Sickle Cell Anemia

The sickle cell disease is an inherited blood
disorder that affects red blood cells. People with
sickle cell have red blood cells that have mostly
hemoglobin\'s, Sometimes these red blood cells
become sickle-shaped or crescent shaped and
have trouble going through small blood vessels.
When sickle-shaped cells block small blood
vessels, less blood can get to that part of the
body. Tissue that does not get a normal blood
flow eventually becomes damaged.This is what
causes the problems of sickle cell disease.As to
this day there is really no cure for sickle cell
disease.Red blood cells take oxygen from the air
we breathe into our lungs to all parts of the body.
Oxygen is carried in red blood cells by a
substance called hemoglobin(Hemoglobin – is the
main substance of the red blood cell. It helps red
blood cells carry oxygen from the air in our lungs
to all parts of the body). Normal red blood cells
contain hemoglobin A. Hemoglobin S and
hemoglobin C are abnormal types of
hemoglobin.Oxygen is carried in red blood cells
by a substance called hemoglobin.The main
hemoglobin in normal red blood cells is
hemoglobin A. Normal red blood cells are soft
and round and can squeeze through tiny blood
tubes (vessels). Normally, red blood cells live for
about 120 days before new ones replace
them.People with sickle cell conditions make a
different form of hemoglobin A called hemoglobin
S (S stands for sickle). Red blood cells containing
mostly hemoglobin S do not live as long as normal
red blood cells (normally about 16 days). They
also become stiff, distorted in shape and have
difficulty passing through the body\'s small blood
vessels. When sickle-shaped cells block small
blood vessels, less blood can get to that part of
the body. Tissue that does not receive a normal
blood flow eventually becomes damaged. This is
what causes the complications of sickle cell
disease. There are several types of sickle cell
disease. The most common are: Sickle Cell
Anemia (SS), Sickle-Hemoglobin C Disease
(SC)Sickle Beta-Plus Thalassemia and Sickle
Beta-Zero Thalassemia. Sickle Cell trait (AS) is
an inherited condition in which both hemoglobin A
and S are made in the red blood cells, there are
always more A than S. Sickle cell trait is not a
type of sickle cell disease. People with sickle cell
trait are generally healthy. Sickle cell conditions
are inherited from parents in much the same way
as blood type, hair color and texture, eye color
and other physical things. The types of hemoglobin
a person makes in the red blood cells depend
upon what hemoglobin genes the person inherits
from his or her parents. Like most genes,
hemoglobin genes are inherited in two sets…one
from each parent(Ex. If one parent has Sickle Cell
Anemia and the other is Normal, all of the children
will have sickle cell trait. 4 If one parent has sickle
cell anemia and the other has sickle cell trait, there
is a 50% chance (or 1 out of 2) of having a baby
with either sickle cell disease or sickle cell trait
with each pregnancy,When both parents have
sickle cell trait, they have a 25% chance (1 of 4)
of having a baby with sickle cell disease with each
pregnancy). HOW DO YOU KNOW IF YOU
BLOOD TEST followed by a laboratory
technique called Hemoglobin Electrophoresis will
determine the type of hemoglobin you have. When
you pass an electric charge through a solution of
hemoglobin, distinct hemoglobins move different
distances, depending on their composition. This
technique differentiates between normal
hemoglobin (A), Sickle hemoglobin (S), and other
different kinds of hemoglobin (such as C, D, E,).
Medical Problems Sickle cells are destroyed
rapidly in the body of people with the disease
causing anemia, jaundice and the formation of
gallstones. The sickle cells also block the flow of
blood through vessels resulting in lung tissue
damage (acute chest syndrome), pain episodes
(arms, legs, chest and abdomen), stroke and
priapism (painful prolonged erection). It also
causes damage to most organs including the
spleen, kidneys and liver. Damage to the spleen
makes sickle cell disease patients, especially
young children, easily overwhelmed by certain
bacterial infections. TREATMENT Health
maintenance for patients with sickle cell disease
starts with early diagnosis, preferably in the
newborn period and includes penicillin
prophylaxis, vaccination against pneumococcus
bacteria and folic acid supplementation. Treatment
of complications often includes antibiotics, pain
management, intravenous fluids, blood transfusion
and surgery all backed by psychosocial support.
Like all patients with chronic disease patients are
best managed in a comprehensive
multi-disciplinary program of care. Promising
Treatment Developments In search for a
substance that can prevent red blood cells from
sickling without causing harm to other parts of the
body, hydroxyurea was found to reduce the
frequency of severe pain, acute chest syndrome
and the need for blood transfusions in adult
patients with sickle cell disease. Hydroxyurea is a
well-known drug,