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    Thalassemia

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    Amanda Scarlet
    Thalassemia is a hereditary blood disorder characterized by decreased production of hemoglobin chains. It is commonly found in populations near the Mediterranean, Middle East, Asia, and Africa. Thalassemia major results from two defective genes and requires regular blood transfusions, while thalassemia minor only has one defective gene and is milder. Nursing care focuses on managing anemia, chelating excess iron, preventing infections, and supporting nutrition and family coping. With treatment, patients can have a normal life expectancy, though heart disease from iron overload is a leading cause of death if chelation therapy is not followed.

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    Thalassemia

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    Amanda Scarlet
    2K views10 pages
    Thalassemia is a hereditary blood disorder characterized by decreased production of hemoglobin chains. It is commonly found in populations near the Mediterranean, Middle East, Asia, and Africa. Thalassemia major results from two defective genes and requires regular blood transfusions, while thalassemia minor only has one defective gene and is milder. Nursing care focuses on managing anemia, chelating excess iron, preventing infections, and supporting nutrition and family coping. With treatment, patients can have a normal life expectancy, though heart disease from iron overload is a leading cause of death if chelation therapy is not followed.

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    Thalassemia is a hereditary blood disorder characterized by decreased production of hemoglobin chains. It is commonly found in populations near the Mediterranean, Middle East, Asia, and Africa. Thalassemia major results from two defective genes and requires regular blood transfusions, while thalassemia minor only has one defective gene and is milder. Nursing care focuses on managing anemia, chelating excess iron, preventing infections, and supporting nutrition and family coping. With treatment, patients can have a normal life expectancy, though heart disease from iron overload is a leading cause of death if chelation therapy is not followed.

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    Download as pptx, pdf, or txt
    2K views10 pages

    Thalassemia

    Uploaded by

    Amanda Scarlet
    Thalassemia is a hereditary blood disorder characterized by decreased production of hemoglobin chains. It is commonly found in populations near the Mediterranean, Middle East, Asia, and Africa. Thalassemia major results from two defective genes and requires regular blood transfusions, while thalassemia minor only has one defective gene and is milder. Nursing care focuses on managing anemia, chelating excess iron, preventing infections, and supporting nutrition and family coping. With treatment, patients can have a normal life expectancy, though heart disease from iron overload is a leading cause of death if chelation therapy is not followed.

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    © All Rights Reserved
    Download as PPTX, PDF, TXT or read online from Scribd
    Download as pptx, pdf, or txt
    of 10

    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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    throughout her career.
    COMPLICATIONS

    Heart and liver diseases


    Infection
    Osteoporosis

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