1. SickleCell
Anemia
Dr. Rafi Ahmed Ghori
FCPS
Professor Medicine
Liaquat University of Medical & Health
Sciences, Jamshoro

2. What We Will Learn?
• What sickle cell anemia is.
• Who worked to understand sickle cell anemia.
• Who is affected by sickle cell anemia.
• What the symptoms of sickle cell anemia are.
• What the causes of sickle cell anemia are.
• What the effects of sickle cell anemia are.
• If there is a cure for sickle cell anemia.

3. What isSickleCell
Anemia?

4. SickleCell Anemia
• Sickle cell anemia is a genetic disorder of
the blood in which the red blood cells curve
into a sickle shape due to hemoglobin S.
• This shape change blocks them from going
through tiny blood vessels (capillaries) and
they clot your blood or break. If they break,
it leads to a insufficient amount of red blood
cells, or anemia.

5. Who Worked to
Understand Sickle
Cell Anemia?

6. William Warrick Cardozo
• Lived from 1905-1962
• In 1937 he concluded that the disease is
inherited and almost always occurs in
people of African descent.
• His works were published in the
Archives of Internal Medicine as
“Immunologic Studies in Sickle Cell
Anemia.”

7. Max F. Perutz
• He constructed a model of hemoglobin
that later helped prove how the
molecule’s structure changes when it
reacts with oxygen.
• His work helped explain why the
change in hemoglobin’s shape causes
the red blood cells to sickle and lessen
their oxygen-carrying capacity.

8. Who isAffected by
SickleCell Anemia?

9. Carriersof SickleCell
Anemia
• Everybody can inherit the sickle cell
trait if both their parents have the trait.
• Hemoglobin S is particularly common
in African Americans, about 8 to 12%
have the trait.
• The trait is also common in people
from Mediterranean countries, the
Middle East and India.

10. Carriers(continued)
• Hemoglobin S is common in the
populations mentioned due to the fact
that carries of sickle cell anemia are
more likely to live through a case of
malaria.
• Because of this, more and more of
those populations are getting the sickle
cell anemia trait from their parents.

11. What arethe
Symptomsof Sickle
Cell Anemia?

12. Symptomsof SickleCell
Anemia
• Symptoms may appear as early as 6
months of age.
• Early symptoms include serious
infections, pain and swelling of the
hands and feet, and enlargement of
the abdomen and heart.

13. What AreThe
Causesof Sickle
Cell Anemia?

14. Causesof SickleCell Anemia
• Sickle cell anemia is caused by the
hemoglobin S gene.
• Both parents must have the trait for there to
be a chance of their child getting sickle cell
anemia.
• If you have one hemoglobin S, and one
hemoglobin A (hemoglobin AS) you will
NOT get sickle cell anemia, because it is a
recessive trait.

15. Causes(continued)
• If and when you get sickle cell anemia,
the hemoglobin S gene causes the red
blood cell to sickle when it gets rid of a
large amount of oxygen.
• The hemoglobin S causes long, rigid
rods to form in the red blood cell when
it gives away oxygen. That is why the
cell sickles.

16. What AreThe
Effectsof Sickle
Cell Anemia?

17. Effectsof SickleCell Anemia
• There are 13 main effects of sickle cell
anemia. They are:
– Pain episodes
– Insufficient amounts of red blood cells
(anemia)
– Strokes
– Increased infections
– Leg ulcers
– Bone damage

18. Effects(continued)
– Yellow eyes or yellowish skin
– Early gallstones
– Lung blockage
– Kidney damage
– Blood blockage in the spleen or liver
– Eye damage
– Delayed growth

19. IsThereaCure
For SickleCell
Anemia?

20. Treatment for SickleCell
Anemia
• There is no definite cure for sickle cell anemia,
but you can get it treated.
• All treatment of sickle cell anemia is geared
toward preventing infection, reducing organ
damage, and minimizing all pain or
discomfort.
• If you are diagnosed early, some of the effects
like severe infections, can be prevented with
antibiotics and vaccinations.

21. Treatment (continued)
• Daily treatment with the cancer drug
hydroxyurea has been proven to reduce the
number of pain episodes and the severity of
the anemia.
• Regular blood transfusions help restore the
number of red blood cells, and make it less
likely that you will have a stroke.

22. Treatment (continued)
• The only proven cure worked on but a
handful of small children.
• These children were able to find an
acceptable bone marrow donor.
• This new bone marrow was able to
produce new red blood cells that didn’t
carry the sickle cell anemia trait.

33. Thank you for watching.