• First, strengthening our efforts to keep BSE out of the American cattle herd and to keep it from amplifying in the herd were it ever to be found in American cows

• Second, strengthening our vigilance to keep potentially TSE-infected foods and other FDA-regulated products from Americans

• Third, enhancing the research needed to better understand TSEs and to develop needed diagnostic tools, therapies, and preventive measures for humans and animals

• Increased enforcement:
• Strengthens efforts to reach goal of 100% compliance with regulations regarding banned cattle feeding practices (first priority);
• Strengthens enforcement of import restrictions on potentially infected products;
• Enhances regulatory efforts with regard to CWD in elk and deer

• Enhanced regulatory tools/legislation:
• Assures enhancements of blood and tissue donation deferral policies;
• Assures enhancements of regulations regarding acceptability of "at risk" bovine materials in various products;
• Assures re-assessment of present policies based on new experience and science.


• Enhanced public communication:
• Assures enhancement of public outreach programs regarding TSEs and potential risks for American public

• Enhances development of new tools to aid in diagnosis of TSEs in animals and humans, to aid in screening of blood and tissues, and to aid in assuring the quality of regulated products with regard to potential TSE contamination

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The group of human and animal diseases known as transmissible spongiform encephalopathies (TSEs) is characterized by a sponge-like appearance of the brain and is associated with deposits in the brain of unique proteins called prions. In some, but not all TSEs, deposits of prions, sometimes detectable pre-clinically, occur in other tissues as well. The function, biology, and pathology of prions are still largely unknown. It appears they are not associated with disease in their "native" state, but only when they become abnormally folded. What causes the abnormal folding to occur, why hosts cannot dispose of or develop immunity to these proteins, and what factors might cause or transmit TSEs are poorly understood.

Prions can be transmitted from one host to another; however, they do not exhibit many other features of traditional infective agents, such as bacteria, viruses, mycobacteria, protozoa, or fungi. As such, prions are not considered "germs" in the classic sense, but rather, are referred to as "agents" of disease. In recent years, research has developed epidemiological evidence of transmissibility of certain of these agents, not only between members of a single species, but also, more ominously, from one species to another.

TSEs occur in several different animal species. Scrapie in sheep and goats has been described for several hundred years. Chronic wasting disease (CWD) in elk and deer has been reported in Colorado, Wyoming, and Canada for several decades. Other TSEs have also been reported in mink and cats.

In the mid-1980’s a new TSE, bovine spongiform encephalopathy (BSE), was first described in cattle in the United Kingdom. The government of the United Kingdom initiated major efforts to determine the origin of the disease and to control the epidemic of BSE (or "mad cow disease") that followed the initial reports. Strong evidence implicates certain feeding practices as the major route of initiation of the disease in cows and of further amplification of the disease throughout the British cattle herd. These feeding practices include certain rendering procedures and the recycling of ruminant animal tissues and byproducts into the cattle food chain through certain livestock feed supplements.

The precautionary slaughter of large numbers of British cows and ever more stringent prohibitions on certain animal feeding and slaughter practices appear to have stemmed, though not eradicated, the BSE epidemic in the United Kingdom. Unfortunately, these measures were not sufficient or enforced stringently enough or instituted soon enough to prevent the spread of the disease to continental European countries. At present, the U. S. Department of Agriculture (USDA) officially recognizes thirty European countries and Oman as either having documented BSE cases or being at high risk for BSE.

Humans are also susceptible to TSEs. Two of the better-documented human TSEs are kuru and classical Creutzfeldt-Jakob disease (CJD). Both of these diseases have been described for decades. Kuru is a disease of the South Pacific Fore people and has been epidemiologically associated with the consumption of human brains. Classical CJD, conversely, occurs worldwide at a stable rate of approximately 1 case per million people. The epidemiology of this disease is not fully understood, although, in certain cases, a specific genetic component appears to increase susceptibility. Transmission of classical CJD between humans via transplants of dura mater and corneas and by handling tissues or injecting pituitary extract from patients with CJD has been documented.

In 1996 a new variant of CJD (vCJD) was described in patients in the United Kingdom. Epidemiological data implicate the consumption of beef products contaminated with the agent of BSE as the probable cause of vCJD in humans. Due to the long incubation time of the agent in humans before vCJD is clinically recognizable, the number of people who harbor the agent of vCJD is unknown. Presently, approximately 100 people in Europe (primarily in the United Kingdom) are known to have died from vCJD.

Unfortunately, the lack of any sensitive, specific pre-mortem diagnostic test TSEs in either humans or animals limits surveillance and other research efforts. Diagnosis is usually only confirmed by special post-mortem examinations of brain tissue.

Presently, animal and human TSEs have no treatments or preventative vaccines. All are invariably fatal.

Routine materials and processes that destroy traditional human and animal pathogens do not appear to destroy prions. Presently, no established methods can reliably decontaminate or sterilize articles contaminated with prions.

There is no evidence of either BSE or vCJD in the United States. However, it is clear that the Atlantic Ocean itself is not a barrier to the spread of this disease. Several issues raise concerns on this side of the Atlantic about potential spread of domestic TSEs, as well as the introduction of BSE and vCJD into the United States. These concerns include: (1) the presence of animal TSEs in North America; (2) certain historic American animal feeding and animal import practices; (3) the continental European experience with BSE and vCJD in the summer of 2000; (4) the international commerce in animals, animal feed, and other products of bovine origin; and (5) the heavy intercontinental travel by Americans.

Based on the European experience with BSE, it is clear that the discovery of BSE in American cattle would be devastating to the U.S. cattle industry. The ultimate magnitude of the public health impact of BSE and vCJD remains to be determined. Nonetheless, due to the invariably fatal nature of the disease, the lack of routine methods to destroy the agent, the ubiquitous presence of bovine products in most people’s daily lives, and the nascent level of scientific knowledge about prion disease, BSE and vCJD cause understandable concerns in the public, the government, and the private sector.

The Department of Health and Human Services has developed a departmental framework document (see Reference 1) that outlines the responsibilities of FDA, CDC, NIH, and the Office of the Secretary regarding TSEs. In that document, the Secretary designates "protection" as the primary responsibility of FDA.

FDA understands its TSE mission is to enhance, sustain, and communicate regulatory safeguards against the agents of TSEs, and, thereby, protect the public health of the U.S.

To fulfill its TSE-related mission, FDA’s TSE program will adhere to the following five principles:

1. Strong science-based knowledge is fundamental to addressing this challenge to public health. FDA’s public health policies will be grounded in that science.
2. The science of these diseases will continue to evolve. FDA’s actions will foster this evolution, and FDA’s policies will respond to and reflect this new knowledge.
3. These diseases uniformly have lethal outcomes, and the level of scientific knowledge about them is quite nascent. Because of these factors, FDA’s public health policies should employ a very cautious approach and should take into account the level of known risk (both real and theoretical).
4. As much as possible, FDA will utilize public advisory committees and other public fora, along with departmental and outside experts, domestic and foreign, to advise concerning the agency’s development of scientific, regulatory, and communication policies.
5. FDA will communicate with all our stakeholders as openly and frankly as possible regarding TSE issues. Science-driven facts and transparent discourse are our best tools to build public trust and understanding.

To fulfill its TSE-related mission, FDA’s program will work to achieve the following six goals:

1. Working with other federal, state, and private sector entities, FDA will protect the U.S. public health by preventing, to the extent possible, the entry of the agent of BSE into U.S. cattle.
2. If the agent of BSE were to enter U.S. cattle, FDA, in coordination with other federal, state, and private sector entities, will have systems in place to identify, isolate, and destroy the affected cattle and to prevent amplification of the disease further in the U.S. herd.
3. FDA will protect the U.S. public health by minimizing exposure of Americans to TSE agents through: (a) potentially contaminated food products, (b) other FDA-regulated products that use potentially infected materials in their manufacture, and (c) other FDA-regulated products or materials which may contain TSE agents.
4. FDA will protect the U.S. public health by facilitating the development of reliable pre-mortem diagnostic tests for and options for the prevention and treatment of TSEs of humans and animals.
5. FDA will protect the U.S. public health by developing and sustaining a strong regulatory infrastructure to assure compliance with TSE regulations and policies.
6. FDA will protect the U.S. public health by developing and sustaining, in coordination with other federal, state, and private sector entities, an effective communications program with our stakeholders on TSE issues.

FDA has identified, and will continue to identify, specific action items that it will undertake in each of these five major focus areas. These action items, as of the date of this document, are listed below. The short description of each action item also includes the designated lead organizational component(s) within FDA. Other organizations, with which the lead will primarily consult, are shown within parentheses.

Ultimate responsibility for oversight of these action items resides with the Commissioner of Food and Drugs, the person designated by the Commissioner as the lead for TSE-activities in the Office of the Commissioner, and the directors of the organizational components identified in this document as being the lead for a specific action item.

It should be noted that FDA might not complete some action items, because FDA, at present, is simply in a fact-finding mode for those items. As further information is gathered regarding certain items, it may be decided that the action item should not be pursued or that the action does not fall within the jurisdiction of FDA. Additional action items may also be identified.

In prioritizing FDA resources to accomplish these specific action items, FDA has allocated the resources it has for TSE purposes first to those activities believed to have the most significant impact on FDA TSE goals.

Many of FDA’s activities to date have focused primarily on (1) preventing the entry of BSE into U.S. cattle and (2) ensuring that if BSE entered the U.S. herd, tissue from the infected animal(s) would not end up in cattle feed and potentially infect other animals (i.e., be amplified through the herd). Cattle that eat feed that does not contain certain animal protein, which might contain BSE's infectious agent, will be free of BSE. Products for human use made from these cattle will be free from the potential risk of causing the human version of BSE (vCJD). Continuous improvement in these activities, based on new science and experience, are now a primary focus of FDA’s efforts in this regard.

More vexing has been the effort to determine the correct policies for protecting Americans from possible exposure to the agent of BSE from FDA-regulated products (drugs, vaccines, medical devices, blood products, foods, cosmetics, dietary supplements) that may be manufactured using BSE-positive or BSE-high risk bovine materials. In addition, FDA and outside experts have spent considerable effort deciding the best policies regarding the potential exposure to the agent of BSE from blood or tissue transplantation from individuals who may be harboring the BSE agent because of past potential exposure to the agent .

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Major Focus Area 1:

Import Initiatives (specific for human and animal foods; also includes general import initiatives applicable to all FDA-regulated products)

Action Item 1: In coordination with USDA and Customs, issue Import Alert for certain animal feed products
Lead: Office of Regulatory Affairs, ORA (Center for Veterinary Medicine, CVM)

Action Item 2: In coordination with USDA and Customs, issue Import Bulletin for certain human food products, including dietary supplements
Lead: ORA (Center for Food Safety and Applied Nutrition, CFSAN)

Action Item 3: Convert the present Import Bulletin for certain human food products, including dietary supplements, to an Import Alert
Lead: ORA (CFSAN)

Action Item 4: Decide on need for further import bulletin or alert regarding veterinary medications or pet foods
Lead: ORA (CVM)

Action Item 5: Increased visual inspections at ports of entry
Lead: ORA

Action Item 6: Task force on smuggling of animal proteins – recommendations
Lead: ORA (Office of Criminal Investigations / Customs)

Action Item 7: Evaluate import filer applications (evaluating manifests submitted versus actual contents of shipments) and determine need for legislation to improve import oversight
Lead: ORA

Action Item 8: Determine need for legislation requiring labeling of country of origin of all components of imported FDA-regulated products – not just country of final assembly
Lead: ORA (Office of Policy, Planning, and Legislation, OPPL)

Action Item 9: Institute import filer training
Lead: ORA

Action Item 10: Upgrade certain aspects of information technology components of the OASIS System
Lead: ORA

Action Item 11: Complete initial inspections of all renderers, licensed feed mills, and known unlicensed feed mills
Lead: ORA (CVM)

Action Item 12: Complete re-inspections of all renderers, licensed feed mills, and known unlicensed feed mills who were found not be in compliance on initial inspection
Lead: ORA (CVM)

Action Item 13: Complete inventory and initial inspections of all unlicensed feed mills
Lead: ORA (CVM)

Action Item 14: Establish periodicity of re-inspections of those subject to 21 CFR 589.2000
Lead: CVM (ORA)

Action Item 15: Re-assess adequacy of feed rule educational program (i.e., strategies for obtaining and maintaining compliance with 21 CFR 589.2000)
Lead: CVM (ORA)

Action Item 16: Develop and implement testing program for imported feed
Lead: CVM (ORA)

Action Item 17: Develop and implement testing program for domestic feed
Lead: CVM (ORA)

Action Item 18: Enhance coordination of federal and state feed inspection activities (including contracts, training, and audits)
Lead: ORA (CVM)

Other action items related to this initiative

Action Item 19: Re-evaluate adequacy of present feed ban regulation (21 CFR 589.2000), including penalties for non-compliance
Lead: OC (OPPL / CVM / ORA / Department of Transpportation)

Action Item 20: Clarify policy on ruminant animals fed prohibited feed / Determine need for legislation relative to this matter.
Lead: OC (ORA / CVM / CFSAN / OPPL)

Action Item 21: Finalize disposition of elk meat from CWD-positive herd
Lead: ORA (CFSAN)

Action Item 22: Develop regulation on meat and other FDA-regulated products from animals with CWD or from CWD-positive herd, including need for legislation for compensation
Lead: CFSAN / Center for Drug Evaluation and Research ,CDER (CVM)

Action Item 23: Consider ban on ruminant brain and spinal cord as human foods or as part of human food (coordinate with CDER) (food elements of this proposal may be subsumed under Action Item 26 if that action item goes forward as proposed)
Lead: CFSAN

Action Item 24: Consider prohibiting meat products produced by mechanical recovery process and mechanical separation process on the head/skull and vertebral column
Lead: Office of the Commissioner, OC / CFSAN (USDA)

Action Item 25: Consider prohibiting meat products obtained from cows killed by air injection method of stunning at slaughter
Lead: OC / CFSAN (USDA)

Action Item 26: Consider prohibiting food products that contain "Specified Risk Products" and consider defining "specified risk materials as "skull, brain, eyes, spinal cord, tonsils of cattle 12 months or older; the entire intestine of cattle of any age, and the vertebral column of cattle 30 months or older"
Lead: OC / CFSAN (USDA)

Action Item 27: Consider prohibiting "Specified Risk Materials" from ALL animal feed (see definition of SRM in previous item)
Lead: CVM

Action Item 28: Re-evaluate status of certain tallow in human food products
Lead: CFSAN

Action Item 29: Re-evaluate status of certain gelatin products
Lead: CFSAN / CDER

Action Item 30: Re-evaluate status of certain milk and milk derivatives
Lead: CFSAN

Action Item 31: Re-evaluate salvagers’ use of certain pet foods in animal feeds
Lead: CVM

Action Item 32: Issue updated guidance on blood deferrals
Lead: CBER

Action Item 33: Develop guidance on cornea deferrals
Lead: CBER

Action Item 34: Develop guidance on bone marrow deferrals
Lead: CBER

Action Item 35: Develop donor suitability guidance on cells, tissues, and cell- and tissue-based products
Lead: CBER (Center for Devices and Radiological Health, CDRH)

Action Item 36: Develop and implement strategy for assuring compliance with guidance recommendations in action items 32, 33, 34, and 35
Lead: CBER (ORA)

Major Focus Area 3:

Major Focus Area 4:

Action Item 46: Establish mechanism for interagency strategic coordination on BSE and other TSE issues
Lead: OC

Action Item 47: Participate with USDA interagency coordination and sharing at staff level on specific issues
Lead: OC

Action Item 48: Establish mechanism for coordinating BSE/TSE efforts within FDA and for sharing information across various FDA components
Lead: OC

Action Item 49: Organize public meeting for consumers on BSE/TSEs
Lead: OC

Action Item 50: Draft letter from Commissioner to feed producers encouraging adherence to feed rule
Lead: OC (CVM / ORA)

Action Item 51: Develop position on private sector certification scheme (determine FDA involvement, if any)
Lead: CVM (ORA)

Action Item 52: Consider recognition in some way of those entities that are helping assure compliance by their own actions (outside FDA’s compliance actions) (e.g., only buying beef in compliance with feeding rule)
Lead: OC

Action Item 53: Establish point people within FDA for various outreach activities (press, legislative, department, international, states)
Lead: OC

Action Item 54: Develop FDA BSE Contingency Plan for BSE positive cow in the USA or person in USA positive for vCJD
Lead: ORA

Action Item 55: Coordinate with other agencies and undertake exercise to "dry run" test the contingency plan
Lead: ORA

Major Focus Area 5:

Action Item 56: Research on possible pre-mortem diagnostic tools
Lead: CBER / CDRH

Action Item 57: Research on possible decontamination / deactivation procedures
Lead: CBER / CFSAN

Action Item 58: Research on possible sterilization procedures
Lead: CBER

Action Item 59: Facilitate outside research on therapeutic, preventative, and diagnostic possibilities
Lead: CDER / CVM / CBER / CDRH

Action Item 60: Research on BSE agent recovery from and identification in foods and cosmetics
Lead: CFSAN

Action Item 61: Research on CWD
Lead: CFSAN

Action Item 62: Research on characterization and elimination of TSE infectivity in blood
Lead: CBER

Action Item 63: Consider contracting with outside party to perform risk analysis regarding TSEs and FDA-regulated products and to offer additional suggestions on ways to minimize such risk
Lead: OC

Action Item 64: Develop or facilitate development of test(s) for detection of prohibited materials in animal feed and feed ingredients
Lead: CVM