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Sickle cell anemia summer 2013

Sickle cell anemia summer 2013






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    Sickle cell anemia summer 2013 Sickle cell anemia summer 2013 Presentation Transcript

    • Hematological Conditions inChildrenNursing Assessment andInterventionsKathryn Kushto-Reese
    • Sickle Cell Disease Inherited: autosomal recessive Hemoglobin (Hgb A) replaced byabnormal sickle hemoglobin(Hgb S) Valine (amino acid) substitutedfor glutamic acid on beta chainof Hgb molecule
    • Course of DiseaseCourse of Disease In healthy infants at about 6In healthy infants at about 6monthsmonths–normally fetal hemoglobin isnormally fetal hemoglobin isreplaced by normal HbAreplaced by normal HbA In infant with sickle cell disease,In infant with sickle cell disease,HbA replaced by HbSHbA replaced by HbS
    • PathophysiologyPathophysiology EITHER:EITHER:–decrease in oxygen tensiondecrease in oxygen tension( hypoxemia)( hypoxemia)OROR–decrease in blood pH ( metabolicdecrease in blood pH ( metabolicacidosis)acidosis) Causes RBCs to lose their normalCauses RBCs to lose their normalshapeshape
    • Normal and Sickle CellsNormal and Sickle Cells
    • Normal & Sickle RBCsNormal & Sickle RBCs
    • Sickled CellsSickled Cells Have a short life span Lodge in small capillaries,break apart Increase the viscosity of blood–slows circulation–occlude vessels–cause tissue ischemia
    • Things that Cause Changes inThings that Cause Changes inOxygen Tension or Blood pHOxygen Tension or Blood pH Fever Infection Dehydration Hypoxia Acidosis Extreme Exercise Serious Cold Exposure High Altitudes
    • Organ Systems AffectedOrgan Systems Affected SpleenSpleen: infection,: infection,sequestrationsequestration LiverLiver: enlargement,: enlargement,gallstones, jaundicegallstones, jaundice BonesBones: osteoporosis,: osteoporosis,lordosis, kyphosis,lordosis, kyphosis,aseptic necrosis ofaseptic necrosis ofhead of the femurhead of the femur SkinSkin: ulcers: ulcers KidneysKidneys: necrosis,: necrosis,scarringscarring CNSCNS: stroke,: stroke,paralysisparalysis EyesEyes: blindness,: blindness,hemorrhagehemorrhage CVCV: cardiomegaly,: cardiomegaly,murmurmurmur LungsLungs: “chest: “chestsyndrome”, infectionsyndrome”, infection
    • Complications of SCDComplications of SCD
    • OstoearthrosisOstoearthrosis
    • Chronic ComplicationsChronic Complications Icteric sclera / jaundice Anorexia Infection Growth retardation Delayed puberty Bone and joint problems
    • Types of Sickle Cell CrisesTypes of Sickle Cell CrisesMost common type of Sickle CellCrisis Vaso-occlusive (VOC)Potentially Life Threatening Acute Chest Syndrome Splenic Sequestration( bloodtrapped in spleen→CV collapse
    • Clinical SymptomsClinical SymptomsVOC CrisisVOC Crisis PAIN: extremely painful, swelling ofPAIN: extremely painful, swelling ofjoints in hands and feet (Hand and footjoints in hands and feet (Hand and footsyndrome), severe abdominal pain.syndrome), severe abdominal pain. FeverFever ANEMIAANEMIA–hemoglobin of 5.5 to 9.5 g/dlhemoglobin of 5.5 to 9.5 g/dl–hematocrit of 19-29 %hematocrit of 19-29 %–Reticulocyte count > 2.5 %Reticulocyte count > 2.5 % Fever, pneumoniaFever, pneumonia HematuriaHematuria
    • Management: Vaso-occlusiveManagement: Vaso-occlusiveCrisisCrisis PAIN MANAGEMENT Bed rest Oxygen Hydration ( 1.5-2 x maintenance fluid) Balance electrolytes Antibiotics for infection Blood transfusions
    • Acute Chest SyndromeAcute Chest SyndromeLeading cause of morbidity andLeading cause of morbidity andmortality.mortality.Presence of a new pulmonary infiltratePresence of a new pulmonary infiltrateassociated with:associated with:– severe chest painsevere chest pain– feverfever– cough, dyspnea, tachypneacough, dyspnea, tachypnea– wheezing, retractionswheezing, retractions– Hypoxia and severe anemiaHypoxia and severe anemia
    • Acute Chest SyndromeAcute Chest Syndrome
    • Management of Acute chestSyndrome Pain Management Oxygen Hydration Pulse oximetry monitoring VS measurement and assessment of BS, WOB, ISand cough and deep breathing PFT,s Antibiotics Transfusion for severe hypoxemia, anemia
    • HydroxyureaHydroxyurea Increases amount of HbgFIncreases amount of HbgF(mechanism is unknown)(mechanism is unknown) Decreases number ofDecreases number ofhospitalizations and episodes ofhospitalizations and episodes ofpneumonia , posssibly CVA’spneumonia , posssibly CVA’s Used in children with > 3 episodes ofUsed in children with > 3 episodes ofVOC requiring hospitalization perVOC requiring hospitalization peryear or recurrent episodes of Chestyear or recurrent episodes of ChestSyndrome, and history of CVA’sSyndrome, and history of CVA’s
    • Risks of HydroxyureaRisks of Hydroxyurea Decreases blood counts– blood work every 2 weeks until dosage isfinalized Can cause infection and bleeding Small risk of cancer or leukemia whenused for several years Teratogenic
    • Nursing DiagnosesNursing Diagnoses Acute pain related to tissue ischemiaAcute pain related to tissue ischemia Risk for Infection related to compromised splenic functionRisk for Infection related to compromised splenic function Activity intolerance related to painActivity intolerance related to pain Deficient fluid volume related to increased fluidDeficient fluid volume related to increased fluidrequirements and po limitationsrequirements and po limitations Risk of ineffective peripheral tissue perfusion related toRisk of ineffective peripheral tissue perfusion related todecreased capillary blood supplydecreased capillary blood supply Fatigue related to inadequate tissue oxygenationFatigue related to inadequate tissue oxygenation Anxiety related to unfamiliar hospital environmentAnxiety related to unfamiliar hospital environment Interrupted family processes related to caring for a childInterrupted family processes related to caring for a childwith a chronic conditionwith a chronic condition
    • Diagnostic TestsDiagnostic Tests Hemoglobin ElectrophoresisHemoglobin Electrophoresis(from newborns cord blood)(from newborns cord blood) Sickle-turbidity test (Sickledex)Sickle-turbidity test (Sickledex)( quick screening > 6 months after fetal( quick screening > 6 months after fetalHgb levels fallHgb levels fall CBC, for drop Hgb and highCBC, for drop Hgb and highreticulocyte countreticulocyte count
    • Newborn Screening Mandated in all 50 states and D.C.Mandated in all 50 states and D.C. Evidence based practice shows that earlyEvidence based practice shows that earlydetection and treatment can prevent lifedetection and treatment can prevent lifethreatening pneumococcal infections.threatening pneumococcal infections.(USPSTF ( 2007).(USPSTF ( 2007). Helpful because parents can start penicillinHelpful because parents can start penicillinprophylaxis by 2 months of age in affected infantsprophylaxis by 2 months of age in affected infantsand pneumococcal conjugate vaccinations andand pneumococcal conjugate vaccinations andparental education about early warning signs ofparental education about early warning signs ofinfectioninfection
    • Well Child CareWell Child Care Nutrition ( folic acid supplements) Adequate hydration Infection prevention Immunizations (vaccines) (Given to High risk children with underlying medicalconditions.)– Pneumococcal ( PCV-13, PPSV)– Meningococcal ( MCV4)– Influenza vaccine ( yearly)