INDEX

 Overview

 Sickle cell anemia

 Symptoms

 When to see a doctor

 Causes

 Complications

 Prevention

 Pathophysiology

 Prognosis

 Conclusion

 Bibliography
 OVERVIEW

Sickle cell anemia is one of a group of inherited

disorders known as sickle cell disease. It affects the
shape of red blood cells, which carry oxygen to all
parts of the body.
Red blood cells are usually round and flexible, so
they move easily through blood vessels. In sickle cell
anemia, some red blood cells are shaped like sickles
or crescent moons. These sickle cells also become
rigid and sticky, which can slow or block blood flow.
There's no cure for most people with sickle cell
anemia. Treatments can relieve pain and help
prevent complications associated with the disease.
 Sickle Cell Anemia

 Red blood cells are usually round and flexible. In

sickle cell anemia, some red blood cells look like
sickles used to cut wheat. These unusually shaped
cells give the disease its name.

 In sickle cell anemia, blood is also chronically low in

oxygen. This lack of oxygen-rich blood can damage
nerves and organs, including kidneys, liver and
spleen, and can be fatal.
 Symptoms
Signs and symptoms of sickle cell anemia usually appear around
6 months of age. They vary from person to person and may
change over time. Signs and symptoms can include:
 Anemia. Sickle cells break apart easily and die. Red blood
cells usually live for about 120 days (about 4 months)
before they need to be replaced. But sickle cells typically
die in 10 to 20 days (about 3 weeks), leaving a shortage of
red blood cells (anemia). Without enough red blood cells,
the body can't get enough oxygen, and this causes fatigue.

 Episodes of pain. Periodic episodes of extreme pain, called

pain 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.

 The pain varies in intensity and can last for a few hours to a
few days. Some people have only a few pain crises a year.
Others have a dozen or more a year. A severe pain crisis
requires a hospital stay.
  Some adolescents and adults with sickle cell anemia also
have chronic pain, which can result from bone and joint
damage, ulcers, and other causes.
 Swelling of hands and feet. The swelling is caused by
sickle-shaped red blood cells blocking blood circulation in
the hands and feet.

 Frequent infections. Sickle cells can damage the spleen,

increasing vulnerability to infections. Infants and children
with sickle cell anemia commonly receive vaccinations and
antibiotics to prevent potentially life-threatening infections,
such as pneumonia.

 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.

 Vision problems. Tiny blood vessels that supply the eyes

can become plugged in with sickle cells. This can damage
the retina — the portion of the eye that processes visual
images — and lead to vision problems.

When to see a doctor!

 " See your health care provider right away if you or your
child has symptoms of sickle cell anemia

"
Because children with sickle cell anemia are prone to
infections, which often start with a fever and can be life-
threatening, seek prompt medical attention for a fever
greater than 101.5 F (38.5 C).
Seek emergency care for symptoms of stroke,
which include:

 One-sided paralysis or weakness in the face, arms or

legs

 Confusion

 Difficulty walking or talking

 Sudden vision changes

 Unexplained numbness

Causes
Sickle cell anemia is caused by a change in the gene that
tells the body to make the iron-rich compound in red
blood cells called hemoglobin.
Hemoglobin enables red blood cells to carry oxygen from
the lungs throughout the body. The hemoglobin
associated with sickle cell anemia causes red blood cells
to become rigid, sticky and misshapen.
For a child to be affected, both mother and father must
carry one copy of the sickle cell gene — also known as
sickle cell trait — and pass both copies of the altered form
to the child.
If only one parent passes the sickle cell gene to the child,
that child will have the sickle cell trait. With one typical
hemoglobin gene and one altered form of the gene, people
with the sickle cell trait make both typical hemoglobin
and sickle cell hemoglobin.
Their blood might contain some sickle cells, but they
generally don't have symptoms. They're carriers of the
disease, however, which means they can pass the gene to
their children.

Risk factors
For a baby to be born with sickle cell anemia, both
parents must carry a sickle cell gene. In the United States,
sickle cell anemia most commonly affects people of
African, Mediterranean and Middle Eastern descent.

A baby born with sickle cell disease inherits a gene for

the disorder from both parents. When both parents have
the genetic defect, there's a 25 percent chance that each
child will be born with sickle cell disease

Complications!
Sickle cell anemia can lead to a host of complications,
including:
Stroke
Sickle cells can block blood flow to an area 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
can be fatal.
Acute chest syndrome
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 breathing. It
might require emergency medical treatment.
Pulmonary hypertension
People with sickle cell anemia 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 can be fatal.

Organ damage
Sickle cells that block blood flow to organs deprive the
affected organs of blood and oxygen. In sickle cell
anemia, blood is also chronically low in oxygen. This lack
of oxygen-rich blood can damage nerves and organs,
including kidneys, liver and spleen, and can be fatal.
Splenic sequestration
Many sickle cells can get trapped in the spleen, causing it
to enlarge and possibly causing belly pain on the left side
of the body. This can be life-threatening. Parents of
children with sickle cell anemia should learn to regularly
feel their child's spleen for enlargement.
Blindness
Sickle cells can block tiny blood vessels that supply the
eyes. Over time, this can lead to blindness.
Leg ulcers
Sickle cell anemia can cause painful open sores on the
legs.
Gallstones
The breakdown of red blood cells produces a substance
called bilirubin. A high level of bilirubin in the body can
lead to gallstones.

Priapism
In this condition, men with sickle cell anemia can have
painful, long-lasting erections. Sickle cells can block the
blood vessels in the penis, which can lead to impotence
over time.
Deep vein thrombosis
Sickling of red cells can cause blood clots, increasing the
risk of a clot lodging in a deep vein (deep vein
thrombosis) or a lung (pulmonary embolism). Either can
cause serious illness or even death.

Pregnancy complications
Sickle cell anemia can increase the risk of high blood
pressure and blood clots during pregnancy. It can also
increase the risk of miscarriage, premature birth and
having low birth weight babies.

Prevention
If you carry the sickle cell trait, seeing a genetic
counselor before trying to conceive can help you
understand your risk of having a child with sickle
cell anemia. A genetic counselor can also explain
possible treatments, preventive measures and
reproductive options.

Sickle cell anemia is an inherited blood disorder.

Because it's a genetic condition someone is born
with, there is no way to prevent the disease, so
scientists are constantly investigating ways that the
disease can be stopped before it passes to the next
generation.

Pathophysiology
The loss of red blood cell elasticity is central to the
pathophysiology of sickle cell disease. Normal red blood cells
are quite elastic and have a biconcave disc shape, which allows
the cells to deform to pass through capillaries.

In sickle cell disease, low oxygen tension promotes red blood

cell sickling and repeated episodes of sickling damage the cell
membrane and decrease the cell's elasticity. These cells fail to
return to normal shape when normal oxygen tension is restored.
As a consequence, these rigid blood cells are unable to deform
as they pass through narrow capillaries, leading to vessel
occlusion and ischaemia.

The actual anaemia of the illness is caused by haemolysis, the

destruction of the red cells, because of their shape. Although the
bone marrow attempts to compensate by creating new red cells,
it does not match the rate of destruction. Healthy red blood cells
typically function for 90–120 days, but sickled cells only last
10–20 days (about 3 weeks)

Prognosis

About 90% of people survive to age 20, and close to 50%

survive beyond age 50.
In 2001, according to one study performed in Jamaica, the
estimated mean survival for people was 53 years for men and 58
years for women with homozygous SCD.
The specific life expectancy in much of the developing world is
unknown. In 1975 about 7.3% of people with SCD died before
their 23rd birthday; while in 1989 2.6% of people with SCD
died by the age of 20.

Conclusion
As our lifestyles are changing, we can escape from
hardships but not fatality. The awareness of this disease is not so
wide, and unawareness leads to the gate of this disease hence we
should stay aware and healthy.
Periodic checkups and a healthy habitual life would make
our society a healthier one! We should practice this not only for
ourselves but also for the future generation!

Bibliography
 https://en.wikipedia.org/wiki/
Sickle_cell_disease

 https://www.mayoclinic.org/diseases-
conditions/sickle-cell-anemia/symptoms-
causes/syc-20355876

 https://simple.wikipedia.org/wiki/
Sickle_cell_disease