Possible CWD/CJD link

Many questions here; how long were they and the herd exposed to CWD, how did they process their deer, did they only eat the muscle (deboned) or did they do bone-in processing. Any organs eaten, any other possible shared exposure like sheep, beef?
 
I have a quite a bit of familiarity with CJD because of my field.

Simplified, a prion is just a misfolded protein that causes other proteins around it to misfold too. It causes a cascade effect that is irreversible with current medical tech.

From a pathological standpoint, CWD and CJD/Mad Cow Disease are the same disease, but when it occurs in humans it is called CJD. The only way to tell the difference is to isolate the individual prion that caused the disease which is very difficult. It has also historically been really difficult to test for in living patients too.

The concern here is that CJD is pretty rare (350 cases in the US per year), so seeing 2 cases occur very close together COULD indicate a common factor.

Definitely something to look into very carefully!
 
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I have a quite a bit of familiarity with CJD because of my field.

Simplified, a prion is just a misfolded protein that causes other proteins around it to misfold too. It causes a cascade effect that is irreversible with current medical tech.

From a pathological standpoint, CWD and CJD/Mad Cow Disease are the same disease, but when it occurs in humans it is called CJD. It has also historically been really difficult to test for in living patients.

The concern here is that CJD is pretty rare (350 cases in the US per year), so seeing 2 cases occur close together COULD indicate a common factor.

Definitely something to look into very carefully!
Agreed. This study is not proof of anything, but with the proximity of the two cases and the statistics of CJD, there could be something to it.
 
Many questions here; how long were they and the herd exposed to CWD, how did they process their deer, did they only eat the muscle (deboned) or did they do bone-in processing. Any organs eaten, any other possible shared exposure like sheep, beef?
You are 100% correct, and I appreciate that the article didn't try to draw conclusions. It did exactly what it was supposed to do which is just to report the cases. Somewhere between 5-15% of CJD cases are thought to be inherited.

This is an very informative article:

Bottom line, we need rapid testing as soon as possible.
 
Good warning about potential transmission, but until they isolate and code the prion in the infected people, we won't know. When humans are infected with Mad Cow Disease (MCD), the prion is different than the natural form of human CJD. For that reason, it is termed CJDv, where the lowercase "v" is for "variant." Until they can show these two people have a variant version of CJD it could just be an incredibly rare coincidence (CJD occurs naturally in 1 in 1,000,000 people).
 
This would be incredibly bad news for deer hunters if it's proven to be related. Only a small percentage of people who consumed mad cows developed CJD but it was enough to create a problem.

Let's just hope and pray these two were unlucky coincidences.
 
This would be incredibly bad news for deer hunters if it's proven to be related. Only a small percentage of people who consumed mad cows developed CJD but it was enough to create a problem.

Let's just hope and pray these two were unlucky coincidences.
Yes it would. And you are correct about very low transmission rate of MCD. Best estimates are, 38 million people in Europe ate MCD infected meat. But only 206 got CJDv. But still a risk and a disaster for those who became infected.
 
Yes it would. And you are correct about very low transmission rate of MCD. Best estimates are, 38 million people in Europe ate MCD infected meat. But only 206 got CJDv. But still a risk and a disaster for those who became infected.
I recall an article that said there may have been a proclivity in those that were affected. Which may account for the low number of those that actually contracted CJDv.
 
The odds of contracting CJD from an infected deer are incredibly low. There are so many other ways to die. More hunters have died from falling out of a tree or from dragging a deer out of the woods. Not to mention hitting a deer on the road. Yet we continue to hunt, climb trees and drive to the woods.

Because I hunt in a highly infectious area where more than half the deer have CWD, I haven't even killed a negative deer in several seasons, I've eliminated venison from my diet altogether. Many of the hunters I hunt with have done the same. It's just easier to donate the deer to HFTH and move on. Fortunately I've reached the age and place in my deer hunting career where it's not that big a deal. But, I hate we have lost this tradition for my grandsons. My sons don't hunt deer anymore somewhat because of CWD. I really hope something can be found to combat this disease.
 
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Who funded the study?
Was just an observational case report, not a study.

It isn't designed to 'prove' anything. Just a possible link between CWD in deer and humans who died from the human version of cwd.

Doesn't change anything for me. I still would never knowingly consume meat from a cwd positive animal. I would never feed a cervid to my kids from a cwd endemic area without having it tested first.

Otherwise, I follow the same procedures I have since CWD began to spread from out west throughout states east of the MS river... only process clean muscle free from contamination of spinal fluids, ensure major lymph glands are removed from the muscle before grinding, etc.
 

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