None other than our very own G.W. Bush.
During his speech in Canada on 12/1, a short sentence uttered for comical effect has since gone on to create a firestorm on mailing lists for families of CJD victims:
Bush: "So much trade there are bound to be some disagreements. I proudly ate some Alberta beef last night and I'm still standing." (emphasis mine)
Well, George, that's because - unfortunately for US - prion diseases can take 30+ years to become obvious. And by then, it's too late.
It was around 1985 that Mad Cow broke in Britian, and government officials rushed to assure the public that New Variant CJD (nvCJD) was a fluke, the beef was safe, and they ate burgers in front of television cameras to prove their point. It took 20 years and 200 deaths for the British to learn the error of their ways. Here in the U.S., we're still in denial.
The next time you go for a burger or steak, venison or squirrel, consider the following facts:
1. Sporadic CJD is thought to occur at a rate of 1 per million mammals, and yet we have a 15 year history of clusters in this country that continue to challenge that statistic:
A. Pennsylvania, 1993
B. Florida, 1994
C. Oregon, 1996
D. Texas, 1996
E. New York, 1999-2000
F. New Jersey, 2002-2004
G. Alaska, 2004
According to our government, these all represent statistical anomolies.
2. CJD is woefully underdiagnosed in this country, often written up as Alzheimer's or Dementia. But, dementia is a symptom, not a disease; and anybody who dies of Alzheimer's in under 4 years either had really fast-acting (and unheard of) Alzheimer's, or something else.
A 1989 study conducted out of Yale University concluded that 3-13% of Alzheimer's cases may actually be CJD. There are about 4 million cases of Alzheimer's each year in this country, which would mean that between 120,000 and 520,000 of them are actually CJD, aka Mad Cow.
- A 2003 study demonstrated that nvCJD prions, injected into animal subjects, resulted in the test subject developing nvCJD half the time; the other half developed Sporadic CJD.
- And finally, this from the CJD Diagnostic and Research Center:
The Creutzfeldt-Jakob Disease (CJD) Diagnostic and Research Center has been established to provide diagnostic support for caring physicians and families of CJD patients. CJD is misdiagnosed in nearly 35% of cases with other conditions (i.e. Lymphomas, gliomas, amyloid angiopathy, etc.) presenting with the same clinical signs and symptoms. Furthermore, there is currently no reliable diagnostic test other than brain biopsy or autopsy to confirm the diagnosis. The 14-3-3 spinal fluid test, currently in vogue, is not reliable and should not be relied upon alone for diagnosis. The situation is further complicated by the extraordinary fear amongst Medical Professionals in regard to this disease and their reluctance to becoming involved in standard diagnostic workup of suspected CJD patients.
People who have watched friends or family die from CJD (myself included) live with these facts and a million others, and the frustration with our government for not being more forthright, on a daily basis. I cannot put into words how life altering it is to come face to face with the enemy, but I can promise you that it is not the sort of death you want to experience.
Educate yourselves and your families, and do not be afraid to question the establishment if your loved one's illness seems to be something a little out of the norm.
Oh, and don't make cracks about how the cow you're eating isn't mad, otherwise you'll be in the same league as our esteemed leader. </sarcasm, anger, hurt>