2. Sickle Cell Disease
Inherited: autosomal recessive
Hemoglobin (Hgb A) replaced by
abnormal sickle hemoglobin
(Hgb S)
Valine (amino acid) substituted
for glutamic acid on beta chain
of Hgb molecule
3. Course of DiseaseCourse of Disease
In healthy infants at about 6In healthy infants at about 6
monthsmonths
–normally fetal hemoglobin isnormally fetal hemoglobin is
replaced 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. PathophysiologyPathophysiology
EITHER:EITHER:
–decrease in oxygen tensiondecrease in oxygen tension
( hypoxemia)( hypoxemia)
OROR
–decrease in blood pH ( metabolicdecrease in blood pH ( metabolic
acidosis)acidosis)
Causes RBCs to lose their normalCauses RBCs to lose their normal
shapeshape
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. Things that Cause Changes inThings that Cause Changes in
Oxygen Tension or Blood pHOxygen Tension or Blood pH
Fever
Infection
Dehydration
Hypoxia
Acidosis
Extreme Exercise
Serious Cold Exposure
High Altitudes
14. Types of Sickle Cell CrisesTypes of Sickle Cell Crises
Most common type of Sickle Cell
Crisis
Vaso-occlusive (VOC)
Potentially Life Threatening
Acute Chest Syndrome
Splenic Sequestration( blood
trapped in spleen→CV collapse
15. Clinical SymptomsClinical Symptoms
VOC CrisisVOC Crisis
PAIN: extremely painful, swelling ofPAIN: extremely painful, swelling of
joints in hands and feet (Hand and footjoints in hands and feet (Hand and foot
syndrome), 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
17. Acute Chest SyndromeAcute Chest Syndrome
Leading cause of morbidity andLeading cause of morbidity and
mortality.mortality.
Presence of a new pulmonary infiltratePresence of a new pulmonary infiltrate
associated with:associated with:
– severe chest painsevere chest pain
– feverfever
– cough, dyspnea, tachypneacough, dyspnea, tachypnea
– wheezing, retractionswheezing, retractions
– Hypoxia and severe anemiaHypoxia and severe anemia
19. Management of Acute chest
Syndrome
Pain Management
Oxygen
Hydration
Pulse oximetry monitoring
VS measurement and assessment of BS, WOB, IS
and cough and deep breathing
PFT,s
Antibiotics
Transfusion for severe hypoxemia, anemia
20. HydroxyureaHydroxyurea
Increases amount of HbgFIncreases amount of HbgF
(mechanism is unknown)(mechanism is unknown)
Decreases number ofDecreases number of
hospitalizations and episodes ofhospitalizations and episodes of
pneumonia , posssibly CVA’spneumonia , posssibly CVA’s
Used in children with > 3 episodes ofUsed in children with > 3 episodes of
VOC requiring hospitalization perVOC requiring hospitalization per
year or recurrent episodes of Chestyear or recurrent episodes of Chest
Syndrome, and history of CVA’sSyndrome, and history of CVA’s
21. Risks of HydroxyureaRisks of Hydroxyurea
Decreases blood counts
– blood work every 2 weeks until dosage is
finalized
Can cause infection and bleeding
Small risk of cancer or leukemia when
used for several years
Teratogenic
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 fluid
requirements and po limitationsrequirements and po limitations
Risk of ineffective peripheral tissue perfusion related toRisk of ineffective peripheral tissue perfusion related to
decreased 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 child
with a chronic conditionwith a chronic condition
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 fetal
Hgb levels fallHgb levels fall
CBC, for drop Hgb and highCBC, for drop Hgb and high
reticulocyte countreticulocyte count
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 early
detection and treatment can prevent lifedetection and treatment can prevent life
threatening pneumococcal infections.threatening pneumococcal infections.
(USPSTF ( 2007).(USPSTF ( 2007).
Helpful because parents can start penicillinHelpful because parents can start penicillin
prophylaxis by 2 months of age in affected infantsprophylaxis by 2 months of age in affected infants
and pneumococcal conjugate vaccinations andand pneumococcal conjugate vaccinations and
parental education about early warning signs ofparental education about early warning signs of
infectioninfection
26. Well Child CareWell Child Care
Nutrition ( folic acid supplements)
Adequate hydration
Infection prevention
Immunizations (vaccines)
(Given to High risk children with underlying medical
conditions.)
– Pneumococcal ( PCV-13, PPSV)
– Meningococcal ( MCV4)
– Influenza vaccine ( yearly)