Congressional Requests

CONGRESSIONAL REQUESTS

Creutzfeldt - Jakob Disease Foundation Advocacy Day

July 16, 2012

Overview

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, Defense, 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 beef contaminated with Bovine Spongiform Encephalopathy (BSE), more commonly known as “Mad Cow” disease.   In April of this year the fourth U.S. case of BSE was announced. The unusual form of BSE found in this cow 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 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.

Key Requests

1. To maintain the important program for national surveillance of human prion diseases, especially

in light of the limited animal prion surveillance carried out by the U.S. Department of Agriculture and the discovery of the fourth BSE positive cow representing a potentially heightened human health risk, we are asking Congress to appropriate $5M in FY13 for the Centers for Disease Control and Prevention (CDC) to continue its critically important human prion disease surveillance program.

Creutzfeldt - Jakob Disease Foundation Advocacy Day

July 16, 2012

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                Background:

In FY 2012 approximately $ 5 million (about an 8% decrease compared to previous year) was appropriated by Congress for the CDC prion program; of this appropriation, $2.5 million is expected to be awarded to the NPDPSC.  For FY 2013 an appropriation of $5M, which, although minimal, would allow the NPDPSC to survive and continue to develop more efficient detection methods while providing the same level of prion surveillance.

2. The President’s budget proposal for FY 2013 combines the prion program with the chronic fatigue syndrome (CFS) program and suggests a 25% cut in the appropriation over that of FY 2012.

We ask that the appropriation for the NPDPSC for FY 2013 be separated from that of the Chronic Fatigue Syndrome (CFS) and be subject to no more than 5-10% reduction. If this combined appropriation and decrease goes forward it would cut the NPDPSC budget for FY 2013 down to  1.875 million jeopardizing its very existence.  

Background:

While CFS is a debilitating and mysterious syndrome it bears no known relationship to prion diseases. CFS is not fatal, does not attack the brain nor is it infectious or transmissible which are all hallmarks of prion diseases.

3. We ask Congress to investigate the US Department of Agriculture (USDA) , Animal Plant Health Inspection Service’s (APHIS’) proposal to drop the significant protections the U.S. has against the importation of cattle and beef from countries where bovine spongiform encephalopathy (BSE or “Mad Cow” disease) is known to exist.  APHIS is proposing to open United States’ borders to cattle and beef from countries that have had thousands of cases of BSE in cattle, more than 200 cases of vCJD in humans, and where new cases of BSE and vCJD continue to be found.  (Docket No. APHIS-2008-0010: Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine Products, (Proposed Rule), published at 77 Fed. Reg. at 15,848-913.)

Further, we ask Congress to investigate the refusal by USDA to have granted our reasonable request for an extension of the public comment period for the Proposed Rule to July 15, 2012,

                Background:    

In March of this year the USDA announced that the United States’ BSE-related import restrictions would be lifted for a number of countries, including the United Kingdom, Ireland, Spain, Portugal, and the Netherlands. According to the World Organization for Animal Health (OIE), those five countries reported 22 new cases of BSE-infected cattle in 2011.  Also in 2011, 5 deaths attributed to vCJD were documented in the United Kingdom.  

The Proposed Rule is voluminous in size and exceedingly complex in its rationale. It represents a sweeping change to U.S. standards regarding the prevention of BSE in U.S. cattle and vCJD in U.S. citizens. To initiate these changes while ignoring our reasonable request for an extension of the public comment period strongly suggests the USDA is favoring special interest groups over human health and safety.