_Thalasemmia _
By: Dickie
Over 2,000,000 American's are carriers of the genetic trait for Thalassemia, a
fatal blood disease. It is also the most common single gene disorder in the
world. This disease which prevents normal hemoglobin production in the blood,
is curently uncurable, and until recently only let its victim live no longer
then the first decade of their life. Thalassemia is a serious genetic disease
that afflicts children and adults all around the worldIn order to
understand Thalassemia, one must understand the physiolgy of blood. Blood, the
carrier of nutrition and waste in our bodies, contains a protein called
hemoglobin. Hemoglobin is solely responsible for the transport of oxygen from
the lungs to cells through out the body. It is imperative that hemoglobin is
readily avaible in the blood to ensure cells can function properly. There are
three types of hemoglobin. One is Hemoglobin A, which is the most prevalent in
adults. Hemoglobin A is composed of two alpha globins and two beta globins.
There are two mior hemoglobins names A2 and F. Hemoglobin A2 is composed of 2
alpha and two delta globins. Finally, hemoglobin F, predominatntly found in
infants, is composed of 2 alpha globins and 2 gamma globins. In a normal human
being the globins that make up hemoglobin would be produced at certain times
in a human's life. Initially, while it is a fetus, the human would contain
high amounts of hemoglobin F, and thus be producing alpha and gamma globins in
majority. However once a baby is born, gamma globin production drops and is
countered by beta globin production, thus allowing hemoglobin A to be created.
Delta globins also increase once a human is growing but they are rather
insignificantly low in numbers In a thalassemia patient, the genes that code
for the production of certain globlins are either mutated or destroyed. This
misinformation in the genetic code, leads to an abnormal ratio of globins
leaving too many unpaired globins and the expression of thalassemia. In
thalamessia, usually one type of globins is produced at normal levels while is
pair is not. The globin produced in normal levels and those that do not becom
paired for red cell aggregates which prove to be harmful to red blood cells.
These aggregrates, destroy the cell membrance of corpuscles which leads to
hemolysis, the destruction of red cells, or eeythropoices, the abnormal growth
of red blod cells. The amount at which these red cell aggregrates and the
properties of them define which type and the serverity of thalassemia a
patient has.
Word Count: 422
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