What Is Sickle Cell Anemia?
Sickle cell anemia (uh-NEE-me-uh) is a serious disease in which the body makes sickle-shaped red blood
cells. “Sickle-shaped” means that the red blood cells are shaped like a "C."
Normal red blood cells are disc-shaped and look like doughnuts without holes in the center. They move
easily through your blood vessels. Red blood cells contain the protein hemoglobin (HEE-muh-glow-bin).
This iron-rich protein gives blood its red color and carries oxygen from the lungs to the rest of the body.
Sickle cells contain abnormal hemoglobin that causes the cells to have a sickle shape. Sickle-shaped cells
don’t move easily through your blood vessels. They’re stiff and sticky and tend to form clumps and get
stuck in the blood vessels. (Other cells also may play a role in this clumping process.)
The clumps of sickle cells block blood flow in the blood vessels that lead to the limbs and organs.
Blocked blood vessels can cause pain, serious infections, and organ damage.
Normal and Sickled Red Blood Cells
Figure A shows normal red blood cells flowing freely in a blood vessel. The inset image shows a crosssection of a normal red blood cell with normal hemoglobin. Figure B shows abnormal, sickled red blood
cells clumping and blocking blood flow in a blood vessel. (Other cells also may play a role in this
clumping process.) The inset image shows a cross-section of a sickle cell with abnormal hemoglobin.
Overview
Sickle cell anemia is one type of anemia. Anemia is a condition in which your blood has a lower than
normal number of red blood cells. This condition also can occur if your red blood cells don’t have enough
hemoglobin.
Red blood cells are made in the spongy marrow inside the large bones of the body. Bone marrow is
always making new red blood cells to replace old ones. Normal red blood cells last about 120 days in the
bloodstream and then die. They carry oxygen and remove carbon dioxide (a waste product) from your
body.
In sickle cell anemia, a lower-than-normal number of red blood cells occurs because sickle cells don’t last
very long. Sickle cells usually die after only about 10 to 20 days. The bone marrow can’t make new red
blood cells fast enough to replace the dying ones.
Sickle cell anemia is an inherited, lifelong disease. People who have the disease are born with it. They
inherit two copies of the sickle cell gene—one from each parent.
People who inherit a sickle cell gene from one parent and a normal gene from the other parent have a
condition called sickle cell trait. Sickle cell trait is different from sickle cell anemia. People who have
sickle cell trait don’t have the disease, but they have one of the genes that cause it. Like people who have
sickle cell anemia, people who have sickle cell trait can pass the gene to their children.
Information above can be found at: http://www.nhlbi.nih.gov/health/dci/Diseases/Sca/SCA_WhatIs.html
How will I know if I have the Trait?
A SIMPLE PAINLESS 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, etc.).
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.
Blood transfusions help benefit sickle cell disease patients by reducing recurrent pain crises, risk of stroke
and other complications. Because red blood cells contain iron, and there is no natural way for the body to
eliminate it, patients who receive repeated blood transfusions can accumulate iron in the body until it
reaches toxic levels. It is important to remove excess iron from the body, because it can gather in the
heart, liver, and other organs and may lead to organ damage. Treatments are available to eliminate iron
overload.
Information above can be found at: http://www.sicklecelldisease.org/about_scd/index.phtml