sickle cell anemia summer 2013
TRANSCRIPT
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 6 In healthy infants at about 6 months months
–normally fetal hemoglobin is normally 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
PathophysiologyPathophysiology
EITHER:EITHER:–decrease in oxygen tension decrease in oxygen tension
( hypoxemia) ( hypoxemia)
OROR–decrease in blood pH ( metabolic decrease in blood pH ( metabolic
acidosis)acidosis) Causes RBCs to lose their normal shapeCauses RBCs to lose their normal shape
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 in Things 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
Organ Systems AffectedOrgan Systems Affected SpleenSpleen: infection, : infection,
sequestrationsequestration LiverLiver: enlargement, : enlargement,
gallstones, jaundicegallstones, jaundice BonesBones: osteoporosis, : osteoporosis,
lordosis, kyphosis, lordosis, kyphosis, aseptic necrosis of aseptic necrosis of head of the femur head of the femur
SkinSkin: ulcers: ulcers
KidneysKidneys: necrosis, : necrosis, scarringscarring
CNSCNS: stroke, : stroke, paralysisparalysis
EyesEyes: blindness, : blindness, hemorrhagehemorrhage
CVCV: cardiomegaly, : cardiomegaly, murmurmurmur
LungsLungs: “chest : “chest syndrome”, 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 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 SymptomsVOC CrisisVOC Crisis
PAIN: extremely painful, swelling of PAIN: extremely painful, swelling of joints in hands and feet (Hand and foot joints 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
Management: Vaso-occlusive Management: Vaso-occlusive CrisisCrisis
PAIN MANAGEMENT Bed rest Oxygen Hydration ( 1.5-2 x maintenance fluid) Balance electrolytes Antibiotics for infection Blood transfusions
Acute Chest SyndromeAcute Chest Syndrome
Leading cause of morbidity and Leading cause of morbidity and mortality.mortality.
Presence of a new pulmonary infiltrate Presence 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
Acute Chest SyndromeAcute Chest Syndrome
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
HydroxyureaHydroxyurea
Increases amount of HbgF Increases amount of HbgF (mechanism is unknown) (mechanism is unknown)
Decreases number of hospitalizations Decreases number of hospitalizations and episodes of pneumonia , and episodes of pneumonia , posssibly CVA’sposssibly CVA’s
Used in children with > 3 episodes of Used in children with > 3 episodes of VOC requiring hospitalization per year VOC requiring hospitalization per year or recurrent episodes of Chest or recurrent episodes of Chest Syndrome, and history of CVA’s Syndrome, 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
Nursing DiagnosesNursing Diagnoses Acute pain related to tissue ischemia Acute pain related to tissue ischemia Risk for Infection related to compromised splenic function Risk for Infection related to compromised splenic function Activity intolerance related to pain Activity intolerance related to pain Deficient fluid volume related to increased fluid Deficient fluid volume related to increased fluid
requirements and po limitationsrequirements and po limitations Risk of ineffective peripheral tissue perfusion related to Risk of ineffective peripheral tissue perfusion related to
decreased capillary blood supplydecreased capillary blood supply Fatigue related to inadequate tissue oxygenation Fatigue related to inadequate tissue oxygenation Anxiety related to unfamiliar hospital environment Anxiety related to unfamiliar hospital environment Interrupted family processes related to caring for a child Interrupted family processes related to caring for a child
with a chronic condition with 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 fetal
Hgb levels fallHgb levels fall CBC, for drop Hgb and high CBC, for drop Hgb and high
reticulocyte countreticulocyte count
EARLY DETECTIONEARLY DETECTION
Newborn Screening
Mandated in all 50 states and D.C.Mandated in all 50 states and D.C. Evidence based practice shows that early detection Evidence based practice shows that early detection
and treatment can prevent life threatening and treatment can prevent life threatening pneumococcal infections.pneumococcal infections.
(USPSTF ( 2007).(USPSTF ( 2007). Helpful because parents can start penicillin Helpful because parents can start penicillin
prophylaxis by 2 months of age in affected infants prophylaxis by 2 months of age in affected infants and pneumococcal conjugate vaccinations and and pneumococcal conjugate vaccinations and parental education about early warning signs of parental education about early warning signs of infectioninfection
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)