Hematology 1

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blood disorders

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<ul><li><p>Pediatric Hematological DisordersWhaley and WongChapters 35, 36</p></li><li><p>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</p></li><li><p>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</p></li><li><p>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</p></li><li><p>Causes of AnemiaNutritional deficiency iron, folate, B12Increased destruction of RBCs sickle cell anemiaImpaired or decreased rate of production aplastic anemiaExcessive blood loss - hemophilia </p></li><li><p>Iron Deficiency AnemiaCauses - inadequate supply of iron - impaired absorption - blood loss - excessive demands for iron reqd for growth - inability for form Hgb </p></li><li><p>Iron Deficiency AnemiaSigns and Symptoms: due to tissue hypoxia &gt; 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</p></li><li><p>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 </p></li><li><p>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</p></li><li><p>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 </p></li><li><p>Sickle Cell AnemiaSigns/Symptoms:Exercise intoleranceAnorexiaJaundiced scleraGallstonesChronic leg ulcersGrowth retardation</p></li><li><p>Sickle Cell AnemiaDiagnosis - Sickledex - Hgb electrophoresis - Stained blood smearVaso-occlusive crisis - mild to severe bone pain - acute abdominal pain - priapism - arthralgia </p></li><li><p>Sickle Cell Anemia Medical management Supportive/symptomatic tx of crises - bed rest - hydration - electrolyte replacement - analgesics for pain - blood replacement - antibiotics - oxygen therapy</p></li><li><p>Sickle Cell AnemiaNursing care:Minimize tissue deoxygenationPromote hydrationMinimize crisesPain managementAdministering blood transfusionsEncourage screening and genetic counselingParent education</p></li><li><p>ThalassemiaAutosomal recessive disorder Greeks, Italians, SyriansSigns/symptoms microcytic anemia &gt; splenomegaly,jaundice,epistaxis, goutDiagnosis Hgb electrophoresisMedical Treatment transfusions, chelation</p></li><li><p>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)</p></li><li><p>HemophiliaNursing care:Prevent bleedingRecognize and control bleeding (RICE) - Rest - Ice - Compression - ElevationPrevent crippling effects of bleedingClient education</p></li><li><p>Idiopathic Thrombocytopenic PurpuraCauses: acquired hemorrhagic disorder of unknown origin, probably an autoimmune response to disease-related antigens Diagnosis: platelet count &lt; 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</p></li><li><p>Idiopathic Thrombocytic PuerperaNursing Considerations:Client/Parent teachingNo contact sportsNo aspirinPrevent infection</p></li><li><p>Blood TransfusionComplications:Hemolytic reactions - chills, shaking, fever - dyspnea - flank pain - progressive signs of shockFebrile reactionsAllergic reactions - urticaria, flushing - wheezingCirculatory overload</p></li><li><p>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</p></li></ul>