Blue Fugates: Difference between revisions
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{{Short description|American family known for having blue skin}} |
{{Short description|American family known for having blue skin}} |
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{{Infobox family |
{{Infobox family |
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|name = |
|name = Fugates |
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|country = United States of America |
|country = United States of America |
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|region = West Virginia |
|region = West Virginia |
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The '''Fugates''', a family living in the hills of [[West Virginia]] starting in the 19th century, were commonly known |
The '''Fugates''', a family living in the hills of [[West Virginia]] starting in the 19th century, were commonly known as the "'''Blue Fugates'''"<ref>{{Cite web |title=Blue-skinned family baffled science for 150 years |url=http://now.msn.com/blue-skinned-family-baffled-science-for-150-years |work=MSN |access-date=10 May 2013 |date=24 February 2012 |url-status=dead |archive-url=https://web.archive.org/web/20130122055829/http://now.msn.com/blue-skinned-family-baffled-science-for-150-years |archive-date=January 22, 2013}}</ref> or the "'''Blue People of West Virginia'''". They are known for being of a [[Introduction to genetics|genetic trait]] that led to the blood disorder [[methemoglobinemia]], causing the skin to appear blue. |
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== Ancestry == |
== Ancestry == |
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[[File:The_Fugate_family.png|thumb|right|The Fugate Family c. 1820]] |
[[File:The_Fugate_family.png|thumb|right|The Fugate Family c. 1820]] |
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Martin Fugate and Elizabeth Smith,<ref name="Trost">{{Cite web|first=Cathy|last=Trost|title=The Blue People of Troublesome Creek|url=https://academic.csuohio.edu/neuendorf_ka/com370/370_blue_people.pdf|url-status=live| |
Martin Fugate and Elizabeth Smith,<ref name="Trost">{{Cite web |first=Cathy |last=Trost |title=The Blue People of Troublesome Creek |url=https://academic.csuohio.edu/neuendorf_ka/com370/370_blue_people.pdf |url-status=live |archive-url=https://web.archive.org/web/20150927104846/https://academic.csuohio.edu/neuendorf_ka/com370/370_blue_people.pdf |archive-date=September 27, 2015 |work=Science 82 |date=November 1982}}</ref> who had married and settled near [[Hazard, Kentucky]], in around 1820, were both carriers of the [[Dominance (genetics)|recessive]] methemoglobinemia (met-H) gene. As a result, four of their seven children exhibited blue skin, and continued reproduction within the limited local gene pool ensured that many descendants of the Fugates were born with met-H.<ref>{{Cite web |last=Adams |first=Cecil |date=July 24, 1998 |title=Is there really a race of blue people?|url=https://www.straightdope.com/21342421/is-there-really-a-race-of-blue-people |access-date=April 22, 2021 |website=The Straight Dope |language=en}}</ref><ref>{{Cite web |url=https://www.newspapers.com/clip/28428591/arizona_republic/ |title=Rare disease turns mountaineers blue |publisher=Arizona Republic |location=Phoenix, Arizona |date=November 7, 1974 |page= 12 |via=Newspapers.com |access-date=April 3, 2019}}</ref><ref name=ABCLakeLouise>{{Cite news |url=https://abcnews.go.com/Health/blue-skinned-people-kentucky-reveal-todays-genetic-lesson/story?id=15759819 |title=Fugates of Kentucky: Skin Bluer than Lake Louise |work=[[ABC News]] |first=Susan Donaldson |last=James |access-date=October 4, 2014 |date=February 22, 2012}}</ref> |
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The disorder can cause heart abnormalities and seizures if the amount of methemoglobin in the blood exceeds 20 percent, but at levels between 10 and 20 percent it can cause blue skin without other symptoms. Most of the Fugates lived long and healthy lives. The "bluest" of the blue Fugates, Luna Stacy, had 13 children and lived to age 84.<ref>{{Cite |
The disorder can cause heart abnormalities and seizures if the amount of methemoglobin in the blood exceeds 20 percent, but at levels between 10 and 20 percent it can cause blue skin without other symptoms. Most of the Fugates lived long and healthy lives. The "bluest" of the blue Fugates, Luna Stacy, had 13 children and lived to age 84.<ref>{{Cite news |title=Fugates of Kentucky: Skin Bluer than Lake Louise |url=https://abcnews.go.com/Health/blue-skinned-people-kentucky-reveal-todays-genetic-lesson/story?id=15759819 |access-date=June 3, 2022 |work=[[ABC News]] |language=en}}</ref> |
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Descendants with the gene continued to live near [[Troublesome Creek (North Fork Kentucky River tributary)|Troublesome Creek]] and [[Ball Creek]] into the 20th century. They eventually came to the attention of the hematologist Madison Cawein III, who with the assistance of the nurse Ruth Pendergrass made a detailed study of their condition and ancestry.<ref name="Trost"/><ref name="ABC2013">{{Cite |
Descendants with the gene continued to live near [[Troublesome Creek (North Fork Kentucky River tributary)|Troublesome Creek]] and [[Ball Creek]] into the 20th century. They eventually came to the attention of the hematologist Madison Cawein III, who with the assistance of the nurse Ruth Pendergrass made a detailed study of their condition and ancestry.<ref name="Trost"/><ref name="ABC2013">{{Cite news |url=https://abcnews.go.com/Health/blue-skinned-people-genetic-connection-kentucky-fugates/story?id=15871929 |title=Blue People Look for Genetic Connection to Kentucky Fugates |work=[[ABC News]] |date=March 8, 2012 |first=Susan Donaldson |last=James |access-date=October 4, 2014}}</ref> Based on a report published in the ''[[Journal of Clinical Investigation]]'' in 1960 by a public health physician named E. M. Scott, who had studied a similar phenomenon among native Alaskans, Cawain concluded that a deficiency of the [[enzyme]] [[Cytochrome b5 reductase|diaphorase]] resulted in an oxygen deficiency in the red blood cells, causing the blood to appear brown, which in turn made the skin of those affected appear blue.<ref name="ABCLakeLouise" /> He treated the family with [[methylene blue]], which eased their symptoms and reduced the blue coloring of their skin.<ref>{{cite web |url=http://www.thecommunitypaper.com/2006/01_19_06/ |title=The Blue People of Troublesome Creek |publisher=The Paper |date=January 19, 2006 |first=Lyle E. |last=Davis |access-date=November 4, 2014 |archive-url=https://web.archive.org/web/20190719220630/http://www.thecommunitypaper.com/2006/01_19_06/ |archive-date=July 19, 2019 |url-status=dead}}</ref> His findings were published in the ''[[JAMA Internal Medicine|Archives of Internal Medicine]]'' in 1964.<ref>{{Cite journal |
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|pmid=14109019 |year=1964 |last1=Cawein |first1=M. |title=Hereditary Diaphorase Deficiency and Methemoglobinemia |journal=[[JAMA Internal Medicine|Archives of Internal Medicine]] |volume=113 |pages=578–85 |last2=Behlen |first2=Charles H. |last3=Lappat |first3=E. J. |last4=Cohn |first4=J. E. |issue=4 |doi=10.1001/archinte.1964.00280100086014}}</ref> |
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| pmid = 14109019 |
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| year = 1964 |
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| last1 = Cawein |
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| first1 = M |
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| title = Hereditary Diaphorase Deficiency and Methemoglobinemia |
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| journal = Archives of Internal Medicine |
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| volume = 113 |
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| pages = 578–85 |
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| last2 = Behlen Ch |
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| first2 = 2nd |
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| last3 = Lappat |
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| first3 = E. J. |
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| last4 = Cohn |
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| first4 = J. E. |
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| issue = 4 |
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| doi = 10.1001/archinte.1964.00280100086014 |
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}}</ref> |
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Benjamin Stacy, born in 1975, was 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 when he became cold or agitated.<ref name=ABCLakeLouise /> |
Benjamin Stacy, born in 1975, was 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 when he became cold or agitated.<ref name=ABCLakeLouise /> |
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In 2021, the novel ''Blue-Skinned Gods'' by [[S. J. Sindu]] references a family from Kentucky with [[methemoglobinemia]] but does not use the surname Fugate. |
In 2021, the novel ''Blue-Skinned Gods'' by [[S. J. Sindu]] references a family from Kentucky with [[methemoglobinemia]] but does not use the surname Fugate. |
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A reference to "the Huntsville subgroup" is made in the American version of television sitcom ''[[Shameless (American TV series)|Shameless]]'' when [[List of Shameless (American TV series) characters#Kevin Ball|Kevin Ball]] (played by [[Steve Howey (actor)|Steve Howey]]) discovers that he may have ancestors from that group.<ref>{{Cite episode |title=God Bless Her Rotting Soul|series=Shameless|network=Showtime|date= |
A reference to "the Huntsville subgroup" is made in the American version of television sitcom ''[[Shameless (American TV series)|Shameless]]'' when [[List of Shameless (American TV series) characters#Kevin Ball|Kevin Ball]] (played by [[Steve Howey (actor)|Steve Howey]]) discovers that he may have ancestors from that group.<ref>{{Cite episode |title=God Bless Her Rotting Soul |series=Shameless |network=Showtime |date=November 19, 2017 |season=8 |number=3 |quote=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?}}</ref> |
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==See also== |
== See also == |
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* [[Family aggregation]] |
* [[Family aggregation]] |
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Revision as of 10:47, 6 May 2023
| Fugates | |
|---|---|
| Country | United States of America |
| Current region | West Virginia |
| Founded | 1820 |
| Founder | Martin Fugate Elizabeth Smith |
| Final head | Benjamin Stacy |
| Members | Benjamin Stacy |
The Fugates, a family living in the hills of West Virginia starting in the 19th century, were commonly known as the "Blue Fugates"[1] or the "Blue People of West Virginia". They are known for being of a genetic trait that led to the blood disorder methemoglobinemia, causing the skin to appear blue.
Ancestry
Martin Fugate and Elizabeth Smith,[2] who had married and settled near Hazard, Kentucky, in 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 reproduction within the 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 exceeds 20 percent, but at levels between 10 and 20 percent it can cause blue skin without other symptoms. Most of the Fugates lived long and healthy lives. The "bluest" of the blue Fugates, Luna Stacy, had 13 children and lived to age 84.[6]
Descendants with the gene continued to live near Troublesome Creek and Ball Creek into the 20th century. They eventually came to the attention of the hematologist Madison Cawein III, who with the assistance of the nurse Ruth Pendergrass made a detailed study of their condition and ancestry.[2][7] Based on a report published in the Journal of Clinical Investigation in 1960 by a public health physician named E. M. Scott, who had studied a similar phenomenon among native Alaskans, Cawain concluded that a deficiency of the enzyme diaphorase resulted in an 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] He treated the family with methylene blue, which eased their symptoms and reduced the blue coloring of their skin.[8] His findings were published in the Archives of Internal Medicine in 1964.[9]
Benjamin Stacy, born in 1975, was 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 when he became cold or agitated.[5]
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.
In 2021, the novel Blue-Skinned Gods by S. J. Sindu references a family from Kentucky with methemoglobinemia but does not 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
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 Trost, Cathy (November 1982). "The Blue People of Troublesome Creek" (PDF). Science 82. Archived (PDF) from the original on September 27, 2015.
- ^ Adams, Cecil (July 24, 1998). "Is there really a race of blue people?". The Straight Dope. Retrieved April 22, 2021.
- ^ "Rare disease turns mountaineers blue". Phoenix, Arizona: Arizona Republic. November 7, 1974. p. 12. Retrieved April 3, 2019 – via Newspapers.com.
- ^ a b c James, Susan Donaldson (February 22, 2012). "Fugates of Kentucky: Skin Bluer than Lake Louise". ABC News. Retrieved October 4, 2014.
- ^ "Fugates of Kentucky: Skin Bluer than Lake Louise". ABC News. Retrieved June 3, 2022.
- ^ James, Susan Donaldson (March 8, 2012). "Blue People Look for Genetic Connection to Kentucky Fugates". ABC News. Retrieved October 4, 2014.
- ^ Davis, Lyle E. (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, Charles H.; 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.
- ^ "God Bless Her Rotting Soul". Shameless. Season 8. Episode 3. November 19, 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?