CJD is NOT "Mad Cow Disease".
Bovine Spongiform Encephalopathy (BSE) occurs only in cows. The first documented case of BSE found in the United States occurred in Washington State in December, 2003 in a cow imported from Canada. The first endemic case was found in Texas and was announced in 2005. Eating infected beef is widely believed to be the cause of the variant form of CJD (vCJD) in humans. The estimated incubation period is 5 to 40 years. vCJD usually affects young people and has not yet been documented as originating in the United States.
Is the public at risk of exposure to CJD?
Although most Americans have never heard of Creutzfeldt-Jakob Disease, they have heard of "mad cow disease" and fear it. They do not know what it is, but know it is catastrophic. The confirmation of a case of vCJD originating in the U.S. would likely lead to a chain reaction of panic-driven decisions and policymaking.
The American Red Cross is denying blood donations from individuals who have spent six months or more in Europe since 1980, and that of any blood relative of a CJD victim because they fear transmitting CJD through the blood supply. The first case of suspected blood transmission of CJD was announced in the U.K in December, 2003. The blood was donated in 1996, 3 1/2 years before the donor became symptomatic. "Even though scientists can't provide positive proof that this case was transfusion-transmitted, in order to protect public health this should be considered the first case." [1] There has been one further case of suspected transfusion-transmission in the U.K. in 2004.
The public is at risk because the federal surveillance system for monitoring CJD and BSE incidence is poorly funded and fragmented. There is no uniform reporting mechanism in place and each state sets its own requirements. The public is at risk because so little is known about how to detect and treat CJD. Until the gap in that continuum is closed, the public will remain at risk. The linkage of CJD, BSE, the blood supply, and the lack of knowledge and reporting, unfortunately, combines to fuel those fears.
From a public policy perspective, a deeper understanding of CJD - both its causes and the rate of incidence would provide direction for science in their goals to better understand prion diseases and the ultimate goal to find the cure.
"CWD was first diagnosed in 1967 in Colorado, and it subsequently had been found in captive herds in nine states and two Canadian provinces and in free-ranging deer and elk in nine states and one province. Earlier this year, the disease was found as far east as New York. The source of infection for wild and captive deer and elk in new geographical areas is unknown in many instances. While it is not known exactly how CWD is transmitted, lateral spread from animal to animal through shedding of the infectious agent from the digestive tract appears to be important, and indirect transmission through environmental contamination with infective material likely." [2]
[1] (Professor Robert G. Will, leading research scientist, CJD Surveillance Unit, Edinburgh Scotland, Director SEAC. Excerpt from speech, FDA regulatory meeting, February, 2004)
[2] Buckmasters Online Magazine. "CWD Found in West Virginia Deer", September 2005. LINK (accessed September 15, 2005).