SICKLE CELL

ANEMIA

By: Princess Joy Galicha

Claire Marie Gonzales
 Sickle Cell Anemia
• Sickle cell anemia 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 are shaped like
sickles or crescent moons.
 Epidemiological Status
• Currently, approximately 250 million people
worldwide carry the gene responsible for
sickle cell disease and other hemoglobin
diseases. Each year about 300,000 infants
are born with a major hemoglobin disease
worldwide. In the United States, about 1 in
every 500 African Americans are born with
the disease.
 SICKLE CELL ANEMIA

Modifiable Risk Factors: Non-Modifiable Risk

•Socioeconomic (education, Factors:
income, insurance status, •age, 13 to 36 months
availability of outpatient care) •sex, (male)
•Both parents have SCD
genes
•ethnicity, (Black or
African-American)
•SCD genotype (mutation
in the β-globin gene)
Diseases Process:
Both parents with SCD gene

abnormal hemoglobin

RBC becomes rigid, sticky and misshapen

Sickle-shaped cell stick to vessel walls

Cause blockage

O2 can't reach nearby tissues

severe pain
CLINICAL MANIFESTASTIONS
• Jaundice
• icterus,
• Fatigue or fussiness
• Painful swelling of the hands and feet, known as dactylitis.

DIAGNOSTIC TEST
Non-Invasive:

Invasive:
•Blood smear
•CBC
•Sickle cell test
•Amniocentesis
 Test Normal Abnormal
Blood Smear biconcave, disc-shaped Sickle-shape
CBC 14-17 g/dL (140-170 g/L) Below
Female: 12-16 g/dL
(120-160 g/L)
Sickle Cell Test A negative test is normal. A positive test result may
It means your mean you have sickle
hemoglobin is normal cell trait or SCD.
Amniocentesis Negative mutations in Positive mutations in the
the HBB gene HBB gene
 Nursing Diagnosis #1: Acute pain related to
tissue hypoxia

INDEPENDENT DEPENDENT COLLABORATIVE

Apply warm, moist
compresses to affected
joints and other painful
areas. Avoid use of ice or
cold compresses.
Rationale:
Warmth causes vasodilation
and increases circulation to
hypoxic areas. Cold causes
vasoconstriction and
compounds the crisis.
Administer and monitor
RBC transfusions.
Rationale:
painful sickle-cell crises
may be reduced by routine
partial exchange
transfusions to maintain the
population of normal RBCs.
 Nursing Diagnosis #2: Risk for infection
INDEPENDENT
Assess for the presence,
existence of, and history of
the common causes of
infection (listed above).
Rationale: These factors
represent a break in the
body’s normal first line of
defense and may indicate
an infection.
DEPENDENT COLLABORATIVE
Administer/monitor
medication regimen (e.g.,
antimicrobials, drip infusion
into osteomyelitis,
subeschar clysis, topical
antibiotics) and note the
client's response.
Rationale: To determine
effectiveness of theraphy or
presence of side effects.
 Nursing Diagnosis #3 Risk for Deficient Fluid
Volume
INDEPENDENT
Maintain accurate I&O and
weigh daily.
Rationale: Patient may
reduce fluid intake during
periods of crisis because of
malaise and anorexia.
Dehydration from vomiting,
diarrhea, and fever may
reduce urine output and
initiate a vaso-occlusive
crisis.
DEPENDENT COLLABORATIVE
Administer IV fluids as
indicated.
Rationale: IV fluids
replaces losses and fills
deficit. It may reverse renal
concentration of RBCs and
presence of failure. Fluids
must be given immediately
to decrease
hemoconcentration and
prevent further infarction.
 Nursing Diagnosis #3 Risk for Deficient Fluid
Volume (con’t)
INDEPENDENT DEPENDENT COLLABORATIVE
Monitor vital signs, comparing with
patient’s previous readings. Measure
BP in lying, sitting, and standing
positions if possible.
Rationale: Reduction of circulating
blood volume can occur from
increased fluid loss, resulting in
hypotension and tachycardia.

Assess patient for fever, changes in

level of consciousness, poor skin
turgor, dryness of skin and mucous
membranes, pain.
Rationale: Symptoms may indicate
dehydration or hemoconcentration
with consequent vaso-occlusive
state.
 Complications
• Hypoxia, ischemia, infection, and poor wound healing leading to skin
breakdown and ulcers
• Dehydration
• Cerebrovascular accident (CVA, brain attack, stroke)
• Anemia
• Renal dysfunction
• Heart failure, pulmonary hypertension, and acute chest syndrome
• Impotence
• Poor compliance
• Substance abuse related to poorly managed chronic pain