Pediatric Hematology

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Pediatric Hematology

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<ul><li><p>PEDIATRIC HEMATOLOGYIron Deficiency Anemia Hemophilia ASickle Cell Anemia</p></li><li><p>IRON DEFICIENCY ANEMIAResults from an inadequate supply of ironMost common type of childhood anemiaInadequate iron supply leads to smaller RBCs, a reduction in the number of RBCs and the quantity of hemoglobin, and a decrease in the oxygen-carrying capacity of the blood</p></li><li><p>Iron Deficiency AnemiaCauses:Large milk intake (milk is a poor source of iron)Inadequate iron supply at birth (prematurity)Impaired absorption (diarrhea, malabsorption)Rapid growthInadequate diet (low in iron)Menses</p></li><li><p>Iron Deficiency Anemia SymptomsINFANTOverweightPaleFrequent infections</p><p>AdolescentsPaleFatigueDecreased attention spanLight-headed</p></li><li><p>Iron Deficiency Anemia - DiagnosticsHemoglobin below 10 gm.Hematocrit below 33Decreased serum iron binding concentration Decreased serum ferritinIncreased total iron binding capacity</p></li><li><p>Priority Nursing DiagnosisKnowledge deficitAltered nutrition, less than body requirementsActivity intoleranceRisk for altered growth and development</p></li><li><p>InterventionsDetectionScreen at 6, 12, 18, 24 months and adolescent femalesNutritional counselingIron-rich foodsDecrease milk intake</p></li><li><p>InterventionsIron supplementsBegin at 4 monthsPremature infants at 2 monthsGive between meals or with citrus juice(Increases absorption)Use a straw or brush teeth immediately after administering to prevent teeth stainingStools will be come green-black</p></li><li><p>SICKLE CELL ANEMIAA hereditary hemoglobinopathy primarily affecting African-AmericansAn autosomal recessive condition whereby normal hemoglobin is partially or completely replaced by the sickle-shaped, abnormal hemoglobin S (Hgb S)</p></li><li><p>SICKLE CELL ANEMIAWhen exposed to diminished levels of oxygen, the hemoglobin in the RBC develops a sickle or crescent shapeCells are rigid &amp; obstruct capillary blood flow, leading to congestion and tissue hypoxiaThis hypoxia causes additional sickling and extensive infarctions</p></li><li><p>Sickle Cell CrisisVaso-occlusive Crises (Thrombotic)Most common type of crisis, painfulCaused by stasis of blood with clumping of cells in the microcirculation, ischemia and infarctionSigns include fever, pain, tissue engorgementHypoxia, low oxygen tension or any condition that increases the bodys need for oxygen may trigger a crisis. Sickling may be triggered by fever, dehydration,emotional or physical stress</p></li><li><p>Sickle Cell CrisisSymptoms of crisis do not usually appear until age 4-6 monthsSickling of cells prevented secondary to the high levels of fetal hemoglobinThose with sickle cell trait (carriers of the disease) rarely experience crises or symptoms </p></li><li><p>SCA - AssessmentHemoglobin electrophoresis confirms the diagnosisNewborns may be diagnosed by hemoglobin electrophoresis of cord bloodSickledex (sickle turbidity test) is a screening test used for children 6 months or olderMultisystem assessment and history</p></li><li><p>SCA - Priority Nursing DiagnosesAltered tissue perfusionRisk for injuryRisk for infectionPainKnowledge deficit</p></li><li><p>SCA - Nursing InterventionsIncrease tissue perfusion: administer oxygenAdminister analgesics around the clockAssist hydration : IV fluids, oral fluidsPromote and encourage restPrevent infection: administer antibiotics as orderedProvide emotional support</p></li><li><p>SCA Client/Family TeachingSigns &amp; symptoms of impending crisisSigns &amp; symptoms of infectionImportance of adequate hydrationPreventing hypoxia from physical and emotional stress</p></li><li><p>HEMOPHILIAA group of disorders characterized by a deficiency in a specific clotting factorThe disease is a chronic inherited bleeding disorderHemophilia is without cure and is a lifelong condition</p></li><li><p>Hemophilia AAccounts for 80% of hemophilia casesInherited as an X-linked recessive traitFemale inherits the hemophilia trait from her father, then has a 50% chance of transmitting it to her sonHemophilia A is the result of factor VIII deficiency1 in 5,000 male births</p></li><li><p>Hemophilia A - AssessmentProlonged bleeding from minor traumaCareful history concerning episodes of bleeding as well as history of familial bleeding tendenciesHematuria or flank painGI, spinal cord bleedsJoint bleeding (hemarthrosis)- most common cause of hospitalization </p></li><li><p>Hemophilia - DiagnosticsProlonged activated PTTDecreased factor VIII levelsGenetic testing to identify carriersAmniocentesisCheck fibrinogen levels Platelets </p></li><li><p>Priority Nursing DiagnosesRisk for injuryPainImpaired physical mobilityKnowledge deficit</p></li><li><p>Nursing InterventionsControl localized bleedingIce, pressure, elevation, local coagulantsTransfuse as neededNO rectal temperatures!!!Manage painIf joint involvedImmobilization, ice packs, elevation Physical therapy when bleeding controlled</p></li><li><p>Nursing InterventionsReplacement of Factor VIIIAdministered IV when bleeding occursParents taught home administrationMay be administered prophylacticallyRisks include AIDS, hepatitis, infectionsExpensiveInjury preventionAvoid aspirin productsMedication: DDAVP Despromesin acetate- Careful bc cause Na levels to decrease and seizures </p></li><li><p>IDIOPATHIC THROMBOCYTOPENIC PURPURA (ITP)Autoimmune hematologic conditionMost common bleeding disorder in childrenPeak age 2-5 yearsUsually develops 1-3 weeks following a viral infection (chicken pox, measles)As platelet destruction exceeds production total number of platelets decreasesBlood clots slows. Can be acute or chronic </p></li><li><p>ITP - AssessmentPetechiaeMultiple ecchymotic areasHematuria, bloody or tarry stoolsNosebleedsLab data: decreased platelet count, increased anti-platelet antibodies Bone marrow to look for platelet precursors and rule out oncologic disorder</p></li><li><p>ITP Priority Nursing DiagnosesRisk for infectionRisk for injuryKnowledge deficit</p></li><li><p>ITP - ManagementMedicationsSteroids to reduce inflammation Admin for 2-3 weeks Intravenous immunoglobulins (IVIG) to reduce the autoimmune problem1/ day for 3-5 days Giving when there is life threatening bleeding - If Steroids and IVIG are not succesful then Spleenectomy (ITP last longer that 8-12 months )</p></li><li><p>ITP Nursing InterventionsFrequent assessment for bruising or active bleedingAssess neurological status every shift and PRNMonitor platelet counts No rectal temperaturesAvoid aspirin productsProvide soft toothbrush</p></li><li><p>LEUKEMIAALLAML</p></li><li><p>LeukemiaCancer of the blood-forming tissuesMost common form of childhood malignancyMore males than females after age 1 yearPeak onset 2 to 6 yearsTwo types recognized in childrenAcute lymphoid leukemia (ALL)Acute non-lymphoid myelogenous leukemia (AML)</p></li><li><p>Leukemia - PathophysiologyUnrestricted proliferation of WBCs occurBone marrow infiltration crowds out stem cells that normally produce red blood cells and platelets; anemia and thrombocytopenia occur; the WBCs that are produced are immature and cannot fight infectionSpleen, liver and lymph nodes become infiltrated and enlarged</p></li><li><p>Leukemia - PathophysiologyCentral nervous system (CNS) is at risk for infiltrationClinical manifestations are directly related to areas of involvement, such as bone pain from marrow proliferation</p></li><li><p>Leukemia - PrognosisBased on the initial WBC count at time of diagnosisMore favorable if below 50,000/mmAge is between 2-10 years at time of diagnosisIf diagnosed with ALL instead of AMLOverall prognosis has improvedMajority of newly diagnosed children who receive multi-agent treatment DO BETTER</p></li><li><p>Leukemia - DiagnosisHistory &amp; physicalPeripheral blood smear reveals anemia, thrombocytopenia, and neutropeniaLeukemic blasts may be seen on smearBone Marrow aspiration is the definitive testNormal marrow contains less than 5% blastsLeukemic marrow has much higher percentage, often 60-100% blastsSpinal tap</p></li><li><p>Leukemia Priority Nursing DiagnosesRisk for infectionRisk for injuryActivity intoleranceAnxietyRisk for ineffective family copingPain</p></li><li><p>Leukemia - TreatmentAim of treatment is to induce a remission (less than 5% blasts in the bone marrow)ChemotherapyCombinations of drugs are usedSeveral stages (CAN LAST 2-3 YEARS)Possible radiation therapy</p></li><li><p>ChemotherapyInductionGoal is to induce a remissionLasts 4-6 weeksIntensification or consolidationServes to maintain the remission and/or further reduce tumor burdenCNS prophylaxis and intrathecal medication</p></li><li><p>ChemotherapyMaintenanceLasts 2 - 3 yearsPreserves the remissionReinduction is used for relapses and adds drugs not previously usedBone marrow transplants are considered after a second relapse occurs</p></li><li><p>Leukemia Nursing ManagementPrepare child/family for diagnostic tests and proceduresRelieve painOpioids are titrated to the childs needsAdministered around the clockProvide emotional support to child and family</p></li><li><p>Nursing ManagementPrevent complications of myelosuppressionInfectionPrivate roomStrict handwashingRestriction of visitorsAdequate nutritionMasks</p></li><li><p>Nursing ManagementHemorrhagePlatelet infusionsAvoid skin punctureMeticulous mouth careNo rectal temperaturesAvoid activities that could cause injury</p></li><li><p>Manage Problems of Drug ToxicityAnemiaBlood transfusionsNausea &amp; vomitingAntiemetics before chemotherapyAnorexiaSmall, frequent feedings</p></li><li><p>Manage Problems of Drug ToxicityMucosal ulcerationBland dietSoft, sponge toothbrushFrequent mouthwashesLocal anestheticsNeuropathyStool softenersGood body alignment, safety measures</p></li><li><p>Manage Problems of Drug ToxicityAlopeciaWarn child and parents of possibility before the hair starts to fall outRecommend cotton head coveringsReaffirm that hair will grow backMood changes</p></li><li><p>Nursing Diagnoses - ChemotherapyRisk for infectionRisk for injuryAltered nutrition: less than body requirementsRisk for altered oral mucous membranesAltered body image related to hair loss</p></li><li><p>**Dont manifet ymptoms until after 6 months *Hemophilia B is called christmas tree disease IX Hemophilia C XI*Factor VIII is produced in the liver and necessary to coagulation </p><p>-If child get injuredPut pressure for 15 min Immobilize Elevate (above the heart)Put ice packs to promote vasoconstriccion. *If bleeding goes into the tissue of the neck, chest and mouth could lead to obstruction of airway. *Not contact sports due to increase risk for bleeding- Recommend swimming *Increase destruction of platelets in spleen. The viral infection, damages the platelets. </p><p>S/S PETECHIAE (MUCUS MEMBRANES AND SCLERA)MUCOSAL BLEEDING HEMATURIA BLACK TARRY STOOLS EPISTAXIS (NOSEBLEEDS)</p><p>*CLININAL MANIFESTATIONS IS EXCESSIVE BLEEDING </p><p>NEUROLOGIC ASSESSMENT DUE TO INCREASE RISK FOR INTRACRANIAL BLEEDING </p><p>If ITP is considered acute recovery to normal platelet count is whitin 6 months *Not give IM medications *Abnormal WBC replace normal WBC ABNORMAL BLEEDING bc WBC take place of platelets and RBC *Most common cause of cancer of kids less that 15 yrs old</p><p>ACUTE LYMPHOBLASTIC LEUKEMIA ALL (MOST COMMON)</p><p>AML has increase incidence of relapse </p><p>*LUMBAR PUNCTURE IS DO TO DETERMINE IF THER IS CSF inflitration </p><p>RAPID FLOW CITOMETRIC RECOGNIZES EVEN SMALL AMOUNT OF LEUKEMIA IN BODY </p><p>WHAT IS BLAST? *STAGES: INDUCTION: MAX DEATH CELL OCCURS CONSOLODATION RECANCIFICATION: CHEMO IS DONE LATE INTENSIFICATION: ADDITIONAL DRUGS TO TARGET LEUKEMIA CELLS REMISSION </p><p>- COMPLICATIONS IRON DEFICIENCY ORGAN FAILURE DEATH *Never immunize children with MMR when they are immunocompromised. Chemo drug are vesicants and cause damage if drug infiltrates *Nausea and Vomiting, give antiemetic before chemo starts Chemo causes mucus ulcerations (mouth) use mouthwashes of Normal Saline. Explain alopecia *If they are receiving corticosteroid they are more susceptible for infections *When doing chemo ALWAYS monitor the kidneys *- Cardinal signs leukemia Petechia Joint pain Flank bleedding (joints)Enlarged liver and spleen Changes in lymph nodes </p><p>ALM IS LESS RESPONSIVE TO TREATMENT. ALL HAVE BETTER PROGNOSIS. *</p></li></ul>