Hematologic-Oncology. Common Hematologic Disorders in Children Iron-Deficiency AnemiaIron-Deficiency Anemia Sickle Cell AnemiaSickle Cell Anemia Beta-Thalasemia

Hematologic-Oncology

Common Hematologic Disorders in Children

• Iron-Deficiency AnemiaIron-Deficiency Anemia• Sickle Cell AnemiaSickle Cell Anemia• Beta-ThalasemiaBeta-Thalasemia

Major (Cooley’s anemia)Major (Cooley’s anemia)• Hemophilia AHemophilia A• Von Willebrand’s DiseaseVon Willebrand’s Disease• ITP (Immune Thrombocytopenic ITP (Immune Thrombocytopenic

Pupura)Pupura)

Common Heme-Oncology Diseases in Children

• Acute Lymphocytic LeukemiaAcute Lymphocytic Leukemia• Hodgkin’s DiseaseHodgkin’s Disease

• Non-Hodgkin’s LymphomaNon-Hodgkin’s Lymphoma• RetinoblastomaRetinoblastoma

• NeuroblastomaNeuroblastoma• NephroblastomaNephroblastoma• Osteogenic SarcomaOsteogenic Sarcoma• Ewing’s SarcomaEwing’s Sarcoma

Complete Blood Count

• WBCWBC

• RBCRBC

• HgbHgb

• HctHct

• PlateletPlatelet

CBC with Differential

• WBCWBC– Neutrophils- phagocytosisNeutrophils- phagocytosis– Lymphocytes – T and B cellLymphocytes – T and B cell– Monoocytes – phagocytosis, antigenMonoocytes – phagocytosis, antigen– Eosophils- allergen Eosophils- allergen – Basophils-inflammatoryBasophils-inflammatory

• RBCRBC– MCV- volumeMCV- volume– MCHMCH– MCHCMCHC– RCW- width RCW- width

• HgbHgb• HctHct• PlateletPlatelet

– MPVMPV

PT, PTT

• The prothrombin time (PT) test measures how long it takes The prothrombin time (PT) test measures how long it takes for a clot to form in a sample of blood. for a clot to form in a sample of blood.

• Prothrombin is one of several clotting factors that are Prothrombin is one of several clotting factors that are produced by the liver. produced by the liver.

• The PT test evaluates the integrated function of these The PT test evaluates the integrated function of these factors and the body’s ability to produce a clot in a factors and the body’s ability to produce a clot in a reasonable amount of time. reasonable amount of time.

• Because the reagents used to perform the PT test vary Because the reagents used to perform the PT test vary from one laboratory to another and even within the same from one laboratory to another and even within the same laboratory over time, the normal values also will fluctuate. laboratory over time, the normal values also will fluctuate.

Other Labs

• Sed Rate (ESR)Sed Rate (ESR)

• IronIron

• TIBC (Transferrin)TIBC (Transferrin)

• FerritinFerritin

• BilirubinBilirubin

Pediatric Laboratory Normal Values: Children age 2-12 Years

• RBC: 3.89-4.96RBC: 3.89-4.96• HgB: 10.2-13.4HgB: 10.2-13.4• Hct: 31.7-39.3%Hct: 31.7-39.3%• Sed: 1-8Sed: 1-8• WBC: 5,400-11,000WBC: 5,400-11,000

• Platelets: 206,000-403,000Platelets: 206,000-403,000

• Fe: 20-105Fe: 20-105

• Ferritin: 47-110Ferritin: 47-110

• TIBC: 240-508TIBC: 240-508

• PT: 10-11 secPT: 10-11 sec

• PTT: 42-54 secPTT: 42-54 sec

• Bilirubin- less than 11.7Bilirubin- less than 11.7

Anemia’s

))

• Reduction of:Reduction of:– number of red blood number of red blood

cellscells– the quantity of the quantity of

hemoglobinhemoglobin– the volume of packed the volume of packed

redred

Types in Children:Types in Children:

• Iron-Deficiency AnemiaIron-Deficiency Anemia• Sickle Cell AnemiaSickle Cell Anemia• Beta-ThalasemiaBeta-Thalasemia

Major (Cooley’s anemiaMajor (Cooley’s anemia

Iron-Deficiency Anemia

The most common hematologic disorder of The most common hematologic disorder of infancy and childhood infancy and childhood

• 9 months- 2 years, adolescence9 months- 2 years, adolescence

• A nutrient deficiency of inadequate A nutrient deficiency of inadequate dietary irondietary iron

Prevention:Prevention: iron fortified products iron fortified products

Children at Risk

• low birth weight infants low birth weight infants

• infants born to mothers with iron infants born to mothers with iron deficiency anemia deficiency anemia

• infants born with GI defects infants born with GI defects

• chronic blood loss in older children chronic blood loss in older children

Pathophysiology

• Dietary Fe is bloodstream binds to Dietary Fe is bloodstream binds to transferrin (TIBC) and is delivered to RBC transferrin (TIBC) and is delivered to RBC in bone marrow, combines with other cells in bone marrow, combines with other cells to make Hgbto make Hgb

• Unused dietary Fe is stored in intestinal Unused dietary Fe is stored in intestinal epithelial cells as ferritinepithelial cells as ferritin

Diagnosis

• Low RBC’s Low RBC’s

• Low HGB Low HGB – Mild ( < 10.2), Moderate (8-9), Severe (< 7)Mild ( < 10.2), Moderate (8-9), Severe (< 7)

• Low HCT Low HCT

• Low Iron Low Iron

• High Transferrin (TIBC) High Transferrin (TIBC)

• Low Ferritin Low Ferritin

Symptoms

Low Hgb=low O2 tissue perfusionLow Hgb=low O2 tissue perfusion

Hgb of 10.2 or lessHgb of 10.2 or less• May seem asymptomatic, not noticed by caregiverMay seem asymptomatic, not noticed by caregiver• Pallor/Pale mucous membranes (low hgb, not enough red color to Pallor/Pale mucous membranes (low hgb, not enough red color to

skin)skin)• Poor muscle tone, decreased activityPoor muscle tone, decreased activity• FatigueFatigue• Increased HR, RRIncreased HR, RR

Hgb < 9Hgb < 9• Above plus irritability, lack of interest in playAbove plus irritability, lack of interest in play

History

• Dietary history usually shows abnormally high milk intake > 32 oz day in toddler

• Ask parents specific questions

• Begin the dietary history at the time the child awoke yesterday; include all activities and exactly what the child ate

Management

• Iron-fortified formula Iron-fortified formula

• Limit cow’s milk to 24-32 oz/day for Limit cow’s milk to 24-32 oz/day for children >12 monthschildren >12 months

• Increase age-appropriate iron-rich foods Increase age-appropriate iron-rich foods and Vit Cand Vit C

• Fe supplements- Ferrous SulfateFe supplements- Ferrous Sulfate

Nursing Considerations

Iron-Rich Foods Vitamin C Rich Foods

Meats, fish, poultry Orange juice

Vegetables Citrus fruits

Dried fruits Strawberries

Legumes Tomatoes

Enriched grain products Broccoli

Whole grain cereal Leafy Green vegetables

Iron-Fortified Cereal Potatoes


• Manage side effects of Ferrous SulfateManage side effects of Ferrous Sulfate– Nausea,Nausea,– AnorexiaAnorexia– ConstipationConstipation– Abdominal distressAbdominal distress– Black stools.Black stools.

• Give on an empty stomach if possibleGive on an empty stomach if possible

• Monitor bowel movements and suggest increased fluid and fiber.Monitor bowel movements and suggest increased fluid and fiber.


• Monitor development, sleep, and activity/fatigue Monitor development, sleep, and activity/fatigue patterns.patterns.

• Monitor hemoglobin to measure effectiveness of Monitor hemoglobin to measure effectiveness of therapy.therapy.

• Instruct families to keep Ferrous Sulfate locked Instruct families to keep Ferrous Sulfate locked and out of reach of children; poisoning is a serious and out of reach of children; poisoning is a serious risk.risk.

Sickle Cell Anemia

Autosomal recessive disorder, African AmericansAutosomal recessive disorder, African Americans Characterized by abnormal hemoglobin (HbS) Characterized by abnormal hemoglobin (HbS) Clinical manifestations caused by obstructions due to Clinical manifestations caused by obstructions due to

the sickled RBC’s and destruction of sickled and the sickled RBC’s and destruction of sickled and normal RBC’snormal RBC’s

Sickle Cell Anemia

Symptoms may not appear until 6 months of Symptoms may not appear until 6 months of ageage

Mortality rate children < 3 yo is 15-35%Mortality rate children < 3 yo is 15-35%

DiagnosisDiagnosis::

Amniocentesis, CVS, Newborn ScreenAmniocentesis, CVS, Newborn Screen

Signs & Symptoms:

Initially: fever & anemia at 6 mosInitially: fever & anemia at 6 mos

• PallorPallor• FatigueFatigue• SOBSOB• IrritabilityIrritability• JaundiceJaundice

Diagnosis

• Moderately low Hcb and HctModerately low Hcb and Hct

• Normal Iron, TIBC, FerritinNormal Iron, TIBC, Ferritin

• Elevated BillirubinElevated Billirubin

3 Sequalea of SCA

1.1. Vaso-Occlusive CrisisVaso-Occlusive Crisis

2.2. Acute Chest SyndromeAcute Chest Syndrome

3.3. Splenic SequestrationSplenic Sequestration

Vaso-occlusive crisis

• Severe, sudden onset of sickling where many new Severe, sudden onset of sickling where many new sickled cells pool in a vessel and cause pain and sickled cells pool in a vessel and cause pain and tissue hypoxia tissue hypoxia

• Caused by: infection, dehydration, anxiety, coldCaused by: infection, dehydration, anxiety, cold• Most common from hypoxia secondary to rapidly Most common from hypoxia secondary to rapidly

destroyed RBCdestroyed RBC

• Lasts for hours to weeksLasts for hours to weeks

Vaso-occlusive Crisis

Early Signs: pallor, Early Signs: pallor, tachycardia, fevertachycardia, fever

Late Signs: acute Late Signs: acute abdominal, back, abdominal, back, extremity painextremity pain

First Crisis in infants: First Crisis in infants: – Dactylitis (hand & Dactylitis (hand &

foot syndrome)foot syndrome)• swelling of hands and swelling of hands and

feet feet

• joints may be warm & joints may be warm & swollen swollen

Management

• Pain reliefPain relief

• Adequate hydrationAdequate hydration

• Adequate oxygenationAdequate oxygenation

Pain

• Assess pain every 1-2h or more Assess pain every 1-2h or more frequentlyfrequently

• Use pain scale appropriate for ageUse pain scale appropriate for age

• Non-pharmacological pain methodsNon-pharmacological pain methods

• AROUND THE CLOCK PAIN MEDSAROUND THE CLOCK PAIN MEDS

• Tylenol for mild painTylenol for mild pain

• Narcotics for mod-severe painNarcotics for mod-severe pain

Prevent Occlusion

• Push PO fluidsPush PO fluids

• IV hydration: IV hydration: 1.5 to 21.5 to 2 times normal times normal raterate

• Risk for fluid overloadRisk for fluid overload

Altered Tissue Perfusion and Prevent Further Sickling

• Administer oxygen to maintain saturation Administer oxygen to maintain saturation of 95% or higherof 95% or higher

• Pulse oximetryPulse oximetry

• Semi-fowler’s position Semi-fowler’s position

• Administer PRBC’sAdminister PRBC’s

Acute Chest Syndrome• Sickle contents Sickle contents

break offbreak off• Bilateral Bilateral

pulmonary pulmonary involvementinvolvement

• Causes chest Causes chest infection, infection, embolismembolism


• Know the Know the symptoms:symptoms:– Chest painChest pain– FeverFever– CoughCough– WheezeWheeze– TachypneaTachypnea

• AnalgesicsAnalgesics

• OxygenOxygen

• Hydration Hydration Incentive Incentive spirometry,spirometry,

• AntibioticsAntibiotics

• PRBCPRBC

Splenic Sequestration• Sickled cells block the spleenSickled cells block the spleen• pooled blood in spleen and/or liver pooled blood in spleen and/or liver

and enlargesand enlarges• Pooled blood leads to a decrease in Pooled blood leads to a decrease in

circulating volume= hypovolemic circulating volume= hypovolemic shock shock

• CVA => comaCVA => coma


• Know the Know the Symptoms:Symptoms:– IrritabilityIrritability– PalePale– TachycardiaTachycardia– Pain to LUQPain to LUQ– Enlarged SpleenEnlarged Spleen

• Life Threatening- Life Threatening- get child to ED get child to ED a.s.a.p.!a.s.a.p.!

• PRBCPRBC

• Remove spleenRemove spleen

Risk for Infection r/t Chronic Immunosuppression

• Administer PCN everydayAdminister PCN everyday• Up-to-date vaccinesUp-to-date vaccines

Educate parentsEducate parents:: s/s infection & respiratory distresss/s infection & respiratory distress

possible triggerspossible triggerstreat pain immediatelytreat pain immediately

adequate fluidsadequate fluids

Beta-ThalasemiaMajor (Cooley’s anemia)

• Hereditary anemia due to abnormal Hereditary anemia due to abnormal synthesis of hemoglobinsynthesis of hemoglobin

• Life long disorderLife long disorder• Mediterranean descentMediterranean descent• Life threatening symptomsLife threatening symptoms

Diagnosis

• Low RBC’sLow RBC’s

• Extremely low Hgb < 5Extremely low Hgb < 5

• Increased serum ironIncreased serum iron

Symptoms

• Facial anomalies Facial anomalies – Frontal bossing (prominent Frontal bossing (prominent

and protruding forehead)and protruding forehead)– Maxillary prominenceMaxillary prominence– Wide-set eyes with a Wide-set eyes with a

flattened noseflattened nose

• Bronze skin color Bronze skin color ((Greenish yellow skin Greenish yellow skin tone)tone)

• Growth and maturation Growth and maturation retardationretardation

Management:

• RBC transfusions q2-4 weeks to get Hgb to 10-12RBC transfusions q2-4 weeks to get Hgb to 10-12• Iron Chelation therapy Iron Chelation therapy

– Desferal (deferoxamine) SQDesferal (deferoxamine) SQ

• SplenectomySplenectomy

• CureCure: bone marrow stem cell transplant: bone marrow stem cell transplant– Estimated 70% do not find a suitable donorEstimated 70% do not find a suitable donor


– Observe for complications of transfusion- iron overloadObserve for complications of transfusion- iron overload

– Supporting the child and family in dealing with a Supporting the child and family in dealing with a chronic life-threatening illnesschronic life-threatening illness

– Monitor Growth and DevelopmentMonitor Growth and Development

– Refer the family for genetic counseling.Refer the family for genetic counseling.

Compare and Contrast

Iron DeficiencyIron Deficiency Sickle CellSickle Cell Thalasemia Thalasemia

•Low RBC’sLow RBC’s•Low HCTLow HCT•Low Hgb Low Hgb •Low ironLow iron•Low ferritinLow ferritin•High TIBCHigh TIBC

•Low RBC’sLow RBC’s•Low HCTLow HCT•Mod low Hgb Mod low Hgb •Normal ironNormal iron•Normal Normal ferritinferritin•Normal TIBCNormal TIBC•Inc BillirubinInc Billirubin

•Low RBC’sLow RBC’s•Low HCTLow HCT•Very low Hgb Very low Hgb •Increased ironIncreased iron•Normal Normal ferritinferritin•Normal TIBCNormal TIBC

Bleeding Disorders

• Hemophilia AHemophilia A

• Von Willebrand’s DiseaseVon Willebrand’s Disease

• ITP (Immune Thrombocytopenic ITP (Immune Thrombocytopenic Pupura)Pupura)

Clotting

• Host of factorsHost of factors

• Platelets aggregation at site of injuryPlatelets aggregation at site of injury

• Tested by coagulation time (PT/PTT)Tested by coagulation time (PT/PTT)

Hemophilia A

• Hereditary blood coagulation deficiency Hereditary blood coagulation deficiency (factor 8)(factor 8)

• Ability to clot is slowerAbility to clot is slower

• X-linked recessive (white, males)X-linked recessive (white, males)

Symptoms

• Vary according to Vary according to concentration of factor concentration of factor 88

• Soft tissue bleeding Soft tissue bleeding and painful and painful hemorrhage into joints hemorrhage into joints

• Severe bleeding may Severe bleeding may occur in GI tract, occur in GI tract, peritoneum or CNSperitoneum or CNS

Interviewing the Child with Hemophilia–Subjective Data

• Recent traumas and measures used to stop Recent traumas and measures used to stop bleedingbleeding

• Length of time pressure was applied before Length of time pressure was applied before bleeding subsidedbleeding subsided

• Whether swelling increased after surface Whether swelling increased after surface bleeding subsidedbleeding subsided

• Whether swelling and stiffness occurred Whether swelling and stiffness occurred without apparent traumawithout apparent trauma

Diagnosis

• Above HistoryAbove History

• Suspected by Labs:Suspected by Labs:– Platelet level: NormalPlatelet level: Normal– PTT: Prolonged (elevated number) > 60PTT: Prolonged (elevated number) > 60

• Confirmed by genetic testing for missing Confirmed by genetic testing for missing factorfactor

Management of Bleeding

Acute therapy:Acute therapy:• Bleeding must be controlled by IV administration of Bleeding must be controlled by IV administration of

factor 8factor 8– After trauma, surgeryAfter trauma, surgery

• Pressure to lacerationPressure to laceration

Prophylactic therapy:Prophylactic therapy: • Children age 1-2 receive PO factor 8 replacement on a Children age 1-2 receive PO factor 8 replacement on a

regular schedule if frequently symptomatic regular schedule if frequently symptomatic • prior to surgery, dental workprior to surgery, dental work

Parental Education

• Primary Goal: Injury PreventionPrimary Goal: Injury Prevention

• Promote oral hygiene, up to date Promote oral hygiene, up to date immunizationsimmunizations

• No aspirin No aspirin

• Avoid activities that induce bleeding Avoid activities that induce bleeding

• Provide activities for normal G&DProvide activities for normal G&D

• Administration of factor replacement prnAdministration of factor replacement prn

Von Willebrand’s Disease• Most commonly inherited bleeding disorder, Most commonly inherited bleeding disorder,

autosomal dominant (Males and Females)autosomal dominant (Males and Females)• Lacks production of VWFLacks production of VWF• Platelets are normal in number Platelets are normal in number • Inability of platelets to aggregateInability of platelets to aggregate• Varying degrees of diseaseVarying degrees of disease

– VWF is deficient to defectiveVWF is deficient to defective

Diagnosis

• Platelets is normalPlatelets is normal

• PT/PTT is normalPT/PTT is normal

• Confirmed by genetic testing for VWFConfirmed by genetic testing for VWF

Signs & Symptoms

Can be so mild that disease Can be so mild that disease is undiagnosedis undiagnosed

EpitaxisEpitaxis Prolonged Prolonged

bleeding from cutsbleeding from cuts Excessive bleeding Excessive bleeding

following surgeryfollowing surgery

• Bleeding from gumsBleeding from gums

Management

• Prophylactic therapy -Replace Prophylactic therapy -Replace dysfunctional factor in blooddysfunctional factor in blood– Treatment of Choice: DDAVPTreatment of Choice: DDAVP

• Injury Prevention Injury Prevention

ITP (Immune Thrombocytopenic Pupura)

• Autoimmune disorder (antiplatelet Autoimmune disorder (antiplatelet antibody) or cause is unknown antibody) or cause is unknown (idiopathic)(idiopathic)

• Occurs most commonly at age 2-4 yearsOccurs most commonly at age 2-4 years

• Reduction in and destruction of plateletsReduction in and destruction of platelets

• Typically seen 2 weeks after a febrile, Typically seen 2 weeks after a febrile, viral illnessviral illness

Signs & Symptoms

• Excessive bruising and Excessive bruising and petechiaepetechiae

• EpitaxisEpitaxis

• Bleeding into jointsBleeding into joints

• Tourniquet test: shows Tourniquet test: shows many petechiae after many petechiae after inflation of BP cuffinflation of BP cuff

Diagnosis

LabsLabs::• Platelets < 150 Platelets < 150 (Marked thrombocytopenia) (Marked thrombocytopenia)

• PT and PTT: NormalPT and PTT: Normal

Management• Prednisone Prednisone • IVIG (IV immunoglobulin)IVIG (IV immunoglobulin)• PLT transfusion (only a temporary solution)PLT transfusion (only a temporary solution)• Most cases are self-limiting Most cases are self-limiting • Avoid when possible:Avoid when possible:

– administering intramuscular injectionsadministering intramuscular injections– aspirin, aspirin-containing products, and nonsteroidal aspirin, aspirin-containing products, and nonsteroidal

antiinflammatory medications (e.g., ibuprofen)antiinflammatory medications (e.g., ibuprofen)– taking temperatures rectally taking temperatures rectally – perform invasive procedures with extreme cautionperform invasive procedures with extreme caution


Hemophilia AHemophilia A VWFVWF ITPITP

Normal PlateletsNormal Platelets

Elevated Elevated PT/PTTPT/PTT

Normal PlateletsNormal Platelets

Normal PT/PTTNormal PT/PTT

Very Low Very Low PlateletsPlatelets

Normal PT/PTTNormal PT/PTT

Oncology

• Cancer in adults Cancer in adults – abnormal cell is transformed by genetic mutation of its abnormal cell is transformed by genetic mutation of its

DNADNA

– usually as a result from exposure to a tetragon usually as a result from exposure to a tetragon

• Cancer in childrenCancer in children– usually arises from chromosomal abnormalities, genetic usually arises from chromosomal abnormalities, genetic

mutations and proliferation of embryonic cellsmutations and proliferation of embryonic cells

Oncology Treatments

• Chemotherapy Chemotherapy – antineoplastic agentsantineoplastic agents

– attempt to destroy tumor cells by interfering with attempt to destroy tumor cells by interfering with cellular functions and reproduction cellular functions and reproduction

– cytotoxic drugs that are designed to cause cell death. cytotoxic drugs that are designed to cause cell death.

• Normal cells that have rapid growth are also Normal cells that have rapid growth are also affected, such as hair growth. affected, such as hair growth.

• Toxic side effectsToxic side effects

Oncology Treatments

• Surgical intervention Surgical intervention – removing the entire cancerous tumor (most removing the entire cancerous tumor (most

ideal and frequently used treatment method)ideal and frequently used treatment method)

• Radiation therapy Radiation therapy – interrupt cellular growth by breaking the DNA interrupt cellular growth by breaking the DNA

stands, leading to cell death. stands, leading to cell death.

Types of Cancer in Children

• Small percentage Carcinoma (opposed to Small percentage Carcinoma (opposed to large percentage in adults)large percentage in adults)

• Mostly LeukemiaMostly Leukemia

• Followed by LymphomaFollowed by Lymphoma

• The rest is solid or soft tissue tumorsThe rest is solid or soft tissue tumors

Clinical Manifestations

• Differ based on type of cancerDiffer based on type of cancer

• Many symptoms are similar to common Many symptoms are similar to common childhood illnesseschildhood illnesses

• Symptoms may be in site other than the Symptoms may be in site other than the cancercancer

• =delay in diagnosis=delay in diagnosis

• Often diagnosis made when cancer is Often diagnosis made when cancer is advancedadvanced

Common Clinical Manifestations

• PainPain

• AnemiaAnemia

• Anorexia, weight lossAnorexia, weight loss

• InfectionsInfections

• BruisingBruising

• Neurological symptomsNeurological symptoms

• Palpable massPalpable mass

Psychosocial Concerns

• Parents in disbeliefParents in disbelief

• Health child suddenly becomes illHealth child suddenly becomes ill

• Potentially life-threateningPotentially life-threatening

• Treatment decisions, can last months-yearsTreatment decisions, can last months-years

• Travel for treatment, heavy financial Travel for treatment, heavy financial responsibilitiesresponsibilities

• Effects of siblingsEffects of siblings

Effects on Child

• Infants- unaware of diagnosisInfants- unaware of diagnosis

• Toddlers- aware they do not feel wellToddlers- aware they do not feel well

• Preschoolers-beginning understanding of Preschoolers-beginning understanding of illness, not cancerillness, not cancer

• School-age-understand cancer, benefit from School-age-understand cancer, benefit from talking about ittalking about it

• Adolescents-mature understanding, benefits Adolescents-mature understanding, benefits from other adolescents with cancerfrom other adolescents with cancer

General Nursing Considerations

• Provide optimal nutrition- high metabolic Provide optimal nutrition- high metabolic rate of cancer depletes storesrate of cancer depletes stores

• Ensure adequate hydration-ice pops, jelloEnsure adequate hydration-ice pops, jello

• Manage painManage pain

• Promote growth and developmentPromote growth and development

• Prevent Infection (next slide)Prevent Infection (next slide)

Risk for infection r/t Immunosuppressed state.

• Monitor vital signs q4hMonitor vital signs q4h• Instruct parents how to measure temp at home Instruct parents how to measure temp at home • Proper handwashingProper handwashing• Inspect child’s skin for breakdownInspect child’s skin for breakdown• Inspect child’s mouth for ulcersInspect child’s mouth for ulcers• Teach child and parents meticulous oral Teach child and parents meticulous oral

hygienehygiene• No live virus administrationNo live virus administration

Leukemia

Leukemia

• Broad term describing a group of malignant Broad term describing a group of malignant diseasesdiseases

• Normal Bone Marrow is replaced by abnormal Normal Bone Marrow is replaced by abnormal immature cellsimmature cells

• Etiology: variety of agents thought to increase risk Etiology: variety of agents thought to increase risk (virus, toxins, drugs) combined with genetics(virus, toxins, drugs) combined with genetics

• Two forms of leukemiaTwo forms of leukemia– ALL: Acute Lymphocytic LeukemiaALL: Acute Lymphocytic Leukemia– AML: Acute Myelogenous LeukemiaAML: Acute Myelogenous Leukemia

Acute Lymphocytic Leukemia

• Most frequently occurring type of cancer in children < 15yo (peak 2-6)

• Distorted and uncontrolled proliferation of immature WBC’s (lymphoblasts)

• Causes decreased RBC’s, platelets, and mature WBC’s production and invasion of body organs by rapidly increasing lymphoblasts

Signs & Symptoms:• FeverFever• Bone or joint painBone or joint pain• BruisingBruising• Decreased RBC’sDecreased RBC’s• Decreased PLT’sDecreased PLT’s• Abnormal high WBC countsAbnormal high WBC counts• LymphadenopathyLymphadenopathy• HepatosplenomegalyHepatosplenomegaly• CNS invasionCNS invasion

Diagnosis:

Based onBased on::

• Signs & symptomsSigns & symptoms

• CBC changesCBC changes

• Bone marrow aspiration (> 25% of Bone marrow aspiration (> 25% of lymphoblast cells present) lymphoblast cells present)

Management

Chemotherapy in 3 stagesChemotherapy in 3 stages

For 2-3 yearsFor 2-3 years

1.1. InductionInduction

2.2. SanctuarySanctuary

3.3. MaintenanceMaintenance

Induction

• 11stst month; aim is to induce remission month; aim is to induce remission (blast cells to < 5%, normal Physical (blast cells to < 5%, normal Physical Findings)Findings)

• Approximately 95% of children Approximately 95% of children achieve remission within 1 monthachieve remission within 1 month

Sanctuary or Consolidation

Begins after remission, 4 weeksBegins after remission, 4 weeks

GoalGoal::

• to maintain remissionto maintain remission

• prevent disease from invading prevent disease from invading sanctuary sitessanctuary sites

Maintenance

• goal to maintain remissiongoal to maintain remission• eliminate residual leukemic cellseliminate residual leukemic cells• combination of drugs, outpatient combination of drugs, outpatient

basisbasis• girls treated for 2 years, boys for 3girls treated for 2 years, boys for 3

Cure: free of disease for 4-5 yearsCure: free of disease for 4-5 years

High Doses of Chemotherapy Can Lead to:

• Tumor Lysis SyndromeTumor Lysis Syndrome– Metabolic emergencyMetabolic emergency

• results from the lysis (dissolving or results from the lysis (dissolving or decomposing) of tumor cells and rapid decomposing) of tumor cells and rapid release of their contents into the bloodrelease of their contents into the blood

Tumor lysis syndrome • Rapid cell destruction releases high levels ofRapid cell destruction releases high levels of

– uric aciduric acid– potassiumpotassium– phosphates phosphates

• Uric acid overloads the kidneysUric acid overloads the kidneys• Leads to Leads to cardiac arrhythmias and renal failurecardiac arrhythmias and renal failure


• Children receiving chemotherapy;Children receiving chemotherapy;

• Monitor for:Monitor for:– HyperuricemiaHyperuricemia– HyperkalemiaHyperkalemia– HyperphosphatemiaHyperphosphatemia– HypocalcemiaHypocalcemia


• Administer vigorous hydration (2–4 times Administer vigorous hydration (2–4 times rate for maintenance fluid)rate for maintenance fluid)

• Administer allopurinol or urate oxidase Administer allopurinol or urate oxidase (rasburicase) to reduce conversion of (rasburicase) to reduce conversion of metabolic by-products to uric acidmetabolic by-products to uric acid

Soft Tissue Tumors

Hodgkin'sHodgkin's

Non Hodgkin'sNon Hodgkin's

RetinoblastomaRetinoblastoma

Lymphomas

• A malignancy that arises from the lymphoid A malignancy that arises from the lymphoid system system

• Two types:Two types:– HodgkinsHodgkins– Non HodgkinsNon Hodgkins

Hodgkin’s Disease• Neoplasm of cervical lymphatic tissueNeoplasm of cervical lymphatic tissue• Starts in a single or group of lymph Starts in a single or group of lymph

nodes then spreads predictably to nodes then spreads predictably to nonnodal sites such as: spleen, liver, nonnodal sites such as: spleen, liver, bone, marrow, lungs, mediastinumbone, marrow, lungs, mediastinum

• Affects adolescents to late 20’sAffects adolescents to late 20’s• Males > femalesMales > females• Etiology unknown- infectious agent Etiology unknown- infectious agent

likelylikely

Signs & Symptoms:

• Painless enlarged Painless enlarged cervical nodecervical node

• Unexplained Unexplained weight loss, weight loss, unexplained unexplained fevers, night fevers, night sweatssweats

Diagnosis and Treatment

DiagnosisDiagnosis• Biopsy of enlarged lymph nodeBiopsy of enlarged lymph node• Staged 1-4Staged 1-4

TreatmentTreatment• ChemotherapyChemotherapy• Radiation-low doses, higher is physiologically matureRadiation-low doses, higher is physiologically mature• Good Prognosis-single originGood Prognosis-single origin

Non-Hodgkin’s Lymphoma• No single originNo single origin• Males > femalesMales > females• Cause unknown Cause unknown • Aggressive proliferation of B or T lymphocytes in Aggressive proliferation of B or T lymphocytes in

lymph nodeslymph nodes• Rapid in onset (ages 5-15)Rapid in onset (ages 5-15)• Usually found with wide-spread involvement via Usually found with wide-spread involvement via

bloodstream (multiple enlarged nodes)bloodstream (multiple enlarged nodes)• Responds quickly to therapyResponds quickly to therapy

Signs & Symptoms:

• Acute abdominal and chest pain, constipation, Acute abdominal and chest pain, constipation, crampingcramping

• Anorexia, weight lossAnorexia, weight loss• Painless enlarged lymph nodes found in cervical Painless enlarged lymph nodes found in cervical

or axillary regionor axillary region• Ascites and obstruction with vomiting a late signAscites and obstruction with vomiting a late sign• Advanced disease: CNS symptoms, HA n/v, Advanced disease: CNS symptoms, HA n/v,

mediastinal mass, petichaie, bruising, bone painmediastinal mass, petichaie, bruising, bone pain

DiagnosisDiagnosis• Biopsy from bone marrow or lymph nodeBiopsy from bone marrow or lymph node• Staging 1-4Staging 1-4

Treatment:Treatment:• Aggressive multi-agent chemo for 6 mos to 2 Aggressive multi-agent chemo for 6 mos to 2

yearsyears• Risk for tumor lysis syndromeRisk for tumor lysis syndrome• Intrathecal chemo and crainal radiationIntrathecal chemo and crainal radiation


HodgkinsHodgkins Non HodgkinsNon Hodgkins

Males>FemalesMales>Females

Late adolescent-20’sLate adolescent-20’s

Single origin of cervical Single origin of cervical glandgland

Good PrognosisGood Prognosis

Males > femalesMales > females

ages 5-15ages 5-15

No single origin-wide-No single origin-wide-spread involvementspread involvement

Aggressive treatmentAggressive treatment

Retinoblastoma• Malignant tumor of retinaMalignant tumor of retina• Inherited autosomal dominantInherited autosomal dominant• Immature retinal cells become malignantImmature retinal cells become malignant• 6 weeks of age to preschool age6 weeks of age to preschool age• Unilateral or bilateralUnilateral or bilateral

Signs & Symptoms

• Absent red reflexAbsent red reflex• Whitish glow to pupilWhitish glow to pupil• Strabismus developsStrabismus develops• Eye painEye pain• Metastases to optic Metastases to optic

nerve, subarachnoid nerve, subarachnoid space, brain, 2space, brain, 2ndnd eye eye

Retinoblastoma

TreatmentTreatment::

• If small: cryosurgery, partial visionIf small: cryosurgery, partial vision

• If mets: chemo & radiationIf mets: chemo & radiation

• If large: enucleation, eye prosthesis 3 If large: enucleation, eye prosthesis 3 weeks post-opweeks post-op

• Survival rate 90%Survival rate 90%

Solid Tumors

NeuroblastomaNeuroblastoma

NephroblastomaNephroblastoma

OsteoscaromaOsteoscaroma

Ewing’s SarcomaEwing’s Sarcoma

Neuroblastoma

• Solid tumor of infants and pre-school Solid tumor of infants and pre-school children (peak 22mos)children (peak 22mos)

• Cancer cells arise from sympathetic Cancer cells arise from sympathetic nervous system called crest cellsnervous system called crest cells– Embryologic cells of adrenal glandsEmbryologic cells of adrenal glands

Etiology: unknownEtiology: unknown

Signs & Symptoms

• Depend on:Depend on:– extent of diseaseextent of disease– location of tumorlocation of tumor

• 65% present with protuberant, firm, 65% present with protuberant, firm, irregular abdominal mass that crosses irregular abdominal mass that crosses midlinemidline

Neuroblastoma

Other manifestationsOther manifestations::

• impaired ROM & mobilityimpaired ROM & mobility

• pain & limpingpain & limping

• large abdominal mass large abdominal mass

• respiratory symptoms if chest tumorrespiratory symptoms if chest tumor

Neuroblastoma

DiagnosisDiagnosis: : • Chest x-rayChest x-ray• CT scan of abdomen, pelvis, spineCT scan of abdomen, pelvis, spine• Bone marrow aspirationBone marrow aspiration

Management:Management:• depends on the presence and extent of depends on the presence and extent of

metastasismetastasis

Wilm’s Tumor (Nephroblastoma)

• Malignant tumor of the kidneysMalignant tumor of the kidneys• Peak age 3-4 yearsPeak age 3-4 years• Girls > boysGirls > boys• Cause is unknownCause is unknown• Other GU problemsOther GU problems

• Occurs in asymptomatic childOccurs in asymptomatic child– May have genetic predispositionMay have genetic predisposition– Is associated with congenital Is associated with congenital

anomaliesanomalies

Nephroblastoma

• Parents usually notice a large, mobile Parents usually notice a large, mobile abdominal mass while bathing or the diaper abdominal mass while bathing or the diaper doesn’t fit anymoredoesn’t fit anymore

• Grows extremely quickly, in a matter of Grows extremely quickly, in a matter of daysdays

• DO NOT PALPATE ABDOMEN DO NOT PALPATE ABDOMEN – can rupture the tumor and cause spreading of can rupture the tumor and cause spreading of

cancerous cellscancerous cells

Other Signs & Symptoms

• microscopic to gross hematuriamicroscopic to gross hematuria

• hypertensionhypertension

• abdominal painabdominal pain

• fatigue, anemia, feverfatigue, anemia, fever

Diagnosis• Suspected from a good historySuspected from a good history

• CT scanCT scan

• Definitive dx made at time of surgeryDefinitive dx made at time of surgery

• Staged 1-5Staged 1-5

Staging 1 through 51.1. tumor confined to the kidney and completely tumor confined to the kidney and completely

removed surgicallyremoved surgically

2.2. tumor extending beyond the kidney but tumor extending beyond the kidney but completely removed surgicallycompletely removed surgically

3.3. regional spread of disease beyond the kidney regional spread of disease beyond the kidney with residual abdominal disease postoperativelywith residual abdominal disease postoperatively

4.4. metastases to metastases to lunglung (primary site), liver, bone, (primary site), liver, bone, distant lymph nodesdistant lymph nodes

5.5. bilateral diseasebilateral disease

Treatment• State 1 and 2 State 1 and 2

– NephrectomyNephrectomy– ChemotherapyChemotherapy

• Stage 3-5Stage 3-5– NephrectomyNephrectomy– RadiationRadiation– ChemotherapyChemotherapy

• Survival rates are good (up to 90%)Survival rates are good (up to 90%)

Bone Cancers

Osteogenic Sarcoma (Osteosarcoma)

• Most common bone malignancy in Most common bone malignancy in children (teenage years)children (teenage years)

• Occurs in distal long bonesOccurs in distal long bones• Attributed to extremity injury or Attributed to extremity injury or

growth spurtgrowth spurt• Originates from bone producing cellsOriginates from bone producing cells• 40-50% occur at distal femur and knee40-50% occur at distal femur and knee

Signs & symptoms• Progressive, insidious or intermittent pain Progressive, insidious or intermittent pain

at site of tumorat site of tumor• Palpable mass & swellingPalpable mass & swelling• Limping, progressive limited range of Limping, progressive limited range of

motionmotion• Pathological fracturesPathological fractures

DiagnosisDiagnosis

• X-ray, CT, MRIX-ray, CT, MRI

• Tumor biopsy Tumor biopsy

• Look for chest metastasesLook for chest metastases

Management• Remove tumor, prevent spread of diseaseRemove tumor, prevent spread of disease

• Combination of surgery & chemoCombination of surgery & chemo

• Amputation my be necessaryAmputation my be necessary

• Limb salvage operationLimb salvage operation

Cure rate: 60-65% without overt metastasesCure rate: 60-65% without overt metastases

Nursing care

• ComfortComfort• InfectionInfection• Potential hemorrhagePotential hemorrhage• Phantom limb painPhantom limb pain• ProsthesisProsthesis• Changes in body image and Changes in body image and

functioningfunctioning

Ewing’s Sarcoma• Highly malignant tumor Highly malignant tumor

in bone marrow of long in bone marrow of long bonesbones

• Can present in any boneCan present in any bone• Spreads longitudinally Spreads longitudinally

through bonethrough bone• Affects young adolescents Affects young adolescents

and older childrenand older children• Metastases is usually Metastases is usually

present at time of dx present at time of dx (lungs, bone, CNS, lymph (lungs, bone, CNS, lymph nodes)nodes)

Signs & Symptoms

• Intermittent pain attributed to injuryIntermittent pain attributed to injury• Swelling at tumor siteSwelling at tumor site• Pain becomes constantPain becomes constant• Progresses intoProgresses into

– Weight lossWeight loss– Fever Fever – Increased sed rateIncreased sed rate

DiagnosisDiagnosis::

• Bone scanBone scan

• Bone marrow aspiration & biopsyBone marrow aspiration & biopsy

• CT of lungsCT of lungs

• Definitive dx: biopsy of tumor siteDefinitive dx: biopsy of tumor site

Treatment:Treatment:

• SurgerySurgery

• Multi agent chemoMulti agent chemo

• RadiationRadiation

Compare and ContrastOsteogenic scaromaOsteogenic scaroma Ewing’s SarcomaEwing’s Sarcoma

Affects long bonesAffects long bones

Older adolescentsOlder adolescents

Intermittent pain Palpable Intermittent pain Palpable mass & swellingmass & swelling

Limping, progressive Limping, progressive limited range of motionlimited range of motion

Pathological fracturesPathological fractures

Metastases not as likelyMetastases not as likely

Surgery and chemoSurgery and chemo

Affects any boneAffects any bone

School-age and adolescentsSchool-age and adolescents

Intermittent pain becomes Intermittent pain becomes constantconstant

Swelling at tumor siteSwelling at tumor site

Progresses into systemic Progresses into systemic symptomssymptoms

Metastases likelyMetastases likely

Aggressive treatmentAggressive treatment

Chronically Ill ChildNursing Diagnosis

• Fear Fear

• Death Anxiety Death Anxiety

• Anticipatory Grieving Anticipatory Grieving

• Hopelessness Hopelessness

Goals for Care of the Chronically Ill Child

• Goals for the childGoals for the child

– Achieve and maintain normalizationAchieve and maintain normalization

– Obtain the highest level of health and function possibleObtain the highest level of health and function possible

• Goals for the familyGoals for the family

– Remain intactRemain intact

– Achieve and maintain normalizationAchieve and maintain normalization

– Maximize function throughout Maximize function throughout the illnessthe illness

Nursing Care for Children with Chronic Conditions

and Their Families

• Attend to the needs of the family systemAttend to the needs of the family system

• Revise goals frequently to meet the child’s Revise goals frequently to meet the child’s changing developmental needschanging developmental needs

• Listen carefully to the child's perception of Listen carefully to the child's perception of the conditionthe condition

Nursing Care for the Dying Child and the Child’s Family

• Be available to assist both child and familyBe available to assist both child and family

• Avoid imposing personal beliefs and expectationsAvoid imposing personal beliefs and expectations

• Provide time and attention to the dying childProvide time and attention to the dying child

• Recognize the need to talk about illness Recognize the need to talk about illness and deathand death

• Provide adequate pain control, oral care, privacy, and Provide adequate pain control, oral care, privacy, and information about the signs of imminent deathinformation about the signs of imminent death

• After death, allow family members as much time as they After death, allow family members as much time as they desire with the childdesire with the child

Practice Questions!

A child is being admitted to the unit with A child is being admitted to the unit with thalassemia major (Cooley’s anemia). In thalassemia major (Cooley’s anemia). In preparing client assignments, the charge nurse preparing client assignments, the charge nurse wants to assign a nurse to this child who can:wants to assign a nurse to this child who can:

1.1. Teach dietary sources of ironTeach dietary sources of iron2.2. Administer blood infusionsAdminister blood infusions3.3. Work with a dying childWork with a dying child4.4. Monitor the child for bleeding tendenciesMonitor the child for bleeding tendencies

A 14-year-old boy with sickle cell anemia is A 14-year-old boy with sickle cell anemia is admitted with severe pain in his abdomen and admitted with severe pain in his abdomen and legs. He asks why the doctor ordered oxygen legs. He asks why the doctor ordered oxygen when he is not having any breathing problems. when he is not having any breathing problems. The nurse states the therapeutic action of O2 is:The nurse states the therapeutic action of O2 is:

1.1. Prevent further sicklingPrevent further sickling2.2. Prevent respiratory complicationsPrevent respiratory complications3.3. Increase O2 capacity of RBCsIncrease O2 capacity of RBCs4.4. Decrease the potential for infectionDecrease the potential for infection

A 10-year old in the ER has a CBC results that A 10-year old in the ER has a CBC results that include a Hgb of 8, and Hct of 24. The nurse include a Hgb of 8, and Hct of 24. The nurse determines that based on the lab results which determines that based on the lab results which nursing action has a high priority?nursing action has a high priority?

1.1. Promotion of skin integrityPromotion of skin integrity2.2. Promotion of hydrationPromotion of hydration3.3. Promotion of nutritionPromotion of nutrition4.4. Conserving energyConserving energy

A 4-year-old is diagnosed with ALL. Following A 4-year-old is diagnosed with ALL. Following teaching about the staging and therapy, the nurse teaching about the staging and therapy, the nurse evaluates the family’s understanding of the evaluates the family’s understanding of the problem. The statement by the family that problem. The statement by the family that indicates appropriate knowledge is “Staging indicates appropriate knowledge is “Staging will:will:

1.1. Determine the extent of the tumor process and Determine the extent of the tumor process and need for palliationneed for palliation

2.2. Help determine if treatment is neededHelp determine if treatment is needed3.3. Determine if surgery is neededDetermine if surgery is needed4.4. Determine the extent of malignant process and Determine the extent of malignant process and

stage the leukemiastage the leukemia

• A 17-year old is being admitted for an A 17-year old is being admitted for an amputation related to a bone tumor. The amputation related to a bone tumor. The nurse is developing a nursing care plan and nurse is developing a nursing care plan and determines the most appropriate age related determines the most appropriate age related diagnosis is:diagnosis is:

1.1. Risk for disuse syndromeRisk for disuse syndrome

2.2. Disturbed body imageDisturbed body image

3.3. Self-care deficitSelf-care deficit

4.4. Activity related intoleranceActivity related intolerance

The nurse is reviewing the lab work on a child admitted for fatigue

– WBC 7,200WBC 7,200

– RBC3.01RBC3.01

– Hgb 9.1Hgb 9.1

– Hct 29.3Hct 29.3

– Platelets 371,000Platelets 371,000

• Iron 64Iron 64• Ferritin 70Ferritin 70• Transferrin 250Transferrin 250• Bilirubin 18.2Bilirubin 18.2

• PTT 45 secondsPTT 45 seconds

• After analyzing the results, the nurse After analyzing the results, the nurse suspects the child may have:suspects the child may have:

• 1. Fe Deficiency Anemia1. Fe Deficiency Anemia

• 2. Cooley’s Anemia2. Cooley’s Anemia

• 3. Sickle Cell Anemia3. Sickle Cell Anemia

• 4. Aplastic Anemia4. Aplastic Anemia

• The nurse is admitting a child for a swollen The nurse is admitting a child for a swollen elbow. The history indicated multiple elbow. The history indicated multiple bruising. Which of the following laboratory bruising. Which of the following laboratory results heightens the nurses suspicion for results heightens the nurses suspicion for Hemophilia?Hemophilia?

• 1. Hbg 12,0001. Hbg 12,000

• 2. WBC 9,0002. WBC 9,000

• 3. Platelets 356,0003. Platelets 356,000

• 4. PTT 73 seconds4. PTT 73 seconds

Documents

Hematologic-Oncology. Common Hematologic Disorders in Children Iron-Deficiency AnemiaIron-Deficiency Anemia Sickle Cell AnemiaSickle Cell Anemia Beta-Thalasemia