The National Prion Disease Pathology Surveillance Center
Sponsored by the American Association of Neuropathologists (AANP)
Office: Advisory Committee:
Dr. Pierluigi Gambetti, Director Dr. Barbara Crain
Dr. Bernardino Ghetti, Co-Director Dr. Stephen DeArmond
Dr. Jiri Safar, Co-Director Dr. Daniel Perl
Dr. Wenquan Zou, Associate Director
Dr. Clive Hamlin, Director, Clinical Laboratory
SUBCOMMITTEE ON LABOR, HHS, EDUCATION AND RELATED AGENCIES CONGRESSIONAL
REQUEST FOR PROGRAMMATIC FUNDING FY2013
July 2012
Dear Representative,
We respectfully ask for consideration of the appropriation of $ 5 M in FY13
for the Centers for Disease Control and Prevention (CDC) to continue the program
for national surveillance of human prion diseases.
The President’s budget proposal for FY 2013 combines the prion program with
that Chronic Fatigue Syndrome (CFS), a non-fatal, non-transmissible condition,
and suggests a 25% cut in the appropriation over that of FY12. We ask that the
prion program appropriation for FY13 be separated from that of the Chronic
Fatigue Syndrome and that it be subject to no more than a 5-10% reduction. Any
larger reduction in one FY would make the current surveillance incomplete
rendering it essentially useless.
Discontinuing national surveillance on human prion diseases will have at
least four major negative consequences: 1. The identification of U.S. cases in
which the prion disease has been acquired from eating prion contaminated meat
will become unlikely. Possible sources of prion infection are cattle as well as
deer and elk affected by BSE (bovine spongiform encephalopathy or Mad Cow
disease) and CWD (chronic wasting disease), respectively. Since the US is not
conducting significant surveillance for BSE and CWD, the occurrence of a BSE or
CWD epidemic may remain completely undetected.
2. Detection of cases of prion disease acquired from the use of
prion-contaminated surgical instruments, tissue and organ transplants, blood
transfusion and other medical procedures will also be jeopardized allowing
potential spread of prion infection to remain unchecked.
3. The occurrences of clusters of cases of prion disease which point to a
possible source of prion infection, which are often sensationalized by the media
will not be definitively resolved possibly maintaining a state of alarm and
eroding confidence in the government’s public health protection and
safety.
4. The U.S. would become the first industrialized country with no prion
surveillance of any kind putting the country at a major disadvantage in the
export of beef and beef products. Indeed the recent resumption of beef
importation by the government of South Korea was largely predicated on the
existence of effective human prion disease surveillance (see attachment).
CONCLUSION: Discontinuing or reducing human prion disease surveillance
would not only eliminate an important safety net to US public health, but it
would also jeopardize the export of US beef.
Sincerely,
Pierluigi Gambetti, MD Florence Kranitz Professor and Director President
National Prion Disease Pathology Surveillance Center CJD Foundation, Inc. Case
Western Reserve University 3632 W. Market St., Ste 105N 2085 Adelbert Road, Room
419, Cleveland, OH 44106 Akron, Ohio 44333 Email: Pxg13@case.edu Phone:
330-665-5590
Wednesday, June 27, 2012
First US BSE Case Since 2006 Underscores Need for Vigilance
Neurology Today 21 June 2012
Tuesday, June 26, 2012
Creutzfeldt Jakob Disease Human TSE report update North America, Canada,
Mexico, and USDA PRION UNIT as of May 18, 2012
type determination pending Creutzfeldt Jakob Disease (tdpCJD), is on the
rise in Canada and the USA
previous USA PRION UNIT reports ;
TSS
