Peer Group Presentation Subject — Medical Topic — T Presented by Mr. Hari Singh Nagar M.Sc Nursing 1*' year Page 7Thalassemia Introduction - It is a group o associated with defective production of one or more i in in the Hb molecules is reduced * Hypochromia (hypo 5, and’Ghromia means — color that means anabnc ecrease in the Hb conte PRBC + Extreme Microcytosis (small then normal RBCs), * Haemolysis (destruction of blood elements) . Page 2Thalassemia Incidence - Thalassemi approximately 4.4 of ever: births throughout the wot that 50% of the patie 35. At that age, 50% patients from an udy were still alive. Page 3Thalassemia Normal Thalassemia Malformed red blood cell _<_— 6 . ite é , ® ite el 0% @ White ood cer aie ee Blood Cell * i) 7Hemoglobin Structure i Four subunits ‘ff two a ij / two 8 om yj red blood call Bchain helical shape of the polypeptide moleculeHEMOGLOBIN Hemoglobin molecule lron 9 (the iron is the site e $Y of oxygen binding) Bs min: i 7 des, ] : h ir i ee Cy Ler Red Oxygen blood cells moleculeNormal level RBC - Male — 4.5-6.2 millio +t (unconjugated) — 0.1-1gm/dl Total — less then 1.5gm/dl erum iron — Male = 65-175mcg/dl Female = 50-170meg/dl Page 7Definition It is the form of inherited b results in improper oxyg destruction of RBCs wh Page &Types Haemoglobin made up of two part * Alpha globins * Beta globins Thalassemia occurs when there i efect in genes that helps control prod Q proteins. Ther of thalassemia. A C1 en a genes related to the alpha AObInS 8 are sary or changed (mutated). . Beta — it occurs when similar gene defect affect production of the B-globin protein. Page 9Both A & B thalassemia including the followin two forms — . Thalassemia major — yo defect from both parents major. It is also called as'Co . Thalassemia minor ccurs if you receive the Page 10Pathophysiology Due to E/F a disturbance of ratio between the alpha & beta globin chain synihiesis then decrease production of one or more globin chains formation of abnormal Hb structure ineffective erythropoiesis excessive RBC destruction Iron overload extramedullary hematopoiesis ‘Thalassemia Page 12Clinical manifestation 1. Thalassemia minor - vho 2 inherited the defective gen a chain of Hb usually don’t sho because the alpha ger 2ctdd€sn’t cause any major problen erfere swith the Hb Page 13Clinical manifestation mild anaemia. The Hb completely normal to normal. If it is oce greater chances a more serious Page 142. Thalassemia major People who have inherite chain of Hb from both t thalassemia major, also Cooley's anaemia. The usually seen within th Anorexia, jaundice Delay growth & puberty due to anaemia. Page 15Weak bone — abnormal facial bone Infection iron overload Enlargement of spleen, Small head Epicanthai folds Smooth — phittrum PADAM Page 16Thalassemia facial bone abnormalities. These changes include bossing of the skull; hypertrophy of the maxilla, exposing the upper teeth; depression of nasal bridge; and periorbital puffiness B-Thalassemia major. Note the pallor, short stature, massive hepatosplenomegaly, and wasted limbs in this undertransfused case of B- thalassemia majorDiagnostic evaluation * History * Physical examination * Blood test - CBC, mic abnormal ae utational analysis — it can be done when results of Hb electrophoresis are inconclusive. Page 19Management It is treated with regular blood trai chelation therapy. 1. Blood transfusion — it i major thalassemia. It is required i 2-3 weeks to supplement RBCs and around 9gmldL. 2. Iron chelation th increase, the condition is called iron overload. Which can create complication and damage other healthy organs include heart & liver. To prevent iron overload medicines are prescribed. Page 20Deferoxamine & wefirasirean are common iron . Supplements — folic acid given to enhance the producti . Surgery Bone marrow tran: ri ed who required ansfusion (assist in relieving the , fcbdominal pressure & increase the life span of supplemental RBCs). Instruct the patient to report sign of infection because of the risk of sepsis. Page 21Complication + Heart disease + Liver disease Endocrine problems Page 22Nursing management 1. Nursing diagnosis — ineffective tis to reduce cellular component: Nursing diagnosis — activi imbalance of oxygen supp evidence by fatigue. Nursing diagnosis — j requirements.re Page 23Nursing management 1. Nursing diagnosis — ineffective tis frequency and B.P. * Provid g Provide oxygen therapy to patients. Monitor vital sign. Observe any restlessness and confusion. Page 242. Nursing diagnosis — activity intolerance related to evidence by fatigue. Nursing goal — To improve Intervention - Assess the ¢ Provide frequent rest periods to reduce oxygen consumption. To administer blood products. Page 25.. Nursing diagnosis — imbalance nutrition le requirements related to lack of appetite weight and skin integrity. Nursing goal — To maintain the Intervention : Allow to eat food that ca io improve the nutritional quality ai Provi 1 ich include high caloric, high protein, vitamin and minerals. Check the weight daily. Provide IV fluid if not able to take by mouth. Page 26Recent study regarding the thalassem thalassemia major has/sig increased in recent years, several groups in dif at quality of life. In a tecent study from the United Kingdom, it was found that 50% of the patients had died before age 35. Page 27Recent study regarding the Thalassen Italian long-term study Heart disease is responsi half of the deaths. rate sities cations are appearing in long- erm survivors. Iron overload of the heart remains the main cause of morbidity and mortality. meeBibliography “Suddarth’s & burnner” tex’ surgical nursing, twelth edi Page no. 925-926 “Saunders” comprehensive RN examination, fifth ed page no. 522-523 sscribed.com www.webmd.com Page 29