Module 13.3 Children Who Have Cancer &/OR Are Dying

Module 13.3 Peggy Kearney MSN, RN

Children Who Have Cancer and/or Are Dying IntroductionNeoplastic Disorders are leading cause of death from disease in children past infancy

Almost of all childhood cancers involve blood or blood-forming organs

Choice of therapy is based on:Type Stage Extent of spread of the malignancy

Prognosis childhood cancer improved -- >70-80% children treated in major cancer centers survive longer than 5 years (average)

Goal of treatment is curative, palliative or supportive

2Page 888Etiology Cause remains unknown General theory is genetic changes Some show pattern of inheritance Children with Immune Disorders -- HIV/AIDSDowns & Klinefelter syndrome greater risk for developing certain cancers Environmental

3Classification of Leukemia's Two forms are generally recognized Acute lymphoblastic leukemia (ALL)Stem or blast cell Acute nonlymphoid (myelogenous) leukemia (ANLL or AML)Complex disease with varying heterogeneity Classifications are increasingly complex

PathophysiologyLeukemia is an unrestricted proliferation of immature WBCs in the blood-forming tissues of the bodyProliferating cells depress the production of all formed elements of the blood in the bone marrowBroad term given to a group of malignant diseases of the bone marrow & lymphatic systemLiver, spleen and lymph glands are the most severely affected organs CNS involvement also which may cause IICP Not a solid tumoracts like one

Leukemia is an overproduction of WBCsbut the cells are immature so consequently in the acute stages numbers are low.

These Immature cells crowd out cells in vascular tissuecompete for metabolic elementsbone marrow crowding causes invasion of the periosteum and gradual weakening of bone and possible fractures

5Clinical ManifestationsLeukemia occurs when stem cells in bone marrow produce immature WBCs that dont function normally

These cells proliferate & cause bone marrow to fill with abnormal WBCs

RBCs, WBCs, platelets, plasma, stem cells decreased 6WBC will be low-----non functioning WBCs----abnormal cells compete for metabolic elements and causes cellular destruction (cacexcia)

Watch for anemia, infection and bleedingSigns &Symptoms LeukemiaAcute lymphoblastic & Acute nonlymphoblastic

Fever PallorOvert signs of bleeding Lethargy and MalaiseAnorexia and/or weight loss Large joint or bone pain Petechiae, frank bleeding, joint pain are cardinal signs of bone marrow failure

Consequences of Leukemia3 Main: 1. 2. 3.

Spleen, liver & lymph glands become infiltrated & enlarged eventually become fibrotic CNS becomes infiltrated IICP

8Anemia from decreased RBCsInfection from neutropeniaBleeding from decreased platelet production

Can infiltrate other organs also.

Incidence of Leukemia 3-4 cases/100,000More common in white malesPeak age for children is between 2 & 5 years of age Most common childrens type of leukemia -- majority of patients are under 10 years old Prognosis: Page 888 (criteria at diagnosis)2-10 year oldsFemale do better than males5 year survival rates are close to >80% for ALL 50-65% for AML

9Diagnostic EvaluationBased on history, physical manifestations

Peripheral blood smear (done with CBC)-- Immature leukocytes -- Frequently low blood counts

Bone marrow aspiration or biopsy Definitive diagnosis

LP to evaluate CNS involvementTherapeutic Management Chemotherapy With or without cranial irradiationIntrathecal meds for CNS involvement 4 Phases InductionCNS prophylactic therapyPrevents invasion into CNSIntensification (consolidation)Eradicates residual leukemia cellsMaintenance

Individualized Road map11Hematopoietic Stem Cell Transplantation (HSCT)Donors may be relatives or non-relatives

Stem cells from umbilical cord blood

This is tx option for child who has a relapse with acute lymphoblastic leukemia, achieves 2nd remission; transplant given when child is in remission 12If issue with transplant---think of issues the sibling donor may haveHematopoietic Stem Cell Transplantation (Reasons for use)Used to establish healthy cells in both malignant & nonmalignant disease

Ablative therapyHigh-dose combination chemo (with or without radiation) to eradicate unhealthy cells & suppress immune system to prevent rejection of transplanted marrow13Nursing Care ManagementPrepare child and family Multiple painful tests/proceduresExplanations /Therapeutic PlayPharmacologic Measures Sedation Non-pharmacologic MeasuresRelieve Pain Prevention of Complications of MyleosuppressionInfectionAdequate nutritionHemorrhage Anemia Manage Problems of Drug Toxicity N & VAnorexiaMucosal Ulceration NeuropathyHemorrhagic Cystitis 14Blood transfusions may be necessary---cmv negative, leukopoor, washed, irradiated

handle chemo with care

N & V---antiemetic, steroids ---given before chemo goal to prevent the child from ever experience prevents development of anticipatory sxsAnorexiamay need TPNMucosal ulcers---bland diet, soft tooth brushes rinses---avoid viscous lido (page 892)Cystitis---fluidsNURSING Diagnosis Activity intolerance Risk for infection Fluid excess Strict I & O Impaired tissue integrity/injury risk/immobility Altered nutrition allow food child likes/toleratesSkin care & mucous membranePain/Anxiety/Fatiguelessen painful proceduresFoster growth & development Body image Family and child with coping 15

Wilms Tumor NephroblastomaMost common malignant renal & intraabdominal tumor of childhood 8 cases/million 500 new cases/yearPeak age at diagnosis is age 3 years1-2.5% have a familial origin Usually a single tumor from renal parenchyma

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No method for identifying gene carriers at this time

Only 6% will have a tumor on both kidneysClinical Manifestations Abdominal Swelling or MassFirmNontenderConfined to one sideSee page 917 Box 27-5 Most common site for metastasis is lungs then liver, kidney, bone (rare)

Tumor can compress up into the diaphragm---causing respiratory issues. 17 Diagnostic Evaluation H & PFamily history Signs of malignancyWeight lossHepato/spleenomegally Abdominal swellingTight diaper Abdominal USCT (chest & abdomen)Hematologic studiesUA

18Bone marrow Dye studies for renal function MEDICAL MANAGEMENT Surgery Kidney removed Provides tissue for dx, histology & stagingExplore lymph nodes & abdominal organsStaging is exact determination of extent of disease at the time of dx

Radiation therapy &/or chemotherapy

19NURSING ALERT!!Post a Sign:

DO NOT PALPATE ABDOMENCareful bathing and handling to prevent trauma and possible rupture to tumor site20SIDS Sudden Infant Death SyndromeSIDS Sudden of death of an infant younger than 1 year of age Unexpected death of any infant where postmortem examination fails to determine cause of death.

Decreased by 53% due to Back to Sleep campaign

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Unexpected/unexplained after ME and crime scene investigation and review of case history Infants at Risk for SIDS Birth Premature neonates VLBWMultiplesLow Apgar scores Male sexRecent viral illnessSiblings of infants who have died of SIDS Infants of American Indian or African-American ethnicity 22No diagnostic test exists to predict which infants will surviveEtiology & PathophysiologyNumerous theories Generally unknown? Brain stem abnormality Genetic predisposition Risk FactorsMaternal Smoking During pregnancy & in infants environmentCosleeping Prone sleepingSoft bedding

23Protective FactorsBreast FeedingPacifier UseSupine Position Back to Sleep www.michigan.gov/safesleep

Autopsies reveal consistent findings pulmonary edema 24Nurse Care ManagementPreventativeEducationRole model while in hospital Not side lyingFollow up at home Written materialsResults of autopsy Home visits Cards, phone calls 25Quit smoking---no smoking environmentBack to sleep--no cosleeping, no soft bedding

hAnd prints/foot printsKeepsake Memories

If the parents need time with the child/baby==give it to them

Some institutions will put a heating pad in the crib or wrapped around the baby to keep the infant warm26Children's Understanding of & Reaction to DeathPage 558Table 18-4Perspective on the Care of Children at the End of Life starts on page 55527