•The word thalassemia is derived from the
Greek words “Thalassa” means the great sea.
The disease was described by Cooley in 1925.
So other name for thalassemia is Cooley’s
• Thalassemia is a group of hereditary hemolytic
anemia characterized by reduction in the synthesis
• Thalassemia can be considered as hemolytic and
hypo proliferative anemia related to abnormal
1st noticed in patients originating from the Mediterranean Sea.
The prevalence of the disease in India is high among Gujaraties, sindhis, and
There are millions of people as carriers of thalassemia gene and every year
thousands of thalassemia children are born in our country.
6000-8000 new cases were detected every year in India.
7. • Thalassemia Major:
• Severe form of illness & associated with homozygous state.
Thalassemic genes are inherited from parents. There is malfunction &
failure of organs like liver, spleen, heart etc.,
8. Thalassemia Intermediate:
• It is a state of chronic hemolytic anemia caused by deficient alpha
or beta chain synthesis. This is a homozygous form.
• These patients may have chronic liver dysfunction, osteoporosis,
mild hepatomegaly & chronic anemia.
9. Thalassemia Minor:
• It is a mild form of illness produced by heterozygosity of either
alpha or beta chain. Asymptomatic form.
Facial- Frontal bossing
o Prominent maxilla
o Depressed bridge of nose
o Puffy eyes
o Malocclusion of teeth
Increased pigmentation skin
Congestive cardiac failure
Transfusion related infections (HIV, HB, HC)
Multi organ dysfunction
14. Diagnostic Evaluation:
1. Blood picture
o Hb percentage- decrease usually 2-6 gm/dl
o Reduced RBC count- 2-3 million/cmm
o Increased Hb-f
o Decreased HCT Values
o Increased reticulocyte count
o In peripheral smear RBC shows as- hypochromia, anisocytosis, poikilocytosis, microcytosis,
nucleated RBCs, and target cells.
o Decreased WBC count
o Increased platelet count
o Increased serum bilirubin level
o Increased serum iron level
15. Bone marrow shows- Hyper cellular & erythroid hyperplasia
Osmotic fragility test shows decreased fragility
o Widening of medulla
o Thinning of cortex
o Skull bones show hair on end appearance
o Coarsening of trabeculations due to bone marrow hyperplasia in the long
bones, metacarpals and metatarsals.
Repeated blood transfusion
• It is given at regular interval to maintain hemoglobin level at least 10-11
• Iron chelation therapy:
o Iron chelating agent desferrioxamine is recommended.
o Route- SC dose 25-50mg/kg/day in 2-3 dose.
o When the child need very frequent blood transfusion
• Folic acid supplements:
o Dietary iron is avoided so that folic acid supplementation given
• Supportive management:
o To treat complications
o Vaccination with Hepatitis-B is necessary to prevent transfusion related infection.
o Emotional support
• Bone Marrow transplantation
o Defective stem cells are replaced by normal stem cells.
18. Nursing Diagnosis:
Risk of infection related to anemia
Activity intolerance related to anemia
Chronic pain related to skeletal changes
Body image disturbance related to bony changes
Ineffective family coping related to poor prognosis
Knowledge deficit related to child care in long term chronic illness
with hemolytic anemia.
19. Nursing Interventions:
Assessment of child condition to prevent complications that can be
done as hospital based or community based
Preparation for repeated hospitalization
Arrange of necessary diagnostic measures
Administration of blood transfusion, iron chelating agent with
Emotional support to the parents & family members.