SICKLE-CELL ANEMIA (SCA) 3.
Hemoglobin SB 0 (Beta Zero)
Sickle-Cell Anemia is a blood disease that
affects red blood cells. Normal red blood cells
are round. In people with sickle cell anemia,
hemoglobin – a substance in red blood cells –
becomes defective and causes the red blood
cells to change shape
Sickle cell anemia is one of a group of
disorders known as sickle cell disease. It is an
inherited red blood cell disorder in which there
aren't enough healthy red blood cells to carry
oxygen throughout your body. Normally, the
flexible, round red blood cells move easily
through blood vessels. In sickle cell anemia,
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. There's no cure for most people with
sickle cell anemia. But treatments can relieve
pain and help prevent complications
associated with the disease.
TYPES of SCA
1. Hemoglobin SS Disease
Hemoglobin SS disease is the most common
and most severe type of sickle cell disease. It
occurs when you inherit the hemoglobin S
gene mutation from both parents. In this type,
the body only produces hemoglobin S. This
type is often called “sickle cell anemia.” As the
most severe form of SCD, individuals with this
form also experience the worst symptoms at a
higher rate.
2. Hemoglobin SC Disease
Hemoglobin SC disease is the second most
common type of sickle cell disease. It occurs
when you inherit the hemoglobin beta S gene
from one parent and the hemoglobin C gene
from the other. Individuals with hemoglobin SC
disease may have similar symptoms to
individuals with hemoglobin SS disease.
However, the symptoms may be less severe
and usually a milder form of SCD.
Thalassemia
Hemoglobin S beta zero thalassemia occurs
when you inherit the hemoglobin beta S gene
from one parent and a hemoglobin beta0
thalassemia gene mutation from the other
parent. It has similar symptoms to hemoglobin
SS disease and is also called sickle cell
anemia because the body only produces
hemoglobin S. However, sometimes the
symptoms of beta zero thalassemia are more
severe. It is associated with a poorer
prognosis.
4. Hemoglobin SB+ (Beta) Thalassemia
Hemoglobin SB+ (beta) thalassemia occurs
when you inherit the hemoglobin beta S gene
from one parent and a hemoglobin beta plus
thalassemia gene from the other parent. In this
type, some normal beta hemoglobin is
produced, but in reduced amounts. Because
the body produces some normal hemoglobin,
this form of sickle cell disease is less severe
than hemoglobin SS disease. Symptoms are
usually milder than hemoglobin SS or SC
disease, but complications can still develop.
5. Hemoglobin SD, Hemoglobin SE, And
Hemoglobin SO
These types of sickle cell disease are less
common and are usually less severe. People
who have these forms of SCD inherit one
sickle cell gene (“S”) and one gene from an
abnormal type of hemoglobin (“D”, “E”, or “O”).
Hemoglobin is a protein that allows red blood
cells to carry oxygen to all parts of the body.
The severity of these rarer types of SCD
varies.
6. Sickle Cell Trait (SCT)
HbAS - People who have SCT inherit one
sickle cell gene (“S”) from one parent and one
normal gene (“A”) from the other parent. This is
called sickle cell trait (SCT). People with SCT
usually do not have any of the signs of the
disease and live a normal life, but they can
pass the trait on to their children. Additionally, vascular damage are likely to be central
there are a few, uncommon health problems components of the pathophysiology of sickle
that may potentially be related to sickle cell cell anaemia.
trait.
CAUSES and RISK FACTORS of SCA
Sickle cell anemia is caused by a 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 (hemoglobin). In
sickle cell anemia, the abnormal hemoglobin
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 cell gene

to the child, that child will have the sickle cell
trait. With one normal hemoglobin gene and
one defective form of the gene, people with the
sickle cell trait make both normal 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: Having a family history of

sickle cell disease increases your risk for the
disease. In the United States, it mainly affects
African Americans.
PATHOPHYSIOLOGY of SCA
Sickle cell disease is caused by a mutation in
the beta-globin chain of the haemoglobin
molecule. Sickle haemoglobin, the result of this
mutation, has the singular property of
polymerizing when deoxygenated. Exactly how
normal tissue perfusion is interrupted by
abnormal sickle cells is complex and poorly
understood. Despite genetic identity at the site
of the sickle haemoglobin mutation, all patients
with sickle cell anaemia are not affected
equally by this disease. Secondary genetic
determinants and acquired erythrocyte and
SIGNS and SYMPTOMS of SCA
1. Anemia. Sickle cells are more fragile than
normal red blood cells and break apart
easily and tend to die in 10-20 days.
Normal red blood cells live for about 120
days. This causes a shortage of red blood
cells, known as anemia and thus, needs to
be replaced. Without enough red blood
cells, the body can't get enough oxygen,
causing fatigue.
2. Episodes of pain. Periodic episodes of
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 the chest, abdomen and joints.
Pain can also occur in the bones.
3. Swelling of hands and feet. Also known
as hand-foot syndrome or dactylitis. The
swelling is caused by sickle-shaped red
blood cells blocking blood flow to the hands
and feet.
4. Frequent infections. Sickle cells
sometimes damage the tissues, leading to
ulcers. If they damage the spleen, you
could get infections. Doctors sometimes
give SCD patients vaccinations and
antibiotics to prevent potentially life-
threatening infections, such as pneumonia.
5. Yellowing of skin, eyes and mouth.
Jaundice is a common sign and symptom
of sickle disease. Sickle cells do not live as
long as normal red blood cells and,
therefore, they are dying faster than the
liver can filter them out. Bilirubin (which
causes the yellow color) from these broken-
down cells builds up in the system causing
jaundice.
6. Vision problems. Sickle cells can get stuck
in the blood vessels that supply the eyes,
causing damage to the retina as well as
vision problems.
7. Developmental delays. 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.
COMPLICATIONS of SCA
o Stroke. Sickle cells can block blood flow to
an area of the brain.
o 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.
o Pulmonary hypertension. People with
sickle cell anemia can develop high blood
pressure in their lungs. This complication
usually affects adults.
o 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 the
kidneys, liver and spleen, and can be fatal.
o Blindness. Sickle cells can block tiny blood
vessels that supply the eyes. Over time,
this can damage the eye and lead to
blindness.
o Leg ulcers. Sickle cell anemia can cause
open sores on the legs.
o Gallstones. The breakdown of red blood
cells produces a substance called bilirubin.
A high level of bilirubin in the body can lead
to gallstones.
o 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.
o 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.
o Splenic sequestration (pooling). Crises
are a result of sickle cells pooling in the
spleen. This can cause a sudden drop in
hemoglobin and can be life-threatening if
not treated promptly. The spleen can also
become enlarged and painful from the
increase in blood volume. After repeated
episodes, the spleen becomes scarred, and
permanently damaged.
LABORATORY and DIAGNOSTIC TESTS
1. Hb electrophoresis
  Identifies any abnormal hemoglobin
types. This test can also differentiate
between sickle cell trait and sickle cell
anemia.
2. CBC – a test that measures and counts the
amount of blood cells. A patient with sickle
cell anemia has the following CBC results:
o Reticulocytosis – there is an
elevation in the number of
reticulocytes or the RBCs that are
still developing or immature RBCs
(count may vary from 30%–50%)
o Leukocytosis – there is an increased
levels of leukocytes in the blood
which indicates infection
o Decreased Hb (5–10 g/dL) and total
RBCs
o Elevated platelet
o Normal to elevated MCV
3. Sickle-turbidity tube test (Sickledex)
 Routine screening test that determines
the presence of hemoglobin S (HbS) –
or an abnormal hemoglobin that causes
red blood cells to become stiff and
abnormally shaped. Normal hemoglobin
in an adult is mostly Hgb A but sickle
cell anemia patient results from the
hereditary presence of abnormal Hgb S
in place of Hgb A
4. Stained RBC examination
 Normally, red blood cells are
disc shaped while patients who have
sickle cell anemia, the stained RBC
examination demonstrates partially
or completely sickled, crescent-
shaped cells.
5. ABGs
 May reflect acidosis and decreased
Po2
MEDICAL and SURGICAL TREATMENT
Management of sickle cell anemia is usually
aimed at avoiding pain episodes, relieving
symptoms and preventing complications.
Treatments might include medications and
blood transfusions but for some children and
teenagers, a stem cell transplant might cure
the disease.
A. Medications
 Hydroxyurea (Droxia, Hydrea, Siklos).
Daily hydroxyurea reduces the frequency of
painful crises and might reduce the need for
blood transfusions and hospitalizations. It
can also increase your risk of infections.
Don't take the drug if you're pregnant.
 L-glutamine oral powder (Endari). The
FDA recently approved this drug for
treatment of sickle cell anemia. It helps in
reducing the frequency of pain crises.
 Crizanlizumab (Adakveo). The FDA
recently approved this drug for treatment of
sickle cell anemia. Given through a vein, it
helps reduce the frequency of pain crises.
Side effects can include nausea, joint pain,
back pain and fever.
 Pain-relieving medications. Your doctor
might prescribe narcotics to help relieve
pain during sickle cell pain crises.
 Voxelotor (Oxbryta). The Food and Drug
Administration (FDA) recently approved this
oral drug to improve anemia in people with
sickle cell disease. Side effects can include
headache, nausea, diarrhea, fatigue, rash
and fever.
B. Surgical and other procedures
 Blood transfusions. In a red blood cell
transfusion, red blood cells are removed
from a supply of donated blood, then given
through a vein to a person with sickle cell
anemia. This increases the number of
normal red blood cells, which helps reduce
symptoms and complications.
 Stem cell transplant. Also known as bone
marrow transplant, this procedure involves
replacing bone marrow affected by sickle
cell anemia with healthy bone marrow from
a donor. The procedure usually uses a
matched donor, such as a sibling, who
doesn't have sickle cell anemia. Because of
the risks associated with a bone marrow
 transplant, the procedure is recommended
only for people, usually children, who have
significant symptoms and complications of
sickle cell anemia. The procedure requires
a long hospital stay. After the transplant,
you'll receive drugs to help prevent rejection
of the donated stem cells.
C. Other Treatments
 Rehydration with intravenous fluids
helps red blood cells return to a normal
state. The red blood cells are more likely to
deform and assume the sickle shape if
you’re dehydration.
 Treating underlying or associated
infections is an important part of managing
the crisis, as the stress of an infection can
result in a sickle cell crisis. An infection may
also result as a complication of a crisis.
 Supplemental oxygen is given through a
mask. It makes breathing easier and
improves oxygen levels in the blood.
 Immunizations can help prevent infections.
Patients tend to have lower immunity.
Childhood vaccinations are important for
preventing disease in all children. They're
even more important for children with sickle
cell anemia because their infections can be
severe. Vaccines against pneumonia and
meningitis and an annual flu shot are also
helpful. Vaccines are also important for
adults with sickle cell anemia.
 A person with sickle cell disease should
begin having regular eye exams at 9 or 10
years old. The exams will be repeated
annually for patients with sickle hemoglobin
C disease and every other year for those
with other types of sickle cell disease.
D. Lifestyle and Home Remedies
 Take folic acid supplements daily, and
choose a healthy diet. Bone marrow
needs folic acid and other vitamins to make
new red blood cells. Ask your doctor about
a folic acid supplement and other vitamins.
Eat a variety of colorful fruits and
vegetables, as well as whole grains.
 Drink plenty of water. Dehydration can
increase your risk of a sickle cell crisis.
Drink water throughout your day, aiming for
about eight glasses a day. Increase the
amount of water you drink if you exercise or
spend time in a hot, dry climate.
 Avoid temperature extremes. Exposure to
extreme heat or cold can increase your risk
of a sickle cell crisis.
 Exercise regularly, but don't overdo it.
Talk with your doctor about how much
exercise is right for you.
 Use over-the-counter (OTC) medications
with caution. Use OTC pain medications,
such as ibuprofen (Advil, Motrin IB,
Children's Motrin, others) or naproxen
sodium (Aleve), sparingly, if at all, because
of the possible effect on your kidneys. Ask
your doctor before taking OTC drugs.
 Don't smoke. Smoking increases your risk
of pain crises.
NURSING MANAGEMENT
1. Prevent tissue deoxygenation – since
sickle cell disease patients frequently
experience low oxygen levels due to the
reduced oxygen-carrying capacity of
hemoglobin S
o Monitor respiratory rate, depth, use
of accessory muscles, and areas of
cyanosis. These are indicators of
adequacy of respiratory function and the
need for therapy.
o Regularly assess level of
consciousness. Brain tissue is very
sensitive to decreases in oxygen. Doing
the assessment may be an early
indicator of developing hypoxia.
o Assist client in turning, coughing,
and deep-breathing exercises.
Promote expansion of chest optimally,
mobilization of secretions, and aeration
of all lung fields.
o Assist with ADLs and mobility as
needed. Limit activities within
patient’s tolerance. Reduces the
 metabolic requirements of the body
would reduce the oxygen requirements.
2. Promote rest
o Schedule rest periods and encourage
patient to alternate rests and activity.
To protect the patient from excessive
fatigue and reduce oxygen demands.
3. Pain management – pain develops when
sickled cells clog small blood vessels. Pain
can occur in the patient’s chest, abdomen,
joints and bones.
o Teach and discuss alternative pain
relief measures. Such as relaxation
techniques, breathing techniques,
meditation, distraction techniques and
use of heating pads to affected areas.
This can help the patient to reduce
reliance on pharmacological means of
pain control. This also enhances the
patient’s sense of control.
o Provide support and carefully
position affected extremities. To
reduce edema, discomfort, and risk of
injury.
o Massage gently affected areas. Helps
reduce muscle tension.
o Inspect skin and pressure points
regularly for redness. Poor circulation
may predispose to rapid skin
breakdown.
o Note and monitor ischemic areas,
cuts, bumps, and bruises closely for
ulcer formation. These serves as
potential entry sites for pathogenic
organisms. In presence of altered
immune system, this increases risk of
infection and delayed healing.
o Protect bony prominences with
pillows. Decreases pressure on tissues,
preventing skin breakdown.
5. Maintain adequate hydration
o Encourage adequate fluid intake (2 to
3 L/day) if not contraindicated.
Adequate intake is necessary to provide
for mobilization of secretions and
prevent hyper viscosity of blood
occlusion.
6. Promote client’s knowledge
o Teach patient about situations that can
precipitate a sickle cell crisis and steps
to take to prevent or diminish such
crises.

o Encourage ROM exercises. Prevents

joint stiffness and possible contracture
formation.

o Administer and monitor RBC

transfusions as indicated. Frequency
of painful sickle-cell crises may be
reduced by routine partial exchange
transfusions to maintain the population
of normal RBCs.

4. Prevent infection - because patients with

sickle cell anemia are susceptible to
infections, they are assessed for the
presence of any infectious process.