The Blue People of Troublesome Creek

Hematologist Madison Cawein, who traveled back and forth, from Lexington to Hazard, KY, explored rumors and legends of the blue people of the hills and hollows. He began his search at the local clinic and from there, explored all over the hills and creeks close by. The trait he searched for was the rumored blue people—no, not smurfs. These people were thought “to have a heart disease, lung disorder, or that simply their blood was closer to their skin.” After concluding that neither of these hypotheses were correct, Cawein did more investigation involving many blood tests. He originally suspected methemoglobinemia, a rare hereditary blood disorder that results in excess methemoglobin—a blue nonfunctional form or the red hemoglobin that carries oxygen to the body. Blue is the color of oxygen-depleted blood cells that you see in veins below the skin. Several causes of this could be abnormal hemoglobin formation, enzyme deficiency, or too much of a particular drug or vitamin. First, Cawein tested for abnormal or a deficiency of hemoglobin; the results were negative. He did some research that led to his new hypothesis of a diaphorase deficiency. Diaphorase is used by the body to break down methemoglobin into hemoglobin; if this is not present, methemoglobin numbers rise disproportionately in the blood giving it a blue tint. This doesn’t mean, however, that the individual is deprived of oxygen but strictly is a condition of pigment.

Cawein further explored the reason for this. He found it a recessive trait and is typically suffered from excessive inbreeding, or contiguous mating. The family tree he studied was that of Martin Fugate, an immigrant orphan from France. The pedigree that Martin made was extensive and displayed said inbreeding, which was common in the isolated area as there was few people in the area to begin with. Three families were mentioned: Fugates, Stacys, and Ritchies. It was only natural for a member of the family to marry “the girl next door” since choice was limited even if they had the same name…

To attempt to fix the embarrassing condition Cawein made 100 mg pills made of methylene blue which would hopefully reverse the blue effects, if only temporary. So the pill must be taken daily, as the methylene blue would be peed out. The study ended here. Cawein then focused on creating a pedigree, a chart displaying the family tree and a trait that is passed down. It is used to help trace back the origination of a gene, in this case Martin Fugate, the father of the Fugate clan, married Elizabeth Smith, a carrier of the trait. Martin was said to be blue thus passing the gene onto his seven children, four of which were blue. These children went on to pass the trait to the other two families as well as keep it in their own, marrying cousins. Once World Ward II rolled around the family began to spread out and marry others. Evidence of the blue gene would soon be lost to carriers of the trait and would be simply coincidence if they married another carrier unless they were distant family. Odds of that would be slim and highly unlikely.

This article interested me because I have family that live in Appalachia very close to Kentucky. I’ve heard stories about blue people but never really understood it or tried to do more research on it. I found it interesting because although the trait dispersed and the last remaining direct Fugate descendant died, the trait can still emerge. This trait was also found in Eskimos and Indians, which I also found very interesting.

By: Kara Ward

Cites:

• Cawein, Madison, Behlen, Charles H., Lappat, E. J., and Cohn, Jerome. Hereditary Diaphorase Deficiency and Methemoglobinemia. 1964. Archives of Internal Medicine. http://archinte.ama-assn.org/cgi/content/summary/113/4/578.
• Trost, Cathy. The Blue People of Troublesome Creek. 1982. Science Magazine, 82. http://www.indiana.edu/~oso/lessons/Blues/TheBlues.htm.
• http://topics.info.com/Who-were-the-Blue-People-in-Appalachia_171