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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 y 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

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PathophysiologyPathophysiology

EITHER:EITHER:

––decrease in oxygen tension decrease in oxygen tension

( hypoxemia)( hypoxemia)( hypoxemia) ( hypoxemia)

OROR

––decrease in blood pH ( metabolic decrease in blood pH ( metabolic acidosis)acidosis)

Causes RBCs to lose their normal Causes RBCs to lose their normal shapeshape

Normal and Sickle CellsNormal and Sickle Cells

Normal & Sickle RBCsNormal & Sickle RBCs

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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

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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 of aseptic necrosis of head of the femur head 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

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Chronic ComplicationsChronic Complications

Icteric sclera / jaundice

Anorexia

InfectionInfection

Growth retardation

Delayed puberty

Bone and joint problems

Types of Sickle Cell CrisesTypes of Sickle Cell CrisesMost 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 19hematocrit of 19--29 %29 %––Reticulocyte count > 2.5 %Reticulocyte count > 2.5 %

Fever, pneumoniaFever, pneumonia HematuriaHematuria

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Management: VasoManagement: Vaso--occlusive occlusive 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 and Leading cause of morbidity and mortality.mortality.

Presence of a new pulmonary infiltrate Presence 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

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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 of Increases amount of HbgFHbgF(mechanism is unknown) (mechanism is unknown)

Decreases number of Decreases number of h it li ti d i d fh it li ti d i d fhospitalizations and episodes of hospitalizations and episodes of pneumonia , pneumonia , posssiblyposssibly CVA’sCVA’s

Used in children with > 3 episodes of Used in children with > 3 episodes of VOC requiring hospitalization per VOC requiring hospitalization per year or recurrent episodes of Chest year 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

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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 andrequirements and popo limitationslimitationsrequirements and requirements and popo limitationslimitations

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)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 high CBC, for drop Hgb and high

reticulocyte countreticulocyte count

EARLY DETECTIONEARLY DETECTION

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Newborn Screening

Mandated in all 50 states and D.C.Mandated in all 50 states and D.C. Evidence based practice shows that early Evidence based practice shows that early

detection and treatment can prevent life detection and treatment can prevent life threatening pneumococcal infectionsthreatening pneumococcal infectionsthreatening pneumococcal infections.threatening 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 Infection prevention

Immunizations (vaccines) (Given to High risk children with underlying medical

conditions.)

– Pneumococcal ( PCV-13, PPSV)

– Meningococcal ( MCV4)– Influenza vaccine ( yearly)