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CONGRESSIONAL REQUESTS
 

2008

Overview  

Individuals from the Creutzfeldt-Jakob Disease (CJD) community are visiting Members of Congress and their staff about CJD, a rare, degenerative and fatal brain disease. At present, there is no treatment or cure. CJD is reported to affect approximately one person, per million, per population, however, the unreported and undiagnosed number of cases remains unclear. One form of this disease (vCJD) is known to be caused by ingesting beef contaminated with Bovine Spongiform Encephalopathy (BSE) or “Mad Cow Disease.”  The discovery of two new strains of BSE in cattle born in the United States raises deep concerns regarding possible risks to humans.

CJD is one of several prion diseases receiving modest support through diffuse federal funding under the Departments of Agriculture and Health and Human Services.  We need your support to strengthen and continue the co-ordination of prion activities, and to assure the safety of the American food supply. 

Key Requests 

(1)    Centers for Disease Control and Prevention: Human Prion Disease Surveillance 

To keep pace with the growing demand for national surveillance of human prion diseases, especially in light of the drastic reduction in animal prion surveillance by the US Department of Agriculture and of the discovery of Bovine Spongiform Encephalopathy (BSE)-positive cows endangering human health, we are asking Congress to appropriate $5.7M in 2008 and $6.2M in 2009 in order for the Centers for Disease Control and Prevention to maintain human prion disease surveillance in FY 09 and FY 10.  This represents an increase of about $0.5M per year over the Administration’s budget request of about $5.2M for FY 09. 

Background:

The Centers for Disease Control and Prevention, Division of Viral and Rickettsial Diseases, part of the Department of Infectious Diseases, is responsible for all surveillance activities related to prion diseases in the United States.  A portion of the annual budget for CDC supported prion disease surveillance is allocated to the National Prion Disease Pathology Surveillance Center (NPDPSC) based at Case Western Reserve University in Cleveland, Ohio. 

The NPDPSC performs histological and immunohistochemical diagnostic examinations, as well as analyses of the prion protein and gene in tissues and body fluids to establish the presence and the type of the prion disease.  Between 350 and 400 cases are examined per year.  Diagnostic tests on cerebrospinal fluid are carried out on close to 3,000 cases per year.  All together, these tests allow for the definitive identification of the so-called variant Creutzfeldt-Jakob disease,  (the form acquired by eating prion-contaminated beef) as well as other possible forms of prion disease acquired by eating prion-contaminated elk and deer meat. All of these tests are free to families. The CDC also provides funding directly to eleven states to support its CJD surveillance activities. In addition, a growing number of states receive funding from the CDC for surveillance of chronic wasting disease (CWD), a prion disease recently discovered in deer and elk, helping the NPDPSC to monitor its possible transmission to humans. 

 In FY 06, the CDC received $5.5M for prion disease related programs. The FY 07 and FY 08 budgets for the same program were cut by $100,000 to $5.4M. The current Administration’s budget requests of $5.2M for FY 09, is a progressive decrease and reveals a disturbing trend which ignores what is arguably an avoidable danger to human health.

(2)    Increased BSE Surveillance, including mandatory inspection of all USDA approved meat packing facilities. 

Background:

With the recent discovery of illegal practices at the Westland/Hallmark meatpacking plant, the very real concern that one plant with five USDA inspectors on the premises wasn’t able to uncover the severe infractions exposed by the National Humane Society, leads to the logical conclusion that there may be other plants not in compliance. 

  

It is well known that cattle over the age of 30 months have a higher risk of developing BSE. The dangers to the American public from ingesting “Mad Cow” infected beef, a known vector of variant Creutzfedlt-Jakob disease (vCJD), have been greatly elevated with this discovery, as well as with the resumption of importation of Canadian cattle over the age of 30 months (there have been 13 cases of BSE in Canadian born cows over the age of 30 months, and one additional Canadian case was discovered in Washington state in 2003, making a total of 14 Canadian BSE cases identified thus far).  Cattle harboring BSE can be asymptomatic but can still transmit vCJD to humans, a 100% fatal disease. 

Presently, the USDA tests 40,000 U.S cattle per year for BSE. This number represents less than 1% of the 35 million head of cattle slaughtered each year for American consumption.  

We ask that Congress investigate the USDA policy regarding BSE surveillance as it affects human safety, and that BSE testing be increased to include all cattle over the age of 30 months.  

We further ask that Congress request the USDA to provide proof of inspection of all usda approved meat packing plants.  

     (3) We ask that Congress demand feasible inspection measures and accountability from the FDA regarding their regulations banning all brains, spinal cords and  other specified risk materials (SRM’s) from animal feed. (Title 21, Part 589.2000 of the Code of Federal Regulations known as the “Ruminant Feed Ban”).  These SRM’s pose a high risk of infectious agents reaching healthy stock.  

     (4) We ask that the FDA’s food functions be unified under one

agency. This one agency should be responsible for food safety, including inspection (at any stage) and accountability. There should be a single person responsible for reporting directly to the Secretary of Health and Human Services.  

Background (for numbers 3 and 4):

With limited USDA BSE surveillance and no incentive for farmers to turn in sick or dead stock for testing, there is a very real risk that BSE positive cows will be missed and/or ground up and utilized as a cost effective measure to feed animals on farms.  

Presently, 31% of our bovine imported products are from Canada, (Table 5 FDA Rules and Regulations, USDA Foreign Agriculture Service) a country which, as of June 2008, had reported 14 cases of BSE. Risks to the U.S food supply, both animal and human, significantly increased with the resumption of Canadian beef importation late last year. 

The recent discovery of illegal practices at the Westland/Hallmark meatpacking plant leads to the logical conclusion that there most likely are other plants not in compliance. The FDA does not conduct inspections in USDA regulated slaughter establishments, and the USDA acknowledges serious inspection problems. This situation can lead to a perfect storm; BSE cases possibly entering both cattle feed and the human food supply. 

Based on previous history, passing FDA regulations and not having feasible inspection measures in place is an exercise in futility.  “Existing food safety laws are vastly outdated” and “current laws focus mainly on reacting to safety hazards rather than preventing them” commented Michael Taylor, research professor of health policy at George Washington University and former administrator of the USDA’s Food Safety Inspection Service (FSIS) in an April 30, 2008 CIDRAP (Center for Infectious Disease Research and Policy) newsletter. 

These issues present a clear argument for stronger regulations and inspection procedures, as well as the need for review and restructuring. Presently, the FDA only conducts end-processing inspections which most likely led to the problems at the Westland/Hallmark Plant in Chino, California.

 
  © 2008 Creutzfeldt-Jakob Disease Foundation, Inc.