Life in a blue skin

A disease where a person can develop dark blue skin without any other symptoms.

BY GARDY CHACHA

Six generations after a French orphan named Martin Fugate settled on the banks of eastern Kentucky’s Troublesome Creek with his redheaded American bride, his great-great-great great grandson was born in a modern hospital not far from where the creek still runs.

It is recorded that the boy inherited his father’s lankiness and his mother’s slightly nasal way of speaking. What he got from Martin Fugate was dark blue skin.

“It was almost purple,” his father recalls. The Fugates are the first known humans to have lived as blue human beings. History has it that doctorswere so astonished by the colour of Benjamin “Benjy” Stacy’s skin that they raced him by ambulance from the maternity ward in the hospital near Hazard to a medical clinic in Lexington in the UnitedStates.Two days of tests produced no explanation for the skin colour of a bruisedplum.

Methemoglobinemia is a blood disorder in which an abnormal amount of methemoglobin — a form of haemoglobin— is produced, according tothe American National Institutes for Health. Haemoglobinis responsible for distributingoxygen to the body. Withoutoxygen, the heart, brain andmuscles can die.The disorder can be inherit-

The Fugates are the first known humans to have lived as blue human beings. History has it that doctors Medical Mysteries ed, as was the case with the Fugate family, or caused by exposure to certain chemicals such as anaesthetic drugs like benzocaine and xylocaine. The carcinogen benzene and nitrites used as meat additives can also be culprits, as well as certain antibiotics, including dapsone and chloroquine. 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.Normally, people have less than about one per cent of methemoglobin, a type of haemoglobin that is altered bybeing oxidised so it’s useless in carrying oxygen in the blood.

When those levels rise to greater than 20 per cent, heart abnormalities, seizures and even death can occur.

ONE CAN LIVE A FULL LIFE

But at levels of between 10 and 20 per cent, a person can develop blue skin without any other symptoms. Most of blue Fugates never suffered any health effects and lived into their 80s and 90s.

In methemoglobinemia, the haemoglobin is unable to carry oxygen and it also makes it difficult for unaffected haemoglobin to release oxygen effectively to body tissues.

Patients’ lips are purple, the skin looks blue and the blood is Tuesday, July 17, 2012 / The Standard  ‘chocolate coloured’ because it is not oxygenated, according to reports.

Martin Fugate came to Troublesome Creek from France in 1820 and family folklore says he was blue. He married Elizabeth Smith, who also carried the recessive gene. Of their seven children, four were reported to be blue. There were no railroads and few roads outside the region, so the community remained small and isolated. The Fugates married other Fugate cousins and families who lived nearby,with names like Combs, Smith, Ritchie and Stacy. That is most likely how the blue skin genes appeared phenotypically in their offsprings.