(continued) Blood Safety

The American Red Cross (ARC) is knowledge and reporting, unfortunately, denying blood donations from combines to fuel those fears. individuals who have spent six months From a public policy perspective, a deeper or more in Europe since 1980, as well understanding of CJD - both its causes and as that of any blood relative of a CJD the rate of incidence, would provide direction victim. Sporadic CJD is not believed to for scientists in their goals to better be transmissible through blood, understand prion diseases and the ultimate however, the ARC is taking strict goal to find the cure. precautions. The first case of suspected blood transfusion transmission occurred in the U.K in 2003 through blood donated from a pre -symptomatic variant CJD victim. Even Chronic Wasting Disease (CWD) though scientists can’t provide positive Chronic Wasting Disease was first proof that this case was transfusion- diagnosed in 1967 in Colorado, and it has transmitted, in order to protect public subsequently been found in captive herds in health this should be considered the several other areas. The source of infection first case. There have been two other for wild and captive deer and elk in new ge- cases of suspected transfusion- ographical areas is unknown in many in- transmission in the U.K. In 2009, the stances. While it is not known exactly how U.K. authorities also reported possible CWD is transmitted, lateral spread from transmission of vCJD through a treat- animal to animal through shedding of the ment for hemophilia produced from infectious agent from the digestive tract ap- blood plasma. pears to be important, and indirect

transmission through environmental contam- Other Concerns ination with infective material likely. CWD

The public is at risk because the has not been proven to cross the species federal surveillance system for mon- itoring CJD and BSE incidence is poor- ly funded and fragmented. There is no uniform reporting mechanism in place and each state sets its own require- ments. The public is at risk because The CJD Foundation, Inc. so little is known about how to detect PO Box 5312 and treat CJD. Until the gap in that Akron, Ohio 44334 HelpLine 1.800.659.1991 continuum is closed, the public will re- 1.800.659.1991 main at risk. The linkage of CJD, BSE, www.cjdfoundation.org the blood supply, and the lack of www.cjdfoundation.org [email protected] [email protected] What is CJD? Public Health Concerns

CJD is NOT “Mad Cow Disease.” Creutzfeldt-Jakob Disease (CJD) Bovine Spongiform Encephalopathy is a rare, fatal brain disorder. The (BSE), the technical term for Mad incidence of CJD cases Cow Disease, occurs only in cows. worldwide is one case, per Causes of CJD million, per population. In the Sporadic CJD The first documented case of BSE United States this statistic  Unknown found in the United States occurred in translates to approximately 300 Washington State in December 2003 in new cases per year. Eighty-five Familial CJD a cow imported from Canada. The first percent of the cases are  Genetic mutation inherited from a parent endemic case was found in Texas and sporadic, meaning there is no was announced in 2005. Eating known cause at present. Iatrogenic CJD infected beef is widely believed to be  Contaminated surgical instruments the cause of the variant form of CJD There are three types of CJD:  Contaminated dura mater transplant (vCJD) in humans. vCJD usually  Contaminated corneal transplant  Sporadic (sCJD) affects young people. An endemic  Contaminated human growth hormone  Familial (fCJD) case has not yet been documented as

 Acquired (vCJD or iCJD) Variant CJD originating in the United States.

 Contaminated beef In the early stages of the disease, Is the public at risk of exposure to  Contaminated blood or blood plasma CJD patients may exhibit failing CJD? transfusion memory, behavior changes, Although most Americans have never impaired coordination and/or heard of Creutzfeldt-Jakob Disease, The sporadic form of CJD is the most visual disturbances. As the they have heard of “Mad Cow Disease” prevalent form of CJD affecting illness progresses, mental and fear it. They do not know what it is, approximately 300 new people in the deterioration becomes more but know it is catastrophic. The United States each year. The familial pronounced, and involuntary confirmation of a case of vCJD orig- form of CJD accounts for 10-15% of the movements, blindness, weakness inating in the U.S. would likely lead to a cases. Acquired CJD, which includes of extremities, and, ultimately, chain reaction of panic-driven deci- iatrogenic and variant CJD, accounts for coma may occur. sCJD usually sions and policymaking. less than 1% of all cases. More occurs later in life and typically information can be found on leads to death within a few weeks www.cjdfoundation.org. As of May or months to one year following 2009, there are no known cases of the onset of symptoms. endemic vCJD in the United States.

Diagnosis of CJD is very difficult and is

often made from clinical observation and/ or process of elimination of other www.cjdfoundation.org HelpLine 1.800.659.1991 diseases. The diagnosis of CJD can on- ly be confirmed through a brain biopsy or autopsy. Cerebral spinal fluid testing