Creutzfeldt-Jakob Disease and Blood

DebbieOney DebbieOney at AOL.COM
Thu May 7 17:01:58 EST 1998

This was sent to me and I want to share it with this list.

The Centers for Disease Control and Prevention (CDC) Creutzfeldt-Jakob
     Disease Program

     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     withdrawn.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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     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
     can do.

     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
     for 1997.

     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
                            flattened sheets

     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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