Sickle cell anemia summer 2013


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Sickle cell anemia summer 2013

  1. 1. Hematological Conditions inChildrenNursing Assessment andInterventionsKathryn Kushto-Reese
  2. 2. 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
  3. 3. 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
  4. 4. 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
  5. 5. Normal and Sickle CellsNormal and Sickle Cells
  6. 6. Normal & Sickle RBCsNormal & Sickle RBCs
  7. 7. 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
  8. 8. 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
  10. 10. 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
  11. 11. Complications of SCDComplications of SCD
  12. 12. OstoearthrosisOstoearthrosis
  13. 13. Chronic ComplicationsChronic Complications Icteric sclera / jaundice Anorexia Infection Growth retardation Delayed puberty Bone and joint problems
  14. 14. 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
  15. 15. 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
  16. 16. Management: Vaso-occlusiveManagement: Vaso-occlusiveCrisisCrisis PAIN MANAGEMENT Bed rest Oxygen Hydration ( 1.5-2 x maintenance fluid) Balance electrolytes Antibiotics for infection Blood transfusions
  17. 17. 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
  18. 18. Acute Chest SyndromeAcute Chest Syndrome
  19. 19. 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
  20. 20. 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
  21. 21. 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
  22. 22. 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
  23. 23. 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
  25. 25. 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
  26. 26. 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)