Thalassaemia

Thalassaemia
Objectives:
1. Describe the medical aspects of thalassaemia
2. Assessment
3. Nursing diagnosis
4. Plan appropriate nursing care using the nursing process approach
5. Prepare to implement and evaluate the care.

Description : Thalassemia is an inherited blood disorder characterized by deficient synthesis of specific globulin chains of the hem globulin molecule . in the case of beta- thalassemia , the most common type , of beta-chains.
Thalassemia is an inherited autosomal recessive disease it occurs in the three major clinical forms : thalassemia major the homozygous form ( also know as cooley s anemia) , thalassemia intermediate , the homozygous form and thalassemia minor the heterozygous form.



Assessment findings:
1. Thalassemia minor commonly produces only mild to moderate anemia that may be asymptomatic and often goes undetected.
2. Thalassmia intermediate produces the following clinical manifestations , usually first noted within two years time .
a. The syndrome is clinically milder than thalassemia major .
b. Patients generally do not require blood transfusions and maintain a hemoglobin between 7 and 10g/dl
c. Marked hepatosplenomegaly , growth retardation , hyperbilirubinemia and iron overload occur.
d. Thalassemia major commonly produce the following clinical manifestation , pallor , poor feeding and protruding abdomen secondary to hepatosplenomegaly ,signs and symptoms of chronic hypoxia such as headache , lethargy , bone pain, anorexia, and exercise intolerance.



Nursing diagnosis:
1. Activity intolerance
2. Body image disturbance
3. Altered health maintenance
4. High risk for injury
5. High risk for infection
6. Knowledge deficit
7. Pain
8. Altered nutrition : less than body requirements