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"We've already taken numerous precautionary steps at the federal and local levels to prevent BSE from occurring in the U.S. food supply, but we must continue to strengthen our understanding of this disease," said Secretary Thompson. "This plan lays out a course of action to expand our understanding of the underlying science of BSE and its potential for transmission to humans."

BSE (bovine spongioform encephalopathy), first identified in 1986 in the United Kingdom, is a fatal disease that causes progressive neurological degeneration in cattle. It is one of a family of diseases called TSEs, or transmissible spongioform encephalopathies, named for the sponge-like gaps that develop in the brain tissue of diseased animals or people. One TSE disease that affects humans is called Creutzfeldt-Jakob Disease (CJD), and a form of this disease, variant CJD (vCJD), is probably related to the BSE disease of cattle. There is strong scientific evidence that the agent that causes BSE in cattle is the agent that causes vCJD in people. So far, there have been cases of vCJD reported in the United Kingdom and elsewhere in Europe, believed to occur in people who consumed beef products contaminated with the infective BSE agent. There are no reported cases of BSE or vCJD in the United States.

"The number of people known to suffer this disease remains extremely small, with no cases involving residents of this country. The basic facts about BSE and other TSEs are not well understood, so it's important for us to learn more as quickly as we can. And at the same time, we must continue to carry out effective steps to keep BSE out of the American food supply," Secretary Thompson said.

The Secreatary's action plan released today outlines four areas of responsibility-surveillance, protection, research and oversight-within HHS. This effort will be coordinated with other government agencies, the private sector, and the international community to contain this epidemic and assist those affected by it.

• Surveillance. The Centers for Disease Control and Prevention (CDC) will enhance its current program to identify and investigate possible cases of vCJD. Through cooperative agreements with state and local health departments, CDC also will enhance and expedite the oversight of illness and deaths from CJD so that any possible vCJD cases will be rapidly detected. CDC will also increase its technical assistance to state and local health personnel, develop new laboratory capacity to support its investigations and enhance its current collaborative agreement with the National Prion Disease Pathology Surveillance Center at Case Western Reserve University.

• Protection. The Food and Drug Administration, in partnership with the U.S. Department of Agriculture (USDA), oversees efforts to prevent transmission of BSE and other TSEs through food products. FDA will review and expand its import inspection programs to keep potentially infected food products out of the U.S., and its animal feed inspection program to prevent the use of mammalian protein in feed for ruminant animals such as cows and sheep. FDA will also continually review and upgrade its policies designed to prevent potential exposure to vCJD and other TSEs through blood transfusions and tissue transplantation. Additionally, FDA will broaden its policies where appropriate to prevent potential transmission through FDA-regulated products including drugs, medical devices, vaccines and other biological products, cosmetics, food and food additives and dietary supplements.

• Research. Under the new action plan, the National Institutes of Health (NIH) will more than double its current spending for research on TSEs, including BSE and vCJD, by the end of fiscal year 2002. Goals of the NIH research program include: understanding prions, the abnormal proteins that cause TSEs; learning how TSEs are transmitted between animals of one species and between different species; developing diagnostic tests for animals and people, including an effective vCJD screening test; and designing drug treatments. NIH plans to double the laboratory facilities available over the next two years and triple the number of investigators involved in this research over the next five years.

• Oversight. In addition to providing effective program support to HHS agencies in these efforts, the department will take any steps necessary to assure the public with timely, accurate and thorough information about the potential threats posed by BSE and vCJD and about the actions each agency is taking to protect the public from these threats. The department also will continue to support several advisory committees that review issues related to BSE and vCJD, as well as those related to the safety and adequacy of the national blood supply.

The Secretary's action plan is on the Web at: frwebgate.access.gpo.gov/cgi-bin/getdoc.cgi?dbname=2001_register&docid=01-21145-filed.

Additional information on BSE is available from the following resources:
HHS Fact Sheet: www.hhs.gov/news/press/2001pres/01fsbse.html
FDA Web site: www.fda.gov/oc/opacom/hottopics/bse.html
CDC Web site: www.cdc.gov/ncidod/diseases/cjd/cjd.htm

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