Sickle Cell Anemia by Mohammed kareem

1. Sickle Cell
Anemia
By: Mohammed .K .R

2. Introduction
Anemia is a blood disorder in which
the blood has a reduced ability to
deliver oxygen due to a lower than
normal number of red blood cells,
or a reduction in the amount of
hemoglobin.

3. 01
02
03
04
05
06
About Sickle Cell anemia
Signs and symptoms
Etiology
Diagnosis
Complications Statistics
Contents
07
Treatment & Prevention
08
Recommendations
09
References

4. About Sickle Cell anemia
Sickle cell disease (SCD) is a group of blood disorders typically inherited
from a person's parents. The most common type is known as sickle cell
anemia. It results in an abnormality in the oxygen-carrying protein
hemoglobin found in red blood cells. This leads to a rigid, sickle-like shape
under certain circumstances. Problems in sickle cell disease typically begin
around 5 to 6 months of age. A number of health problems may develop, such
as attacks of pain (known as a sickle cell crisis), anemia, swelling in the
hands and feet, bacterial infections and stroke. Long-term pain may develop
as people get older. SCD affects African at much higher rates than people of
other ethnicities.
01

6. 02
• Sickle cell crisis
• Vaso-occlusive crisis
• Splenic sequestration crisis
• Acute chest syndrome
• Aplastic crisis
• Hemolytic crisis
• Frequent infections
• Swelling of hands and feet
• Delayed growth or puberty
• Vision problems
Signs and symptoms

7. Symptoms of sickle cell anemia
Acute chest syndrome
Fatigue
Distracted
Dizziness
Swollen hand
Irritable

8. 03
• Stroke
• Acute chest syndrome
• Pulmonary hypertension
• Organ damage
• Blindness
• Leg ulcers
• Gallstones
• Priapism (for males)
• Pregnancy complications
Complications

9. Etiology
Sickle cell disease occurs when a person inherits two abnormal copies of the
β-globin gene (HBB) that makes hemoglobin , one from each parent. This
gene occurs in chromosome 11. Several subtypes exist, depending on the
exact mutation in each hemoglobin gene. An attack can be set off by
temperature changes, stress, dehydration, and high altitude. A person with a
single abnormal copy does not usually have symptoms and is said to have
sickle cell trait. Such people are also referred to as carriers. Diagnosis is by
a blood test, and some countries test all babies at birth for the disease.
Diagnosis is also possible during pregnancy.
04

11. Diagnosis
The best way to check for sickle cell trait or sickle cell disease is to
look at the blood using a method called high-performance liquid
chromatography (HPLC). This test identifies which type of
hemoglobin is present. To confirm the results of HPLC, a genetic
test may be done.
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12. Statistics
There is no reliable estimate of global SCD cases. However, about 300,000
babies are born every year with sickle cell anemia. Most of these cases
occur in Nigeria, the Democratic Republic of the Congo, and India.
The World Health Organization and United Nations recognize sickle cell
disease as a global health issue. Many advances made in the treatment of
sickle cell disease have not yet reached countries with high poverty levels.
06

13. Sickle cell disease around
the world
African Americans are at a much higher risk of experiencing SCD.
Researchers believe this could be because SCD evolved in human populations
living where malaria is common.
• As high as 40 percent of people in some African countries have sickle cell
trait
• In countries with poor resources, more than 90 percent of children with
SCD do not survive to adulthood
• About 1,000 children in Africa are born with SCD every day, and more
than half will die before they reach 5 years old
• About 20 percent of children with SCD in India die by 2 years old
• By 2050, the number of people with SCD will increase by 30 percent

14. Data are based on estimates from Piel et al.1 Alaska is shown
separately from the rest of the United States.

15. Treatment & Prevention
Management of sickle cell anemia is usually aimed at
avoiding pain episodes, relieving symptoms and preventing
complications. Treatments might include medications and
blood transfusions. For some children and teenagers, a stem
cell transplant might cure the disease.
07

16.  Treatment
Medications
• Hydroxyurea : Daily hydroxyurea reduces the frequency of painful
crises.
• L-glutamine oral powder: It helps in reducing the frequency of pain
crises.
• Pain-relieving medications
• penicillin : From birth to five years of age, penicillin daily
• Folic acid

17. Surgical and other procedures
• Blood transfusions.
• Stem cell transplant. Also known as bone marrow transplant.
 Prevention
• Preventing infections
• Malaria prevention
• Stroke prevention
• Vaso-occlusive crisis prevention
• Acute chest syndrome prevention

18. Recommendations
The patient should taking the following steps to stay healthy and may help to
avoid complications of sickle cell anemia:
● Take folic acid supplements daily, and choose a healthy diet.
● Drink plenty of water.
● Avoid temperature extremes.
● Exercise regularly, but don't overdo it.
● Don't smoke.
● Finding someone to talk with.
● Exploring ways to cope with the pain.
● Learning about sickle cell anemia to make informed decisions about care.
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19. References
● Williams Hematology /Ninth Edition/CHAPTER 49 DISORDERS OF HEMOGLOBIN
STRUCTURE: SICKLE CELL ANEMIA AND RELATED ABNORMALITIE / Kavita
Natrajan and Abdullah Kutlar
● Fischbach FT, Dunning MB III, eds. (2009). Manual of Laboratory and Diagnostic Tests, 8th
ed. Philadelphia: Lippincott Williams and Wilkins.
● Kato GJ, Piel FB, Reid CD, et al. Sickle cell disease. Nat Rev Dis Primers. 2018;4:18010.
doi:10.1038/nrdp.2018.10.
● Serjeant GR. The natural history of sickle cell disease. Cold Spring Harb Perspect Med.
2013;3(10):a011783. doi:10.1101/cshperspect.a011783.
● Piel FB, Hay SI, Gupta S, Weatherall DJ, Williams TN. Global burden of sickle cell anemia in
children under five, 2010-2050: modelling based on demographics, excess mortality, and
interventions. PLoS Med. 2013;10(7):e1001484. doi:10.1371/journal.pmed.1001484.
● "What Causes Sickle Cell Disease?". National Heart, Lung, and Blood Institute. 12 June 2015.
Archived from the original on 24 March 2016. Retrieved 8 March 2016.
● Sickle-cell disease and other haemoglobin disorders Fact sheet N°308". January 2011.
Archived from the original on 9 March 2016. Retrieved 8 March 2016.

20. References
● Adams RJ, Ohene-Frempong K, Wang W (2001). "Sickle cell and the brain". Hematology.
American Society of Hematology. Education Program. 2001 (1): 31–46.
doi:10.1182/asheducation-2001.1.31. PMID 11722977.
● Chrouser KL, Ajiboye OB, Oyetunji TA, Chang DC (April 2011). "Priapism in the United
States: the changing role of sickle cell disease". American Journal of Surgery. 201 (4): 468–74.
● Lindenbaum J, Klipstein F: Folic acid deficiency in sickle-cell anemia. N Engl J Med
269:875–882, 1963
● Elzouki AY (2012). Textbook of clinical pediatrics (2 ed.). Berlin: Springer. p. 2950. ISBN
9783642022012

21. THIS IS THE END
OF PRESENTATION

22. Thanks You
For Four Attention