Statement of Principles concerning porphyria cutanea tarda (Reasonable Hypothesis) (No. 69 of 2021) (Cth)

Statement of Principles concerning PORPHYRIA CUTANEA TARDA (Reasonable Hypothesis) (No. 69 of 2021) The Repatriation Medical Authority determines the following Statement of Principles under subsection 196B(2) of the Veterans' Entitlements Act 1986.

Dated 21 May 2021

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 porphyria cutanea tarda (Reasonable Hypothesis) (No. 69 of 2021). 2 Commencement This instrument commences on 21 June 2021. 3 Authority This instrument is made under subsection 196B(2) of the Veterans' Entitlements Act 1986. 4 Repeal The Statement of Principles concerning porphyria cutanea tarda No. 43 of 2012 (Federal Register of Legislation No. F2012L01357) made under subsection 196B(2) of the VEA is repealed. 5 Application This instrument applies to a claim to which section 120A of the VEA or section 338 of the Military Rehabilitation and Compensation Act 2004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 porphyria cutanea tarda and death from porphyria cutanea tarda. Meaning of porphyria cutanea tarda (2) For the purposes of this Statement of Principles, porphyria cutanea tarda means a skin disorder resulting from decreased activity of the enzyme uroporphyrinogen decarboxylase in the liver and accumulation of porphyrins in the circulation. Note: Typical clinical manifestations of porphyria cutanea tarda include blistering of the skin in sun-exposed areas, hyperpigmentation and facial hypertrichosis. (3) While porphyria cutanea tarda attracts ICD‑10‑AM code E80.1, in applying this Statement of Principles the meaning of porphyria cutanea tarda 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 porphyria cutanea tarda (5) For the purposes of this Statement of Principles, porphyria cutanea tarda, in relation to a person, includes death from a terminal event or condition that was contributed to by the person's porphyria cutanea tarda. Note: terminal event is defined in the Schedule 1 - Dictionary. 8 Basis for determining the factors The Repatriation Medical Authority is of the view that there is sound medical‑scientific evidence that indicates that porphyria cutanea tarda and death from porphyria cutanea tarda can be related to relevant service rendered by veterans, members of Peacekeeping Forces, 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 as a minimum exist before it can be said that a reasonable hypothesis has been raised connecting porphyria cutanea tarda or death from porphyria cutanea tarda with the circumstances of a person's relevant service: (1) for males, consuming a total of at least 100 kilograms of alcohol within the 10 years before the clinical onset of porphyria cutanea tarda; Note: Alcohol consumption is calculated utilising the Australian Standard of 10 grams of alcohol per standard alcoholic drink. (2) for females, consuming a total of at least 50 kilograms of alcohol within the 10 years before the clinical onset of porphyria cutanea tarda; Note: Alcohol consumption is calculated utilising the Australian Standard of 10 grams of alcohol per standard alcoholic drink. (3) having alcohol use disorder at the time of the clinical onset of porphyria cutanea tarda; (4) having cirrhosis of the liver or steatohepatitis at the time of the clinical onset of porphyria cutanea tarda; (5) having chronic renal failure at the time of the clinical onset of porphyria cutanea tarda; Note: chronic renal failure is defined in the Schedule 1 - Dictionary. (6) having hepatic iron overload at the time of the clinical onset of porphyria cutanea tarda; Note: iron overload is defined in the Schedule 1 - Dictionary. (7) having a viral infection from the specified list of viral infections at the time of the clinical onset of porphyria cutanea tarda; Note: specified list of viral infections is defined in the Schedule 1 - Dictionary. (8) taking a drug from the specified list of drugs within the 30 days before the clinical onset of porphyria cutanea tarda; Note: specified list of drugs is defined in the Schedule 1 - Dictionary. (9) taking a drug which is associated in the individual with the clinical onset of porphyria cutanea tarda during drug therapy and either: (a) the improvement of porphyria cutanea tarda within 2 months of discontinuing or tapering drug therapy; or (b) the redevelopment of porphyria cutanea tarda on rechallenge with the same drug; and where taking the drug continued for at least the 7 days before the clinical onset of porphyria cutanea tarda; (10) inhaling, ingesting or having cutaneous contact with a chemical agent contaminated by 2,3,7,8-tetrachlorodibenzo-para-dioxin (TCDD) within the 1 year before the clinical onset of porphyria cutanea tarda; Note: inhaling, ingesting or having cutaneous contact with a chemical agent contaminated by 2,3,7,8-tetrachlorodibenzo-para-dioxin (TCDD) is defined in the Schedule 1 - Dictionary. (11) ingesting food contaminated with hexachlorobenzene within the 1 year before the clinical onset of porphyria cutanea tarda; (12) having smoked tobacco products: (a) in an amount of at least 10 cigarettes per day or the equivalent thereof in other tobacco products; and (b) for at least the 6 months before the clinical onset of porphyria cutanea tarda; and if smoking has ceased before the clinical onset of porphyria cutanea tarda, then that onset occurred within 30 days of cessation; Note: cigarettes per day or the equivalent thereof in other tobacco products is defined in the Schedule 1 - Dictionary. (13) for males, consuming a total of at least 100 kilograms of alcohol within the 10 years before the clinical worsening of porphyria cutanea tarda; Note: Alcohol consumption is calculated utilising the Australian Standard of 10 grams of alcohol per standard alcoholic drink. (14) for females, consuming a total of at least 50 kilograms of alcohol within the 10 years before the clinical worsening of porphyria cutanea tarda; Note: Alcohol consumption is calculated utilising the Australian Standard of 10 grams of alcohol per standard alcoholic drink. (15) having alcohol use disorder at the time of the clinical worsening of porphyria cutanea tarda; (16) having cirrhosis of the liver or steatohepatitis at the time of the clinical worsening of porphyria cutanea tarda; (17) having chronic renal failure at the time of the clinical worsening of porphyria cutanea tarda; Note: chronic renal failure is defined in the Schedule 1 - Dictionary. (18) having hepatic iron overload at the time of the clinical worsening of porphyria cutanea tarda; Note: iron overload is defined in the Schedule 1 - Dictionary. (19) having a viral infection from the specified list of viral infections at the time of the clinical worsening of porphyria cutanea tarda; Note: specified list of viral infections is defined in the Schedule 1 - Dictionary. (20) taking a drug from the specified list of drugs within the 30 days before the clinical worsening of porphyria cutanea tarda; Note: specified list of drugs is defined in the Schedule 1 - Dictionary. (21) taking a drug which is associated in the individual with: (a) the clinical worsening of porphyria cutanea tarda during drug therapy; and (b) the improvement of porphyria cutanea tarda within 2 months of discontinuing or tapering drug therapy; and where taking the drug continued for at least the 7 days before the clinical worsening of porphyria cutanea tarda; (22) inhaling, ingesting or having cutaneous contact with a chemical agent contaminated by 2,3,7,8-tetrachlorodibenzo-para-dioxin (TCDD) within the 1 year before the clinical worsening of porphyria cutanea tarda; Note: inhaling, ingesting or having cutaneous contact with a chemical agent contaminated by 2,3,7,8-tetrachlorodibenzo-para-dioxin (TCDD) is defined in the Schedule 1 - Dictionary. (23) ingesting food contaminated with hexachlorobenzene within the 1 year before the clinical worsening of porphyria cutanea tarda; (24) having smoked tobacco products: (a) in an amount of at least 10 cigarettes per day or the equivalent thereof in other tobacco products; and (b) for least 6 months before the clinical worsening of porphyria cutanea tarda; and if smoking has ceased before the clinical worsening of porphyria cutanea tarda, then that worsening occurred within 30 days of cessation; Note: cigarettes per day or the equivalent thereof in other tobacco products is defined in the Schedule 1 - Dictionary. (25) inability to obtain appropriate clinical management for porphyria cutanea tarda. 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(13) to 9(25) apply only to material contribution to, or aggravation of, porphyria cutanea tarda where the person's porphyria cutanea tarda 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(2) 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.