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By Jeanie Ward

Iron Deficiency Anemia

Reduction in the number of RBCs caused


by:
Decreased iron intake
Blood loss
Increased internal demands during growth
periods
Iron Deficiency Anemia

Manifestations:
Pallor; Pale mucus membranes
Enlarged spleen and heart
Poor muscle tone with decreased activity
Fatigue

Diagnosis
low hemoglobin (<11g/100mL)
Low hematocrit (<33%)
RBC are small and hypochromic
Serum iron levels are low ( 30g/mL)
Iron Deficiency Anemia
Treatment
Oral iron supplements
Ascorbic acid (vitamin C)
Diet high in iron
Iron-fortified formula or supplements
Limit cows 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

Sickle-shaped
Disc-shaped
Soft (like bag of
Hard (like piece
of wood)
jelly)
Often get stuck
Easily flows
in small blood
through small
vessels
blood vessels
Lives for 20 days
Lives for 120 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 RBCs 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
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
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
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
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
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
Chest pains
Fever, cough
Dyspnea, retractions
Leading cause of death in SCD.
Priapism

Persistent painful erection


of the penis occurring when
penile blood flow is obstructed.
Hepatomegaly

Enlarged liver with jaundice and


hepatic coma.

Hematuria

related to damage to the kidneys


Aplastic Crisis

Diminished RBC production resulting


in severe anemia
Manifested by:
Malaise, lethargy
Headache
Pallor
fainting
Aplastic Crisis
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 RBCs than
normal
Acute Sequestration Crisis

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 RBCs from the
bloodstream and recycles them

Minor Role:
Manufacturers RBCs toward end of fetal
life
and after birth, taken over by bone marrow.
Acts as a blood reservoir.
Spleen in the Infant

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

Blood transfusion

Spleen removal
Effects of Chronic Crisis
States on the Childs Body
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 Childs Body
Eyes bleeds in retina.

Leg Ulcers
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

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

The is NO CURE for the disease

Treat the Symptoms


Relieve the pain

Provide oxygenation

Adequate hydration
Pain Management

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

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?
Allergic reaction

Circulatory overload

Iron Overload
Give Desferal, an iron-chelating agent,
to decrease the iron levels
Oxygenation

Prevent infection, dehydration


Avoid emotional stress, overfatigue
Avoid prolonged exposure to heat
and cold
Avoid low oxygen environment
Hydration/ Electrolyte
Replacement
Encourage fluid intake, at least the
minimum amount of fluid required daily for
that childs 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
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

Hepatitis B series
Recipient of blood and blood products.
Pneumococcal
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
Assess baseline knowledge and teach
accordingly:
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
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 vaso-occlusive crisis in a 14
year old with sickle cell anemia?
A. Alteration in comfort
B. Ineffective individual coping
C. Decreased cardiac output
D. 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 Isolate child from known sources of
infection
B Avoid contact with all children
C Restrict childs intake during the night
D 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.
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
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
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.

Often diagnosed when infant is circumcised

Bleeding in the mouth from a cut, bitten tongue


or loss of a tooth (especially in children)
Assessment
Surface bruising - Bleeding from trauma into
soft tissues and muscles (the ileopsoas muscle
around the hip, calf, forearm, upper arm,
achilles tendon, buttocks, retroperitoneal.

Hematuria

Hemarthrosis - bleeding into joints (knees,


elbows, ankles, shoulders, hips, wrists in
descending order of frequency)
How to Assess Bleeding in a
Joint
The first sign is a feeling of tightness in the joint but no real pain. The joint
feels a little puffy to the touch.

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.

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
CNS bleeding - Major cause of death
Signs and Symptoms of CNS bleeds
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

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

Prevent and control Bleeding

Prevent crippling effects / Decrease


pain

Child/ Parent Teaching


Interventions to Prevent
or Control Bleeding
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:


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.
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
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. on-
demand therapy?
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
During bleeding episodeelevate
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:
loss of range of motion
muscle loss
destruction of the joints themselves
Child / Parent Teaching

Measures to prevent injury/


providing a safe environment

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 childs 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 nurses
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
Childhood Cancers
Brain tumors second leading cause of death from
childhood cancer.
Most are cerebellar and brain stem tumors
Lymphomas
Non-Hodgkins lymphomasone-third present with a
mass in the neck or mediastinal area. Also have
dyspnea, wheezing, abdominal mass or pain and
lymphadenopathy.
Hodgkins 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.

Rhabdomyosarcomamalignant 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 Immune defects


carcinogens
Genetics
Physical Discovered
carcinogens gene for
leukemia on
Viruses 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
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
Treated with antiemetics (Zofran or Anzimet). Cool
cloth to forehead, provide distraction, accurate I&O.

Fatigue
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
May be on reverse isolation

Mucositis- inflammation of mucus


membranes mainly the mouth
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
Place in reverse isolation, keep anyone exposed
to a virus away from patient.
Monitor temperature
Should not receive live-virus vaccines
Bleeding Tendency (thrombocytopenia)
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
Encourage video games, movies, etc
Allow visits from friends
Nausea, vomiting, diarrhea, anorexia
Give anti-emetics
Small frequent meals
Monitor for dehydration
Altered mucous membranes
Stomatitis
Rectal ulcerations
Side effects of chemotherapy
Renal involvement
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.

Body Image changes


Alopecia

Pain
Nursing Responsibilities
Related to Chemotherapy
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
(Contd)
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 Radiation side effects
Bone marrow Skin reactions
suppression Fatigue
Alopecia Bone marrow
Malaise/fatigue suppression
Nausea Nausea
Vomiting Vomiting
Anorexia Anorexia
Stomatitis 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 patients immunologic
function and help kill remaining cancer cells.
Types of Transplantations
Syngeneic
bone marrow comes from identical twin

Allogeneic
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 WBCs.
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. Decrease in mature WBCs - fever
2. Decrease RBCs, Anemia- pallor, lethargy, anorexia
3. Decreased Platelets/ thrombocytopenia- bleeding
4. 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 dont)

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 signsB/P and temperature
Monitor GI function assess bowel sounds and stool
production
NG tube to drainage. Measure abdominal girth.
Ewings 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 agents
(VAC)
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:

Whatare the priority psychosocial


needs of the child/family
diagnosed with cancer

What are nursing interventions to


assist with stressors that influence
the child/familys 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 darknesswant 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. Elicit child's understanding of death before discussing
2. Encourage children to express feelings in own way
through play, drawings, or verbalization to promote
free expression.
3. Provide a safe, acceptable outlet for expressions of
feelings
4. Structure care of child to allow child choices and
participation in process within constraints of physical
condition
5. Help child maintain independence and control; normal
ADL as much as possible (set realistic goals)
6. Realize that they will go through the stages of dying:
denial, bargaining, anger, depression, acceptance
Nursing Care
Parents
1. Spend time with family to listen, answer questions, and
provide information. Discuss issues with parents before
discussing with child.
2. 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.
3. Reactions may depend on relationship with child and
circumstances of illness or injury
4. Reactions depend on degree of guilt felt by parents-help
them sort out
5. Assist parents in expressing fears, concerns, grief to
enable them to appropriately support child
6. 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
7. Support, enhance parent-child communication,
enhance parents' ability to support child
8. 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.

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