THALASSEMIA

Presented by :
Smt. Priya B. shinde,
M.Sc. Nursing 1st Year
D.Y. Patil College of Nursing,
Kolhapur
PATHOPHYSIOLOGY
of
thalassemia
 Absence / reduce or β globin

Impaired α - β globin ratio

Affect red cell pathology

Haemolysis Ineffective
erythropoiesis
Anaemia

Erythropoietin Iron absortion Blood

increased increased transfusion
Bone marrow
expansion Iron loading
Skeletel changes Heart failure
hypermetabolic
 Types
of
Thalassemia
 Types according to protein chain
• Alpha Thalassemia
• Beta Thalassemia
Thalassemia patient
 MEDICAL
MANAGEMENT
1.Repeated Blood Transfusion
 10 to 15 ml/kg every 2 to 3 weeks
2. Iron Chelation Therapy
Iron chelating agent desferrioxamine
(Desferal)
25 to 50 mg/kg/day over a period of 8
hours to 12 hours (subcutaneous infusion)
Deferiprone 75 to 100 mg/kg/day in 2 to
3 divided doses.(Orally)
3. Folic acid supplementations
 To prevent more iron deposition
4.Supportive Management
 Treats complication.
 Hep B vaccination
 Basic supportive nursing care.
 surgical
MANAGEMENT
Bone Marrow Transplantation
 New
Approaches
• Gene therapy and gene manipulation.
• In gene therapy insertion of normal gene is
done in the stem cells to to underlying defect.
 Nursing
Management
• Early assessment
• Preparation of child for diagnostic
procedures
• Care during blood transfusion
• Administration of chelation therapy
• Prevention of infection
• Education and Support of parents and
child
Preventive
Measures
• Antenatal screening
• Genetic counselling
• marital counselling
 Prognosis
 The outcome depends on the severity of
disease.
 Thalassemia minor may lead near normal life
 If the child has Thalassemia major, he
requires frequent blood transfusion and
chelation therapy. With chelation therapy the
child can survive for up to 30 years of age.
 Death usually occurs from cardiac
complications during 2nd decade of life.im
 ASSIGNMENT

Q.1 Short answer question (10 marks)

1. Define thalassemia.
2.write types of thalassemia & clinical manifestation of
thalassemia major.

Q.2 Long answer question

(10 marks)
1. Explain in brief about the management of thalassemia.