Blue Fugates
| Fugates, | |
|---|---|
| Country | United States of America |
| Founder | Martin Fugate Elizabeth Smith |
| Final head | Benjamin Stacy |
| Members | Benjamin Stacy |
The Fugates, a family that lived in the hills of Kentucky, commonly known as the "Blue Fugates"[1] or the "Blue People of Kentucky", are notable for having been carriers of a genetic trait that led to the blood disorder methemoglobinemia, which causes the appearance of blue-tinged skin.
Ancestry
Martin Fugate and Elizabeth Smith[2] who had married and settled near Hazard, Kentucky, around 1820, were both carriers of the recessive methemoglobinemia (met-H) gene. As a result, four of their seven children exhibited blue skin, and continued progenation within the very limited local gene pool ensured that many descendants of the Fugates were born with met-H.[3][4][5]
Descendants with the gene continued to live in the areas around Troublesome Creek and Ball Creek into the 20th century, eventually coming to the attention of the nurse Ruth Pendergrass and the hematologist Madison Cawein III, who made a detailed study of their condition and ancestry.[2][6] He found that a report from 1960 by a public health doctor, E. M. Scott, who published in the Journal of Clinical Investigation his research on the phenomenon among native Alaskans, based on the theory that a deficiency of the enzyme diaphorase is the cause of the oxygen deficiency in the red blood cells, causing the blood to appear brown, which in turn made the skin of those affected appear blue.[5]
Cawein treated the family with methylene blue, which eased their symptoms and reduced the blue coloring of their skin.[7] He eventually published his research in the Archives of Internal Medicine in 1964.[8]
Benjamin Stacy, born in 1975, is the last known descendant of the Fugates to have been born exhibiting the characteristic blue color of the disorder, though he quickly lost his blue skin tone, exhibiting only blue tinges on his lips and fingertips if he was cold, or agitated.[5]
It has been speculated that some other Americans who inherited methemoglobinemia may also have had Fugate ancestors, but searches for direct links have so far proved inconclusive.[6]
In popular culture
In 2019, the novel The Book Woman of Troublesome Creek, by Kim Michele Richardson, described a fictional version of the Fugate family during the Great Depression.
References
- ^ "Blue-skinned family baffled science for 150 years". MSN. 24 February 2012. Archived from the original on January 22, 2013. Retrieved 10 May 2013.
- ^ a b Cathy Trost. "The Blue People of Troublesome Creek". Science 82, November, 1982
- ^ Straight Dope article on the Fugates of Appalachia, an extended family of blue-skinned people
- ^ "Rare disease turns mountaineers blue". Phoenix, Arizona: Arizona Republic. November 7, 1974. p. 12. Retrieved 3 April 2019 – via Newspapers.com.
- ^ a b c Susan Donaldson James (February 22, 2012). "Fugates of Kentucky: Skin Bluer than Lake Louise". ABC News. Retrieved 2014-10-04.
- ^ a b Susan Donaldson James (March 8, 2012). "Blue People Look for Genetic Connection to Kentucky Fugates". ABC News. Retrieved 2014-10-04.
- ^ Lyle E. Davis (January 19, 2006). "The Blue People of Troublesome Creek". The Paper. Archived from the original on July 19, 2019. Retrieved November 4, 2014.
- ^ Cawein, M; Behlen Ch, 2nd; Lappat, E. J.; Cohn, J. E. (1964). "Hereditary Diaphorase Deficiency and Methemoglobinemia". Archives of Internal Medicine. 113: 578–85. doi:10.1001/archinte.1964.00280100086014. PMID 14109019.
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