Hematologic-Oncology Stressors and Adaptation

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

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

• Non-Hodgkin’s LymphomaNon-Hodgkin’s Lymphoma• NeuroblastomaNeuroblastoma• Osteogenic SarcomaOsteogenic Sarcoma• Ewing’s SarcomaEwing’s Sarcoma• RetinoblastomaRetinoblastoma

Complete Blood Count

• WBCWBC

• RBCRBC

• HgbHgb

• HctHct

• PlateletPlatelet

CBC with Differential

• WBCWBC– Neutrophils Neutrophils – Lymphocytes Lymphocytes – Monoocytes Monoocytes – Eosophils Eosophils – BasophilsBasophils

• RBCRBC– MCVMCV– MCHMCH– MCHCMCHC– RCWRCW

• 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

Pediatric Laboratory 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: 5.4-11WBC: 5.4-11– Neutrophils: 34.3-72.9Neutrophils: 34.3-72.9

– Esinophils: 2.4-4.8Esinophils: 2.4-4.8

– Basophils: 1Basophils: 1

– Lymphocytes: 13.5-52.8Lymphocytes: 13.5-52.8

– Monocytes: 3.5-13.4Monocytes: 3.5-13.4

• Platelets: 206-403Platelets: 206-403

• 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

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

Based on levels of Based on levels of

• RBCRBC

• HgbHgb

• HctHct

• IronIron

• Transferrin (TIBC)Transferrin (TIBC)

• FerritinFerritin

Diagnosis

• RBC’s RBC’s – hypocytic hypocytic – hyochromichyochromic

• HGB < 10-11gHGB < 10-11g– Mild ( < 11), Moderate (7-11), Severe (3-7)Mild ( < 11), Moderate (7-11), Severe (3-7)

• HCT < 33%HCT < 33%• Iron 30ug /100mlIron 30ug /100ml• Transferrin (TIBC) > 350ugTransferrin (TIBC) > 350ug• Ferritin < 10ugFerritin < 10ug

Symptoms

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

Hgb of 10Hgb of 10• 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 < 10Hgb < 10• Above plus irritability, lack of interest in playAbove plus irritability, lack of interest in play

History

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

• Ask parents specific questionsAsk parents specific questions• Begin the dietary history at the Begin the dietary history at the

time the child awoke yesterday; time the child awoke yesterday; include all activities and exactly include all activities and exactly what the child atewhat the child ate

Management

Dietary ModificationsDietary Modifications• Iron-fortified formula or supplements 4-6 Iron-fortified formula or supplements 4-6

weeksweeks• 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 supplementsFe supplements

– Monitor complianceMonitor compliance

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• JaundiceJaundice• SOBSOB• IrritabilityIrritability

3 Sequalea of SCA

1.1. Vaso-Occlusive CrisisVaso-Occlusive Crisis

2.2. Acute Chest SyndromeAcute Chest Syndrome

3.3. Splenic SequestrationSplenic Sequestration

Sickle Cell Crisis Vaso-occlusive crisis

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

• caused by: infection, dehydration, anxiety, caused by: infection, dehydration, anxiety, hypoxia, coldhypoxia, cold

• 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 of Crisis

Three primary needs:Three primary needs:

• 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 AROUND THE CLOCK PAIN

MEDSMEDS• Tylenol for mild painTylenol for mild pain

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

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

• Administer blood transfusions to Administer blood transfusions to increase Hgb increase Hgb

• BedrestBedrest

Altered Tissue Perfusion

• Assess vital signs and respiratory status Assess vital signs and respiratory status q4h and PRNq4h and PRN

• Pulse oximetryPulse oximetry

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

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

• Administer PRBC’sAdminister PRBC’s

Acute Chest Syndrome• Sickle contents break offSickle contents break off

• bilateral pulmonary involvementbilateral pulmonary involvement

• chest pain, fever, coughchest pain, fever, cough

Splenic Sequestration• Sickled cells block the spleenSickled cells block the spleen

• pooled blood in spleen and/or liver and enlargespooled blood in spleen and/or liver and enlarges

• Pooled blood leads to a decrease in circulating Pooled blood leads to a decrease in circulating

volume= hypovolemic shockvolume= hypovolemic shock • CVA => comaCVA => coma

• TX: PRBC, SplenectomyTX: PRBC, Splenectomy

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

• RBC’sRBC’s– hypochromic hypochromic – microcyticmicrocytic

• Hgb < 5gHgb < 5g

• 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 tone)Greenish yellow skin tone)

• Growth and maturation Growth and maturation retardationretardation

• HepatosplenomegalyHepatosplenomegaly• Diabetes MellitusDiabetes Mellitus• Cardiac dilatationCardiac dilatation

Management:

• RBC transfusions q2-4 weeks to get Hgb to 10-RBC transfusions q2-4 weeks to get Hgb to 10-1212

• Iron Chelation therapy Iron Chelation therapy – Desferal (deferoxamine) SQDesferal (deferoxamine) SQ

• SplenectomySplenectomy• Digitalis, diuretics, low salt dietDigitalis, diuretics, low salt diet

• CureCure: bone marrow stem cell transplant: bone marrow stem cell transplant

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 concentration of factor Vary according to concentration of factor 88

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

• Severe bleeding may occur in GI tract, Severe bleeding may 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

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

Management

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

• Injury PreventionInjury 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 replacementAdministration of factor replacement

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• 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

Signs & Symptoms

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

EpitaxisEpitaxisProlonged bleeding from cutsProlonged bleeding from cutsExcessive bleeding following surgeryExcessive bleeding following surgeryBleeding from gumsBleeding from gums

Management

• Replace dysfunctional factor in Replace dysfunctional factor in bloodblood

• Treatment of Choice: DDAVPTreatment of Choice: DDAVP• Prophylactic therapyProphylactic therapy• Injury Prevention Injury Prevention

ITP (Immune Thrombocytopenic Pupura)

• Autoimmune disorder (antiplatelet antibody) Autoimmune disorder (antiplatelet antibody) or cause is unknown (idiopathic)or cause is unknown (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, viral Typically seen 2 weeks after a febrile, viral

illnessillness• Congential: if mother had ITP during Congential: if mother had ITP during

pregnancypregnancy

Signs & Symptoms

• Excessive bruising and petechiaeExcessive bruising and petechiae• EpitaxisEpitaxis• Bleeding into jointsBleeding into joints• Tourniquet test: shows many petechiae after Tourniquet test: shows 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

Aplastic AnemiaBone marrow stops production of RBC, WBC and Bone marrow stops production of RBC, WBC and platelets (pancytopenia)platelets (pancytopenia)

CongenitalCongenital– inherited autosomal recessiveinherited autosomal recessive– Fanconi’s Syndrome: pancytopenia plus skeletal & renal Fanconi’s Syndrome: pancytopenia plus skeletal & renal

abnormalities, hypogenitalism, short statureabnormalities, hypogenitalism, short stature

AcquiredAcquired– IdopathicIdopathic– Excessive exposure to radiation or drugs causing bone marrow Excessive exposure to radiation or drugs causing bone marrow

damagedamage

Signs & Symptoms

Mean age is 6-8 yearsMean age is 6-8 years• BruisingBruising• AnemiaAnemia

– PallorPallor– WeaknessWeakness

• PetechiaePetechiae• EpitaxisEpitaxis• Multiple infectionsMultiple infections• Cardiac decompensationCardiac decompensation

Management:• Bone marrow transplant (if donor available)Bone marrow transplant (if donor available)• Platelet and RBC transfusionsPlatelet and RBC transfusions

– Maintain platelets > 20,000/mmMaintain platelets > 20,000/mm– HBG >7g/dlHBG >7g/dl

• Suppress autoimmune responseSuppress autoimmune response– CortiosteroidsCortiosteroids

• TestosteroneTestosteroneParent educationParent education: : Prevent infection, protect from injuryPrevent infection, protect from injury

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.

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

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

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

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)

• Possibly a lumbar puncturePossibly a lumbar puncture

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 PE)(blast cells to < 5%, normal PE)

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

Common drugs used in Induction

• Steroids (prevent cell formation)Steroids (prevent cell formation)

• Vincristine (prevents cell replication)Vincristine (prevents cell replication)

• L-Asparaginase (destroys existing cells)L-Asparaginase (destroys existing cells)

• Allopurinol (uric acid)Allopurinol (uric acid)

Tumor lysis syndrome • Can occur with WBC > 50,000Can occur with WBC > 50,000• Intracellular contents are shifted into the Intracellular contents are shifted into the

extracellular fluid as the lymphocytes are extracellular fluid as the lymphocytes are destroyed with chemo, release uric acid into destroyed with chemo, release uric acid into serumserum

• Uric acid overloads the kidneysUric acid overloads the kidneysPrevention:Prevention:• Allopurinol and IV fluids with sodium Allopurinol and IV fluids with sodium

bicarb are given to decrease the serum uric bicarb are given to decrease the serum uric acid level and alkalazine in urineacid level and alkalazine in urine

Sanctuary or Consolidation

Begins after remission, 4 weeksBegins after remission, 4 weeksGoalGoal::• to maintain remissionto maintain remission• prevent disease from invading prevent disease from invading

sanctuary sitessanctuary sites• Intrathecal MethotrexateIntrathecal Methotrexate

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

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

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 cervical nodesPainless enlarged cervical nodes• Unexplained weight loss, Unexplained weight loss,

unexplained fevers, night sweatsunexplained fevers, night sweats

Diagnosis and Treatment

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

TreatmentTreatment• ChemotherapyChemotherapy• RadiationRadiation• Good PrognosisGood Prognosis

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• StagingStaging

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

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

Neuroblastoma

Staging (1-4)Staging (1-4) extent & location of metasteses, lymph node extent & location of metasteses, lymph node

involvement, unilateral or bilateral involvementinvolvement, unilateral or bilateral involvementEarly stagesEarly stages::• Stage 1 or 2: tumor is confined to area of originStage 1 or 2: tumor is confined to area of origin• Surgical excision of tumor, follow-upSurgical excision of tumor, follow-upLater stagesLater stages::• Stage 3 and 4: tumor infiltrated out of original areaStage 3 and 4: tumor infiltrated out of original area• Surgery plus, radiation and/or chemoSurgery plus, radiation and/or chemo

Neuroblastoma

Nursing careNursing care::

• Support of family membersSupport of family members

• Pain managementPain management

• 5 year survival rate, recurrence 5 year survival rate, recurrence common in 1common in 1stst year year

• Better prognosis for children <1yoBetter prognosis for children <1yo

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 in bone marrow of Highly malignant tumor in bone marrow of long boneslong bones

• Can present in any boneCan present in any bone• Spreads longitudinally through boneSpreads longitudinally through bone• Affects young adolescents and older childrenAffects young adolescents and older children• Metastases is usually present at time of dx Metastases is usually present at time of dx

(lungs, bone, CNS, lymph nodes)(lungs, bone, CNS, lymph 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

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 nerve, subarachnoid Metastases to optic 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%

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

Case Study

• You are caring for an You are caring for an 11-month-old male 11-month-old male who has been admitted who has been admitted through the ER for through the ER for treatment of treatment of hemarthrosis of the hemarthrosis of the right elbow and right elbow and confirm a diagnosis of confirm a diagnosis of hemophiliahemophilia

• What are the most pertinent questions you What are the most pertinent questions you should ask his parents in your admission should ask his parents in your admission history?history?

• What diagnostic tests and medical What diagnostic tests and medical interventions would you anticipate being interventions would you anticipate being ordered?ordered?

• What information will you give his parents What information will you give his parents explaining hemophilia?explaining hemophilia?

• What information will you give his parents What information will you give his parents upon discharge?upon discharge?

A child is being admitted to the unit with A child is being admitted to the unit with thalassemia major. In preparing client thalassemia major. In preparing client assignments, the charge nurse wants to assign a assignments, the charge nurse wants to assign a nurse to this child who can: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 testing and therapy, the nurse teaching about the testing 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 “Tests will:indicates appropriate knowledge is “Tests 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