WEBVTT - What's the deal with blue people? 0:00:00.960 --> 0:00:03.200 Hey, everybody, we are so glad to be coming back 0:00:03.279 --> 0:00:06.000 to s F Sketch Fest this week, the best comedy 0:00:06.000 --> 0:00:08.280 festival in the land, for our first live show in 0:00:08.280 --> 0:00:11.120 two years. Yep, we're gonna be there on Friday, January 0:00:11.160 --> 0:00:14.640 twenty one, seven thirty pm at the Sydney Goldstein Theater 0:00:14.720 --> 0:00:17.320 to be specific. And if you want tickets then head 0:00:17.360 --> 0:00:19.840 on over to s F Sketch Fest dot com or 0:00:19.880 --> 0:00:22.480 City box Office dot com and search stuff you should know. 0:00:22.960 --> 0:00:25.880 That's right. It's a vaccinated only show and a masking 0:00:25.960 --> 0:00:28.319 up show, so everyone be safe, get out there and 0:00:28.320 --> 0:00:31.560 come check us out live. We'll see you on January one. 0:00:33.400 --> 0:00:36.199 Welcome to Stuff you Should Know, a production of I 0:00:36.280 --> 0:00:45.520 Heart Radio. Hey, and welcome to the podcast. I'm Josh Clark, 0:00:45.560 --> 0:00:48.559 and there's Charles w Chuck Bryan over there, and uh, 0:00:48.640 --> 0:00:52.720 Jerry was just here. She said peace out, suckers, and 0:00:52.840 --> 0:00:55.640 this is stuff you should know. Of course, it was 0:00:55.720 --> 0:01:00.680 nice of her to log on to say goodbye. Right now, 0:01:00.680 --> 0:01:04.160 we're just getting Jerry. She's been busy lately. Yeah, what happens. 0:01:04.640 --> 0:01:07.640 It totally happens in this busy, go go world of 0:01:09.080 --> 0:01:14.160 almost two The Double Deuce this year has just flown by. 0:01:14.920 --> 0:01:19.040 It really has I think mercifully because this is going 0:01:19.080 --> 0:01:22.520 to be part of the lost years when we look back, 0:01:22.760 --> 0:01:27.760 you know. Yeah, it felt like eighteen years and somehow 0:01:28.600 --> 0:01:31.000 one has just like been in a snap. Yeah. I 0:01:31.040 --> 0:01:35.160 don't know what's going on. I don't think anyone does anymore, Chuck, 0:01:35.240 --> 0:01:37.960 So don't don't feel bad. Uh. Can we shout out 0:01:38.000 --> 0:01:42.240 to the people and sources that helped us along for 0:01:42.280 --> 0:01:47.920 this one? Well, our buddy Dave Rouse wrote an article 0:01:48.040 --> 0:01:52.560 for our Stuff Works. Uh, And I found a great 0:01:52.640 --> 0:01:55.040 article from Wired. I forgot how much I love Wired. 0:01:55.120 --> 0:01:59.080 That's from Mallory Pickett and BBC News, ABC News, NPR 0:01:59.200 --> 0:02:02.000 Morning a Dish, which is always a fun listen. Uh, 0:02:02.160 --> 0:02:04.920 National Library of Medicine here in the US. And then, 0:02:05.080 --> 0:02:07.559 as always, I feel like it makes appearance in every 0:02:07.560 --> 0:02:13.519 episode Jamaica Hospital dot Org. That's right, surprisingly helpful? Yeah, 0:02:13.600 --> 0:02:17.239 maybe not surprisingly? I wonder is that Jamaica Queens or 0:02:17.320 --> 0:02:21.440 Jamaica Jamaica. Oh you know what I was thinking, Jamaica Jamaica. 0:02:21.480 --> 0:02:25.800 Maybe Jamaica Queens. Yeah, should I Jamaica scene Jamaica Queens. 0:02:26.400 --> 0:02:30.360 My neighbor across the street, neighbor has uh is from Jamaica, 0:02:30.440 --> 0:02:33.280 the country, and I feel like a heel now because 0:02:33.320 --> 0:02:38.000 I was like, hey, that hospital websites great. Pat, She's like, 0:02:38.040 --> 0:02:41.360 what are you talking about? She's like, okay, great, glad 0:02:41.440 --> 0:02:46.959 you like some rando hospital website. Oh boys, so we're 0:02:47.000 --> 0:02:50.079 talking today. You went and found some medical stuff. That's 0:02:50.080 --> 0:02:52.440 why you went to look at Jamaica Hospital dot org. 0:02:52.480 --> 0:02:57.520 I'm guessing because we're talking about a really interesting condition 0:02:57.560 --> 0:03:01.560 and actually a few kinds of conditions that have something 0:03:02.480 --> 0:03:04.960 unusual in common, and that is that the people who 0:03:05.000 --> 0:03:10.600 suffer from these conditions have blue skin. Blueskin. You heard 0:03:10.600 --> 0:03:14.079 it here first, everybody, even though this really first started 0:03:14.160 --> 0:03:17.000 hitting the presses in two thousand eight. Yeah, I mean 0:03:17.000 --> 0:03:19.200 you didn't hear it here first. This, I mean, this 0:03:19.280 --> 0:03:22.800 is all over the internet. But we're talking about not 0:03:22.880 --> 0:03:26.880 the Blue Man Group, but the Blue People Group of Kentucky. 0:03:27.680 --> 0:03:30.160 It's a family that is I guess it's pronounced few Gate. 0:03:31.320 --> 0:03:35.840 It's not. No, that's Italian. He was from France. Well 0:03:35.880 --> 0:03:39.160 that's that's a spoiler. Oh is it? I'm sorry? I 0:03:39.200 --> 0:03:42.600 take it back. Everyone. You know it's not a spoiler. 0:03:42.640 --> 0:03:45.440 But for some reason, when I saw that the original 0:03:46.080 --> 0:03:50.200 Martin Fugate was from France. From uh, I'm not sure 0:03:50.200 --> 0:03:52.400 what part of France, but I was like, that's surprising. 0:03:52.560 --> 0:03:55.960 It was like, I don't know why it would be. Uh. Yeah. 0:03:56.040 --> 0:03:57.840 He could have come from anywhere in the world, and 0:03:57.880 --> 0:04:01.880 he would have been like a completely overlookable person, I think, 0:04:02.000 --> 0:04:05.880 totally lost to history had it not been for the 0:04:06.000 --> 0:04:10.840 rare um rare genetic mutation that he happened to carry 0:04:10.880 --> 0:04:13.520 with him. And even that wouldn't have gotten him into 0:04:13.520 --> 0:04:17.920 the history books, Chuck, Even that wouldn't have. Instead, it 0:04:17.920 --> 0:04:23.200 took Martin Fugate to happen, upon against all odds, to 0:04:23.560 --> 0:04:26.400 have found a wife who he was not related to, 0:04:26.440 --> 0:04:29.679 who was a total stranger from a totally different country, 0:04:29.920 --> 0:04:34.320 who also happened to have that same rare genetic mutation. 0:04:34.400 --> 0:04:38.080 And when the two get together, capal blue kids start 0:04:38.160 --> 0:04:42.680 popping out. That's right. He came over from France to Kentucky, 0:04:42.720 --> 0:04:45.480 to the Blue Hills of Kentucky. Ironically, Oh wow, I 0:04:45.480 --> 0:04:49.600 hadn't thought about that. In And like I said, he 0:04:49.640 --> 0:04:51.320 was from France when he was an orphan, so he 0:04:51.360 --> 0:04:53.760 didn't know a lot about his his family as far 0:04:53.839 --> 0:04:56.559 as uh, whether or not any of them were blue, 0:04:56.720 --> 0:04:59.640 I guess, or if so, he wasn't he wasn't piping 0:04:59.720 --> 0:05:02.960 up a out. Yeah, maybe he just kept it quiet. 0:05:03.680 --> 0:05:07.600 But he did marry a redheaded American named Elizabeth Smith, 0:05:08.080 --> 0:05:11.240 and they headed on over to Troublesome Creek near Hazard 0:05:11.240 --> 0:05:14.039 County in Kentucky and set up a homestead there. And 0:05:14.240 --> 0:05:17.120 it is a I mean the one of the doctors 0:05:17.160 --> 0:05:20.200 that uh from one of these articles said, you know, 0:05:20.240 --> 0:05:23.440 it's maybe one and a one hundred thousand chance that 0:05:23.480 --> 0:05:26.760 you have to even have this genetic mutation. And he said, 0:05:26.760 --> 0:05:30.039 but you know, once you start, I guess there's no 0:05:30.080 --> 0:05:32.560 other way to say it. Once you start in breeding, 0:05:33.680 --> 0:05:38.159 which can happen in the eighteen hundreds in the rural 0:05:38.200 --> 0:05:40.680 nous of Kentucky, he said, that can go down all 0:05:40.680 --> 0:05:43.000 the way to like one in eight chance. And that's 0:05:43.000 --> 0:05:45.600 exactly what happened. Yeah, I was surprised there was even 0:05:45.640 --> 0:05:49.640 one in eight. But there's some little fluke of genetics that, um, 0:05:49.720 --> 0:05:53.280 that you have to when you're talking about probability in genetics, 0:05:53.279 --> 0:05:58.200 it's a little more difficult than half in a straight math. Yeah. Um. 0:05:58.680 --> 0:06:03.680 So apparently Martin himself possibly had like a little bit 0:06:03.680 --> 0:06:07.440 of blue to him. His wife Elizabeth had no blue 0:06:07.480 --> 0:06:10.960 to her um. She apparently had very very pale skin 0:06:11.040 --> 0:06:14.159 and bright red hair. But when um the two got 0:06:14.160 --> 0:06:19.440 together and those two genetic mutations donated UM their mutations 0:06:19.440 --> 0:06:22.720 to that specific gene, they the kids that they had 0:06:22.839 --> 0:06:28.000 were like, um, irresistibly blue. Like there was no confusing. 0:06:28.000 --> 0:06:30.479 It's not like, yeah, Martin's kind of blue. If you 0:06:30.480 --> 0:06:34.160 ever noticed, maybe you need to breathe more deeply. This 0:06:34.320 --> 0:06:38.040 was like straight up blue kids. I've even seen him 0:06:38.080 --> 0:06:41.400 described as dark blue, not even a bluish hue or 0:06:41.480 --> 0:06:46.000 tint or tinge dark blue skinned children. Yea, four of 0:06:46.160 --> 0:06:49.240 seven were blue. Uh. And it's funny he said that 0:06:49.279 --> 0:06:52.919 she was not. The mother was not blue tinted. But 0:06:53.240 --> 0:06:58.000 I imagine, like before they knew about genetics and in breeding, 0:06:58.880 --> 0:07:00.720 if he would, if she would been a little blue, 0:07:00.760 --> 0:07:03.839 that might have been a reason to get together, you 0:07:03.880 --> 0:07:07.880 know what I mean? No, I don't explain. Well, blue 0:07:07.920 --> 0:07:10.920 man happens to meet blue woman and they're like, jeez, 0:07:11.000 --> 0:07:14.160 there's another one like me, and you know we've been 0:07:14.240 --> 0:07:16.760 sort of you know, sort of embarrassing and maybe we're 0:07:16.800 --> 0:07:19.760 cast out a little bit. So let's hitch our wagons 0:07:19.760 --> 0:07:23.960 together because there's probably nothing wrong with us having kids, right, 0:07:25.000 --> 0:07:27.680 His medical science hadn't known that at that point. It's 0:07:27.720 --> 0:07:30.400 a story as old as time, Chuck, but it was. 0:07:32.000 --> 0:07:35.920 It was just complete happenstance though, right. Yeah, So, UM, 0:07:35.920 --> 0:07:38.440 And I wonder also, like it was significant to me 0:07:38.520 --> 0:07:42.320 that they pointed out that his wife, Elizabeth Smith um 0:07:42.480 --> 0:07:45.680 few Gates skin was almost translucent. It was so pale, 0:07:46.000 --> 0:07:50.520 because I wonder if she donated that to her children, UM, 0:07:50.560 --> 0:07:53.080 which allowed the blue to really shine through even more. 0:07:53.960 --> 0:07:58.440 Maybe it's possible, like the ultimate recipe for blueness exactly. Um. 0:07:58.520 --> 0:08:01.480 And they actually became owns, this blue family, and they 0:08:01.480 --> 0:08:05.240 were not particularly proud of their blue skin. I think 0:08:05.240 --> 0:08:07.160 they were a little freaked out about it. I think 0:08:07.160 --> 0:08:10.360 that their neighbors may not have particularly treated them well, 0:08:10.440 --> 0:08:13.640 and so they already lived in like a pretty isolated 0:08:13.720 --> 0:08:17.480 part of Appalachia, UM, but they took pains to actually 0:08:17.480 --> 0:08:20.640 isolate themselves even further. And one of the one of 0:08:20.680 --> 0:08:24.440 the consequences of that was something you touched upon. That 0:08:24.440 --> 0:08:28.200 that meant that, Um, their son married their aunt, and 0:08:28.320 --> 0:08:31.400 cousins married cousins, even if they were way too close 0:08:31.440 --> 0:08:34.320 to be marrying, and so one of the one of 0:08:34.360 --> 0:08:38.600 the products of these this inner marriage was an entire 0:08:39.440 --> 0:08:43.240 small regional population of blue families or families who have 0:08:43.360 --> 0:08:47.880 blue children or Blue family members, because these neighbor families 0:08:48.120 --> 0:08:51.880 were still blood relatives of the few Gates who initially 0:08:52.240 --> 0:08:57.280 settled this area and just started reproducing through inbreeding. Uh. 0:08:57.320 --> 0:09:00.200 And the condition that they had is not a skin 0:09:00.240 --> 0:09:02.880 condition at all. It's actually a blood disorder like you mentioned, 0:09:03.240 --> 0:09:08.600 and it's called medima globinemia. It's so pretty good. Wow, 0:09:09.000 --> 0:09:12.959 you're so it's not meth now, Okay, Oh, I'm glad 0:09:13.000 --> 0:09:16.600 you said it first. Then I actually took great pains 0:09:16.600 --> 0:09:19.040 today to look up between this and the other episode 0:09:19.080 --> 0:09:25.640 we're recording pronunciations, and when you look at medima globinemia, 0:09:25.880 --> 0:09:29.559 that is seventeen letters of it's a mouthful of letters, 0:09:30.040 --> 0:09:33.080 pure hell. And it's really one of those that I 0:09:33.120 --> 0:09:35.000 looked at over and over and just could not get 0:09:35.000 --> 0:09:36.800 it right in my head until I, you know, went 0:09:36.840 --> 0:09:38.240 and looked it up on the internet and I was like, 0:09:38.480 --> 0:09:39.960 that's not so hard, all right, let me try. Let 0:09:40.000 --> 0:09:44.080 me try mediema globinemia. I think it's more of a 0:09:44.120 --> 0:09:47.800 t than a d uh, okay, what about a t 0:09:49.240 --> 0:09:53.320 you want me try that one? This is like the 0:09:53.400 --> 0:10:01.520 worst acting audition ever. What's my motivation here? Uh to 0:10:01.559 --> 0:10:05.120 get through this episode? Uh So we're gonna touch a 0:10:05.120 --> 0:10:08.720 little bit on melanin throughout this episode. But you remember 0:10:08.760 --> 0:10:13.160 melanin from our I think pretty good episode on sunscreen 0:10:13.320 --> 0:10:18.200 and sun tans, right. Uh? And this is uh, you know, 0:10:18.240 --> 0:10:21.280 special cells in the body, in the skin make melanin 0:10:21.760 --> 0:10:24.640 and they are Melanin is responsible for coloring your skin. 0:10:25.360 --> 0:10:28.840 And there are all kinds of different uh melanin disorders, 0:10:28.880 --> 0:10:31.640 whether you have too much or too little, whether you 0:10:31.679 --> 0:10:33.840 can you know, we'll make your skin lighter or darker, 0:10:34.600 --> 0:10:37.679 or whether or not it happens in patches. I had 0:10:37.679 --> 0:10:41.959 a friend in Los Angeles with bid eligo, which oh 0:10:42.000 --> 0:10:46.080 you do, which you know leads to uh, patches of 0:10:46.160 --> 0:10:49.160 light skin on the body. In't that what Michael Jackson 0:10:49.160 --> 0:10:53.600 claimed to have had to Yes? Was that verified? I 0:10:53.679 --> 0:10:56.520 don't know. I don't know either. I really don't know, 0:10:56.559 --> 0:10:58.280 because I just remember there was a big stink when 0:10:58.320 --> 0:11:00.320 that came out and everyone was like, yeah, right, the 0:11:00.480 --> 0:11:03.520 well the reason why it was like, really it's you 0:11:03.600 --> 0:11:07.600 got videlago because it's usually um patches like the melano sites. 0:11:07.600 --> 0:11:10.360 The melanin producing cells just kind of poop out or 0:11:10.360 --> 0:11:14.040 crap out in an area, and it it leaves like 0:11:14.120 --> 0:11:18.679 patches without pigment, not your entire face, um without pigment. 0:11:18.800 --> 0:11:21.679 But who knows, well, I think his I think what 0:11:21.720 --> 0:11:24.800 he said was that he bleached his skin to match 0:11:24.840 --> 0:11:28.760 the light patches. Was his reasoning. Oh, well, that's fairly reasonable. 0:11:29.080 --> 0:11:32.400 You know, if you're self conscious about videlago and you 0:11:32.440 --> 0:11:34.920 have a ton of money, you could do something like that. 0:11:35.440 --> 0:11:39.280 Who knows, Who knows? Should we talk about skin color? 0:11:39.360 --> 0:11:42.480 Should we take an awkward Michael Jackson break? I think 0:11:42.520 --> 0:11:44.880 we just did. But the point is this that I 0:11:44.920 --> 0:11:48.480 think you're making, Chuck, is that changes in like your 0:11:48.520 --> 0:11:51.800 skin color typically have to do with a lack of 0:11:52.040 --> 0:11:56.000 or an overproduction of melanin. Right, And that what we're 0:11:56.040 --> 0:12:02.520 talking about, uh, mattiemoglobinemia right, is actually a blood disorder. 0:12:02.600 --> 0:12:05.080 It's not has nothing to do with the skin. It 0:12:05.240 --> 0:12:09.880 just shines through that super translucent skin that we have. Right, 0:12:09.960 --> 0:12:14.559 So what's going on here in the body? Okay, So 0:12:15.280 --> 0:12:18.880 are you surprised, I'm asking you. I'm a little surprised. 0:12:19.080 --> 0:12:22.720 I thought we maybe tagging team this one. But okay, okay, so, 0:12:23.000 --> 0:12:26.240 uh you have like a pink is shoe too, typically 0:12:26.760 --> 0:12:31.760 being being Caucasian. Um, because you're there's not a ton 0:12:31.760 --> 0:12:37.120 of melonin that's blocking the blood supply underneath and your tissues. 0:12:37.160 --> 0:12:40.400 It's kind of shining through and your blood is red. 0:12:40.480 --> 0:12:45.680 And the reason it's red, Chuck, is because because the 0:12:45.760 --> 0:12:48.520 reason why blood is red, well, remember in school they 0:12:48.520 --> 0:12:51.120 teach you that your blood is blue until you get 0:12:51.120 --> 0:12:53.520 cut and then it hits the air. Yeah, that's not true, 0:12:53.720 --> 0:12:55.120 and then it turns red. That's what you hear on 0:12:55.160 --> 0:12:58.680 the playground in elementary school from dumb kids. But that 0:12:58.840 --> 0:13:00.679 is not true. And I bet you anything, kids are 0:13:00.679 --> 0:13:04.040 still spreading that mistruth on the playground. Yeah. It's got 0:13:04.080 --> 0:13:06.839 some staying power for sure, as far as as far 0:13:06.880 --> 0:13:09.600 as rumors go. And you know what, my dad will 0:13:09.640 --> 0:13:13.600 sue your dad for all the money he's got. Did 0:13:13.600 --> 0:13:18.559 you guys threaten lawsuits? Sure? We were very lutitious kids 0:13:18.600 --> 0:13:20.360 did though. It was very funny. We were like, we're 0:13:20.400 --> 0:13:24.520 like little Puritans in in Salem town. That's right. Uh 0:13:24.600 --> 0:13:28.880 so blood is red? That was a question, right? Uh? Yeah, yeah, 0:13:28.880 --> 0:13:31.920 why is blood red, right, because I don't know we've 0:13:31.920 --> 0:13:34.840 talked about this before, but it's because our red blood 0:13:34.880 --> 0:13:38.400 cells have hemoglobe and it's a protein and it gets 0:13:38.480 --> 0:13:42.239 its red color from something called hem It's a compound 0:13:42.280 --> 0:13:45.160 that has an iron atom in there, and that iron 0:13:45.200 --> 0:13:48.079 atom is the key. That's what binds with oxygen and 0:13:48.240 --> 0:13:51.560 allows red blood cells to get oxygen all over the body, 0:13:52.200 --> 0:13:55.480 and that's sort of what makes us tick. Yeah, it 0:13:55.559 --> 0:13:58.520 definitely is. I mean that oxygen is super important, so 0:13:58.640 --> 0:14:02.480 we want to have super red rich hemoglobin blood. Right. 0:14:03.120 --> 0:14:08.040 Our bodies also produced something else called metemoglobin's I guess 0:14:08.120 --> 0:14:10.000 is how you would pronounce it. That is not how 0:14:10.080 --> 0:14:13.000 I've been pronouncing it in my head. But these metema 0:14:13.000 --> 0:14:17.320 globins are basically the same thing as hemoglobins, but they 0:14:17.360 --> 0:14:20.040 have a different kind of iron attached to them. The 0:14:20.080 --> 0:14:23.040 iron they have is like, there's really another way to 0:14:23.040 --> 0:14:24.880 put it. Actually, there's plenty of other ways to put it, 0:14:24.880 --> 0:14:26.920 but one way you could put it is that is rusted. 0:14:27.360 --> 0:14:32.160 It's oxidized iron, which means that it's missing an electron 0:14:32.640 --> 0:14:35.160 that it would need to bind oxygen, which means it's 0:14:35.320 --> 0:14:38.760 useless for transporting oxygen. Through the blood. So if you 0:14:38.760 --> 0:14:41.160 have a bunch of metemoglobin built up in your blood, 0:14:41.200 --> 0:14:44.040 your blood can reasonably turn blue. You also may have 0:14:44.080 --> 0:14:48.239 some problems breathing um because you don't have any oxygen 0:14:48.280 --> 0:14:50.600 in your blood, although that's not true, I'm sorry. You 0:14:50.640 --> 0:14:53.560 would be able to breathe, breathe perfectly fine, but it 0:14:53.640 --> 0:14:58.000 wouldn't clear up necessarily the lack of oxygen your blood 0:14:58.000 --> 0:15:01.080 because it's on a blood level um rather than something 0:15:01.120 --> 0:15:04.160 to do with your lungs. Yeah, that was my one question, 0:15:04.240 --> 0:15:09.480 because doctor after doctor is confirmed that mativa globinemia doesn't 0:15:09.520 --> 0:15:13.040 present any health issues. But how can that be possible 0:15:13.080 --> 0:15:17.120 that your blood has is having trouble carrying oxygen to 0:15:17.200 --> 0:15:19.560 your body and it doesn't matter. I had a really 0:15:19.560 --> 0:15:21.440 hard time with that too. And the best I could 0:15:21.440 --> 0:15:24.000 come up with as far as an answer, which is 0:15:24.040 --> 0:15:26.040 not definitive, but the best that can come up with, 0:15:26.160 --> 0:15:29.720 is that there is a there's a threshold where your 0:15:29.800 --> 0:15:33.480 blood will turn blue, where you have enough metema globins 0:15:33.520 --> 0:15:37.600 in there that it's blue, but not so there's not 0:15:37.680 --> 0:15:40.880 so many metema globins that you're actually having trouble with 0:15:40.960 --> 0:15:44.400 your oxygen in your body. So it can turn blue 0:15:44.440 --> 0:15:47.360 long before you actually like suffer from the effects of 0:15:47.440 --> 0:15:52.080 low oxygen um. Does that make sense? Yeah, I guess 0:15:52.080 --> 0:15:55.040 that makes sense. And isn't the threshold to turn your 0:15:55.080 --> 0:15:58.320 skin blue like a really low percentage, even like one 0:15:58.360 --> 0:16:00.880 percent of metemaments. If you're blood is one percent in 0:16:00.920 --> 0:16:03.200 team of globins, you can start to turn blue right 0:16:03.280 --> 0:16:06.360 So it doesn't take very much, which means that you 0:16:06.360 --> 0:16:10.520 have of of the global the globins in your blood 0:16:10.560 --> 0:16:14.360 are are hemoglobin, which means you're still able to get 0:16:14.360 --> 0:16:16.880 all the oxygen you need. You just have maybe a 0:16:16.920 --> 0:16:19.640 little less than somebody else. That's my best guess from 0:16:19.680 --> 0:16:23.200 what I saw UM, and I think at this point 0:16:23.200 --> 0:16:26.400 in our understanding of my team of globalemia, it's as 0:16:26.440 --> 0:16:29.560 good as anybody's. Well. That sounds like a perfect place 0:16:29.600 --> 0:16:32.600 for a break, Okay, and uh, we'll be right back 0:16:32.640 --> 0:16:35.000 to talk a little bit more about this and other 0:16:35.520 --> 0:16:58.680 interesting blood conditions right for this, So, Chuck, where're talking 0:16:58.680 --> 0:17:02.800 about my team globe and temia um and the reason like, 0:17:02.800 --> 0:17:04.679 like I think one of us said earlier that the 0:17:04.720 --> 0:17:10.280 body naturally produces these things, um, these metema globins um, 0:17:10.359 --> 0:17:14.720 but we also produce an enzyme that can convert excess 0:17:14.760 --> 0:17:19.159 metema globins into hemoglobin, and it's got a doozy of 0:17:19.160 --> 0:17:22.480 a name which I just love. May I take this one? Yes, 0:17:23.720 --> 0:17:29.320 this enzyme is called reduced nicotinamide adenine dionucleotide cytochrome B 0:17:29.520 --> 0:17:33.960 five reduct as one of the great all times nicknames. 0:17:34.000 --> 0:17:35.800 It has no nickname. You have to say the entire 0:17:35.880 --> 0:17:41.240 thing every time. And so this enzyme, reduced nicotinamine ad 0:17:41.240 --> 0:17:44.840 aine dionucleotide side of chrome B five reduct days is 0:17:44.840 --> 0:17:50.280 actually capable of converting the iron from its um ferric 0:17:50.359 --> 0:17:54.720 state in metema globin's into its fairest state, which is 0:17:55.000 --> 0:17:57.720 capable of binding with oxygen like the kind that's found 0:17:57.760 --> 0:18:01.680 in hemoglobin. So, for all intents and purposes, reduced nicotinamide 0:18:01.720 --> 0:18:06.480 adenine dinucleotide cytochrome B five reductase is capable of converting 0:18:06.720 --> 0:18:10.399 metemoglobins into hemoglobin. And most of us are able to 0:18:10.440 --> 0:18:13.320 do that. But if you're like a few gate and 0:18:13.440 --> 0:18:17.080 you have that rare genetic condition where both parents donated 0:18:17.119 --> 0:18:21.960 that mutation to you and you developed metemoglobinemia, but there's 0:18:22.000 --> 0:18:27.320 some reduced amount of reduced nicotinamide adenine dionucleotide cytochrome B 0:18:27.480 --> 0:18:32.000 five reductas, which means that that metemoglobin's that you naturally 0:18:32.040 --> 0:18:35.280 produced are able to build up and accumulate in your blood. 0:18:35.320 --> 0:18:37.520 And when they do that, and they they they that's 0:18:37.520 --> 0:18:39.600 how you start to turn blue. That's how you cross 0:18:39.680 --> 0:18:42.920 that one percent threshold. That's right. And this is one 0:18:42.960 --> 0:18:46.119 of the rare times where you can actually have a 0:18:46.160 --> 0:18:50.760 genetic condition that's pretty easily solved. And in this case, 0:18:50.800 --> 0:18:53.240 it's just a pill. Uh. And the guy who figured 0:18:53.280 --> 0:18:56.159 this out has sort of an interesting story in his 0:18:56.200 --> 0:19:01.159 own right. His name is Madison Coween or Coine Kaween. 0:19:01.600 --> 0:19:08.320 I'm going with col Wayne cal Wine, Colween, c A 0:19:08.560 --> 0:19:13.359 w Ei and Coween, although I like cow Wine. That 0:19:13.440 --> 0:19:15.639 doesn't sound like you'd be tasty, but I'll beg it 0:19:15.640 --> 0:19:18.919 gives you a pretty good bus. Uh. He was the 0:19:19.880 --> 0:19:22.879 h He was the grandson of a pretty famous at 0:19:22.880 --> 0:19:26.679 the time poet out of Kentucky. Uh. And Madison was 0:19:26.720 --> 0:19:30.679 the third of of the Madison's. And this is just 0:19:30.720 --> 0:19:34.639 a side note, very sad and interesting. His wife was 0:19:34.800 --> 0:19:39.000 poisoned and murdered by poison in the nineteen sixties and 0:19:39.000 --> 0:19:41.520 no one was ever caught in charge with a crime 0:19:41.520 --> 0:19:45.000 and it kind of remains one of Kentucky's um, you know, 0:19:45.040 --> 0:19:47.440 sort of biggest cold cases. Wow, I did not see 0:19:47.440 --> 0:19:50.639 that one coming. Yeah, he and his wife and this 0:19:50.720 --> 0:19:53.359 other couple were out drinking all night supposedly like just 0:19:53.400 --> 0:19:58.840 got slashed and at a at a at a country club. Um. 0:19:58.960 --> 0:20:01.040 He Madison didn't even make at home. I think he 0:20:01.119 --> 0:20:05.840 stayed the night there, but the other man, the man 0:20:05.880 --> 0:20:09.680 from the other couple, drove his wife home. Um, apparently 0:20:09.720 --> 0:20:12.240 just dropped. They had another nightcap and she woke up dead. 0:20:12.280 --> 0:20:15.680 One didn't wake up dead. She died and they thought 0:20:15.720 --> 0:20:18.680 it was just you know, alcohol or some natural cause. 0:20:18.720 --> 0:20:20.639 But then they found these two like little needle pricks 0:20:20.680 --> 0:20:25.359 in her where they think she was I guess given 0:20:25.400 --> 0:20:28.040 a shot to pass out and then poisoned. And I 0:20:28.040 --> 0:20:30.280 can't remember the poison. It was some sort of like 0:20:30.640 --> 0:20:34.800 acid or something. But yeah, really really interesting. Neither here 0:20:34.800 --> 0:20:36.960 nor there as far as this goes. But that's the 0:20:37.000 --> 0:20:42.399 sad story of Dr Madison. Uh cow wines wife. Was 0:20:42.440 --> 0:20:45.840 he a suspect because I would have suspected him. I 0:20:45.880 --> 0:20:48.240 don't think so, because he wasn't even there. That's the 0:20:48.240 --> 0:20:50.879 first thing I thought but apparently it was pretty verified 0:20:50.920 --> 0:20:53.280 that he slept it off at the country club. I 0:20:53.320 --> 0:20:56.239 guess crazy because yeah, well, I mean one of the 0:20:56.280 --> 0:20:58.680 things that he became famous for, if not the thing 0:20:58.720 --> 0:21:01.520 he became famous for. It wasn't jacting people with with 0:21:01.880 --> 0:21:06.080 some fairly weird stuff. I know, isn't that weird? It 0:21:06.240 --> 0:21:09.680 is weird. You're not buying it. No, I don't want 0:21:09.680 --> 0:21:11.960 to like cast persions on somebody. I don't know enough 0:21:12.000 --> 0:21:15.399 about the story, but that's just very odd, especially like 0:21:15.920 --> 0:21:20.400 judging from this ninety second story, exactly I can conclusively 0:21:20.480 --> 0:21:22.840 solve this case. I've seen enough law in order to 0:21:22.920 --> 0:21:25.840 know that the husband almost always did it else that 0:21:25.960 --> 0:21:28.280 is probably true, but probably not in this case. But 0:21:28.480 --> 0:21:32.320 at any rate, he was a hematologist at you at 0:21:32.440 --> 0:21:36.960 the University of Kentucky, and he heard about these people 0:21:37.040 --> 0:21:39.879 throughout the world and really wanted to kind of get 0:21:39.920 --> 0:21:42.440 to the bottom of this. When a couple of them, 0:21:42.680 --> 0:21:44.480 uh well, I don't think these were few gates, but 0:21:44.520 --> 0:21:47.920 Patrick and Rachel Ritchie, I guess you know it happened 0:21:47.920 --> 0:21:50.880 in some other family. Know the Combs is. We knew 0:21:50.880 --> 0:21:53.520 it Combs from Kentucky. We had a friend that we 0:21:53.600 --> 0:21:56.840 worked with for a little while. Really he was never 0:21:57.400 --> 0:22:00.399 struck me as blue. Yeah, he never struck you. But 0:22:00.440 --> 0:22:02.320 I wonder if he is related to the combs is, 0:22:03.720 --> 0:22:08.439 which would make him vibe some somehow. Few Gate because again, 0:22:08.760 --> 0:22:11.040 these these families in the few Gate area where all 0:22:11.080 --> 0:22:14.399 if you went back far enough, few Gates exactly. All right, 0:22:14.440 --> 0:22:16.520 So these people walk into his office and what happened? 0:22:16.800 --> 0:22:18.880 So here here's the thing, Like, they didn't walk into 0:22:19.000 --> 0:22:21.239 his office, he went and found them. I read an 0:22:21.359 --> 0:22:25.240 article about him, and he became maybe a little obsessed 0:22:25.240 --> 0:22:27.960 with finding these people. Wanted to know what the heck 0:22:28.040 --> 0:22:29.840 was going on. So he actually went out in the 0:22:29.840 --> 0:22:33.000 woods and would wait for them, and every once in 0:22:33.000 --> 0:22:34.920 a while would see one and he chased them, telling 0:22:34.960 --> 0:22:36.800 him to stop, and of course they would run off. 0:22:37.200 --> 0:22:39.000 So he figured out another way to do it. He 0:22:39.040 --> 0:22:42.399 moved to Hazard County near Troublesome Creek, and he started 0:22:42.440 --> 0:22:45.800 asking around at clinics, like had anybody ever treated a 0:22:45.840 --> 0:22:50.639 blue patient? And he finally struck gold Um blue Gold 0:22:50.840 --> 0:22:53.360 when he spoke to one nurse at a clinic that said, yeah, 0:22:53.400 --> 0:22:55.199 this this one woman came in with blue skin and 0:22:55.240 --> 0:22:57.400 she was really self conscious about her skin. She came 0:22:57.440 --> 0:22:59.919 to the back door. Was really just kind of me, 0:23:00.000 --> 0:23:03.520 he can embarrassed. Um, but yeah, we've treated a blue 0:23:03.520 --> 0:23:07.600 person before. So Colween basically hung around this clinic until 0:23:08.080 --> 0:23:10.240 more blue people came in and that this is where 0:23:10.320 --> 0:23:14.639 he met the Richie siblings. Yeah, so he started to 0:23:14.680 --> 0:23:17.280 dig into their family a little bit. Uh. It became 0:23:17.320 --> 0:23:19.919 pretty obvious to him that it was a genetic disorder, 0:23:20.600 --> 0:23:24.080 and he started digging around other stories around the North 0:23:24.160 --> 0:23:28.280 America and found an Inuit population in Alaska who had 0:23:29.560 --> 0:23:31.960 who had the same condition. I'm just gonna call it 0:23:32.000 --> 0:23:34.919 this condition now. I think we said it enough, at 0:23:35.000 --> 0:23:38.160 least I have. And he said, well, you know these 0:23:38.160 --> 0:23:42.280 people were interbreeding a lot because they were very remote 0:23:42.320 --> 0:23:45.120 as well. And so he said, I think we understand 0:23:45.200 --> 0:23:49.280 the problem here. How do we fix it? Yeah, I 0:23:49.320 --> 0:23:53.320 don't know if he where he he got this idea, 0:23:54.000 --> 0:23:57.520 but he used something called methylene blue, which was already 0:23:57.560 --> 0:24:01.639 like in wide use. It's used to staying tissue because 0:24:01.680 --> 0:24:05.439