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Creutzfeldt - Jakob Disease Foundation Advocacy Day
July 14, 2014
The Creutzfeldt-Jakob Disease (CJD) community is on Capitol Hill today to inform Members of Congress and their staff about Creutzfeldt-Jakob Disease (CJD), a rare,100% fatal, degenerative brain disease which presently has no treatment or cure. CJD is reported to affect approximately one person per million per population worldwide, however, the unreported and undiagnosed number of cases remains unclear.
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 coordination of prion activities and to assure the safety of the American public and the U.S. food supply.
One form of this disease in humans, variant CJD (vCJD), is known to be caused from ingesting tissues in beef contaminated with Bovine Spongiform Encephalopathy (BSE), more commonly known as “mad cow” disease. In April of 2013 the fourth U.S. case of BSE was announced. The unusual form of BSE found in this cow and the limited and random testing for BSE by the USDA (one cow per 40,000) adds another layer to the already deepening concerns regarding possible risks to humans.
Human surveillance through brain tissue examination is the only way to determine the incidence and possible changes occurring in human prion diseases and also to determine if the spread of a similar disease, Chronic Wasting Disease (CWD) found in U.S elk and deer has become a human risk. The National Prion Disease Pathology Surveillance Center (NPDPSC) which performs all of the necessary testing, is funded through the Centers for Disease Control and Prevention (CDC). The NPDPSC, located at Case Western Reserve University in Cleveland, Ohio acts as our first line of defense against the possibility of an undetected U.S. human prion disease epidemic as experienced in the United Kingdom.
- We ask for Congressional support in maintaining the National Prion Disease Pathology Surveillance Center’s (the NPDPSC) appropriation for FY 2015 at a level that does not decrease below the FY 2014 appropriation. This would allow the NPDPSC to meet increasing autopsy costs and continue to develop more efficient detection methods while providing an acceptable level of prion surveillance. Reduction of funding to the NPDPSC would eliminate an important safety net to US public health making the US the only industrialized country lacking prion surveillance which in turn could jeopardize the export of US beef.
The NPDPSC is funded entirely by the Centers for Disease Control and Prevention (CDC) from funds allocated by Congress. The CDC traditionally keeps approximately half of the appropriation. Below are the FY10-13 Congressional appropriations allocated to CDC for the prion program.
FY10 Funding: ~$5.474M
FY11 Funding: ~$5.373M
FY12 Funding: ~$4.969M
FY13 Funding: ~$4.960M
FY14 Funding: ~$4.9M
Although the 2014 appropriation of $4.9M is a reduction it will allow the Center to survive and continue to develop more efficient detection methods while providing an acceptable level of prion surveillance. If the reductions are higher, rigorous national prion surveillance will be impossible.
The National Prion Pathology Surveillance Center (NPDPSC) is the only organization in the US that monitors human prion diseases and is able to determine whether a patient acquired the disease through the consumption of prion contaminated beef (“mad cow” disease) or meat from elk and deer affected by chronic wasting disease (CWD). CWD is an endemic prion disease of elk or deer in the United States.
The NPDPSC also monitors cases in which a prion disease might have been acquired by infected blood transfusion, from the use of contaminated surgical instruments or from contaminated human growth hormone.
The NPDPSC also plays a decisive role in resolving suspected cases or clusters of cases of food acquired prion disease that are often magnified by the media stirring intense public alarm. To date, the NPDPSC has examined over 5,000 suspected incidents of prion diseases and has definitely confirmed presence and type of prion disease in 3,061 cases.
The NPDPSC represents the last line of defense in safeguarding US public health against prion diseases because the United States ─ unlike other BSE affected countries such as the United Kingdom, the European Union and Japan ─ does not have a sufficiently robust animal prion surveillance system.
The NPDPSC offers assurances, to the countries that import (or are considering importing) meat from the United States, that the U.S is free of indigenous human cases of “mad cow” disease. Recently South Korean health officials resumed importation of US beef to their country after a visit to the NPDPSC provided assurances regarding rigorous human prion surveillance (see letter).
(2) We ask for a Congressional investigation regarding the adequacy of the USDA's BSE surveillance program and its oversight of human safeguards against BSE.
Currently the primary USDA’s safeguard against BSE infected beef entering the U.S food supply is by the removal of specified risk materials (SRMs) - or the parts of an animal that would contain BSE - from every animal at slaughter.
On June 11, 2014, the Department of Agriculture’s Food Safety and Inspection Services (FSIS) announced that Fruitland American Meat of Jackson, MO, issued a recall of approximately 4,012 pounds of fresh beef products because “the dorsal root ganglia may not have been completely removed, which is not compliant with agency regulations that require their removal in cattle 30 months of age and older”.
Although the agency said that none of the animals displayed signs of BSE before slaughter the reality is that unless an animal shows advanced signs of illness prior to entering the slaughter process, it would be very difficult to detect a sick animal due to the huge number of animals moving together towards slaughter. In her letter to USDA Under Secretary Brian Renholm, Congresswoman Rosa DeLauro (CT) questioned why it took 10 months for the error to be discovered, citing other recent examples of beef recalls over an extended period of time, such as the Wolverine Packing Co. and the Rancho Feeding Corporation recalls.
The incubation period for the human form of “Mad Cow” Disease or variant CJD after exposure to contaminated beef can be up to 20 years. The USDA in their statement regarding the June 11th beef recall characterized the situation as a “low-risk, class two recall because no illnesses have been reported”.
The USDA BSE surveillance system depends on independent processing plants with, what appears to be, more interest in protecting their USDA contract than protecting the U.S. public from possible exposure to this fatal disease.
A 2014 FSIS audit report of Brazilian meatpacking plants that export beef to the United States reveals that in 2007 Brazil relaxed its SRM removal policies by issuing a notice that inexplicably removed the skull, trigeminal ganglia, vertebral column, and dorsal root ganglia in cattle 30 months of age or older from the list of SRMs that must be removed at slaughter.
Despite Brazil’s failure since 2007 to consistently implement the United States’ mandatory requirement that all SRMs be excluded from the human food chain, FSIS officials nevertheless determined that Brazil “continues to meet FSIS equivalence criteria at an adequate level for this component (the SRM removal component).”
Given the United States’ modest animal prion surveillance program and the known breaches of mad cow disease safeguards for humans, both domestically and internationally, Congress should call for an investigative hearing to determine the appropriate standards that USDA must meet to adequately protect the United States from a BSE outbreak.