The Fugate Family – The Real Blue Bloods of Kentucky

Kermit, the frog, hit the pop charts lamenting the difficulty of being green, but the Fugate family of Kentucky suffered for almost 150 years from being blue – and not in the emotional sense. A rare genetic disorder caused some family members to be born with blue skin.

A blue-skinned man / Blue fingers / Blue grandmother
Source: YouTube

The Fugate story began in 1820 when a French orphan named Martin Fugate settled in Troublesome Creek in the hills of eastern Kentucky. He married a woman named Elizabeth Smith, who was as pale and white as the mountain laurel that blooms every spring around the creek hollows.

Four Children Born with Blue Skin

Husband and wife both possessed a recessive gene that led to four of their seven children being born with blue skin. Rural Kentucky was as isolated then as in the days of Daniel Boone. There were no roads, and a railroad wouldn’t reach that part of the state until the early 1910s.

An old painting of the Fugate family.
Source: YouTube

As a result, many Fugates began to marry and have children within their bloodline. This is not so unusual in eastern Kentucky, which has the highest rate of inbreeding in the state. The Whitaker family, the most inbred family in the U.S., also lives in that region.

Zachariah Marries Mom’s Sis

One of Martin and Elizabeth Fugate’s blue boys, Zachariah, married his mother’s sister, Elizabeth. Zechariah and his wife were not blue-skinned, but when their children married his younger siblings, they produced blue children. The family continued passing this genetic disorder to their generations through interbreeding.

A woman poses with her grandmother, who has blue skin.
Source: Facebook

One of their sons, Levy, married a Ritchie girl and had eight children, one of them Luna. Luna married John E. Stacy, and they had 13 children. Not all the children had blue skin. It did not seem to cause any health problems for family members. Luna, who was said to be the “bluest” family member, died at 84 after bearing 13 children.

The Stigma of Backwoods Inbreeding

As coal mining increased in the Troublesome Creek area, it became less isolated. The Fugates became more self-conscious about their skin color, partly because of the Blue itself and partly because of the stigma of inbreeding it evoked.

A family tree of the Fugate family.
Source: Facebook

In the early 1960s, University of Kentucky hematologist Madison Caweiin became interested in studying the “blue people.” He and American Heart Association nurse Ruth Pendergrass started searching for them in the area around Hazard. Pendergrass had met Luna once when the older woman came in for a blood test.

Seeking Out the Blue People

Pendergrass described her as “indigo” in color and said she had scared her to death because it looked like she had a heart attack because of her color. Now, however, whenever she and Caweiin spotted a “blue person,” the person would run from them.

A portrait of a woman with a blue tint to her skin.
Source: Tumblr

However, two Fugate descendants, Rachel and Patrick Ritchie, had had enough. They met with Caweiin and explained to the doctor that they were embarrassed by their blue skin. The doctor ran the tests on the couple and observed high methemoglobin levels in the .

A Diagnosis of Methemoglobinemia

According to the National Institutes for Health, methemoglobinemia is a blood disorder in which an abnormal amount of methemoglobin — a form of hemoglobin — is produced. Hemoglobin is responsible for distributing oxygen to the body, and without oxygen, the heart, brain, and muscles can die.

A woman stands beside a blue-skinned man.
Source: YouTube

In methemoglobinemia, the hemoglobin cannot carry oxygen, making it difficult for unaffected hemoglobin to release oxygen effectively to body tissues. Patients’ lips are purple, the skin looks blue, and the blood is “chocolate-colored” because it is not oxygenated.

Disorder Can be Caused by Exposure

As was the case with the Fugates, the disorder can be inherited or caused by exposure to certain drugs and chemicals such as anesthetic drugs like benzocaine and xylocaine. The carcinogen benzene and nitrites used as meat additives can also be culprits and certain antibiotics, including dapsone and chloroquine.

Paul Karason takes part in an interview.
Source: ABC

The genetic form of methemoglobinemia is caused by one of several genetic defects. The Fugates probably had a deficiency in the enzyme called cytochrome-b5 methemoglobin reductase, which is responsible for recessive congenital methemoglobinemia.

Methylene Provided the Cure

Caweiin injected them with methylene, which indeed cured them. Their skin color turned normal in a short period. They were both desperately happy at this simple solution and continued to take methylene pills to ensure that the blue color never returned.

Hands that have blue fingertips.
Source: Tumblr

The Fugates no longer live in isolation, and with the widening of the gene pool, the blue-skin affliction has vanished. The last known case of Fugate blue-skin was in 1975 when Benjamin “Benji” Stacey scared the Kentucky maternity doctors by popping out with blue skin.

Last of the Blue Fugates

He was rushed just hours after his birth to the University of Kentucky Medical Center. As a transfusion was being readied, Benji’s grandmother suggested to doctors that he looked like the “blue Fugates of Troublesome Creek.” And indeed, his great-great-great-grandmother was the famous Luna.

Paul Karason is a guest on a talk show.
Source: YouTube

Doctors say Benji likely carried only one gene for methemoglobinemia because he eventually had normal skin tones. The likelihood of him marrying a woman with the same recessive gene would have been small. Benji has vanished into the mists of time and has not responded to media follow-ups regarding the color of his skin.