Download “The Blue People of Troublesome Creek” In this interesting article

“The Blue People of Troublesome Creek”
In this interesting article, “The Blue People of Troublesome Creek”, it explains how a six
generations back, Martin Fugate’s great-great-great-great grandson was born in a modern
hospital. This baby, Benje Stacy, of Kentucky’s Troublesome Creek was born with dark blue
skin, which almost reached a purple color. Doctors really didn’t know what to do at this point
because they had never really seen anything like this. They wondered if this was due to blood
inherited from generations back. However, after a few weeks, he had lost his blue tint and only
dark blue lips and fingernails were left in the boy. The recessive gene that many of the Fugates
had was for the past 162 years. There were many speculations going on about the “blue people”
and had a lot of people wondering what was the actual cause. After ruling out speculations such
as heart disease and lung disease, the doctor came up with the idea that it might be
methemoglobinemia, which is a rare hereditary blood disorder that results from excess levels of
methemoglobin in the blood. Methemoglobin, which is blue, is a nonfunctional form of the red
hemoglobin that carries the oxygen to the body. It’s also the color of oxygen-depleted blood that
we see in the blue veins just below our skin that are easily seen with normal colored skin. This is
just what they came up with, and now they had to actually find the cause, which could have been
brought on by several things including abnormal hemoglobin formation, or enzyme deficiency.
Scott who was a Public Health Service doctor thought that it was due to the absence of enzyme
diaphorase from their red blood cells so now this was another speculation that they needed to
look into. They knew they needed to figure out what cure was going to do the work.
They came to the conclusion that diaphorase converts methemoglobin back to
hemoglobin, so to get this “blue people” disorder, a person would have to inherit two genes for
it, one from each parent so therefore somebody with only one gene would not have the condition
but could pass the gene to a child. They found that in normal people, hemoglobin is converted to
methemoglobin at a slow rate and if this conversion had continued at all, the body’s hemoglobin
would have just ended up being useless, because diaphorase converts methemoglobin back to
hemoglobin. Long story short, studies show that the body had an alternative method of
converting methemoglobin back to normal and this required adding to the blood a substance that
acts as an electron donor. Madison Caewin decided to inject them with 100 milligrams of
methylene blue and they changed colors. Cawein knew that using methylene blue had an
alternative method of converting methemoglobin back to normal and it required him adding this
substance to the blood of the donor that these people would be injected with. He knew this
substance was successful with other cases, so he thought he would try it and see if it would act
quickly to cause this cure. They found the resolution of it all and the study of blue people had
ended, but before this, Cawein returned to the mountains to resolve the confusing journal of
Martin Fugate’s recessive gene. The doctors had finally figured out what was going on with
these “blue people” and they did find a way to cure it. However, after this crazy, confusing
journey that the doctors and patients went through, Martin Fugate who was a French Orphan
from Kentucky in 1820 and carried the dominant gene, married a lady Elizabeth. They had 7
children and 4 of them were blue. After this, they just kept multiplying allowing blue people to
get multiplying. Fugates were marrying Fugates and marrying within the family. They basically
formed their own desolate community and completely built their own world with log cabins and
all. Now looking at the chemistry side to it all, we can figure out what this disease is and how it
turned these people blue.
Methemoglobinemia is a Beta-globin type that is an inherited blood disorder. It is a
disruption of hemoglobin in red blood cells, in which they carry oxygen to cells throughout the
body. This disease occurs when red blood cells contain methemoglobin at levels higher than 1%.
This results from presence of iron and the decreased availability of oxygen to the tissues. These
symptoms could include skin color changes and blood color changes and cardiac symptoms
could rise as a consequence of hypoxia as well. Functional Anemia refers to red blood cells
mass, which is the amount of hemoglobin of packed red blood cells that determined as a
hemoglobin concentration. 3-15% of levels of symptoms could include slight discoloration of
skin, which includes, pale, gray, or blue. Some other symptoms of methemoglobin accumulation
could include Cyanosis, headaches, lightheadedness, weakness, confusion, chest pain, altered
mental status, seizures, and sometimes death. Persons who are heterozygous for this disease have
no clinical or laboratory abnormalities and have the normal methemoglobin concentrations in
their blood. They have normal arterial oxygen saturation but have a certain amount of
methemoglobin in their blood, and this is usually 12% to 20%. Heterozygotes have slight lower
results than the reference range and homozygotes demonstrate little to no methemoglobin
activity and increased levels of methemoglobin.
According to the Pedigree of Hereditary
Methemoglobinemia chart in the article, it showed that Luna Fugate in the 1900s ended up with
the homozygous of the disease and it looks like the heterozygous carriers were on both sides with
the Fugates and the Ritchies within the 1900-year period. From the 1700s when this started,
somehow the homozygous dominant gene made its way down to Martin Fugate in which this
disease started, and then by the 1800s, there were at least 5 people of the Fugate family that were
homozygous dominant. By 1950, this is when Ben Stacy had the disease but was a carrier of it
by Luna Fugate’s son Alva who had Ben. Alva Stacy and Hilda Godsey had Ben after Alva
having the disease, had passed it on to ben who was now the heterozygous carrier.
I found this article very interesting! I thoroughly enjoyed reading through this article and
how it actually had doctors confused on to what it was and how they were going to figure it out.
I found it particularly interesting to look at the chart of how the blue gene started out and traced
where it went and from which person it started out with and how it got to the next person in the
family. I also wondered in the beginning how the doctors were going to go about this. How
were they going to figure out what substance they needed to place in these people’s body to
eliminate the blue color of their skin. When it talked about the blue dye and how that solution
might work to eliminate this problem and how they were going to inject the people with this to
turn them pink, it might work! I definitely didn’t think it was going to work but Cawein knew
that it had worked for something in the past and he knew that it probably wouldn’t have hurt to
try it. When I kept reading and found out that it worked for the people and they actually changed
colors within a few minutes from blue to no blue in their skin, I was surprised! I had no idea it
was going to work but I enjoyed reading how they went through the whole process of trying to
figure out a way and this way worked to take away the color. After this, one thing in this article
that really stood out for me is that the Fugates started marrying Fugates and multiplying and
marrying first cousins. It was strange to me that they started this marriage group within the
family and then lived in complete isolation from the world. I just took this part of the article into
deep thought just because thinking of the society in today’s world and how anyone who heard
this type of thing would find such weirdness in it. Overall, I definitely enjoyed reading this
article and learning about a “blue people” cause like this. I’m glad we were able to read it and
figure out certain aspects through out this paper from the genetic basis of methemoglobinemia to
describing the chemistry behind this unique type of disease.
Additional Resources
Denshaw-Burke, M. (1994, January 1). Methemoglobinemia. Methemoglobinemia. Retrieved
April 13, 2014, from http://emedicine.medscape.com/article/204178-overview
Test ID: METR
Methemoglobin Reductase, Blood. (1995, January 1). METR. Retrieved April
12, 2014, from http://www.mayomedicallaboratories.com/testcatalog/Clinical+and+Interpretive/9322