Thalasemia patient

Thalassemia

Thalasemia patient

A.    Definitions

   Thalassemia is a disease anemia hemolitikdimana damage to red blood cells in the blood vessel so that the life erirosit be short (less than 100 days).Thalassemia is a hereditary disease hemolytic anemua recessive inheritance, molecularly divided into alpha and beta thalassemia, while clinically divided into thalassemia major and minor (Mansjoer, Capita Selecta Medicine, 2000: 497)

B.     Process pathology

   Postnatal normal hemoglobin consists of two alpha chains and beta polipeptide. In beta thalassemia, there is a decrease in part or in whole in the process of synthesis of beta chain of hemoglobin molecules, consequence of an increase in the compensatory process chain pensintesisan alpha and gamma chain production remains active, and lead to imperfections hemoglobin formation. Polypeptide that is not balanced is not very stable, permissible apart and destroys red blood cells which can cause severe anemia. To cope with hemolytic process, red blood cells formed in jmlah that much, or at least suppressed bone marrow with trannfusi process. Iron overload from transfusions as well as the addition of RBCs in the rapid destruction of cells defectif deposited in various organs (hemosiderosis)

C.     clinical manifestations

·   Lethargy

·   Pale

·   weakness

·   Anorexia

·   Diarrhea

·   Shortness of breath

·   Enlarged spleen and liver

·   mild jaundice

·   The thinning of the cortex of the long bones , hands and feet .

·   The thickening of the cranial bone

D.    Investigations

·   Laboratory tests of blood :

-          Hb : Hb 3-9 g %

-          Staining HR : Anisocytosis , poikilositosis , hypochromia weight , the target cell , tear drop cells.

·   description of the bone marrow eritripoesis hyperactivity

·   Hb Electrophoresis :

o   Thalassemia alpha : found Hb Bart 's and Hb H

o   Beta Thalassemia : Hb F varies between 10-90 % (N < = 1 % )

E.     The focus of the assessment

1. Physical Assessment

a.       perform a physical examination

b.      review of medical history, especially those associated with anemia (paleness, weakness, spasms, rapid breathing, hypoxia, bone pain, and chest, decreased activity, anorexia, epistaxis berlang)

c.       Assess family history.

2. Assessment of the common

a.       Stunted growth

b.      anemia of chronic

c.       Delayed sexual maturity.

3. vaso occlusive crisis

a.       Pain that is felt

b.      Perceived symptoms associated denganischemia related areas:

o   The extremities: the hands and feet skin peeling accompanied by pain that radiates.

o   Abdomen: ache

o   The cerebrum: troke, impaired vision.

o   Liver: obstruction, jaundice, coma hepaticum.

o   Renal: hematuria

c.       Effects of vaso occlusive crisis is:

·         Cast: cardiomegaly, systolic murmur.

·         Lungs - Pulmonary: Pulmonary function disorders, can easily become infected.

·         Kidney: The inability to break down the compound urine, kidney failure.

·         Genital: sore, tense.

·         · Liver: hepatomegaly, cirrhosis.

·         Eyes: Abnormalities that lead to impaired vision lens, sometimes causing disruption of retinal layers and can cause blindness.

·         Extremities: Changes in bone - the bone is mainly caused slouch, easily infected with Salmonella, Osteomyelitis.

F.      Nursing Diagnosis:

1. Changes in tissue perfusion b.d reduced cellular component that is essential to pure oxygen to the cells.

3. Changes in nutrition less than body requirements b.d lack of appetite.

4. Coping family inefektif b.d childhood diseases impact on the function of the family.