Any news item headlined Deaths probed for rare brain disease is bad; news items with that headline from towns a short drive away are worse; and news items with that headline in a town a short drive away, when that “rare brain disease” can be related to mad cow disease, are the worst. Color me disturbed.

A rare degenerative brain disorder was suspected in the deaths of four people in northeastern Indiana during the past five months, health officials said.

Allen County Health Commissioner Deborah McMahan said the deaths were suspected to have been caused by Creutzfeldt-Jakob Disease. After the third death, McMahan contacted the state health department and asked that the federal Centers for Disease Control and Prevention be notified.

That would probably be because CDC figures suggest the Fort Wayne area “might expect one death from the disease a year,” making four rather alarming.

Testing of brain tissue from two victims was planned as that is considered the only definitive way to determine whether a person had CJD. Health officials said the four deaths appear to be from classic CJD and not related to mad cow disease, which is linked to the rare variant CJD found in humans.

…State epidemiologist Bob Teclaw said he was not drawing any conclusions from the deaths in northeastern Indiana. At this point, “we’re in the wait-and-see mode,” he said.

Good news. When there’s an outbreak of a rare and incurable disease nearby, what I like to hear is “wait and see.”

That’s actually just a bit of snark. I’m glad they’re paying attention and trying to sort it out, and I hope quite fervently that whatever Teclaw and pals at the Indiana Statehouse find out is the same information that we eventually get. Ahem.

[Thanks for the heads-up to Steph Mineart, one of whose readers passes on some more information about the lack of agricultural/DNR oversight in Indiana on canned deer hunting, which has resulted in blind eyes being turned to the mad cow-related Chronic Wasting Disease.]



Filed under 01_shakespeares_sister

26 responses to “Disturbing

  1. My reader sent me even further information on the connections between venison and potential links to humans:

    “Originally, there were great plans to monitor for CWD – in Lake County, IN there was even an additional feel for picking up roadkill because CWD was reported in nearby Illinois. Most of those precautions have been done away with once the ten operations were allowed to operate because of a special court injunction. It just all went off the radar screen.

    “Yes, these cases can be sporadic – but the sudden appearance of these “sporadic” cases defies the odds. Venison is ground up and fed to cattle. We have rendering plants that do this. At first there were strict requirements for animal feed plants in Indiana that used venision (road kill) and the DNR even wrote the Feds a complaining letter because they were not able to monitor the rendering plants because the plants knew that if even one case of CWD was found, they would be shut down. So – everyone took their chances and it all just blew over. Maybe …… who knows.

    “Anyway, I have that letter that was sent to the feds and also the DNR said they would check thousands of deer for CWD and, of course, did not. I am by no means saying this is the reason. Not at all. But – it is a dirty little secret in Indiana. If everyone’s luck holds out, no one will get the CJD that would result from chronic wasting disease. The domestic deer are monitored minimally and only recently. The secret shooter buck industry works behind the scenes. The motto of the shooter buck farmers in WI was – this is in print (shoot shovel and shut up). Of course, prion borne disease cannot be sterilized or buried. That is the threat of it. Incineration of the elk out west that had CWD was astronomicaly expensive. Anyway, I could go on and on. Just some food for thought. Venison can be put in chili, etc – the blades used to cut through deer spines can be infected – no sterilization works. That is why the rendering plants did NOT ALLOW the INdiana DNR to come in and inspect. Interesting, no?”

  2. Melissa McEwan

    Interesting, no?

    Yeah. “Interesting.”

    All part of the great deregulation-a-thon that’s been turning the entire state into a privatized free-for-all ever since Mitch “the Blade” Daniels left Bush’s budget office and took up residence in the Indiana governor’s mansion.

    Fuckity fuck fuck.

  3. jenn

    Yikes. I would say,”Vegetarianism anyone?” (being one myself) but considering last year’s spinach contamination, I’m really wondering if there’s anything left for us to safely eat. Just imagine what we DON’T find out about. How can we protect oursleves from this cost/benefit approach to food safety and public health?

  4. Jeez, what a horror story. I hadn’t even thought about contaminated implements – blades and such.

    So what are you and Iain eating this week?

  5. Nik E Poo

    All part of the great deregulation-a-thon…

    Bumper sticker:

    First in racing,
    to the bottom.”

  6. Nik E Poo

    All part of the great deregulation-a-thon…

    hmmm … no? … I’ll try again (this might be a double post)

    Bumper sticker:

    Indiana: First in racing … to the bottom.

  7. Sounds like a good time to go veggie.

  8. In Wisconsin, where they are starting to find CWD in deer, they are starting to give hunters special instructions for hanging the deer after it is killed to reduce the potential for CWD transmittal. In some places, hunters can bring the deer head and spine in for testing, and almost no one I know goes to the meat plant to have their deer processed anymore. Rather, they are doing it by hand.

  9. Melissa McEwan

    So what are you and Iain eating this week?

    Mashed potatoes.

  10. christine

    And people wondered why I was pissed off when we found a poached yearling deer on our farm. The poacher stripped off the ‘good’ meat and left the rest of the carcus on the ground. The white tailed deer can have the CWD and it’s been found in Minnesota, NE Iowa, Wisconsin, Illinois, Michigan, and Indiana for the last 5 to 10 years. One of the speculated reasons for this happening is that the deer population is way, way too high due to lack of natural preditors. Cull them!!!

    There’s a huge ‘tourist’ thing going on here in Iowa for trophy buck hunting in deer season. Can you say 14 point deers??? These guys are paying thousands of dollars to come here to hunt them. Do you think that these ‘hunters’ (and I use the term loosely) want to turn the heads over so the brains can be tested??

  11. I wonder if Steph Mineart’s knowledgeable commenter has the initials LZ.

  12. DBK

    My understanding is that these diseases require consumption of central nervous system tissue and that steaks, like venison steaks from a legitimately killed deer, is safe. Anyone have different information?

    I’m throwing the venison sausage we were given into the garbage as a result of this, but we just had venison stew last night from stew meat (no central nervous system tissue). Also, we’re in NJ, but I don’t know what difference that makes because Lyme Disease spread across the country, so why couldn’t these other diseases? Now I’m nervous. I like my brain. It’s my second favorite organ.

  13. Melissa McEwan

    the initials LZ


  14. PortlyDyke

    DBK — When I was taught to hunt as a child, I was always told to never eat any part of an animal that was observably unhealthy. Problem is, CWD is a long-term progressive disease, so an animal may not show external symptoms, and gung-ho trophy shooters with high powered, scoped rifles may only be counting points from a long way off, rather than worrying about whether the deer is healthy.

    I realize that what I’m about to say may bring up the “eeewww” factor for many, but if you’ve ever seen or participated in field-dressing a deer, you’ll probably know that there are many ideas about the “right way” to do it, and also, many hunters that don’t have a clue.

    Personally, I wouldn’t eat any venison that did not come from an experienced hunter whose knowledge and skill I trusted, or that I had killed and dressed myself (and those days are a long way off for me now).

    I do eat meat sparingly. I now eat only locally-raised organic meat, though.

    The biggest “duh” point in the whole problem with prion disease amongst beef-herds, to me is this: Don’t feed meat to herbivores. Duh.

  15. Don’t feed meat to herbivores. Duh.

    No fuckin’ shit!

  16. Nik E Poo

    Don’t feed meat to herbivores.

    Especially not the animal’s parents!

  17. PortlyDyke

    And, lest we get too awfully paranoid:

  18. That was some funny shit, PortlyDyke!

  19. Well, for what it’s worth, going from one to four cases could be a matter of pure randomness in the incidence of (human) CJ disease. When the numbers are that low, the statistics don’t tell you much. Now, if it went from 100 to 400…. The real data will come in once they’ve done the tissue tests. As you say, hopefully what you hear will be the same things they find out.

    The whole business of spongiform encephalopathies (scrapie, BSE, chronic wasting disease) jumping species barriers is really scary. Scrapie hasn’t, so far. That’s one of the reasons they didn’t worry too much about BSE, way back when. The old can’t-happen-here syndrome. It seems beyond ga-ga to take the same attitude now to CWD, after even the meanest intelligence should be to draw a lesson or two from BSE.

    (DBK: yes, you need to eat CNS tissue. If you have expertly and cleanly slaughtered animals and are eating only muscle tissue, I believe there’s not supposed to be any risk. The problem is when there’s no way to be sure of the “expertly and cleanly” aspect.)

  20. Come on gang, what’s the problem? All ya gotta do is brush your teeth with some of that Chinese toothpaste after eatin this shit and you’ll be OK. I wonder if the Rethugs haven’t been lapping this stuff up for years and not tellin anybody since they all seem to suffer from brain meltdown to some degree.
    (This is very early morning snark for those who can’t figure it out.)

  21. Jo

    A couple-five points about CJD versus vCJD:

    vCJD and CJD have different symptomatology–way different. The variant sort, the kind you get from eating infected neural tissue, takes much longer than the spontaneous sort to kill you. It’s also actively painful, and tends to hit much younger people. The distribution of lesions in the brain differs, as does the pattern of damage to individual cells.

    What these folks are wait-and-seeing on, then, is probably tissue path reports from Mayo. That’s where we send our suspected CJD samples (I work as a neuroscience nurse).

    Spontaneous CJD can also be transmitted via infected surgical instruments. The prions (protein bits) that cause it can only be inactivated with a really intense, chemical-heavy, heat-blasting sort of sterilization that practically nobody uses. It seems much more likely to me, therefore, that a patient with CJD, not yet showing symptoms (or maybe showing minor ones) had some sort of neurosurgery (like a spinal operation for back pain)…and the instruments were sterilized and reused. Yeah, scalpels and what-all are generally tossed post-surgery, but retractors and other stuff isn’t. We keep kits at the hospital for brain biopsies in which everydamnthing gets thrown away.

    In short, if there were four deaths in five months, I’d bet my boots it was spontaneous CJD (since it only takes six weeks to nine months to kill you, versus several years) that was transmitted by something in somebody’s operating theater. There was a situation like this at a hospital in Atlanta several years ago, in which it was discovered that a patient who’d had brain surgery had CJD after the instruments used in surgery had been traditionally sterilized and used on other folks.

  22. morpheme


    You’re right, iatrogenic CJD is more likely (though incubation times, depending on what was operated on can range from months to decades).

    On the other hand, we also don’t know what CWD mediated via a human species barrier would look like. Scrapie (the sheep prion disease) doesn’t cause CJD in humans, BSE apparently does. CWD may or may not, and CWD-induced CJD might look like regular CJD or like vCJD, or like something else, and the infection timeframes may not parallel either.

    I don’t have access to all my notes to see how CWD compares to BSE or Scrapie in mice or hamsters or monkeys for comparison, unfortunately.

    but the species barrier differences explain why wait-and-see is the right approach from a scientific viewpoint.

    I don’t think it’s the right approach from a public health standpoint, though. From a public health standpoint, I think you have to assume CWD is infectious, and proceed from that.

  23. Nora

    All this sounds like a good reason to make friends with organic farmers for your meats. Growing salad stuff is easy in containers, even in a small apartment. Other, larger items can be sought out from farmers’ markets. Eating local and known is turning out to be good advice.

  24. Kevin

    Then there’s my favorite paranoia — having your brain eaten by raccoon roundworms. Same symptoms, and someone at the CDC quietly wondered if maybe a greater number of younger dementia victims might have been roundworm victims.

  25. Arkades

    Jo and Morpheme… I was thinking something similar, that the cluster of co-located but (presumably?) not-genetically-related cases sounds to me suspiciously like iatrogenic spread.

    In addition to contaminated surgical implements, certain kinds of organ transplantation like corneal grafts or dura matter can transmit CJD from an infected person to the new recipient. HGH (human growth hormone) preparations can also carry CJD.

    Though one imagines if the four cases were linked by having received transplants from a common donor, the investigators would have figured out that particular scenario right away. At least, one would hope so.

  26. Pingback: My Left Nutmeg :: Where Connecticut Dems Scratch That Progressive Itch

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