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Is there a disease that kills by preventing sleep? · Stuff You Should Know

0:01
Speaker 1: Welcome to you stuff you should know from How Stuff
0:04
Speaker 1: Works dot com. Hey, and welcome to the podcast. I'm
0:12
Speaker 1: Josh Clark with Charleston, Chuck, Bryan and Jerry and this
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Speaker 1: is not a day for podcast. I feel like I
0:21
Speaker 1: sound like um one of the the public radio gals
0:27
Speaker 1: from the early two thousands. Saturday. Oh yeah, yeah, yeah,
0:32
Speaker 1: I remember the uh shwetty balls. It's a classic bed
0:37
Speaker 1: good times right the Yeah, I was feigning like I
0:40
Speaker 1: was yawning, as if I had been up all night
0:42
Speaker 1: with insomnia. That's oh, I thought it was because of
0:45
Speaker 1: the weather outside. Just a little play acting I got
0:48
Speaker 1: you to get us going. You know, it's weird. I
0:49
Speaker 1: got a little tired just studying this. Yeah, it's crazy.
0:53
Speaker 1: How suggestive How Stuff Works articles are. Well, yeah, not
0:57
Speaker 1: in that way, No, no, on that way. I mean, like,
1:01
Speaker 1: you know, suggestive in that way. Jerry did not get
1:06
Speaker 1: any sleep last night. We were talking about it before
1:08
Speaker 1: we recorded. She got like a couple of hours, like
1:11
Speaker 1: one of those deals where you wake up and then
1:14
Speaker 1: you just stay awake for hours and hours. And I
1:16
Speaker 1: told her, I said, you may have been sleeping during
1:18
Speaker 1: some of that time, because you know, sometimes you'll be
1:21
Speaker 1: distressed about not sleeping, and you'd be like, man, I'm
1:23
Speaker 1: just awake, and then you wake up and you're like,
1:25
Speaker 1: was I just dreaming about being stressed about being awake? Yeah? Well,
1:28
Speaker 1: what's the answer, Jerry, she's tired. I'm going to answer
1:32
Speaker 1: for I think you should. But um, yeah, I don't
1:36
Speaker 1: typically get insomnia, but sometimes I can psych myself out
1:38
Speaker 1: a little bit mentally with about of it. And that's
1:42
Speaker 1: when I'm laying there going like, oh man, not this.
1:45
Speaker 1: Oh yeah, once you start thinking about it, it is over.
1:49
Speaker 1: So that's that is what one might call typical standard insomnia. Yeah,
1:54
Speaker 1: that's not what this is about. No, no, Well we're
1:56
Speaker 1: talking about is a very very rare genetic disorder. Well
2:02
Speaker 1: it's not even genetic disorder. It's a neurodegenerative disorder, I
2:05
Speaker 1: guess is what you call it. It's called fatal familial insomnia. Fatal.
2:10
Speaker 1: That should tell you all you need to know. This
2:12
Speaker 1: is insomnia that will kill you exactly like without know
2:16
Speaker 1: if sands are but you will die. And like I said,
2:18
Speaker 1: it's very very rare, Chuck. They think that possibly a
2:22
Speaker 1: hundred people since they started analyzing this or you noticed it.
2:28
Speaker 1: I think in the eighteenth century probably have died from
2:32
Speaker 1: fatil familial insomnia. So it's a very rare disease. And
2:35
Speaker 1: but it's also you'll notice, familial. It's very frequently passed
2:40
Speaker 1: down along family lines, almost always, so they think tops
2:45
Speaker 1: forty families are touched by it. Yeah, and I already misspoke,
2:50
Speaker 1: which will correct later. But I said it's insomnia that
2:54
Speaker 1: will kill you, and that's not really a case. The
2:56
Speaker 1: insomnia is just a symptom of a larger problem in
2:59
Speaker 1: your that will kill you. Yes, but the insomnia does
3:03
Speaker 1: not help. Sure, it makes everything worse. So Um, the
3:08
Speaker 1: history of this is a little murky, but they have
3:10
Speaker 1: traced it back to one of two people. One is
3:14
Speaker 1: a guy who they just referred to as patient zero,
3:17
Speaker 1: who possibly died in seventeen sixty five in Venice, Venice, Italy,
3:22
Speaker 1: that is uh. And the other one is a guy
3:25
Speaker 1: who died in eighteen thirty six, probably of fatal familial insomnia. Um,
3:32
Speaker 1: also in Venice, Italy. What's going on over there, Well,
3:35
Speaker 1: there's probably maybe so tainted pasta sauce, who knows what
3:43
Speaker 1: what happened, But the whole thing, there are different ways
3:46
Speaker 1: that it could get started Um, but the these whoever
3:52
Speaker 1: the patient zero was, it's been passed down along their family.
3:56
Speaker 1: Those two families especially have did not farewell over the RS.
4:01
Speaker 1: It's very sad. It well, the diseases extremely sad. Yeah,
4:05
Speaker 1: And like I said, it's not you're not dying just
4:07
Speaker 1: because you can't sleep night after night. Um, that is
4:10
Speaker 1: just a symptom. But what we're talking about in a
4:13
Speaker 1: larger sense is something called a preon disease. These are
4:17
Speaker 1: super rare and uh characteristic of a few things that
4:22
Speaker 1: they call it a sponge AFM disease, which means you're
4:26
Speaker 1: gonna get tiny little holes in your brain. You get
4:28
Speaker 1: a sponge sponge brain and uh there is uh neural loss.
4:34
Speaker 1: And one of the weird things is a failure to
4:37
Speaker 1: induce inflammatory response, Yeah, which I guess is that's the
4:41
Speaker 1: body first saying, hey, something's wrong here. I'm gonna puff up.
4:46
Speaker 1: Whether it's an ankle sprain or you know, like a
4:49
Speaker 1: disease that will that will make something in flame. That's
4:52
Speaker 1: a sign of your body trying to fight something else.
4:55
Speaker 1: There's actually a lot of controversy about prion diseases because
4:58
Speaker 1: it doesn't make an any sense. It's an infectious agent, right,
5:04
Speaker 1: But it's really just a misfolded protein. And it's really
5:08
Speaker 1: bizarre because with any other kind of infection, you have
5:12
Speaker 1: a viral infection, a bacterial infection, and a preon infection.
5:17
Speaker 1: Viral infections and bacterial infections have DNA or RNA. A
5:22
Speaker 1: protein again, is just a preon. It's just a misfolded protein,
5:26
Speaker 1: so it shouldn't be able to infect anything, but it does.
5:30
Speaker 1: So it's this crazy medical mystery that they're still trying
5:34
Speaker 1: to get to the bottom of. But as they do,
5:36
Speaker 1: diseases like fatal familial insomnia or um uh crips Field
5:42
Speaker 1: Jacob's disease, which is like human mag cow disease. It's
5:46
Speaker 1: a spongefformed disease, spongebrain. It happens with animals and humans.
5:51
Speaker 1: I don't I think that's pretty important. So in in
5:55
Speaker 1: in many ways, it makes a lot of sense that
5:57
Speaker 1: you would be able to pass down this problematic um
6:02
Speaker 1: it's an autosomal dominant neurodegenerative disease, fatal familial insomnia. It
6:09
Speaker 1: makes sense that it would pass down along family lines
6:11
Speaker 1: because there's a gene that UM in under normal circumstances,
6:17
Speaker 1: expresses a protein the pr PC protein, which is a
6:20
Speaker 1: normal protein. We don't know what it does, but we
6:23
Speaker 1: do know that it probably ei there has to do
6:26
Speaker 1: with copper ion transport into the cell. It prevents cellular
6:31
Speaker 1: death until the time is right, or it helps create
6:36
Speaker 1: the sheets around your nerve endings so you're not in
6:39
Speaker 1: constant pain. They think it's one of those, right, But
6:43
Speaker 1: because it's the brain, there's still some mystery. And I
6:45
Speaker 1: don't even think it was like the midneties when they
6:48
Speaker 1: finally name this, right, Yeah, so it's pretty new on
6:51
Speaker 1: the scene as far as because it's so rare, right,
6:53
Speaker 1: but not even just fatal familial insomnia. It wasn't until
6:56
Speaker 1: two thousand five that they the these res riachers at
7:00
Speaker 1: the University of Texas in Austin. Basically, ir irrefutally proved
7:05
Speaker 1: that preons misfolded proteins are an infectious agent. Even though
7:10
Speaker 1: we have no idea how this is happening, It's it's true.
7:14
Speaker 1: There's three ways of getting an infection in A misfolded
7:17
Speaker 1: protein is one of them. Yeah, like you said, they
7:20
Speaker 1: can occur in three ways. One is um acquired and
7:24
Speaker 1: that means you have an infection. Well, in the case
7:28
Speaker 1: of kuru, which I guess we should talk about k
7:30
Speaker 1: you are you? That is when you get an infection
7:33
Speaker 1: because you ate someone's brain. Yeah, I think we talked
7:36
Speaker 1: about that in the cannibalism episode, doesn't you. It seemed
7:39
Speaker 1: familiar in Papua New Guinea in the fifties, Like I
7:42
Speaker 1: guess a local I think British governor or agent started
7:47
Speaker 1: noting that there is this thing that the four A
7:49
Speaker 1: people who practiced funereal cannibalism that included the eatings of
7:54
Speaker 1: the deceased brain, um, would come down with the disease
7:58
Speaker 1: that they called kuru, which meant like trembling in fear right,
8:02
Speaker 1: And they started investigating it a little more. And that's
8:05
Speaker 1: when we started to get the idea that there was
8:07
Speaker 1: such a thing as prion disease, that you could catch
8:10
Speaker 1: a preon uh well disease from eating brain. Yeah, and
8:16
Speaker 1: it would render them you know, they couldn't eventually walk
8:18
Speaker 1: or talk or eat. They would just lose all their
8:21
Speaker 1: motor function basically and waste away and die because they
8:25
Speaker 1: can't swallow or chew or anything exactly. So um, that's
8:30
Speaker 1: when we first started really noticing in humans this whole
8:33
Speaker 1: idea of uh, spongebrain problems, and that's the acquired version.
8:39
Speaker 1: There's also one called sporadic which basically you just all
8:44
Speaker 1: of a sudden develop it was fatal familial insomnia, and
8:48
Speaker 1: they don't know where it comes from or how it's caused.
8:51
Speaker 1: But doesn't it doesn't the fact that like one of
8:53
Speaker 1: those two, that there are those two different ways, and
8:57
Speaker 1: I know there's a third one, but don't they make
9:00
Speaker 1: you very suspicious of what the heck was going on
9:02
Speaker 1: in Venice in the late eighteenth century? Totally because they
9:06
Speaker 1: were both of those original patient zeros were unrelated, right yeah,
9:10
Speaker 1: because one was seventeen sixty five was six. But even
9:13
Speaker 1: still they if they were in the same family, they'd
9:15
Speaker 1: be like, well, these guys are in the same family,
9:17
Speaker 1: so probably the one from earlier. From what I've read,
9:20
Speaker 1: they're not in the same family. They just happened to
9:21
Speaker 1: live in the same town. I think that is correct.
9:23
Speaker 1: So I wonder if people were eating some weird stuff
9:26
Speaker 1: there and one of them sporadically developed fatal familial insomnia.
9:32
Speaker 1: That's nutty. So there's a third way to right, yeah, inherited,
9:36
Speaker 1: which is for as far as fatal familial insnia it's
9:40
Speaker 1: almost always inherited UH from from your family's genetics. Almost always,
9:47
Speaker 1: but not all prion diseases are, which is by the way,
9:50
Speaker 1: it's derived from the words protein and infection. Yeah, there's
9:54
Speaker 1: a guy in two who um coined the term UH
10:01
Speaker 1: and I ended up winning the Nobel Prize, and I
10:04
Speaker 1: think because of his early studies. Name was Stanley Pruss Prisoner.
10:11
Speaker 1: He won the Nobel in nine um And even after
10:15
Speaker 1: he won the Nobel, people are like, this is what
10:17
Speaker 1: you're talking about is impossible. There's no way a protein
10:20
Speaker 1: can infect other proteins. But that's exactly what happens. So,
10:24
Speaker 1: like I said, there's a there's a normal protein, the
10:27
Speaker 1: pr PC protein, right, and then there's the sponge aformed
10:31
Speaker 1: version of it, the prp sc and that's after it's
10:35
Speaker 1: been folded. Yes, that's the folded basically mutated version. So
10:41
Speaker 1: the whole thing comes down to what's called a polymorphism
10:45
Speaker 1: on a code on which is a sequence of nucleotides
10:50
Speaker 1: amino acids on your DNA right on a gene, and
10:55
Speaker 1: the specific code on these say three nucleotides say you
10:59
Speaker 1: got eyes, mean that we're going to code this protein
11:03
Speaker 1: and under normal circumstances. On this gene, the pr PC
11:07
Speaker 1: protein is coded, but if you have a different nucleotide combination,
11:15
Speaker 1: you start coding the pr p SC protein and that's
11:21
Speaker 1: the misfolded one. Normal enough, right, Okay, Yes, we can
11:25
Speaker 1: code abnormally folding proteins. It's what cancers and all that stuff.
11:29
Speaker 1: The thing is, once the patient starts folding these proteins abnormally,
11:36
Speaker 1: those proteins go in and somehow infect the already properly
11:42
Speaker 1: folded proteins that were expressed in the brain elsewhere before. Yeah,
11:46
Speaker 1: they bind to them, and they don't know how or why.
11:49
Speaker 1: Isn't that bizarre? Yeah, it's totally bizarre. And once that happens,
11:53
Speaker 1: you're in big trouble. You are in big trouble. And uh,
11:56
Speaker 1: we'll get into some of the symptoms and stages right
11:59
Speaker 1: after this message break. All right, So we are back
12:05
Speaker 1: with some symptoms and stages. And this is in general
12:09
Speaker 1: for all prion diseases. They're gonna share some symptoms, like
12:13
Speaker 1: a handful of them. Um fatigue, it is one cognitive decline,
12:18
Speaker 1: like you're gonna lose some memory. You might develop dementia. Uh,
12:22
Speaker 1: rigidity with like movement and walking hallucinations. Um, but they
12:27
Speaker 1: don't all have the same symptoms. You might get some
12:30
Speaker 1: of these and not get others. Right. The hallmark of
12:32
Speaker 1: fatal familial insomnia is appropriately enough insomnia. Yeah, and that's
12:38
Speaker 1: like when you start. One of the really sad things
12:40
Speaker 1: is once you start to notice that you're already although
12:43
Speaker 1: they can't cure it anyway, but um, sometimes it's nice
12:47
Speaker 1: to know these things early on, but once you start
12:48
Speaker 1: noticing insomnia, it's pretty far along. Yeah. And most people
12:52
Speaker 1: don't know that they have it early on because the
12:55
Speaker 1: mean age of onset is fifty years old, and it's
12:59
Speaker 1: kind of all over place, Like they have seen cases
13:01
Speaker 1: as early as nineteen. They've seen him come on as
13:04
Speaker 1: late as seventy two. You're doing pretty good. But for
13:07
Speaker 1: the most part, it strikes you around late late forties,
13:11
Speaker 1: early fifties, and once it happens, you've got between one
13:15
Speaker 1: to maybe three years of basically a living hell before
13:19
Speaker 1: you die of this. Yeah it Um. There's generally four
13:23
Speaker 1: stages of f f i U, the first of which
13:26
Speaker 1: is gonna start with the insomnia, and over about four
13:29
Speaker 1: months is gonna get worse and worse. But like I said,
13:32
Speaker 1: some people only notice it later on, UM, after other
13:35
Speaker 1: UH symptoms become known, you might start to have like
13:40
Speaker 1: panic attacks, phobia's paranoia. When you do manage to sleep,
13:45
Speaker 1: you supposedly have like super vivid dreams, which is interesting,
13:48
Speaker 1: but for the most part, you're having bouts of insomnia
13:51
Speaker 1: big time. Stage two it gets even worse. This stage
13:55
Speaker 1: lasts about five months on average or typically, which is
13:59
Speaker 1: to say it has in less than a hundred people
14:02
Speaker 1: in the history of Earth. UM. But the the you
14:06
Speaker 1: enter this stage called sympathetic hyperactivity, which is where you're
14:10
Speaker 1: just keyed up all the time. UM. Do you remember
14:14
Speaker 1: we've talked about insomnia and sleep deprivation and a couple
14:18
Speaker 1: of podcasts before and how just totally unhealthy it is.
14:22
Speaker 1: One of the reasons it is so unhealthy is because
14:24
Speaker 1: your body enters a state of constant stress reaction. And
14:29
Speaker 1: that's what UM sympathetic hyperactivity is. It's like, UM, your
14:33
Speaker 1: breathing is elevated, your heartbeat is elevated, your core body
14:37
Speaker 1: temperature is elevated. UM, you're just tuned up all the
14:41
Speaker 1: time and you're not getting the sleep to knock yourself
14:44
Speaker 1: out of that state and to regroup and regenerate and rest. Yeah,
14:48
Speaker 1: and you're gonna at this stage you're gonna have um
14:51
Speaker 1: some memory loss, short term memory loss, mood changes, a
14:55
Speaker 1: lot of anxiety and depression, and you're gonna start to
14:59
Speaker 1: have some motor issues as well, like the way you
15:02
Speaker 1: move and the way you walk. So things are starting
15:04
Speaker 1: to get pretty bad at this point, and you're probably
15:06
Speaker 1: pretty freaked out, especially if this doesn't run in your
15:09
Speaker 1: family and you either acquired it or it's a sporadic case.
15:13
Speaker 1: Well yeah, and you're not going to a doctor and
15:15
Speaker 1: they're going, hey, this sounds like ff I to me
15:18
Speaker 1: because I've never heard of it in my life exactly. Um,
15:21
Speaker 1: well that's not true. Doctors have heard of this, but
15:22
Speaker 1: you know what I mean, like two of them. Half. Yeah,
15:25
Speaker 1: it's not the first go to I think when you
15:27
Speaker 1: say I've been having trouble sleeping and I'm agitated, they're
15:30
Speaker 1: probably gonna ask, like, what kind of drugs you've been doing? Yeah,
15:34
Speaker 1: you know, like just lay off the pot. Well that
15:37
Speaker 1: should make you sleep, though, right, I don't know. I
15:40
Speaker 1: guess it probably depends on what type of pot. I
15:43
Speaker 1: would think it's more I like lay off the speed.
15:46
Speaker 1: Oh yeah, you know, I probably should be gone there
15:49
Speaker 1: all right. Uh so the third stage is pretty short,
15:53
Speaker 1: it's about three months long, and that's when you're uh
15:57
Speaker 1: really delving into the hardcore. And so what's what's that
16:01
Speaker 1: in stage one? In stage two, appropriately enough, you're sleeping,
16:06
Speaker 1: but you're only entering stage one. In stage two of sleep,
16:10
Speaker 1: which is stage one is considered um where you're just
16:15
Speaker 1: very relaxed. Stage two is where you're starting to sleep,
16:20
Speaker 1: but you can be woken up very easily, and you're
16:23
Speaker 1: not Yeah, and you're not going beyond that. You're not
16:26
Speaker 1: going into stage three or stage five, which is R
16:29
Speaker 1: A M. Sleep. They combine stage three and stage four apparently.
16:33
Speaker 1: But you're not getting to sleep. So by the third
16:36
Speaker 1: stage of fatal familiar insomnia, you're not even going to
16:39
Speaker 1: one or two. You're just not sleeping at all. And
16:43
Speaker 1: it's been like this for um nine ten months already,
16:47
Speaker 1: so you're just basically losing it at this point. Yeah,
16:51
Speaker 1: and that will deliver you to the end stage stage four,
16:54
Speaker 1: and it's called end stage. You know where you're headed there,
16:57
Speaker 1: um serious decline in dementia, UM in brain activity. Maybe
17:03
Speaker 1: you've got about six months at that point, but you're
17:06
Speaker 1: going to lose the ability to speak and move. It's
17:09
Speaker 1: called a kinetic mutism and basically fall into a comma
17:12
Speaker 1: and death. So with with a kinetic mutism, you actually
17:16
Speaker 1: have the ability to move and speak, but you lack
17:20
Speaker 1: the basic will to do so. Apparently, like patients who
17:25
Speaker 1: have come out of this have reported that will not
17:28
Speaker 1: necessarily f FI because it's always fatal in a hundred
17:31
Speaker 1: percent of cases. Um, But people who have had a
17:34
Speaker 1: kinetic mutism for other reasons have said, like, like I
17:37
Speaker 1: knew I could, but in any time I got the
17:40
Speaker 1: will up to move, right, there was something else just
17:44
Speaker 1: counteracting that that was stronger, and I just couldn't move
17:47
Speaker 1: and couldn't talk. Yeah, and then like you said, you
17:51
Speaker 1: go from that into a coma right, and then death. Yes,
17:55
Speaker 1: and all prion diseases are fatal at this point and uncurable,
17:59
Speaker 1: correct I guess? So yeah, yeah, as far as they know. So, Chuck,
18:03
Speaker 1: what's what's going on in the brain here? Uh? Well,
18:06
Speaker 1: basically your central nervous system is starting to break down. Um.
18:11
Speaker 1: The anterior ventral and medio dorsal thalamic neurons. So those
18:16
Speaker 1: are neurons in your thalamus that basically manage your motor functions,
18:20
Speaker 1: they start to die out, and instead of being replaced
18:24
Speaker 1: like your body likes to do when cells die out
18:26
Speaker 1: with healthy ones, they don't your glial cells, which when
18:30
Speaker 1: don't we talk about glial cells. We've talked about them
18:32
Speaker 1: a few times. Yeah, we've in some brain wine before.
18:34
Speaker 1: They're basically the cleaning service for your central nervous system. Uh,
18:39
Speaker 1: and they help out with communication there they start to
18:42
Speaker 1: die and form scar tissue and the thalamus and once
18:45
Speaker 1: that happens, it's called gliosis. You've just got scar tissue
18:49
Speaker 1: instead of healthy cells exactly, pretty rapid decline from there.
18:52
Speaker 1: It kills the communication between cells. Right. Yeah. So, um,
18:57
Speaker 1: with fatal familial insomnia like what just described as the
19:01
Speaker 1: result of any spongeform prion disease, right, which again, we're
19:05
Speaker 1: just tiny little holes in your brain. Right. So it
19:08
Speaker 1: seems like the distinction between the different spongeyform diseases is
19:13
Speaker 1: what part of the brain specifically they attack. Fatal familial
19:17
Speaker 1: insomnia they attack the thalamus, specifically the hypothalamus and specifically
19:23
Speaker 1: the parts of the hypothalamus that help regulate sleep. Um,
19:28
Speaker 1: And there's this part of your hypothalamus that is an
19:33
Speaker 1: it has it creates what's what you could call an
19:35
Speaker 1: anti waking system, to where not only are the neurons
19:39
Speaker 1: shut off in one respect, in another, a bunch of
19:45
Speaker 1: neurons that are off while you're asleep are on and
19:49
Speaker 1: just keeping you asleep. So when you're waking normally, those
19:53
Speaker 1: neurons are off, and when you're sleeping they're on. The
19:57
Speaker 1: problem is, if you have fatal familiar insomnia, Uh, the
20:01
Speaker 1: prions have eaten away at this system, and now all
20:06
Speaker 1: of a sudden that that anti waking system that keeps
20:09
Speaker 1: you asleep when you're asleep allows you to go to sleep,
20:11
Speaker 1: to transition from one stage of sleep to deeper stage
20:15
Speaker 1: of sleep is no longer active any longer. And so
20:18
Speaker 1: the only thing that is active is your wakefulness and
20:21
Speaker 1: it is on all the time. So you know you're
20:24
Speaker 1: dying and there's this it's got to be some sort
20:27
Speaker 1: of madness from not being able to sleep. It just
20:30
Speaker 1: exacerbates everything, like that's just one of the symptoms. It is,
20:35
Speaker 1: and it is a symptom, but it's also uh, it's
20:38
Speaker 1: it's also directly related to the mechanism of this disease. Yeah,
20:43
Speaker 1: and it's got to speed up the process because your
20:45
Speaker 1: body is not getting the rest it needs exactly top
20:47
Speaker 1: of everything else. And and that's the that's the devious
20:50
Speaker 1: part of the whole thing is not only is your
20:52
Speaker 1: body not getting the rest it needs, it's on all
20:55
Speaker 1: the time. So it's it's kind of like a doubly
21:00
Speaker 1: hardcore as far as diseases go. So, like we said,
21:04
Speaker 1: diagnosing it is tough a because it's so rare. Uh
21:08
Speaker 1: be the symptoms are, you know, they're always patient reported.
21:13
Speaker 1: So like I said, a doctor is not gonna like
21:16
Speaker 1: look at this first thing. Uh, You're gonna go in
21:18
Speaker 1: with your family history and maybe get some blood test
21:21
Speaker 1: on UM, an I exam, a spinal tap. You might
21:25
Speaker 1: get an m R I or a pet scam or
21:27
Speaker 1: pet scam pet scan you've never believed in, or an
21:31
Speaker 1: e G which measures measures electrical activity in your brain.
21:36
Speaker 1: But um, it's really tough to diagnose. Well, yeah, and
21:39
Speaker 1: none of that is gonna work until you've already entered
21:42
Speaker 1: that stage. Yeah, like you've already entered the first stage
21:45
Speaker 1: at least, because those those tests show, oh, yeah, you
21:49
Speaker 1: have insomnia. And then once they established yeah, you have insomnia,
21:53
Speaker 1: then they have to further establish that it's fatal familial insomnia. Yeah.
21:58
Speaker 1: And by this time, also, Chuck, it's say about fifty
22:03
Speaker 1: year about fifty years old, you've probably already had kids.
22:06
Speaker 1: And so now once you find out you have fatal
22:09
Speaker 1: familiar in samnia, you're also terrified that you've passed it
22:12
Speaker 1: onto your children, you get about. Yeah, and since it's
22:15
Speaker 1: an autosomal dominant trait or disease condition, um, all you
22:20
Speaker 1: need is one parent with it to pass it on
22:22
Speaker 1: to you. Yeah. It's so sad. It's like, basically, what
22:25
Speaker 1: am I dying of? Because you can't fix me? And
22:30
Speaker 1: are my kids going to die this as well? And
22:32
Speaker 1: then their kids? Very horrible disease. All Right, we're gonna
22:36
Speaker 1: get into I guess, finish up with a few a
22:40
Speaker 1: little information on a few more of these pre owned diseases,
22:42
Speaker 1: right for this break all right, before we get onto
22:53
Speaker 1: the other preonn diseases, we do need to talk about treatment.
22:56
Speaker 1: Like we've said over and over, sadly, there is no
23:01
Speaker 1: curative treatment, but there is palliative care, which basically means
23:05
Speaker 1: we're gonna try and help you out as much as
23:08
Speaker 1: we can to be comfortable as you die. Uh. And
23:11
Speaker 1: weirdly one of the things that they are looking into
23:15
Speaker 1: and trying is giving patients g h by the club
23:19
Speaker 1: drug that you hear about to help people sleep, and
23:22
Speaker 1: they're actually prescribing that in some certain cases. Yeah, and
23:25
Speaker 1: they it apparently gives them quick, small, short bouts of sleep.
23:31
Speaker 1: But that's I'm sure incredibly wonderful sleep. Nonetheless, yeah, it
23:37
Speaker 1: might decrease your heart rate and body temperature a little
23:39
Speaker 1: bit too, but um, at that point, you know, when
23:43
Speaker 1: you're taking g HB to get twenty minutes of sleep,
23:45
Speaker 1: you've got a pretty sad end coming very soon. It's
23:49
Speaker 1: very sad. The other great hope is gene therapy, where
23:53
Speaker 1: basically the delete the gene that's responsible for um making
23:59
Speaker 1: this protein misfold and insert the correct version of it.
24:04
Speaker 1: That's got to be the future of these cures, don't
24:07
Speaker 1: you think. Yeah, that's gonna be the future of a
24:08
Speaker 1: lot of cures when we can just rewrite the code
24:12
Speaker 1: of our genes to you know, make it express properly. Yeah,
24:16
Speaker 1: But until then, there's gonna be some problems for people
24:20
Speaker 1: with spongeformed diseases because there's nothing you can do, including
24:25
Speaker 1: with fatal familial insomni you can give somebody g HP.
24:28
Speaker 1: That's about it. Should we go over a few more
24:30
Speaker 1: of these? Yeah, they're all equally devastating. Um, I think
24:34
Speaker 1: you did mention c J D quitz felt jacob or
24:37
Speaker 1: yakup disease yaup. Maybe sure. Anytime I see j A
24:44
Speaker 1: k O B, I think of the like you know,
24:46
Speaker 1: German pronunciation. Yeah, it's probably right. This one's the most
24:50
Speaker 1: prevalent um and it is a spontaneous occurrence, which is
24:53
Speaker 1: really creepy. Well, you can get it too from eating it.
24:57
Speaker 1: Oh is that? I think ten perc and as spontaneous
25:01
Speaker 1: and the rest you get from eating it's acquired. Oh no,
25:04
Speaker 1: it's just ten percent are inherited. Okay. So that's the one,
25:08
Speaker 1: if I'm not mistaken, that was directly related to the
25:12
Speaker 1: outbreak of mad cow. That's like human mad cow disease.
25:15
Speaker 1: Remember back in the nineties with the mad cow outbreak. Well,
25:18
Speaker 1: that all came from feeding a bunch of cattle a
25:21
Speaker 1: lot of ground up beef that included cattle brains that
25:25
Speaker 1: had pre on diseases in it, and so the cattle
25:28
Speaker 1: got mad cow disease, and from eating that cattle we
25:31
Speaker 1: got kuuz felled yakub Okay, so that's the human version. Yes,
25:36
Speaker 1: and that's that kind of points out a huge problem
25:38
Speaker 1: with preon since they're not biological, not not living in
25:43
Speaker 1: the sense that we consider an effective agent typically like
25:47
Speaker 1: a bacteria or a virus. There's no genetic information to destroy,
25:52
Speaker 1: like using heat or bleach or whatever. It can't be
25:56
Speaker 1: killed as I saw it put somewhere else. It's the
25:58
Speaker 1: perfect pathogen. Yes, that's a mouthful. It is, but it's
26:02
Speaker 1: also horribly scary. It is another spongeform diseases. Scrapy that
26:09
Speaker 1: just sounds good. Yeah, that is just in goats and
26:12
Speaker 1: cheap though, So humans don't need to worry about it. Um.
26:16
Speaker 1: And it is also genetic and they have no evidence
26:18
Speaker 1: right now that that humans can get it. No, we've
26:21
Speaker 1: got enough to worry about. We talked about mad cow
26:24
Speaker 1: that is officially called bob and spongebform uh encephalopathy. Nice,
26:31
Speaker 1: Is that right? I think so? Yeah, UM, deer and
26:34
Speaker 1: elk might get chronic wasting disease in the Western US.
26:38
Speaker 1: If you see a deer that is um really skinny
26:41
Speaker 1: and drooling and can't swallow, um, they may have chronic
26:45
Speaker 1: wasting disease. I've seen that that humans can have that
26:49
Speaker 1: as well, But um, I don't know if it's the
26:51
Speaker 1: same version. No, it doesn't seem like it, because I
26:53
Speaker 1: think it's in patients that have like uh, that have
26:56
Speaker 1: died from AIDS. They'll often like waste away, like no
27:00
Speaker 1: amount of nutrients will like keep them from just losing
27:04
Speaker 1: weight until they basically just die from wasting away. But
27:07
Speaker 1: I don't think it's related to a brain disease. And
27:09
Speaker 1: then chuck also going back to um c j D,
27:13
Speaker 1: which is what we call it now officially forever c
27:18
Speaker 1: j D. Ok Um. There was an outbreak of it
27:21
Speaker 1: in the eighties and no the nineties. These French doctors
27:25
Speaker 1: were using it in a growth hormone that they were
27:27
Speaker 1: giving as injections to kids who had stunted growth, and
27:32
Speaker 1: they were getting them from pituitary glands harvested from humans
27:36
Speaker 1: and sold along the black market. So basically a bunch
27:40
Speaker 1: of dead people in Bulgaria had their pituitary glands removed
27:46
Speaker 1: and sold the doctors in France who were using them. Yeah,
27:49
Speaker 1: and these growth things or these growth hormone shots that
27:53
Speaker 1: they were giving kids, and like a sixty or eighty
27:55
Speaker 1: kids died the nineties. Yeah, yeah, it sounds like seventeen nineties.
28:03
Speaker 1: So it's it's very weird because we go from not
28:06
Speaker 1: even recognizing diseases like fatal familial insomnia as a disease
28:10
Speaker 1: until the eighties, having an outbreak of mad cow disease,
28:14
Speaker 1: having an outbreak of c j D, all within a
28:17
Speaker 1: couple of decades, and all the while people are saying like, no,
28:22
Speaker 1: preons can't exist. What you're saying is is it can't
28:26
Speaker 1: be possible. And a lot of people tried to disprove
28:31
Speaker 1: preons by saying, Okay, there are different types of scrapey,
28:36
Speaker 1: and say one hasn't a different incubation period. In this
28:41
Speaker 1: other one, you'll get this symptom, but you won't like
28:43
Speaker 1: the The sheep will talk, will speak Russian, but in
28:48
Speaker 1: this other form of scrapy they speak Swedish. And they're
28:51
Speaker 1: saying like, this proves that these preons have some sort
28:55
Speaker 1: of virus associated with them that we're just missing. Right,
28:59
Speaker 1: Another group or another argument against preons was well, Alzheimer's
29:04
Speaker 1: disease is technically a sponge ofform disease. It's a misfolded
29:09
Speaker 1: proteins creating plaque build up scar tissue in the brain
29:14
Speaker 1: that leads to all these same symptoms as say C J. D. Right,
29:19
Speaker 1: it's not infectious. What's going on here? So they said
29:23
Speaker 1: that there is one way to prove this, and that
29:25
Speaker 1: is to create a pre on in a test tube
29:30
Speaker 1: completely from from the whole cloth and put it in
29:35
Speaker 1: someone's brain and put in a healthy brain. And that's
29:37
Speaker 1: exactly what they did in two thousand and five at
29:39
Speaker 1: the University of Texas, and it infected the other proteins.
29:42
Speaker 1: That's right. So it's like, prions are this weird thing
29:45
Speaker 1: that we didn't know that is now infectious, was even possible,
29:49
Speaker 1: and we're finally wrapping our heads around it. Are spongy,
29:53
Speaker 1: whole filled heads. I think this has gotta be not
29:56
Speaker 1: this particular, but uh, this has gotta be what's gonna
29:59
Speaker 1: wipe out the human race one day. I don't know.
30:02
Speaker 1: Bowl is making a pretty good case for itself lately. No,
30:04
Speaker 1: that's what I'm saying, Just some disease, Like, Uh, I
30:07
Speaker 1: don't think we're gonna blow ourselves up with or like
30:10
Speaker 1: run out of food or blow ourselves up with nuclear bombs.
30:13
Speaker 1: I think it's just gonna be another like weird plague
30:15
Speaker 1: or something that we don't understand due to our close
30:18
Speaker 1: association with live Stock, and it's not medtime soon. I
30:22
Speaker 1: don't know. Yeah, you're worried. I'm not worried. I'm just more,
30:27
Speaker 1: you know, realistic. You give your safe room, you're building
30:30
Speaker 1: in the side of a mountain. No, that would indicate
30:32
Speaker 1: that I was worried. Okay, I'm building mine. You got anything, well?
30:36
Speaker 1: Can I come over? Good? Are you got anything else? No? Okay, Well,
30:40
Speaker 1: that's fatal familial insomnia and prions. If you want to
30:45
Speaker 1: learn more about those things, you can type those words
30:47
Speaker 1: into the search bar at how stuff works dot com.
30:50
Speaker 1: And since I said that, it's time for a listener mail,
30:53
Speaker 1: and they call this Canadian email from a Canadian kid
30:57
Speaker 1: from Canadia. From Canadia, Hey guys, my name is Ben
30:59
Speaker 1: matt as him a seventeen year old Canadian A. He
31:02
Speaker 1: said that I didn't add that. Then that makes me
31:05
Speaker 1: think he's probably not Canadian. I've been well, he was
31:09
Speaker 1: in jest. I think I've been working my way through
31:11
Speaker 1: the backlog of episodes, and I recently listened to the
31:12
Speaker 1: episode on serial Killers. And then he spoke of a
31:15
Speaker 1: man who killed by feeding his victims to pigs picked
31:19
Speaker 1: in huh, rob is that his name? Do you remember
31:23
Speaker 1: that because it sounds like pig pen. No, he's a
31:26
Speaker 1: pretty famous serial killer from Vancouver. This reminded me of
31:30
Speaker 1: a true story from my dad's childhood on a farm
31:32
Speaker 1: in Holland, Michigan. While he was growing up, he was
31:36
Speaker 1: often given the job of feeding pigs, a job he despised.
31:39
Speaker 1: Much of the stem back to a horrible accident that
31:41
Speaker 1: happened to a nearby farmer. When this particular farmer was
31:44
Speaker 1: out in his barn feeding pigs, he had a heart
31:46
Speaker 1: attack and collapsed among them. When his family found him
31:49
Speaker 1: a few hours later, all that remained of his body
31:51
Speaker 1: were the palms of his hands and the soles of
31:53
Speaker 1: his feet. Hate those parts. This experience understandably and stilled
31:58
Speaker 1: fearing to my dad for many years to come. And
32:00
Speaker 1: to be honest, I don't blame him anyway. Thanks for you, guys,
32:04
Speaker 1: Thanks for all you do. A shout out would make
32:06
Speaker 1: my day, maybe even my year. So been masked, shout out,
32:10
Speaker 1: shout out. Thanks for the email. Nice Thanks a lot. Ben.
32:14
Speaker 1: You haven't heard much about Robert Pickton. I don't think so.
32:18
Speaker 1: He was operating in like the nineties. I think and
32:22
Speaker 1: he was just like having prostitutes over and then he'd
32:25
Speaker 1: murder him and do horrible stuff like stuff like that.
32:29
Speaker 1: And he was supplying the public because you know, he
32:32
Speaker 1: had a pig farm. He's applying the public with pork.
32:35
Speaker 1: And they think that he ground up people in it
32:37
Speaker 1: to the pork ate that and then people ate the pork.
32:40
Speaker 1: No no, no, like he ground up people and mixed
32:44
Speaker 1: it together with ground pork and then sold that at
32:46
Speaker 1: his ground pork. Oh wow, it was even worse. This
32:49
Speaker 1: is the nineteen nineties. He was a bad man. Where
32:52
Speaker 1: was he Vancouver? Jeez? I know, I thought those people
32:56
Speaker 1: are nice. Not Robert picked him. Man horrific. So if
33:02
Speaker 1: you like Ben masked shout out, I want to get
33:06
Speaker 1: in touch with Chucker me. You can tweet to us
33:08
Speaker 1: at s y s K podcast. You can join us
33:10
Speaker 1: on Facebook dot com, slash stuff you Should Know. You
33:13
Speaker 1: can send us an email to Stuff Podcast at how
33:15
Speaker 1: stuff Works dot com, and as always, joined us at
33:18
Speaker 1: home on the web Stuff you Should Know dot com.
33:26
Speaker 1: For more on this and thousands of other topics, visit
33:28
Speaker 1: how Stuff Works dot com.