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

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Presentation on theme: "Hematologic-Oncology. Common Hematologic Disorders in Children Iron-Deficiency AnemiaIron-Deficiency Anemia Sickle Cell AnemiaSickle Cell Anemia Beta-Thalasemia."— Presentation transcript:

1 Hematologic-Oncology

2 Common Hematologic Disorders in Children Iron-Deficiency AnemiaIron-Deficiency Anemia Sickle Cell AnemiaSickle Cell Anemia Beta-Thalasemia Major (Cooley’s anemia)Beta-Thalasemia Major (Cooley’s anemia) Hemophilia AHemophilia A Von Willebrand’s DiseaseVon Willebrand’s Disease ITP (Immune Thrombocytopenic Pupura)ITP (Immune Thrombocytopenic Pupura)

3 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

4 Complete Blood Count WBCWBC RBCRBC HgbHgb HctHct PlateletPlatelet

5 CBC with Differential WBCWBC –Neutrophils- phagocytosis –Lymphocytes – T and B cell –Monoocytes – phagocytosis, antigen –Eosophils- allergen –Basophils-inflammatory RBCRBC –MCV- volume –MCH –MCHC –RCW- width HgbHgb HctHct PlateletPlatelet –MPV

6 PT, PTT The prothrombin time (PT) test measures how long it takes for a clot to form in a sample of blood.The prothrombin time (PT) test measures how long it takes for a clot to form in a sample of blood. Prothrombin is one of several clotting factors that are produced by the liver.Prothrombin is one of several clotting factors that are produced by the liver. The PT test evaluates the integrated function of these factors and the body’s ability to produce a clot in a reasonable amount of time.The PT test evaluates the integrated function of these factors and the body’s ability to produce a clot in a reasonable amount of time. Because the reagents used to perform the PT test vary from one laboratory to another and even within the same laboratory over time, the normal values also will fluctuate. Because the reagents used to perform the PT test vary from one laboratory to another and even within the same laboratory over time, the normal values also will fluctuate.

7 Other Labs Sed Rate (ESR)Sed Rate (ESR) IronIron TIBC (Transferrin)TIBC (Transferrin) FerritinFerritin BilirubinBilirubin

8 Pediatric Laboratory Normal Values: Children age 2-12 Years RBC: RBC: HgB: HgB: Hct: %Hct: % Sed: 1-8Sed: 1-8 WBC: 5,400-11,000WBC: 5,400-11,000 Platelets: 206, ,000 Fe: Ferritin: TIBC: PT: sec PTT: sec Bilirubin- less than 11.7

9 Anemia’s ) Reduction of: –number of red blood cells –the quantity of hemoglobin –the volume of packed red Types in Children: Iron-Deficiency Anemia Sickle Cell Anemia Beta-Thalasemia Major (Cooley’s anemia

10 Iron-Deficiency Anemia The most common hematologic disorder of infancy and childhood 9 months- 2 years, adolescence9 months- 2 years, adolescence A nutrient deficiency of inadequate dietary ironA nutrient deficiency of inadequate dietary iron Prevention: iron fortified products

11 Children at Risk low birth weight infantslow birth weight infants infants born to mothers with iron deficiency anemiainfants born to mothers with iron deficiency anemia infants born with GI defectsinfants born with GI defects chronic blood loss in older childrenchronic blood loss in older children

12 Pathophysiology Dietary Fe is bloodstream binds to transferrin (TIBC) and is delivered to RBC in bone marrow, combines with other cells to make HgbDietary Fe is bloodstream binds to transferrin (TIBC) and is delivered to RBC in bone marrow, combines with other cells to make Hgb Unused dietary Fe is stored in intestinal epithelial cells as ferritinUnused dietary Fe is stored in intestinal epithelial cells as ferritin

13 Diagnosis Low RBC’sLow RBC’s Low HGBLow HGB –Mild ( < 10.2), Moderate (8-9), Severe (< 7) Low HCTLow HCT Low IronLow Iron High Transferrin (TIBC)High Transferrin (TIBC) Low FerritinLow Ferritin

14 Symptoms Low Hgb=low O2 tissue perfusion Hgb 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 skin)Pallor/Pale mucous membranes (low hgb, not enough red color to skin) Poor muscle tone, decreased activityPoor muscle tone, decreased activity FatigueFatigue Increased HR, RRIncreased HR, RR Hgb < 9 Above plus irritability, lack of interest in playAbove plus irritability, lack of interest in play

15 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

16 Management Iron-fortified formulaIron-fortified formula Limit cow’s milk to oz/day for children >12 monthsLimit cow’s milk to oz/day for children >12 months Increase age-appropriate iron-rich foods and Vit CIncrease age-appropriate iron-rich foods and Vit C Fe supplements- Ferrous SulfateFe supplements- Ferrous Sulfate

17 Nursing Considerations Iron-Rich FoodsVitamin C Rich Foods Meats, fish, poultryOrange juice VegetablesCitrus fruits Dried fruitsStrawberries Legumes Tomatoes Enriched grain products Broccoli Whole grain cerealLeafy Green vegetables Iron-Fortified CerealPotatoes

18 Nursing Considerations Manage side effects of Ferrous SulfateManage side effects of Ferrous Sulfate –Nausea, –Anorexia –Constipation –Abdominal distress –Black stools. Give on an empty stomach if possible Give on an empty stomach if possible Monitor bowel movements and suggest increased fluid and fiber.Monitor bowel movements and suggest increased fluid and fiber.

19 Nursing Considerations Monitor development, sleep, and activity/fatigue patterns.Monitor development, sleep, and activity/fatigue patterns. Monitor hemoglobin to measure effectiveness of therapy. Monitor hemoglobin to measure effectiveness of therapy. Instruct families to keep Ferrous Sulfate locked and out of reach of children; poisoning is a serious risk. Instruct families to keep Ferrous Sulfate locked and out of reach of children; poisoning is a serious risk.

20 Sickle Cell Anemia  Autosomal recessive disorder, African Americans  Characterized by abnormal hemoglobin (HbS)  Clinical manifestations caused by obstructions due to the sickled RBC’s and destruction of sickled and normal RBC’s

21 Sickle Cell Anemia Symptoms may not appear until 6 months of age Mortality rate children < 3 yo is 15-35% Diagnosis: Amniocentesis, CVS, Newborn Screen

22 Signs & Symptoms: Initially: fever & anemia at 6 mos PallorPallor FatigueFatigue SOBSOB IrritabilityIrritability JaundiceJaundice

23 Diagnosis Moderately low Hcb and HctModerately low Hcb and Hct Normal Iron, TIBC, FerritinNormal Iron, TIBC, Ferritin Elevated BillirubinElevated Billirubin

24 3 Sequalea of SCA 1.Vaso-Occlusive Crisis 2.Acute Chest Syndrome 3.Splenic Sequestration

25 Vaso-occlusive crisis Severe, sudden onset of sickling where many new sickled cells pool in a vessel and cause pain and tissue hypoxiaSevere, sudden onset of sickling where many new sickled cells pool in a vessel and cause pain and tissue hypoxia Caused by: infection, dehydration, anxiety, coldCaused by: infection, dehydration, anxiety, cold Most common from hypoxia secondary to rapidly destroyed RBCMost common from hypoxia secondary to rapidly destroyed RBC Lasts for hours to weeksLasts for hours to weeks

26 Vaso-occlusive Crisis Early Signs: pallor, tachycardia, fever Late Signs: acute abdominal, back, extremity pain First Crisis in infants: –Dactylitis (hand & foot syndrome) swelling of hands and feet joints may be warm & swollen

27 Management Pain reliefPain relief Adequate hydrationAdequate hydration Adequate oxygenationAdequate oxygenation

28 Pain Assess pain every 1-2h or more frequentlyAssess pain every 1-2h or more frequently 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

29 Prevent Occlusion Push PO fluidsPush PO fluids IV hydration: 1.5 to 2 times normal rateIV hydration: 1.5 to 2 times normal rate Risk for fluid overloadRisk for fluid overload

30 Altered Tissue Perfusion and Prevent Further Sickling Administer oxygen to maintain saturation of 95% or higherAdminister oxygen to maintain saturation of 95% or higher Pulse oximetryPulse oximetry Semi-fowler’s positionSemi-fowler’s position Administer PRBC’sAdminister PRBC’s

31 Acute Chest Syndrome Sickle contents break offSickle contents break off Bilateral pulmonary involvementBilateral pulmonary involvement Causes chest infection, embolismCauses chest infection, embolism

32 Nursing Considerations Know the symptoms:Know the symptoms: –Chest pain –Fever –Cough –Wheeze –Tachypnea Analgesics Oxygen Hydration Incentive spirometry, Antibiotics PRBC

33 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 volume= hypovolemic shockPooled blood leads to a decrease in circulating volume= hypovolemic shock CVA => comaCVA => coma

34 Nursing Considerations Know the Symptoms:Know the Symptoms: –Irritability –Pale –Tachycardia –Pain to LUQ –Enlarged Spleen Life Threatening- get child to ED a.s.a.p.! PRBC Remove spleen

35 Risk for Infection r/t Chronic Immunosuppression Administer PCN everydayAdminister PCN everyday Up-to-date vaccinesUp-to-date vaccines Educate parents: s/s infection & respiratory distress s/s infection & respiratory distress possible triggers treat pain immediately adequate fluids

36 Beta-Thalasemia Major (Cooley’s anemia) Hereditary anemia due to abnormal synthesis of hemoglobinHereditary anemia due to abnormal synthesis of hemoglobin Life long disorderLife long disorder Mediterranean descentMediterranean descent Life threatening symptomsLife threatening symptoms

37 Diagnosis Low RBC’sLow RBC’s Extremely low Hgb < 5Extremely low Hgb < 5 Increased serum ironIncreased serum iron

38 Symptoms Facial anomaliesFacial anomalies –Frontal bossing (prominent and protruding forehead) –Maxillary prominence –Wide-set eyes with a flattened nose Bronze skin color (Greenish yellow skin tone)Bronze skin color (Greenish yellow skin tone) Growth and maturation retardationGrowth and maturation retardation

39 Management: RBC transfusions q2-4 weeks to get Hgb to RBC transfusions q2-4 weeks to get Hgb to Iron Chelation therapyIron Chelation therapy –Desferal (deferoxamine) SQ SplenectomySplenectomy Cure: bone marrow stem cell transplantCure: bone marrow stem cell transplant –Estimated 70% do not find a suitable donor

40 Nursing Considerations –Observe for complications of transfusion- iron overload –Supporting the child and family in dealing with a chronic life-threatening illness –Monitor Growth and Development –Refer the family for genetic counseling.

41 Compare and Contrast Iron Deficiency Sickle Cell Thalasemia Low RBC’sLow RBC’s Low HCTLow HCT Low HgbLow Hgb Low ironLow iron Low ferritinLow ferritin High TIBCHigh TIBC Low RBC’sLow RBC’s Low HCTLow HCT Mod low HgbMod low Hgb Normal ironNormal iron Normal ferritinNormal ferritin Normal TIBCNormal TIBC Inc BillirubinInc Billirubin Low RBC’sLow RBC’s Low HCTLow HCT Very low HgbVery low Hgb Increased ironIncreased iron Normal ferritinNormal ferritin Normal TIBCNormal TIBC

42 Bleeding Disorders Hemophilia AHemophilia A Von Willebrand’s DiseaseVon Willebrand’s Disease ITP (Immune Thrombocytopenic Pupura)ITP (Immune Thrombocytopenic Pupura)

43 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)

44 Hemophilia A Hereditary blood coagulation deficiency (factor 8)Hereditary blood coagulation deficiency (factor 8) Ability to clot is slowerAbility to clot is slower X-linked recessive (white, males)X-linked recessive (white, males)

45 Symptoms Vary according to concentration of factor 8Vary according to concentration of factor 8 Soft tissue bleeding and painful hemorrhage into jointsSoft tissue bleeding and painful hemorrhage into joints Severe bleeding may occur in GI tract, peritoneum or CNSSevere bleeding may occur in GI tract, peritoneum or CNS

46 Interviewing the Child with Hemophilia–Subjective Data Recent traumas and measures used to stop bleedingRecent traumas and measures used to stop bleeding Length of time pressure was applied before bleeding subsidedLength of time pressure was applied before bleeding subsided Whether swelling increased after surface bleeding subsidedWhether swelling increased after surface bleeding subsided Whether swelling and stiffness occurred without apparent traumaWhether swelling and stiffness occurred without apparent trauma

47 Diagnosis Above HistoryAbove History Suspected by Labs:Suspected by Labs: –Platelet level: Normal – PTT: Prolonged (elevated number) > 60 Confirmed by genetic testing for missing factorConfirmed by genetic testing for missing factor

48 Management of Bleeding Acute therapy: Bleeding must be controlled by IV administration of factor 8Bleeding must be controlled by IV administration of factor 8 –After trauma, surgery Pressure to lacerationPressure to laceration Prophylactic therapy: Children age 1-2 receive PO factor 8 replacement on a regular schedule if frequently symptomaticChildren age 1-2 receive PO factor 8 replacement on a regular schedule if frequently symptomatic prior to surgery, dental workprior to surgery, dental work

49 Parental Education Primary Goal: Injury PreventionPrimary Goal: Injury Prevention Promote oral hygiene, up to date immunizationsPromote oral hygiene, up to date immunizations No aspirinNo aspirin Avoid activities that induce bleedingAvoid activities that induce bleeding Provide activities for normal G&DProvide activities for normal G&D Administration of factor replacement prnAdministration of factor replacement prn

50 Von Willebrand’s Disease Most commonly inherited bleeding disorder, autosomal dominant (Males and Females)Most commonly inherited bleeding disorder, autosomal dominant (Males and Females) Lacks production of VWFLacks production of VWF Platelets are normal in numberPlatelets are normal in number Inability of platelets to aggregateInability of platelets to aggregate Varying degrees of diseaseVarying degrees of disease –VWF is deficient to defective

51 Diagnosis Platelets is normalPlatelets is normal PT/PTT is normalPT/PTT is normal Confirmed by genetic testing for VWFConfirmed by genetic testing for VWF

52 Signs & Symptoms Can be so mild that disease is undiagnosed  Epitaxis  Prolonged bleeding from cuts  Excessive bleeding following surgery Bleeding from gums

53 Management Prophylactic therapy -Replace dysfunctional factor in bloodProphylactic therapy -Replace dysfunctional factor in blood –Treatment of Choice: DDAVP Injury PreventionInjury Prevention

54 ITP (Immune Thrombocytopenic Pupura) Autoimmune disorder (antiplatelet antibody) or cause is unknown (idiopathic)Autoimmune disorder (antiplatelet antibody) 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 illnessTypically seen 2 weeks after a febrile, viral illness

55 Signs & Symptoms Excessive bruising and petechiaeExcessive bruising and petechiae EpitaxisEpitaxis Bleeding into jointsBleeding into joints Tourniquet test: shows many petechiae after inflation of BP cuffTourniquet test: shows many petechiae after inflation of BP cuff

56 Diagnosis Labs: Platelets < 150 (Marked thrombocytopenia)Platelets < 150 (Marked thrombocytopenia) PT and PTT: NormalPT and PTT: Normal

57 Management PrednisonePrednisone IVIG (IV immunoglobulin)IVIG (IV immunoglobulin) PLT transfusion (only a temporary solution)PLT transfusion (only a temporary solution) Most cases are self-limitingMost cases are self-limiting Avoid when possible:Avoid when possible: –administering intramuscular injections – aspirin, aspirin-containing products, and nonsteroidal antiinflammatory medications (e.g., ibuprofen) – taking temperatures rectally –perform invasive procedures with extreme caution

58 Compare and Contrast Hemophilia A VWFITP Normal Platelets Elevated PT/PTT Normal Platelets Normal PT/PTT Very Low Platelets Normal PT/PTT

59 Oncology Cancer in adultsCancer in adults –abnormal cell is transformed by genetic mutation of its DNA – usually as a result from exposure to a tetragon Cancer in childrenCancer in children –usually arises from chromosomal abnormalities, genetic mutations and proliferation of embryonic cells

60 Oncology Treatments ChemotherapyChemotherapy –antineoplastic agents –attempt to destroy tumor cells by interfering with cellular functions and reproduction –cytotoxic drugs that are designed to cause cell death. Normal cells that have rapid growth are also affected, such as hair growth.Normal cells that have rapid growth are also affected, such as hair growth. Toxic side effectsToxic side effects

61 Oncology Treatments Surgical interventionSurgical intervention –removing the entire cancerous tumor (most ideal and frequently used treatment method) Radiation therapyRadiation therapy – interrupt cellular growth by breaking the DNA stands, leading to cell death.

62 Types of Cancer in Children Small percentage Carcinoma (opposed to large percentage in adults)Small percentage Carcinoma (opposed to 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

63 Clinical Manifestations Differ based on type of cancerDiffer based on type of cancer Many symptoms are similar to common childhood illnessesMany symptoms are similar to common childhood illnesses Symptoms may be in site other than the cancerSymptoms may be in site other than the cancer =delay in diagnosis=delay in diagnosis Often diagnosis made when cancer is advancedOften diagnosis made when cancer is advanced

64 Common Clinical Manifestations PainPain AnemiaAnemia Anorexia, weight lossAnorexia, weight loss InfectionsInfections BruisingBruising Neurological symptomsNeurological symptoms Palpable massPalpable mass

65 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 responsibilitiesTravel for treatment, heavy financial responsibilities Effects of siblingsEffects of siblings

66 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 illness, not cancerPreschoolers-beginning understanding of illness, not cancer School-age-understand cancer, benefit from talking about itSchool-age-understand cancer, benefit from talking about it Adolescents-mature understanding, benefits from other adolescents with cancerAdolescents-mature understanding, benefits from other adolescents with cancer

67 General Nursing Considerations Provide optimal nutrition- high metabolic rate of cancer depletes storesProvide optimal nutrition- high metabolic rate 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)

68 Risk for infection r/t Immunosuppressed state. Monitor vital signs q4hMonitor vital signs q4h Instruct parents how to measure temp at homeInstruct 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 hygieneTeach child and parents meticulous oral hygiene No live virus administrationNo live virus administration

69 Leukemia

70 Broad term describing a group of malignant diseasesBroad term describing a group of malignant diseases Normal Bone Marrow is replaced by abnormal immature cellsNormal Bone Marrow is replaced by abnormal immature cells Etiology: variety of agents thought to increase risk (virus, toxins, drugs) combined with geneticsEtiology: variety of agents thought to increase risk (virus, toxins, drugs) combined with genetics Two forms of leukemiaTwo forms of leukemia –ALL: Acute Lymphocytic Leukemia –AML: Acute Myelogenous Leukemia

71 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

72 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

73 Diagnosis: Based on: Signs & symptomsSigns & symptoms CBC changesCBC changes Bone marrow aspiration (> 25% of lymphoblast cells present)Bone marrow aspiration (> 25% of lymphoblast cells present)

74 Management Chemotherapy in 3 stages For 2-3 years 1.Induction 2.Sanctuary 3.Maintenance

75 Induction 1 st month; aim is to induce remission (blast cells to < 5%, normal Physical Findings)1 st month; aim is to induce remission (blast cells to < 5%, normal Physical Findings) Approximately 95% of children achieve remission within 1 monthApproximately 95% of children achieve remission within 1 month

76 Sanctuary or Consolidation Begins after remission, 4 weeks Goal: to maintain remissionto maintain remission prevent disease from invading sanctuary sitesprevent disease from invading sanctuary sites

77 Maintenance goal to maintain remissiongoal to maintain remission eliminate residual leukemic cellseliminate residual leukemic cells combination of drugs, outpatient basiscombination of drugs, outpatient basis girls treated for 2 years, boys for 3girls treated for 2 years, boys for 3 Cure: free of disease for 4-5 years

78 High Doses of Chemotherapy Can Lead to: Tumor Lysis SyndromeTumor Lysis Syndrome –Metabolic emergency results from the lysis (dissolving or decomposing) of tumor cells and rapid release of their contents into the bloodresults from the lysis (dissolving or decomposing) of tumor cells and rapid release of their contents into the blood

79 Tumor lysis syndrome Rapid cell destruction releases high levels ofRapid cell destruction releases high levels of – uric acid – potassium – phosphates Uric acid overloads the kidneysUric acid overloads the kidneys Leads to cardiac arrhythmias and renal failureLeads to cardiac arrhythmias and renal failure

80 Nursing Considerations Children receiving chemotherapy;Children receiving chemotherapy; Monitor for:Monitor for: –Hyperuricemia –Hyperkalemia –Hyperphosphatemia –Hypocalcemia

81 Nursing Considerations Administer vigorous hydration (2–4 times rate for maintenance fluid)Administer vigorous hydration (2–4 times rate for maintenance fluid) Administer allopurinol or urate oxidase (rasburicase) to reduce conversion of metabolic by-products to uric acidAdminister allopurinol or urate oxidase (rasburicase) to reduce conversion of metabolic by-products to uric acid

82 Soft Tissue Tumors Hodgkin's Non Hodgkin's Retinoblastoma

83 Lymphomas A malignancy that arises from the lymphoid systemA malignancy that arises from the lymphoid system Two types:Two types: –Hodgkins –Non Hodgkins

84 Hodgkin’s Disease Neoplasm of cervical lymphatic tissueNeoplasm of cervical lymphatic tissue Starts in a single or group of lymph nodes then spreads predictably to nonnodal sites such as: spleen, liver, bone, marrow, lungs, mediastinumStarts in a single or group of lymph nodes then spreads predictably to nonnodal sites such as: spleen, liver, bone, marrow, lungs, mediastinum Affects adolescents to late 20’sAffects adolescents to late 20’s Males > femalesMales > females Etiology unknown- infectious agent likelyEtiology unknown- infectious agent likely

85 Signs & Symptoms: Painless enlarged cervical nodePainless enlarged cervical node Unexplained weight loss, unexplained fevers, night sweatsUnexplained weight loss, unexplained fevers, night sweats

86 Diagnosis and Treatment Diagnosis Biopsy of enlarged lymph nodeBiopsy of enlarged lymph node Staged 1-4Staged 1-4Treatment ChemotherapyChemotherapy Radiation-low doses, higher is physiologically matureRadiation-low doses, higher is physiologically mature Good Prognosis-single originGood Prognosis-single origin

87 Non-Hodgkin’s Lymphoma No single originNo single origin Males > femalesMales > females Cause unknownCause unknown Aggressive proliferation of B or T lymphocytes in lymph nodesAggressive proliferation of B or T lymphocytes in lymph nodes Rapid in onset (ages 5-15)Rapid in onset (ages 5-15) Usually found with wide-spread involvement via bloodstream (multiple enlarged nodes)Usually found with wide-spread involvement via bloodstream (multiple enlarged nodes) Responds quickly to therapyResponds quickly to therapy

88 Signs & Symptoms: Acute abdominal and chest pain, constipation, crampingAcute abdominal and chest pain, constipation, cramping Anorexia, weight lossAnorexia, weight loss Painless enlarged lymph nodes found in cervical or axillary regionPainless enlarged lymph nodes found in cervical or axillary region Ascites and obstruction with vomiting a late signAscites and obstruction with vomiting a late sign Advanced disease: CNS symptoms, HA n/v, mediastinal mass, petichaie, bruising, bone painAdvanced disease: CNS symptoms, HA n/v, mediastinal mass, petichaie, bruising, bone pain

89 Diagnosis Biopsy from bone marrow or lymph nodeBiopsy from bone marrow or lymph node Staging 1-4Staging 1-4Treatment: Aggressive multi-agent chemo for 6 mos to 2 yearsAggressive multi-agent chemo for 6 mos to 2 years Risk for tumor lysis syndromeRisk for tumor lysis syndrome Intrathecal chemo and crainal radiationIntrathecal chemo and crainal radiation

90 Compare and Contrast Hodgkins Non Hodgkins Males>Females Late adolescent-20’s Single origin of cervical gland Good Prognosis Males > females ages 5-15 No single origin-wide- spread involvement Aggressive treatment

91 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

92 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 space, brain, 2 nd eyeMetastases to optic nerve, subarachnoid space, brain, 2 nd eye

93 Retinoblastoma Treatment: If small: cryosurgery, partial visionIf small: cryosurgery, partial vision If mets: chemo & radiationIf mets: chemo & radiation If large: enucleation, eye prosthesis 3 weeks post-opIf large: enucleation, eye prosthesis 3 weeks post-op Survival rate 90%Survival rate 90%

94 Solid Tumors NeuroblastomaNephroblastomaOsteoscaroma Ewing’s Sarcoma

95 Neuroblastoma Solid tumor of infants and pre-school children (peak 22mos)Solid tumor of infants and pre-school children (peak 22mos) Cancer cells arise from sympathetic nervous system called crest cellsCancer cells arise from sympathetic nervous system called crest cells –Embryologic cells of adrenal glands Etiology: unknown

96 Signs & Symptoms Depend on:Depend on: –extent of disease –location of tumor 65% present with protuberant, firm, irregular abdominal mass that crosses midline65% present with protuberant, firm, irregular abdominal mass that crosses midline

97 Neuroblastoma Other manifestations: impaired ROM & mobilityimpaired ROM & mobility pain & limpingpain & limping large abdominal masslarge abdominal mass respiratory symptoms if chest tumorrespiratory symptoms if chest tumor

98 Neuroblastoma Diagnosis: Chest x-rayChest x-ray CT scan of abdomen, pelvis, spineCT scan of abdomen, pelvis, spine Bone marrow aspiration Bone marrow aspirationManagement: depends on the presence and extent of metastasis depends on the presence and extent of metastasis

99 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 predisposition –Is associated with congenital anomalies

100 Nephroblastoma Parents usually notice a large, mobile abdominal mass while bathing or the diaper doesn’t fit anymoreParents usually notice a large, mobile abdominal mass while bathing or the diaper doesn’t fit anymore Grows extremely quickly, in a matter of daysGrows extremely quickly, in a matter of days DO NOT PALPATE ABDOMENDO NOT PALPATE ABDOMEN –can rupture the tumor and cause spreading of cancerous cells

101 Other Signs & Symptoms microscopic to gross hematuriamicroscopic to gross hematuria hypertensionhypertension abdominal painabdominal pain fatigue, anemia, feverfatigue, anemia, fever

102 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

103 Staging 1 through 5 1.tumor confined to the kidney and completely removed surgically 2.tumor extending beyond the kidney but completely removed surgically 3.regional spread of disease beyond the kidney with residual abdominal disease postoperatively 4.metastases to lung (primary site), liver, bone, distant lymph nodes 5.bilateral disease

104 Treatment State 1 and 2State 1 and 2 –Nephrectomy –Chemotherapy Stage 3-5Stage 3-5 –Nephrectomy –Radiation –Chemotherapy Survival rates are good (up to 90%)Survival rates are good (up to 90%)

105 Bone Cancers

106 Osteogenic Sarcoma (Osteosarcoma) Most common bone malignancy in children (teenage years)Most common bone malignancy in children (teenage years) Occurs in distal long bonesOccurs in distal long bones Attributed to extremity injury or growth spurtAttributed to extremity injury or growth 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

107 Signs & symptoms Progressive, insidious or intermittent pain at site of tumorProgressive, insidious or intermittent pain at site of tumor Palpable mass & swellingPalpable mass & swelling Limping, progressive limited range of motionLimping, progressive limited range of motion Pathological fracturesPathological fractures

108 Diagnosis X-ray, CT, MRIX-ray, CT, MRI Tumor biopsyTumor biopsy Look for chest metastasesLook for chest metastases

109 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 metastases

110 Nursing care ComfortComfort InfectionInfection Potential hemorrhagePotential hemorrhage Phantom limb painPhantom limb pain ProsthesisProsthesis Changes in body image and functioningChanges in body image and functioning

111 Ewing’s Sarcoma Highly malignant tumor in bone marrow of long bonesHighly malignant tumor in bone marrow of long 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 (lungs, bone, CNS, lymph nodes)Metastases is usually present at time of dx (lungs, bone, CNS, lymph nodes)

112 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 loss –Fever –Increased sed rate

113 Diagnosis: 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

114 Treatment: SurgerySurgery Multi agent chemoMulti agent chemo RadiationRadiation

115 Compare and Contrast Osteogenic scaroma Ewing’s Sarcoma Affects long bones Older adolescents Intermittent pain Palpable mass & swelling Limping, progressive limited range of motion Pathological fractures Metastases not as likely Surgery and chemo Affects any bone School-age and adolescents Intermittent pain becomes constant Swelling at tumor site Progresses into systemic symptoms Metastases likely Aggressive treatment

116 Chronically Ill Child Nursing Diagnosis FearFear Death AnxietyDeath Anxiety Anticipatory GrievingAnticipatory Grieving HopelessnessHopelessness

117 Goals for Care of the Chronically Ill Child Goals for the childGoals for the child –Achieve and maintain normalization –Obtain the highest level of health and function possible Goals for the familyGoals for the family –Remain intact –Achieve and maintain normalization –Maximize function throughout the illness

118 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 changing developmental needsRevise goals frequently to meet the child’s changing developmental needs Listen carefully to the child's perception of the conditionListen carefully to the child's perception of the condition

119 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 and deathRecognize the need to talk about illness and death Provide adequate pain control, oral care, privacy, and information about the signs of imminent deathProvide adequate pain control, oral care, privacy, and information about the signs of imminent death After death, allow family members as much time as they desire with the childAfter death, allow family members as much time as they desire with the child

120 Practice Questions!

121 A child is being admitted to the unit with thalassemia major (Cooley’s anemia). In preparing client assignments, the charge nurse wants to assign a nurse to this child who can: 1.Teach dietary sources of iron 2.Administer blood infusions 3.Work with a dying child 4.Monitor the child for bleeding tendencies

122 A 14-year-old boy with sickle cell anemia is admitted with severe pain in his abdomen and legs. He asks why the doctor ordered oxygen when he is not having any breathing problems. The nurse states the therapeutic action of O2 is: 1.Prevent further sickling 2.Prevent respiratory complications 3.Increase O2 capacity of RBCs 4.Decrease the potential for infection

123 A 10-year old in the ER has a CBC results that include a Hgb of 8, and Hct of 24. The nurse determines that based on the lab results which nursing action has a high priority? 1.Promotion of skin integrity 2.Promotion of hydration 3.Promotion of nutrition 4.Conserving energy

124 A 4-year-old is diagnosed with ALL. Following teaching about the staging and therapy, the nurse evaluates the family’s understanding of the problem. The statement by the family that indicates appropriate knowledge is “Staging will: 1.Determine the extent of the tumor process and need for palliation 2.Help determine if treatment is needed 3.Determine if surgery is needed 4.Determine the extent of malignant process and stage the leukemia

125 A 17-year old is being admitted for an amputation related to a bone tumor. The nurse is developing a nursing care plan and determines the most appropriate age related diagnosis is:A 17-year old is being admitted for an amputation related to a bone tumor. The nurse is developing a nursing care plan and determines the most appropriate age related diagnosis is: 1.Risk for disuse syndrome 2.Disturbed body image 3.Self-care deficit 4.Activity related intolerance

126 The nurse is reviewing the lab work on a child admitted for fatigue –WBC 7,200 –RBC3.01 –Hgb 9.1 –Hct 29.3 –Platelets 371,000 Iron 64 Ferritin 70 Transferrin 250 Bilirubin 18.2 PTT 45 seconds

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

128 The nurse is admitting a child for a swollen elbow. The history indicated multiple bruising. Which of the following laboratory results heightens the nurses suspicion for Hemophilia?The nurse is admitting a child for a swollen elbow. The history indicated multiple bruising. Which of the following laboratory results heightens the nurses suspicion for Hemophilia? 1. Hbg 12,0001. Hbg 12, WBC 9,0002. WBC 9, Platelets 356,0003. Platelets 356, PTT 73 seconds4. PTT 73 seconds


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