CREUTZFELDT-JAKOB DISEASE FACT SHEET 1/1998
BY DEBORAH ONEY, MASTER OF SOCIAL WORK
CO-FOUNDER, BLOOD RECALL/WITHDRAWAL - CJD
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Creutzfeldt-Jakob Disease (CJD) is an infectious rapidly progressive fatal brain-deteriorating disease for which there is no treatment or cure. Most scientists believe CJD is caused by a prion, which is a protein. People in the United States and throughout the world get CJD. One strain nvCJD is thought to be related to bovine spongiform encephalopathy (i.e. Mad Cow Disease) in the United Kingdom. To date no nvCJD cases have been observed in the United States. Normal sterilization procedures do not inactivate the CJD infectious agent. The incubation period varies greatly with symptoms emerging in known cases in 15 months to 30 years after exposure. There is no preclinical test to determine if infection took place after a possible exposure.
WHO GETS CJD AND HOW?
CJD most frequently appears in people ages 50-75. However, there are victims in their 40s, 30s and even 20s. The officially stated mortality rate of about one person per million per year appears to be an under statement since CJD often escapes detection. In a Yale University study 13% of clinically-diagnosed Alzheimer patients were found on autopsy to have CJD. There are three forms of CJD: familial (about 5-10% of cases), sporadic (cause unknown, about 90-95%) and iatrogenic (caused by a medical procedure such as contaminated cadaver-derived growth hormones, dura mater implants, and cornea transplants, less than 1%). Athletes who use cadaver-derived growth hormones are at risk for CJD.
People receive blood products in transfusions, vaccines, diagnostic tests and InVitro Fertilization cultures. The Centers for Disease Control is currently conducting a study to determine if CJD is transmitted to humans by blood. The infectious agent has been found in blood, but there have been no documented cases of blood transmission in humans. As a precaution blood relatives of CJD victims and people at risk for iatrogenic or familial CJD should not donate blood.
Blood product withdrawals due to theoretical CJD risk have resulted in many people receiving blood product withdrawal notifications. These notifications can cause a great deal of anxiety for recipients.
WHAT ARE THE SYMPTOMS OF CJD AND HOW IS IT DIAGNOSED?
Classical CJD symptoms are a rapidly progressive presenile dementia; involuntary, irregular jerking movements; and progressive motor dysfunction. Early symptoms can include failing memory, behavior changes, visual disturbances, and lack of coordination. The duration of CJD from the onset of symptoms to death is usually less than a year. 14-3-3 spinal fluid tests and brain biopsies are tests used to support a clinical diagnosis of CJD in symptomatic patients. However, an autopsy is the only definitive way of diagnosing CJD.
CJD AND THE INTERNET
Blood-CJD is an e-mail group which was formed to support blood withdrawal notification recipients. The group website has CJD information and is located at http://members.aol.com/debbieoney/blood.htm If you do not have internet access, you can join by e-mailing DebbieOney@aol.com or email@example.com.
The Many Faces of CJD website is a collection of personal experiences with CJD. The website is at http://members.aol.com/stacy91434/cjd/cjd.htm
The CJD Watch website attempts to track cases throughout the world. The website also has a Victims' Profiles Database. It is at http://members.aol.com/stacy91434/watch/watch.htm
RESOURCES USED IN WRITING THE FACT SHEET
Brainum, Jerry. Growth Hormone: Certain Forms Could Be Deadly: GH Derived from Cadavers Could Incubate Lethal Brain Cancer - And It's Still Available in Europe! Muscular Development. September, 1998; 146.
The Centers for Disease Control and Prevention (CDC) Creutzfeldt-Jakob Disease Program: A Congressional Mandate (This is at http://members.aol.com/larmstr853/cjdvoice/cdcprog.htm)
Dealler, Dr. Steven, "Terms used by the scientists and explained as simply as possible." (This is available at http://www.airtime.co.uk/bse/defb.htm).
The Food and Drug Administration Center for Biologics Evaluation and Research "Revised Precautionary Measures to Reduce the Possible Risk of Transmission of Creutzfeldt-Jakob Disease (CJD) by Blood and Blood Products - December 11, 1996." (This is available at http://www.fda.gov/cber/bldmem/mem121196.txt)
Hill, Andrew F.; Melanie Desbruslais; Susan Joiner, Katie C. L. Sidle, Ian Gowland; John Collnge; Lawrence J. Doey; and Peter Lantos. "The same prion strain causes vCJD and BSE". Nature.389 (October 2, 1997). 448 - 450.
Hsich, Gary; Kimbra Kenney; Clarence J. Gibbs, Jr.; Kelvin H. Lee; Michael G. Harrington. The New England Journal of Medicine -- September 26, 1996 -- Volume 335, Number 13. "The 14-3-3 Brain Protein in Cerebrospinal Fluid as a Marker for Transmissible Spongiform Encephalopathies."
Manuelidis, Elias E. and Laura Manuelidis, "Suggested Links between Different Types of Dementias: Creutzfeldt-Jakob Disease, Alzheimer Disease, and Retroviral CNS Infections" Alzheimer Disease and Associated Disorders_ 3 (1989): 100-109
National Institute of Neurological Disorders and Stroke. "Creutzfeldt-Jakob Disease: Fact Sheet". (This is available at http://www.ninds.nih.gov/healinfo/DISORDER/CREUTJAB/cjd.htm#gethelp)
National Institute of Neurological Disorders and Stroke, "Creutzfeldt-Jakob Disease for Health Care Workers and Morticians", July, 1997. (This is available at http://members.aol.com/larmstr853/cjdvoice/ninds.htm)
Ricketts, Maura N., Neil R. Cashman, Elizabeth E. Stratton, Susie ElSaadany, Laboratory Centre for Disease Control, Health Canada, Ottawa, Ontario, Canada; Montreal Neurological Institute, Montreal, Canada "Is Creutzfeldt-Jakob Disease Transmitted in Blood?" 05/14/97 Emerging Infectious Diseases National Center for Infectious Diseases Centers for Disease Control and Prevention Atlanta, GA. (This is available at http://www.cyber-dyne.com/~tom/blood_cjd.html)
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