People in the Hazard, Kentucky area have been perplexed by a group of people with blue skin for 150 years. Methemoglobinemia, the cause of the illness, was discovered to mostly affect members of the Fugate family of Troublesome Creek and a kindred family in neighbouring Ball Creek. Not until the middle of the twentieth century, when the number of people affected by the hereditary condition had drastically dropped, was the local celebrity family of Blue Fugates—also known as the Huntsville subgroup—identified.
Although the Blue People of Kentucky and the highly unusual genetic mutation that gave them their distinctive blue skin hue continue to be topics of conversation, little is known about methemoglobinemia. The story of the Fugates and the met-H gene they passed down is instructive because it teaches us about genetics and disease.
From history, the Blue People of Kentucky are from the Fugate Family.
Around the year 1820, an orphan from France named Martin Fugate tied the knot with a woman whose name was Elizabeth. They decided to make their home in Troublesome Creek, which is located in Kentucky. Unbeknownst to the couple, they were both carriers of a recessive gene (met-H) that caused methemoglobinemia. This condition causes sufferers' skin to turn a bluish-grey colour, and it can be passed down through families. Four of Martin and Elizabeth's children were born with blue skin, giving the couple a total of seven children. They were taken aback and perplexed by the news, especially considering that neither parent exhibited any of the symptoms of methemoglobinemia. No one was able to explain.
In the nineteenth century, this region was characterized by its severe rurality and isolation. There were no roads, and it would not be until 1910 that the railroad would reach the area. The only other family in the area were connected to Elizabeth through marriage or blood. As a result of their seclusion, the Fugates started mating among themselves, which led to a greater chance of the met-H gene being passed down to subsequent generations and producing infants with blue skin. They were given the name Blue Fugates by the people in the area.
Zacariah, the son of the Fugates, wed Elizabeth's older sister, who was also his aunt. Both Zacariah and his wife, who did not have blue skin, had the gene for having non-blue skin, and subsequently, Zacariah's children married his younger siblings and their offspring. As a result, the quality was handed down from generation to generation in the family. Unfortunately, this meant that other families from neighbouring towns were less reluctant to marry into the Fugate family out of concern that their children would also produce blue-skinned children if they did so.
Later on, the Fugates were able to marry outside of their genetic group because their forebears dispersed and were replaced by newcomers over the generations. The risk of having a kid affected with met-H was reduced as a result of this.
Who and how did this disorder get fixed?
In the year 1960, Two Ritchie blue-skin descendent, Rachel and Patrick Ritchie, crossed paths with Dr Madison Cawein III. In Cawein's memory, he could tell how the two were ashamed of their blue complexion because of the stigma that had developed in the community around interracial marriage. Cawein carried out a series of tests, and the results revealed that the amounts of methemoglobin present in the blood were abnormally elevated. He concluded that administering injections of methylene blue would be the most effective treatment for them (more later on about why that worked). After injecting both patients with methylene blue, nurse Ruth Pendergrass observed that their skin "transformed before her very eyes" as a result of the medication. The doctors advised both patients to keep popping their methylene pills to prevent their blue colour from returning.
Benjamin Stacy, who was born in 1975, is the most recent known member of the Huntsville subgroup to have been born with blue skin. Benjamin appeared to be practically purple when he was born, which prompted a panic at the medical facility where he was delivered. The only other blue Fugate before Benjamin was Benjamin's grandmother, Luna, who remained a dark blue throughout her entire life. This is something that Benjamin's father recalled from their family history. Within a short period, Benjamin lost the blue tint to his fur. Even though they had methemoglobinemia, many Fugates enjoyed excellent health; in fact, it was said that several of them lived well into their 90s. There are no blue-skinned Fugates known to exist in today's world.
What is Methemoglobinemia?
Methemoglobinemia is a condition that affects the blood and is characterized by abnormally high amounts of protein methemoglobin in the blood. Methemoglobin is a type of haemoglobin that incorporates the iron atom Fe3 into its structure. Methemoglobin levels rise when the defence systems against oxidative stress are overpowered by iron 3, and haemoglobin is the protein in red blood cells that is responsible for delivering oxygen throughout the body. Following that, it transforms from haemoglobin into methemoglobin. Less than one per cent is considered to be normal for methemoglobin levels.
What are the symptoms
People whose methemoglobin levels are above 10% may suffer from cyanosis, which is characterized by blue skin and shortness of breath. Cyanosis can also cause fatigue, headaches, dizziness, and loss of consciousness. Rarely does this condition cause cyanosis that affects the entire body, as seen in the Fugate family. In most cases, the blue only appears in one particular region of the body.
Seizures, coma, and even death have been linked to methemoglobin levels that are higher than 50%. Those individuals who are otherwise healthy and have levels that are lower than 15% may display just mild symptoms or none at all. Patients who have methemoglobinemia are more likely to have blood that is a chocolate brown tint rather than a vibrant red tone.
Causes of this disorder
Experts have tried to explain the cause of these disorders in two different aspects. These include
- The Genetic aspect
An enzyme deficit (NADH) causes the Met-H recessive gene, which prevents oxygen from being transported across the body via the circulatory system. There is a one in four probability that a child of two carriers will be born with methemoglobinemia, a two in four chance that the child will be an unaffected carrier, and a one in four risks that the child will not acquire the gene at all.
As a result, the Fugates were struck by tragedy more often than average. There was an increased risk of passing on the disorder to offspring when both parents were carriers or were affected. A recessive trait like this would have been eliminated from the gene pool or seen very infrequent occurrences if the gene pool was sufficiently diversified. Isolation made this challenging and provided the ideal conditions for the perpetuation of the inherited disease.
The risk of passing met-H down through generations among the Fugates declined as they expanded their family and had children with people from beyond the remote Kentucky mountains where they had first resided. As a recessive trait, the chances of passing it on the decrease with each generation that marries outside the Fugate family.
- Acquired aspect
Methemoglobinemia can be brought on by many factors, including antibiotic use, local anaesthetics, and nitrate use. Methylated haemoglobin is produced when the body's defences are unable to keep up with the oxidizing medicines. Not only is it highly improbable that this would be permanent, but it also isn't likely to be the root cause of the Fugates' infamous "blue all over" appearance.
Naturally, infants have lower concentrations of the enzyme NADH. In carriers of the mutation, this enzyme prevents oxygen from entering the body's tissues and organs. For this reason, methemoglobinemia is more common in babies, especially those who have been exposed to nitrates. This condition, often known as "blue baby syndrome," has been linked to exposure to nitrates; those with private water systems are at more risk. In most cases, this is temporary and will resolve itself.
Replacing the Cause with a Remedy
Both Patrick Ritchie and his cousin Rachel Ritchie, both of the Fugate family, were cured after receiving methylene blue injections.
That brings us to the conclusion. I want to express my gratitude to you for taking the time to read this post, and I pray that God will richly reward you.
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