CLASS PRESENTATION

ON

THALASSEMIA

Presesnted by:
Amanda Kharsamai
Roll no- 02
MSc Nursing Ist yr
DEFINITION
Thalassemia is a hereditary disorder characterized
by a decrease in the synthesis of goblin chains
(alpha or beta).
Impaired goblin synthesis causes impaired
production of hemoglobin and eventually results in
a hypochromic microcytic anemia because of
defective hemoglobinization of RBCs
Weatherall, 2010
 INCIDENCE
Thalassemia is commonly found in members of ethnic groups whose
origin are near the Mediterranean Sea and equatorial regions of Asia, the
Middle East and Africa.
Carrier frequency varies from 3% to 17% in different populations, with
over 30 million people carrying the defective gene.
It is the commonest group of autosomal recessive disorder in India.
Over 9000 thalassemic children are born every year in the country.
Majority of the patients are severe tranfusion dependent; about 10% to
15% of cases have milder phenotypes.
An individual with thalassemia may have a heterozygous types or homozygous
form of the disease.
A person who is heterozygous has one thalassemic gene and one normal gene and is
said to have thalassemia minor which is a mild form of the disease.
A homozygous person has two thalassemic genes, causing a severe condition known
as thalassemia major.
 ETIOLOGY
 Thalassemia is due to decreased production of at least one globin
polypeptide chain ( beta, alpha, gamma, delta) which results in
unbalanced hemoglobin synthesis.
Beta thalassemia results from decreased production of beta-
popypeptide chains.
Alpha thalassemia is a result of decreased production of alpha
globins.
 PATHOPHYSIOLOGY

Anemia,
bone marrow
Globins in
expansion,
Decreased β excess
Imbalance extramedullar
or α precipitate Ineffective
between α and y
production of and damage erythropoiesis
β globins hematopoiesi
or globins the RBC
s, increased
membranes
intestinal iron
absorption
 NURSING MANAGEMENT
Nursing assessment:
Thalassemia major
Thalassemia intermediate
Thalassemia minor
Nursing diagnosis:
Ineffective tissue perfusion
Imbalanced nutrition
Activity intolerance
Ineffective family coping
Risk for infection
 PROGNOSIS
Patients with mild thalassemia can expect a normal life expectency.
Patients with moderate, or severe thalassemia have a good chance of long-term survival
as long as they follow their treatment program.
Heart disease from iron overload is the leading cause of death in patients with
thalassemia, so keeping up with the iron chelation therapy is extremely important.
A bone marrow transplant may cure thalassemia.
Patients with thalassemia may need surgery to correct skeletal problems.

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 COMPLICATIONS

Heart and liver diseases

Infection
Osteoporosis