College of Health Sciences

Nursing Division
WHO collaborating center for Nursing
Development

Thalassemia major

2010
 Objectives

 By the end of this class the students will be

able to:
- Define thalassemia
- Identify the incidence in Bahrain and worldwide
- Recognize types of thalassemia
- Explore the clinical manifestations in relation to
pathophysiology
- Identify the complication of thalassemia
 Objectives

- Identify the diagnostic tests done to diagnose

thalassemia

- Explore the collaborative management of

thalassemia

- Educate patients and their families regarding

thalassemia.

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 Incidence
 (hemolytic anemia 2007):
Male Female Total
2 1 3

 In day case center:

 19969-2008:
 Total of 106 cases

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 Types of thalassemia

 2 common types caused by decrease in alpha or

beta chain.
 Thalassemia minor or major

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 Pathophysiology
 Normal hemoglobin composed of 2 alpha and 2 beta
polypeptide chains.

 Genetically reduction in beta chain and high alpha

level

 Result in hemosiderosis

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 Pathophysiology-Cont’d

 Clinical affects attributed to:

1. Defective synthesis of HbA

2. Structurally impaired RBC’s

3. Shortened life span of erythrocytes

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 Clinical Manifestations

• Anemia

• DM

• Bone changes

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 Complications

 Splenomegaly
 Growth retardation & delay sexual maturation
 Cardiac enlargement
 Hepatomegaly
 Gallstones
 Folic acid deficiency

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 Diagnostic Test

 Electrolyte, Ca, Mg, ferritin, Sugar, oral GTT

 RBC:
 Low Hb, Hct:
 Hb electropherosis:
 HbF level

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 Medical Management

 Maintain Hb level

 Blood transfusion

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 Surgical Management

 Splenectomy

 Bone marrow transplantation

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 Pharmacological

 Desferal tablets (deferoxamine)

 Exjade:

 Folic acid

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 Nursing Management

 Promote compliance with transfusion and

chelation
 Assist coping with anxiety
 Observe for complications
 Explained about untreated iron overload
 Genetic counseling
 Teach how to give chelation

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 Health Education

 Chelation at home:

 Diet:

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 Health Education

 Improved self image:

 Prevention of infection:

 Follow up care:

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