Entries in Fugates (1)


Man With Blue Skin Looks for Genetic Connection to Kentucky's Fugates

Kerry Green pictured on far right. Courtesy Kerry Green(SEATTLE) -- Born in 1964, Kerry Green of Tulsa, Okla., was given little hope that he would live because of a malformed aorta.

But by 3-years-old and several heart surgeries later, Green was being described by doctors as a "miracle child," small for his age at 23 pounds, but a "real live wire."

What doctors didn't know then was that the boy had a more serious underlying condition, a rare blood condition called methemoglobinemia -- the same disorder that affected the Blue Fugates of Kentucky.

"I was picked on as a kid in elementary school because I am blue," said Green, who is now 46.  "I look dead.  My lips are purple and my fingernails and toes are dark."

Today, Green lives in Seattle and is disabled, but he said he believes finding a genetic connection to the Fugates may help him learn more about the father he never knew.

"I am positive my father had the condition -- they all told me," said Green.  "I did see one kind of blurry picture of him and you could almost see it.  He's got the pale look I do."

Raised by his grandparents, Green said he doesn't even know if his father is alive.  Bob Green, who would be 73, had been a long-haul truck driver with relatives who had migrated west from Tennessee.

One of Green's sister was put up for adoption and the whereabouts of two brothers are unknown.  His mother, meanwhile, wandered in and out of his life.

"I just want to know where I came from and to know that side of my family history," said Green.  "It's hard to describe and it's kind of weird not knowing where the condition of mine came from.  People have pointed out the Fugates to me before."

Seven generations of the Fugates lived in an isolated pocket of Appalachia, passing down a recessive gene that turned their skin blue through in-breeding.

In the 1980s and 1990s, they dispersed, and the family gene pool became much more diverse.  Other relatives, perhaps like Green's paternal relatives, scattered throughout Virginia and Arkansas.

Even today, "you almost never see a patient with it," said Dr. Ayalew Tefferi, a hematologist from Minnesota's Mayo Clinic.  "It's a disease that one learns about in medical school and it's infrequent enough to be on every exam in hematology."

In the mildest form, methemoglobinemia causes no harm, and most of the Fugates lived well into their 80s.  But in Green's case, his body is starved of oxygen and every organ is affected.

Methemoglobinemia is a blood disorder in which an abnormal amount of methemoglobin -- a form of hemoglobin -- is produced.  Methemoglobin cannot effectively release oxygen.

Hemoglobin is responsible for distributing oxygen to the body and without oxygen, the heart, brain and muscles can die.

Copyright 2012 ABC News Radio

ABC News Radio