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
By Jeanie Ward
Iron Deficiency Anemia

Reduction in the number of RBC’s
caused by:
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Decreased iron intake
Blood loss
Increased internal demands during growth
periods
Iron Deficiency Anemia
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Manifestations:
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Pallor; Pale mucus membranes
Enlarged spleen and heart
Poor muscle tone with decreased activity
Fatigue
Diagnosis
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low hemoglobin (<11g/100mL)
Low hematocrit (<33%)
RBC are small and hypochromic
Serum iron levels are low ( 30μg/mL)
Iron Deficiency Anemia

Treatment

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Oral iron supplements
Ascorbic acid (vitamin C)
Diet high in iron
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Iron-fortified formula or supplements
Limit cow’s milk to 24oz/day for children >12
months
Increase age-appropriate iron-rich foods
Definition

A group of genetic disorders
characterized by production of
elongated, crescent shaped
erythrocytes, or abnormal form of
hemoglobin, designated as sickle
hemoglobin (HbS) in the place of the
normal hemoglobin.
Etiology and Pathophysiology

Autosomal recessive inherited disease

Occurs almost exclusively among African Americans

Defect of beta chain of hemoglobin

Disease usually not apparent until 6 months of age –
fetal hemoglobin contains a gamma, not a beta chain.
Hemoglobin changes from fetal form to adult of HgbA
at that time.
Normal vs Sickle Hemoglobin

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Disc-shaped
Soft (like bag of
jelly)
Easily flows through
small blood vessels
Lives for 120 days

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Sickle-shaped
Hard (like piece of
wood)
Often get stuck in
small blood
vessels
Lives for 20 days
or less
Genetics
Both Parents Have Sickle Cell Trait
25% will be normal
25% will have
Sickle cell Disease
50% will have the Trait
What causes the cell to sickle?

Low Oxygen tension (less than 60%-70%)

Low blood pH (acidosis)

Increased blood viscosity (dehydration,
fever, hypoxia)
Result of Sickling Process
The sickled RBC’s do not move freely
through the microcirculation.
Blockage in vessel / blood flow halts
Tissue distal to the blockage becomes
ischemic
Acute pain, cell destruction,
tissue death
Assessment
General Manifestations
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Chronic anemia (Hgb. 6-9 g/dl.), pallor,
weakness. Unable to do physical activities
because of lack of stamina
Fatigue, malaise
Anorexia
Jaundice
Possible delayed sexual maturation
Marked susceptibility to sepsis
Possible growth retardation – with long bones
disproportionately long.
Types of Sickle Cell Crisis
VasoOcclusive
Aplastic
Crisis
Crisis
Acute
Sequestration
Crisis
Vaso-Occlusive Crisis

Occurs from the pooling of many of the
new sickled cells in vessels with resulting
tissue hypoxia

May last from one
day to several weeks
Manifestations of
Vaso-Occlusive Crisis

Bone Pain
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Most outstanding symptom
Mainly in the bones of the extremities and
joints, but can occur anywhere
Abdominal and back pain is common
Related to necrosis of bone tissue
Parents may notice this with refusal to move
an extremity, crying out with joint movement
or joint touched.
Hand and foot Syndrome
Dactylitis
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Painful swelling of
the hands and /or
feet.
May be the first
symptom of crisis
Warmth in affected
areas.
Fever
Cerebrovascular accident
Vaso-occlusion in the brain
results in cerebral infarction
which causes variable degrees
of neurologic damage
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Hemiplegia
Aphasia
Seizures
Vision changes
Headaches
Alterations in level of consciousness
Acute Chest Syndrome
Blockage of blood vessels
in the chest leads to
Pneumonia and
acute chest syndrome
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Chest pains
Fever, cough
Dyspnea, retractions
Leading cause of death in SCD.
Priapism
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Persistent painful erection
of the penis occurring when
penile blood flow is obstructed.
Hepatomegaly
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Enlarged liver with jaundice and hepatic
coma.
Hematuria
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related to damage to the kidneys
Aplastic Crisis
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Diminished RBC production resulting in
severe anemia
Manifested by:
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Malaise, lethargy
Headache
Pallor
fainting
Aplastic Crisis
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Profound anemia- low RBC count
is lifelong. The average RBC life
is down to an average of 10-20
days in SCA.
Jaundice, elevated bilirubin
Reticulocytosis
Bone marrow producing 3-4
times more RBC’s than normal
Acute Sequestration Crisis
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Sickled cells become trapped
in the spleen
obstructing
blood flow with pooling and
enlargement of the spleen.
Leads to shock and
hypovolemia
Circulatory collapse and
death can occur in less than
30 minutes
Spleen Function


Acts as a filter against foreign
organisms that infect the
bloodstream.
Filters out old RBC’s from the
bloodstream and recycles them
Minor Role:
Manufacturers RBC’s toward end of fetal life
and after birth, taken over by bone marrow.
Acts as a blood reservoir.

Spleen in the Infant
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Soft, purplish-red organ
that lies under the
diaphragm on the left
side of the abdominal
cavity
Filters old blood and
clears bacteria
Teach Parents How to Measure
Spleen


A tongue depressor
can be used to
measure and track
spleen size
Place tip on left
nipple and make
mark where the
spleen tip is felt
Treatment for Sequestration Crisis

Intravenous fluids
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Blood transfusion
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Spleen removal
Effects of Chronic Crisis States
on the Child’s Body
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Spleen – more susceptible to infection. Gradual
fibrosis and scarring with reduction in spleen
activity. Asplenia.
Liver – enlarged, firm, tender
Brain – single episode of sickling
CVA, seizures
Heart – enlarges and murmurs develop
Lungs – pulmonary edema and stasis
Kidneys – hematuria, unable to concentrate urine
Bone Infarcts – hands and feet swell.
Effects of Chronic Crisis States on
the Child’s Body
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Eyes – bleeds in retina.
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Leg Ulcers
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Are seen in 10-15% of older
children
May start as simple insect bite
or cut that does not heal
With poor circulation develops
into leg ulcer
Treated with dressings, leg
elevation and elastic stockings.
Diagnostic Tests

CBC – low hgb and hct
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Hemoglobin electrophoresis –
used to
determine type of hemoglobin. When you pass an
electric charge through a solution of hemoglobin,
distinct hemoglobins move different distances,
depending on their composition. This technique
differentiates between normal hemoglobin (A), Sickle
hemoglobin (S).
Goals in Care
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The is NO CURE for the disease
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Treat the Symptoms
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Relieve the pain
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Provide oxygenation
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Adequate hydration
Pain Management
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Give analgesics as ordered
Schedule nursing care to allow for
optimal rest
Position joints with pillows
Application of warmth
Promote circulation through passive ROM
Oxygenation
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Assess Oxygen saturation- pulse
oximetry. Should be 95% or >.
Administer Oxygen for short period of
time to keep saturation levels up.
Bedrest to minimize energy expenditure
and oxygen consumption
Administer blood transfusions
What are Complications associated
with frequent blood transfusions?
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Allergic reaction
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Circulatory overload
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Iron Overload
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Give Desferal, an iron-chelating agent, to
decrease the iron levels
Oxygenation
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Prevent infection, dehydration
Avoid emotional stress, overfatigue
Avoid prolonged exposure to heat and
cold
Avoid low oxygen environment
Hydration/ Electrolyte Replacement
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Encourage fluid intake, at least the minimum
amount of fluid required daily for that child’s
weight. (1-2x maintenance)
Record I & O. Monitor electrolyte balance
Give parents written instructions on specific
amount of daily intake needed.
Assess signs of dehydration (decreased
urination, thirst)
Teach parents measures to prevent dehydration
such as avoiding heat / stress.
Prevent Infection
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With the spleen damaged, has greater
chance of getting sepsis.
Avoid know sources of infection
Assess Vital Signs and report elevated
temperatures. Treat with Ibuprofen, NOT
ASA.
Place on prophylactic antibiotics such as
Penicillin VK 250 mg twice a day, through
age 5 yrs
Prevent Infection
Immunizations
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Hepatitis B series
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Pneumococcal
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Recipient of blood and blood products.
Pneumovax for now at age 24 months and 5
years, with an improved vaccine coming on
the market soon for administration to
infants.
Meningococcal vaccine
H. influenzae vaccine
Child / Parent Teaching
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Assess baseline knowledge and teach accordingly:
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Causes of disease and consequences, genetic counseling
Situations that cause sickling
Signs of developing crisis, infection,
When to call the doctor
Keep in school, promote normal g&d as much as
possible
Allow for decreased endurance - let the child set his
or her own pace during strenuous exercise, and to take rest
breaks when fatigue
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Explain some complications: CVA, anemia,
swollen spleen, liver problems
Support child and family
Critical Thinking

Which of the following nursing diagnoses
should receive priority during a vasoocclusive crisis in a 14 year old with
sickle cell anemia?
A.
B.
C.
D.
Alteration in comfort
Ineffective individual coping
Decreased cardiac output
Ineffective airway clearance
Critical Thinking

Which of these instructions should the
parents of a child who has recovered
from a sickle cell crisis receive?
A
B
C
D
Isolate child from known sources of infection
Avoid contact with all children
Restrict child’s intake during the night
Reinforce the basics of trait transmission
Hemophilia

A group of bleeding disorders in which there
is a deficiency of one of the factors
necessary for coagulation of the blood.
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Hemophilia A – most common bleeding disorder.
Deficiency of factor VIII.
Hemophilia is an hereditary condition in
which the mother may pass the defect to her
male child.
With deficiency of coagulation factor VIII,
blood does not coagulate as it should.
Do Hemophiliacs bleed more
Profusely than people
without Hemophilia?
Answer:

The blood of a person with hemophilia
does not clot normally. He does not
bleed more profusely or more quickly
than other people; however, he bleeds
for a longer time.
Hemophiliacs bleed mainly from
minor cuts

True or False
False
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This is a myth. External wounds are
usually not serious.

Far more important is internal bleeding
(hemorrhaging). These hemorrhages are
in joints, especially knees, ankles and
elbows; and into tissues and muscles.

When bleeding occurs in a vital organ,
especially the brain, a hemophiliac's life
is in danger.
What Causes a Bleeding Episode?

Bleeding is often caused by minor injury - a bump or a
slight twist of a joint. However, many hemorrhages,
especially among severe hemophiliacs, happen for no
apparent reason. This is even truer in joints that have
bled often in the past. The more a joint has bled, the
easier it bleeds again with no external cause.

Even hemorrhages in the brain often have no
apparent cause. Brain hemorrhages are the leading
cause of death from bleeding in hemophilia. Therefore
it is important to recognize the symptoms of a brain
hemorrhage very quickly.
Related to the deficiency of AHF
Interviewing the Child with
Hemophilia – Subjective Data
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Recent traumas and measures used to stop
bleeding
Length of time pressure was applied before
bleeding subsided
Was swelling increased after surface bleeding
subsided
Did swelling and stiffness occur without
apparent trauma
Assessment
Prolonged bleeding anywhere from
or in the body.
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Often diagnosed when infant is circumcised
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Bleeding in the mouth from a cut, bitten tongue
or loss of a tooth (especially in children)
Assessment
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Surface bruising - Bleeding from trauma into
soft tissues and muscles (the ileopsoas muscle
around the hip, calf, forearm, upper arm,
achilles tendon, buttocks, retroperitoneal.
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Hematuria
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Hemarthrosis - bleeding into joints (knees,
elbows, ankles, shoulders, hips, wrists in
descending order of frequency)
How to Assess Bleeding in a Joint
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The first sign is a feeling of tightness in the joint but no real
pain. The joint feels a little puffy to the touch.
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As the hours pass, the joint becomes hot to the touch. Fully
flexing or extending the joint becomes painful. Weight bearing
becomes difficult. By this time, the joint is visibly swollen.
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As the bleeding continues and the swelling increases, all
movement in the joint is lost. The joint becomes fixed in a
slightly flexed position in an attempt to relieve the interior
pressure in the joint. Pain at this point can be excruciating.

The bleeding slows after several days when the joint is so full
of blood that the pressure inside the joint cavity is equal to the
pressure inside the broken blood vessels. Slowly, the
bleeding stops and the long process of absorbing the blood in
the joint cavity begins.
Assessment
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CNS bleeding - Major cause of death
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Signs and Symptoms of CNS bleeds
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Persistent or increasing headache
Repeated vomiting
Sleepiness or a change in normal behavior
Sudden weakness or clumsiness of an arm or leg
Stiffness of the neck or complaints of pain with neck
movement
Complaints of seeing double
The development of crossed eyes
Poor balance when walking, a lack of coordination
Convulsions or seizures
Assessment of Child
Bleeding in the
mouth
Hemarthrosis
Surface bruising
Diagnostic Tests
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DNA testing for the trait
Blood Tests
1. Partial Thromboplastin Time (PTT) –
Prolonged
2. Bleeding time – Prolonged
> 2 hrs.
3. Platelet count and Prothrombin time –
Normal
4. Low levels of Factor VIII
Goals
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Prevent and control Bleeding
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Prevent crippling effects / Decrease pain
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Child/ Parent Teaching
Interventions to Prevent or
Control Bleeding
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The basic treatment to stop or prevent bleeding
in people with hemophilia A is:
Give Factor VIII replacement therapy
via IV.

These concentrates come from two sources:
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human plasma (a component of blood), Fresh whole
blood, fresh or frozen plasma may be used.
a genetically engineered (artificial) Factor VIII blood
product made by DNA technology.
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In both cases, the Factor VIII protein is nearly
identical to the protein which is lacking in the blood
of hemophiliacs. After an infusion of the concentrate,
all the proteins needed for clotting are in place. A
hemophiliac's blood becomes "normal", at least for a
few hours. This allows the time for a clot to form at
the site of the damaged blood vessel.

Unfortunately, the replacement of the missing
clotting factors is not permanent. Half of the clotting
factor activity which was infused is removed by the
body every 24 hours. This means that within 3 days
almost none is left. The hemophiliac's blood is again
unable to clot normally.
Advantages of Factor VIII concentrate
•They are very concentrated. A small amount contains
enough Factor VIII to control bleeding, even in major
surgery. Thus, they are very effective.
•They are convenient. The concentrates can be stored in a
home refrigerator for up to a year or kept at room
temperature for 3 to 6 months.
•They are easy to mix. Five to 10 ml. of sterile water is
added to the powdered concentrate. One minute later the
mixture is ready to be infused.
•They are quick to infuse. From beginning to end, the
infusion takes about 15 to 20 minutes.
•They are very safe. Hepatitis is rarely, if ever, transmitted
by the concentrates made from human plasma and the
concentrates made by genetic engineering are even safer
because they are not made from human plasma, which
can carry blood-borne infections.
Additional Interventions to
Prevent and Control Bleeding
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Administration of DDAVP (desmopressin acetate)
nasal spray used to stimiulate release of factor
VIII. Used with mild hemophilia
Apply local pressure for 10-15 minutes
Elevate joint above level of heart
Apply cold compresses to promote vasoconstriction
Prophylactic administration of factor VIII
What is prophylactic vs. ondemand therapy?
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In prophylaxis therapy, hemophiliacs receive factor
concentrates several times a week to prevent bleeding.
The goal is to keep the levels of Factor VIII or IX in the
blood high enough that bleeding does not happen. This
therapy is common with children.
On-demand therapy is the infusion of factor concentrates
immediately after the beginning of a bleed. The goal is to
stop the bleeding quickly, before any damage is done to
the joint or muscle.
Research has shown that prophylaxis therapy gives
children the best chance to reach adulthood without
damage to their joints.
Interventions to Prevent
Crippling effects / Decrease Pain
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During bleeding episode—elevate joint
and immobilize
ROM after acute episode
Exercise unaffected joints and muscles.
Give analgesics before Physical therapy.
Do NOT give Aspirin.
Watch diet/ weight – excessive weight
stresses the joints
What is the crippling Effect of
Repeated bleeds into a Joint?

Bleeding in joints, especially knees,
ankles and elbows can lead to:
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loss of range of motion
muscle loss
destruction of the joints themselves
Child / Parent Teaching

Measures to prevent injury/ providing a
safe environment
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Early recognition of bleeding episodes

Keep current on immunizations

Hepatitis B – because recipient of blood and
blood products
Child / Parent Teaching

How to administer factor VIII.

Children often learn how to infuse
themselves at the age of eight or
ten. Then, the hemophiliac is able to
treat himself at home, at school, at
camp or on vacation.
Critical Thinking

A 12 year old hemophiliac child has been
admitted to the hospital for hemarthrosis.
Which of these expected outcomes should
receive priority in the child’s care?
A. Family will receive genetic counseling
B. Child will participate in appropriate activities for
present condition
C. Child and family will seek support from National
Hemophilia Foundation
D. Maximum function of the joint will be restored
Review

Following administration of factor VIII to
a nine year old child admitted to the
hospital with hemarthrosis, the nurse’s
next action would be to:
A. Elevate and immobilize the affected joint
B. Institute passive range of motion to the
affected joint during the acute phase
C. Apply warm compresses to the affected
joint
D. Apply pressure to the area as needed
Cancer

Leading cause of death from disease
in children

Almost all childhood cancers involve
blood or blood-forming tissues
Types and Incidence of Cancers
Leukemia
Brain Tumors
Lymphomas
Neuroblastoma
Wilms
Rhabdomyosarcoma
Retinoblastoma
Othes
Childhood Cancers
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Brain tumors – second leading cause of death from
childhood cancer.
 Most are cerebellar and brain stem tumors
Lymphomas
 Non-Hodgkins lymphomas—one-third present with a
mass in the neck or mediastinal area. Also have
dyspnea, wheezing, abdominal mass or pain and
lymphadenopathy.
 Hodgkin’s disease – arises in single lymph node with
painless nodal enlargement, followed by extension to
adjacent nodes and into spleen, liver, lungs, bone
marrow.
Neuroblastoma – malignant tumor arising from
sympathetic NS ganglion cells outside the cranium and
and can arise from anywhere along the sympathetic
nervous system chain. Can also occur in retroperitoneal
area, pelvis, neck.

Wilms Tumor – solid tumor of kidney.

Rhabdomyosarcoma—malignant tumor of the
striated muscle cells.
 occur in muscles around eye, head, neck,
extremities, GU system.
Retinoblastoma – intraocular malignancy of the
retina of eye. Usually unilateral. If bilateral ,
hereditary. First sign is white pupil.


Others – osteogenic sarcoma/ Ewings sarcoma –
tumor of bones of the trunk. Often seen in
adolescence growth spurt. Found in distal femur,
proximal tibia.
Theories of Etiology

Intrauterine
carcinogens

Physical carcinogens

Viruses

Immune defects

Genetics
 Discovered gene
for leukemia on
chromosome 22
Warning Signs of Childhood Cancer
C = continual unexplained weight loss
H = headaches with vomiting (early morning)
I = increased swelling of pain in joints
L = lump or mass
D = development of whitish appearance in pupil
R = recurrent or persistent fevers, night sweats
E = excessive bruising or bleeding
N = noticeable paleness or tiredness
Diagnostic Tests
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Biopsy
Blood Tests
 CBC
 Uric Acid
Bone Marrow Aspiration
PET, SPECT
MRI, CT, ultrasound
Interventions

Radiation therapy

Chemotherapy

Surgery

Bone Marrow and Stem cell transplantation
Radiation Therapy
Changes the DNA component of
a cell nucleus
The cell cannot replicate which
Inhibits further cell division and growth
Effects of Radiation Therapy

Radiation sickness- anorexia, nausea,
vomiting

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Fatigue
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Treated with antiemetics (Zofran or Anzimet).
Cool cloth to forehead, provide distraction,
accurate I&O.
allow for naps an rest periods (coordinate care),
encourage parent to cuddle in bed with child,
pillow, blankets, favorite toys
Skin reactions –erythema, tenderness

Bone marrow suppression – anemia,
neutropenia, thrombocytopenia
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Mucositis- inflammation of mucus
membranes mainly the mouth

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May be on reverse isolation
Offer soft foods, and cold foods.
Frequent mouth care. Lidocaine oral to swish
in mouth (older child)
Long term – depends on part of body
receiving radiation
Chemotherapy

There are several categories of antineoplastic
drugs used in treating childhood cancers.

Scheduled at set times and days and by
different predetermined routes.

May remain in hospital for few days at first,
then later report on specific day for therapy.

Children and Parents must be taught about
what to do and not to do during therapy.
Side effects and Toxic Reactions to
Chemotherapy

Bone Marrow Suppression – neutropenia,
anemia, thrombocytopenia
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Place in reverse isolation, keep anyone exposed
to a virus away from patient.
Monitor temperature
Should not receive live-virus vaccines
Bleeding Tendency (thrombocytopenia)
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Apply pressure to puncture site
No contact sports
Check urine and stool for blood
Give stool softeners. WHY?
Soft objects in mouth

Malaise and fatigue
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Nausea, vomiting, diarrhea, anorexia
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Encourage video games, movies, etc
Allow visits from friends
Give anti-emetics
Small frequent meals
Monitor for dehydration
Altered mucous membranes
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Stomatitis
Rectal ulcerations
Side effects of chemotherapy

Renal involvement
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Body Image changes

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Uric acid levels rise as a result of breakdown of cells.
The renal tubules causing renal failure.
If kidney affected/damaged- chemo drugs will not be
excreted as usual and may limit drugs given.
Alopecia
Pain
Nursing Responsibilities
Related to Chemotherapy
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Know OSHA guidelines for administering
antineoplastic agents
Confirm all measurements and calculations
Double-check ordered dosages
Obtain complete blood count within 48 hours of
chemotherapy administration
Note white blood cell and platelet levels
before chemotherapy begins
Know side effects of chemotherapeutic agents
and ways to alleviate these effects
Nursing Responsibilities Related
to Chemotherapy (Cont’d)

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Ensure patency of IV tubing by checking
for blood return
Ensure needle placement for
implantable infusion device
Give vesicants (agents that can cause
tissue necrosis) only through a fresh IV
site
Have emergency drugs available
Review of Common Side Effects of
Chemotherapy and Radiation

Chemotherapy

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Bone marrow
suppression
Alopecia
Malaise/fatigue
Nausea
Vomiting
Anorexia
Stomatitis

Radiation side effects

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Skin reactions
Fatigue
Bone marrow
suppression
Nausea
Vomiting
Anorexia
Mucositis
Surgery

Curative
 Remove the tumor and cancerous
tissue

Palliative
 Relieve complications due to the
cancer
Bone Marrow and Stem Cell
Transplantation

The goal of therapy is to administer a lethal dose
of chemotherapy and radiation therapy that will
kill the cancer and then re-supply the body with
bone marrow and stem cells to reconstitute
immunologic function.

Healthy bone marrow or stem cells are infused
into the bloodstream and migrate to the marrow
space to replenish the patient’s immunologic
function and help kill remaining cancer cells.
Types of Transplantations

Syngeneic


Allogeneic


bone marrow comes from identical twin
bone marrow comes from matched sibling (one in
four chances) or someone who is histocompatible.
Autologous

own bone marrow. May be harvested at time of
remission in preparation for relapse or when bone
marrow is free of malignant cells. Also being used
so toxic doses of chemotherapy and radiation can
be administered and the bone marrow rescued.
Procedure

First --All potential donors are typed for HLA
(human leukocyte antigen) compatibility.
Collection of bone marrow is a surgical
procedure.



The donor undergoes anesthesia for aspiration of
the bone marrow
The bone marrow is then processed and frozen
When patient ready - it is infused into the
recipient.

Where is the most common site for bone
marrow aspiration in children?

Iliac crest
Side effects of Transplantation
1. Graft-Versus-Host Disease (GVHD) – potentially
lethal immunologic response of donor T cells
against the tissue of the recipient.
 Signs and symptoms – rash, malaise, high fever,
diarrhea, liver and spleen enlargement.
 Because there is no cure, prevention is
essential. Careful tissue typing, irradiation of
blood products which helps to inactivate mature
T lymphocytes.
2. Rejection of the transplant
Post Therapeutic Disabilities










CNS – cognitive disorders, seizures, headaches,
coordination problems
Bone – asymmetric growth of bones, easy fractures,
scoliosis, kyphosis
CV – cardiomyopathy (pericardial thickening) ,
pericardial damage
Respiratory – pneumonitis, pulmonary fibrosis
GI – enteritis, bleeding, hepatic fibrosis
Urinary – hemorrhagic cystitis, reflux
Endocrine – decrease in growth, thyroid and gonadal
dysfunction
Reproductive – decrease sperm
Dental - caries
Secondary malignancies
Ask Yourself?

What is the most common form of childhood
cancer?
a. leukemia
b. brain tumors
c. lymphoma
d.osteosarcoma
Leukemia

A malignancy of the blood forming
tissues/ bone marrow in which normal
bone marrow is replaced by malignant
immature WBC’s.
Pathophysiology of Leukemias

The WBC's are produced so rapidly that immature cells
(blast cells) are released into the circulation.

These blast cells are nonfunctional, can't fight
infection, and are formed continuously without
respect to the body's needs

The blasts cells then invade other organs and interfere
with metabolism / function. The production of red
blood cells and platelets decreases leading to anemia
and thrombocytopenia.
Leukemia
Signs & Symptoms
Bone marrow Depression results in:
1.
2.
3.
4.
Decrease in mature WBC’s - fever
Decrease RBC’s, Anemia- pallor, lethargy, anorexia
Decreased Platelets/ thrombocytopenia-  bleeding
Increase cell metabolism which deprives cells of
nutrients
5. Enlargement of organs infiltrated with blast cells
results in:
 Bone pain
 Spleenomegaly. Hepatomegaly, Nephromegaly
 Lymphadenopathy
 CNS infiltration – increased ICP
Diagnostic Tests

History and Physical

Blood Work
 Leukocytes are > 10,000
 Platelet count, Hgb and Hct low
 Blast cells appear (where they normally don’t)

Bone Marrow Aspiration
 Used to identify the type of WBC involved,
therefore, type of leukemia

X-rays of long bones
 Show lesions caused by invasion of abnormal cells

Lumbar Puncture – blast cells in the CSF
Chemotherapy



A combination of antineoplastic drugs
are given for about a month
A different combination is given for
about 2-3 years
Advantages of using a combination of
drugs:



Decrease resistance to one drug
Lessening of severe side effects of massive
doses of one drug
Breakdown of the tumor cell cycle at
multiple sites
Chemotherapy Phases

1. Remission induction- most intense treatment.
Large doses of antineoplastic drug administered in
an effort to destroy as many proliferating cells as
possible. Lasts 4-6 wks. About 95% respond.

2. Consolidation- method of destroying leukemic
cells in the CNS- for children who have CNS
involvement or are high risk. Given Intrathecal.

3. Remission maintenance- drugs given at specific
intervals. If remain in remission for 3 yrs, treatment
is discontinued. Approx 80% of children who sustain
remission for 2-3 yrs continue to remain in remission
and appear to be cured.
Administration of Chemotherapy

When is intrathecal administration
of chemotherapeutic medications
required?
Intrathecal chemotherapy is
instilled in to the spinal canal for
cancers that have metastasized to
the brain

What is the rationale for
the use of cranial
radiation in addition to
chemotherapy?

Bypass the blood brain barrier
Nursing Care
 Prevent infection (neutropenia, anemia)
 Pain Relief
 Nausea and vomiting
 Mouth discomfort-mucositis / stomatitis
 Fatigue
 Alopecia
 Prevent blood loss-platelet low- nose
bleed most common kind of bleed
 Support child and family
 Assist with referrals to social services,
home health agency, chaplain
Nursing Care

Community Resource

Candlelighters Childhood Cancer Foundation® (CCCF) is a
national non-profit membership organization whose mission
is to educate, support, serve, and advocate for families of
children with cancer, survivors of childhood cancer, and
the professionals who care for them.
 http://www.candlelighters.org/

American Cancer Society
Make a wish Foundation
Leukemia Society
Church and Schools



Tumor Lysis Syndrome

Caused by the breakdown (lysis) of malignant
cells which release intracellular contents into
the blood.

Intracellular electrolytes overload the kidneys
and can lead to kidney failure.

Further severe electrolyte imbalances cause
metabolic acidosis, hyperkalemia, increase uric
acid levels, hypocalcemia and cardiac
arrythmias.

Treat with IV fluids, electrolyte replacement,
allopurinol.
An embryonic tumor of the kidney.
Etiology and Pathophysiology

Cause is unknown

Originates from immature
renoblast cells

Tumor is vascular
Assessment

Palpable abdominal mass

Firm and smooth
**The abdomen should not be palpated once the
diagnosis is made. Avoid palpating the tumor mass
during assessment because of the risk of rupturing the
protective capsule. Excessive manipulation can cause
seeding of the tumor and spread of cancerous cells




Abdominal distention
Fever
Fatigue
Late signs
 Anemia
 Hematuria, dysuria
 Hypertension
Diagnostic Tests

Abdominal ultrasound

CT, MRI

Biopsy
Interventions

Nephrectomy and removal of lymph nodes

Post-op chemotherapy and / or radiation
therapy

CT every 6 months for 3 years

Chest x-ray every 3 months for 3 years
Nursing Care

Pre-op



Sign on bed ”Do Not Palpate Abdomen”
Child / Parent teaching
Post-op




Monitor kidney function, Strict I & O
Monitor vital signs—B/P and temperature
Monitor GI function– assess bowel sounds
and stool production
NG tube to drainage. Measure abdominal
girth.
Ewing’s Sarcoma

Clinical manifestations
Treatment sequence
 How does this treatment differ from
osteosarcoma?


Why is metastases more of a
concern with this cancer?
Rabdomyosarcoma
 Clinical
manifestations-
 Treatment
sequence-
 Chemotherapeutic
(VAC)
agents
Osteosarcoma

Clinical manifestations

Treatment sequence

Why is the sequence of treatment
important?
Review of Nursing Care:

Pre-operative nursing goals for the child
diagnosed with cancer



Disturbed sensory perception
Anticipatory grieving
Risk for infection
Post-operative nursing goals



Risk for infection
Impaired skin integrity
Impaired adjustment- disturbed body image
Pain
Review of Nursing Care:

What is the rationale for treating some
cancers with chemotherapy prior to
removal of the tumor?

What specific teaching should the nurse
include as priority regarding
chemotherapy?

What lab values does the nurse monitor
carefully in the client undergoing
chemotherapy?
Review of Nursing Care:

What are the priority psychosocial
needs of the child/family
diagnosed with cancer

What are nursing interventions to
assist with stressors that influence
the child/family’s response to the
diagnosis and treatment of cancer.
Caring for a child who is dying can be one
of the hardest tasks in nursing
Understanding of Death

Children under 3
 Have no understanding of own impending death
 May perceive family anxiety, sadness

Preschool to 5 years
 More afraid of separation from parents than of
thought of dying. Greatest fear is separation.
 Envision death as temporary, and have little of
adults’ fear of it
 Think of it as a long sleep, not a final process.
Nightmares increase.
 May feel pain / illness is a punishment for misdeeds
or thoughts
 May ask questions about death
 In long term illness – may simulate adult response
with depression, withdrawal, fearfulness, anxiety

School Age 5-9






Begin to understand that death is permanent
May think it is something that only happens to
adults
Become aware of what is happening to them
when their disorder has a fatal prognosis.
Concerns center around fear of pain, fear of
being left alone and leaving parents and
friends.
May associate death with sleep and may be
afraid to go to sleep without someone near
them.
May associate death with darkness—want light
left on in room
Understanding Death

Adolescent, older school age






By age 10 have an adults concept of death, realizing
that it is inevitable, universal, and irreversible. Have
more understanding than adults realize.
Understand that death is the cessation of life.
Emotional outbursts may reflect anger
View death as fearsome and fascinating (increase in
adolescent suicide).
May feel immune to death and deny symptoms for
longer than usual because they believe it is
impossible that anything serious could happen to
them.
Some adolescents consider themselves alienated
from their peers and unable to communicate with
their parents for emotional support feeling alone in
their struggle.
Nursing Care
Child
1.
2.
3.
4.
5.
6.
Elicit child's understanding of death before discussing
Encourage children to express feelings in own way
through play, drawings, or verbalization to promote
free expression.
Provide a safe, acceptable outlet for expressions of
feelings
Structure care of child to allow child choices and
participation in process within constraints of physical
condition
Help child maintain independence and control; normal
ADL as much as possible (set realistic goals)
Realize that they will go through the stages of dying:
denial, bargaining, anger, depression, acceptance
Nursing Care
Parents
1.
2.
3.
4.
Spend time with family to listen, answer questions, and
provide information. Discuss issues with parents before
discussing with child.
Provide opportunities for family to express their
emotions and deal with their feelings. Parental
reactions: continuum of grief process and usually
depend on previous experiences with loss,
intellectualization.
Reactions may depend on relationship with child and
circumstances of illness or injury
Reactions depend on degree of guilt felt by parents-help
them sort out
5.
6.
7.
8.
Assist parents in expressing fears, concerns, grief
to enable them to appropriately support child
Assist parents to understand sibling' possible
reactions to terminally ill child
· Guilt- believing they caused the problem or
illness
· Jealousy- wanting equal attention from parents
· Anger- feelings of being left behind
Support, enhance parent-child communication,
enhance parents' ability to support child
Refer to parent, family support groups- not alone,
help focus, open communication, provide
information
Nursing Care
Nurse
 Nurse needs to care for self.
 Care of the caregiver is imperative if
the nurse is to provide physical and
psychosocial care for families at such a
difficult time.
 Caring for dying children and their
families can be stressful and
emotionally demanding.