Rare disease killed Tri-City man (Creutzfeldt-Jakob (Mad Cow))

[DuctapeFatwa]

The family of Dennis Jean Willett has waited four years to learn how he contracted Creutzfeldt-Jakob disease. The rare disease, which can be caused by consuming meat infected with mad cow disease, ate away at the Kennewick man's brain for four months, leaving the family to watch helplessly as he died Aug. 27, 1999.

As his body deteriorated, Willett's pain and dizziness got so bad that he was taken to Kadlec Medical Center in Richland. Doctors didn't know what was causing his condition, so he was flown in late April 1999 to Harborview Medical Center in Seattle.

Doctors at the state's main trauma hospital conducted numerous tests, but even they couldn't determine what was wrong. A sample of Willett's spinal tissue was sent to the National Institutes of Health in Maryland for testing, his family said.

Experts there determined Willett had Creutzfeldt-Jakob disease, but they don't know exactly how he contracted it.

http://www.tricityherald.com/tch/local/story/4552332p-4526738c.html

[Nlighten1]

1. I suppose we will see more of these soon...ala Firestone...

A defective product kept quiet by the media until the problem can't be ignored anymore...

[ET Awful]

2. There's only one proven way to contract Creutzfeldt-Jakob

and that's by eating contaminated meat, and not just meat, it must be tissue from the neurological system, meaning brain, spinal fluid, etc.

The only other way is only theorectical, and only one case (that occurred a few weeks ago that I heard on NPR) is a blood transfusion from an infected individual.

This isn't a communicable disease that spreads by touch or air, etc. It must be consumed.

Someone is bullshitting someone.

[demsrule4life]

3. I spent 20 years in Europe in the military

And have been banned from donating blood by the Red Cross.

[nadinbrzezinski]

5. This reminds me of England

After they found the first cases in cows and made the connection to the human form, did we see more human cases come out

This is what will happen here, and yes this means your and my worst fears

The food supply has been contaminated for the last 20 + years

[NickB79]

9. There are two forms of CJD

One is vCJD, the form transmitted by eating Mad Cow-contaminated beef, but there is a second form that occurs rarely but spontaneously in often healthy, almost always aged individuals. So, technically there are two proven ways to contract CJD. Considering this man's age, it seems possible he could have been infected with the spontaneous form rather than the transmittable one. The thing that started raising red flags in Britain when their Mad Cow outbreak occurred was that young people, including teenagers, were testing postive for CJD, instead of the typical 40+ yr old patients. A detailed study of the prions found in his brain would reveal exactly what form the prion is and solve the mystery of how he contracted the disease, since the protein configurations between the two forms of CJD are different.

[sabbat hunter]

17. maxine postal

is or i should say the presiding officer of the suffolk county legislature, having just won reelection in november, despite already being ill. she has been diagnosed with CJD. but the non-mad cow variety.

from what i understand about mad cow disease, one in one million cattle will come down with it, for no apparent reason, spontaneously.

we do not yet know if there is an outbreak in seattle or just an isolated case

there is also a way to eat safer meat.. eat kosher meat. by kosher law cattle have to be mobile before being slaughtered, unlike USDA regulations that allow a cattle that is a downer to be killed for meat.

also kosher meat is more carefully checked for signs of disease

i think the federal government should adopt some of the kosher rules to make our food supply safer

peace
david
:hippie:

[shanti]

35. i agree

a kosher rating (or something similar) seems to be the wave of the future as far as food safety now...

[NIGHT TRIPPER]

43. Kosher? not gonna help

at all-
What do you think They''ll do differently?
Look under a microscope as they bleed tha animal to death?

[chasqui]

20. woah there

It seems like CJD is really vCJD. When scientists say something like 'spontaneous' it generally means 'we are not exaclty sure how.'

[ConcernedCanuk]

10. Ahem - a wee bit of info - "unlike Alzheimer’s, CJD is infectious.”

.
.
. . ““Health officials have maintained there are only about 250 new cases of
CJD in this country each year, but several autopsy studies suggest this disease
has been under-diagnosed,” explained ABC’s John McKenzie. “The studies
show that when pathologists actually did autopsies and examined brain tissue
from patients with Alzheimer’s and other brain disorders, they uncovered
hidden cases of CJD, anywhere from about 1% to 13%. These preliminary findings
suggest a public health problem is being overlooked. If larger autopsy
studies at more hospitals in this country confirmed that even 1% of Alzheimer’s
patients had CJD, that would mean 40,000 cases, and each undetected case is
significant because, unlike Alzheimer’s, CJD is infectious.”

The math is obvious, and the potential ramifications are disturbing. If the
true number of CJD cases in the United States turns out to be 40,000 instead
of 250, the implications for human health would be severe. It could mean that
a deadly infectious dementia akin to Britain’s problem has already entered the
U.S. population. And since CJD has an invisible latency period of up to 40
years in humans, 40,000 cases could be just the beginning of something much
larger.

/snip/ - that's on page 7 out of 253 in the book

Mad cow U.S.A. Could the nightmare happen here?

Written in 1997 !! (complete version at above link)

So the Administration KNEW !

Somehow I ain't surprized - - SCARY - yes ???

YuBetcha sweet bippie !

[sam sarrha]

30. Independant studies have shown that 12-16% of alzhimers cases are Prion

disease. they are misdiagnosed.. I took an alzhimers test, it was 5 stupid questions, like my name where i was, what day it was..F'n stupid. Really pissed me off, it cost $485.

[sam sarrha]

31. Downer cows are FED TO OUR CHILDREN IN PUBLIC SCHOOL LUNCH ROOMS!!!

Check it out at www.organicconsumers. Campaign Contributions can do a lot to subsidize the beef and slaughter house industries...

[nadinbrzezinski]

4. I can see the panic a-coming folks

Most definitely

[shanti]

36. yep

i think this is it, folks. if this smoking gun doesn't get the chimp, i dunno what else will!

[Mrs. Overall]

6. This hasn't been stated explicitly in this thread yet,

but the Tri-Cities in Washington State where this man contracted the disease, is in the same area as Yakima where the mad cow case has been confirmed.
This is very odd coincidence, and as a Washington State resident, I find this news extremely disturbing.

[DuctapeFatwa]

8. I think this, the KC woman etc will be dismissed as a "conspiracy theory"

I'm not sure what is up with the announcement the other day, but I would expect coverage to now taper off, and focus on assurances that the food supply is safe.

[proud2BlibKansan]

12. What KC woman?

did someone in KC have mad cow?

[DuctapeFatwa]

26. Sorry, the town's name is Lucas, link inside

http://www.saljournal.com/index.shtml

[aquart]

41. They can focus on anything they want. Will you buy beef?

Will your neighbor buy beef?

Will you risk your life on the off-chance BushCo is telling the truth about something?

Not to mention, all those nations with good governments that are refusing to buy beef until we prove we're safe. And we can't.

[Junkdrawer]

7. If he was 50 yrs. old or older, I wouldn't suspect Mad Cow...

I did some Googling on Creutzfeldt-Jakob syndrome and the necessary link to Mad Cow hasn't been proven. The link was made in Britain because young people got this disease, which they almost never do.

On Edit: Added this link:

http://www.ninds.nih.gov/health_and_medical/pubs/creutzfeldt-jakob_disease_fact_sheet.htm#causes

[seventhson]

11. Radiation mutates these proteins into the prions that cause this

Notice this guy was a nuke engineer. A Long Island woman (near Brookhaven nuclear facility) also got the damn thing.

Halliburton is a HUGE nuclear firm.

THAT is why no one is stating the obvious reported by Sakharov almost 50 years ago: radiation pollution globally is mutating not only human genes but viruses, bacteria and all sorts of other cellular matter.

The point is: mad cow started after Chernobyl in Britain(which was heavily irradiated by the plume from Chernobyl)

The South Pacific Islanders who got it were, imho, exposed also to radiation from nuke testing there.

The prions are a new development in irradiated and exposed populations of people and animals.

I'd say keep an open mind on this and pay attention to it.

I think no meat is on my resolution list for the new year.

[WannaJumpMyScooter]

13. I hope you are not refering to this Long Island woman...

http://www.democraticunderground.com/discuss/duboard.php?az=show_topic&forum=102&topic_id=281493

cause she lives no where near Brookhaven


and Radiation only causes natural mutations, there are NO mutations that have ever been found to be caused by radiation that have not been identified in nature. this is what makes proving radiation-related disease so hard.

[NickB79]

25. Well, considering that the original known prion disease, scrapies

Has been known in sheep for hundreds of years, I doubt the Scottish were using nuclear reactors in the 1700's. If, by South Pacific Islanders, you mean Papau New Guinea people, kuru (prion-transferred brain-eating disease caused by cannibalism) was present and the people suffering from this disease well before WWII and the accompanying nuclear radiation. It reached its height of spread in the 1960's, but the tribal people knew of and recognized the disease for generations before this. I kind of doubt that radiation has much of a role here. This is a protein-transmitted disease, not a bacteria or virus. Since it takes over 500F to denature the prion protein, it appears to be in a very stable configuration. This would make it exceptionally difficult to "mutate" even with potentially lethal amounts of radiation (can you even mutate proteins?). The only way I could think of that prions could arise from mutation is for the radiation to induce mutations in the cell's DNA, and this mutated DNA is then misread by the cellular machinery and an improperly constructed protein chain is produced. But, when compared to the naturally occurring protein chain that the prion arises from, it was found that the amino acid sequences are the same. The only difference between them was the way in which the virulent prion protein folds.

[spinbaby]

28. Coupla points here

Prion-caused diseases have probably been around way longer than hundreds of years. In fact, because many people are genetically immune to them, there is even a theory going that these diseases were endemic in ancient times and, also, that many of our ancestors were cannibals.

Also, prions do not have DNA to mutate. Nor do they have RNA. There is a theory that they may "hide" genetic material in hosts but the prevailing theory is that they are pure proteins.

[chasqui]

45. Makes no sense.

The original prionic disease, Scrapie, has been known for ages, at least 300 years.
What you are observing there is just a refining of our understanding of the issue.

[NashVegas]

14. Was He a Hunter?

Apparently, venison is also a good way to pick it up.

[alarimer]

32. chronic wasting disease

this is a similar disease to BSE found in deer and elk. It has spread through the deer populations in many states. I am not sure if a human can get it from eating deer or elk.

[robertarctor]

15. Um, aren't the Tri-Cities in Washington State ...

...right next to the Hanford Nuclear Reservation? I assume you're talking about Richland, Pasco and whatever the third town is?

[regnaD kciN]

16. Yep, and...

...not too far from the farm where the Mad Cow was found.

However, I'd hesitate before making a linkage. If the cow in question was killed a couple of weeks ago, how could this guy have contacted the disease more than four years ago from eating its meat? And if he contracted it from some other local cow, why didn't we hear about that one?

The fact is that this disease is "long-onset," in other words, many years or even decades can pass in humans between infection and noticeable symptoms. If, as seems likely from what's written here, the man did contact this disease from eating meat from a BSE-infected cow, it may have been one from any time in his life, whether or not he was near Washington state at the time.

[SheilaT]

18. In early June of 2001

a man in the Kansas City area died of Creuzfeldt-Jakob disease. This was someone I knew personally.

Perhaps one in a million people spontaneously develop that CJD every year. It's not known why this happens, but is apparently just one of those weird things that happens.

The man was in his early sixties, had been in perfectly fine health until he started showing signs of dementia in the late fall of 2000. His decline was steady and unstoppable.

It's REALLY important to understand that CJD that arises spontaneously in humans and mad cow disease that arises from cow cannibalism are two totally different things. The first is simply not contagious. The second can be passed along through the food chain.

A useful book to read is "Deadly Feasts" by Richard Rhodes. I was, in fact, in the middle of reading that book when my friend was diagnosed, which made it a little creepy.

[kutastha]

19. IIRC

in my pathology class just last month, we discussed that people are genetically predisposed to CJD, that if they have a certain gene and are infected with prion-containing material, the protein product of the gene is susceptible to becoming 'transformed' into a prion as well, and then a cascade results. If people don't have that specfic allele, then the likelihood of contracting CJD is drastically reduced.

[RainDog]

21. prions

http://www.sfgate.com/cgi-bin/article.cgi?file=/chronicle/archive/2000/09/15/MN18012.DTL&type=science

Despite their forbidding reputation, prions are found throughout the normal nervous system of humans and other mammals, and are dangerous only when they appear in an abnormal shape. Prions may also be involved in normal functioning immune systems.

The mystery has been to determine what the harmless prions are there for. In the latest experiments, the French team used a special line of developing neurons -- budding nerve cells in the process of differentiating into a particular form -- derived from mice to work out an answer.

The particles sit on the surface of neuronal dendrites, the long, spindly arms of nerve cells that receive incoming signals. Somehow, an arriving message kicks the prions into action, triggering other chemical intermediaries within the cell in a cascade of biochemical events.

---

I think Tues. NYTimes also had an article about prions, not the disease version, and their possible function in creating long-term memory.

I should go find the link.

Since I read that prions were just discovered in 1997, I do wonder if prions could behave like cancerous cells? Can proteins do that?

edited to add NYTimes link-

http://www.nytimes.com/2003/12/25/science/25MEMO.html

Research With Sea Slugs and Yeast May Explain How Long-Term Memories Are Stored
By SANDRA BLAKESLEE

Published: December 25, 2003

by tinkering with yeast and sea slugs...Those lowly creatures possess an unusual protein that exists in two shapes. In one shape, the protein is sluggish or inactive. In its second shape, the protein perpetuates itself indefinitely but can also harmlessly switch back to the inactive form.
Advertisement


Researchers believe that in higher organisms the same protein may exploit this second shape to confer lasting stability to sites on brain cells, called synapses, that store the memories of a lifetime.

Surprisingly, the shape-shifting protein in yeast and slugs has all the hallmarks of another protein, the infamous prion, found in humans and other animals. Such prions also assume two shapes. One serves a normal function in the brain. The second sets into motion a runaway process that converts normal prions into a toxic form. As a result, deadly clumps of protein leave holes in the brain and cause disorders like mad cow disease.

The disease-causing prion and the memory-storage protein are not identical, said Dr. Eric R. Kandel, a neuroscientist at Columbia University who shared the 2000 Nobel Prize in Physiology or Medicine for his research on memory formation. But they share attributes that make prionlike behavior a perfect mechanism for storing memories.

--so this article also leads me to wonder if alzheimer's is someone affected...who knows, I certainly don't understand the way these work.

[kutastha]

23. Excellent links

>>Since I read that prions were just discovered in 1997, I do wonder if prions could behave like cancerous cells? Can proteins do that?<<

Kind of. You can think of them as proteins that function like a cancer cell, multiplying out of control, but have no genome or DNA dictating how they work. That's what makes them such a scary entity.

>>so this article also leads me to wonder if alzheimer's is someone affected...who knows, I certainly don't understand the way these work.<<

Alzheimer's is kind of similar as people also have a genetic predisposition to it and both CJD and Alzheimer's lead to dementia. Alzheimer's also has something to do with protein function and what results are tangles and plaques in neurons that interferes with their function. This occurs initially in the areas responsible for memory.

[jmcgowanjm]

27. raise your own, or go w/ family small farms

Dr. Prusiner's view, Ms. Veneman is getting poor scientific advice. "U.S.D.A. scientists and veterinarians, who grew up learning about viruses, have difficulty comprehending the novel concepts of prion biology," he said. "They treat the disease as if it were an infection that you can contain by quarantining animals on farms. It's as though my work of the last 20 years did not exist."
http://www.nytimes.com/2003/12/25/national/25WARN.html?pagewanted=1&hp



According to this chart, I think the industry has
been preparing for this since the first week in November.

[confusionisnext]

42. Alzheimer's

is due to a different protein, A(beta). CJD, Alzheimer's, Parkinson's, and Senile Systemic Amyloidosis, among others, are all due to their own misfolding protein. Scientists are just as of recently beginning to appreciate this new way of being diseased that is not due to viruses or bacteria.

[sam sarrha]

22. Go to www.maddeer.org and http//whyfiles.org/156cwd_deer/ and

surf Prion disease or cwd prion disease. TYPE IN: Downer cows lunch program action alert. cows to sick to get into the slaughter house on their own are being fed to your children in school?????????!!!!! It was downed cows fed to mink in Minnesota that got loose and spread CWD in to the deer herds!!!!!!!!!!!! FUCKING CAMPAIGN CONTRIBUTIONS TO INFLUENCE BEEF INDUSTRY AND USDA TO SELL SICKNESS AND DEATH TO OUR CHILDREN IN SCHOOLS THEY ARE FORCED TO ATTEND.!!!!!!!!!! I have seen these sick animals fall and be electro prodded through the door to become your food.. i never ate beef again after seeing that.

[this_side_up]

24. about 10 or 12 years ago

minister died from either original CJD or the variant.

His wife wanted an autopsy done but the state refused
saying that all of the instruments, table, and the
entire autopsy room would have to be permanently
sealed off as there is no way to disinfect anything.

Living as we all do, this man obviously had been to
the dentist, restuarants, maybe had minor surgery?
He had never been out of the country.

Before that a woman died from it. She was an
avid gardener who used liberal amounts of
bone meal and did not wear a mask or gloves.
Don't remember if she ever left the country.

Back in the 80s a certain hospital was treating
so-called fibromyalgia patients with bovine
growth hormone. They got sick. Hospital
switched to synthetic. these people also
went to the dentist, maybe had some surgery done?

[Blaze Diem]

29. Bovine Growth Hormone added to herds of milk cows?

Wasn't there a controversy a while ago over the use of BGH added to the production of cows for milk production increase? The Gov't itself approved its use over much protest Any info on this?

[donsu]

33. yes, some states ban it and some don't

nt

[Native]

34. Banned in some states?

Are you sure? With regard to banning products from cows given rBGH, it is my understanding that the U.S. is the only country that allows these products, and they are allowed in all states. I have read, however, that some states do require labels on dairy products from cows given rBGH to differentiate between products with & without. Here in Florida, no labeling is required. I've been told that in Wisconsin, labeling is quite evident - in addition to labeling on the products, signs are also placed on shelves/sections of the dairy area in grocery stores.

[this_side_up]

38. Monsanto allow

rBGH to be banned in various states? I don't think so.

The last I heard is that they sued various farm(ers)
or butter or milk companies for having "non-rBGH milk"
on their labels.

Cute.

May all of the people involved in this mess rot in
hell with the pro-Iraq war bastards.

It will be difficult for me to become vegan and
I don't think hubby will go for it. But I'll work
on him and I guess we will have to eat what we
have as he still does not have a job.

But hey! without a job, ya can't afford to buy
meats and chickens and turkey and cheese and milk
and eggs.

Thanks thieving * and your crooked supporters!
There will be millions of new vegans.

I gotta sign out before I say stuff I shouldn't.

To everyone who is keeping up with this and posting
articles, links and comments: Thank You.

[NashVegas]

39. States' Seals of Quality

That's how some states are dealing with it. Maine, for instance, won't allow its dairies to use the state seal if they use the hormones.

FYI, last I heard, Monsanto wants to settle with Oakhurst dairy.

[this_side_up]

37. I haven't read

anything about rBGH with regards to CJD in people or
Mad Cow in animals.

Intereting thought, though.

[Must_B_Free]

40. Does this Mad Cow episode remind anyone of the Romans

and their lead cups?

I bet they didn't believe that it was bad for them either...

[NIGHT TRIPPER]

44. reminds me, we surround ourselves in blood daily

and wonder why bad things happen to good people.
Ever heard of what comes around goes around?
Some basis for that old saying is obvious truth.
This is what I think.--it is not fact--it is what I think--

I say IT IS TIME TO CHANGE OUR WAYS !
the year Two thousand and what?
Nature always recoils against unnatural abuse.
Is this just the start of something bigger?

Each year is like a holocaust for animals in the U.S.!
Industrialized farming kills faster than ever before in history.
Less cruelly? NO.

Much of this world casually bathes daily in the blood of those murdered (animals) by hired hands,---safely distancing ourselves from the sick horror, we buy the body parts all neatly wrapped in cellophane and serve them up at the family barbeque and at the wholesome little family restaurants.
How quaint.

In almost every building we hoard the dismembered body parts in refrigerators...Seems like a bad science fiction movie when you think about it.

An advanced society?
Who's kidding who?
Mass horor nowadays/ unlike the old days of limited killing with occasional consumption.
America=6% of the world's population and consumes, gnaws, chews, ingests 60% of the worlds' cow body parts.
This is out of hand.
Advanced technology provides much more efficient means of food production and "taste friendly" products.
Nutritionally there is no necessity.
It's only myth that you "need" to eat animals.
Ever wonder why the myth?
BIG BUSINESS is why.
Don't be a sucker and believe these clowns who tell you you have to eat that shit!
Think for yourself.
When you see a cow if you have the impulse to go blow his brains out and chain saw him up into pieces then you have been to the wrong schools, the wrong churches, and had the wrong parents...and you currently have the wrong pResident.

It doesn't taste that good --"TO DIE FOR"