Sickle cell anemia

1. SICKLE CELL
SICKLE CELL
ANEMIA
Muhammad Waheed
Sir Rafique Syal

2. What Is Sickle Cell
Anemia?
Sickle cell anemia 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."

3. What Is Sickle Cell Anemia?
• 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

4. What Is Sickle Cell
Anemia?

5. Sickle cell anemia
• 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.

6. Sickle cell anemia
• 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..

7. Sickle cell trait
• 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.

8. Example of an Inheritance
Pattern for Sickle Cell Trait

9. What Causes Sickle Cell Anemia?
• Sickle cell anemia is an inherited disease.
People who have the disease inherit two copies of
the sickle cell gene—one from each parent.
• The sickle cell gene causes the body to make
abnormal hemoglobin.
• Hemoglobin is the iron-rich protein that gives
blood its red color and carries oxygen from the
lungs to the rest of the body.

10. What Causes Sickle Cell Anemia?
• In sickle cell anemia, the hemoglobin sticks together
when it delivers oxygen to the body’s tissues. These
clumps of hemoglobin are like liquid fibers.
• They cause the red blood cells to become stiff and
shaped like a sickle, or “C.”
• The sickled red blood cells tend to stick together and get
caught in the blood vessels.

11. Who Is At Risk for Sickle Cell
Anemia?
• Sickle cell anemia is most common in people
whose families come from
Africa, South or Central America (especially Panama),
Caribbean islands, Mediterranean countries (such as
Turkey, Greece, and Italy), India, and Saudi Arabia.

12. What Are the Signs and Symptoms of
Sickle Cell Anemia?
The most common symptom of anemia is
• fatigue (feeling tired or weak).
• Shortness of breath
• Dizziness
• Headache
• Coldness in the hands and feet
• Pale skin
• Chest pain

13. What Are the Signs and Symptoms of
Sickle Cell Anemia?
• Sudden pain throughout the body is a common symptom
of sickle cell anemia.
• This pain is called a "sickle cell crisis." Sickle cell crises
often affect the bones, lungs, abdomen, and joints.
• A sickle cell crisis occurs when sickled red blood cells
form clumps in the bloodstream
• These clumps of cells block blood flow through the small
blood vessels in the limbs and organs. This can cause
pain and organ damage.

14. Complications of Sickle Cell
Anemia
Hand-Foot Syndrome
• Sickle cells can block the small blood vessels in the
hands or feet.
• This condition is called hand-foot syndrome.
• It can lead to pain, swelling, and fever. One or both
hands and/or feet may be affected at the same time.

15. Complications of Sickle Cell
Anemia
Splenic Crisis
• The spleen is an organ in the abdomen.
Normally, it filters out abnormal red blood
cells and helps fight infection.
• In some cases, the spleen may trap cells
that should be in the bloodstream. This
causes the spleen to grow large and leads to
anemia.

16. Complications of Sickle Cell
Anemia
Acute Chest Syndrome
• Acute chest syndrome is a life-threatening condition
linked to sickle cell anemia.
• It’s similar to pneumonia. The condition is caused by an
infection or sickle cells trapped in the lungs.

17. Complications of Sickle Cell
Anemia
Priapism
• Males who have sickle cell anemia may have painful and
unwanted erections.
• It happens because the sickle cells block blood flow out
of an erect penis.
• Over time, priapism can damage the penis and lead to
impotence.

18. Complications of Sickle Cell
Anemia
Multiple Organ Failure
• Multiple organ failure is rare, but serious. It happens if
you have a sickle cell crisis that causes two out of three
major organs (lungs, liver, or kidney) to fail.
• Symptoms of this complication are a fever and changes in
mental status, such as sudden tiredness and loss of
interest in your surroundings.

19. How Is Sickle Cell Anemia
Diagnosed?
• Early diagnosis of sickle cell anemia is very important.
Children who have the disease need prompt and proper
treatment.
• The test uses blood from the same blood samples used for
other routine newborn screening tests. It can show
whether a newborn infant has sickle cell anemia or sickle
cell trait.

20. How Is Sickle Cell Anemia
Treated?
• Sickle cell anemia has no widely available cure.
However, treatments can help relieve symptoms
and treat complications.
• The goals of treating sickle cell anemia are to
relieve pain; prevent infections, eye damage, and
strokes; and control complications (if they
occur).

21. How Is Sickle Cell Anemia
Treated?
Bone Marrow Transplant
• A bone marrow transplant can work well for treating
sickle cell anemia. This treatment may even offer a cure
in a small number of cases.
• However, the procedure is risky and can lead to serious
side effects or even death. Because of this, only some
people can or should have this procedure.

22. How Is Sickle Cell Anemia
Treated?
Gene Therapy
• Gene therapy is being studied as a possible treatment for sickle
cell anemia. Researchers want to know whether a normal gene
can be put in the bone marrow of a person who has sickle cell
anemia. This would cause the body to make normal red blood
cells.
• Researchers also are studying whether they can "turn off" the
sickle cell gene or "turn on" a gene that makes red blood cells
behave normally.

23. How Is Sickle Cell Anemia
Treated?
• Researchers are studying several new medicines for sickle cell
anemia. These include:
• Butyric acid. This is a food additive that may increase normal
hemoglobin in the blood.
• Nitric oxide. This medicine may make sickle cells less sticky
and keep blood vessels open. People who have sickle cell
anemia have low levels of nitric oxide in their blood.
• Decitadine. This medicine increases hemoglobin F levels (this
type of hemoglobin carries more oxygen). It may be a good
choice instead of hydroxyurea.

24. How Can Sickle Cell Anemia Be
Prevented?
• You can't prevent sickle cell anemia because it’s an
inherited disease. However, you can take steps to reduce
its complications.
• People who are at high risk for sickle cell anemia and are
planning to have children may want to consider genetic
counseling.
• A counselor can help you understand your risk of having
a child who has the disease and help explain the choices
that are available to you.