Hey listeners. Women have more wealth than ever before. There also way less likely to invest their money. That's why there's a giant wealth gap that gets bigger every year. Wealth simple, makes investing easy for everyone, and just five minutes, though build you, a low fee, personalized portfolio, that's right for you and you can talk to their financial experts whenever you want and when you get started now at wealth, simple that come slash, breakfast you'll get your first. Ten thousand dollars managed free welcome to stop. You should know from house networks that are more than just part with throws. The jury not a day for podcasting I guess I'm like one yeah public radio, gals

the early two, thousands, Sarah yea area, member Thea, sweaty balls, the classic bed yeah I was failing like I was yawning as if I had been up all night with insomnia. That's ok because of the weather out some simple play. Acting, I gotta get us going. You know it's weird. I got a little tired studying this yeah, it's crazy! How suggestive house of works articles are well you're, not That way, now I mean like a suggestive now why Jerry did not get any sleep last night we're talking about before we recorded she got like a couple hours like one of those. Were you wake up and then you to stay awake for hours and hours, and I told us that you may have been sleeping during some of that time. Casinos sometimes you'll be distressed

not sleeping and you'd, be a man, I'm just awake, and when you wake up- and I was just dreaming about being stressed about being awake yeah well, what's the Answer- Jerry she's, tired, I'm gonna answer, for I think you should buy them typically get insomnia, but sometimes I can cite myself out a little bit mentally with a bout of it. That's what I'm saying. They're gone like our man, not this oh yeah, once you start, can about each of us. It is over. So let's that is. Why might call typical standard insomnia get us nowhere about no know what we're talking about is a very very rare genetic disorder whilst Ivan genetic Disorders, Neuro degenerative disorder. I guess it's what you call it yeah, it's called fatal, familial insomnia fatal that should take You need to know this is and saw me that will kill you exactly like without know its answer, but you will die.

Like I said, it's very, very rare, Chuck yeah, they think that possibly a hundred people since they started analyzing. This crazy noticed it. I think in the eighteenth century, probably have died from fail. Familial insomnia says a very rare disease and but it's also yonder familial. It's very frequently passed down along family lines. He almost always so they think tops forty families are touch. Yeah and I already misspoke, which will correct later, but at its insomnia. That will kill you and that's not really Casey Insomnia, just a symptom of a larger problem in your body. That will kill you. Yes, but the insomnia does not help sure had makes everything worse. So the history of this is a little murky, but they

traced back to one of two people. One is a guy who they just refer to his patient zero, who possibly died in seventeen sixty five in Venice, Dennis ITALY. That is right yes and the other one is a guy who died in eighteen, thirty, six, probably of fatal familial insomnia, also in Venice ITALY. What's going on over there, Well, there's probably these may be tainted past us asked who knows with what happened, but the whole thing. There are different ways that it could get started, but the this, however, the patient zero was it's been passed down along their family wines. Those two families especially have the did not farewell bright over the years through said it. I have no other.

Extremely said. Yeah, like I said it's, not you not dying just cause. You can't sleep at night after night, that is just a symptom, but what we're talking about in a larger senses, something called a pre on disease. These are super. Air and characteristic of a few things that the colonists Spongiform Disease, which means you get tiny little holes in your brain spy sponge brain and there is no and one of the weird things is, a failure to induce inflammatory response, yeah, which I guesses, the body first saying hey, something's wrong here, I'm gonna pop up, It's an angle sprain or you know, like a disease. Little that'll make something inflame. That's a sign of your body trying to fight

something else that is actually a lot of controversy about pre on diseases, because it doesn't make any sense. Yeah, it's an infectious agent right It's really just a miss folded. Protein yeah, it's real! Bizarre because, with any other kind of infection, you have a viral infection bacterial infection in a pre on infection, viral infections. In bacteria, factions have dna or RNA. A protein again is just a pre honest, just a misfortune protein, so it shouldn't be able do in fact anything, but it does so it's this crazy medical misery that are still trying to get to the bottom of, but as they do diseases fatal, familiar insomnia or Creutzfeldt Jakob Disease, which is like me, human mad cow disease, is spongiform disease

sponge brain yoga happens with animals and humans, and I think it's pretty important yeah so in in In many ways it makes a lot of sense that you would be the past down this problematic is the autism more dominant, neuro, degenerative disease, fatal familial inside it makes sense that it would pass down along similar lines, because there is a gene that, in under normal circumstances, expresses a protein, the pr pc protein, which is an all party, we don't know what it does, but we do know that it probably. There has to do with a copper ion transport into the cell. Thea it prevents cellular death until the time is right or it helps create, the sheets around your nerve ending, so you not inconstant pain, yet they think it's those right yeah, but because it's the brain there still some mystery and anything. It was like the middle,

in eighties, when they finally name that's right, yeah, so pretty on the scene as far as this is a rare right, but not even just fatal. Yo insomnia. It wasn't until two thousand five that they the these risk. Just at the University of Texas in Austin. Basically, Aruf irrefutable proved that premiums Miss folded, proteins, are in infectious agent, even though we have no idea how this is happening here. It's it's true. There's three: ways of getting an infection in a mist folded. Protein is one of em. Like you said they can occur in three ways. One is acquired, It means you haven't infection well in the case of through, and I guess we should, about K. You are you that is when you get infection because you ate someone's brain yeah, I think we talked about them. Anabolism episode, don't you seem familiar in Papua New Guinea in the fifties like, I guess, a local? I think british, governor or agent

started noting that there is a thing that the four people who practise funeral Cannibalism, they included the evenings of the deceased brain would come down with a disease that they called Currer, which meant like trembling inferior, yeah and they started investigating a little more and that's when we started to get the idea that there was such a thing is prior disease. So you could catch a pre on well disease from eating brain yeah it would render them. You know they couldn't eventually walk or talk or eat. You they would just lose all their motor function basically and waste away and die because they can't swallow or to anything. Exactly so, that's when we first started really noticing in humans. This whole idea of sponge brain problems here and as the acquired rights right. There is also one called sporadic which basically you just

the sudden, develop fatal, familiar insomnia, yeah nowhere comes from and how its cause no but doesn't know didn't effect. Like one of those two, but that there are those two different ways- and I know there's a third one, but don't they make you suspicions of what the heck was going on in venison lily eighteenth century totally, because it the they were both of those No patience, zeros were unrelated right as one would seventeen sixty five, almost eighteen, thirty six, but even still, if they were in the same family they'd, be like, while these guys were of the same family. So probably right one from earlier from what I've read: they're, not in the same failures happen to live in the same town. I think that is correct, so I wonder people were eating some weird stuff there, and maybe one of em sporadically developed, fatal, familial insomnia, that's nutty! So there's a third. Way to right, yeah inherited, which is before us this fatal familiar insomnia. Its

always inherited from from your family's genetics. Almost always yeah, but not all pre on diseases are which is by the way, is derived from the words protein. Infection, yeah, there's a guy in nineteen, eighty two who arm coin the term. I ended up winning the Nobel Prize and I think, ninety ninety seven, because of his early studies, name Stanley proves prison even the Nobel and ninety seven and even after he won the Nobel people like this weird about impossible, there's no way a protein can in fact other proteins right, but that's exactly what happened so

like, I said, there's a there's, a normal protein, the pure pc protein night. Yet and then there's the spongiform version of it, the p r p s see right and it's after it's been folded. Yes, the folded, basically mutated, verge right, so the whole thing comes down to what's call the polymorphism, I'm a code on which is it a sequence of nuclear tides, amino acids, yeah, I'm your dna, I own a gene and the specific code on these say three nucleotides say you guys mean we're gonna code, this protein and under normal circumstances, on this gene, the pr pc protein is coated. But if you have a different nucleotides combination gear, you start coding the p r p s c.

That's right protein! That's the miss folded, one normal enough right! Ok, yes, we can cope abnormally. Falling proteins is what cancers and all that stuff. The thing is once the patient starts, building these proteins abnormally those parties going and somehow infect the already properly folded proteins that were expressed in the brain, swear before yet a bind to him there. How or why, in the bizarre near the solely buzz and what's that happens, you're in big trouble, you aren't they double and we'll get into some of the symptoms and stages. Right after this message right, hello stuff, you should nurse. Do you want a new year's resolution? That's easy to keep yes resolve to help. Put to your identity and personal info with lifelike identity theft, protection, lifelong alert, you to potential threats to

I didn't he and they see more than what you can see by just monitoring your own credit like your info on the dark web and that's a big deal yeah, and if you the problem. Lifelike? U S based restoration specialists, no, the steps it take to help resolve your case after all one in five identity theft, victims who had accounts open in their names discovered their theft through bank or credit card company, and, of course no one can prevent all identity theft or monitor all transactions at all businesses, but lifelike is the new year's resolution does not only easy to keep it'll help. You protect what you ve worked so hard for get lifelike for up to twenty five percent off your first year, gonna lifelike, dot, com, slash stuff, that's lifelike, dot, com slashed stuff. For The twenty five percent off all right, so we are back with some symptoms in stages, and this is

general for all pre on diseases. They're gonna share some symptoms like a handful of em, yet fatigue. It's one cognitive Why, unlike you, can lose some memory? You might develop dementia rigidity with like men walking hallucinations, but they don't all have the same symptoms. You might get some of these and not get others right. The whole a fatal, familial insomnia is appropriately enough for insomnia, yeah, that's like when you one of the really sad things once you start to notice that you're already, although they can't cured anyway, but sometimes it's nice now these since early on, but once you start noticing, saw me it's pretty far along yeah and most don't know that they have an early on, because the mean age of answer is fifty years old and it can all over the place like they have seen cases as early as nineteen,

I ve seen him come on his latest seventy two year to do in pretty good, but for the most part strikes you around late, late, forties, early fifties and once it happened between one may be three years of basically living hell. The edifice you die of this year, there's generally forced stages of effort by the first of which is in us, with the insomnia and over about four months, is going worse and worse but, like I said some people, how does it later on after other symptoms become known, but you might start to have like attacks. Phobias paranoia, we need do manage to sleep. You supposedly have like super vivid drink, which is it but for the most part you're having bouts of insomnia big time you stay,

to get even worse. The some states less about five months on average are typically yet, which is to say it has in less than a hundred people in the history of earth sure, but the the you enter the stage called Syn that a hyper activity, which is where you just keep up all the time. Do you remember we ve talked insomnia and sleep deprivation. In a couple of pike ass before and how just totally unhealthy, it is one of the reasons it is sold This is because your body entered a state of constant stress reaction yet and that's what sympathetic hyperactive is it like your breathing? Is elevated? Your heart beat his elevated your core body temperature is elevated. You're just turned up all the time and you're not getting the sleep to knock yourself out of that state in the regroup and regenerating rest yeah you're gonna. At this stage, you're gonna have some memory loss

short term memory. Loss mood, changes a lot of anxiety, oppression and you're gonna start to have some motor issues as well like the way you move in way, walk right, two things are starting to get pretty bad. At this point, you probably pretty freaked out Firstly, if this doesn't run in your family required under its sporadic case. We are united. I went to a doctor and they're gone. It sounds like ever find me because I'm heard of it my life exactly well, that's not your doctors of her this, but you know I mean like two of them have yeah it's not the the first go to. I think when you say I've been having trouble sleeping and I'm agitated the Publican ass, like what kind of drugs you ve been doing. Yeah I like to slay off the pie, that'll be fine. Well, that should make you sleep there right now. I guess employee depends on what type of path. I would think it's more like lay off the speed. Oh yeah, you know I am, I probably should be gone

all right. So the third stage is pretty short about three months long. That's when you're really delving into hard core insomnia, whimsically withstand stage one stage to appropriate enough, you're sleeping but you're only entering stage, one in states to sleep right, which is stage one is considered or you're just very relaxed here. Stage too, is where you're starting to sleep, but you can be woken up very easily like Jerry and you're, not yeah, and you. Going beyond that, you're, not going in stage three or stage five, which is our aim sleep. They combine states three stage for apparently, yet you're, not getting the sleep, so vice the third stage of emu, Familial insomnia you're not even going to one or two bridges, not sleeping at all, and it's been like this for nine ten months already, so you just pay

losing it at this point a year, and that will deliver you to the end stage stage. Four has got end stage Your headed, they're, serious dick in dementia in brain activity, maybe you ve got about six months at that point, the chicken Is the ability to speak and move yet it's called a kinetic monetarism and basically fall into a coma death. So, with with economic me You actually have the ability to move and speak, but you lack the basic will to do so. Apparently, like patients have come out of this, have reported that will not necessarily ever five because it always fatal and how two percent of cases but people who have had a canary commuters and for other reasons, have said like I like it, you, I could a brain, each time I got the will to move right. There was something else does

reacting that there was stronger and this could move and couldn't talk, she's yeah, then, unlike you said, you go from there into a coma right and then death, yes and all pre on diseases are fatal. At this point and unclearable correct, I guess so. Yes, further now so he took with what's going on in the brain here. Well, basically, your central nervous system is starting to break down the end your ventral and media dorsal thou neurons. So there's a neurons in your thalamus that basically manage your motor functions. They start to die out and instead of being replaced, like you, He likes to do and sells die out with healthy ones. They don't you ugly ourselves, which wouldn't we talk about glee ourselves. We ve talked about him a few times a year. We ve been some very annoying before that there be Equally, the cleaning service for your central nervous system. And they help out with communication there they start to die. In Florence. Our tissue

the thalamus and once that happens is called closest. You just got scar tissue instead of healthy cells, writings, acclivity, rapid decline from there. It kills the communication between cells right yeah. So with fatal, familiar insomnia like what you ve just described, is there, results of any spongiform pre on disease right, yet which again we're just tiny little holes in your brain so it seems like the distinction between the different spongiform diseases is what part of the brain. Specifically they right, fatal, familial insomnia. They attack the thalamus, specifically the hypothalamus and specifically the parts of the hypothalamus, their help, re regulate sleep and theirs is part of your hypothalamus that isn't it had it hey. What's what you could call an anti awaking system yet too, where not only on the nerves, shut off in one respect in another,

a bunch of neurons that off while you're asleep, are on re interest. Keeping you asleep so your waking. Normally those neurons are off and when you're sleeping there on the problem is fatal. Familial insomnia, the pre ins, have eaten away at this and now, all of a sudden that their anti awaking system that keeps you asleep when your sleep allows you to go to sleep. The transition from one stages, deeper stages. There is no longer active any longer and so the only that is active is your wakefulness and it is on all the time. You know you're dying and there's this it's gotta be some sort of madness for not being able to sleep. It just exacerbates everything yeah they came is just one of them

it is in it is a symptom, but it's also it's. It's also directly related to the mechanism of this disease, and it's got to speed up the process, because your bodies not get the rest. It needs exact on top of everything else in in that's the that's, the devious, The whole thing is not only is your body not getting the rest. It needs its on all the time. So it's it's kind of like doubly hard core. His farmers disease, go so like we said diagnosing, it is tough because it's a rare, be the symptoms, are in other always patient reported. So, like a said, doctors not gonna, like look at this first thing go on with your family history and maybe get some blood test on exam spinal tat. You might an mri or a pet scammer pets game tat scan you ve, never believed in Paris,

or an e g which measures, electrical activity in your brain, but it's really tough, it I now saw yet none of that is gonna work until you ve already entered that stage. Yeah like you, ve already entered the first stage, at least because those those tests oh yeah, you have insomnia and then once they establish You haven't seen near than they have two further established. That is fatal, familial insomnia and, by this time, also chuck its say about if EU about fifty years old yeah, you probably already had kids and so now what find out you have fatal familial insomnia, you're, also terrified that you ass it on your children. They get about fifty fifty two yeah incentives in order, so more dominant trade disease condition. All you need is one with it to pass it on to you yet so sad, it's like a wolf. Basically,

am I dying of because he can't fix me an hour. My kids gonna die. This is well and then their kids very horrible disease. All right we're gonna get into, I guess finish up with a few. Information on a few more these pre on diseases right for the spring. new year's resolutions, are very, very difficult to keep git exercise save more money. What about this? We have a resolution that you can really work with stop wasting time going to the post office that right, you stamps dot com instead because they bring all the services of the. U S: postal service, right to your computer, with your small business sinning invoices

or packages or an online seller shipping out products stamps that can handle it all with ease yank it. This was simply come you get five cents off every first class stamp in up to forty percent of priority mail. This right? U S, postage twenty four, seven for any letter, any package, any class anywhere you want to send it! There's no risk and using our promo code, ass white, ass, gay you're gonna get this offer, afore weak trial, plus free postage and a digital scale, and there are no long term commitments or contracts just go to stamp that come click on the microphone off the top of the home page and type in s. Why, as K that stance, I come from a goat s. Why, as K stamps that come ever go to the post office again before we get on this. The other pre on diseases we do need to talk about treatment, we ve said over and over sadly there

is no curative treatment, but there is palliative care, which basically means we're gonna, try and help you out. It is much as we can to be comfortable as you die here and weirdly, one of the things that they are looking into and trying his giving patients g h be the club drug that you hear about to help people sleep and there actually prescribing that in certain cases they ve it apparently gives them quick, small short, sleep, but it's I'm sure, incredibly wonderful, sleep. Nonetheless, yeah might decrease your heart rate and body temperature little bit too, but I'm at that point Mina, when you're taking G h be to get twenty minutes asleep. You ve got a pretty sad and coming in

Harrison very said. The other great hope is gene therapy or basically, the delete the gene that responsible for making this protein miss Fold and insert the correct version of it. That's gotta be the future of these cures. Yes, Kennedy the Future Auditors will weaken this Bree right that code of our Jeanne, the ETA in M, make it expressed properly. But until then, there's gonna be some problems for people with spongiform diseases, because there's nothing you can do yeah he's a including with fatal familial insomnia gives somebody Jake be that's about it. Shall we go where few more these yet they're, all equally devastating? I think you did mention see. J D creates felt Jacob or yak up the disease life. The outcome, maybe any jealousy J, a k, o b, I think of it.

Like? You know german pronunciation yeah, it's probably right. This is most prevalent and it is the spawning its occurrence, which is really creepy. Well, you can get it to from eating it. I was that really think ten percent spontaneous and the rest you get from its acquired knows his temper centre inherited. Ok. So that's the one, if I'm not mistaken, there was directly related to the outbreak of mad cow disease. Like you, mad cow disease out, remember back in the nineties with the mad cow outbreak area, while that all came from feeding a bunch of cattle, a lot of ground beef that included cattle brains, yeah that have pre on diseases in it. And so the cattle got mad cow disease and from eating that cattle we got Creutzfeldt Jakob. Ok, that's the human version. Yes, and that's it that's kind of points out, the problem with priority since they're, not biological or not.

Living in the sense that we consider and infective agent. Typically like a bacteria or a virus right, there's no information to destroy like using heat or bleach wherever it can't be killed, as I saw it put somewhere else. It's the perfect pathogen. Yes, that's a mouthful, it is, but is also horribly scary, it is another spongiform diseases are scraping. That sounds dead men here. That is just and goats and cheap those of humans. Don't need to worry about it, It is also genetic and have no evidence right now, but that humans can get it now. Got enough to wish her talk about mad cow. That is officially called Bovine spongiform and set foe apathy. The right I think so dear and Elk might get chronic wasting disease western. You ass. If you see a dear, that is

We skinny and ruling can't swallow they may have chronic wasting I've seen that humans can have that is well, but It's the same version. No, it doesn't seem like a kid. They think it sooner. Patients that have like that have died from AIDS, though often like waste away in like no amount of nutrients would keep him from this losing wait until he basically to die from wasting away, but I do not think is related to a brain disease and then check also going back to see J D, which is what we call it now officially forever. Creutzfeldt Jakob J D out. There is an outbreak of it in the eighties and know the nineties. These french doctors were using it in growth, that. They were giving as injections, two's kids, who had stunted growth

and they were getting them from pituitary glance harvested from humans and sold along the black market. Westley bunch of dead people in Bulgaria had their pituitary once removed and sold the doctors and France who are using them. That's crazy, yeah. In these growth, things deserve growth, hormone shots that they were giving kids like a sixty year. Eighty kids die what uses the nineties, while yeah that ninety nine like seventy nine is so it's very weird because we go from nothing recognizing diseases like fatal familial insomnia as a disease until the eighties right having an outbreak of mad cow disease having an outbreak of sea J D, always in a couple

ok there and all the while people are saying like no pre odds can exist. What you're saying is, is it can't be possible in a lot of people tried to disprove pre on yeah by saying? Ok, there are different types of scraping and see one has an indifferent incubation period. Southern one you'll get the symptom, but you won't like that. The sheep or talk will speak Russian. In the cellar farmers creepy. They speak swedish here and there a thing like this proves that these prayers have some sort of virus associated with a network. Just missing write another group for another argument against freons was well. Alzheimer's disease is technically a bunch of form disease again, Miss folded, proteins, creating plaque build up.

Scar tissue in the brain that leads to all these same symptoms is say, see J D guy. It's an infectious the what's going on here, so they said that there is one way to prove this net is to create a pre on in a test to completely from from the whole cloth and put it someone's brain in a healthy brain is exactly that they didn't. He doesn't five at University of Texas infected the other proteins. That's right so like the odds are this weird thing that we didn't know that is now infectious was even possible were finally wrapping our heads around it spongy whole filled heads. I think this is gonna, be not this particular, but that this is gonna, be what's going to wipe out the human race one day I'll know a bullet making a pretty good case for itself. Lately now ass, we say and do some disease yeah like, and I think we're gonna blow ourselves up with her

like round the oder blower ourselves up with nuclear bombs. I think it's just can be another like weird plague or something that we don't understand due to our close association with livestock and it's not meet up soon enough yeah, you worried, worried, I'm. More realistic, give your safer, I'm your building in the sight of a mountain. No, that would indicate I was worried. Ok, I'm building my! U guarding them, or can I come over to get you got anything else now? Ok, let's fatal, familial insomnia, embryonic if you want to learn more about the things you can take those words into the search bar at house efforts that and camp said that, aside for listening Michael canadian email from our canadian kid from Cambodia from Canadian hey guys. My name has been master a seventeen year old, Canadian, a he said that I didn't know that, then that makes me think is probably not can

I've been worthwhile. He was just. I think, I've been working my way backlog of episodes in a recently listen to the absurd and serial killers in it. You spoke of the man who killed by feeding his victims to pigs, Dixon Rubber picnic says I do remember that cause it something bigger than now he's pretty serial killer from Vancouver. This reminded me of a true story from my dad's childhood on a farm in HOLLAND Michigan. He was growing up you as often given the job of feeding pigs, a job he despised. Much of this back to a horrible accident that happen to a nearby farmer on this particular farmers out in his barn feeding, pigs had a heart attack. Collapsed among them. I want to say we found him years later, other remained of his body where the palms of his hands and the souls of this feat yet pigs hate. Those parts is, experience, understandably instil fear into my dad for many years to come. In the pianist item by item anyway, thing are you guys thanks Roy, you do a shout

would make my day, maybe even my year so been mask shout shot up. Thanks for the email I stay. Flappin ever heard. Much about rubber pitted. I don't think so, Hughes operating in like their ninety ninety. They think the news is like having passed, it's over and then he'd murderer undue horrible stuff but so do I m, like their new supplying the public is a pig farms is applying the public with pork I think that he ground up people and to point out that when people, the port, no, no like he ground up people in mixed together with ground port and then sold as ground. Pork asked was even worse in the ninety nine. It is a bad man. Where was he Vancouver S, Father speak or not,

Now, however, picked them in Arabic it. So if you like Ben Mass, shout out one I get in touch with checker me, you can t to us it s my escape. I guess you can join us on Facebook, complex that we should now begin. Citizen, email to stuff, Pakistan, hausa foresight, CUP and, as always, at home on the web stuffy should know doc.

For more on this and thousands of other topics for the House of workshop com. Dear young rockers applaud, cast coming of age story about finding a home and rock music and learning to flourish in your own wheeled way, it's also a series of letters of advice to my younger self ass. She navigates ashes, adolescence seals with social inside each body, issues and relationships and discovers a transformative power. Visa during rocker comes from double Elvis productions is created and hosted by mutual citizen and executive produced by Jig Brennan of disgrace. Land episode, one drops ones: a genuine fifteen, listen dear young locker. On the I hurt radio up apple pod costs, or were you get your podcast