This was sent to me and I want to share it with this list.
The Centers for Disease Control and Prevention (CDC) Creutzfeldt-Jakob
A Congressional Mandate
In response to concerns that CJD may be transmitted through blood or
blood products, the U.S. Congress requested that the
CDC conduct a study to assess whether CJD is a threat to the safety of
the nation's blood supply. Researchers believe that
the risk of transmission to humans through blood products is very
small since there have been no known cases of CJD
contracted by humans in this manner. However, precautions are being
taken to assure that the blood supply is safe from this
infectious agent. Current blood safety policy requires that any blood
products made from blood donated by a person who
later develops CJD, or is found to have risk factors for CJD, must be
withdrawn. Until the question of transmissibility is
resolved, the availability and the price of blood products will
continue to be adversely impacted by shortages caused by
recalls and the destruction of blood products which, to date, has cost
over $100 million.
The Program: How You Can Help
Because the signs and symptoms of CJD may not develop for up to 30
years, a person could be infected and not show any
symptoms during his or her lifetime. Furthermore, there is no
screening test available for CJD and the only sure way to test
for CJD is by analyzing brain tissue after death. The CJD program asks
families of individuals who have received blood
products to donate brain tissue after their death. The brain tissue
will be shipped to Stephen J. Armond, M.D., Ph.D.,
Professor of Neuropathology, University of California, San Francisco
to test for evidence of CJD. Other brain tissue will be
stored at CDC in Atlanta for analysis in the future when more is known
about the cause of CJD.
Participation in the CJD program is voluntary. The CDC is aware that
the death of a family member is a difficult time to make
important decisions. Therefore, the CDC is working through your
regional coordinator, physician, and treatment center staff to
provide support to you and your family as you discuss brain tissue
donation. To help with making an informed and rational
decision, the CDC has developed information packets which answer
commonly asked questions about the program including:
how to authorize the donation; how confidentiality will be assured;
the ability to retrieve the brain tissue without disfigurement
and without affecting funeral arrangements; and the time frame for
receiving test results.
Why Should I Participate?
Participation in this project is the only current way to help
determine whether CJD can be or has been transmitted by blood
products. If results show that CJD is not transmitted through blood
products, needless recalls and shortages could be
avoided. On the other hand, if this study shows that CJD may be spread
through blood, research could then focus on the
development of screening tests in an effort to maintain a safe blood
supply. This project is the response of Congress and the
CDC to avoid a potential threat to the current members of our
community who use blood products and future generations
who will rely on a safe blood supply.
If you would like more information or would like to participate in the
CJD program, please contact Tami Wood-Lively,
HFM's Regional Coordinator, in Michigan at (800)482-3041, ext.29 or if
you are outside Michigan, at (734)761-2535,
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WHAT CAN YOU DO?
1. Stay informed. Read the information you receive, and the news
reports about CJD research. Call HFM to get the latest
information and access to resources on the internet.
2. Participate in the CDC Creutzfeldt-Jakob Disease Program. While it
will not directly help you, it will provide valuable
information that can protect future generations. PLEASE read the
following article about the program.
3. Become an activist. The National Hemophilia Foundation (NHF) and
other groups are working on efforts to promote an
improved system of patient notification for product recalls and
advocacy to reduce the size of plasma pools, thus lessening the
risk of exposure to pathogens. Call HFM or NHF to find out what you
4. Be prepared for change. Be aware of when safer products come on the
market. If appropriate, discuss changing to these
products with your health care providers.
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About the Prion......
Stanley B. Prusiner, MD, building on decades of research by many other
scientists, proposed the idea of a "prion" over 20
years ago. The idea that an infectious agent could be caused by
nothing but protein met with much skepticism. A protein
cannot replicate itself like a virus or bacteria, so any ability for
it to be infectious was considered impossible. Although Prusiner
could not explain at the time exactly how these abnormal prion
proteins caused other normal proteins around them to mutate
into disease causing
abnormal proteins, he still felt it was the best explanation for
certain degenerative disorders of the central nervous system.
Even today, the exact mechanism for this mutation is unclear. However,
despite the fact that some scientists still do not believe
the prion theory, Dr. Prusiner was awarded the Nobel Prize in Medicine
Prusiner comments that he first suspected that an infectious agent
could lack nucleic acid (which drives replication) in the early
1970's, when studying "scrapie," the spongiform encephalopathy that
occurs in sheep. There were many clues - evidence that
scrapie could not be inactivated by procedures known to damage nucleic
acid, an inability to find any evidence of a virus, and
others. If this infectious agent lacked nucleic acid, what WAS it made
of? Thus the notion of a prion was born. This idea has
fostered decades of research and discovery. Dr. Prusiner has continued
his investigation of prions at his laboratory at the
University of California School of Medicine, San Francisco. He states
that his studies, among others "argue persuasively that
the prion is an entirely new class of infectious pathogen."
Scientific American, January 1995, Volume 272, Number 1 Pages 48-57
Normal prion protein
twisted helical shape
Abnormal prion protein
From: Huang, Z., Prusiner, S.B. and Cohen, F.E. (1996) Structures of
prion proteins and conformational models for prion diseases IN Prions
Prions Prions (ed. S.B. Prusiner) Berlin: SpringerVerlag.
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