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Sickle-Cell

Anemia

Definition
Sickle cell anemia is a
disease passed down
through families in which
red blood cells form an
abnormal crescent shape.

Causes/Etiology
Sickle Cell Anemia is an inherited disorder in which a child
inherits one abnormal gene from each parent. This is known as
autosomal recessive inheritance.

These abnormal genes produce an abnormal type of blood


hemoglobin called hemoglobin S.

RBC containing HbS loses its round, pliable,


biconcave disk shape and becomes deformed,
rigid and sickled-shaped
Sickle-shaped cells deliverO2 to the body's
tissues. They also can clog more easily in small
blood vessels, and break into pieces that disrupt
blood flow, the patient may have pain, swelling
and fever
If the RBC is again exposed to adequate amount of
oxygen before the membrane becomes too rigid, it
can revert to a normal shape
Cold can aggravate the sickling process

Risk Factor
NON MODIFIABLE
1.

Hereditary

2.

Race

Sickle cell anemia is most common in people of African,


Indian, Middle Eastern, or Mediterranean descent.
Nevertheless, it can occur in all peoples.

Signs and Symptoms


Signs and symptoms of sickle cell anemia usually show up after
an infant is 4 months old
Includes:
Anemia
RBCs usually live for about 120 days before they die and
need to be replaced. However, sickle cells die after only 10 to 20
days.
Without enough red blood cells in circulation, your body can't get
the oxygen it needs to feel energized.

Delayed growth
RBCs provide your body with the oxygen and nutrients you need
for growth
Shortage of healthy red blood cells can slow growth in infants and
children and delay puberty in teenagers

Jaundice
It is a yellowing of the skin and eyes that occurs because of liver
damage or dysfunction
Sickle cell anemia have some degree of jaundice because the liver,
which filters harmful substances from the blood, is overwhelmed by the
rapid breakdown of red blood cells

Hand-foot syndrome
Swollen hands and feet may be the first
signs of sickle cell anemia in babies. The
swelling is caused by sickle-shaped red
blood cells blocking blood flow out of their
hands and feet.
Frequent infection
Sickle cells can damage your spleen, an
organ that fights infection. This may make
you more vulnerable to infections.

1year old and up


Vision Problem
Tiny blood vessels that supply your eyes may become
plugged with sickle cells. This can damage the retina
(portion of the eye that processes visual images).
Episodes of pain (Sickle Cell Crisis)

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.
The pain may vary in intensity and can last for a few hours
to a few weeks.

Diagnostic Test
Test

Definition

Hemoglobin
Hemoglobin
electrophore
electrophoresis sis is a test
that
measures
the different
types of the
oxygencarrying
substance
(hemoglobin
) in the
blood.

Purpose

You may
have this
test if your
health care
provider
suspects
that you
have a
disorder
caused by
abnormal
forms of
hemoglobin

Normal
result

Abnormality
with
condition

No HbS

Presence of
HbS

A sickle
Sickle Cell
cell test
Test
looks for
the
presence
or
absence
of
abnormal
hemoglob
in in the
blood that
causes
sickle cell
anemia.

This test
is done to
tell if you
have
abnormal
hemoglob
in that
causes
sickle cell
disease
and sickle
cell trait.

Negative

Sickle
Cell Trait
Sickle
Cell
Anemia

Complete
Blood
Count
(CBC)

-It measures
the # of
RbCs, # of
WBCs, total
It is a
amount of
screening
Hb in the
test used to
blood, % of
diagnose
the blood
and manage
composed of
numerous
RBCs(hemat
diseases.
ocrit)
-provides
specific
information
the size and
hemoglobin
content of
individual
red blood
cells.

RBC:
Male:
4.7 to
6.1million
cells/mcL

decrease
(indicates

Female: 4.2
to 5.4 million
cells/mcL

WBC:4,500
to 10,000
cells/mcL

anemia)

Hgb:
Male:

13.8 to
17.2 gm/dL

Female:
12.1 to 15.1
gm/dL

Chronic
neutrophili
a is often

present
Platelets:
1500,000400,000/L

decrease
5-10g/dL
often
increase

Nursing Management
Provide information about what the client may feel
Encourage questions and dialogue about fears and apprehensions
Inform the client of the time period before the results will be available

Management
Medical
Patients with sickle cell disease need ongoing treatment, even when they
are not having a painful crisis.
1.

Acetaminophen

Classification: Analgesic
Action: It is used for the relief of fever as well as aches
and pains associated with many conditions.
Indication: Mild Pain/ Fever
Generic (Brand) names: Acetaminophen( Tempra, Tylenol)

2.

Hydroxyurea
Classification: Antimetabolite
Action: inhibits production of Hb F, which inhibits sickling
Indication: to reduce frequency of painful crises and
need for blood transfusions in adult

patient with Sickle Cell Anemia with


moderate to severe painful

recurrent
crises

Generic (Brand) names: Hydroxyurea (Droxia, Hydrea)

3.

Blood Transfusion
In a red blood cell transfusion, red blood cells are

removed from a supply of donated blood. These donated


cells are then given intravenously to a person with sickle cell
anemia.
Blood transfusions increase the number of normal red
blood cells in circulation, helping to relieve anemia. In
children with sickle cell anemia at high risk of stroke, regular
blood transfusions can decrease their risk of stroke.

4. Supplemental oxygen

Breathing supplemental oxygen through a breathing


mask adds oxygen to your blood and helps you breathe
easier.

Non Pharmacological-Highlights
1.

Supplements of Folic Acid(Vitamin B)


-essential for producing red blood cells

2.

Fluids
-help prevent dehydration
- given either by mouth or through a vein

3.

Heating pads

Surgical
Bone marrow transplant
This procedure replaces bone marrow affected by sickle cell
anemia with healthy bone marrow from a donor who doesn't have
the disease. It can be a cure, but the procedure is risky, and it's
difficult to find suitable donors.
Bone marrow used for a transplant must come from a closely
matched donor. This is usually a close family member who doesn't
have sickle cell anemia.
Bone marrow transplant requires a lengthy hospital stay. After
the transplant, you'll need drugs to help prevent rejection of the
donated marrow.

Nursing Diagnosis
Diagnosis: Acute pain related to tissue hypoxia
Interventions:
-promote calm and relaxing environment to divert clients
awareness to pain
-divertional activities like watching television, use of imagery etc.
-perform warm compress on the site of pain to provide relief
-encourage families to maintain a normal life for the child with SC
Anemia
Outcome: Client will be able to relieve pain.

Diagnosis: Risk for infection related to inadequate secondary defense


Interventions:
-promote childhood immunization program
-encourage adults to update immunizations to reduce the
transmission of global infection
-emphasize necessity of taking antiviral/antibiotics
-review individual nutritional needs, appropriate exercise program
and need for rest
-encourage frequent proper hand hygiene
Outcome: Client will be able to prevent the spread of potentially
infectious microorganism.

Diagnosis: Impaired gas exchange related to oxygen carrying


capacity of the blood
Interventions:
-review oxygen-conserving techniques
-instruct in the use of relaxation, stress reduction techniques
-encourage appropriate activity and exercise to improve
quality of life
-emphasize the importance of nutrition in improving stamina
and reducing the work of breathing
Outcome: Client will be able to have an adequate oxygenation
of tissue.

Prognosis
In the past, sickle cell patients often died from organ failure
between ages 20 and 40. Thanks to a better understanding and
management of the disease, today, patients can live into their 50s
or beyond.
Causes of death include organ failure and infection. Some
people with the disease experience minor, brief, infrequent
episodes. Others experience severe, long-term, frequent episodes
with many complications.

Research
Red grape skin may help sickle cell sufferers(October 31, 2009)
"AUGUSTA, Ga. An extract in red grape skin may be a new treatment for
sickle cell disease, Medical College of Georgia researchers say.
Study Using Viagra Ingredient to Treat Sickle Cell
Cut Short (July 28, 2009, 2:03 PM)

Viagra treats erectile dysfuntion by widening arteries. Viagras


active ingredient also has been approved, under the name
Revatio, to treat pulmonary hypertension, the condition raising
blood pressure in the lungs. And thats why researchers were
studying whether Revatio might also work in patients with sicklecell disease, a genetic blood disorder.
www.newstin.com.uk

References
Brunner and Suddaraths Textbook of Med-Surgical Nursing 11th
Edition
Kozier ans Erbs Fundamentals of Nursing
Tabers Cyclopedia Medical Dictionary, F.A. Davis
Disease and disorders Nursing Therapeutic Manual
Http://en.wikipedia.org/wiki/Sickle_Cell_Disease
http://www.nlm.nih.gov/medlineplus/ency/article/000527.htm
http://www.mayoclinic.com/health/sickle-cell-anemia/DS00324
Nursess Pocket Guide 11th Edition