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Finding The Famous Painting of the Blue People of Kentucky

Most stories about the blue people of Kentucky include an eerie, compelling drawing of a family, with the stark faces of 5 of the 9 members a striking bluish-gray, due to an inherited disease. Most stories also borrow heavily from a terrific article by Cathy Frost from Science 82, a long-gone magazine that I quite liked. Frost’s piece, “The Blue People of Troublesome Creek,” is usually credited, but the painting not, or misattributed to ABC News, various newspapers, or simply deemed “unknown.”

The artist, Walt Spitzmiller
The artist, Walt Spitzmiller

The artist Walt Spitzmiller in fact painted the portrait of the Fugate family (see Walt Spitzmiller Fine Art). A Science 82 editor asked him in 1982 to draw a family, who lived in rural Kentucky, in which the father and some of the children had blue skin. “That’s all I knew about it. I did research on the period they talked about and took old photos and put them together. I added the hunting dog in the lower right, the rooster, that type of thing to add authenticity,” Walt told me.

A BRIEF HISTORY OF THE FUGATES
The blue people of Troublesome Creek had methemoglobinemia, a metabolic condition affecting hemoglobin, the four-part protein that carries oxygen bound to an iron atom at each subunit’s core. Like my recent post about the deaf community on Martha’s Vineyard, it is a tale of an autosomal recessive disease that has dissipated over time as the descendants of the original carrier couple left home. The community in Chilmark so embraced the hearing impaired among them that everyone used their own local form of sign language. The Kentucky families did not experience such acceptance, according to the sparse literature on them. Their blue hue was a genetic badge of inbreeding.

I’ve written about the blue people in nearly every edition of my human genetics textbook. Because part of the blue people tale is about plagiarizing, I’ll plagiarize myself:

“A rare but very noticeable condition of abnormal hemoglobin affects the “blue people of Troublesome Creek”. Seven generations ago, in 1820, a French orphan named Martin Fugate who settled in this area of Kentucky brought in an autosomal recessive gene that causes methemoglobinemia. Martin’s mutation was in the CYP5R3 gene, which encodes an enzyme (cytochrome b5 methemoglobin reductase) that normally catalyzes a reaction that converts a type of hemoglobin with poor oxygen affinity, methemoglobin, back into normal hemoglobin by adding an electron. Martin was a heterozygote but still slightly bluish. His wife, Elizabeth Smith, was also a carrier for this very rare disease, and four of their seven children were blue. After extensive inbreeding in the isolated community—their son married his aunt, for example—a large pedigree of “blue people” of both sexes arose.

Hemoglobin
Hemoglobin

In “blue person disease,” excess oxygen-poor hemoglobin causes a dark blue complexion. Carriers may have bluish lips and fingernails at birth, which usually lighten. Treatment is simple: A tablet of methylene blue, a commonly used dye adds the electron back to methemoglobin, converting it to normal hemoglobin. In most members of the Fugate family, blueness was the only symptom. Normally, less than 1 percent of hemoglobin molecules are the methemoglobin form, which binds less oxygen. The Fugates had 10 to 20 percent in this form. People with the inherited condition who have more than 20 percent methemoglobin may suffer seizures, heart failure, and even death.”

Once young people began leaving the hollows of Kentucky, disease incidence there plummeted. Methemoglobinemia is also seen in Alaska and Algeria, and among Navajo Indians.

TRACKING DOWN THE PORTRAIT
Writing a science textbook entails much more than explaining; there’s also art, photos, the pedagogical elements like questions and summaries, and layout to consider. I’m encouraged to find freebie photos in a “digital asset library” the publisher maintains, but something as specific as the blue Fugates wouldn’t be among the stock photos.

I’d requested Walt’s image, which I’d seen everywhere, for past editions, with no luck — McGraw-Hill required permission from the copyright holder, and photo editors inevitably ended up with newspapers or TV stations that had run the image and claimed it as their own. That wasn’t good enough.

My day job is writing textbooks.
My day job is writing textbooks.

I’m working on the 12th edition now, so thought I’d try again, dragging the famous image from a Google search into the digital publishing platform that has replaced dead trees, in the part of the chapter on mutation about hemoglobin. A few weeks later, I heard from a photo editor, one I hadn’t worked with before, Molly Berke. I’d left a note that the image, grainy and faded, appeared everywhere, but I’d had trouble getting permission to use it. Molly, an artist herself, instantly recognized the talent behind the painting and, sympathetic to the forgotten artist, was determined to track it down. And she did.

The only assistance I could provide was to explain that Science 82 was not Science magazine from 1982, but a stand-alone magazine. From there, googling, Molly finally found an obscure source that had a name. Bingo! “I do photography, paint, and collect vintage photographs. I just figured this guy, as good as he is, had his own studio. So, I googled him. He does!” she emailed me.

Walt Spitzmiller answered her e-mail right away and they spoke. Then he offered to snail mail the “only original slide of the painting on earth,” Molly told me. She helped him find someone to replicate the slide and create a digital image, which opens up this post. “I’m old school, having done photo research since the late ‘80s, and I know what that slide was worth, not just monetarily, but in legacy to his family because of his artistry. A badly scanned copy has proliferated on the Internet, stolen and reused without permission countless times. The real image is so gorgeous, a feast for the eyes. Photo research is cool!”

Walt graciously gave permission for me to use the image here, knowing it would be zapped around the Internet to join its blurry echoes, and I’ve ordered a print for myself.

Screen Shot 2016-09-20 at 3.26.42 PMTHE BIGGER PICTURE
I like to end DNA Science posts with general musings and opinion, and a few things come to mind with the story of the blue people of Kentucky.

1. On plagiarism and fair use. The publishing world has turned upside down, and while I still earn most of my income from textbooks (I’ve also written an inexpensive, short human genetics book for a different publisher, second edition coming in December), much of my shorter work is republished with my earning little or nothing, websites not even letting me know they’ve used my work. I’ve gotten used to it. But photographs and original paintings are another matter. They’re more lasting than blog posts.

2. Editors are important. I’ve learned nearly all I know about writing from copyeditors. Developmental editors guide book-long projects. Magazine, journal, and newspaper (remember those?) editors help a writer get to the point and make it well. Art editors manage the dozens of illustrations that go into a science textbook. And photo editors sometimes solve decades-long mysteries, as did Molly Berke. These talented professionals, editors, should not be replaced with digital image libraries, databases, and worst of all, the aggregator algorithms that butcher science news on a daily basis, regurgitating news releases with little nuance or context.

3. On the prevalence of rare recessive diseases. Certain recessive diseases persist because carrier status protects against a different disease: sickle cell protecting against malaria is the classic example of this balancing selection.

facebookMight a new mechanism of balancing selection be emerging, as the ease of travel disperses carriers of the same disease, while social media unites them? The blue people of Kentucky and the deaf of Martha’s Vineyard illustrate the dilution of genetic disease. Yet Facebook groups have enabled the meeting of many people who have mutations in the same gene. This is wonderful, but it could facilitate transmission of the disease when carriers have children together. A sad recent example is the “Fault in Their Stars” couple, Dalton and Katie Prager, who hadn’t had children, but met on Facebook at age 18 and married at 20. He died at 25 a few days ago, from complications following a double lung transplant for cystic fibrosis that had kept him from staying with Katie in hospice care back home in, ironically, Kentucky.

methylene blue treats methemoglobinemia
Methylene blue treats methemoglobinemia

4. On the importance of phenotype, not just genotype. The straightforward treatment for methemoglobinemia came from Madison Cawein, a young hematologist who drove hours from the University of Kentucky in Lexington to visit the blue people in the hollows of Appalachia in the early 1960s. He sampled their blood, recognized the enzyme deficiency, and hypothesized that the harmless dye methylene blue might provide the missing electrons to restore the ferrous state of iron in the hemoglobin molecules. The lesson: Sometimes a treatment arises from understanding the biochemistry of an inherited disease – not just sequencing DNA. I’ll return to that theme soon.

Discussion
  1. […] Finding The Famous Painting of the Blue People of Kentucky – My day job is writing textbooks … for parents-to-be at CareNet Medical Group in Schenectady, NY and teaches "Genethics" an online course for master’s degree students at the Alden March … […]

  2. This is so interesting!! Had never heard of them. My daughter has been reading a book about the “Blue People of Kentucky”!! It is obviously a “medical condition” that has probably become almost gone with time. Anyway, what information you can send me about them from time to time would be greatly appreciated. Thanks

    1. Martin Fugate is my 6 gr grandfather. i waa surprised as anyone to learn of them and this year to discover them in my Kentucky family tree!

  3. Hey, Dr. Lewis, I just wanted to let you know that your link to the Science 82 article is no good, as it seems to have been moved to another URL or deleted by the host. I tried to find an older version of it on Wayback Machine, but the earliest capture is June 2020, after its removal. I’d really like to read it, and it should be preserved, so if you have any way of finding or publishing another online copy, that would be great. You’re a remarkable writer, I really enjoyed the read.

    1. Thanks, John and Ricki. The compelling Science82 article is a nice complement to Ricki’s post. Isn’t science intriguing?!

  4. I love this painting. You mentioned you’d ordered a print for yourself, but I’m wondering if prints are actually available for purchase somewhere? I’m a bit fussy when it comes to make sure an artist is credited and compensated for his work but I’ve love to hang it on my wall.

  5. I discovered through DNA matches that I have a cousin who is a related to the blue Fugates and apparently one of my uncles from Ireland in the 1800s married a Fugate .They must have been hillbillies at the time, I also discovered quite to my surprise that I have Romani and Kale ancestors and also European royalty going back to the Plantagenets, At the end of the day everyone on the Earth is connected one way or another.By doing a DNA test you can really discover who you are. I always knew that I was half Irish with some Indian and African as that was my most recent history. I didn´t however imagine that i was also Scandanavian , German Dutch, Russian, Native American, Maya and many many more. Noone on the planet is 100% of one country as families tended to mix over thousands and thousands of years.

  6. Thank you, Dr. Lewis, for your very informative blog. I recently saw a fictional book titled, The Book Woman of Troublesome Creek, in my selection list from BookBub. My parents grew up in the Appalachian mountains of Kentucky. I dimly remember my grandfather once telling us about some people on another creek who had blue skin. I didn’t believe him because my brother had just asked him if he’d ever seen an alien. This was some 62+ years ago. When I was looking for information on this topic, I found your really interesting article. Now I need to find how close Troublesome Creek is to Smith Creek. Again, thank you! By-the-by, I grew up in the desert of Arizona.

  7. I love the book “The Book Woman of Troublesome Creek”. It opened my eyes to methemoglobinemia. I am a retired nurse and had not heard of it before.

    The book points out discrimination against “coloreds” both black people and blue people. Quite a shock to me, but the book is well written and I am enjoying the historical fiction very much. Well worth a read!

  8. Wow the science here is exact, I’ve done all the research my self… I was drawn tto these people for 2 or more reasons.. I personally have methemoglobinemia.. (blue blood)… But like it’s more deep brown (or purplish to me, I personally see a bit of purple).. especially when I was younger.. that stuff Methelyn Blue, the “cure”.. not sure how it would work and sounds way ahead of its time, especially when it was made…also how it’s related to cyanide aslo ahead of its time… “Cyan” is Blue, so in otherwords cyannide is “Bluenide”.. but also how B12 is related to cyanide, and then how much B12 and other b complex vitamins people are taking apart from their natural diet…

    It got me thinking about chemical dependency, as well as the location dependency of your diet, and how chemical dependency can ultimately affect your chemical reliance to certain things or even people in the case of monopolizing health through said dependencies if said dependencies can be controlled.

    Second reason is my family Name, and our motto “without stain”…so I took to taking it literally, as if there was a blue family that was naturally blue without stain, and it just so happens that there was..”the Blue fugates”

    To me most of the research done on this family and their blood condition seems to be ignorant and looks at the family as if there is a condition where their blood was not able to carry as much oxygen efficiently..

    The thing about that is I have the blue blood condition and my blood happens to carry 110% of the normal amount of oxygen per blood capita, ultimately suggesting that the science behind the blood condition is wrong, otherwise my blood oxygen level would be sitting at 75 to 80 at a minimum, any lower is definitely not optimal.

    It is also accounted for in Mythos all over the Eastern side of the world, blue skinned characters include Osiris, Loki, Shiva, Hades.. to me this suggests that blue people did exist based on they are accounted for by several different people, most of the stories seem to be overembellished and dissimilar in so many ways, but one thing does remain in all these ancient Mythos is that there was a blue skinned people that existed in a so-called underworld, to me this underworld was the Americas or the western side of the planet from the eastern.

    Seeing this and knowing that I put about a year investigating into these blue people learning about DNA and it’s particulars, and that this Blood condition can be isolated to Native American people, which could negate the Bering strait land crossing as to being the other way around where blue people had made their way from the Americas to both European/African and the Asiatic continents..

    India happens to have the clearest records and indications of who these people were apart from where their actual location was and they’re regards to a place known as “Agartha”, it is in the tales of a gartha that you hear of electrical trans systems as well as fiber optic capabilities…

    To me this makes sense why we would live in a Romanesque empire that worships which seems to be an alternative history, and keeps this alternative history of blue people and the Free World from us in order to keep us all a slaves…

    I don’t really believe in aliens but I think that there is still an advanced race of humans that do have blue skin and perhaps even pink skins too.. who are in hiding currently due to a genocide of their people and you know unwary set of human beings who are set to kill anything they don’t understand.

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