Blue Fugates

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Fugates
CountryUnited States of America
Current regionKentucky
Founded1820
FounderMartin Fugate
Elizabeth Smith
Final headBenjamin Stacy
MembersBenjamin Stacy

The Fugates, a family who 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[edit]

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]

The disorder can cause heart abnormalities and seizures if the amount of methemoglobin in the blood rises above 20%. But the ranges between 10 and 20 percent can cause blue skin without other symptoms. Most of the Fugates lived long and healthy lives. The "bluest" of the blue Fugates, Luna Stacey, had 13 children and lived to age 84.[6]

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][7] 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.[8] He eventually published his research in the Archives of Internal Medicine in 1964.[9]

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.[7]

In popular culture[edit]

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.

In 2021, the novel Blue-Skinned Gods by S. J. Sindu references a family from Kentucky with methemoglobinemia but doesn't use the surname Fugate.

A reference to The Huntsville Subgroup is made in the American version of television sitcom Shameless when Kevin Ball (played by Steve Howey) discovers that he may have ancestors from that group.[10]

See also[edit]

References[edit]

  1. ^ "Blue-skinned family baffled science for 150 years". MSN. 24 February 2012. Archived from the original on January 22, 2013. Retrieved 10 May 2013.
  2. ^ a b Trost, Cathy. "The Blue People of Troublesome Creek" (PDF). Archived (PDF) from the original on 2015-09-27. Science 82, November, 1982
  3. ^ Adams, Cecil (1998-07-24). "Is there really a race of blue people?". The Straight Dope. Retrieved 2021-04-22.
  4. ^ "Rare disease turns mountaineers blue". Phoenix, Arizona: Arizona Republic. November 7, 1974. p. 12. Retrieved 3 April 2019 – via Newspapers.com.
  5. ^ a b c Susan Donaldson James (February 22, 2012). "Fugates of Kentucky: Skin Bluer than Lake Louise". ABC News. Retrieved 2014-10-04.
  6. ^ "Fugates of Kentucky: Skin Bluer than Lake Louise". ABC News. Retrieved 2022-06-03.
  7. ^ a b Susan Donaldson James (March 8, 2012). "Blue People Look for Genetic Connection to Kentucky Fugates". ABC News. Retrieved 2014-10-04.
  8. ^ 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.
  9. ^ Cawein, M; Behlen Ch, 2nd; Lappat, E. J.; Cohn, J. E. (1964). "Hereditary Diaphorase Deficiency and Methemoglobinemia". Archives of Internal Medicine. 113 (4): 578–85. doi:10.1001/archinte.1964.00280100086014. PMID 14109019.
  10. ^ "God Bless Her Rotting Soul". Shameless. Season 8. Episode 3. 19 November 2017. Showtime. You have a very rare chromosomal pattern, Mr. Ball. You are, more specifically, one in two billion. Were you aware you are from the Huntsville subgroup?