Sickle cell anemia summer 2013


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Sickle cell anemia summer 2013

  1. 1. 1Hematological Conditions inChildrenNursing Assessment andInterventionsKathryn Kushto-ReeseSickle Cell Disease Inherited: autosomal recessive Hemoglobin (Hgb A) replaced byabnormal sickle hemoglobin(Hgb S) Valine (amino acid) substitutedfor glutamic acid on beta chainof Hgb moleculeCourse of DiseaseCourse of Disease In healthy infants at about 6In healthy infants at about 6monthsmonths––normally fetal hemoglobin isnormally fetal hemoglobin isy gy greplaced 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
  2. 2. 2PathophysiologyPathophysiology EITHER:EITHER:––decrease in oxygen tensiondecrease in oxygen tension( hypoxemia)( hypoxemia)( hypoxemia)( hypoxemia)OROR––decrease in blood pH ( metabolicdecrease in blood pH ( metabolicacidosis)acidosis) Causes RBCs to lose their normalCauses RBCs to lose their normalshapeshapeNormal and Sickle CellsNormal and Sickle CellsNormal & Sickle RBCsNormal & Sickle RBCs
  3. 3. 3Sickled CellsSickled Cells Have a short life span Lodge in small capillaries,break apart Increase the viscosity of blood–slows circulation–occlude vessels–cause tissue ischemiaThings 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 AltitudesSUMMER CAMPSUMMER CAMP
  4. 4. 4Organ Systems AffectedOrgan Systems Affected SpleenSpleen: infection,: infection,sequestrationsequestration LiverLiver: enlargement,: enlargement,gallstones, jaundicegallstones, jaundice KidneysKidneys: necrosis,: necrosis,scarringscarring CNSCNS: stroke,: stroke,paralysisparalysisgallstones, jaundicegallstones, jaundice BonesBones: osteoporosis,: osteoporosis,lordosis, kyphosis,lordosis, kyphosis,aseptic necrosis ofaseptic necrosis ofhead of the femurhead of the femur SkinSkin: ulcers: ulcersparalysisparalysis EyesEyes: blindness,: blindness,hemorrhagehemorrhage CVCV: cardiomegaly,: cardiomegaly,murmurmurmur LungsLungs: “chest: “chestsyndrome”, infectionsyndrome”, infectionComplications of SCDComplications of SCDOstoearthrosisOstoearthrosis
  5. 5. 5Chronic ComplicationsChronic Complications Icteric sclera / jaundice Anorexia InfectionInfection Growth retardation Delayed puberty Bone and joint problemsTypes 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 collapseClinical 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 19hematocrit of 19--29 %29 %––Reticulocyte count > 2.5 %Reticulocyte count > 2.5 % Fever, pneumoniaFever, pneumonia HematuriaHematuria
  6. 6. 6Management: VasoManagement: Vaso--occlusiveocclusiveCrisisCrisis PAIN MANAGEMENT Bed rest Oxygen Oxygen Hydration ( 1.5-2 x maintenance fluid) Balance electrolytes Antibiotics for infection Blood transfusionsAcute 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:associated with:associated with:–– severe chest painsevere chest pain–– feverfever–– cough, dyspnea, tachypneacough, dyspnea, tachypnea–– wheezing, retractionswheezing, retractions–– Hypoxia and severe anemiaHypoxia and severe anemiaAcute Chest SyndromeAcute Chest Syndrome
  7. 7. 7Management of Acute chestSyndrome Pain Management Oxygen Hydration Pulse oximetry monitoringPulse oximetry monitoring VS measurement and assessment of BS, WOB, ISand cough and deep breathing PFT,s Antibiotics Transfusion for severe hypoxemia, anemiaHydroxyureaHydroxyurea Increases amount ofIncreases amount of HbgFHbgF(mechanism is unknown)(mechanism is unknown) Decreases number ofDecreases number ofh it li ti d i d fh it li ti d i d fhospitalizations and episodes ofhospitalizations and episodes ofpneumonia ,pneumonia , posssiblyposssibly CVA’sCVA’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’sRisks 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
  8. 8. 8Nursing 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 andrequirements and popo limitationslimitationsrequirements andrequirements and popo limitationslimitations 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 conditionDiagnostic TestsDiagnostic Tests Hemoglobin ElectrophoresisHemoglobin Electrophoresis(from newborns cord blood)(from newborns cord blood)Si klSi kl t bidit t t (Si kl d )t bidit t t (Si kl d ) SickleSickle--turbidity test (Sickledex)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 countEARLY DETECTIONEARLY DETECTION
  9. 9. 9Newborn 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 infectionsthreatening pneumococcal infectionsthreatening 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 ofinfectioninfectionWell Child CareWell Child Care Nutrition ( folic acid supplements) Adequate hydration Infection prevention Infection prevention Immunizations (vaccines) (Given to High risk children with underlying medicalconditions.)– Pneumococcal ( PCV-13, PPSV)– Meningococcal ( MCV4)– Influenza vaccine ( yearly)