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St. Maarten Medical Center
Sickle-cell Anemia is a life-long disease of the blood that makes the normally round red blood cells turn stiff and sickle-like in shape.
1 in every 1,000 live births may develop sickle-cell anemia in St. Maarten. Signs of sickle-cell anemia is usually present themselves in childhood.
It is commonly seen in people from South and Central America, the Caribbean, the Middle East and those of African-descent.
Hemoglobin is the part of a Red Blood Cell that
carriers oxygen. Hemoglobin A (HbA) is found normally in most
people and does not cause any harm. But a certain type of hemoglobin causes Sickle-cell Anemia and
it is called Hemoglobin-S (HbS).
When the person is under a low-oxygen environment, or when it gets too cold,
the HbS changes the normally-round blood cells into something that look like crescents or sickles.
These fragile, sickle-shaped cells deliver less oxygen to the body.
They also clog the small veins in the body and stop a healthy blood flow. This causes pain. A
laboratory test is needed to determine if a person has this HbS in their body.
When both parents have HbA; their children would not have the sickle-cell disorder.
A child may have Sickle-cell Anemia only when both parents have an
abnormality in their blood.
If one of the parents has the HbS, there is a chance that one of their children would carry the Sickle-cell
trait, but would not get sick with it. This is what we call a Carrier.
But when both parents are Carriers, there is a high chance that their children would get sick with Sickle-cell Anemia.
Carriers and those positive with Sickle-cell Anemia may pass this disease to their
children, starting the chain again.
Diagram showing how the normal
hemoglobin-A (HbA) and the abnormal
hemoglobin-S (HbS) is passed by parents.
Both parents are carriers.
Almost all patients with sickle cell anemia have painful episodes called crises,
which can last from hours to days. These crises can affect the back, arms, legs and/or the chest.
Some patients have one episode every few years. Others have many episodes per year.
The crises can be severe enough to require a hospital stay.
Here are two common types of Crisis:
The Vaso-occlusive Crisis:
This occurs when an organ’s blood supply is blocked by sickled-cells (see diagram). This
causes pain and damage to the organ.
The frequency, severity, and duration of the crises vary considerably.
The “Spleen Crisis”:
This is painful enlargement of the spleen. ‘Sickled” blood accumulates into the
spleen, making it swollen, tender and very painful. It may also lead to a fatal drop of blood
volume from other parts of your body, especially from the brain.
Signs and symptoms of a sickle-cell crises vary from one persons to another. However, common symptoms include:
Pain management is one of the highest priorities until the crisis stops.
Strong pain medications incl. morphine-like substances, usually given
through an injection, is prescribed.
For milder crisis, common pain medications are given instead.
Cold air and water can start a painful crisis. Keeping the body warm,
especially during cold, moist days can prevent a crisis from happening.
It is a good advice to avoid cold showers, pools or the winter season
Since one of the common causes of a sickle- cell crisis is the lack
of oxygen in the body, providing oxygen offers some degree of
pain relief and it prevents the crisis from worsening.
Being even slightly dehydrated gives the body enough stress to cause a
To prevent this from happening, patients are given more fluids through an
intravenous line. These fluids also have the right amount of minerals and
sugar which the body needs.
Drinking more liquids like water and juice helps too.
Blood transfusions are sometimes ordered by the Internist when the amount of
blood in the body drops too low.
Hydroxyurea is the first approved drug for the management of sickle-cell
anemia. It was shown to decrease the number and severity of attacks in
This drugs is, however, relatively new and is not suitable nor works for each
sickle- cell case.
With good health care, many people who have sickle cell anemia can live productive lives. They
also can have reasonably good health most of the time and live longer today than in the past.
You can reduce the risk of a crisis occurring by:
Take medication as prescribed by your doctor.
Sickle cell anemia may be the result of a genetic mutation that happened in malaria-prone
regions like Africa thousands of years ago. People with sickle cell trait may have been
more likely to survive malaria epidemics - and because they survived when others did not,
this allowed the trait to be passed down through generations.