CJD is a disease caused by a misfolding protein, called a prion. (PREE-on) Prion diseases are a group of rare and fatal brain diseases which occur in both humans and animals. In humans, it is known as CJD. Cows get BSE, which stands for bovine spongiform encephalopathy. Deer and elk contract CWD, or chronic wasting disease. There is no cure or clinical diagnosis for CJD. There is no cure for any of the diseases in the prion family.

Tuesday, February 28, 2006

Watch that iatrogenic contamination

I have seen studies showing prions present in the muscle of cows, (still hotly debated in the scientific community) so why should we humans be any different? Check this out...I know this is more for the nerdy CJD family members such as myself, but good stuff nonetheless. If you're new to reading these things and can't make it past the first sentence, relax. Just jump to the last sentence for the results!

Here's the link:

http://ajp.amjpathol.org/cgi/content/abstract/168/3/927

And the study, from The American Journal of Pathology:


American Journal of Pathology. 2006;168:927-935.)
© 2006 American Society for Investigative Pathology

Detection and Localization of PrPSc in the Skeletal Muscle of Patients with Variant, Iatrogenic, and Sporadic Forms of Creutzfeldt-Jakob Disease
Alexander H. Peden, Diane L. Ritchie, Mark W. Head and James W. Ironside
From the National Creutzfeldt-Jakob Disease Surveillance Unit and Division of Pathology, School of Molecular and Clinical Medicine, University of Edinburgh, Western General Hospital, Edinburgh, United Kingdom


Variant Creutzfeldt-Jakob disease (vCJD) differs from other human prion diseases in that the pathogenic prion protein PrPSc can be detected to a greater extent at extraneuronal sites throughout the body, principally within lymphoid tissues. However, a recent study using a high-sensitivity Western blotting technique revealed low levels of PrPSc in skeletal muscle from a quarter of Swiss patients with sporadic CJD (sCJD). This posed the question of whether PrPSc in muscle could also be detected in vCJD, sCJD, and iatrogenic (iCJD) patients from other populations. Therefore, we have used the same high-sensitivity Western blotting technique, in combination with paraffin-embedded tissue blotting, to screen for PrPSc in muscle tissue specimens taken at autopsy from 49 CJD patients in the United Kingdom. These techniques identified muscle PrPSc in 8 of 17 vCJD, 7 of 26 sCJD, and 2 of 5 iCJD patients. Paraffin-embedded tissue blotting analysis showed PrPSc in skeletal muscle in localized anatomical structures that had the morphological and immunohistochemical characteristics of nerve fibers. The detection of PrPSc in muscle tissue from all forms of CJD indicates the possible presence of infectivity in these tissues, suggesting important implications for assessing the potential risk of iatrogenic spread via contaminated surgical instruments.

Monday, February 27, 2006

U.S. Farmers & Our Government

Many of the CJD Families I've talked to have a certain bitterness towards the U.S. governemnt. Ok, just to be fair there is animosity towards the British government as well. Why? For putting profit above the safety of the people. Proper testing procedures cost money. And you wouldn't want to caution the public in the name of harming the beef industry when a cow is testing positive for BSE. The U.S. government could fix the mad cow issue in this country by testing every animal for human consumption. They could take the money they spend on beef public relations and use it for testing. It's such an easy solution!

Considering all the bitterness I've heard from CJD families all over the world about their respective governments valuing profit over human lives, this article didn't surprise me at all. Yes, the government worries about trade above your safety. That's why the U.S. government is trying so hard to get our beef into Japan, but Japan very wisely keeps saying no.

http://newstribune.com/articles/2006/02/26/business/309news23.txt

Here's a tidbit from the Jefferson City, Missouri News Tribune:

Rep. Jerry Moran, R-Kan., called Johanns' comments against extension “premature,” though he conceded the option should not be eliminated altogether. He said the farm bill should be driving negotiators at the WTO, not the other way around.

“It seems better for us to develop what a farm bill should look like for the benefit of American farmers, as compared to letting our WTO negotiators and the rest of the world determine what our farm policy should be in this country,” said Moran, a member of the House Agriculture Committee.

Sunday, February 26, 2006

Extra Credit & Enrichment Beyond CJD

Since I've been so busy working around 70 hours (or more!) each week, I started this blog nearly a month ago and it's been terribly neglected ever since. So I'm going to make a quick post of "extra credit" for you. One of the things I've become interested in since learning about BSE, CJD and CWD is food safety. It's a fascinating subject that probably warrants a blog of its own, and I'm sure one exists somewhere.

Are you going to get mad cow disease from eating beef? Who knows? It's not like there are a whole lot of people keeping track of that worldwide, although we're doing our best. I say "we" because there are plenty of CJD families trying to keep track of CJD deaths across America, whether connected to BSE or not. It's the families who have lost loved ones to CJD who are trying to keep track of who died of what and who got an autopsy to prove it.

If a family gets an autopsy back and it says the patient died of "sporadic" CJD, well, then they have to look at the person's medical history and see if there were blood donations, world travel, surgeries, eating wild game, etc. So then if you never left the U.S., never had a surgery, never had a blood transfusion, never took HGH in the 1970's, never had a dura matter graft, never ate wild game, or in any other way never exposed yourself to a prion disease, then how did you die of "sporadic" CJD? Sporadic is supposed to not be caused by eating beef. If it were, it would be "variant" CJD, or vCJD, and would most certainly show up on your autopsy. We hope. Sporadic is CJD-speak for not knowing where it came from. But that's a whole other post about testing procedures, and there's a whole school of thought on that.

Just for the record, I think my mom's autopsy came out correct because it showed her as fCJD, E200K, 129M. That is the Polish mutation, and she's 100% Polish. Two others I know of died in the family, so there's no way her autopsy could have come out as "sporadic," although it did initially. The NPDPSC, or National Prion Disease Pathology Surveillance Center told me that was POSSIBLE, but not PROBABLE and went on to do more testing with more DNA. Nine months after my mom's death, I had an autopsy that said "familial," with a mutation that made sense.

So back to food safety. Meat equals e. coli, food poisoning and all sorts of things that can make you ill or mame you for life, as is the case of e. coli. Then again, so can a lot of foods and once you really start looking into this stuff, it does get a little scary. Which is why I find this guy's post quite interesting, which is about Hexane. I'd never heard about Hexane until I read about this today, and thought this was cool enough to post a link to. So here's your extra credit for the day and I PROMISE to get some more posts up here!

http://groups.google.com/group/eHolistic/browse_thread/thread/de5cee243c8e4a2d/8f73babd71c8df6f?q=mad+cow+disease&rnum=1#8f73babd71c8df6f

Here's a little hunk of it, in case that link doesn't work for you. I found it on Google Groups. Yes, input from cattle ranchers are more than welcome here at CJDTalk. I have much respect for the ones who are doing the right thing here in America, but I remain a vegan nonetheless.

**************************************************************************

Hello Group
I think that this is information that you already know, but I want to post
it here in case there are some people who do not know the information that I
only recently discovered.
Thank you for reading my letter and if you feel that my letter raises valid
questions that have not yet been resolved will you forward my letter to
deserving people who you know?
Thank you,
Dale Baney


Feb 2006


Hello,


My name is Dale Baney. I am a cattle-producer from Indiana, near South Bend.
We raise beef cattle, and have for fifteen years. While reading material on
the internet in the last month I have learned that straight vegetable oil
can be used as a many ways superior-quality fuel over petroleum diesel!


In my researching vegetable oils and the methods involved in their
'creation' or processing, I learned that 95 percent of all vegetable oils in
the US are created using the very reactive neuro-toxins Hexane and
Methyl-Chloride that solve tight budget desires for consumer to get cheap
food.


The neurotoxin Hexane is said to be the most adversely effecting toxin of
all of the toxic solvents, and Methylene Chloride is also a chemical that
damages the immune systems, & causes birth defects of animals who eat it.
The occupational exposure limit for inhalation of Hexane has been set by
OSHA at 50 ppm (TWghAvg 8 -10 hours). How much Hexane & Methylene Chloride
remain in the oils and the high fiber meals produced using Hexane and
Methylene Chloride as the solvents?


One company selling Hexane-Free soybean oil says that a trace quantity of
Hexane remains in the meals and the oils processed with Hexane. That website
named the concentration number as "a 0.5% Hexane concentration" in meal.


http://www.alaffia.com/ingredients/oil_extraction.ph
p


The EPA has published the following document on the internet
that reveals what the average concentration found for Hexane in various
stages of production of the meal, though which stage is the stage for
production of high fiber, high protein meals for human consumption, and
which stage and which plant & location produce for high-protein animal foods
is not stated: http://www.epa.gov/ttn/chief/ap42/ch09/bgdocs/b9s11-1.pdf