Transcript Sickle Cell Anemia

Sickle Cell Anemia
Roxbury Community College
ADN 253
Honors Presentation
Adanna Uwandu, Shadia Laurent, Salwa Said
05/01/07
Objective
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To apply the nursing process to care for patients
and families with sickle cell anemia.
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To learn the community resources available to
patients and families with sickle cell anemia and
sickle cell trait.
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To provide care to:
-individuals with the disease
-individuals planning to have a family.
Definition of Sickle Cell
Anemia
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It is an autosomal recessive inherited disorder affecting the beta
chain of hemoglobin
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It is a congenital hematological disease.
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Hemoglobin:
-iron-containing portion of RBC
-allows RBC to carry O2 from the lungs to tissues
-normal levels in g/100mL (p.1383):
*Birth; 13.7- 20.1
*3 mths 9.5-14.5
*adult levels 11-16
Pathophysiology
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Erythrocytes (RBC) become elongated and crescent-shaped
(sickled) when they are submitted to
-low O2 tension/levels (< 60%-70%)
-a low blood pH (acidosis)
-increased blood viscosity (thick blood)
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Dehydration and hypoxia can trigger these effects on the
blood
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Sickle cells can accumulate in capillaries and smaller blood
vessels causing occlusions, impair normal circ., tissue
infarctions (tissue death), swelling and anoxic changes.
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Defective hemoglobin molecule is produced called
Hemoglobin S.
Differences in Red Blood Cells
Pathophysiology
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Sickled RBC’s are thick and clump together.
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RBC’s do not move freely through the blood
vessels causing stasis and further sickling to
occur.
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Blood flow stops and the tissue distal to the
blockage becomes ischemic thus causing:
acute pain, cell destruction.
Fetal Hemoglobin
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Is present until about six months and the child with
sickle cell anemia will not usually have clinical
symptoms before such time.
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Fetal hemoglobin changes to adult hemoglobin
around 6 mths.
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Can be diagnosed in utero by
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Child with sickle cell disease has hemoglobin SS,
and produces no normal hemoglobin and thus
shows characteristic symptoms of the disease.
Sickle Cell Trait
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25-50% of the hemoglobin produced is
abnormal.
Patients who are carriers (heterozygous),
can pass the gene to their offspring.
It is the benign type of SC disease.
Their normal hemoglobin outnumber the
abnormal hemoglobin thus; they have
Assessment

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Hemoglobin electrophoresis: few drops of blood that have
converted to their adult form
Show signs ~ 6mths of disease
- fever, anemia, stasis of blood and infarction > local disease.
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Other signs incl:
-hand-foot syndrome
-slight built/thin, long arms & legs
-protruding abdomen, atrophic spleen in adolescence > inc risk
for infection, prophylactic antibx given
Assessment
-chest syndrome ~ pneumonia occurs, enlarged liver
> cirrhosis from infarctions and scarring tissue.
-dec kidney function, yellowed sclerae
-dec vision in children, from small retinal occlusions,
regular eye
exams.
-priapism
Sickle Cell Crisis
Definition: Sudden, severe onset of sickling.
Types: sequestration crisis, aplastic crisis, megaloblastic crisis
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Symptoms result from:
-vaso-occlusive crisis (pooling > tissue hypoxia past the
blockage point.
Triggers include:
- dehydration, respiratory infection, lowered O2 exchange,
dec arterial O2 level
Sickle Cell Trait