Arizona Republic from Phoenix, Arizona • Page 12

REPUBLIC CITY C-8 The Arizona Republic 0 Nov. 7, H74 Rare disease turns mountaineers blue 551 El' 1 M'ABAVA 7- i Just taste Windsor-and you may never go back to your ifcual whisky. Windsor is the only Canadian made exclu-sivelywith Canadian grain, with water from glacier-fed springs, and aged in the clear dry air of the Canadian Rockies. Very remarkably priced. She said her lips and skin do not become bluish unless she is sick or tired.

Her father said he was always able to tell if his daughter 'Is coming down sick or ill because her lips will turn blue." Rachel Ritchie Patrick, an elderly woman who lives near Dwarf In the little coal mining town of Hardburly, said she "shows blue" when she takes "some of these medicines, like aspirins." She and her sister, Rosa Ritchie Day, said their brother and other Ritchie relatives "show blue" when they are angry or have too much to drink. When a film crew arrived in Dwarf a while back to film a story on the unusual blood condition, Mrs. Patrick refused to be photographed. "I got enough to contend with; I don't want any television," she said. She said the blue condition has never bothered her: "I never paid it no mind." Dr.

Madison Cawein, the senior author of the medical study on the "blue" families, said the color of several of the people he saw was similar to that of "an oxford cloth blue shirt." Family members describe the color variously as gun metal" or "blue as fish hooks" or "blue as indigo." But the coloring Is not always that noticeable. Farley tells about the film crew that arrived in the Troublesome Creek area. When they located one person who carried the trait, "the director looked at her and said, Rut she isn't blue Without a word, the crew packed up, went back to Lexington, and flew off." The "Blue Fugate" traits have spawned storytelling and mountain mythmaking. "Big Man" Fugate said that he was told the blue originated because the family was Swedish. Lee Fugate says, ''My grandfather always said there was a blue race of people in Europe, but I've never read that in no history book." The "blue people" have had some problems that are more than cosmetic: Tom Combs and others were rejected for military service because Army doctors thought their color indicated a serious physical problem.

"Big Man" Fugate said a cousin was the object of a flurry of tests when she was hospitalized with 8n illness. When she realized what the doctors were after, she told them, "If you're doctoring for that blue, you can stop now. I've had it all my life." The bluish complexion caused by methemoglobinemia can be readily treated by the drug "methylene blue," which Behelen describes as "a simple dye which causes a chemical reaction in the blood." Behlen said doctors gave methylene blue pills to people affected by the condition when they were studiyng the illness more than 10 years ago. They said some of the patients were "elated to be white for the first time." However, taking methylene blue, also turns a patient's urine blue. Behlen said doctors have explained to the "blue people" how the drug works and telling them they're getting rid of the blue with the urine.

While not technically correct, the explanation seemed to satisfy 3 4L a. tVi 4 21 1974 thru Sunday, Reserved ed in the mountains as being either black people or as being hexed," Behlen said. "Some of the blue people wouldn't go farther than Dwarf to a store. Some of them said to us, 'We don't go to town; people think we look Ruth Pendergrass, a Perry County health nurse, said she knew about the "blue" families as early as 1948. She said she asked doctors at the University of Kentucky to do studies to determine why they were blue.

She said there was a rumor that the families had Negro ancestry. "I never believed that," she explained. "It's not a mountain custom for blacks and whites to intermarry. I was curious. I wanted to know what made them blue.

"They weren't sick; it was just the way they look. They're normal people; they're good people." Many members of the "blue" families have com-, plexions that differ very little in color from those of other Caucasians, and family members who "show blue" vary widely in the amount of coloring. Zach "Big Man" Fugate reminisced about one of his relatives as he sat along Ball Creek recently. "My great uncle, John Fugate, he was right blue," he said. Another relative, he said, was "just as blue as you can get." Fugate said he doesn't "show blue" except in cold weather or when he takes a medicine for his blood circulation.

The amount of coloring varies with physical condition and age. Carol Ann Fugate Sloane's husband said his wife "has a bluish complexion." He said her coloring was much more pronounced in the first five years of her life, when she was mistakenly diagnosed as a "blue baby." But the Fugate family trait is not related to the congenial heart or lung defects which cause people to be termed "blue babies." "These doctors just laugh at you when you tell them your family's marked. Doctors don't know about marks," said Lee Fugate. Carols' father. (Both of Carol Ann's parents come from Fugate families).

Carol needlessly spent weeks in a hospital as an infant while doctors tried vainly to determine the heart condition they thought was making her blue. Carol Ann, a young teacher who lives near her parents on Clear Creek, said she still has to wear polish to hide her blue fingernails. Los Angeles Times Service DWARF, Ky. Luke Combs, a man who lives in the mountains around Troublesome Creek, took his ailing wife to the University of Kentucky Hospital in Lexington in 1958. But it was Combs the doctors noticed.

"Luke was just as blue as Lake Louise on a cool summer day," said Dr. Charles H. Behlen II, one of he physicians in attendance. said to him, 'Luke, there's something going on here. Let's find out what it It was organized medicine's first known brush with Appalachia's "blue people." It led to a comprehensive study of the rare genetic trait.

A few of the "blue people" still live near Troublesome Creek and its tributaries in the isolated mountain hollows oi southeastern Kentucky. Doctors say members of the affected families Sometimes called "The Blue Fugates" or "The Blue Combses" have a rare hereditary blood disease called methemoglobinemia. trait illustrates a a victim's coloring, but it is other relatives in the ex-harmless. train illustrates a wider Appalachian phenomenon: Genetic illnesses are apparently more common in the mountains, according to doctors, because of the intermarriage of first cousins and other realtives in the extreme isolation of the area in the 19th and early 20th centuries. Some of the hereditary illnesses and syndromes found in eastern 'Kentucky are much more serious than the coloring.

They include: A lack of sweat glands, a birdlike face, lack of immunity to major diseases and blindness. With the improvement of roads and other amenities, the Intermarriage seems to be declining, which could lead to a reduction in genetic illnesses. "But back in the old days there was no one to marry except your cousin who lived up the hollow," said a woman from the "Blue Filiate" family that lives on Ball Creek. Doctors have discovered many of the genetic illnesses only recently. They say there are probably other mountain families with genetic illnesses that have never been discovered" or studied by modern medicine.

"It's a very fertile field for inquiry," said Dr. Claude H. Farley, a Kentucky medical scholar. "These people were treat- The smoothest whisky ever to come out of Canada. Dorothce Poison whets the appetite every Wednesday in The Republic's Food Section.

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