This document outlines learning outcomes and content related to alterations in hematologic function and childhood malignancies. It begins by describing the functions of red blood cells, white blood cells, and platelets. It then discusses differences in pediatric hematopoiesis compared to adults. Specific topics covered include anemia, sickle cell disease, hemophilia, and childhood cancers. Nursing care is discussed for conditions such as vaso-occlusive crises, bleeding episodes, and cancer diagnosis and treatment.

1. Joyce Buck, PhD(c), MSN, RN-C, CNE
Joy A. Shepard, PhD, RN-C, CNE
Alterations in Hematologic
Function/ Childhood Malignancies
1

2. Learning Outcomes
 Describe functions of red blood cells, white blood cells, and
platelets
 Distinguish pathophysiology and clinical manifestations for
chronic disorders of red blood cells
 Plan nursing care for a child with a chronic disorder of red blood
cells
 Distinguish pathophysiology and clinical manifestations for the
major bleeding disorders affecting the pediatric population
 Prioritize nursing interventions for a child with a major bleeding
disorder
 Summarize nursing implications for a child receiving
hematopoietic stem cell transplantation (HSCT)
2

3. Learning Outcomes Cont’d…
• Differentiate between characteristics of cancer in childhood and
adulthood
• Describe the incidence, etiologies, and common manifestations
of childhood cancer
• Synthesize information about diagnostic tests and clinical
therapies to plan comprehensive care
• Integrate information about oncologic emergencies to plan care
• Recognize common solid tumors in children, describe their
treatment, and plan comprehensive nursing care
• Plan care for children and adolescents with a diagnosis of
leukemia
• Describe the impact of cancer survival and its effect on
physiologic and psychosocial care
3

4. 4

5. Blood Cells
 Red Blood Cells (RBC)
 Carry O2 via hemoglobin (Hgb)
 Release CO2 in lungs–pick up O2
 White Blood Cells (WBC)
 Called leukocytes
 Responsible for immune responses
 Phagocytic, inflammatory, antigen-producing
 Platelets
 Clotting component
 Stored in spleen
5

6. Figure 23-1 Types of blood cells.
6

7. 7

8. 8

9. Pediatric Differences (RBC’s)
9
At birth hematopoiesis (blood cell production) occurs in
bone marrow
At birth the newborn has ↑ RBC’s due to high level of
erythropoietin (stimulates RBC production)
Once the newborn starts breathing air & O2 level in blood
increases, this production slows
RBC levels ↓ til 2-3 months of age (to 9-11 g/dL) and then
begin increasing
Adult levels are reached during adolescence (male teens
slightly higher than females)

10. Pediatric Differences (WBC’s)
10
 WBC count is highest at birth
 Levels decline after 12 hours and cont. to due so for
the 1st week
 By 1 week levels stabilize & remain so til ~ 1 yr.
 At 1 yr. WBC ct. slowly decreases til adult value is
reached in adolescence

11. Pediatric Differences (Plts)
11
 Platelet levels in newborns are lower than in older
children & adults
 Levels of many clotting factors are also lower in
infants
 Vitamin K is required for synthesis of clotting factors
II, VII, IX, & X

12. Anemia
12

13. Definition of Anemia
 Decreased number of red blood cells (RBC)
 Decreased quantity of hemoglobin (HgB)
 Decreased volume of packed red blood cells
(PRBC).
13

14. Causes of Anemia
 Increased loss or destruction of existing RBCs
 Impaired or decreased rate of RBC production
 Clinical manifestation of lead poisoning or
hypersplenism
14

15. Chronic Diseases Causing Anemia
 Sickle Cell Disease
 Thalassemia Major
 Cancer
 Aplastic anemia
 Folate deficiency
 Inflammatory bowel disease
 Infection
 Chronic renal disease
 Liver disease
15

16. Types of Anemia
 Impaired production (e.g., iron deficiency anemia) –
not going to cover
 Increased destruction: hemolytic anemia
Sickle cell anemia
16

17. Symptoms of Anemia
 Tachycardia
 Tachypnea
 Pallor
 Pale mucous membranes
 Poor appetite
 Pica
 Poor muscle tone
17

18. Symptoms of Anemia Cont’d
 Fatigue
 Exercise intolerance
 Irritability
 Hepatomegaly
 Splenomegaly
18

19. Prolonged Anemia
 Nailbed deformities
 Systolic heart murmur and/or heart failure
 Growth retardation
 Developmental delay
19

20. Tests for Anemia:
 Hgb 
 Hct 
 Mean corpuscular volume 
 RBC count 
 Reticulocyte count 
20

21. Sickle Cell Disease (SCD)
21

22. Sickle Cell Disease (SCD)
 SCD is a hereditary hemoglobinopathy disease involving
partial or complete replacement of normal Hgb with abnormal
Hgb S in red blood cells.
 It is an autosomal recessive disorder.
 Sickle cell trait affects 1 in 12 African Americans
 Sickle cell trait affects 1 in 16 Hispanics
 Also occurs in people of Mediterranean, South American,
Arabian, & East Indian descent
 Approximately 2 million Americans carry the sickle cell gene
 SCD usually diagnosed in infants. Otherwise, in toddlers or
preschool age children with the first crisis after an infection
22

23. Pathophysiology of Sickle Cell
Disease
23
 Hgb acquires an elongated crescent or
sickle shape
 The sickled cells are rigid and obstruct
capillary blood flow resulting in
engorgement and tissue ischemia
 The spleen is the first organ affected;
90% of children with HSD have
functional asplenia
 Many undergo splenectomy in early
childhood leading to severely
compromised immunity
 Bacterial infections are the leading
cause of death in young children
 Significant risk of stroke
http://www.nhlbi.nih.gov/health/health-topics/topics/sca/

24. 24
Pathophysiology Illustrated: Sickle
Cell Disease

25. 25
Pathophysiology Illustrated: Sickle
Cell Disease

26. Figure 23-5 The clinical manifestations of sickle cell anemia result from pathologic changes to
structures and systems throughout the body.
26

27. 27

28. 28

29. Acute Chest Syndrome
29
 2nd most common reason for hospitalization
 Life-threatening
 A new pulmonary infiltrate is present on CXR
 S/S: fever, chest pain, tachypnea, SOB, coughing,
wheezing, crackles

30. Sickle Cell Disease
 Sickling of RBCs can be triggered by any type stress
including fever, infection, hypoxia, emotional, or
physical
 Any condition resulting in increased blood viscosity
and hypoxia can trigger a crisis
 Sickle cells can resume a normal shape when
rehydrated or reoxygenated
 Sickle cells have a shortened life span of about 15
days in comparison to the normal 120 day lifespan of
an RBC
30

31. Box 23-2 Precipitating Factors Contributing to Sickle Cell Crisis
31

32. Clinical Manifestations of Sickle
Cell Disease
 Usually asymptomatic until 6 months when high levels of fetal Hgb
no longer inhibits sickling.
 Symptoms of anemia as a result of shortened lifespan of RBCs.
 Vaso-occlusive crisis:
 Severe pain and/ or swelling in areas affected
 Fever
 Joint pain
 Splenic sequestration crisis
 Blood is trapped or pooled in the spleen
 Mimics blood loss/ shock
 Ultimately can cause death from profound anemia and cardiovascular
collapse
32

33. Common Organs Affected in Sickle
Cell Disease
 Brain
 Cerebrovascular accident
 Eyes
 Retinopathy
 Retinal detachment
 Diminished vision
 Bones
 Chronic bone ischemia leading to infection and bone
degeneration
 Chest
 Acute Chest Syndrome
33

34. Common Organs Affected in Sickle
Cell Disease
Liver
 Hepatomegaly
 Scarring
 Cirrhosis
 Spleen
 Infarcts leading to fibrosis and increased risk of
infection
34

35. Common Organs Affected in Sickle
Cell Disease
 Kidneys
 Enuresis
 Hematuria
 Inability to concentrate urine
 Penis
 Microcirculatory obstruction possibly causing priapism
 Extremities
 Weakness
 Peripheral neuropathy
 Arthralgias
 Skin
 Ulcerations
35

36. Diagnosis of Sickle Cell Disease
 Newborn screening
 Cord blood testing using Hgb electrophoresis
 Hgb electrophoresis (verifies positive sickledex)
 Sickledex in child over 6 months of age
36

37. Clinical Management of Sickle Cell
Disease
 Pain control
 Hydration (aggressive)
 Oxygenation
 Prevention of infection
 Prevention of associated complications
 Transfusions
37

38. Growth & Development
38
 To encourage fluid intake in a small child:
 Use favorite cup or glass
 Use straws
 Take advantage of times child is thirsty
 Leave cup within easy reach
 Offer frozen juice pops and crushed ice drinks

39. Clinical Management of SCD
cont’d
39
 Frequent blood transfusions cause overload of Fe in
the body (hemosiderosis)
 Fe becomes stored in tissues & organs
 The body cannot excrete it
 Deferoxamine (Desferal) is a chelating agent given
by infusion
 Binds excess Fe and excretes it via the kidneys
 Deferasirox (Exjade) oral drug given once a day

40. Clinical Management of SCD
cont’d
40
 Annual transcranial Doppler testing
 Ages 2-16
 If abnormal: prophylactic transfusions
 Prevent of decrease risk of stroke

41. Clinical Management of SCD
cont’d
41
 Splenectomy- to prevent splenic sequestration
(trapping of RBC’s within spleen)
Folic Acid
 Helps make new RBC’s

42. Hydroxyurea
Improves fetal Hgb
levels
Increases total Hgb
concentration
Decreases sickling &
painful crises
Cytotoxic
Off-label use in
children
42

43. Experimental Treatment
43
 Rivipansel
 Study drug for prevention of vaso-occlusive crisis
(VOC)
 Decreases cell adhesion, activation, & inflammation
 May decrease pain
 IV

44. Hematopoietic Stem Cell
Transplantation (HSCT)
44
 Only known cure for SCD
 Limited to children with a human leukocyte antigen
(HLA) compatible sibling
 Survival rate 93-97%

45. Patient Education
45
 Regular health promotion visits/ immunizations UTD
 Scheduled appointment with hematologist
 Periodic special testing: heart & eye exams
 Follow instructions for antibiotic administration
 Assess child’s pain & give pain meds as prescribed
 Ensure extra fluids (hot weather, physical activity, travel)
 Inform school personnel
 Contact HCP for fever

46. Review Question
46
 A nurse is caring for a child with sickle cell anemia
who has a vaso-occlusive crisis. Which of the
following interventions should improve tissue
perfusion?
 A. Limiting oral fluids
 B. Administering oxygen
 C. Administering antibiotics
 D. Administering analgesics

47. Hemophilia
47

48. Hemophilia
 Hemophilia is a bleeding disorder inherited as a sex link (X-
linked) recessive trait. The disease occurs in males only but is
transmitted by symptom free female carriers.
 It results in a deficiency of one or more clotting factors.
Classic or Hemophilia A is a deficiency of clotting factor VIII
and is responsible for 85% cases of all cases of hemophilia.
 Hemophilia B (Christmas disease) is caused by a deficiency
of clotting factor IX and is responsible for 10-15% cases of all
cases of hemophilia.
48

49. 49

50. Symptoms of Hemophilia
 Symptoms are related to excessive bleeding as a
result of poor clotting.
 There are different levels of involvement related to
the level of deficiency of clotting factors
 Hemophilia can be mild to severe.
50

51. Symptoms of Hemophilia
 Newborn boy
 Abnormal amount of bleeding from the umbilical cord, circumcision, or
injections.
 Toddler boy
 Excessive bruising, intracranial bleeding, prolonged bleeding from cuts,
lacerations, injections.
 General symptoms
 Hemarthrosis
 Petechiae
 Epistaxis
 Frank areas of hemorrhage
 Anemia
51

52. Diagnosis of Hemophilia
 PTT (partial thromboplastin time) increased or
prolonged
 Analysis of clotting factors
 PT, TT, fibrinogen, & plt count all normal
52

53. 53

54. Treatment of Hemophilia
 Desmopressin (DDAVP) is effective in mild cases
 Administration of IV clotting factors (administer during bleeding episodes as
quickly as possible)
 RICE and immobilization of the affected part
 Pain management
 ROM once bleeding controlled
 Avoid rectal temps or suppositories
 Individualized School Health Plan
54

55. 55

56. Review Question
56
 Which of the following will be abnormal in a child
with a diagnosis of hemophilia?
 A. The platelet count
 B. The hemoglobin level
 C. The white blood cell count
 D. The partial thromboplastin time

57. Childhood Cancers
57

58. Incidence
58
 In the U.S. a diagnosis of cancer is made in ~
11,000 children under age 15 annually
 About 1500 die from cancer each year
 Cancer is the leading cause of disease-related
death in children under age 15
 Also second leading cause of death overall

59. Pediatric Cancers
 Neoplasm = “New Growth”
 Benign = no danger to life or health
 Malignant = can grow and spread
 Metastasis = spread of malignancy
 Nonepithelial or embryonal cell types predominate
 Fast-growing
 Less frequent than adult cancer
59

60. Pediatric Cancers (cont’d)
 Different types of cancers predominate at various
ages
 Immune system involvement – functions immaturely
in the young child
 Fast rate of cell growth in children – can lead to
proliferation of cancerous and normal cells
60

61. Figure 24-1 Percentage of primary cancers by site of origin for different age groups. Data from: Kadan-Lottick,
N. S. (2007). Epidemiology of childhood and adolescent cancer. In R. M. Kliegman, R. E. Behrman, H. B. Jensen,
& B.F. Stanton (Eds.), Nelson textbook of pediatrics (18th ed., p. 2098). Philadelphia: Saunders.
61

62. Cancer Causes
 External Stimuli
 Chemicals, radiation
 Cause of more adult cancers
 Immune or Gene Abnormalities
 Congenital or triggered by virus
 Chromosomal Abnormalities
 Congenital risk for specific cancers
 Eg. leukemia and Down syndrome link
62

63. Pediatric Cancer Signs
 Pain
 Cachexia
 Anemia
 Infections
 Bruising
 Neurologic Changes
 Palpable Mass (most commonly abdominal)
63

64. Cancer Diagnostic Tests
64
 CBC with differential
 Bone marrow aspiration
 Bone marrow biopsy
 Lumbar puncture
 Peripheral blood studies
 Radiographic examination
 Magnetic resonance
imaging
 Computed tomography
 Ultrasound
 Biopsy of tumor

65. CBC w/differential
65
 Examines cellular components of blood
 Normal: WBC < 10,000
 Platelets 150-400 K
 Hgb 12-16
 Diagnostic: WBC > 10,000 or depressed levels
 Platelets 20-100 K
 Hgb 7-10

66. ANC (Absolute Neutrophil Count)
66
 Examines blood component ratio: (% segmental
neutrophils + % bands (immature neutrophils) X
WBC count / 100
 Normal: ANC > 1000
 Diagnostic: ANC < 1000 = risk of infection

67. Bone Marrow Aspiration
67
 Examines bone marrow
 Normal: < 5% blast cells
 Diagnostic: > 25% blast cells (ALL)

68. Lumbar Puncture
68
 Examines CSF
 Normal Cell count:
polymorphonuclear leukocytes 0
Monocytes 0-5
RBC’s 0-5
 Diagnostic: presence of malignant cells indicates
CNS involvement

69. Cancer Treatment Goals
 Remove the cancer
 Surgery
 Inhibit growth of rapidly growing cells
 Chemotherapy and radiation
 Assist immune system to destroy cancer
 Biotherapy
 Replace cancerous bone marrow
 Hematopoietic stem cell transplant
69

70. Surgery
70
 Remove or debulk ( size) of a solid tumor
 Eg. Wilms tumor
 Also used to determine stage & type of cancer

71. Chemotherapy
71
 Administration of specific drugs that kill both normal
& cancerous cells
 Administration is timed to achieve greatest cellular
destruction
 Cell’s cycle of replication determines schedule
 Several chemo drugs administered simultaneously
to maximize lethal impact on cells

72. Chemotherapy (cont’d)
72
 Protocol: plan of action for chemo based on type of
cancer, stage & cell type
 Meds usually oral, IV, or intrathecal
 Colony-stimulating factors, antiemetics & nutritional
supplements

73. Biotherapy
73
 Treatment that uses &/or enhances the body’s ability
to fight disease with biologic agents that promote an
immune response
 Substances called biological response modifiers
(BRM’s)
 Egs. Interferon and colony-stimulating
factors(CSF’s)

74. Colony-Stimulating Factors
74
 Hormone-like glycoproteins that enhance blood cell
production & counteract the myelosuppressive
effects of chemo
 Eg. Erythropoitin produced in kidney; epoetin
(Epogen) (recombinant form) used to treat anemia
 Filgrastim (Neupogen)  production of neutrophils by
bone marrow
 Oprelvekin (Neumega)  platelet count

75. Radiation
75
 Unstable isotopes that release varying levels of
energy to cause breaks in DNA molecule & thereby
destroy cells
 Used for local & regional control & in combination
with surgery & chemo
 Curative or palliative
 Treatment field includes tumor site & sometimes
other areas (ie. lymph nodes)

76. Radiation (cont’d)
76
 Goal: irradiate tumor, not healthy tissue
 Total dose is divided (fractionated) over several
weeks
 Eg. Once daily 4-5 days/week X 2-7 weeks
 Egs of cancer treated w/radiation (Hodgkin disease,
Wilms tumor, retinoblastoma, rhabdomyosarcoma, &
CNS disease in leumkemia

77. Bone Marrow Transplant
77
 Autologous: uses the child’s own bone marrow
previously removed (stem cells)
 Stem cells can also be derived from the umbilical cord
 Allogeneic: compatible donor
 Hematopoietic stem cell transplant (donor stem cell)
transplant
 Only known cure for sickle cell anemia
 Only for kids with family member who is genotypically
identical (survival rate 93%)

78. Bone Marrow Transplant (cont’d)
78
 Used leukemia, neuroblastoma, & noncancerous
conditions (aplastic anemia)
 Goal: Administer lethal dose of chemo & radiation to
kill the cancer, then resupply the body w/stem cells
 Kills all circulating blood cells & bone marrow
contents

79. Childhood Cancers
 There are more than 250 different types of childhood
cancers
 The most common types are brain tumors and
leukemia
79

80. Cancer
 Cancer is a group of diseases in which there is out-of-
control growth and spread of abnormal cells (anaplasia).
 Anaplastic cells resist normal growth controls. This
abnormal cellular growth is also known as neoplasm and
is caused by one or a combination of three factors
 External stimuli or environment
 Viruses that alter the immune system
 Chromosomal and gene abnormalities
80

81. Tumor
 Benign
 Slow, limited, noninvasive growth (not cancerous)
 Malignant
 Progressive virulent growth (cancerous)
81

82. Differences Between Childhood
and Adult Cancers
 Childhood cancers
 Arise from primitive embryonic tissue (environmental
link)
 Cure rate is better
 Cancers affect stem cells
 More aggressive and faster growing
 Respond more readily to chemotherapy and radiation!
 Treated at major cancer centers in the United States
82

83. Differences Between Childhood
and Adult Cancers (cont’d)
83
Children are:
More resilient
Tolerate more aggressive therapy
Less other physiological problems

84. Common Symptoms of Cancer
 Unresolved fevers
 Discomfort, pain
 Fatigue
 Anemia
 Anorexia
 Weight loss
 Failure to thrive
 Pallor
 Lymphadenopathy
84

85. Blood Neoplasms
 Acute Lymphocytic Leukemia (ALL)
 Acute Myelogenous
Leukemia (AML)
85

86. Leukemia
 Leukemia is the most common form of cancer in
children.
 Invades the bone marrow, lymphatic systems and
can cause tumors in other parts of the body.
 Characterized by a proliferation of abnormal white
blood cells in the body.
 Rapidly produced WBCs are immature, often
referred to as blasts. They crowd out the good cells/
stem cells that produce RBCs and platelets.
86

87. Symptoms of Leukemia
87
 Fever
 Pallor
 Overt signs of bleeding
 Lethargy
 Malaise
 Anorexia
 Large joint or bone pain
 Petechiae, frank bleeding
& joint pain (bone marrow
failure)
 Hepatosplenomegaly
 Lymphadenopathy
 CNS infiltration

88. Treatment of Leukemia
 Chemotherapy
 Radiation
 Intrathecal chemotherapy
 Bone marrow and hematopoietic stem cell
transplantation (HSCT)
 Complication: Graft Versus Host Disease (GVHD)
88

89. Brain Tumors
Neuroblastoma
Nephroblastoma
Tumors of the bone
Solid Tumors
89

90. Brain Tumors
 Tumors of the brain and CNS are the most common
solid tumor found in childhood. It is the second most
common cancer after leukemia
 Brain tumors in children usually occur below the roof of
the cerebellum involving the cerebellum, midbrain, and
brainstem.
 In comparison, brain tumors in adults occur more
frequently above the area between the cerebrum and
cerebellum.
90

91. Four Types of Brain Cancer
 Astrocytomas: seizures, visual disturbances,  ICP,
vomiting; Tx: surgery, chemo & radiation
 Glioma (brain stem): cranial nerve VI & VII signs,
nystagmus, ataxia, motor symptoms; Tx: surgery &
radiation
 Ependymoma: hydrocephalus; Tx: surgery & radiation
 Medulloblastoma (medulla) external layer of
cerebellum: H/A, vomiting, ataxia; Tx: surgery, chemo
& radiation
91

92. Symptoms of Brain Cancer
92
 Visual changes
 Irritability
 Headaches
 Nausea & vomiting
 Abnormal Gait
 Weakness
 Educational or behavioral
changes
 Clumsiness
 Seizures
 Bulging fontanel and
widened cranial suture
line in infants
 Increasing cranial size
 Cranial nerve palsies
 FTT (brainstem tumors)

93. Diagnosis of Brain Cancer
93
 CT scan (computed
tomography)
 MRI (magnetic resonance
imaging)
 PET (positron emission
tomography)
 SPECT (single-photon
emission computed
tomography)
 Myelography
 Angiography
 MRA (magnetic resonance
angiography)
 MRS (magnetic resonance
spectroscopy)
 DSA (digital subtraction
angiography)
 CTA (CT angiography)
 Electroencephalography
 Brainstem evoked potentials
 Tumor markers
 CSF cytology

94. Treatment of Brain Cancer
 Surgical excision (common)
 Radiation (> age 3)
 Chemotherapy
 Intrathecal chemotherapy (via LP or scalp)
 Ommaya Reservoir
 Corticosteroids (prednisone, prednisolone or dexa
methasone)
94

95. Nursing Care of the Child with
Brain Cancer
 HOB up as ordered
 Minimal sensory stimulation including quiet, dimly lit
environment
 Neurological checks
 Pain assessment
 Assessment and prevention of infection in surgical sites
95

96. Organ Neoplasms
96

97. Neuroblastoma
 The most common solid tumor outside of the
cranium in children. Most common tumor in infants
prior to one year of age.
 Occurs anywhere along the sympathetic nervous
system chain
 Frequently occurs in the abdomen
 50% develop in the adrenal medulla
 30% develop in the cervical, thoracic, or pelvic areas
97

98. Signs & Symptoms of
Neuroblastoma
 Based on location of the mass
 Retroperitoneal mass
 Altered bowel and bladder function
 Weight loss
 Abdominal distention
 Enlarged liver
 Irritability
 Fatigue
 Fever
98

99. Signs & Symptoms of
Neuroblastoma
99
 Mediastinal
 Dyspnea
 Infection
 Neck & facial edema (superior vena cava syndrome)
 Intracranial
 Periorbital ecchymosis
 Metastasis to bone
 Malaise
 Fever
 Limp

100. Signs & Symptoms of
Neuroblastoma
100
 Bone marrow disease
 Pancytopenia: abnormal depression of all cellular
blood components
 Neutropenia
 Anemia

101. Diagnosis of Neuroblastoma
 Biopsy of tumor (initial diagnosis)
 Metastases
 Bone marrow biopsy
 Radio-labeled scanning
 MRI
 CT scan
 XR
101

102. Diagnosis of Neuroblastoma
102
 Tumor markers:
 Vanillylmandelic acid (VMA)*
 Homovanillic acid (HVA)*
 Dopamine
 Ferritin
 NSE (enzyme in neural tissue)
 LDH (lactic dehydrogenase
 Ganglioside GD2 (sugar & lipid molecule on surface
of neural cells)

103. Pheochromocytoma
103
 Tumor that arises from adrenal gland
 Usually benign & curable
 Familial (autosomal dominant)
 Males>females
 Ages 6-14
 Tumor causes excessive release of catecholamines
  BP, palpitations, sweating, anxiety, tremors, & H/A
 Diagnosis: VMA & HVA

104. VMA & HVA
104
 By-products of adrenal hormones
 Urine & blood levels 
 Urine test*
 Dietary restrictions prior to test:
 Avoid excess physical exercise & stress
 Discard 1st void & then start timing for 24 hours
 Avoid coffee, tea, chocolate, cocoa, licorice, bananas,
citrus fruits, foods & fluids containing vanilla, & ASA 2-
3 days prior

105. Nephroblastoma
 Intrarenal tumor
 Wilms' Tumor: most common type
 Most frequently ages 2-3
 Bilateral or unilateral
 Wilms' Tumor is often associated with severe
congenital anomalies suggesting a genetic link.
 Most children with Wilms' Tumor do not have
associated anomalies
105

106. Nephroblastoma
106
 Usually asymptomatic
 Fast-growing (doubles in size every 11-13 days)
 90% survival rate

107. Signs & Symptoms of
Nephroblastoma
 Asymptomatic, firm mass located to 1 side of the
midline in the abdomen
 Hypertension (25% of the cases)
 Hematuria*
 Abdominal pain*
107

108. Nephroblastoma
108
DO NOT PALPATE ABDOMEN!!!!!

109. Diagnosis of Nephroblastoma
 US (diagnostic)
 IVP (diagnostic)
 CT scan or MRI (identify metastasis)
109

110. Treatment of Nephroblastoma
 Surgery (removal of tumor & kidney)
 Radiation
 Chemotherapy
110

111. Bone Cancer

112. Osteosarcoma (OS)
 Most common bone malignancy
in children
 Very aggressive tumor
 Originates in osteoblasts
 Dx: biopsy
 Seen in adolescent boys
 50% cases—femur
 Pain
 Pathologic (“stress”) fractures
 Tx: Amputation, chemo

113. OS—Nursing Care
 Amputation care postoperatively
 Do not elevate the stump; keep it straight
 Control bleeding
 Pt will have phantom-limb pain
 Chemotherapy (before and after)
 N/V
 Loss of hair

114. Ewing Sarcoma
 Second most common bone cancer in children, rare
 Can affect the legs, pelvis, arms, ribs, skull or spine
 Ages 10-20, affects slightly more boys than girls, rarely
found in African Americans or Asian Americans
 Extremely aggressive
 Dx: biopsy
 70% overall cure rate
 Teens ages 15-19 yrs: survival rate 56%
 If metastasis has already occurred: < 30% survival
rate (spreads to lungs, bones and bone marrow)
 Pain and swelling, palpable lump, fever, pathologic
fracture
 Tx: chemo, surgery, radiation

115. General Cancer Care
115

116. Treatment of Cancer
 Chemotherapy
 Radiation
 Intrathecal chemotherapy (leukemia, CNS cancers)
 Corticosteroids
 Biotherapy
 Bone marrow and hematopoietic stem cell
transplantation (leukemia, neuroblastoma, aplastic
anemia)
 Complementary therapies
 Palliative care
116

117. Common Side Effects of
Chemotherapy and Radiation
 Immunosuppression
 Infection
 Anemia, thrombocytopenia, neutropenia
 Nausea, vomiting, diarrhea, weight loss
 Mucositis, stomatitis, and ulceration
 Alopecia
 Chemotherapy drug-specific side effects include
pulmonary fibrosis, hemorrhagic cystitis, and renal
disease
117

118. Complementary Therapies
118
 Nutritional supplements
 Herbal supplements
 Touch therapy
 Mind/body interventions

119. Management of Symptoms of the
Cancer and Treatment
 Pain management
 Antiemetic therapy (ondansetron-Zofran)
 Colony stimulating factors
 Epoetin/ Epogen: RBC production
 Filgrastin/ Neupogen/ Neulast: granulocyte production
 Oprelvekin/ Neumega: platelet production
119

120. Support for Cancer Treatment
120
 Provide extra rest periods to offset fatigue due to
chemo & radiation
 Treat appropriately according to age
 Maintain contact w/ child’s peer group & family
members via phone, video, etc. during periods of
immune suppression
 Respite care for families

121. Oncologic Emergencies
 Tumor lysis syndrome
 Septic shock
 Bone marrow suppression
 GI & CNS bleeding
 DIC
 Brain herniation
 Spinal cord compression
 Superior vena cava syndrome
121

122. Tumor Lysis Syndrome
122
 Breakdown of malignant cells releases intracellular
components into blood
 Results in hyperuricemia, hyperkalemia,
hyperphosphatemia, & hypocalcemia
 Treatment: IVF’s, I & O, daily weights, urine SG,
correct electrolytes

123. Tumor Lysis Syndrome (cont’d)
123
 Electrolyte imbalance causes metabolic acidosis &
serious abnormalities
 Results in cardiac arrhythmias, impaired renal fx,
tetany, neuro and mental status changes
 Treatment: ECG monitoring, Lasix for K+ excretion,
electrolyte administration

124. Superior Vena Cava Syndrome
124
 Compression of the SVC (returns blood from upper
body back to the right atrium)
 S & S: SOB, swelling of arms & face

125. Nursing Care of the Child with
Cancer
 Prevention of infection during periods of immunosuppression
 Implementation of bleeding precautions
 Interventions to prevent wasting syndrome/ cachexia
 Education on care of central lines, transfusion therapy, and
symptom management
 Support and educate the family
 Side effects of medications and treatments
 Infection prevention
 Orchestrate an interdisciplinary approach
 Provide distraction through play therapy
125

126. Neutropenic Precautions
126
 Neutropenia-absolute neutrophil count (ANC) <
500 cells/mm3
 Or when ANC count is 500-1000 when
chemotherapy is given & ↓ levels are anticipated

127. Neutropenic Precautions
127
 No raw fruits & vegetables
 No fresh flowers or live plants
 No pets
 No contact with infections persons
 Strict handwashing

128. Nursing Care of the Child with
Cancer
 Monitor for s/s of infection
 Alleviate pain
 Oral hygiene
 Nutrition
 Provide education on disease, diagnostic tests, &
treatments, & prognosis
 Assess and promote development & growth
 Assess the child and family’s coping and support
systems
128

129. Review Question
129
 A nurse is caring for a 10-year-old with leukemia who is
receiving chemotherapy. The child is on neutropenic
precautions. Friends of the child come to the desk and ask for
a vase for flowers they have picked from their garden. Which
of the following is the best response?
 A. “I will get you a special vase that we use on the unit.”
 B. “The flowers from your garden are beautiful but should not
be placed in the room at this time.”
 C. “As soon as I can wash a vase, I will put the flowers in it
and bring it to the room.”
 D. “Get rid of the flowers immediately. You could harm the
child.”

130. Thrombocytopenia
130
 Assess for signs of bleeding
 Minimize needle sticks
 Soft toothbrush or foam oral cleanser
 No razors

131. Anemia
131
 Encourage iron-rich foods
 Nutritional supplements
 Blood transfusions

132. Emotional Care
132
 Prepare for hair loss w/plans (hats, wigs, etc.)
 Continue contact w/ friends via phone, internet, in
person when possible
 Relaxation techniques for sleep & management of
treatments
 Talk w/clergy, teachers, parents, counselors, friends,
or other supportive people
 Journaling to record feelings & experiences

133. End of Life Care
133
 Undertreatment of symptoms (pain, dyspnea,
nutrition, elimination, & fatigue)
 Fragmentation of care
 End-of-Life of palliative care team
 Advanced care directive

134. Body Image
134
 Hair loss, surgical scars, amputation, cushingoid
symptoms
 Provide emotional support
 Refer to other children with similar concerns

135. Ways to  Cancer in Children
135
  intake of fruits & veggies (5 servings daily min)
 Protect skin w/ sunscreen
 Avoid tanning beds
 Discourage smoking
 Avoid tobacco smoke
 Early screening if family Hx of cancers
 Pap test, breast self-exam, testicular exam, HPV
vaccine