Thalassemia is an inherited disorder characterized by abnormal hemoglobin synthesis resulting from a defect in a gene controlling globin protein production. It causes hypochromic anemia and affects thousands of children born in India each year, being most common in those with Asian, Mediterranean, or African ancestry. Treatment involves regular blood transfusions to maintain hemoglobin levels along with chelation therapy to remove excess iron from previous transfusions.

1. THALASSEMIA
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2. OUTLINE
 Introduce the topic
 Define thalassemia
 Incidence
 Risk factors
 Etiology
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 Types of Thalassemia
 Pathophysiology
 Clinical Manifestation
 Diagnostic Evaluation
 Management

3. DEFINITION:
“Thalassemia is a inherited chronic disorders, characterized by absence or
decreased synthesis of one or more globin chains of haemoglobin.”
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4. INCIDENCE
About more than 9000 thalassaemic children
are born every year in country.
It was named so, because the disease had
highest incidence among people living around
Mediterranean sea like Italians, Greeks, and
Syrians.
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5. RISK FACTOR:
-Family history of the disorder.
-Asian, Chinese, Mediterranean, or African
American ethnicity.
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6. ETIOLOGY
Hb is made up of two proteins: Alpha globin, Beta
globin.
Thalassemia occurs when there is a defect in a gene
that helps in control production of one of these
proteins. (abnormal Hb synthesis).
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7. DIAGNOSTIC EVAUATION
Physical examination
History collection
Haemoglobin estimation
Complete blood count
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 Bone marrow
examination
 Serum Iron and serum
bilirubin level
 Amniocentesis (foetal
diagnosis)

8. MEDICAL MANAGEMENT
MEDICAL MANAGEMENT :( Supportive therapies)
1.Transfusion therapy
2.Chelation therapy
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9. MEDICAL MANAGEMENT
Transfusion therapy:
Blood transfusion is the mainstay of managing thalassemia. Children who
cannot maintain a haemoglobin level of about 7 g/dL, should receive
regular transfusions to maintain haemoglobin level between 10-12 g/dL.
Chelation therapy:
Rapid haemolysis and repeated blood transfusions result in iron overload.
Excessive iron deposits in various organs leading to multiple organ failure.
Chelation therapy is used to excrete excessive iron an urine.
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10. SURGICAL MANAGEMENT:
Bone marrow transplantation (especially in children).
Splenectomy: Splenectomy may be done to decrease transfusion
requirements (because RBC's may be withdrawn in spleen, if iron
supplements used during blood transfusion).
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11. NURSING MANAGEMENT:
Assessment:
- Assess client's skin, heart, spleen, liver, pancreas, lymph nodes,
endocrine, skeletal, etc.
- Heredity.
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12. NURSING DIAGNOSIS:
• Risk for infection related to decreased resistance secondary to hypoxia.
• Impaired nutritional pattern less than body requirement related to inadequate nutritional intake and
anorexia.
• Activity intolerance related to impaired oxygen transport.
• Disturbed body image related to skeletal changes.
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13. NURSING INTERVENTION:
- Assess the client's general conditions.
- Monitor vital signs.
- Monitor intake output.
- Manage fluid overload.
- Monitor regular blood transfusion to keep Hb at 10.5 gm/dl.
- 24 hours urine collection after chelating therapy to estimate amount of iron excreted.
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14. SUMMARY:
It is an autosomal recessive disorder which is
characterized by absence or decreased synthesis of
one or more globin chains of haemoglobin.
The patients show a variable degree of hypochromic
anaemia, with evidence of haemolysis and
ineffective erythropoiesis.
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