hematology 1
DESCRIPTION
blood disordersTRANSCRIPT
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Pediatric Hematological DisordersWhaley and WongChapters 35, 36
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Components of the BloodBlood:Plasma water, albumin, electrolytes, clotting factorsCellular ComponentsRBCs, WBCs, PlateletsAll formed in the red bone marrow (after birth)In utero- spleen, thymus, liverlymphatic system regulates maturation
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ErythrocytesRBCs carry hemoglobin which is attached to oxygen- provides O2 to the tissueslife span 120 daysmanufacture regulated by erythropoetinNormal Hematocrit- 35-45%Normal Hemoglobin- 12-16 grams
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Problems of Erythrocyte ProductionAnemia reduction of RBC volume or Hgb concentration below normal Classifications: 1. Etiology/Pathophysiology causes of RBC/Hgb depletion 2. Morphology changes in RBC size, shape, and color
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Causes of AnemiaNutritional deficiency iron, folate, B12Increased destruction of RBCs sickle cell anemiaImpaired or decreased rate of production aplastic anemiaExcessive blood loss - hemophilia
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Iron Deficiency AnemiaCauses - inadequate supply of iron - impaired absorption - blood loss - excessive demands for iron reqd for growth - inability for form Hgb
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Iron Deficiency AnemiaSigns and Symptoms: due to tissue hypoxia > lack of energy, easy fatigability, pallorDiagnosis: CBC with diff, red cell indices (MCV, MCH, MCHC), iron studies, physical examMedical Treatment: supplement with ferrous sulfate (dosages vary with age), dietary counseling
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Iron Deficiency AnemiaNursing Assessment and Interventions: - educate parents about nutrition - explain laboratory testing - teach parents proper administration of iron preparations, caution about high toxicity of iron
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Sickle Cell AnemiaCauses: genetic transmission, 2 parents with the trait have 25% chance of having child with SCD, found primarily in Blacks, occ HispanicsHgb A is partly or completely replaced by Hgb SWith dehydration,acidosis, hypoxia, and temp elevations, Hgb S sickles
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Sickle Cell AnemiaPathophysiology: - vaso-occlusion from sickled RBCs - increased RBC destruction - splenic congestion and enlargement - hepatomegaly, liver failure - renal ischemia, hematuria - osteoporosis, lordosis, kyphosis - cardiomegaly, heart failure, stroke
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Sickle Cell AnemiaSigns/Symptoms:Exercise intoleranceAnorexiaJaundiced scleraGallstonesChronic leg ulcersGrowth retardation
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Sickle Cell AnemiaDiagnosis - Sickledex - Hgb electrophoresis - Stained blood smearVaso-occlusive crisis - mild to severe bone pain - acute abdominal pain - priapism - arthralgia
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Sickle Cell Anemia Medical management Supportive/symptomatic tx of crises - bed rest - hydration - electrolyte replacement - analgesics for pain - blood replacement - antibiotics - oxygen therapy
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Sickle Cell AnemiaNursing care:Minimize tissue deoxygenationPromote hydrationMinimize crisesPain managementAdministering blood transfusionsEncourage screening and genetic counselingParent education
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ThalassemiaAutosomal recessive disorder Greeks, Italians, SyriansSigns/symptoms microcytic anemia > splenomegaly,jaundice,epistaxis, goutDiagnosis Hgb electrophoresisMedical Treatment transfusions, chelation
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HemophiliaFactor 8 or factor 9 deficiencyprolonged bleeding any where in the body!Cause: X-linked recessive disorder, defects in platelets and clotting factorsDiagnosis: history of bleeding episodes, evidence of x-linked inheritence, labsMedical Management: Factor VIII concentrate, DDAVP (vasopressin)
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HemophiliaNursing care:Prevent bleedingRecognize and control bleeding (RICE) - Rest - Ice - Compression - ElevationPrevent crippling effects of bleedingClient education
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Idiopathic Thrombocytopenic PurpuraCauses: acquired hemorrhagic disorder of unknown origin, probably an autoimmune response to disease-related antigens Diagnosis: platelet count < 20,000, abnl bleeding time and clot retractionSigns and Symptoms: petechiae, bruising, bleeding from mucous membranes, prolonged bleeding from abrasionsMedical management: supportive, steroids, Anti-D antibody, splenectomy
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Idiopathic Thrombocytic PuerperaNursing Considerations:Client/Parent teachingNo contact sportsNo aspirinPrevent infection
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Blood TransfusionComplications:Hemolytic reactions - chills, shaking, fever - dyspnea - flank pain - progressive signs of shockFebrile reactionsAllergic reactions - urticaria, flushing - wheezingCirculatory overload
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Blood TransfusionsNursing CareTake VS BEFORE administering bloodCheck ID of recipient with donors blood typeAdminister 50 mL or 1/5 volume SLOWLY STAY WITH THE CHILDAdminister with NS on piggyback set-upUse appropriate filterUse within 30 mins infuse within 4 hrsIf reaction suspected: Stop the transfusion, maintain patent IV line with NS, take VS, notify practitioner