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The 'Blue People' of Appalachia

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  • multiracialbookclub
    THE BLUE PEOPLE OF TROUBLESOME CREEK The story of an Appalachian malady , an inquisitive doctor, and a paradoxical cure. by Cathy Trost © Science 82,
    Message 1 of 3 , Feb 22, 2010
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      THE BLUE PEOPLE OF
      TROUBLESOME CREEK
       


      The story of 'an Appalachian malady', an 
      inquisitive doctor, and a paradoxical cure.

      by Cathy Trost © Science 82, November, 1982



      Illustration of Martin Fugate and his family. (circa 1900)
      Some reports say Martin was ... a carrier of the methemoglobinemia gene


      The Fugate Family

      Six generations after a French orphan named Martin 
      Fugate settled on the banks of eastern Kentucky's 
      Troublesome Creek with his redheaded American bride, 
      his great-great-great great grandson was born in a 
      modern hospital not far fromwhere the creek still runs.

      The boy inherited his father's lankiness and his 
      mother's slightly nasal way of speaking.

      What he got from Martin Fugate was dark blue skin. 

      "It was almost purple," his father recalls.

      Doctors were so astonished by the color of Benjy Stacy's skin 
      that they raced him by ambulance from the maternity ward 
      in the hospital near Hazard to a medical clinic in Lexington. 

      Two days of tests produced no explanation
      for skin the color of a bruised plum.

      A transfusion was being prepared 
      when Benjy's grandmother spoke up. 
      "Have you ever heard of the blue Fugates
      of Troublesome Creek?"
       she asked the doctors.

      "My grandmother Luna on my 
      dad's side was a blue Fugate. 
      It was real bad in her," AlvaStacy,
      the boy's father, explained.
       

      "The doctors finally came to the conclusion
      that Benjy's color was due to blood
      inherited from generations back."

      Benjy lost his blue tint within a few weeks, 
      and now he is about as normal looking a 
      seven-year-old boy as you could hope to find. 

      His lips and fingernails still turn a shade of 
      purple-blue when he gets cold or angry a quirk that 
      so intrigued medical students after Benjy's that they 
      would crowd around the baby and try to make him cry. 

      "Benjy was a pretty big item in the 
      hospital," his mother says with a grin.

      Dark blue lips and fingernails are the only traces
      of Martin Fugate's legacy left in the boy; that, and 
      the recessive gene that has shaded many of the 
      Fugates and their kin blue for the past 162 years.



      They're known simply as the "blue people" in the
      hills and hollows around Troublesome and Ball Creeks. 

      Most lived to their 80s and 90s without serious 
      illness associated with the skin discoloration. 

      For some, though, there was a pain not seen in lab tests. 

      That was the pain of being blue in a world
       
      that is mostly shades of white to black.

      There was always speculation in the hollows 
      about what made the blue people blue: 
      heart disease, a lung disorder, the 
      possibility proposed by one old-timerthat 
      "their blood is just a little closer to their skin." 

      But no one knew for sure, and doctors rarely paid visits 
      to the remote creekside settlements where most of 
      the "blue Fugates" lived until well into the 1950s. 

      By the time a young hematologist from the University 
      of Kentucky came down to Troublesome Creek in the 
      1960s to cure the blue people, Martin Fugate's 
      descendants had multiplied their recessive 
      genes all over the Cumberland Plateau.

      lorenzo and eleanor fugate
      Lorenzo and Eleanor Fugate.
      Lorenzo was also known as `Blue Anze' and was
      mentioned in Trost's 
      The Blue People of Troublesome Creek.


      Madison Cawein began hearing rumors about the blue 
      people when he went to work at the University of 
      Kentucky's Lexington medical clinic in 1960. 

      "I'm a hematologist, so something like that perks up my ears," 
      Cawein says, sipping on whiskey sours and letting
      his mind slip back to the summer he spent 
      "tromping around the hills looking for blue people."

      Cawein is no stranger to eccentricities of the body. 
      He helped isolate an antidote for cholera, 
      and he did some of the early work on 
      L-dopa, the drug for Parkinson's disease. 

      But his first love, which he developed as an Army 
      medical technician in World War II, was hematology. 
      "Blood cells always looked so beautiful to me," he says.

      Cawein would drive back and forth between 
      Lexington and Hazard an eight-hour ordeal 
      before the tollway was built and scour the hills 
      looking for the blue people he'd heard rumors about. 

      The American Heart Association had a clinic 
      in Hazard, and it was there that Cawein met 
      "a great big nurse" who offered to help.

      Her name was Ruth Pendergrass, and she had 
      been trying to stir up medical interest in the 
      blue people ever since a dark blue woman walked 
      into the county health department one bitterly 
      cold afternoon and asked for a blood test.

      "She had been out in the cold and she was just blue!"
      recalls Pendergrass, who is now 69 and retired from nursing. 

      "Her face and her fingernails were almost indigo blue. 
      It like to scared me to death! 
      She looked like she was having a heart attack. 
      I just knew that patient was going to die right there in 
      the health department, but she wasn't a'tall alarmed. 

      She told me that her family was the blue 
      Combses who lived up on Ball Creek. 

      She was a sister to one of the Fugate women." 

      About this same time, another of the blue 
      Combses, named Luke, had taken his sick 
      wife up to the clinic at Lexington. 

      One look at Luke was enough to "get those doctors 
      down here in a hurry," says Pendergrass, who 
      joined Cawein to look for more blue people.

      Trudging up and down the hollows, fending off 
      "the two mean dogs that everyone had in their front yard," 
      the doctor and the nurse would spot someone at the top 
      of a hill who looked blue and take off in wild pursuit. 

      By the time they'd get to the top, the person would be gone. 

      Finally, one day when the frustrated doctor was idling inside 
      the Hazard clinic, Patrick and Rachel Ritchie walked in.

      "They were bluer'n hell," Cawein says. 
      "Well, as you can imagine, I really examined them. 

      After concluding that there was no evidence of heart 
      disease, I said 'Aha!' I started asking them questions: 
      'Do you have any relatives who are blue?' 
      then I sat down and we began to chart the family."

      Cawein remembers the pain that showed on 
      the Ritchie brother's and sister's faces. 

      "They were really embarrassed
      about being blue," he said. 

      "Patrick was all hunched down in the hall. 
      Rachel was leaning against the wall. 
      They wouldn't come into the waiting room. 
      You could tell how much it bothered them to be blue."

      After ruling out heart and lung diseases,
      the doctor suspected 'methemoglobinemia',
      a rare hereditary blood disorder that results
      from excess levels of methemoglobin in the blood. 
      Methemoglobin which is blue, is a nonfunctional form 
      of the red hemoglobin that carries oxygen. 
      It is the color of oxygen-depleted blood seen 
      in the blue veins just below the skin.

      If the blue people did have methemoglobinemia, 
      the next step was to find out the cause. 

      It can be brought on by several things: 
      abnormal hemoglobin formation, an enzyme deficiency, 
      and taking too much of certain drugs, including 
      vitamin K, which is essential for blood clotting 
      and is abundant in pork liver and vegetable oil.

      Cawein drew "lots of blood" from the 
      Ritchies and hurried back to his lab. 

      He tested first for abnormal 
      hemoglobin, but the results were negative.

      Stumped, the doctor turned to the 
      medical literature for a clue. 

      He found references to methemoglobinemia 
      dating to the turn of the century, but it wasn't 
      until he came across E. M. Scott's 1960 report 
      in the Journal of Clinical Investigation (vol. 39, 
      1960) that the answer began to emerge.

      Scott was a Public Health Service doctor at the 
      Arctic Health Research Center in Anchorage who
      had discovered hereditary methemoglobinemia
      among Alaskan Eskimos and Indians. 

      It was caused, Scott speculated, by an absence of 
      the enzyme diaphorase from their red blood cells. 
      In normal people hemoglobin is converted 
      to methemoglobin at a very slow rate. 
      If this conversion continued, all the body's 
      hemoglobin would eventually be rendered useless. 
      Normally diaphorase converts 
      methemoglobin back to hemoglobin. 

      Scott also concluded that the condition 
      was inherited as a simple recessive trait

      In other words, to get the disorder, a person would 
      have to inherit two genes for it, one from each parent. 
      Somebody with only one gene would not have 
      the condition but could pass the gene to a child.

      Scott's Alaskans seemed to match Cawein's blue people... 

      Cawein needed fresh blood to do an enzyme assay. 
      He had to drive eight hours back to Hazard 
      to search out the Ritchies, who lived in a 
      tapped-out mining town called Hardburly. 
      They took the doctor to see their uncle, who was blue, too. 
      While in the hills, Cawein drove over to see Zach 
      (Big Man) Fugate, the 76-year-old patriarch 
      of the clan on Troublesome Creek. 
      His car gave out on the dirt road to Zach's house, and 
      the doctor had to borrow a Jeep from a filling station.

      Zach took the doctor even farther up Copperhead 
      Hollow to see his Aunt Bessie Fugate, who was blue
      Bessie had an iron pot of clothes boiling in her 
      front yard, but she graciously allowed the 
      doctor to draw some of her blood.

      "So I brought back the new blood and 
      set up my enzyme assay," Cawein continued. 
      "And by God, they didn't have the enzyme diaphorase. 
      I looked at other enzymes and nothing was wrong with them. 
      So I knew we had the defect defined.''

      Just like the Alaskans, their blood had accumulated 
      so much of the blue molecule that it over- whelmed 
      the red of normal hcmoglobin that shows through 
      as pink in the skin of most Caucasians.

      Once he had the enzyme deficiency isolated, 
      methylene blue sprang to Cawein's mind 
      as the "perfectly obvious" antidote. 

      Some of the blue people thought the doctor was slightly 
      addled for suggesting that a blue dye could turn them pink. 

      But Cawein knew from earlier studies that 
      the body has an alternative method of 
      converting methemoglobin back to normal. 

      Activating it requires adding to the blood a 
      substance that acts as an "electron donor." 

      Many substances do this, but Cawein chose methylene 
      blue because it had been used successfully and 
      safely in other cases and because it acts quickly.

      Cawein packed his black bag and rounded 
      up Nurse Pendergrass for the big event. 

      They went over to Patrick and Rachel 
      Ritchie's house and injected each of them 
      with 100 milligrams of methylene blue.

      ''Within a few minutes. the blue color 
      was gone from their skin," the doctor said. 
      "For the first time in their lives, they were pink. 
      They were delighted."

      "They changed colors!" remembered Pendergrass. 
      "It was really something exciting to see."

      The doctor gave each blue family a supply of 
      methylene blue tablets to take as a daily pill. 

      The drug's effects are temporary, as methylene 
      blue is normally excreted in the urine. 

      One day, one of the older mountain men cornered the doctor. 
      "I can see that old blue running out of my skin," he confided.

      Before Cawein ended his study of the blue people, he 
      returned to the mountains to patch together the long 
      and twisted journey of Martin Fugate's recessive gene. 

      From a history of Perry County and some 
      Fugate family Bibles listing ancestors, 
      Cawein has constructed a fairly complete story.

      Martin Fugate was a French orphan who emigrated 
      to Kentucky in 1820 to claim a land grant on 
      the wilderness banks of Troublesome Creek. 

      No mention of his skin color is made in the 
      early histories of the area, but family lore 
      has it that Martin himself was blue.

      The odds against it were incalculable, but 
      Martin Fugate managed to find and marry a 
      woman who carried the same recessive gene

      Elizabeth Smith, apparently, was as 
      pale-skinned as the mountain laurel that 
      blooms every spring around the creek hollows.

      Martin and Elizabeth set up housekeeping on 
      the banks of Troublesome and began a family. 

      Of their seven children, four were reported to be blue.

      The clan kept multiplying. 

      Fugates married other Fugates. 
      Sometimes they married first cousins. 

      And they married the people who lived closest to 
      them, the Combses, Smiths, Ritchies, and Stacys. 

      All lived in isolation from the world, 
      bunched in log cabins up and down the
      hollows, and so it was only natural that
      a boy married the girl next door,
      even if she had the same last name.

      "When they settled this country 
      back then, there was no roads. 
      It was hard to get out, so they intermarried,"
      says Dennis Stacy, a 51-year-old coal miner
      and amateur genealogist who has filled a
      loose-leaf notebook with the laboriously
      traced blood lines of several local families.

      Stacy counts Fugate blood in his own veins. 
      "If you'll notice," he observes, tracing lines on his 
      family's chart, which lists his mother's and his 
      father's great grandfather as Henley Fugate, 
      "I'm kin to myself."

      The railroad didn't come through eastern Kentucky
      until the coal mines were developed around 1912,
      it took another 30 or 40 years to
      lay down roads along the local creeks.

      Martin and Elizabeth Fugate's blue children 
      multiplied in this natural isolation tank

      The marriage of one of their blue boys, 
      Zachariah, to his mother's sister triggered 
      the line of succession that would result in the 
      birth, more than 100 years later, of Benjy Stacy.

      When Benjy was born with purple skin, 
      his relatives told the perplexed doctors 
      about his great grandmother Luna Fugate. 

      One relative describes her as "blue all over," and 
      another calls Luna "the bluest woman I ever saw."

      Luna's father, Levy Fugate, was 
      one of Zachariah Fugate's sons. 

      Levy married a Ritchie girl and bought 
      200 acres of rolling land along Ball Creek. 

      The couple had eight children, including Luna.

      A fellow by the name of John E. Stacy spotted
      Luna at Sunday services of the Old Regular
      Baptist Church back before the century turned. 

      Stacy courted her, married her, and moved 
      over from Troublesome Creek to make
      a living in timber on her daddy's land.

      Luna has been dead nearly 20 years 
      now, but her widower survives. 

      John Stacy still lives on Lick Branch of Ball Creek. 
      His two room log cabin sits in the 
      middle of Laurel Fork Hollow. 
      Luna is buried at the top of the hollow. 
      Stacy's son has built a modern house next door, but the old 
      logger won't hear of leaving the cabin he built with timber 
      he personally cut and hewed for Luna and their 13 children.

      Stacy recalls that his father-in law, Levy Fugate,
      was "part of the family that showed blue
      All them old fellers way back then was blue. 
      One of 'em I remember seeing him when 
      I was just a boy Blue Anze, they called him. 
      Most of them old people went by that the blue Fugates. 
      It run in that generation who lived up and down Ball [Creek]."

      "They looked like anybody else,
      'cept they had the blue color," 
      Stacy says, sitting in a chair in his 
      plaid flannel shirt and suspenders, next to
      a cardboard box where a small black piglet,
      kept as a pet, is squealing for his bottle. 
      "I couldn't tell you what caused it."

      The only thing Stacy can't or won't 
      remember is that his wife Luna was blue. 

      When asked ahout it, he shakes his 
      head and stares steadfastly ahead. 

      It would be hard to doubt this gracious man except 
      that you can't find another person who knew 
      Luna who doesn't remember her as being blue.

      "The bluest Fugates I ever saw was Luna and her kin,"
      says Carrie Lee Kilburn, a nurse who works at
      the rural medical center called Homeplace Clinic. 

      "Luna was bluish all over. 
      Her lips were as dark as a bruise. 
      She was as blue a woman as I ever saw."

      Luna Stacy possessed the good health
      common to the blue people, bearing at
      least 13 children before she died at 84. 

      The clinic doctors only saw her a few times 
      in her life and never for anything serious.

      As coal mining and the railroads brought progress 
      to Kentucky, the blue Fugates started moving out 
      of their communities and marrying other people. 

      The strain of inherited blue began to disappear 
      as the recessive gene spread to families where 
      it was unlikely to be paired with a similar gene.

      Benjy Stacy is one of the last of the blue Fugates. 
      With Fugate blood on both his mother's and his 
      father's side, the boy could have received genes 
      for the enzyme deficiency from either direction. 

      Because the boy was intensely blue at birth but then 
      recovered his normal skin tones, Benjy is assumed 
      to have inherlted only one gene for the condition. 

      Such people tend to be very blue only at birth, 
      probably because newborns normally 
      have smaller amounts of diaphorase. 

      The enzyme eventually builds to normal 
      levels in most children and to almost normal 
      levels in those like Benjy, who carry one gene.

      Hilda Stacy (nee Godsey) is fiercely protective of her son... 
    • Heather Stimmel
      I just wanted to thank whomever posted this story! Talk about an interesting read! The lesson we all can learn from this story, is this: it doesn t matter who
      Message 2 of 3 , Feb 23, 2010
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        I just wanted to thank whomever posted this story! Talk about an interesting read! The lesson we all can learn from this story, is this: it doesn't matter who we are, how much money we have, where we live or what a person looks like on the outside. It's our heart condition that matters most. The "blue people" are just as "normal" as anyone else. They have close-knit families they enjoy spending quality time with, they are a self-sufficient people and with strong character, they go to church and are very protective of the people they care about. As I was reading the story, I began to wonder (the few times that they were seen by other people)... were they treated as lepers, with disgust and racism, the same way people are today? Afterall, that was the early-mid 1800's. According to the story, it didn't sound as if they ventured far from the isolated area in which they lived. Then, again, I'm sure if most people today encountered one of the blue people, they would run into stares, taunting, harassment and so-forth. So... for people today who say racism is gone- it's a thing of the past- I say, put your feet in the shoes of someone who experiences it to know exactly what it feels like. When I read the part of the story that says, there's only one, remaining, blue person living today... I must admit, I was a little sad. Will the blue people and their lives be something that dies off with the last, remaining family member... to be thrown away and forgotten, like so many other geneological histories (particularly, "different" ones)? I believe, learning of other's histories is a way for people to grow and take away lessons, knowledge and, hopefully, empathy for what people lived through. It is the beginning of bringing people closer together, and... if that could truly happen, then it's all worth it. Thanks, again, for such a wonderful story!!!

        "Our lives begin to end the day we
        become silent about things that matter."
        -Martin Luther King, Jr.

      • multiracialbookclub
        Hi Heather, Glad you enjoyed the post and thanks so much for sharing your insight and thoughts on this story of a people who are knownfor their extremely
        Message 3 of 3 , Feb 23, 2010
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          Hi Heather,

          Glad you enjoyed the post and thanks so much for sharing your 
          insight and thoughts on this story of 'a people' who are known
          for their extremely rare (and often misunderstood) phenotype.

          In case you are interested, listed below are a few links to more
          articles on '
          The Blue People' (found in the U.S. and worldwide).

          Thanks again and have a great day,

          -- AP

          ~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~


          The Blue People of Appalachia
          (The case of a close-knit clan of people with blue skin in the U.S.)



          The Blue Men of Lurgan 
          (The case of the two siblings in Ireland with blue skin in 1942)

          http://ije.oxfordjournals.org/cgi/content/full/dym140v1



          In Generation-Mixed@yahoogroups.com,
          Heather Stimmel <heather21230@...> wrote: 

          I just wanted to thank whomever posted this story! Talk about an interesting read! The lesson we all can learn from this story, is this: it doesn't matter who we are, how much money we have, where we live or what a person looks like on the outside. It's our heart condition that matters most. The "blue people" are just as "normal" as anyone else. They have close-knit families they enjoy spending quality time with, they are a self-sufficient people and with strong character, they go to church and are very protective of the people they care about. As I was reading the story, I began to wonder (the few times that they were seen by other people)... were they treated as lepers, with disgust and racism, the same way people are today? Afterall, that was the early-mid 1800's. According to the story, it didn't sound as if they ventured far from the isolated area in which they lived. Then, again, I'm sure if most people today encountered one of the blue people, they would run into stares, taunting, harassment and so-forth. So... for people today who say racism is gone- it's a thing of the past- I say, put your feet in the shoes of someone who experiences it to know exactly what it feels like. When I read the part of the story that says, there's only one, remaining, blue person living today... I must admit, I was a little sad. Will the blue people and their lives be something that dies off with the last, remaining family member... to be thrown away and forgotten, like so many other geneological histories (particularly, "different" ones)? I believe, learning of other's histories is a way for people to grow and take away lessons, knowledge and, hopefully, empathy for what people lived through. It is the beginning of bringing people closer together, and... if that could truly happen, then it's all worth it. Thanks, again, for such a wonderful story!!!

          "Our lives begin to end the day we 
          become silent about things that matter." 
          -Martin Luther King, Jr.

          ~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~


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