Sickle cell anemia is a genetic blood disorder where red blood cells take on an abnormal crescent shape, making them rigid and prone to getting stuck in small blood vessels. This can cause pain, organ damage, and other serious complications. The disease is caused by a defect in the hemoglobin gene and is inherited from one's parents. Symptoms include anemia, periodic pain crises, delayed growth, and vision problems. Treatments include medications to prevent clotting and open blood vessels, bone marrow transplants, and potential future treatments like gene therapy. More awareness is needed about sickle cell anemia and ways to prevent its progression.

1. -Presented By
SHUVADEEP HALDER
Roll No- 20PT011042
Sec- “A”
B.PHARM 1ST YEAR
JIS UNIVERSITY, KOLKATA
Sickle Cell Anemia

2. Table of
Contents
1. Introduction
2. Characteristics of sickle cells
3. Causes
4. Symptoms
5. Complications
6. Treatment
7. Conclusions
8. Reference

3. Introduction
Sickle cell anemia, or sickle cell disease (SCD), is
a genetic disease of the red blood cells (RBCs).
Normally, RBCs are shaped like discs, which gives
them the flexibility to travel through even the
smallest blood vessels.
However, with this disease, the RBCs have an
abnormal crescent shape resembling a sickle. This
makes them sticky and rigid and prone to getting
trapped in small vessels, which blocks blood from
reaching different parts of the body. This can
cause pain and tissue damage.

4.  120 days life span
 Hb has normal oxygen
carrying capacity
 12-14 g/dl of Hb
Characteristics of sickle cells
 30-40 day life span
 Hb has decreased oxygen
capacity
 6-9 g/dl of Hb
RBCS SICKLED CELLS

5. Causes
o Sickle cell disease is caused by a problem in the hemoglobin-
beta gene found on chromosome 11. The defect forms
abnormal hemoglobin.
o It can be passed from generation to generation but is only
majorly presented if the offspring has both allele for the
disorder.
There is a one in two (50%)
chance that any given child
will get one copy of the HbAS
gene and therefore have the
sickle cell trait.

6. There is also a one in four chance
that any given child could be
completely unaffected
No children will be completely
unaffected.

7. None will have sickle
cell anemia.

8. Symptoms
 Anemia
 Episodes of pain:
Periodic episodes of pain, called crises, are a major
symptom of sickle cell anemia.
Pain develops when sickle-shaped red blood cells block
blood flow through tiny blood vessels to your chest, abdomen and
joints. Pain can also occur in your bones.

9. Symptoms
 Painful swelling of hands and feet.
 Delayed growth:
A shortage of healthy red blood cells can slow
growth in infants and children and delay puberty in teenager.
 Vision problems:
Tiny blood vessels that supply your eyes may
become plugged with sickle cells. This can damage the retina — the
portion of the eye that processes visual images, leading to vision
problems.

10. Complication
s
 Stroke: A stroke can occur if sickle cells block blood
flow to an area of your brain. Signs of stroke include
seizures, weakness or numbness of your arms and legs,
sudden speech difficulties, and loss of consciousness.
 Organ damage: Sickle cells that block blood flow
through blood vessels immediately deprive the affected
organ of blood and oxygen. In sickle cell anemia, blood is
also chronically low on oxygen. Chronic deprivation of
oxygen-rich blood can damage nerves and organs in your
body, including your kidneys, liver and spleen.
 Acute chest syndrome: Acute chest syndrome can
be caused by a lung infection or by sickle cells blocking
blood vessels in your lungs.

11. Complication
s
 Pulmonary hypertension: People with sickle cell
anemia can develop high blood pressure in their lungs
(pulmonary hypertension).
 Blindness: Sickle cells can block tiny blood vessels
that supply your eyes. Over time, this can damage
the portion of the eye that processes visual images
(retina) and lead to blindness.

12. Treatmen
t
 Nitric oxide: Nitric oxide is a gas that helps keep blood
vessels open and reduces the stickiness of red blood cells.
 Bone Marrow Transplant:
Bone marrow transplant, also known as stem cell
transplant, offers the only potential cure for sickle cell anemia. It's
usually reserved for people younger than age 16 because the risks
increase for people older than 16. Finding a donor is difficult, and
the procedure has serious risks associated with it, including death.
If a donor is found, the person with sickle cell
anemia receives radiation or chemotherapy to destroy or reduce his
or her bone marrow stem cells. Healthy stem cells from the donor
are injected intravenously into the bloodstream of the person with
sickle cell anemia, where they migrate to the bone marrow and begin
generating new blood cells.

13. Treatment
 Gene therapy: Researchers are exploring whether
inserting a normal gene into the bone marrow of
people with sickle cell anemia will result in normal
hemoglobin.

14. Conclusion
s
Sickle cell anemia is a disease, which
requires immediate attention; if
unattended the results may be
fatal. Many still till this day are
unaware of the risks and affects of
such a lethal disease. People all
over the world need to be aware of
the possible dangers sickle cell holds
and ways to prevent it from
getting worse or spreading.

15. REFERENCE
Dr. C. M. JANGME, R. D.
WADULKAR, Dr. B. N. POUL
“PATHOPHYSIOLOGY” (NIRALI
PRAKASHAN 3rd EDITION’ 2019) ,
CHAPTER:- “HAEMATOLOGICAL
DISEASES” ,
PAGE NO:- “125-128”

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