IntroductionIn Australia we are very lucky – we have no Bovine Spongiform Encephalopathy (BSE) or ‘mad cow disease’ in our cattle and no people have been diagnosed with vCJD. When the first case of vCJD is identified in Australia, it will most likely be in a person who has travelled regularly to or lived for some time in the United Kingdom (UK) in the relevant years (1980 to 1996) or another BSE endemic country, and eaten meat infected with the BSE agent.
This document is a guide to show Australians how the Australian Government will respond to this event and to outline measures already undertaken to protect the Australian public from vCJD.
What is vCJD?Variant CJD was first described in the UK in 1996. vCJD is different from other forms of human CJD (also known as classical CJD, or cCJD) which occur naturally at a rate of approximately 1 - 1.5 cases per million population (or about 20 people) per year in Australia. CJD is a disease that affects the brain and can usually only be accurately identified following an autopsy after death.
Variant CJD is different from cCJD, as it is thought that people catch vCJD from eating meat or offal from cattle infected with the BSE agent. The incubation period is usually greater than five years. To date, over 200 people have been infected with vCJD, in 11 countries with the majority being in the UK. There is some evidence that vCJD may also spread from person-to-person by blood transfusion.
Despite the encouraging decline in numbers of BSE and vCJD cases around the world, the serious consequences of vCJD means that Australia must remain vigilant to ensure that BSE does not become established in cattle herds and the risk of transmission of the BSE agent to humans is reduced. The Department of Health and Ageing continues to work closely with other Australian Government Departments and Agencies to minimise the potential risk to humans from BSE through contamination of food supplies, blood supplies, medicines and therapeutic devices.
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Key Milestones in Australia’s Response so far:Australia has taken a measured and balanced approach in responding to the unlikely but potential threat of vCJD in Australia. The aim is to respond appropriately to the latest scientific information, by implementing effective options to manage any risks posed by the BSE agent. Our responses have included:
- 1996 The livestock industry adopted a voluntary ban on the feeding of ruminant-derived meat-and-bone-meal to ruminants (cattle). This ban was legislated in all States and Territories in 1997 and further extended in 1999 to include banning of additional mammalian material in ruminant feed.
- 1996 The Australian Government banned the importation of beef and beef products from the UK following recognition of vCJD and its links to consumption of beef contaminated with the BSE agent.
- 1996 The Therapeutic Goods Administration (TGA) initiated a comprehensive review of the source of materials of animal origin used in the manufacture of medicines and medical devices, in order to minimise the risk of transmitting vCJD via therapeutic goods.
- November, 2000 Australia’s then Chief Medical Officer, Professor Smallwood announced that an expert committee had undertaken a review of vaccines in Australia that were "grown" in calf serum originally sourced from UK cattle and had considered them to be safe. Professor Smallwood said the findings followed a similar verdict from the American Food and Drug Administration and that the benefit of immunising children outweighed the remote theoretical risk of vCJD.
- December, 2000 Australia's peak public health advisory and medical research body, the National Health and Medical Research Council, announced the establishment of a Special Expert Committee on Transmissible Spongiform Encephalopathies (SECTSE) to provide the Australian Government with direct access to independent and expert advice on BSE and its links to vCJD.
- January, 2001 The Federal Government extended the ban on the importation of foods containing beef or beef products from all countries in Europe except for countries classified as free of Bovine Spongiform Encephalopathy (BSE).
- March, 2001 Australia's then Chief Medical Officer, Professor Smallwood announced that a detailed audit of bovine (beef derived) insulin used in Australia had shown that some beef insulin available in Australia may contain small amounts of bovine insulin derived from Dutch cattle. The NHMRC Special Expert Committee on TSEs had reviewed this information in the light of available evidence and an evaluation of insulin manufacturing processes. The Committee concluded that there was a negligibly small risk of BSE transmission through these products. The theoretical risk from this product was determined to be less than the very real risk to the health of people with diabetes if their current treatment using bovine insulin was disrupted.
- July, 2001 The then Australia New Zealand Food Authority announced a new certification system that determine the conditions under which beef and beef products may enter Australia. Beef sourced from countries with indigenous cases of BSE cannot be imported into Australia. This action followed a voluntary withdrawal of suspect products from supermarket shelves in January, 2001.
- July, 2002 Australian Health Ministers collectively agreed to take precautionary action over possible links between vCJD and blood and announced that a temporary ban would be placed on blood donations from people who lived in the UK for a cumulative total of six months or more during the height of the BSE outbreak - from 1980 to 1996.
- July, 2003 All Australian State and Territory Health Ministers agreed to a change that extended the deferral of people donating blood who lived in the UK for six months from 1980 to 1996 to include people who received a transfusion of blood or blood products in the UK from 1980 onwards.
- January, 2004 The Communicable Disease Network of Australia endorsed the addition of CJD to the list of Nationally Notifiable Diseases.
- October, 2006 SECTSE renamed the Transmissible Spongiform Encephalopathies Advisory Committee (TSEAC).
The Response PlanThe Australian Government, in conjunction with all State and Territory Governments, has developed a vCJD Response Plan to respond to the threat of BSE/vCJD in Australia.
The following draws relevant information from the Plan to give the Australian community an understanding and overview of the sequence of events that will be undertaken on their behalf when a first case of vCJD is found or suspected in Australia.
- STEP 1: The role of doctors/clinicians: The first case of vCJD in Australia would most likely be discovered by a doctor working in general practice or in a hospital, or by a neurologist who is treating a patient for abnormal neurological symptoms. Suspicions may be raised about vCJD by a psychiatrist or psychologist as people presenting with vCJD may be referred for psychiatric treatment in the absence of a clear diagnosis.
- STEP 2: The importance of the CJD Registry: Doctors suspecting such a diagnosis will be expected to report their findings to their State or Territory Health Department and to obtain advice from the Australian National CJD Registry, based at the University of Melbourne. Funded by the Australian Government, the CJD Registry is Australia’s key surveillance watchdog for naturally occurring or medically acquired CJD and is constantly on the lookout for the first case of vCJD.
- STEP 3: Health Authorities talking to each other: It will be important to inform all health departments about the first case of vCJD. There are regular telephone hookups between the Australian Government Department of Health and Ageing and health departments in every State and Territory, via a network called the Communicable Diseases Network Australia, which is a highly effective avenue for sharing information across all Australian health departments.
- STEP 4: Testing the patient: The early symptoms of vCJD are similar to a number of other diseases so, with advice from vCJD experts, the physician could undertake a number of tests to exclude other diseases. A full range of blood tests may be carried out, as well as some scans of the brain, such as Magnetic Resonance Imaging (MRI), Computed Tomography (CT) and an electroencephalogram (EEG). A tonsil biopsy may also be requested to try to indicate vCJD.
- STEP 5: Travel and medical history of the patient: If vCJD is confirmed, an accurate travel history of the person is crucial to verify that the vCJD was contracted overseas. It is likely that families of the patient will be contacted to assist in establishing travel history, foods eaten both overseas and within Australia and if there is any history of blood transfusion or blood donation by the patient. Families may also be asked about the patient’s recent medical or dental treatments.
- STEP 6: If there is no record of overseas travel: In the unlikely event that the patient has never been overseas, medical authorities will investigate a range of other possibilities including medical procedures that the person may have had in the past or the range of foods eaten.
- STEP 7: Involvement of Australian Government Departments and authorities, an important part of the process: Australian Government Department of Agriculture, Fisheries and Forestry (DAFF), Food Standards Australia and New Zealand (FSANZ), Therapeutic Goods Administration, National Blood Authority and the State and Territory Health Departments will work to establish whether the disease was acquired in Australia or overseas and any potential spread within Australia.
- STEP 8: The role of the Chief Medical Officer: The Chief Medical Officer will act as a key spokesperson and senior liaison point between the Federal and State and Territory health authorities and will be the spokesman to the public, keeping Australians informed of what is happening.
- STEP 9: Media involvement: When the first case of vCJD is discovered there is bound to be media interest, both within Australia and from media outlets internationally. The Australian Government Department of Health and Ageing’s Media Unit and media units in the States and Territories will be working closely with local media to ensure that factual information is provided to the public. Every effort will be made to keep private the identity of the patient and his or her family.
- STEP 10: Public education about vCJD transmission risk: It will be important for the public to understand that a person with vCJD cannot spread the disease through normal social or sexual contact. There is a small risk that vCJD can be transmitted by blood transfusion. Australia’s blood donation deferral policy for people who have lived in the UK between 1980 to 1996, means it is highly unlikely that the Australian blood supply has been affected by anyone who has contracted vCJD from eating contaminated beef in the UK. It will be important to minimise public anxiety.
- STEP 11: Web site: The current web sites that give comprehensive information about BSE/vCJD and Australia’s planned responses will be regularly updated to inform the public of latest developments. These web sites include:
www.daff.gov.au – Australian Government Department of Agriculture, Fisheries and Forestry
www.foodstandards.gov.au – Food Standards Australia and New Zealand (FSANZ)
www.tga.gov.au – Therapeutic Goods Administration
www.nba.gov.au – National Blood Authority
www.cjdsupport.org.au – CJD Support Group Network