As little as everyone may know… I have a huge obsession with vampires. I have traveled to New Orleans so that I could go on a vampire tour, I have gone to museums, and I watch many and many of vampire shows. I even have a picture of one hanging from my wall in my apartment and have been one the past three Halloweens. I am a super nerd when it comes to them. Therefore, I decided to choose the topic of Xeroderma pigmentosum for this weeks blog.
Xeroderma pigmentosum, also known as vampire syndrome, is
an inherited condition. This condition is characterized by extreme sensitivity
to the ultraviolet rays from sunlight just like vampires are known to be. When
in the sun the areas that are receiving sunlight and the eyes are affected. In
extreme conditions the nervous system is also affected.
The symptoms of this condition usually appear during
early childhood and infancy. It is usually diagnosed after a child gets a severe
sunburn after only being in the sun for a couple minutes. The sunburn leads to
blistering that will last for weeks at a time. Some children will tan very
easily and over time develop freckles on their skin in the areas that are exposed
to the sun. The name itself comes from the sunlight causing dry skin which is
xeroderma and the skin changing color which is pigmentation.
It is easy to tell that people with this condition will
have and extremely high risk of developing skin cancer. In most cases if the
correct sun protection isn’t used the children will develop skin cancer by the
age of ten. The skin cancer that is obtained is usually on sensitive areas such
as the face, lips, eyelids, scalp, and tip of the tongue. Not only are these
people at the risk of getting skin cancer, they also develop the risk of brain
tumors and lung cancer. It is essential that the people with this condition
wear eye protection because the sun will cause their eyes to get cloudy, their
eyelids to thin, and their eye lashes to fall out. It is also normal for these
people to develop eye abnormalities effecting their vision.
This condition is
caused be mutations in nine different genes. Eight of which make up the
nucleotide excision repair pathway which controls the repairs of UV induced DNA
damage. The ninth gene is what bypasses unrepaired damage. XP is an autosomal
recessive disorder meaning that it is inherited when two copies of a
non-working genes for the same trait is present. If a person only has one non-working
gene and one working gene they are a carrier of the condition but do not
express the symptoms. Parents that are blood relatives have an increased chance
to carry the same non-working genes which increases the risk of the child
getting the condition.
This condition would
be extremely hard to live with as you can tell from all the protection that
must be worn at all times. I don’t have this condition and I will burn after
being in the sun for less than an hour. Therefore, I couldn’t even imagine what
it would be like burning after minutes. It is also scary that you will most
likely develop skin cancer multiple times throughout your lifetime. I guess
this is why vampires immediately burst into flames after they get in the sun.
Very interesting post. I have heard of the vampire syndrome before and knew the basics of it, but I didn't know that cases could get so extreme. It's definitely sad to think that some children have to suffer and be without a childhood because of the sun, something we see as a normality.
ReplyDeleteWow, I've heard of this condition before, but I never knew much about it. I've know some people sunburn really fast, but I never knew that for some people it could happen within minutes. I feel bad for these children because they will never have the same experiences of childhood as the rest of us.
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