Thalasemia

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حمزة الحرايزة
جامعة العلوم والتكنولوجيا الاردنية - اربد
كلية التمريض..قسم تمريض صحة الاطفال
مناقشة مرض الثلاسيميا والانيميا
اعداد حمزة الحرايزة
الدكتورة هدى غرايبة

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Thalasemia

  1. 1. Prepared By HamzeH HarayzeHjust Uni
  2. 2. Outline-Introduction.-Sickle Cell Anemia.-Hemoglobin electrophoresis.-sickle cell crises.-Nursing care for cickle cell anemia children.-Thalassemia.-Minor Thalassemia.-Major Thalassemia.-Nursing care for Thalassemia children- conclusion.-Article.
  3. 3. ObjectiveAfter we have finshed presentation, you should ableto:-Describe and discuss sickle cell anemia and andknow signs and symptoms and nursing care for pt.-Assess child with sickle cell crises.-Know thalassemia classification and managementtherapeutic.-Identify expected outcome for child withthalassemia major.-Formulate nursing diagnosis for a child with cicklecell anemia and thalassemia disoders.
  4. 4. Sickle Cell Anemia
  5. 5. IntroductionSickle cell anemia is a severe chronic anemia in people having abnormalhemoglobin called hemoglobin –S(sickle(.People who are homozygous for the gene will be affected and those whoare heterozygous will be carriers of disease.Patho physiologyWhen the sickle hemoglobin loses its oxygen , it forms rigid rods calledpolymers that change the red blood cells into a sickle shapethese abnormal shaped cells are both rigid and sticky. They stick to thewalls and cannot squeeze through the capillaries. Thus blood flowbecomes slow or stopped in small blood vessels. In the immediatesettings, hypoxia causes severe pain , but overtime it leads to gradualdestruction in the organs and tissues. Cell dehydration is another factorin sickling process.Sickle cells have a short life span of 10-20 days compared to the 120days in normal cells. This causes a drop in the number of cells resultingin anemia.
  6. 6. But Why.. !!Most commonly affected populations arefrom:o Africao Mediterraneano Indiao Middle East
  7. 7. -sickle-cell anemia is the presence of abnormally shapedRBCs.Autosomal recessive inherited defect of Beta chain Hb-Erythrocytes becomes elongated and crescent in shape(sickle(when-submitted to low o2 tension.. with low bld ph acidosis andincrease bld viscosity … such as occurs dehydration or hapoxia.So RBCs sickle, they don’t move freely through vessels.Stasis~~~> sickle cell crisesNO Clinical symptoms for anemia until chlid reach 6 month at his age..Why..??Fetal hb contains a gamma, not a beta chain,the ds usually will notresult in clinical symptoms until the childs hb change from the fetalto adult from at approximatly 6 months.However it can diagnosed during prenatal life(chorionic villi samling &cord bld during amiocentesis(
  8. 8. AssessmentHemoglobin electrophoresis(screening test(-some infant have swelling of the hands and feet(hand-footsyndrome)~~~caused by~~~~~>aseptic infraction of the bones inhands and feet.-slight build and ch-ch long arms and leg.-protruding abdomen ~~~~>( enlarge in spleen & liver).-pneumococcal meningitis,osteomyelitis become frequent illness.-lever enlarge bcoze bld stasis….(liver cirrhosis)- ↓kidney function- ↓vision bcoz occlusion of bld vessels in small retinal.( So Regular eyeexam necessary for pt child).
  9. 9. Sickle cell crisis-this term used to denote sudden ,severe onset of sickling~~~> it’soccur from pooling of many new sickled cells in vessels andconsequent tissue hypoxia beyond the blockage .( vaso-occlusivecrisis).~~~>(The vaso-occlusive crisis is caused by sickle-shapedred blood cells that obstruct capillaries and restrict blood flow toan organ, resulting in ischemia, pain, necrosis and often organdamage. )Can occur when the child has an illness causing dehydration orrespiratory infection (↓ O2 level ) or from strenous exersize.-Aspetic necrosis of the head of femur or hummers increase jointpain-. -- Hb in Lab Result ~~~> 6 – 8 g|100ml …(wat’s hbnormal v(-WBS count elevated to 12.000- 20.000mm3.- ↑Bilirubin level.-Comma.-seizures.-loss motor fuction.-Hematuria-Mybe Death.
  10. 10. Theraputic ManagementThe child with sc crises has three primary needs: pain relief andadequate hydration and o2 to prevent further sickling and halt thecrises.-Acetaminophen (Tylenol)~~~> may be adequate pain relief for somechildrenFor others we have to use:-Narcotic analgesic~~~> Iv morphine.Wen pt child has a pain free.he able to relax.reducing the metabolicdemand for o2 and helping to end the sickling.Hydration is generally accomplished with intensive intravenous fluidreplacment thearpy….Tissue hypoxia~~~>acodosis.The acodosis must be corrected by electrolyte replacment.
  11. 11. Kidney infraction ~~~> so don’t adminster potassium intravenously untilkidney function has been determined (the child is voiding)Excessive potassium levels maybe occur ~~~~>cardiac arrhythmias-if infection appears to be the precipitator for sickling crises,bld andurine culture,chest x-ray,and comlete bld count will be taken and theinfection will be treated by antibiotics…and have to use bldtransfusion to maintain hb above 12gdl.
  12. 12. NursingCareASSESSMENT and nursing intervention-Assess if pt child understand if he will need to remain in bed..bcoz inmost cases with cs crises need to hospital and restrict to bedrest..Bed rest reduce the need to o2 in body cells.-Assess degree of child pain and give prescribed narcotic as requried•Pain scaleso FLACC: nonverbal and young children, usually < 3 years oldo Faces: 3-7 years oldo Numeric scale (0-10): 8 years and older (must understand rank orderandnumeric value)-Assess o2 sat by pulse oximetry..maybe we have to use face mask tokeep o2 saturation above 95%.-Mybe we have to give child folic acid as Dr order.-Intake&Output-Vs
  13. 13. Nursing Dx-Ineffective tissue perfusion rt generalized infract due sickling.-Ineffective health maintenance rt lack of knowledge regarding long-term needs to child with sickle-cell anemia.-pain rt chronic physicl disabilty.-Risk for infection rt chronic disease and splenic malfunction..Activity intolerance rt the decreased oxygen carrying capacity of theblood.Interrupted family process rt frequent medical care, and chronicillness
  14. 14. Thalassemia-The thalassemias are autosomal recessiveanemias associated with abnormalities of thebeta chain of adult hemoglobin (Hgba(.}Inherited disorders of Hb production{-Production of the protein portions(globin( of theHb molecule is ↓..and the globin protein itself isabnormal.Also Rbcs are small ..more pale fromand fragile and early destruction.
  15. 15. -thalassemias classified to main groub1-Thalassemia Minor ..{Heterozygous Beta-Thalassemia{.2-Thalassemia Major ..{Homozygous Beta-Thalassemia{
  16. 16. Thalassemia Minor-Children with thalassemia minor~~> mild form of thisanemia.Produce both defective beta Hb and normal Hb.Becausethere is some normal production,the RBC count will benormal.but the Hb concentrattation will be ↓ 2 to 3g100ml below normal levels.Rbc =hypochromic and microcytic~~>bcoz poorHb formation.-Children with minor thalassemia may have nosymptoms.They require no treatment, and lifeexpectancy is normal.And no iron supplement.-Thalassemia widespread in mediterranean sea andmiddle east ,african and asian heritage.
  17. 17. Major Thalassemia-It’s much more serious condtion..it’s calledto cooley’s anemia or Mediterraneananemia.becoz thalassemia is a beta chainHb defect.symptoms do not become apparent until thechild’s fetal Hb has largely been replacedby adult Hb during second half of the firstyears of life.
  18. 18. Signs and Symptoms-Pallor-Arritabilty-Anorexia-Rbcs are hypochromic(pale(..andmicrocytic(small(.-Fragmented poikilocytes and basophilic stippling.-Hb level is less 5g100ml-The serum iron level high …~~~> why…??
  19. 19. Body organ Effect of abnormal cellproductionBone marrow Overstimulation of BM leads to ↑facial-mandibular growthSkin Bronze-colored fromhemosiderosis and jaundiceSpleen SplenomegalyLiver and gallbladder Cirrhosis and cholelithiasisPancreas Destruction of islet cells anddaibetes mellitusHeart Failure from circulatory overloadEffects of Thalassemia Major
  20. 20. Assessment-Change in the shape skull-protrussion of the upper teath(marked maloccusion maloccusion(.-Base of the nose may be broad and flattened.-Eyes may be slanted with epicanthal fold(as down syndrome(.-X-ray show marked osteorporotic(of lessened density( tissue,possiblyresulting in fractures.-Abdominal pressure from the enlarged spleen may cause anorexiaand vomiting.-Arrhyhamias and HF are frequent causes of death.-Hepatosplenomegaly
  21. 21. Management-Digitalis,diuretis and low sodium diet …~~> why-Transfusion of packed RBCS every 2 to 4 weeks (hypertransfusionthearpy) will maintain hemogloubin between 10 and 12 g100ml-Iron chelating agent to remove the excessive store of iron such asDeferoxamine given subcutaneously over 6 to 8hours as they sleepnight.-Splenectomy to reduce discomfort and reduce the rate RBChemolysis and the number of transfusions needed.-Bone marrow stem cell transplantations can offer cure.
  22. 22. Nursing Care And Dx-Risk for situational low self-esteem related to changedphysical appearance.*child states he can accept altered appearance andinteracts with peers.Children with thalsssamia major may have delayed growthand sexual maturation. they usually develop markedchange in facial appearance because of the overgrowthof marrow-producing centers of facial bones.Child who receives frequent bld transfusion may developsuch hemosiderosis that his or her skin appears bronzein color.
  23. 23. Nursing daignosis for thalsassemia ds:*Deficient knowledge (treatment regimen)* Delayed growth and development* Disturbed body image* Ineffective tissue perfusion: Cardiopulmonary* Interrupted family processes* Activity intolerance* Risk for infectionNursing Goal for ThalassemiaPatient and parents will:Demonstrate age-appropriate skills and behaviors to theextent possible.* Express positive feelings about himself.* Develop no cardiac arrhythmias.* Voice feelings and concerns related to the patient’s illness.* Verbalize the importance of balancing activity, as tolerated,with frequent rest periods.* Remain free from signs and symptoms of infection
  24. 24. Nursing interventions for Thalassemia*Provide an adequate diet,* Encourage the patient to drink plenty of fluids.* Provide emotional support* Help the patient and his family cope for chronic nature ofThalassemia* Watch for adverse reactions during and after RBC transfusions.* Collaborative an antibiotic, and observe the patient for adversereactions.* Explain the need for lifelong transfusions.
  25. 25. conclusion-Thalassemia children with treatment, theoverall progonsis of thalassemia isimproving but still grave,most children withthe disease fie of cardiac failure duringadolescence or as young adults if theydon’t recive a marrow transplant.
  26. 26. References-Maternal and chold Health nursing Textbook (fifthedition) lippincott williams&wilkins.-www.kidshealth.org-www.thalassaemia.org

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