Blue People

When I stumbled across this article on the Blue Fugates of Troublesome Creek, Kentucky I just had to keep reading... So what if it was written in 1982?

Click the title for the link :-) I've quoted the more interesting bits...

In summary (if you can't be bothered to go read the whole article -which you should) it's the story of an Appalachian malady, an inquisitive doctor, and a paradoxical cure.

I'd heard about people who turn blue. They had argyria, a known complication of chronic use of colloidal silver, but this was something different, something genetic -and that intrigued me more.

It started with a baby boy, Benjamin Stacy.
"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 from where the creek still runs."

Born with "dark blue skin "It was almost purple,"

"raced by ambulance from the maternity ward... to a medical clinic... Two days of tests produced no explanation for skin the color of a bruised plum."

"A transfusion was being prepared when Benjamin'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," Alva Stacy, the boy's father, explained. "The doctors finally came to the conclusion that Benjamin's color was due to blood inherited from generations back." "

Benjamin lost his blue tint within a few weeks... 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 Benjamin's birth that they would crowd around the baby and try to make him cry...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-timer that "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... Martin Fugate's descendants had multiplied their recessive genes all over the Cumberland Plateau."

About one patient who walked in...
"She had been out in the cold and she was just blue! 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."

"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... Patrick and Rachel Ritchie walked in."

"After concluding that there was no evidence of heart disease... 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. You could tell how much it bothered them to be blue." "

"...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."

"...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... He found references to methemoglobinemia dating to the turn of the century"

"...discovered hereditary methemoglobinemia among Alaskan Eskimos and Indians... caused... 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...

"... concluded that the condition was inherited as a simple recessive trait... 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... it would appear most often in an inbred line."

"Cawein needed fresh blood to do an enzyme assay. He had to drive eight hours to search out the Ritchies, who lived in a tapped-out mining town... They took the doctor to see their uncle, who was blue, too. 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."

"...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."

"...methyleneblue sprang to 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."

"... and injected each of them with 100 milligrams of methylene blue."

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

"...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."


Here's how it all started...

"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... family lore has it that Martin himself was blue."

"...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... 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, "If you'll notice..."I'm kin to myself." "

"The railroad didn't come through eastern Kentucky until the coal mines were developed around 1912, and 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 Benjamin Stacy."

"...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."

"Benjamin Stacy is one of the last of the blue Fugates. Because the boy was intensely blue at birth but then recovered his normal skin tones, Benjamin is assumed to have inherlted only one gene for the condition."

All Things Agricultural & Pastoral, All Creatures Great & Small

Today I thought it would be nice to take myself along to the Royal Canterbury A&P Show, so that is precisely what I did. I packed a picnic and slathered on the sunscreen, though I really needn't have bothered (I knew I should have packed an umbrella or windbreaker at least).

Highlights: old farm machinery, tractor pull event, wood-chopping, whip-cracking and cows.

What I came across first was some working antiquated farm machinery. It was great because usually this kinda thing just delapidates in a shed or museum somewhere passing itself off for a boring unuseful piece of junk. Most were manufactured during the early part of last century, but there was one at least from 1895. Some ran on oil, others on gas but it was all smelly and great! So many of these ancient beasts congregated in one place attended by their equally aged sapien counterparts. All hissing, slapping, clacketting and spluttering, trying in vain to rattle free of their wooden constraints.

I walked around and got a feel for where everything was. Bumped into Steve all dolled up in a clown costume selling balloons.

Then it was time for the Super-Modified V8 Tractor Pull Competition. Great stuff!! My ears are still pounding. Two tractors drag off towing a massive sled and slabs of concrete. The very earth quaking and the air trembling with as they say "Lots of loud raw noise". Clods of earth flying up. Those vehicles are fast super fast. Oh-so entertaining. Then it started to rain, but somehow that just didn't matter.

For lunch I settled down on a hummock overlooking the Wood-chopping arena to watch the underhand (where the axeman stands on block whist chopping it in half) and standing block chop heats and finals. Also, the jill and jill woodsawing. I always like the wood-chopping events. I was not disappointed.

The sideshows and trade pavillions are pretty much like the Auckland Easter Show -filled with overpriced goods that you'll only use maybe once.

Had a good half hour of whip-cracking entertainment by the Manwaring family, before being lured into the food tent. I, propitiously sucumming at the precise moment when the acceptible ratio of precipitation became unbearably high. There I sampled a whitebait (ah, haven't had those lil' fishys for years, ?decades, maybe even) pattie open sandwich. Kaituna goat cheese from Gruff Junction (in fact I'm fixing to have some right now) and my first sip of Himalayan Goji juice (but at $85 a litre {yes, $85 that's no typo!} that's probably the last time I'll have it). It tasted very berryish, surprise, surprise for that is indeed what it is derived from (the Goji Berry) but with the sweetness and tartness somewhat akin to apple juice and then on the verge of swallowing it became all thick and chilli-like. I approve, yes, it was much to my liking. Such an exoticity my tastebuds have never before encountered.

Then I traipsed round the cattle stalls. It was good to be able to see what different breeds look like in the flesh (as opposed to just reading about them on the net). I love bovine eyes. Saw an alpaca getting shorn/shaved/having a full body hair-cut. At about one-fifth the size of the largest male, some of the banty roosters' crows were so twee. The ducks just wanted outa there.

Then the clouds that had been re-brewing all afternoon cracked open just when I had to find the bus to take me home, but I missed he last bus, much to my dismay, so I resigned myself to trudging along in the gutter, in the rain.

After all that dirt, hay, cows, gas, oil, dampness, my hair now smells really gross, if I may say so myself. And I just did. So I'll probably wash it tomorrow.