Sickle Cell Anemia: A Curse and a Blessing
Sickle Cell Anemia is a disease found right here in America, but in low levels compared
to some areas of the world. The rate for this disease is around five times greater in certain places
in Africa. That is because the potentially fatal disease Sickle Cell Anemia can also work as a
sort of vaccination for another disease called malaria. First the mechanics of Sickle Cell Anemia
will be discussed, then its possible benefits.
Sickle Cell Anemia is an inherited condition. One gene for Sickle Cell Anemia must be
inherited from each parent in order to have the disease. A person who receives a gene for Sickle
Cell Anemia from one parent and a normal gene from the other has a condition called sickle cell
trait. Sickle cell trait produces no symptoms or problems for most people. Sickle Cell Anemia
can neither be contracted nor passed on to another person. The severity of sickle cell disease
varies greatly. Some people with sickle cell disease lead lives that are nearly normal. Others are
less fortunate, and can suffer from a variety of conditions.
Sickle Cell Anemia is caused by a change in the chemical composition of the protein
hemoglobin which is responsible for delivering oxygen throughout our bodies. Normal
hemoglobin is found as a round shape, and is composed of four proteins – two alpha chains and
two beta chains. The change that causes Sickle Cell Anemia occurs when an amino acid called
valine is substituted for glutamic acid in both of the beta chains. This change in the composure
of hemoglobin causes the shape to change when under certain conditions. Two of these
conditions are low oxygen and dehydration. The hemoglobin of a person with Sickle Cell
Anemia then becomes elongated and curved, hence the name sickle cell. When this happens
many problems can occur, primarily blood clotting which leads to a lack of oxygen in body
tissues. Other negative affects of Sickle Cell Anemia are a weakened heart because it is
constantly overworked. Also, bone marrow is affected and bones become softer than usual.
While there is no cure for Sickle Cell Anemia there is treatment. The primary goal is to reduce
the frequency of the Sickle Cell Anemia crisis episodes and maintain enough red blood cells to
keep body tissues healthy. Adequate hydration, oxygenation, bone marrow stimulation, and
blood transfusions are commonly used to treat Sickle Cell Anemia.
In the United States, about one in five hundred African Americans develop sickle cell
anemia. In Africa, about one in one hundred individuals develop the disease. Why is the
frequency of a potentially fatal disease so much higher in Africa? The answer is related to
another potentially fatal disease, malaria. Malaria is characterized by chills, fever, vomiting, and
severe headaches. Malaria is caused by a parasite called Plasmodium that is transmitted to
humans by a mosquito.
When malaria parasites invade the bloodstream, the red cells that contain defective
hemoglobin because of Sickle Cell Anemia become sickled and die. This effectively protects the
individual with Sickle Cell Anemia from an infection of malaria. This explains why areas if the
world with a high rate of malaria, such as Africa, also have a high rate for Sickle Cell Anemia.
Sickle Cell Anemia is nature’s version of irony. On one side of the coin is the answer to
malaria, a disease that has plague regions of the world for centuries; but sadly the other side of
the coin is possibly death.