Sickle Cell Anaemia PDF

SICKLE CELL ANAEMIA
Definition: Sickle cell anaemia is an inherited red

blood cell disorder in which there are not enough
healthy red blood cells to carry oxygen round the
body.
Normally, the flexible, round red blood cells move
easily through blood vessels. In sickle cell anaemia,
however, the red blood cells are shaped like sickles
or crescent moons. These rigid, sticky cells can get
stuck in small blood vessels, which can slow or block
blood flow and oxygen to parts of the body.
Causes of sickle cell anaemia

Sickle cell anaemia is caused by the mutation in the
gene that tells the body to make the iron-rich
compound that makes blood red and enables red
blood cells to carry oxygen from the lungs
throughout the body. This compound is known as
haemoglobin
In sickle cell anaemia, the abnormal haemoglobin
causes red blood cells to become rigid, sticky and
misshapen.
Both mother and father must pass the defective
form of the gene for a child to be affected. If only one
parent passes the sickle cells gene to the child, that
child will have the sickle cell trait. With one normal
haemoglobin and one defective form of the gene
(which is the A S gene); people with the sickle cell
trait make both normal haemoglobin and sickle cell
haemoglobin.
Signs and Symptoms

Signs and symptoms of sickle cell anaemia usually
appear around five months of age. These include;
1) Anaemia: Sickle cells break apart easily and die
leaving the patient with very few red blood cells.
Red blood cells usually live for about 120 days
before they need to be replaced and destroyed by
the liver. But sickle cells usually die in 10 or 20
days, leaving a shortage of red blood cells.
Without enough red blood cells, the body can’t get
enough oxygen, causing serious fatigue.
2) Episodes of Pain: Periodic episodes of pain
called pain crisis, are major symptoms of sickle
cell anaemia. Pain develops when sickle-shaped
red blood cells block flow of blood through tiny
blood vessels to the chest, abdomen and joints.
Pain can also occur in the bones.
The pain varies in intensity and can last for a
few days to weeks.
3) Swelling of Hands and Feet: The swelling is
caused by sickle-shaped red blood cells blocking
flow of blood to the hands and feet.
4) Frequent Infections: Sickle cells can damage
the spleen, leaving a patient more vulnerable to
infections. Doctors commonly give infants and
children with sickle cell anaemia vaccinations
and anti-biotics to prevent potentially life-
threatening infections, such as pneumonia.
5) Delayed Growth or Puberty: Red blood cells
provide the body with the oxygen and nutrients
needed for growth. A shortage of healthy red
blood cells can slow growth in infants and
children and delay puberty in teenagers.
6) Vision Problems: Tiny blood vessels that
supply the eyes with ‘visual acquity’ can become
plugged by sickle cells. This can damage the
retina_ the portion of the eye that processes
visual images_ and lead to vision problems.
7) Fever: People with sickle cell anaemia have an
increased risk of serious infection, and fever can
be the first sign of an infection.
8) Abdominal swelling, especially if the area is
sensitive to touch.
9) Pale skin or nail beds.
Complications Involved in Sickle Cell

Anaemia
Sickle cell anaemia can lead to a host of
complications including;
1. Stroke: Sickle cells can block blood flow to a

portion of the brain. Signs of stroke include
seizures, weakness or numbness of the arms and
legs, sudden speech difficulties and loss of
consciousness. If your child has any of these
signs and symptoms, seek medical treatment
immediately. A stroke, whether partial or total
stroke, may be fatal.
2. Acute Chest Syndrome (A.C.S): A lung infection
or sickle cells blocking blood vessels in the lungs
can cause this life-threatening complication,
resulting in chest pain, fever and difficulty in
breathing. It might require emergency medical
treatment.
3. Pulmonary Hypertension: People with sickle
cell anaemia can develop high blood pressure in
their lungs. This complication usually affects
adults. Shortness of breath and fatigue are
common symptoms of this condition, which also
can be fatal.
4. Organ Damage: Sickle cells that block blood
flow to organs deprive the affected organs of
blood and oxygen. In sickle cell anaemia, blood
is also chronically low in oxygen. This lack of
oxygen rich blood can damage nerves and organs,
including the kidneys, liver and spleen, and can
be deadly.

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SICKLE CELL ANAEMIA

Definition: Sickle cell anaemia is an inherited red

Causes of sickle cell anaemia

Signs and Symptoms

Complications Involved in Sickle Cell

1. Stroke: Sickle cells can block blood flow to a

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