Science Friday - Fetal Cell Research, Schadenfreude, Deer Disease. Dec 21, 2018, Part 2

Episode Date: December 21, 2018

The Trump administration is cracking down on federal scientists seeking fetal tissue for their work, while it conducts a “comprehensive review” of research involving fetal cells. One HIV research program that uses fetal tissue to create humanized mice has already been halted by the order. The Department of Health and Human Services said in a statement that it’s performing the audit due to the “serious regulatory, moral, and ethical considerations involved” in this type of research. And a spokesperson for the HHS said the agency is “pro-life, pro-science.” But what does that mean, exactly?  Schadenfreude, or deriving pleasure from someone else’s misfortune (which you have not caused), may seem to be everywhere in the modern era of internet trolls, but the misunderstood emotion is not a modern phenomenon. The German word first appeared in English text back in 1852, although people in English-speaking countries were so scared of what it would mean to admit to feeling schadenfreude that they never came up with a comparable English word for it. Over the years people have tried to analyze why we feel schadenfreude—evolutionary psychologists say it’s a way for us to assess risk and 19th-century Darwinian scholars suggested it was a behavior associated with “survival of the fittest”—but people have never really gotten comfortable with those academic explanations. You might outwardly protest that you don’t feel joy in seeing another person suffer, before returning to “fail” videos on YouTube. But according to Tiffany Watt Smith, a cultural historian of emotions, you don’t have to feel shame about feeling this way. Schadenfreude doesn’t make us psychopaths, or internet trolls—it just makes us human. And if we are living through an “age of schadenfreude,” as some have suggested, perhaps there’s something useful to be learned from it.  You’ve heard of viruses, bacteria, and fungal infections. But what happens when disease is caused by misfolded proteins? Prion diseases, as they’re called, infect the central nervous systems of animals all over the world, including sheep scrapie, Mad Cow Disease, and even a new one recently discovered in camels. In deer, the prion that causes Chronic Wasting Disease will stay undetected for years before a deer suddenly stops eating and begins to waste away. Always fatal, the infection spreads from deer to deer, and even lurks in soil—and it’s reaching new parts of the U.S. and the world every year. Judd Aiken, a professor at the University of Alberta, explains how prions like those that cause CWD interact with different soil types to bind to minerals and become more infectious… or pass harmlessly through. He describes new research about how humic acid, a product of organic matter in soil, seems to degrade prions and reduce the infectivity of CWD.     Subscribe to this podcast. Plus, to stay updated on all things science, sign up for Science Friday's newsletters.

This is Science Friday. I'm Ira Flato. Later in the hour, we'll be talking about Schadenfreude, when you get satisfaction out of witnessing the misfortune of someone else. What kind of Schadenfreude are you guilty of indulging in? We'd like to know. Give us a call. Our number 844-724-8255-844-724-8255. Or you can tweet us at SciFri.

But first, the Trump administration, is cutting back on funding for scientists involved in fetal tissue research, while the administration conducts a, quote, comprehensive review of research involving fetal cells. One HIV research program has already been halted by the order. Why? The Department of Health and Human Services directed us to a statement, which says the department is doing the audit due to the, quote, serious regulatory moral and ethical considerations involved in this type of research. And a spokesperson for the HHS has said the department is, quote, pro-life, pro-science. But what does that mean exactly?

And what might the implications be for fetal tissue research going forward? Meredith Wadman has been covering the story for Science magazine. She's also written a book about fetal cell research and vaccines called the vaccine race She joined us now from Washington. Welcome to Science Friday. Thanks, Ira. It's a pleasure to be with you. Thanks to have you with us.

I know you've been reporting on this for months. Please take us back to the beginning. How did all of this start? It began really with letters to the FDA commissioner and to Alex Azar, the Secretary of Health and Human Services, from abortion opponents, including members of Congress back in September, saying, hey, we don't like the idea that FAA, the President. FDA just executed a contract with a small company in California to obtain fetal tissue from elective abortions donated by the mothers because that just isn't okay for the U.S. to be involved in that in a funding way. And FDA had been obtaining the tissue in order to create mice with human-like immune systems that would allow them to test drugs for safety and effectiveness. So almost immediately, Health and Human Services canceled that FDA contract, which was a small one, about $16,000. But it also announced that it was going to review the entire portfolio of U.S. funded research that involves fetal tissue donated after elective abortions.

And it has been doing that under the aegis of the Assistant Secretary of Health for about four months now. So what does it mean by an audit? Is that just a term of ambiguity? I think it means we're scrutinizing this. And what they're saying is we want to make sure it comports with existing laws and regulations that are very specific about in what circumstances and how and under what ethical regulations this research may be funded by the U.S. government. and those have been written in law since 1993. The department did not in September produce any evidence that this particular company in California, whose contract with the FDA it killed, was not abiding by those guidelines,

and it has not done so since, nor has it produced any evidence that researchers funded at the National Institutes of Health, which does the lion's share of funding of this research, are not abiding by the guidelines. So it's not really clear, clear what except political pressure induced the administration to take on this review. The administration has brought scientists forward.

Congressmen have in hearings on Capitol Hill Republican congressmen have said that the stem cell research, fetal stem cell research is not important. Fetal cells are not important for doing meaningful work. There are other tools they may use. How logical or how real is that argument? Well, I think, in fairness, they didn't say it's not important. They said it's not necessary. They said there are alternatives. Scientists as a whole strongly debate that and contest that.

There are some kinds of research that can be done without human fetal cells. And researchers, goodness, knows, would prefer not to use them were there better alternatives. But the fact is, for many applications, really important ones like discovering drugs against HIV, There are no better alternatives at the moment. Thus saith the scientific community. Your book, The Vaccine Race, is actually about how fetal cells were used to make vaccines, too, right? That's correct. And in fact, many vaccines, but in this country, most importantly,

they're still used to make the Rubella vaccine that protects about 4 million toddlers every year against Rubella, which people may not remember, but back in the day, Rubella, also known as German measles, regularly caused tons and tons of damage babies to be born because Rubella, like Zika, attacks fetuses in the womb. I should draw a distinction, though. The fetal cells used to make vaccines were obtained from two legal abortions way back in the 1960s, and they were propagated so they still exist and can be used and are used. However, the dispute currently is over the obtaining of fresh fetal tissue from new abortions in the present day. So that's an important distinction. And an important point about this is that this is tissue that's going to be thrown out otherwise, correct?

Correct. Abortions lead to fetal tissue that is typically disposed of. I should add, though, that in the framework, the legal framework that allows the U.S. to fund this research, a woman's decision to proceed with an abortion must be divorced from her decision then to donate that tissue for research so that there's not a situation in which she's undecided about the abortion, but, oh, well, I can donate the tissue for research, therefore that makes me take the plunge. No, a researcher or any institution or any facility providing the services of providing an abortion may not even so much as mentioned donation of the tissue until her decision is independently taken. How strongly have scientists involved in research who know great details about the importance of fetal cell research?

How strongly have they been speaking out, pushing back? They've been speaking out pretty strongly. Sally Temple, who represented the International Society for Stem Cell Research at the Congressional Hearing on December 13th, was really adamant to the members of Congress who challenged the notion that this tissue could just be not used, and alternatives already existed. She pointed out that really important drugs like Truvada for HIV and really important discoveries like how the genetic, sorry, how the genetic, go wrong in this horrible childhood eye cancer called retinoblastoma have depended on access to human fetal tissue. It seems like, you know, even even Francis Collins, the head of the National Institutes of Health, he has spoken out strongly in favor.

He did speak out strongly in favor, and now, unfortunately, abortion opponents are calling for him to be fired. It's a real irony because, as many people know, Francis Collins is an evangelical Christian who thinks about these things very strongly and clearly and is what he would call pro-life himself. So calling for his head may be asking for the head of someone who really is the most sympathetic among many biomedical scientists to people who oppose abortion.

And, you know, we can respect both sides in this debate. I think what's really sad about calling for axing this research is doing so who would not present. prevent any abortions, it would just mean that tissue already being thrown away could no longer be put to use to try to make the lives better of people who are living. Are there any alternatives for the states? For example, California was funding its own fetal cell research for some time to get around these issues.

Is that still going on? Is there funding available from the states? Yeah, that was for human embryonic stem cell research, the California Institute for Regenerative medicine. That is going to be sunsetting. So trying to fund this privately would really be, I think, difficult. There's literally dozens and dozens of projects listed in a publicly accessible database that the National Institutes of Health funds, and it's everything from trying to understand what goes wrong in the brain and down syndrome, to probing how Zika virus crosses the placenta and damages the brains of children in utero.

So there's tons of research going on that halting would have a really serious impact on. All right. So let's talk about what's the next move on this, who we should be watching. The president, someone at HHS? I think Brett Girouar, the Assistant Secretary for Health, initially a pediatric critical care doctor who knows his stuff and who's also been involved in research administration and big universities. systems and so on. He's in charge of this review, and he is the one, and I suppose his boss,

Alex Azar, who will be making the final call on if and to what degree the government does end up shutting down any of this research. Every time he's been pressed about, you know, what are you going to do? It's been like the jury is still out or still conducting this review. No decisions have been made. So is he the one who said he's both pro-lis? and pro-science? Yes, that is the catchphrase that the department is using. Is that I can have my cake and eat it too? Yeah, or I have to tow both sides of a very thin political line, and it's obviously tough. Can the public weigh in on this somehow? Well, letters to members of Congress, letters to the HHS assistant secretary, I think

public statements, petitions, and the anti-abortion lobby is. making itself very loudly felt in this respect. They wrote a letter to President Trump yesterday saying, you've got to shut down this research. So voices on all sides are being heard to a degree. Will the new Congress with the Democrats of control of the House have any influence on this? You know, I don't know because Congress is about law, really. And this is not just because you can fund something as, say, NIH, doesn't mean you must. It's not really, a question of law so long as the 1993 law that makes this kind of research legal still stands. Now, I suppose Congress, a very conservative Congress, could rewrite that law, but clearly with

the House going to the Democrats in January, that's not going to happen. Well, we'll watch it with you. I want to thank you for being a referee on this, Meredith. It's been a pleasure. Thanks for having me, Ira. Have a happy holiday. You too. Meredith Wadman is a reporter for Science Magazine and author of the vaccine race.

After the break, do you take pleasure in seeing your political opponents fail? Is 2018 the year of Schadenfreude? We consult an historian of emotions on this who has written a whole book about it. Stay with us. We'll be right back after this break. This is Science Friday. I'm Ira Flito. Do you find pleasure in seeing the quarterback of your despised sports rival getting sacked, even hurt? Do you give a fist pump when another White House staffer resigns or is finding, Well, you may be practicing schadenfreude.

It means to derive pleasure from someone else's misfortune. It's not an English-sounding word, is it? In fact, years ago, English speakers were so scared of what it would mean to admit to feeling Schadenfreude that they never came up with an English word to describe it. They had to borrow from the German. And over the years, people have tried to analyze why we feel Schadenfreude. Evolutionary psychologists say, it's a way for us to assess risk. Darwinian scholars suggest it was a behavior associated with survival of the fittest.

But we've never really gotten comfortable with those academic explanations. We outwardly protest that we don't feel joy in seeing another person suffer before returning to watch a stream of fail videos on YouTube. But according to my next guest, we don't have to feel shame about feeling this way. Schadenfreude doesn't make us psychopaths or internet trolls. it just makes us human. And if we're living through an age of Schadenfreude, perhaps there's something useful to be learned from it.

Tiffany Watts-Smith is a cultural historian of emotions at Queen Mary University in London, and she's also the author of a great little book called Shadenfreude, The Joy of Another Misfortune. Dr. Smith, welcome to Science Friday. Hi, thanks for having me. I want to ask our listeners first before we begin our conversation, when was the last time you had a moment of Shadenfreude?

How did you feel about it? Did you feel guilty or ashamed, satisfied, and smug? Give us a call. Our number 844-724-825-8-4-Sy-Talk, or as always, you can tweet us at SciFri. Let me begin with, let's define some terms and pronunciation first. What is the accepted way to say the term?

The word. So, Chardon-Froidurida, so four syllables, usually, in German. But, you know, this is a word that has been co-opted from the German into English. So in some senses we can be free, I think, to kind of say it as we want to. This is one of the interesting things about loan words, isn't it? That they do change shape as they travel. And define the term exactly for me. Well, in German, Chardon means damage, and Freuda means joy.

So damage joy, which is kind of broad. When the word was first started to be used in English in the middle of the 19th century, It was used to describe quite a lot of pleasures in other people's misfortune. So some that we would think of as very familiar to us. So, you know, enjoying politicians make a mess of something, enjoying seeing your sports rival mess up, etc. But some of them which wouldn't quite sit with what we think of a Schroeder today. So, for example, in the late 1890s, someone says Scharden Freud is the pleasure

that people feel watching cats being tortured on the street. and that to me sounds more like what we think of as sadism. What happens in the 19th century is that this new term sadism comes in and Chardonfreda gets all the fun, sort of silly stuff, and sadism gets a more serious, kind of brutal delight. I think you write that the digital age, social media, it's a perfect time, it's a perfect place for Chadenfreude now, and it seems quite obvious.

Yes, that was one of the things I found really interesting about the research for the book. So I had one of the things that prompted me to write it is I kept on coming across this phrase, you know, we're living in an age of Shardin-Froeder. And as a historian, I'm very interested in those kinds of claims, you know, that when one emotion is thought to define, you know, an entire public mood or spirit. But yes, the internet seems to have an awful lot to do with it. We, we, one of the things that causes us to feel Shardin-Froid is, is injustice. Sorry, excuse me. seeing people who have done something bad get their comeuppance. Now, of course, if you wander up and down your street,

you don't see an awful lot of injustice, but you spend five minutes hanging around online and you see terrible atrocities and any chance that we get to then see those people punished and get their comeuppance, I think fills us with a certain amount of glee and satisfaction. So, yes, online we experience a lot more of this kind of Sean Vrida, but also it's a lot easier for us to express it. You know, I was thinking actually about an animal,

anecdote that I read now, you know, I don't follow American football, but I read an anecdote about a new England Patriots player called Tom Brady, who fell and tore a ligament. And apparently everyone in the stadium was absolutely silent when he screamed and he fell. But everyone listening on their radios and especially online, we're really whooping and celebrating at this. I think it's much easier to express that kind of glee when we're not sort of in the presence of the person who's suffering. You know, he's about as famous as Harry Kane is in your... Sorry, who's this guy, Tom Brady?

It's all like Beckham. He's that level, you know? Apologies. That's quite all right. Let me go to the phone. So many people want to talk about this. Let's go to Gueni in San Antonio, Texas. Hi, Quennie.

Hi, thank you so much for letting me through. Wow. Okay, this is awesome. So, yes. So I first discovered about Chauden for you. Actually, a month or two back when I was in Avenue Q at the Woodlawn. And so that musical contains this song called Shotden, Florida. And it explains what I think is examples of stuff.

And I was wondering, because there was a part that I really wanted in that musical. And I was sitting in the audition room. And, you know, something in me just wanted to be better than everybody else so that I would get part. I didn't. Oh, well. And so I was wondering if wanting to get something over somebody else so kind of then with fortune, like indirectly, would that count as an indirect of shot in Freud as a great question.

Yeah, that's a great question. And I'm sorry that the part was not yours, but I'm sure it will come around again. But that interesting, that's a really interesting point. I think that rivalries are where we get our most intense, Schaden Freud. think, you know, we can feel ourselves to be inferior to someone in some way, whether that's, you know, just being less successful or rich or beautiful or whatever it is that you're competing over. And then, you know, their kind of minor disappointment or them experiencing some sort of setback is a great moment of psychic compensation, I think, for us, a little win, you know,

and a feeling, I think, that gives us a certain boost. I think that in this way, Schaunfroeder it can be, is in fact very useful to us and we experience it a lot in that context because it gives us a little bit of confidence back when we're feeling at our lowest and gives us, makes us feel a little bit more sprightly and ready to take on the world again. Let's go to Robert in Sioux Falls, South Dakota. Hi, Robert. Good afternoon. Good afternoon. The best quote that the one that we all can live by is the one that Mark Twain made. It said, I've never killed anyone, but I've read many an obituary with a great amount of satisfaction.

Thanks for that addict, though. Have you heard of that one, Tiffany? No, I wish I had. That's absolutely wonderful. Yeah. Is there a time, you say we're living in the age of Schadenfreude, is there a time when the pendulum might swing in the other direction where we've

had enough of Schadenfreude and we want the anti-Shardens-Fraude? Is there such a thing? Well, that's very, yeah, that's very interesting because on one level I think that one of the reasons why we've got so interested in Schaunfroeder at the moment is not just because there's more of it washing around, but because we're more anxious about it. And one of the reasons why we're more anxious about it, I think, is because of a real interest in the last, really since the 1990s, I suppose,

in empathy and recognizing how valuable empathy is, you know, not just in our personal relationships, but in our public relationships and how we learn, how we do business, how we manage and so on. And in that kind of context where empathy is really being celebrated, you know, Chardon Freude looks really, really problematic, I think. You know, certainly when people were discussing Chardon Freude, you know, in the 18th and 19th century people were saying, you know, this is a terrible emotion, it's really ungodly and it's fearful and so on.

But I think in a way we're kind of slightly turning back to that kind of quite moralistic stance around Chardonfreude. Actually, I read, I published only a few weeks. ago an article that brought together a lot of existing research and said, you know, Shardun Freud really brings out our inner psychopath. And I thought that that was really problematic language, actually, because in this context, Sharden Freud gets defined as the opposite of empathy, as if those two emotional states were fundamentally incompatible. But that's not really how our emotions work. You know, emotions kind of all tumble together in a big sort of mess. And it's perfectly

reasonable that you might have an experience where you, you know, you're genuinely sorry for your friend who is very successful and happens to have not got their latest promotion, but there's also some terrible sly smile creeping up on the side of your lips because it makes you feel slightly better about, you know, your job. I mean, we can feel two emotions at once. You know, from a personal experience, I was driving a while back and there was a crazy driver behind me and it was zigzagging all over the place and he zipped right by me almost clipped me at 90 miles and I said, oh, I hope that guy gets caught by a cop. And literally, coming around the corner,

I saw that his car had flipped over three times. He couldn't take the turn. And suddenly I felt, you know, maybe that's just too much. That's too bad a sentence to have for that person who, you know, that's just too much punishment to take for something that's silly. And I immediately, you know, switched over to empathy from having a disgust for the driver. Is that a common sort of thing? I think that's very common.

I think Shardin Freud is an interesting emotion, not just because it shows up all kinds of hidden ways in which we relate to one another, but also because it's what psychologists call a cognitive emotion. So instead of something like fear, which is kind of trigger response type of thing, Shard & Freud really involves us thinking and calibrating our response and judging the situation and trying to work out what's fair and what's deserved and so on. And so we often find ourselves in these kind of awkward predicaments. where we think that one person, initially we might think, oh, that person deserved it,

and I'm really pleased, and then immediately you think, oh, actually, no, they've suffered more than I think they deserve, and now I'm not pleased anymore. This kind of situation, I think, happens all the time with Chardon Freud. It's very interesting. Let me go to the phones to Creston, Iowa. Julie, welcome to Science Friday. Julia. My question is just along those lines.

Does it count that Statenforda, if someone has already done something nasty to you and they're jerks about it, And then you just feel like, oh, well, karma's going to get you. It's just desserts. And what goes around comes around. Absolutely. I mean, karma, I think, is the most sort of basic form of Schadenfreude, isn't it? I mean, I think we enjoy seeing justice carried out. But I think it's particularly delicious when the universe seems to have intervened.

And, you know, the person who's shoved past you on, you know, on the station stairs still manages to miss their train. I mean, these moments are just spectacularly. pleasing, I think. You're right that the Schadenfreude is part of what drives fake news. Why is that? Well, I think that there's a particular desire and hunger to see our politicians and those in power, humiliated or suffer kind of setbacks. I mean, politics is one of the areas where Schauner is particularly rife. There's been several studies on this, but it was very clear that Schaude has very intense when we divide ourselves into tribes, particularly into rival tribes. The Chardonfreude is part of what allows us to bond together in those groups and sort of

confirm our social identities. But it's also really important for us to denigrate the other side and therefore feel like we're sort of coming out on top. So this landscape is really fertile both for Chardonfreude and therefore for sort of offering the possibility of enjoying fake news or, you know, sort of blatantly made up terrible stories about the other side because, you know, the Chardon Fretter is so irresistible, really. And I think this is the other thing that I was really interested in when I was writing the book was that, you know, on the whole, I think Schoenfrette is pretty harmless and it's just a bit of fun and it's fine and we can cut ourselves some slack.

But I do think it is worth understanding it better and understanding the kinds of situations which kind of provoke it in ourselves because we do find ourselves, you know, at the mercy of whether it's the news or our politicians who recognizes this is a very powerful emotion and try to elicit it in us. So it's good for us to understand how it works so that we can recognize when, you know, our Chardonfreude strings are being plucked and we can kind of take a step back. Talking with Tiffany Watt-Smith, author of Chadenfreude, The Joy of Another's Misfortune. On Science Friday from WNIC Studios. A little bit along this idea about tribalism and being with your cohorts.

I got a tweet from Michael who says, as a relatively new parent, I do feel some pleasure or kinship when I see in the other parents struggling or dealing with the trials of parenthood with their children. Misery loves company. Yes, you can go two ways with this, can't you? Like sometimes I'm a parent of young children as well. And I definitely feel camaraderie when I see another parent, you know, with their child having a tantrum in the middle of the shops or whatever it is. But sometimes you do get that moment where you just feel a little bit smug because your child is being well behaved temporarily and their child isn't.

I suppose it just depends on your mood at the time and how generous you're feeling and perhaps how naughty your child has been in the previous few minutes. What's the difference between Schadenfreude and slapstick comedy? You know, we grew up with a I Love Lucy, which was a sitcom about a woman who was always getting into trouble. It was the most popular show on TV. We watched her getting in and out of trouble, and we laughed at it. I mean, I think Chardon Frider is the pleasure that we get when we watch Slapsdick. I think that, in fact, that's probably the most fundamental earliest form of Shardin Freud. Actually, I came across a really intriguing study that was done at the University of Oxford in 2011.

and they found that there was, you know, the particular kind of laughter, belly laughter, which is that kind of laughing so much it hurts type of laughter. And they found, among all the different lafters that they studied, that this was very unique to humans. And they also found that this sort of laughter only seemed to ever arise in response to slapstick. And they particularly found in response to Mr. Bean, if you're familiar with him. Right, I know Mr. Bean, absolutely. And the difference is that we know they're not being hurt, right?

If we know that it's just comedy, then no one's going to go home with a broken leg or something. Well, I think you know that it's, absolutely, you know that it's being choreographed, but it doesn't mean that it's not funny. I mean, one of the things this study found was that it actually lowered people, and raised people's tolerance to pain. So they suggested that that kind of laughter is really important for bonding groups together, but also helping people survive in very hostile environments. And I think it doesn't really matter in that sort of situation,

whether it's, you know, someone's pretending to be banged over their head with a sarspan or whether someone really accidentally trips and falls into a sourcepan. It just, it's the moment of bonding that happens as a result of it that seems to matter in that situation. No, you certainly have covered every situation I could think of in the book of Schadenfreude. And every, you know, just when I think, oh, she's never done this. Oh, yeah, next turn the next page. You're talking about it. Tiffany Watt Smith, author of the new book Schadenfreude,

the joy of another's misfortune. If you want to read an excerpt, you can find one on our website at ScienceFriety.com slash emotion. Thank you for taking time to be with us today. And happy holiday to you. Thanks for having me. And to you too. We're going to take a break, and when we come back,

we're going to talk about a deadly protein, a preon. It continues to spread chronic wasting disease in deer herds around the country and the world, even the reindeer. Can soil scientists help slow the spread? some answers in your questions coming up after the break, so stay with this. This is Science Friday. I'm Ira Flato.

Where does disease come from? Maybe you think the answer is a virus, bacteria, maybe fungi. Would you ever stop to think that a protein could make you sick? Well, that's what's happening in preon diseases, like mad cow, sheep's scrappy, Kreuzfeld-Yacob disease, misshapen proteins become infectious and eventually get into the brain where they cause all kinds of degenerative symptoms. And in the world of prion diseases, one is really leaving its mark here in the U.S. Chronic wasting disease, which has been found in deer.

More than two dozen states now, three Canadian provinces, as well as Korea and Finland, and even reindeer are infected. One line of attack into slowing the spread? Well, it's the soil. Preons in soil are a key to chronic wasting disease, but not all soils are created equal. Here to explain more is Jud Aiken, Professor in the Center of Preon and Protein Folding Diseases at the University of Alberta in Edmonton, Alberta. Welcome to Science Friday. Very good to be here. Thank you.

Nice to have you. It's still so mysterious to me that something as simple as a protein can actually cause disease. Describe how preons actually work to infect a hose. Yeah, it's not simple, but basically with regard to chronic wasting disease, the disease agent is taken up, this abnormal form of the prion protein, is taken up by the animal. It enters, we first detected in the lymph system, it eventually migrates into the brain. The abnormal protein is neurotoxic to brain cells. Now, this is a protein that all mammals have.

the sequence varies from species to species. It's not an issue, the normal form of the protein, where the problem comes in is if an animal or a human, for that matter, gets exposed to this abnormal form, which is structurally different. It's an aggregate of a protein, and it is very difficult to degrade, and as I said, it is neurotoxic. Why then is the soil such a key component of chronic wasting disease? cervids deer moose elk shed infected deer moose and elk shed infectivity into the environment it's released in saliva

urine feces and so it will interact with soils and it binds to soils and can be a source of infection to other animals and your group found that organic matter might help determine if preons become infectious or not Yeah, well, what we, we've been working on this for many, many, many years, and some of our studies looked at soil minerals. This was looking, more recently we've looked at organic material, and what we found was the humic acid, a component of soil, organic material, a component of soil, has the ability to degrade the prion protein, reduce the amount of abnormal protein, and reduce infectivity. I want to stress that this does not completely eliminate infectivity, but we certainly saw a reduction. So it's not the sole answer, then, is what you're saying? I think it's a great starting point. It's not easy to find something that effectively degrades the abnormal form of the protein.

They're very resistant to all sorts of treatments. The soil is not the only way the deer get. The CWD, they also get it from each other, correct? Yeah, there's clearly animal-to-animal transmission that can occur. The disease agent is in saliva, for example. And then there's animal environment to animal. A lot of debate in the scientific in the CWD world about the ratio of those, but both certainly do occur.

Do we know the origin of where it first started? Well, I don't really know the origin. We know the location it first started was in Colorado and Wyoming, probably in the mid-1960s. But as you indicated in your initial comments, it has spread quite a bit since then. How do we know about getting back to treating the soil with humic acid? How do we know, how much do we know to prevent the spread of CWD? I'm not proposing treating the soil with humic acid. I'm saying is that humic acid in the soil can be.

degrade prion protein. We have really few options right now on controlling it, and this is always a fatal disease, I should emphasize. Yeah, and I guess that's a call out to Wisconsin people, people who are around deer, about being careful about eating and the hunting season. Let me talk about the human risk, because this is important. I mean, this disease, I mean, we kind of get lumped with mad cow disease, which is a known zoonotic, i.e. Mad cow boving spongiform and cephalopathy does,

has been documented to transmit to humans. There is no evidence chronic wasting disease transmits to humans. That's not to say it can't happen or hasn't happened, but we have no evidence it does. The disease, maybe putting a pot more positive, the disease appears to be more like sheep scrappy. And sheep scrapy has been around for a couple of hundred years, and we've not seen a human case linked to that. So we have no evidence. I'm sorry, we have no evidence it

transmits to humans, but there's uncertainty there. I'm going to zoom in for a second on the practical side of chronic wasting disease. Sick deer and how to manage them. In Wisconsin, CW.E was first detected in 2002, and the state has been trying to manage the spread

ever since. And one area where that gets complicated, the state has many captive breeding operations where deer are provided for hunters and those deer two are getting sick. Wisconsin Public Radio reporter Rich Kramer is here to explain more. Welcome to Science Friday. Thanks for having me. Let's talk about why are captive deer a disease management problem in Wisconsin.

Aren't they behind fences? They are. Wisconsin has, like many other states, has a number of deer farms around 380, and those are broken down generally into two categories. You've got your breeding farms and then hunting preserves where they sell hunts for trophy deer. So there has long been a concern about these farms as a potential source of CWD because it's a concentrated area. Deer are in contact with each other close quarters. So the thought is that if it gets on these farms, it can spread quite quickly.

And through some reporting, we found out that in 2013, our state's Department of Agriculture, Trade, and consumer protection changed policy to allow some of these facilities to continue operating, even though they've got the disease on the landscape. You also talked to a deer farmer whose deer seem to just get sick out of thin air, right? That's right. I talked with Greg Fleece. He's considered one of the biggest names in deer farming, not only in Wisconsin, but also nationwide. He said that he had trouble believing that one of his animals tested positive,

and he suspected that the disease was already on the land when he bought it, fenced it in, and turned it into a hunting preserve. We never took a deer from anywhere else other than this farm that's never had a positive. We put them onto that property, and once they were on that landscape for a while, all of a sudden, we started getting some positives,

and it was one in the first year, a handful of second year, and it's gotten to be more and more. So did the deer farmers see CWD is a problem for their businesses? Oh, absolutely. Wisconsin has rules that if you do contract CWD, you cannot move deer to other farms.

And for a breeding facility that either sells animals to these hunting preserves or to other deer farms for genetic, you know, to incorporate some new genetics into their lines, that's a death sentence for that business. So they are concerned about it, but they also stress that they're not the source. They try to distance themselves as much as possible from the accusations that they are just contributing.

So what about then the wild deer, did they need to be kept safe? from the captive deer, or is it vice versa? Like, that's what the farmer, the deer herder seemed to be saying. Absolutely, yeah. That same concern about the CWD coming from the outside in, you know, it's the same concern from the inside, going from the inside of the farm to the outside, to the wild deer.

And this is why a lot of talk has been happening in Wisconsin about requiring an additional fence, you know, two fences that are eight feet tall, but even that has been tough to get any action. action on. Right now there are rules in the works, but there's a lot of pushback from the deer farmers because they say it's very expensive and could put them out of business. I see that. I know the deer hunting season is wrapping up for the year, so let's talk about what's next for the CWD problem in Wisconsin. Can it be contained?

Well, it is spread. As you mentioned, the first cases were in 2002. Right now, we've got 55 counties across our state that have been listed as CWD affected. Just this year alone, there was a record number, I believe. It was 900 positives. And overall, since 2002, there have been 5,000. So the disease is definitely spreading. There have been efforts to try to contain it, and we've got new leadership in the governor's office.

And this new governor has been pledging to do more science-of-a-science-based approach to try to keep the disease from continuing it spread across the state. Well, thank you for enlightening us, Rich. Thanks for having me.

Wisconsin Public Radio reporter Rich Kramer, reporting on what's going on in O'Clair, Wisconsin. I want to also bring back our guest, Judd-Akin, professor in the Center of Preon and Protein Folding Diseases at the University of Alberta in Edmonton. We just heard from the managing problems about them in Wisconsin. Are Prion diseases easy to manage in general? Oh, geez. There are a huge, huge challenge. I mean, first of all, we have no treatment. Second, the animals appear healthy for most of the disease, i.e., if you're shooting an animal in a area that has CWD, you can't look at that animal and say whether it's infected or uninfected unless it's very far into the disease clinically.

So that's a problem. The testing requires a laboratory. And generally, you've got to be looking at a large number of animals. If it's early in the disease in an area, you've got to be looking at a large number of animals. number of animals. And you've got a detection system that is rather clunky at best because we have to be able to distinguish abnormal form of a protein from a protein that is already there, normal form of the protein. They're just structured differently. What about are there, are there prion diseases that are threatening to people? You said this is a very low chance of people getting infected

from CWD. What about other preon diseases? I didn't really mean to say low. I I just said we really don't know the risk. We're cautiously optimistic that it might be low, but we certainly don't know. Mad cow disease is clearly a zonotic disease, i.e. it can transmit to humans. Sheep scrapy, peers not to. There's a recent preon disease discovered in camels, of all things, last year in Algeria. We know very little about that. It was only a very, very recent discovery.

Wow. I'm Ira Plato. This is Science Friday from WNYAC Studios. Is it possible then that there are health problems that prions could be causing that we have not identified yet? We have not attributed them or isolated them to prions. That's an interesting question. And I'm not probably the one to answer that. They're clearly neurodegenerative diseases. I mean, they're not preon diseases. But, I mean, there's an argument that Alzheimer's, beta amyloid, tau, they can accumulate by a preon-like mechanism, perhaps. Regarding chronic wasting disease, if it moves into humans, we don't know exactly what we're in humans, what we would be looking for. I'm not sure I'm answering your question.

No, no, you are. The best you're going to get right now. Well, you're basically saying there's no great news about it. And maybe there's hopeful news. I mean, is it possible that we might be able to get any closer to a vaccine, for example? Yeah, no, and that's a very good point. There was a recent vaccine that failed miserably. For chronic wasting disease, I do know of a couple groups that are actively trying.

They're very innovative, very smart people. I'm hoping that something comes because we need something. We need something to deal with the disease. Here in Alberta, the disease is moving north and moving west. As it's moving both directions, it's going to start overlapping caribou. Caribou are the same species as reindeer, and we know from Norway that reindeer can get the disease. Wow. So it's in three provinces in Canada right now?

Yep, it is. Initially it was in Saskatchewan. This was a game farm animal that was brought. infected elk into Saskatchewan. Eventually it moved into wild, wild cervids there. It spread into Alberta. It's close to Manitoba, but it's a disease that is just gradually spreading unless we're moving the disease agent unintentionally vehicularly.

And I do want to emphasize that the problem with the disease is the nature of this infectious agent, it's difficult to detect, and the disease doesn't show as a clinical disease until way toward the end of the infection process. So there's no mass screening, then, for this in the animals? Not easy, no. No. And it's just, and a future of soil research, perhaps, hopeful? I'm hoping.

I mean, clearly we're looking at what is in this organic component that's degrading prions. And I'm quite optimistic about it. I think it's a bit speculative at this point, but certainly something we are actively looking into. Well, we'll be following it with you. You know, the right time of the year to talk about it since you say reindeer are also infected by it. Anything that gets the public attention, right?

I mean, you don't hear people talking about preons very much. So thank you very much. And have a happy holiday. A.T. Jung, and thank you for taking time to be with us today. You're very, very welcome. Take care. You too, sir. Judd Aiken is Professor at the Center for Priyons and Protein Folding Disease at the University of Alberta in Edmonton. One last thing. Robots, they need nimble fingers and hands if they're going to be more like us. And researchers at the University of Cambridge unveiled a prototype

hand this week that plays the piano with literally just the flick of a wrist. There are no actuators in the fingers, according to lead author Josie Hughes. just the natural mechanics of human bones and tendons. Think of thing, that unattached hand in the Adams family, and you get the picture as unveiled in the journal Science this week. Here's a little holiday sample. Yes, on that note, I don't want the year to end before I thank all of you, Science Friday's donors and folks supporting your local stations.

Individual contributions are a large part of public radio success, and if you haven't made your year-end donation, please, Consider doing it now. So public radio needs your support. Please do that as soon as you can. BJ Lehman composed our theme music, and of course we're active all week in social media. You have a smart speaker also. You can ask it to play Science Friday whenever you want.

Every day now is Science Friday. In New York, I'm Ira Flato. Merry Christmas to all, and to all a good night.