Stuff You Should Know - Are there people who can't feel pain?

Episode Date: April 20, 2010

There are less than a hundred documented cases of people who cannot feel pain and suffer from a condition called congenital insensitivity to pain. Josh and Chuck discuss the dangers and symptoms of CIP and related disorders in this episode. Learn more about your ad-choices at https://www.iheartpodcastnetwork.comSee omnystudio.com/listener for privacy information.

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Welcome to Stuff You Should Know from HowStuffWorks.com. Hey, and welcome to the podcast. I'm Josh Clark. I can feel pain. With me is always is Charles W. Schuck Bryant, and he, hold on. Oh, dude.

Schuck can feel pain as well. I just tested him. I wanted to make 100% sure. That's the second take, though, and you didn't really have to do it twice. We could have faked it this time. No, I wanted a realistic response. Well, that's real.

Josh pinched me, it hurt. Yeah, you could tell. You all right? Yeah, I'm fine. How are 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 patient from some ward where he had surgery or something on his brain. I didn't goof my hair. That's right. Yeah.

The little lady did. I have an Eastern block haircut now. Right. Yeah. 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. It is. You knew that because of a yet to be released audio book. That's right. It's forthcoming. It's a little teaser for everybody. That's right. But there are actually people out there, Chuck,

who cannot feel pain. Straight up, don't feel pain. Yeah. It's amazing. It's very rare, but it happens. It is rare, Chuck. Actually, the only hard number that I've seen is 60. 60 reported cases worldwide.

60 since 1983. Actually, I've got 35 in the United States as of now. Yeah. I saw that number as well. I've seen people who just plumb said we have no idea how many could be underreported, especially with younger kids. What we're talking about is called congenital insensitivity to pain with anhydrosis.

Right. Otherwise, we're just going to call it SEPA for short. Right. Because that's much easier. And that is actually part of a, it's a subtype of a larger disorder called hereditary sensory and autonomic neuropathy. And this is actually the type four.

SEPA is type four H-S-A-N. Right? And it's the most severe, well, we'll get into what it all means, but it's the most severe. We will. There's actually kind of a recent, famous case of it. There is a British physician named Dr. Joffrey Woods. You heard of him, Chuck?

No, but is he one of the G-E-O-F guys? Yeah. Yeah. He's British. Yeah. He's both programmed with two M's and an E. Right. But Dr. Woods 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, walk on hot coals. Right. And showed no signs of feeling pain whatsoever. And he wasn't in the Jim Rose circus? No. OK.

He wasn't, actually. But he probably could have done a lot better for himself than just on the streets of Lahore. Yeah. Right? What Dr. Woods heard about this guy, 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 14th birthday died. Actually, 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.

Oh, really? Yeah, which is also called analgesia. Oh, that's the other word for it? I didn't know that. Yeah, painkillers are called analgesics. Oh, sure. So this would be like the state of not having pain or having reduced pain would be analgesia.

But this isn't just reduced pain, is it, Chuck? No, we're talking about no, you can feel pressure, but you can feel no pain. 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. OK, let's talk about the pain part first.

Let's talk about pain in general. OK. How do we feel pain? Well, Josh, your nervous system is where all this happens. And we're talking about your brain. Right, this is your brain on pain. Yeah, your cranial nerves, spinal cord, spinal nerves, and other things like your gangly and receptors,

sensory receptors, stuff like that. Right. 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 burned.

It hurts. Right, and it sends a response message that moves your hand away real quick, if you're touching a hot stove or something like that. Yeah, you can take it from here, though, because you're Mr. Doctor Guy, Dr. Clark. Thank you, Chuck. Actually, the sense of pain, the experience of pain,

is called nociception, right? Yeah. 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? And we have actually specialized nerve endings, receptors, called nociceptors, that are responsible just

for sending pain signals. These peripheral nerves. Right, thank God for that. Yes, actually, as painful as pain is, we have it for a reason. Sure. Like you said, you're touching a hot stove, and your brain says, get your hand off of it, right?

We also developed one theory of how we develop fear, which is good in and of itself as well, is through direct conditioning. That's one of three ways we learn fear responses, is through direct conditioning. And part of direct conditioning is experiencing physical pain. Right, 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 going to pick it up, right? Now, what happens with SIPA, as far as I understand? And actually, did you notice as well, there's not a pat explanation of, if you have SIPA, this is exactly what happens to you. Yeah, well, probably because it's so rare.

Right, but wouldn't you think that if they could get the group of patients together, they would find like, oh, OK, well, yeah, all of you people are lacking nociceptors. Yeah, yeah. In some patients, the nociceptors aren't there. In other patients, the peripheral nerves that the nociceptors are attached to or uses the information highway aren't there, or else they're not

innervated. There's a lot of different ways that this can come about, but it all results in this cardinal diagnosis or diagnostic tool of SIPA, which is you feel no pain, and you aren't responsive to temperature either. Right, but nerve conduction is there, right? It depends on some of them. There's innervation.

In others, the nerves aren't conducting or they're not capable of conducting. And specifically with anhedrosis, I think 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. A is anhedrosis after the SIP part, and that's the most severe case, and that's when you don't sweat, which, you know how I sweat. I know, you definitely don't have anhedrosis. Remember at the aquarium, I sweated underwater? In like 40 degree water, too. I mean, there was like little chunks of ice floating

on the surface of the water, and you had cartoon sweat jumping off your forehead. I did. So I could handle the anhedrosis part, or at least a slight version of that. I could deal with that. Right. And these are our eccrine sweat glands,

or our eccrine glands that are responsible for regulation of body temperature. So what they found with the anhedrosis, it seems fairly uniform in that respect, that the SIPA patients don't have innervation, meaning the nerves aren't becoming activated in their sweat glands. Yeah, and that's especially, I mean, it's bad enough to overheat

because you can overheat just as an adult, but it's really scary with babies with SIPA, because a baby can overheat and die very easily. Well, yeah, actually, I think 20% of people who have SIPA die by the age of three. Yeah, that's all that's that. And I think another quarter of those deaths are from sepsis, from an untreated infection.

Right. 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 better treat this cut. Right. And at the same time, you're not crying, so your parents aren't alerted.

Right. And so this cut can go untreated, become infected. When the cut is infected, the body's immunological response raises your body temperature. Right. It can lead to a fever, and unchecked, this fever can actually lead to brain damage. Right.

So a lot of patients with SIPA who've made it past three or so have some form of mental retardation. Yeah, I also saw where we'll get to the tips later. But since you brought it up, one of the tips for small kids is they'll 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 find mom and dad. Yeah, and I thought these tips that, like you said, we'll get to in a minute, they're pretty clever. Right. Or come find mom and mom, or dad and dad. Nice, Chuck. You're a 21st century guy, aren't you? Yeah, but mind it around here.

Or just mom, or just dad. Or Uncle Charlie, who's a- Or grandma. Or grandma. Yeah. Or grandpa and grandpa. There's all kinds of scenarios. No, somebody, the first person to be legally pronounced

asexual was legally pronounced asexual in the last couple of weeks, I believe. Really? Yeah, it's true. Very interesting. Let's do one on that. Yeah. OK.

Where are we, Chuck? Let's talk about SIPA, the medical history of SIPA, OK? OK. As far as I could find, it was first described in 1847. And described as what is going on here? Yeah, exactly. And actually, in the 60s, I read an article in 1960 that was based on a hypothesis that people who feel no pain

were actually, it was actually a psychological disturbance. Oh, really? There had been such a massive affront to their ego that as a response, the response of their ego mounted resulted in a loss of the sensation of pain. That's such a 60s notion, too. It totally is. So that cat just can't feel pain.

He's so out there. He's so far out. There was a doctor who was surveying this literature of certain patients. And he was describing how the diagnosis went. And there's this one poor guy in the 50s who he was described as a pretty nice, upbeat, bright guy. And he goes to the doctor and is like, I can't feel pain.

And the doctor's like, oh, yeah? So you can see this half-drunk doctor, right? He's got like a glass of scotch in his hand or something. He has a cigarette in the other. All right. And the little reflective thing on his forehead. 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. Did he do each of these? And was he like, what about that? No, I feel that. Can't you imagine this doctor's like, oh, yeah? That didn't hurt. We'll try this.

He keeps ratcheting it up. Exactly. Wow. Yeah. 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 mention, too, that they think they have narrowed it down to a mutation of the TRKa parentheses NTRK1 gene, which appears to control nerve growth. But again, they don't know for sure. They just think that might be the culprit. Chuck, that seems to be the likeliest candidate, because that gene, actually, is responsible for producing nerve growth factor, which goes and recruits neurons and nerves.

I guess it would be neurons to become nociceptors. Oh, OK. And so that wouldn't happen. Right. But they're kind of speculating still a little bit, right? Yeah. We still don't have an idea. I think that's the case is reported worldwide.

Right. 35 in the US, maybe. Yeah. Although it does seem like it would be genetic. Like that kid in Lahore, 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.

That suggests either something environmental, close to the home, or genetic. And that's kind of what they're leaning to. Yeah. Is the genetic part. Right, Josh. Or do they know for sure that it's an autosomal recessive disorder?

I don't know how they do, but 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. Right.

So 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 doesn't affect every ethnic group. Oh, really? Yeah. But I was reading another study on this from, I think, 2000. And it was in, I think, Jerusalem, or Tel Aviv, one of the

two, and they got together a bunch of kids who had SIPA. And some of them were related. And they took a family history and found that, I think, nine of the 13 participants' parents were first or second cousins. Uh-huh. So I wonder how much that has to do with something, too. Yeah. Interesting.

Yeah. Very interesting. So should we talk about some of the problems? I mean, they're pretty obvious, but there are some that I didn't actually consider. Well, yeah, let's talk about raising a baby with SIPA. How would you first start to recognize that your kid has SIPA? Well, that's a problem because the first way you would probably

recognize it is witnessing an accident that should cause your baby to cry and your baby doesn't cry. Right. That's like, have you ever heard the discovery of ether and nitrous oxide as anesthetics? No. Right here. Well, actually, it happened in New York City, but a physician from

our fair city named Dr. Crawford Long. Yeah. I did a little book report on him when I was like the fourth grade. He was like our 1960s Scotch and cigarette doctor. Yeah. And he noticed that people are in the depths of an etherbender 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. Wow. Yeah. So there's a similarity there. I know 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 that will happen is a parent season accident and the child doesn't cry. So you're going to have some problems getting diagnosed though because it is so rare. In the meantime, the kid could end up with an infection and die

of overheating or suffer some sort of brain damage. Yeah. True. One of the other big problems with a baby is when a baby tees, a baby just gnaws 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 eye scratching. Yeah. Well, they said though on the teeth, they said that some parents elect to have the teeth, baby teeth removed all together.

Right. Yeah. 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.

Right. But it's like eat baby food for many, many years or chew right through your tongue. Right. Right. You know? So what was the one you just mentioned? Well, you are just rubbing your eye or whatever.

You pretty much know when to stop. Right. If you are a patient, a SIPA 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, but she had one eye removed and scratched the other eye. Wow. And she was five and could barely see and she'd done it to herself.

Well, another tip since we're there, a lot of parents elect to have little baby goggles for their child so they can't get to their eyes. Right. Which is sad. Yeah. Hunger pains. This is interesting. They don't feel hunger pains.

Right. So eating isn't something they realize they should do. So they sometimes SIPA 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. Right.

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, 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. Fractures are very common, obviously.

You can slam your hand at the door, won't feel the pain, won't realize you got a broken hand. And joints too. This is something I hadn't thought of either. Yeah, me too. But consider this. 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 our weight on another joint. Yeah, that's your body telling you to shift so you're not putting too much stress on anything. Right. But 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 to develop charcot joints because 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 grating in the joint. It fills with fluid. And there's definitely a point of no return that can lead to amputation, limb replacement, that kind of stuff.

Jeez. Well, one of the, since we're kind of doing the problem tip thing, one of the tips... Are we? Well, 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.

Or they may say your kid needs to be in a wheelchair a little more 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. If you have a baby that has sepa, 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 you should also check your child until they're old enough to do this on their own. Check them for injuries a couple of three times a day. Also, because of the anhedrosis, a lot of families opt to move to cooler climates just to protect against overheating. Yeah. And Katie Lambert wrote this article of stuff you miss in history class. Right.

Our cohorts. And Katie, suppose that as far as getting exercise, which everyone needs, that swimming might be a good thing for them to do. And that kind of made sense to me. Yeah, but have you ever swam and sweated underwater? Well, you know. Well, yeah. But I wondered about that because 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. Yeah, true. So it could still lead to trouble. I guess get in cool water. And maybe don't swim so much that you're going to be sweating. That'd 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. There is no cure. No. That has happened yet. And it's so rare that 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. If it's a genetic flaw, usually that means that an enzyme or a protein isn't being produced. Right. So you just use stem cells to generate that enzyme or protein and booyah.

Dr. Clark. No more sippa. It says booyah. Yeah. There are a couple of websites. Should we go ahead and plug those? Sure. 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 helproberto.com is another one that details a young boy with sippa. And Chuck, also, there is, from what I understand, a pretty good documentary called A Life Without Pain by director Melody Gilbert, and it's about sippa. Oh, really? Uh-huh. It's supposed to be pretty cool. I haven't seen it, but anybody interested might want to rent that.

Yeah, I'll check that out. 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 going to pinch you during that thing? It's going to be rough. Hopefully no more than once. Oh, it'll be more than once.

All right. If you want to learn more about sippa, type in C-I-P-A in the handysearchparthowstuffworks.com. That means it's time for listener mail. Yes, sir. Wait. Really? Yeah, exactly. Chuck, I want us to give a special shout out to 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. Echo's a cool name. Yeah. I like it. Spence goes by zebra, apparently. This came in when I was on vacation, huh?

Yeah, yeah. Because you replied. I did. And you promised a shout out and never gave it. Yeah, we didn't do a shout out. We are now, though. I mean, come on. They raked me over the coals and I responded.

Yeah, and you know, we typically don't do shout outs because we get requests 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. Exactly. So you guys keep the fort down and thanks for listening. Thank you, girls. And we'll get the listener mail eventually, I think.

Yes. But we should probably plug Facebook and Twitter. Tweet, tweet. Children of the next generation. I don't know. Modern, modern dudes. What generation now? Y, I think.

No, you're X. I'm Y. No, seriously. That says it all. We're definitely not millennials. So yes, we are on Facebook now for sure. We are on Facebook. We have consolidated our, we had multiple pages.

It was kind of a mess, but now we're all consolidated. We are personally putting stuff on Facebook and talking to people. It's kind of fun, actually. Yeah, it is. You're doing a heck of a job, Chuck. Thank 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, I'm fine. Yeah. And the same goes for Twitter, too. Do you have that same, that same desire, that same goal?

Yeah. 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. S-Y-S-K podcast. Right. The little at symbol. Sure. For those of you who don't know.

How that works. Right. So you can check those out. Join up. Follow us. And we will be interacting, right? Right. We're going to come down from our iFreeTower.

Right. Yeah. All right. So, Josh, I'm going to call this Earthquake Survivor. Did you read this one? No. Pretty remarkable. You're back.

You're not on vacation anymore. Hey, Josh and Chuck. I just wanted to e-mail 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 Isla Moja. Do you like my phonetic pronunciation?

You do. It's nice. 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, 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 8.8 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 to 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. 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'd saved in my fanny pack, and just listen with my eyes closed, really happy, because that was for the first time

the only connection to normal I had. That is awesome. Really cool. Wow. That was really different, 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. That feels good. Yeah. How often do we just sit and be quiet? Never. Never, because that'd be really bad radio. It's like an opposite of what we're supposed to do.

You have a loyal listener for years to come. That is from Ignacio. Thanks, Ignacio. Who's alive and well. Thank God. Do you hear about the Des Armes family, or the Des Arms? No. They're a family from Haiti who survived the Port-au-Prince quake, and they had a son

who lived in San Diego who's like, come live with me. You're kidding. They moved to San Diego just in time to survive the Chili Quake. Survived both though. Yeah. Wow. Yeah. And since you said that that way, what did you say the name of the place was?

San Diego, Port-au-Prince. No. Des Armes. The Des Arms? Yeah. They're the family. De Plain, Illinois, we should say. We goofed that one.

We didn't know. It's apparently. Des Plains. Des Plains. That's 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 De Plain, De Plain. So.

I think I said it. No. We're the same. Yeah. We're interchangeable. My pain is your pain. You can switch our guts. Right.

Our bellies. Well, officially, I would like to switch bellies, yours is slightly smaller than mine. Thank you. Officially, we'd like to make that correction. It is Des Plains, Illinois. And it's Eau Claire, Eau Claire, Wisconsin. No, it's not. And in Canada, they call 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 email combat over a topic and issue something like that, send us an email. Do not cite Wikipedia to support your claims or else you've lost right out of the gate. Yeah. Do better. Wrap it up.

Send it to stuffpodcast at howstuffworks.com. For more on this and thousands of other topics, visit howstuffworks.com. Want more howstuffworks? Check out our blogs on the howstuffworks.com homepage. Brought to you by the reinvented 2012 Camry. It's ready. Are you? You're ready to travel in 2023 and since 1981, Gate One travel has been providing more

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