidence available, the Repatriation Medical Authority is of the view that it is more probable than not that hereditary haemochromatosis and death from hereditary haemochromatosis can be related to relevant service rendered by veterans or members of the Forces under the VEA, or members under the MRCA. Note: MRCA, relevant service and VEA are defined in the Schedule 1 - Dictionary. 9 Factors that must exist At least one of the following factors must exist before it can be said that, on the balance of probabilities, hereditary haemochromatosis or death from hereditary haemochromatosis is connected with the circumstances of a person's relevant service: (1) for males, consuming a total of at least 60 kilograms of alcohol within any five year period before the clinical worsening of hereditary haemochromatosis; Note 1: Alcohol consumption is calculated utilising the Australian Standard of ten grams of alcohol per standard alcoholic drink. Note 2: The clinical worsening of hereditary haemochromatosis typically manifests as hepatic fibrosis or cirrhosis. (2) for females, consuming a total of at least 40 kilograms of alcohol within any five year period before the clinical worsening of hereditary haemochromatosis; Note 1: Alcohol consumption is calculated utilising the Australian Standard of ten grams of alcohol per standard alcoholic drink. Note 2: The clinical worsening of hereditary haemochromatosis typically manifests as hepatic fibrosis or cirrhosis. (3) having infection with hepatitis C virus at the time of the clinical worsening of hereditary haemochromatosis; Note: The clinical worsening of hereditary haemochromatosis typically manifests as hepatic fibrosis or cirrhosis. (4) having steatohepatitis at the time of the clinical worsening of hereditary haemochromatosis; Note: The clinical worsening of hereditary haemochromatosis typically manifests as hepatic fibrosis or cirrhosis. (5) inability to obtain appropriate clinical management for hereditary haemochromatosis. 10 Relationship to service (1) The existence in a person of any factor referred to in section 9, must be related to the relevant service rendered by the person. (2) The factors set out in subsections 9(1) to 9(5) apply only to material contribution to, or aggravation of, hereditary haemochromatosis where the person's hereditary haemochromatosis was suffered or contracted before or during (but did not arise out of) the person's relevant service. 11 Factors referring to an injury or disease covered by another Statement of Principles In this Statement of Principles: (1) if a factor referred to in section 9 applies in relation to a person; and (2) that factor refers to an injury or disease in respect of which a Statement of Principles has been determined under subsection 196B(3) of the VEA; then the factors in that Statement of Principles apply in accordance with the terms of that Statement of Principles as in force from time to time. Schedule 1 - Dictionary Note: See Section 6 1 Definitions In this instrument: hereditary haemochromatosis—see subsection 7(2). MRCA means the Military Rehabilitation and Compensation Act 2004. relevant service means: (a) eligible war service (other than operational service) under the VEA; (b) defence service (other than hazardous service and British nuclear test defence service) under the VEA; or (c) peacetime service under the MRCA. Note: MRCA and VEA are also defined in the Schedule 1 - Dictionary. terminal event means the proximate or ultimate cause of death and includes the following: (a) pneumonia; (b) respiratory failure; (c) cardiac arrest; (d) circulatory failure; or (e) cessation of brain function. VEA means the Veterans' Entitlements Act 1986.