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
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
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
16. Types of Anemia
Impaired production (e.g., iron deficiency anemia) –
not going to cover
Increased destruction: hemolytic anemia
Sickle cell anemia
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17. Symptoms of Anemia
Tachycardia
Tachypnea
Pallor
Pale mucous membranes
Poor appetite
Pica
Poor muscle tone
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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
effected, 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. Figure 23-4 The etiology, pathophysiology, and disease process of sickle cell anemia.
24
25. Figure 23-5 The clinical manifestations of sickle cell anemia result from pathologic changes to
structures and systems throughout the body.
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28. Acute Chest Syndrome
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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
29. 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.
29
31. 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
31
32. 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
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33. Common Organs Affected in Sickle
Cell Disease
Liver
Hepatomegaly
Scarring
Cirrhosis
Spleen
Infarcts leading to fibrosis and increased risk of
infection
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35. Diagnosis of Sickle Cell Disease
Cord blood testing using HgB electrophoresis
Sickledex in child over 6 months of age
Hgb electrophoresis (verifies positive sickledex)
35
36. Clinical Management of Sickle Cell
Disease
Pain control
Hydration
Oxygenation
Prevention of infection
Prevention of associated complications
Transfusions
Iron chelating drugs as needed for increased iron
levels (hemosiderosis)as a result of frequent
transfusions
36
37. Clinical Management of SCD
cont’d
37
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
38. Clinical Management of SCD
cont’d
38
Splenectomy- to prevent splenic sequestration
(trapping of RBC’s within spleen)
Hydroxyurea (cytotoxic)
Improves fetal Hgb levels
Decreases sickling & painful crises
40. Review Question
40
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
42. 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.
42
43. 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.
43
44. 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
44
45. Diagnosis of Hemophilia
PTT (partial thromboplastin time)
Analysis of clotting factors
PT, TT, fibrinogen, & plt count all normal
45
47. Treatment of Hemophilia
Desmopressin (DDAVP) is effective in mild cases
Administration of IV clotting factors needed
RICE and immobilization of the affected part
Pain management
47
49. Review Question
49
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
51. Incidence
51
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
52. 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
52
53. 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
53
54. 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.
54
55. 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
For example, leukemia and Down syndrome link
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59. 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
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60. Biotherapy
60
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)
61. Bone Marrow Transplant
61
Autologous: uses the child’s own bone marrow
previously removed (stem cellsl)
Stem cells can also be derived from the umbilical cord
Allogeneic: compatible donar
Hematopoietic stem cell transplant (donar stem cell)
transplant
Only known cure for sickle cell anemia
Only for kids with family member who is genotypically
identical (survival rate 93%)
62. Childhood Cancers
There are more than 250 different types of childhood
cancers
The most common types are brain tumors and
leukemia
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63. Cancer
Cancer is a group of diseases in which there is out-of-
control growth and spread of abnormal cells (anaplasia).
Anaplasic 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
63
65. 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
More resilient
Tolerate more aggressive therapy
Less other physiological problems
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66. Common Symptoms of Cancer
Unresolved fevers
Discomfort, pain
Fatigue
Anemia
Anorexia
Weight loss
Failure to thrive
Pallor
Lymphadenopathy
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68. 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.
68
69. Symptoms of Leukemia
Limp in gait
Anemia
Thrombocytopenia
Petechiae
Bruising
Hepatomegaly
Splenomegaly
Bone pain
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70. Treatment of Leukemia
Chemotherapy
Radiation
Intrathecal chemotherapy
Bone marrow and hematopoietic stem cell
transplantation (HSCT)
Complication: Graft Versus Host Disease (GVHD)
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72. 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.
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73. Four Types of Brain Cancer
Dependent upon site of origination:
Astrocytoma: arise from glial cells & can either be above
or below the area between the cerebrum & cerebellum
(35-40% of childhood brain tumors)
Glioma (brain stem) located in the pons & typically
spreads (10-15%)
Ependymoma (the thin epithelial membrane lining the
ventricular system of the brain and the spinal cord
responsible for production of cerebrospinal fluid)(10-15%)
Medulloblastoma (medulla) external layer of cerebellum
(35-40%) commonly ages 5-6
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74. Symptoms of Brain Cancer
Visual changes
Irritability
Headaches
Emesis
Gait changes
Weakness
Clumsiness
Seizures
Bulging fontanel and widened cranial suture line in infants
Increasing cranial size
Cranial nerve palsies
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75. Diagnosis of Brain Cancer
CT scan
MRI
PET
SPECT
Myelography
Angiography
75
77. 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
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79. 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
79
80. 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
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81. Diagnosis of Neuroblastoma
MRI
CT scan
XR
Elevated vanillylmandelic acid (VMA) and
homovanillic acid (HVA) in the blood and urine
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82. Nephroblastoma
A nephroblastoma is an intrarenal tumor of which
Wilms' Tumor is the most common type. Wilms'
Tumor is often associated with severe congenital
anomalies suggesting a genetic link.
Though most children with Wilms' Tumor do not
have associated anomalies.
It is an asymptomatic, fast growing tumor.
82
83. Signs & Symptoms of
Nephroblastoma
Hypertension in 25% of the cases
Hematuria
Abdominal pain
Usually presents as an abdominal swelling or mass
that crosses the midline
Weight loss, anemia, & fatigue
83
89. 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
89
90. 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
90
91. Management of Symptoms of the
Cancer and Treatment
Pain management
Antiemetic therapy
Colony stimulating factors
Epoetin/ Epogen: RBC production
Filgrastin/ Neupogen/ Neulast: granulocyte production
Oprelvekin/ Neumega: platelet production
91
93. Tumor Lysis Syndrome
93
Lysis or decomposing of tumor cells & rapid release
of their contents into the blood
Releases increased amounts of uric acid, K+, &
phosphates in the blood
Can decrease serum Na levels & may result in
cardiac arrythmias & renal failure (can occur with
ALL)
Treatment: IVF’s, I & O, daily weights, urine SG,
correct electrolytes
94. Superior Vena Cava Syndrome
94
Compression of the SVC (returns blood from upper
body back to the right atrium)
S & S: SOB, swelling of arms & face
95. 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
95
96. Nursing Care of the Child with
Cancer
Monitor for s/s of infection
Alleviate pain
Oral hygiene
Provide education on disease, diagnostic tests, &
treatments, & prognosis
Assess and promote development & growth
Assess the child and family’s coping and support
systems
96
98. Neutropenic Precautions
98
No raw fruits & vegetables
No fresh flowers or live plants
No pets
No contact with infections persons
Strict handwashing
99. Review Question
99
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.”
102. Body Image
102
Hair loss, surgical scars, amputation, cushingoid
symptoms
Provide emotional support
Refer to other children with similar concerns
