presentation ms1 (group 7) thalessemia
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Thalassemia
MEDICAL SURGICAL NURSING I
HNS 214
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ThalassemiaDefinitionInherited blood disorder that cause the body to
produce less hemoglobin.
Cause
One of more gene defected that passed from parents.
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Types
Alpha Thalassemia
Missing or damage of one or more Alpha Globins
of chromosome 16.
I. Silent carrier
II. Alpha Thalassemia Minor
III. Hemoglobin H disease
IV. Alpha Thalassemia Major
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Cont
Beta Thalassemia
Missing or damage of one or more Beta
globins gene of chromosome 11.
I. Beta Thalassemia Minor
II. Beta Thalassemia IntramediaIII. Beta Thalassemia Major
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Alpha Thalassemia
Silent carrier
One Alpha-globins gene defected.
Asymptomatic.
No need treatment.
Blood cell more small than normal cell.
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Cont
Alpha Thalassemia Minor
Two Alpha-globins gene defected.
Mild anemia.
No need treatment.
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Cont
Hemoglobin H Disease
Three Alpha-globins gene defected.
Moderate to severe anemia.
Need blood transfusion.
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Cont
Alpha Thalassemia Major
Four Alpha-globins gene defected.
Die shortly after born.
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Beta Thalassemia
Beta Thalassemia Minor
One of Beta-globins gene defected.
Mild anemia.
No need treatment.
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Cont
Beta Thalassemia Intramedia
Two of Beta-globins gene defected.
Moderate anemia.
Need blood transfusion.
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Cont
Beta Thalassemia Major
Two of Beta-globins gene defected.
Severe anemia.
Cant live until adulthood without treatment.
Live with blood transfusion since child. Maybe death due to damage organ such as heart or
liver.
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Clinical Manifestation
Fatigue
Weakness
Light headaceness
Slow growth and delay puberty
Short of breath during exercise
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Diagnosis
Complete Blood Count ( CBC )
Amount of RBC, WBC, Platelet and Plasma.
Gene TestDNA comparison between patient and parents.
Iron Serum Test
Identify the level of iron.
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Cont
Hemoglobin electrophoresis
Detect or identify the abnormal Hb in blood. Quantitative G6PD
Quantity of enzyme and protein in blood.
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Cont
Prenatal testingcan be done before a baby is born to find out if he or she hasthalassemia and determine how severe it may be. Tests used todiagnose thalassemia in unborn babies include:
Chorionic villus sampling. This test is usually done around the11th week of pregnancy and involves removing a tiny piece of the
placenta for evaluation.
Amniocentesis. This test is usually done around the 16th week ofpregnancy and involves taking a sample of the fluid that surrounds
the baby. Fetal blood sampling. This test can be performed after 18 weeks of
gestation and involves taking blood from the fetus or the bloodvessels in the umbilical cord using an ultrasound-guided needle.
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Treatment
Depend on the type and sign and symptom of
Thalassemia.
Mild anemia
No need treatment.
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Cont
Moderate anemia
Folic acid supplement.
Help in build healthy RBC.
Blood transfusion
Supply healthy RBC and normal Hb.
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Cont
Severe anemia
Regular blood transfusion.
Every 2 4 weeks.
Folic acid supplement.
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Complication
Iron overload
Infection Bone Deformities
Enlarged spleen
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Risk for infection
related to invasive procedure
Intervention
Assess the puncture site for any
inflammation.
Assess any sign and symptom of
infection. (V.S)
Rational
Inflammation is a normal
reaction of body for infection.
To detect any abnormality.
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Intervention
Maintain aseptic technique
when do any procedure to
patient.
Cover the puncture site after
procedure.
Administered medication asordered by doctor such as
antibiotic for bacteria infection
and antipyretic for fever.
Rational
To prevent any cross infection
between patient and hospital
staff.
To prevent expose to
microorganism.
To kill bacteria and relief thefever.
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Fatigue related to Disease
ProcessIntervention
Assess patient condition by
seeing physical activity.
Assisting patient by daily
activity example giving bed pan.
Encourage patient rest in bed.
Rational
To know any abnormality for
next intervention.
Easier patient to void and
reduce fatigue to ambulation.
To restore back the energy.
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Intervention
Instruct client how to use call
bell and ask client to press it if
required.
Make sure the client bed is not
to high.
Rational To reach immediate help from
staff nurse.
Easier client to move and
prevent any injuries.
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Low Self Esteem related to
inherited diseaseIntervention
Assess the cause of low self
esteem and acceptance of
patient for the disease.
Establish relationship with
patient and provide and
opportunity for patient to
verbalize feelings.
Rational
To understand patient condition
and guide intervention.
As a therapeutic therapy.
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Intervention
Provide treatment for patients
disease blood transfusion.
Encourage family and peer
support.
Refer patient to supportive
community.
Rational
Treat cause to build patients
self esteem.
To build patients confidence
and feel of acceptance.
Help patient to deal with
embarrassment and learncoping mechanism.
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Thank You
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