Brought to you by the reinvented two thousand twelve Camray. It's ready. Are you welcome to stuff you should know from house Stuff Works dot Com. Hey, and welcome to the podcast. I'm Josh Clark. I can feel pain with me as always A Charles W. Chuck Bryant and he hold on, dude, Chuck can feel pain as well. I just tested him. I wanted to make a hund percent sure. That's the second take though, and you didn't really have to do it twice. We could effaked it this time.
I wanted a realistic response. Well that's real, Josh, pinch me hurt? Yeah, yeah, you could tell um you are right? Yeah, I'm fine. Okay, how you doing besides the throbbing forearm? I'm great, sir, and you I'm doing pretty good. Good. Everything's just peachy, including with my hair. Josh has a he goofed He shaves his head and he kind of goofed it so he looks like a a patient from some word where he had to surgery or something in his on his brain. I didn't goof my hair, that's right. Yeah,
and I have an eastern black haircut now right. Yeah. Um. So other than that I'm feeling pretty good. You're right, So you felt emotional pain with your odd haircut. I did, which, by the way, Chuck, is different from physical pain. You knew that because of a yet to be released audiobook. That's right, it's forthcoming. It's a little teaser for everybody. Um, But there are actually people out there, Chuck, who cannot feel pain straight up, don't feel pain. It's amazing. It
is very rare, but it happens. It is rare, Chuck. Actually, the only hard number that I've seen is UH sixty sixty reported cases worldwide, sixties since three. Actually, I've got thirty five in the United States as of now. Yeah, I've seen I saw that number as well. I've seen people who just plumb said, we have no idea how many could be under reported, especially with younger kids. UM with what we're talking about is called congenital insensitivity to
pain with an hydrosis. Otherwise, we're just gonna call it SIPA for short, right, because that's much easier. And that is actually part of a it's a sub type of a larger disorder called hereditary sensory and autonomic neuropathy UH. And this is actually the type four SIPPA is type four s H s A N. Yes, and it's the most severe. Uh. Well, we'll get into what it all means, but it's the most severe. We will. Um. There's actually kind of a recent famous case of it. There's a
British physician named Dr Jeoffrey Woods. You heard of him, chuck, No, But is he one of the g o F guys. Yeah, he's British. Yeah, he's spilled programmed with two ms in an e UM. But Dr Woods uh travels to Pakistan fairly regularly, and while he was there he heard about a kid who used to pass like thick needles right through his arm walking on hot coals. Uh, and showed no signs of feeling pain whatsoever. And he wasn't in
the gym Rows circus. No, he wasn't, actually, but he probably could have done a lot better for himself than just on the streets of Lahore right. Um with Dr Woods heard about this guy and this little kid and was kind of interested and finally got around to trying to go see him. Right before he could see him, the kid, around his fourteenth birthday died. Actually, Um, he was he was. I don't know if he was dared or he was doing it for money or whatever, but
he jumped off a roof and died. But Dr Woods got ahold of this kid's family and actually found out that a bunch of them have no sensitivity whatsoever to pain. Yeah, which is also called analgesia. Oh that's the other word for it. Didn't know that. Yeah, pain killers are called analgesics. So this would be like the state of not having pain or having reduced pain would be analges yet. But this isn't just reduced pain as a chuck, No, we're talking about no. Uh. You can feel pressure, but you
can feel no pain. Uh. And you can feel no heat or cold. You can feel no temperature on your skin either. Right, that's the one with anhydrosis, Yes, exactly. Okay, let's talk about the pain part first. Let's talk about pain in general. Okay, how do how do we feel pain? Well, Josh, your your nervous system is where all this happens. And we're talking about your brain here. This is your brain
on pain. Yeah, your cranial nerves, spinal cord, spinal nerves, and other things like your gangli and receptor sensory receptor stuff like that, and your nerves carry messages to your spine through your body to your spine. Spine carries it
to your brain and the receptors. If you burn your finger or something will send the message up to the spine to the brain that says you got burnt, It hurts, right, and it sends a response message that moves your hand away real quick, touching a hot stove or something like that that. You can take it from here though, because your Mr Doctor guy Dr Clark, thank you chuck. Um. Actually, the sense of pain, the experience of pain is called no susception,
right um. And this is the experience of physical pain as a response to noxious stimuli. And there's actually four categories of noxious stimuli. There's mechanical, electrical, thermal, and chemical, right um. And we have actually specialized uh nerve endings receptors called no susceptors that are responsible just for sending pain signals. They use peripheral nerves. Yes. Actually, as as painful as pain is, we have it for a reason.
Like you said, you know you're touching a hot stove and your brain says, get your hand off of it, right um. We also all uh what one one theory of how we develop fear, which is good in and of itself as well, UM, is through direct conditioning. That's one of three ways we we learn fear responses is
through direct conditioning. And part of direct conditioning is experiencing physical pain because once you've hit the hot stove, once you know that the stove is always going to burn you exactly, or if you're a dumb kid, maybe two or three times, but eventually you're gonna pick it up. Right now, what happens with sippa, as far as I understand, um, and actually did you notice as well? There's there's not a pet explanation of if you have SIPPA, this is
exactly what happens to you. Yeah, well probably because it's so rare. Right. But when you think that if they could get this the group of patients together, they would find like, okay, well yeah, all of you people are lacking no susceptors. Uh So in some patients the no susceptors aren't there. In other patients, UM, the peripheral nerves that the nose acceptors are attached to, or uses the
information highway UM aren't there, or else they're not innervated. UM. There's a lot of different ways that this this can come about. But it all results in this cardinal diagnosis or diagnostic tool of SIPPA, which is you feel no pain and you aren't responsive to temperature either. But nerve conduction is there, right, It depends on some of them. There's innervation and others the nerves aren't conducting, they're not
capable of conducting. And specifically with anhydrosis, I think, um, there's no there's no innervation of the nerves in the sweat glands. So when you start to get hot, there's nothing sending your brain the message, Hey, you're getting hot. We need to start sweating so we can cool down. Well, yeah, we should go ahead and say that that's the A is anhydrosis and after the sip part, and that's the most sever your case, and that's when you don't sweat,
which you know how I sweat. I know you definitely don't have anhydrosis. Remember at the aquarium, I sweated underwater in like forty degree water too. I mean there was like little chunks of ice floating on the surface of the water and you had cartoons sweat jumping off your forehead did so is I could handle the anhydrosis part or at least a slight version of that. I could deal with that, right. Uh, and this is our These are ekron sweat glands or ekron glands that are responsible
for regulation of body temperature. So what they they found with the anhydrosis, it seems fairly uniform in that respect that we the a sip of patients don't have um innervation, meaning the nerves aren't becoming activated in their sweat glands. Yeah, and that's especially I mean it's bad enough to over you know, because you can overheat just as an adult, but it's really scary with babies with SIPPA because a
baby can overheat and die like very easily. Well yeah, actually, I think of people who have SIPPA die before or by the age of three, and I think another quarter of those deaths are um from sepsis from an untreated infection. One of the things about not being able to feel pain is you can cut yourself and if you're a kid, you don't think, well, I'd better treat this cut. And at the same time you're not crying, so your parents aren't alerted, and so this cut can go untreated become infected.
When they cut is infected, the bodies immunological response UH raises your body temperature. It can lead to a fever, and unchecked, this fever can actually lead to um brain damage. So a lot of patients with SIPPA who have made it past you know, three or so have some form of mental retardation. Yeah, they also saw where um. We'll get to the tips later, but since you brought it up. One of the tips for small kids as they will teach the child to recognize blood and teach them what
that means. So if you see blood, you need to come find mommy and daddy real quick because you don't know you're hurt. But that's a bad thing to happen, So come come find mom and dad. Yeah, and I thought these tips that, like you said, we'll get to in a minute, or they were pretty clever, right, or come fine mom and mom or dad and dad. Chucky guy, I've been minded around here. Or just mom or just dad or uncle Charlie, who's uh grandma or grandma or
grandpa and grandpa. There's all kinds of scenarios, A lot of know somebody the first person um to be legally pronounced a sexual was legally pronounced a sexual in the last couple of weeks, I believe. Really, yeah, it's true. Very interesting that. Yeah, Okay, where are we, Chuck, Let's talk about sipa. The medical history of sippa Okay, as far as I could find, it was first described in eighteen forty seven, uh and described as what is going
on here? Yeah, exactly. And actually in the sixties. I read an article in nineteen sixty that was based on UM a hypothesis that people who feel no pain were actually it was actually a psychological disturbance. There's been such a massive affront to their ego that as a response, the response to their ego mounted resulted in a loss of the sensation of pain. That cat just can't feel pain.
He's so out there, so far out. There is a doctor who was who was surveying this literature of UM certain patients, and he was describing like how the diagnosis went, the diagnosis went. And there's this one poor guy in the fifties who he was described as a pretty nice, upbeat, bright guy, and he goes to the doctor and it's like, I can't feel pain. And the doctors like, oh yeah, so you can see this half drunk doctor, right, he's got like a glass of scotch and a right and
uh right, and the little reflective thing on his forehead. Um, and he ran this guy through a battery of tests, including squeezing his testicles, running an electrical current across his lower teeth, and Chuck, it gets worse, taking a skin and muscle biopsy without anesthetic. What did he do each of these? And was he like what about that? You know? I feel that? Can't you imagine like this? Doctors like, oh, yeah, that didn't yeah. Um, So the psychological idea was discarded
pretty quickly. And now, because we're in the age where the genome has been cracked, it's all genetics, right, yeah, And I guess we should go ahead and mentioned too that they think they have narrowed it down to a mutation of the t r K A parentheses in t r K one gene, which appears to control nerve growth,
but again they don't know for sure. They just think that might be the culprit, right, Chuck, that seems to be the likeliest UM candidate, because that gene actually is it's responsible for producing nerve growth factor, which goes and recruits UM neurons and nerves, isn't I guess it would be neurons uh to become no susceptors, and so that wouldn't happen, right, right, But they're kind of speculating still a little bit, right. Yeah, we still don't have an idea.
I mean, sixty cases reported worldwide, thirty five in the US. Maybe, although, um, it does seem like it would be genetic, like that kid in the Little Horror Pakistan when he died and the doctor went to go see his family and found out he had siblings that had the same condition. Sure, Um, that suggests either something environmental close to the home or uh, genetic, And that's kind of what they're leaning to. That's the genetic part, right, Josh. They do think or do they
know for sure that it's an autosomal recessive disorder. I don't know how they do, but that's that's how I take it, right, Which means that both of your parents must pass down a copy of this gene. So each one of your parents has this mutation and it's not related to gender or anything. But they do know that both of your parents have to give it to you, so that that's probably what makes it rare as well. Well, yeah, both of your parents have to have this really rare thing. Apparently,
also it hasn't It doesn't affect every ethnic group. Oh really Yeah, but I was reading another study on this from I think two thousand and it was in I think Jerusalem or Tel Aviv, one of the two, and they got together a bunch of kids who had sippa and um, some of them were related, and they took a family history and found that I think nine of the thirt participants parents were first or second cousins. So I wonder how much that has to do with something
too interesting? Yeah, very interesting? So should we talk about some of the problems. I mean, they're pretty obvious, but there are something that I didn't actually well, yeah, let's talk about raising a baby with sippa. How would you first start to recognize that your kid has sippa? Well, that's a problem because the first way you would probably recognize it is seeing witnessing an accident that should cause
your baby to cry, and your baby doesn't cry. Right, that's like, did you have you ever heard the discovery of ether and nitrous ox side is anesthetics right here? Well, actually it happened in New York City, but a physician from our fair City name ms Dr Crawford Long. Yeah. I did a little book report on him when I
was like the fourth grade. He was like our nineteen sixties Scotch and cigarette doctor, and that he liked the party and he noticed that people on in the depths of an ether bender would run into walls and gash their foreheads and not have any response to it whatsoever, aside from laughing hysterically. Right exactly, look at all this blood. Yeah, so there's there's a similarity there. And well for me to add that tangent, we have Crawford Long Hospital here too,
I know, look at you with the sidebars. So, like I said, the first thing it will happen is apparent season accident and the child doesn't cry. Um, so you're gonna have some problems getting diagnosed. So because it is so rare. Uh. And in the meantime, the kid could end up with an infection and die of overheating or
suffer some sort of brain damn. One of the other big problems with the baby is um when a baby tease, a baby just naws like crazy, but if they don't feel pain, they may gnaw like straight through their little baby finger without even realizing right, or their tongue or their lips. Those are common injuries as well, yeah, which
is just awful. There's also um ice scratching. Yeah. Well, they said though on the teeth, they said that some parents it's elect to have the teeth baby teeth removed altogether and wait for the adult teeth to come in, at which they're in age where they can understand like don't bite through your finger. Yeah, but that's not good either, because then your child has no teeth and it makes it harder to eat and right, but it's like eat baby food for many many years, or she right through
your tongue right right? You know? So what was the one you just mentioned? I scratching? Yeah, you know you if you are just rubbing your eye or whatever, you pretty much know when to stop. If you are a patient, a sip of patient, you don't know when to stop. I was reading about this five year old girl who blinded herself. She can still see shapes, I believe, um. But she had one eye removed and scratched the other eye and she was five and could barely see. She'd
done it to herself. Well, another tips, since we're there, a lot of parents elect to have little baby goggles for their for their child, so they can't get to their eyes, which is sad. Yeah, um, hunger pains. This was interesting. They don't feel hunger pains, so eating isn't something they realized they should do. So uh they sometimes sip A patients will set a clock to remind them
to eat or to use the potty. Right, Because one of the ways that you know that you have to go number one or go number two is from the discomfort involved. Your body is alerted like you need to evacuate your bowels, and so you go evacuate your bowels. But if you don't have any sense of discomfort whatsoever, Um, you aren't going to go to the bathroom. You can suffer constipation all manner of horrible results from that from holding it too long. Yeah for sure, Um, fractures are
very common. Obviously. You can slam your hand in the door, won't feel the pain, won't realize you've got a broken hand and joints to This is something I hadn't thought of either. But consider this, like, how many times have either one of us moved in our chairs during this podcast? And the reason we're moving shifting is because it's become uncomfortable to lean on that joint, so we move and put the our weight on another joint. That's your body telling you to to shift, so you're not putting too
much stress on anything. Right. If you don't shift, then all of that weight is on that joint, and this can actually result in charcot joints. These are joints where
basically you can't feel pain. Uh, to develop charcot joints because when you it's the result of like a prolonged and repeated destruction of a joint or several joints, And what happens is like little bone fragments break off and then you have bone pieces grading in the joint, it fills with fluid and UH, there's definitely a point of no return that can lead to amputation, um limb replacement, that kind of stuff. Gez, Well, one of the since we're kind of doing the problem tip thing, one of
the tips don't think we have so far. One of the things that they recommend is occupational therapy, so they can teach your child different ways to sit and do physical tasks to like put the least amount of stress possible on those joints, right or they may say your kid needs to be in a wheelchair a little more than than here. She is true and again very sad. This whole thing is just one of the most depressing
rare disorders I can think of. Uh, if you have a baby that has um SIPA, you should probably do a regular check over your child, like you need a baby proof the heck out of your house. Like it's baby proof anyway, but you really need to go overboard with softening corners and things like that, making sure the stove and anything dangerous isn't accessible the knife drawer, that
kind of thing. But um, you should also check your check your child for until they're old enough to do this on their own, check them for injuries, you know, a couple of three times a day. Also, um, because of the anhydrosis. Uh that a lot of families opt to move to cooler climates just you know, to protect
against overheating. Yeah, and um, Katie Lambert wrote this article of stuff you miss in history class are cohorts And uh Katie supposed that as far as getting exercise, which you know everyone needs, that swimming might be a good good thing for them to do. And that kind of made sense to me. Yeah, but have you ever like
swam and sweat it underwater? Well you know it? Well, yeah, but now I I wondered about that because I mean, if you think about it, if you are sweating underwater, then your body's saying you need to be cooler than the ambient water temperature. So it could still lead to trouble. I guess getting cool water and maybe don't swim so much that you're gonna be sweating could be my advice. Dr Chuck's advice. That's good advice, Dr Chuck. Although the
water is a good medium because it's easier on the joints. Yeah, well, I think that's why she recommended it. Sure, Uh, there is no cure, no that that has happened yet, and it's so rare that it's it's one of these things it's hard to get a cure when you don't have anyone to test it on. Yeah, although Chuck, don't you think the advent of stem cells will probably cure everything?
I hope so. Well, I mean think about it, it's a if it's a genetic flaw measually that means that an enzyme or protein isn't being produced, so you just use stem cells to generate that enzyme or protein and R Clark no more sippa. There are a couple of websites should we go ahead and plug those. Gift of Pain is a website set up by a family whose daughter has SIPPA, and if you want to have your heart broken and learn a little bit, you can go visit there and help. Roberto dot com is another one
that details a young boy with SIPPA and Chuck. Also there is, from what I understand um, a pretty good documentary called A Life Without Pain by director Melody Guild and it's about SIPPA. Huh, it's supposed to be pretty cool. I haven't seen it, but anybody interested, we want to rent that? And I say look for a pain podcast in the future. I think we should do one on pain Josh and Chuck's House of Pain. Do you know how many times I'm gonna pinch you during that thing?
It's gonna be rough, hopefully no more than once. It will be more than once, all right. If you want to learn more about SIPPA, type in c I p A in the handy search bar at how Stuff works dot com. That means it's time for listener mayle yes sir, exactly, Chuck, I want us to give a special shout out the three sisters triplets. In fact, we have triplets triplet listeners. Yeah, Helen, Spence and Echo, and they all three are voracious stuff. You should know listeners A cool name, Yeah, yeah, I
like it. A Spence goes by Zebra apparently just came in when I was on vacation. Huh yeah, yeah, because you replied I did, and you promised the shout out and never gave it. Yeah. They we didn't do a shout out and we are now though, I mean, come on, they break me over the coals and I responded, yeah, And you know, we typically don't do shoutouts because we get request and there's just too many of them. Yeah, but how many triplets do we have listening to? Exactly?
So if it's something remarkable, or if you want your name read so bad, just tell us your quadruplets and make up three names. So you guys keep the fourth doubt and thanks for listening. Thank you girls, and we'll get the listener mail eventually, I think. But we should probably plug Facebook and Twitter tweet tweet. We are children of the next generation. I don't know modern why I think no your x M. Why no, seriously, we're definitely not millennial. So, yes, we're on Facebook now, for sure.
We're on Facebook. We have can validated are We had multiple pages. It was kind of a mess, but now we're all consolidated. We're personally putting stuff on Facebook and talking to people. It's kind of kind of fun. Actually, yeah, it is. You're doing a heck of a job, Chuck. You you can type in stuff you should know on Facebook's handy search bar and that should bring up our page. You go ahead and join. I'm very curious to see
how many people join. Yeah, you know, as long as we have more than all the other podcasts combined, and I'm fine. Yeah. And the same goes for Twitter too. Do you have that same that same desire, the same goal. Yeah, so I've embraced Twitter. I can't believe it. Yeah, I'm tweeting. Yeah, Chuck's first tweet was hilarious, as I imagine all successive tweets will be. Our Twitter name is s y s K podcast. That's our Twitter name at yeah past right, the little at simbulture for those of you who don't
know how that works. Right, Uh, so you can check those out join up, follow us, and we will be interacting. Right, We're gonna come down from our ivory tower, right, yeah, all right? Uh so, Josh, I'm gonna call this um earthquake survivor. Did you read this one? Pretty remarkable? You're You're back. You're not on vacation anymore. Hey, Josh and Chuck. I just wanted to email and say thanks for unknowingly doing a lot to keep things normal for me in
the least normal of times. Let me explain. I live in Santiago, Chile, and in late February I was invited to the camping trip in a place down south called Mocha. Do you like my phonetic pronunciations. It was all really last minute, but before leaving I managed to grab ahold of your latest podcast, which was How Braille Works, put it on the old iPod. I thought I might need it for the long bus ride, and I only grabbed that one because, being a true fan, I had already
listened to the others, So good for you, Ignacio. On our second night camping on the island, we had an eight point eight Richter earthquake, if you remember, and we were pretty near the epicenter. We had to run from a tsunami, which only gave us about seven minutes to get the higher ground. After the earthquake, our tents and all of our stuff was washed away. We never saw
it again. Turned out we had it easy. Most of the homes on the island were taken by the wave, and we camped without tents of course, with the locals up on a hill for a couple of days before being able to even take a plane to start our journey back home, which was difficult, Josh, because the bridges were down, streets were cracked, and there was no gas at the gas stations, that kind of thing for like
five days. Um. In the middle of it all, I was feeling really bad about everything, though grateful for being alive, and I was not really knowing about my friends or family since cell phones were down and I was in the middle of the least normal situation I'd ever been. Then I remembered I had your podcast, pulled out my iPod which I had saved in my Fannie pack and just listen with my eyes closed, really happy because that was, for the first time the only nection to normal I had.
That is awesome, really cool. Uh. It made the hugest difference. So I wanted to say thank you so much like you guys to stop and think what a difference you can make in the listeners lives. So let's stop and think for a moment. All right, all right, nice? I feel good. How often do we just sit and be quiet? Never? Never what we're supposed to do? Uh? You you have a loyal listener for years to come. That is from Ignatio. Thanks. Ignatio was alive and well thank god. Did you hear
about the Day's Army Family or the Day's Arms. They're a family from Haiti who survived the Porter Prince quake and they had a son who lived in Saniago who's like, come live with me. You're kidding. They moved to Saniago just in time to survive the Chili quake, survived both though. Yeah, yeah, and since you said that that way, it's the name. What did you say? The name of the place was Saniago. The Day's Arms, Yeah, they're the family. That's that's the
plane Illinois. We should say, well we goofed that one. We didn't know. It's apparently planes death planes. Dutch Boot sounds very strange because it would seem like it would be silent, but everyone made fun of me saying you sounded like tattoo with the plane deplane. I think we're the same. We're interchangeable. My pain is your pain. You can you can switch our guts, right bellies. So officially, UH, I would like to switch bellies. You're a slightly smaller
than mine. Thank you. UM. Officially, we'd like to make that correction. It is de Planes, Illinois, and it's yo. Claire yeo. Claire was continent, it's not. And in Canada they called Canadian bacon bacon. Yes, Canadians. We have corrected that for two years now, so you can stop with the emails. It's a joke. If you have a correction you want to send Chuck or me, or you want to get into some UH email combat over a topic
and issue something like that, send us an email. Do not cite Wikipedia UH to support your claims, or else you've lost right out of the gate. Do better wrap it up. Send it to stuff podcast at how stuff works dot com for more on this and thousands of other topics. Is that how stuff works dot com. Want more how stuff works, check out our blogs on the house stuff works dot com home page. Brought to you by the reinvented two thousand twelve camera. It's ready, are you