CONGRESSIONAL REQUESTS
Creutzfeldt-Jakob Disease Foundation Lobby Day
July 10, 2006
Overview
The CJD community is on Capitol Hill today to educate Members of Congress and their staff about Creutzfeldt-Jakob Disease (CJD), a rare, degenerative and fatal brain disease. At present there is no 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 from ingesting beef infected with Bovine Spongiform Encephalopathy (BSE) or "Mad Cow Disease". The discovery of an atypical strain of BSE in U.S. born cows raises deep concerns regarding increased possible risks to humans.
CJD is one of several prion diseases receiving modest support through diffuse federal funding under the Departments of Agriculture, Defense, and Health and Human Services. We need your support to strengthen and continue the co-ordination of prion activities.
Key Requests
(1) Centers for Disease Control and Prevention
To keep pace with the growing demand for national surveillance of prion diseases (especially in light of the discovery of BSE positive cows which create risks to human life, and the growing number of states with CWD infected animals) we are asking for $6.5 million in funding for prion disease surveillance and research at the Centers for Disease Control and Prevention in FY 07, an increase of $1M. We ask that a significant amount of this funding be used for human prion disease surveillance.
Background:
The Centers for Disease Control and Prevention, Division of Viral and Rickettsial 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. The NPDPSC performs special diagnostic tests, all of which are free to families. These tests include both pre and post mortem testing for variant CJD (the human form of “mad cow disease”). The CDC also provides funding directly to ten states to support its CJD surveillance activities. A growing number of states also receive allocations from the CDC for surveillance of chronic wasting disease (CWD) in deer and elk and research into its possible transmission to humans.
In FY '06 the CDC, Division of Viral and Rickettsial Diseases received $5.5 million for prion disease related programs and activities. This amount was an increase of $800,000 over FY '05.
(2) Federal Prion Disease Interagency Working Group on Prion Science
We ask that the federal Interagency Working Group on Prion Diseases continue its work for a further two year period.
Background:
The Department of Health and Human Services created an Interagency Working Group (IWG) on Prion Diseases in January 2005 to be in effect for a two year period and is scheduled to be dissolved in January 2007. The IWG was created to oversee the work of government agencies involved in prion disease research, surveillance and prevention. Its goals are to improve communication and coordination among all federal agencies engaged in prion research and management of associated transmissible spongiform encephalopathies.
(3) Heightened BSE Surveillance
a) We ask that the USDA initiate a BSE testing pilot program allowing cattle processors to voluntarily test cattle they slaughter with USDA supplied or approved test kits in government accredited laboratories.
b) We ask that the USDA test 1) all cattle of any age showing signs of central nervous system disease; 2) continue high intensity testing of all cattle 30 months or older; and 3) test a random sampling of healthy animals 20 to 30 months old (which reflects the standards of the European Union).
Background:
The dangers to the American public from ingesting "Mad Cow" infected beef have now been elevated with the discovery of an atypical strain of Bovine Spongiform Encephalopathy (BSE) in two U.S. cows. Presently the USDA has increased its BSE testing from 40,000 in 2003 to approximately 740,000 cattle over the past 18 months. 35 million head of cattle are slaughtered each year for American consumption.
We acknowledge the increased BSE testing initiated by the USDA and are aware of the high costs needed to implement this increased testing. We understand that the USDA must fund numerous other efforts to control animal diseases and protect human health in addition; therefore, we are calling for a program of voluntary government validated outsourcing for BSE testing. Some private cattle processors have already stated publicly that they would pay the costs of voluntary testing thereby providing significant cost reduction to the federal government. This pilot program would help to increase beef safety and elevate the confidence of the American public and our trading partners.
(4) Mandatory Animal Identification System
We ask that Congress require immediate implementation of a mandatory animal identification and tracking system to be monitored by the USDA.
Background:
Contaminated animal feed is the recognized source of most BSE infections. It is most unlikely that only one animal per herd would ingest contaminated feed, a known vector of BSE. Because there was no mandatory tracking system in place, the USDA was not able to locate the birth herds for the two BSE positive cows (in spite of the rigorous efforts of APHIS) and the risk to consumers that these cattle might enter the food chain greatly increased. A mandatory animal tracking system (such as those in Japan, the United Kingdom and other EU countries) will ensure limited exposure to BSE, help to reassure our trading partners and serve to eliminate the possible erosion of consumer confidence.
