Question

Compare sickle cell anemia and β-thalassemia major in relation to pathophysiology and nursing care.

Answer 1

sickle cell anemia: it is a genetic disorder caused by the errors in the genes of the haemoglobin .

• it has sickle shaped erythrocytes.
• Pain experienced by the patients due to blockage of tiny blood vessels.
• pathophysiology: red cells with Hbs in oxygenated state bahave normally but in deoxygenated state it aggregares ,polymerises and distort the red cell to sickle .these sickle shaped cells block the micro circulation due to their rigid structure .thesr are having shorter life span and more fragile ,increased destruction leads to lmhaemolysis ,anaemia and jaundice.
• Nursing care: preconceptional counselling ,antenal care during pregnancy, prophylactic folic acid 1mg given daily ,iron supplementation ,blood transfusions up to 6 months
• treatment : bone marrow transplantation and hydroxy urea medication will help to stimulate the fetal hemoglobin
• thalassaemia major:it is also called as cooley anaemia .there is a red cell destruction as there is on beta cell production ,erythropoisis is ineffective.infabnt need repeated blood transfutions
• there is progprogressive hepato spleeno megaly ,impaired growth,anaemia ,ccf,infection.
• the chance of survival beyond teens uncommon .
• nursing care:
• preconceptional counselling .
• iv iron therapy contraindicated
• oral folic acid supplementation
• careful monitoring needed .