Thalassemia – Nursing Diagnosis and Treatment

Thalassemia
Thalassemia

•    Autosomal recessive disorder characterized by deficiency in the synthesis of globin chains.
•    Types
–    Thalassemia minor (trait) : Heterozygous
–    Thalassemia intermedia      : Manifests with splenomegaly and severe anemia
–    Thalassemia major (Cooley’s anemia) : Homozygous form, can not survive  without regular blood transfusions

Assessment of Thalassemia

•    Skin
•    Heart
•    Spleen
•    Liver
•    Pancreas
•    Lymph nodes
•    Endocrine
•    Skeletal
•    Bronze skin, freckles from deposition of Iron, jaundice
•    Cardiac failure from myocardial fibrosis, and prolonged transfusion therapies
•    Enlarged spleen, abdominal distention and breathing difficulty
•    Fibrosis, increased absorption of iron from GIT leading to increased iron stores
•    Fibrosis, DM
•    Enlarged lymph nodes
•    Delayed or absent sexual maturation, absence of secondary sexual characteristics, endocrine abnormalities
•    Bone marrow expansion, frontal and parietal bossing, prominent cheek bone, enlargement of maxilla, protruding upper lip and incisors, malocclusion, osteoporosis, pathological fractures

Stressor

•    Heredity
•    Spontaneous mutation
•    Homozygous form of the disease
•    Pathologic fractures
•    Changes of facial bone structure
•    Discoloration of skin due to iron deposits
•    Severe anemia
•    Absence of β-chain synthesis in Hb production
•    Hyperplasia of bone marrow
•    Negative body image
•    Autosomal recessive disorder

Treatment of Thalassemia

•    Regular blood transfusion to keep Hb at 10.5 g/dL
•    Manage fluid overload
•    Iron chelating therapy (Desferral)
•    24 h urine collection after chelating therapy to estimate the amount of iron excreted
•    Reassure the orange color is normal

Nursing diagnoses for Thalassemia

•    Activity intolerance R/T impaired oxygen transport
•    High risk for infection R/T decreased resistance secondary to hypoxia
•    Altered body image R/T skeletal changes
•    High risk for altered health maintenance R/T lack of knowledge

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