This document discusses sickle cell disease in children. It describes sickle cell disease as an inherited condition caused by a mutation in the hemoglobin gene, causing red blood cells to take on a sickle shape. This leads to blockages in small blood vessels and pain crises. It outlines the pathophysiology, common complications affecting different organ systems, types of sickle cell crises including vaso-occlusive and acute chest syndrome, diagnostic tests, nursing care plans, and treatment and management strategies including hydroxyurea and newborn screening.

1. 1
Hematological Conditions in
Children
Nursing Assessment and
Interventions
Kathryn Kushto-Reese
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
Course of DiseaseCourse of Disease
 In healthy infants at about 6In healthy infants at about 6
monthsmonths
––normally fetal hemoglobin isnormally fetal hemoglobin isy gy g
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

2. 2
PathophysiologyPathophysiology
 EITHER:EITHER:
––decrease in oxygen tensiondecrease in oxygen tension
( hypoxemia)( hypoxemia)( 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
Normal and Sickle CellsNormal and Sickle Cells
Normal & Sickle RBCsNormal & Sickle RBCs

3. 3
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 in
Oxygen Tension or Blood pHOxygen Tension or Blood pH
 Fever
 Infection
 Dehydration
 Hypoxia
 Acidosis
 Extreme Exercise
 Serious Cold Exposure
 High Altitudes
SUMMER CAMPSUMMER CAMP

4. 4
Organ 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 of
head of the femurhead of the femur
 SkinSkin: ulcers: ulcers
paralysisparalysis
 EyesEyes: blindness,: blindness,
hemorrhagehemorrhage
 CVCV: cardiomegaly,: cardiomegaly,
murmurmurmur
 LungsLungs: “chest: “chest
syndrome”, infectionsyndrome”, infection
Complications of SCDComplications of SCD
OstoearthrosisOstoearthrosis

5. 5
Chronic ComplicationsChronic Complications
 Icteric sclera / jaundice
 Anorexia
 InfectionInfection
 Growth retardation
 Delayed puberty
 Bone and joint problems
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
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 19hematocrit of 19--29 %29 %
––Reticulocyte count > 2.5 %Reticulocyte count > 2.5 %
 Fever, pneumoniaFever, pneumonia
 HematuriaHematuria

6. 6
Management: VasoManagement: Vaso--occlusiveocclusive
CrisisCrisis
 PAIN MANAGEMENT
 Bed rest
 Oxygen Oxygen
 Hydration ( 1.5-2 x maintenance fluid)
 Balance electrolytes
 Antibiotics for infection
 Blood transfusions
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: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
Acute Chest SyndromeAcute Chest Syndrome

7. 7
Management of Acute chest
Syndrome
 Pain Management
 Oxygen
 Hydration
 Pulse oximetry monitoringPulse oximetry monitoring
 VS measurement and assessment of BS, WOB, IS
and cough and deep breathing
 PFT,s
 Antibiotics
 Transfusion for severe hypoxemia, anemia
HydroxyureaHydroxyurea
 Increases amount ofIncreases amount of HbgFHbgF
(mechanism is unknown)(mechanism is unknown)
 Decreases number ofDecreases number of
h it li ti d i d fh it li ti d i d fhospitalizations and episodes ofhospitalizations and episodes of
pneumonia ,pneumonia , posssiblyposssibly CVA’sCVA’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
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

8. 8
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 andrequirements and popo limitationslimitationsrequirements andrequirements and popo limitationslimitations
 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
Diagnostic 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 fetal
Hgb levels fallHgb levels fall
 CBC, for drop Hgb and highCBC, for drop Hgb and high
reticulocyte countreticulocyte count
EARLY DETECTIONEARLY DETECTION

9. 9
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 infectionsthreatening pneumococcal infectionsthreatening 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
Well Child CareWell Child Care
 Nutrition ( folic acid supplements)
 Adequate hydration
 Infection prevention Infection prevention
 Immunizations (vaccines)
 (Given to High risk children with underlying medical
conditions.)
– Pneumococcal ( PCV-13, PPSV)
– Meningococcal ( MCV4)
– Influenza vaccine ( yearly)