Talk about blue in the face! The extraordinary tale of the Blue Family in Appalacia

  • Isolated family in Kentucky started producing blue children in 1800s
  • French orphan Martin Fugate married pale American Elizabeth Smith
  • Had seven children, four were blue; they intermarried with local family
  • Genetic mutation reduces individual’s ability to carry oxygen in blood
  • Intermarriage led to ‘pure’ gene pool which often met ‘met-H’ gene

By
Daily Mail Reporter

Last updated at 6:03 PM on 16th February 2012

In the Appalachian Mountains rests a medical oddity so unusual that it at first seems a massive hoax.

Dating back to the early 1800s, an isolated family in eastern Kentucky – who can trace their roots back to a French orphan – started producing children who were blue.

As a result of a coincidental meeting of recessive genes, intermarriage and inbreeding, members of the Fugate family were born with a rare condition that made them visibly discoloured.

Due to inbreeding and inter-marriage, a rural family developed a rare blue discolouration

Feeling blue: Due to inbreeding and inter-marriage, a rural family developed a rare skin discolouration

The mystery behind the astonishing picture of the Fugates, which has been baffling people for years, appears to have finally been solved.

It began when Martin Fugate, a French orphan, settled on the banks of eastern Kentucky’s Troublesome Creek to claim a land grant in the early 19th century.

He married a red-haired American named Elizabeth Smith – who had a very pale complexion – and their union formed a genetic mutation that resulted in their descendants being born with blue skin.

Looking at the portrait, they appear to have been either Photoshopped or made up to mimic characters from children’s cartoon The Smurfs, but science proves that the condition is in fact real.

Rural: The Fugates lived in an isolated part of eastern Kentucky and had little opportunity to mix with other families

Rural: The Fugates lived in an isolated part of eastern Kentucky and had little opportunity to mix with others

Relations: A photo of Lorenzo 'Blue Anze' Dow Fugate and Eleanor Fugate, taken around 1915

Relations: A photo of Lorenzo ‘Blue Anze’ Dow Fugate and Eleanor Fugate, taken around 1915

Called methaemoglobinaemia (commonly
known as met-H), the condition reduces the individual’s ability to carry
oxygen in their blood. As a result, their blood is darker than the colour typically found running through people’s veins.

HOW ‘MET-H’ DARKENS BLOOD

The methaemoglobinaemia condition, or ‘met-H’, reduces someone’s ability to carry oxygen in the blood, leaving it darker than the colour typically found in veins.

The Fugate family intermarried with a neighbouring family for generations, which led to a relatively ‘pure’ gene pool, often including the met-H gene.

But as Kentucky became more populated and more genes came into the Fugate family tree, there were far fewer children born blue.

The gene is still around today but has now become statistically insignificant and there are no serious medical problems associated with it.

Because
the Fugate family lived in such an isolated part of the Kentucky, they
intermarried with a neighbouring family for generations which led to a
relatively ‘pure’ gene pool where the met-H gene appeared much more
frequently.

The family was first discovered in 1958
when one of the blue men, Luke Combs, who was a descendant of another
branch of the Fugate family, took his white wife to the University of Kentucky
Hospital and doctors paid more attention to him than his wife.

‘Luke was just as blue as Lake Louise on a cool summer day,’ doctor Charles H. Behlen II told the Tri-City Herald in 1974.

Aside from the stark discoloration of the carrier’s skin, there are no serious problems associated with the disease.

In 1980, a counter-intuitive solution was discovered where the blue person drinks a chemical-filled solution that is itself blue. This then turns the carrier’s blood into a ‘normal’ red hue which is then reflected in a change in skin tone.

Methaemoglobinaemia (commonly known as met-H) is a recessive gene (shown by the small r) so only produces 'blue' people in very rare circumstances

Methaemoglobinaemia (commonly known as met-H) is a recessive gene (shown by the small r) so only produces ‘blue’ people in very rare circumstances

Because of the dispersion of fluids, the
solution only lasts for about a day so the carrier would have to drink a
serving every day.

‘They weren’t sick; it was just the way they look. They’re normal people – they’re good people’

Nurse Ruth Pendergrass

As eastern Kentucky has become vastly more populated than the early 19th century, and as more genes are married into the Fugate family tree, there were far fewer children born with the condition.

That said, the recessive met-H gene lingers to this day, but it is statistically insignificant now.

‘They weren’t sick; it was just the way they look,’ said nurse Ruth Pendergrass in the Tri-City Herald article. ‘They’re normal people – they’re good people.’

 

Here’s what other readers have said. Why not add your thoughts,
or debate this issue live on our message boards.

The comments below have not been moderated.

An blue-skinned orphan from France, trying to find a new life, where he is accepted, works hard in the middle of know-where and finds himself a wife, they lead a very harsh lives, hunting for food. Children spread over the neighbouring mountains, marrying and then cousins sometimes marry ……. this is totally natural and legal (even in UK). The gene pool naturally expands and the likelihood of both Recessive genes creating a ‘blue person’ becomes less likely. But hats off to Martin Fugate, a survivor of lifes’ cruelties, against the odds.

Would you like to have another go at this? I know you are being insulting but don’t understand how or why.
– tynegirl, Bromley UK, 16/2/2012
Very amusing – I did laugh – upon referal to the original comment I think Tiziana had an upset English customer whose toast was burnt and didn’t think much of the orange marmalade on offer !!! And how dare they complain – after all having bed and breakfast one should be grateful with whatever breakfast is served to them – very strange !!!

The only problem here is the first picture is clearly altered. It says the condition still exists today, so why no current picture of a family member with this condition?

They’re Blue, dabah dee, dabah dah, da dabah deebee dabah dah, dabah dee, dabah dah, da dabah deebee dabah dah, dabah dee, dabah dah, da dabah deebee dabah dah, dabah dabah da dahhhhhh…
– Dave, Glasgow, 16/2/2012 11:37
———
Bump. This joke needs to be seen by everyone!!!

Avatar part 2

What a load of bol***ks … What the hell is wrong with the DM management team? …

“I’m just curious as to why methaemoglobinaemia is commonly known as met-h ?
– bb, north, 16/2/2012 17:59”
===== Simples…. it is actually MetHb which is derived thus:
MET from the first three characters of METhaemoglobinaemia
+
Hb from Haemoglobin (the molecule that transports oxygen in red blood cells).

Methemoglobinemia can also be acquired after birth. Nitrates used in agricultural fertilisers may leak into the ground and may contaminate well water.

Infants under 6 months of age are particularly susceptible to methemoglobinemia caused by nitrates ingested in drinking water (called blue-baby syndrome). Benzocaine applied to the gums or throat (as commonly used in baby teething gels) can cause methemoglobinemia.

Isn’t Kentucky the Blue Grass State.

I agree withTiziana U.S.A. born an Italian,escaped at 6 years old. 09:05 . We are truly amazing people ! dont know what the breakfast order is about though.

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