Transcript Document 7131228

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Iron-Deficiency Anemia
Sickle Cell Anemia
Beta-Thalasemia
Major (Cooley’s anemia)
Hemophilia A
Von Willebrand’s Disease
ITP (Immune Thrombocytopenic Pupura)
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Acute Lymphocytic Leukemia
Hodgkin’s Disease
Non-Hodgkin’s Lymphoma
Retinoblastoma
Neuroblastoma
Nephroblastoma
Osteogenic Sarcoma
Ewing’s Sarcoma
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WBC
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RBC
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Hgb
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Hct
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Platelet
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WBC
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RBC
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Neutrophils- phagocytosis
Lymphocytes – T and B cell
Monoocytes – phagocytosis, antigen
Eosophils- allergen
Basophils-inflammatory
MCV- volume
MCH
MCHC
RCW- width
Hgb
Hct
Platelet
◦ MPV
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PT/PTT
Sed Rate (ESR)
Iron
TIBC (Transferrin)
Ferritin
Bilirubin
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RBC: 3.89-4.96
HgB: 10.2-13.4
Hct: 31.7-39.3%
Sed: 1-8
WBC: 5,400-11,000
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Platelets: 206,000403,000
Fe: 20-105
Ferritin: 47-110
TIBC: 240-508
PT: 10-11 sec
PTT: 42-54 sec
Bilirubin- less than
11.7
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Reduction of:
◦ number of red
blood cells
◦ the quantity of
hemoglobin
◦ the volume of
packed red
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Iron-Deficiency
Anemia
Sickle Cell Anemia
Beta-Thalasemia
Major (Cooley’s
anemia
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A nutrient deficiency of inadequate dietary
iron
The most common hematologic disorder of
infancy and childhood
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Peaks at 9 months- 2 years, adolescence
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Prevented by use of iron fortified productsn
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low birth weight infants
infants born to mothers with iron deficiency
anemia
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infants born with GI defects
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chronic blood loss in older children
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Dietary Fe is bloodstream binds to transferrin
(TIBC) and is delivered to RBC in bone
Marrow, combines with other cells to make
Hgb
Unused dietary Fe is stored in intestinal
epithelial cells as ferritin
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Dietary history usually shows abnormally high
milk intake > 32 oz day in toddler
Ask parents specific questions
Begin the dietary history at the time the child
awoke yesterday; include all activities and
exactly what the child ate
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Low RBC
Low HGB
◦ Mild ( < 10.2), Moderate (8-9), Severe (< 7)
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Low HCT
Low Iron
High Transferrin (TIBC)
Low Ferritin
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Low Hgb=low O2 tissue perfusion
Hgb of 10.2 or less
◦ May seem asymptomatic, not noticed by caregiver
◦ Pallor/Pale mucous membranes (low hgb, not enough red
color to skin)
◦ Poor muscle tone, decreased activity
◦ Fatigue
◦ Increased HR, RR
Hgb < 9
◦ Above symptoms plus irritability, lack of interest in play
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Consume Iron-fortified formula
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Limit cow’s milk to 24-32 oz/day for children
>12 months
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Increase age-appropriate iron-rich foods and
Vit C
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May be ordered to take Fe supplementsFerrous Sulfate
Iron-Rich Foods
Vitamin C Rich Foods
Meats, fish, poultry
Orange juice
Vegetables
Citrus fruits
Dried fruits
Strawberries
Legumes
Tomatoes
Enriched grain products
Broccoli
Whole grain cereal
Leafy Green vegetables
Iron-Fortified Cereal
Potatoes
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Manage side effects of Ferrous Sulfate
◦ Nausea,
◦ Anorexia
◦ Constipation
◦ Abdominal distress
◦ Black stools.
Give on an empty stomach if possible
Monitor bowel movements and suggest increased
fluid and fiber.
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Monitor development, sleep, and
activity/fatigue patterns
Monitor hemoglobin to measure
effectiveness of therapy
Instruct families to keep Ferrous Sulfate
locked and out of reach of children;
poisoning is a serious risk
Autosomal recessive disorder
 Seen in African Americans
 Characterized by abnormal hemoglobin
(HbS)
 Clinical manifestations caused by
obstructions due to the sickled RBC’s and
destruction of sickled and normal RBC’s
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Can be diagnosed in-utero
Symptoms may not appear until 6 months of
age
Mortality rate children < 3 years old is 35%
 Pallor
 Fatigue
 SOB
 Irritability
 Jaundice
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Moderately low Hcb and Hct
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Normal Iron, TIBC, Ferritin
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Elevated Billirubin
1.
Vaso-Occlusive Crisis
2.
Acute Chest Syndrome
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Splenic Sequestration
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Severe, sudden onset of sickling where
many new sickled cells pool in a vessel and
cause pain and tissue hypoxia
Caused by: infection, dehydration, anxiety,
cold
Most common from hypoxia secondary to
rapidly destroyed RBC
Lasts for hours to weeks
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Early Signs
◦ fever
◦ tachycardia
◦ pallor
Late Signs
◦ abdominal pain
◦ back pain
◦ extremity pain
First Crisis in infants
◦ Dactylitis (hand &
foot syndrome)
 swelling of hands and
feet
 joints may be warm &
swollen
 Pain
relief
 Prevent/Treat occulsions
 Adequate oxygenation
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Assess pain every 1-2h or more frequently
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Use pain scale appropriate for age
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Non-pharmacological pain methods
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AROUND THE CLOCK PAIN MEDS
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Tylenol for mild pain
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Narcotics for mod-severe pain
 Prevents
and treats occlusion
 Push PO fluids
 IV hydration 1.5 to 2 times
normal rate
 Risk for fluid overload
◦ Listen for crackles
 Administer
oxygen
 Maintain saturation of 95% or
higher
 Semi-fowler’s position
 Sickle contents
break off
 Bilateral
pulmonary
involvement
 Causes chest
infection,
embolism
 Symptoms
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Chest pain
Fever
Cough
Wheeze
Tachypnea
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Analgesics
Oxygen
Hydration
Incentive
spirometry
Antibiotics
PRBC
 Sickled cells block the spleen
 Blood pools in spleen and/or
liver and enlarges
 Pooled blood leads to a decrease
in circulating volume
 Can lead to hypovolemic shock
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Symptoms
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Irritability
Pale
Tachycardia
Pain to LUQ
Enlarged Spleen
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Life Threateningget child to ED
a.s.a.p.!
PRBC
Remove spleen
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Risk for Infection r/t Chronic
Immunosuppression
Administer PCN everyday
Up-to-date vaccines
Educate parents
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Signs of infection & respiratory distress
possible triggers
treat pain immediately
adequate fluids
(Cooley’s Anemia)
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Hereditary anemia due to abnormal
synthesis of hemoglobin
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Life long disorder
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Mediterranean descent
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Life threatening symptoms
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Low RBC’s
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Extremely low Hgb < 5
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Increased serum iron
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Facial anomalies
◦ Frontal bossing
(prominent and
protruding forehead)
◦ Maxillary prominence
◦ Wide-set eyes with a
flattened nose
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Bronze skin color
(Greenish yellow skin
tone)
Growth and
maturation
retardation
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RBC transfusions q 2-4 weeks
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Iron Chelation therapy
◦ Desferal (deferoxamine) SQ
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Splenectomy
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Cure isbone marrow stem cell transplant
 Estimated 70% do not find a suitable donor
◦ Observe for complications of transfusion- iron
overload
◦ Supporting the child and family in dealing with a
chronic life-threatening illness
◦ Monitor Growth and Development
◦ Refer the family for genetic counseling
Iron Deficiency Sickle Cell
Thalasemia
•Low RBC’s
•Low HCT
•Low Hgb
•Low iron
•Low ferritin
•High TIBC
•Low RBC’s
•Low HCT
•Very low Hgb
•Increased iron
•Normal
ferritin
•Normal TIBC
•Low RBC’s
•Low HCT
•Mod low Hgb
•Normal iron
•Normal
ferritin
•Normal TIBC
•Inc Bilirubin
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Host of factors
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Platelets aggregation at site of injury
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Tested by coagulation time (PT/PTT)
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Hemophilia A
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Von Willebrand’s Disease
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ITP (Immune Thrombocytopenic Pupura)
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Hereditary blood coagulation deficiency
(factor 8)
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Ability to clot is slower
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X-linked recessive (white, males)
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Recent traumas and measures used to stop
bleeding
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Length of time pressure was applied before
bleeding subsided
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Whether swelling increased after surface
bleeding subsided
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Whether swelling and stiffness occurred
without apparent trauma
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Above History
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Suspected by Labs:
◦ Platelet level: Normal
◦ PTT: Prolonged (elevated number) > 60
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Confirmed by genetic testing for missing
factor
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Vary according to
concentration of
factor 8
Soft tissue bleeding
and painful
hemorrhage into
joints
Severe bleeding may
occur in GI tract,
peritoneum or CNS
Acute therapy
 Acute bleeding stopped by IV administration of
factor 8
 Pressure to laceration
Prophylactic therapy
 PO factor 8 replacement on a regular schedule if
frequently symptomatic (prior to surgery, dental
work)
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Primary Goal is Injury Prevention
Promote oral hygiene, up to date
immunizations
No aspirin
Avoid activities that induce bleeding
Provide activities for normal G&D
Administration of factor replacement prn
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Most commonly inherited bleeding disorder,
autosomal dominant (Males and Females)
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Lacks production of VWF
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Platelets are normal in number
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Inability of platelets to aggregate
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Varying degrees of disease
◦ VWF is deficient to defective
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Platelets is normal
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PT/PTT is normal
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Confirmed by genetic testing for VWF
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Can be so mild
that disease is
undiagnosed
 Bleeding from
gums
 Epitaxis
 Prolonged
bleeding from
cuts
 Excessive
bleeding
following surgery
 Primary
Goal: Injury Prevention
 Prophylactic
therapy
◦ Replace dysfunctional factor in
blood when bleeding with
DDAVP
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Autoimmune disorder (antiplatelet antibody)
or cause is unknown (idiopathic)
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Occurs most commonly at age 2-4 years
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Reduction in and destruction of platelets
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Typically seen 2 weeks after a febrile, viral
illness
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Excessive bruising
and petechiae
Epitaxis
Bleeding into joints
Tourniquet test:
shows many
petechiae after
inflation of BP cuff
 Platelets
 PT
< 150
(Marked thrombocytopenia)
and PTT is Normal
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PLT transfusion (only a temporary solution)
Injury Prevention
Avoid when possible:
◦ administering intramuscular injections
◦ aspirin, aspirin-containing products, and
nonsteroidal antiinflammatory medications
(e.g., ibuprofen)
◦ taking temperatures rectally
Hemophilia A
VWF
ITP
Normal Platelets Normal Platelets Very Low
Platelets
Elevated
PT/PTT
Normal PT/PTT Normal PT/PTT
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Cancer in adults
◦ abnormal cell is transformed by genetic mutation
of its DNA
◦ usually as a result from exposure to a tetragon
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Cancer in children
◦ usually arises from chromosomal abnormalities,
genetic mutations and proliferation of embryonic
cells
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Surgical intervention
◦ Removing the entire cancerous tumor
◦ Most ideal and frequently used treatment method
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Chemotherapy
◦ destroy tumor cells by cause cell
death
◦ normal cells that have rapid growth
are also affected, such as hair growth
◦ toxic side effects
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Radiation therapy
◦ Least preferred treatment in children
◦ Interrupt cellular growth by breaking the DNA
stands, leading to cell death
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Small percentage Carcinoma (opposed to
large percentage in adults)
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Mostly Leukemia
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Followed by Lymphoma
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The rest is solid or soft tissue tumors
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Differ based on type of cancer
Many symptoms are similar to common
childhood illnesses
Symptoms may be in site other than the
cancer
=delay in diagnosis
Often diagnosis made when cancer is
advanced
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Pain
Anemia
Anorexia, weight loss
Infections
Bruising
Neurological symptoms
Palpable mass
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Parents in disbelief
Health child suddenly becomes ill
Potentially life-threatening
Treatment decisions, can last months-years
Travel for treatment, heavy financial
responsibilities
Effects of siblings
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Infants- unaware of diagnosis
Toddlers- aware they do not feel well
Preschoolers-beginning understanding of
illness, not cancer
School-age-understand cancer, benefit from
talking about it
Adolescents-mature understanding, benefits
from other adolescents with cancer
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Provide optimal nutrition- high metabolic rate
of cancer depletes stores
Ensure adequate hydration-ice pops, jello
Manage pain
Promote growth and development
Prevent Infection (next slide)
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Monitor vital signs q4h
Instruct parents how to measure temp at
home
Proper handwashing
Inspect child’s skin for breakdown
Inspect child’s mouth for ulcers
Teach child and parents meticulous oral
hygiene
No live virus administration
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Broad term describing a group of malignant
diseases
Normal bone marrow is replaced by
abnormal immature cells
Develops from a variety of agents thought
to increase risk (virus, toxins, drugs)
combined with genetics
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Occurs in children< 15 years old (peak 2-6
years old)
Distorted and uncontrolled proliferation of
immature WBC’s (lymphoblasts)
Causes decreased RBC’s, platelets, and
mature WBC’s production
Invasion of body organs by rapidly
increasing lymphoblasts
CBC Changes
Physical Exam
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Fever
Bone or joint pain
Bruising
Lymphadenopathy
Hepatosplenomegaly
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Decreased RBCs
Decreased PLTs
Very high WBC
 Signs
& symptoms
 CBC changes
 Confirmed with bone marrow
aspiration (> 25% of lymphoblast
cells present)
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High dose chemotherapy
administered for 2-3 years
Returns blast cells in bone
marrow to less than 5%
Physical assessment findings
are normal
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Metabolic emergency
Lysis (dissolving or decomposing) of
tumor cells
Rapid release of their contents into
the blood
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Rapid cell destruction releases high
levels of
◦ uric acid
◦ potassium
◦ phosphates
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Uric acid overloads the kidneys
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Leads to cardiac arrhythmias and renal failure
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receiving chemotherapy
monitor for
◦ Hyperuricemia
◦ Hyperkalemia
◦ Hyperphosphatemia
◦ Hypocalcemia
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Administer vigorous hydration (2–4 times rate
for maintenance fluid)
Administer allopurinol or urate oxidase
(rasburicase)
Reduce conversion of metabolic by-products
to uric acid
Hodgkin's
Non Hodgkin's
Retinoblastoma
A
malignancy that arises from the
lymphoid system
 Two
types
◦ Hodgkins
◦ Non Hodgkins
 Neoplasm
 Affects
 Males
of the lymph tissue
adolescents to late 20’s
> females
 Etiology
unknown- infectious
agent likely
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Begins as a
single painless
enlarged cervical
node
Spreads
predictably to
nonnodal sites
◦ spleen, liver, bone,
marrow, lungs,
mediastinum
 As
cancer progresses
◦Unexplained weight loss
◦Unexplained fevers
◦Night sweats
Diagnosis
 Biopsy of enlarged lymph node
Treatment
 Chemotherapy
 Radiation-low doses, higher if
physiologically mature
 Good Prognosis-single origin
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Aggressive neoplasm of many lymph nodes
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No single origin
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Rapid in onset
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Affects younger children ages 5-15
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Males > females
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Cause unknown-infectious agent likely
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Multiple enlarged painless lymph nodes
Acute abdominal and chest pain,
constipation, cramping
Anorexia, weight loss
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As cancer progresses
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CNS symptoms,
Headache
Nausea
Vomiting
Mediastinal mass
Petichaie
Bruising
Bone pain
Diagnosis
 Biopsy from bone marrow or lymph node
Treatment
 Aggressive multi-agent chemo for 2 years
 Risk for tumor lysis syndrome
 May need crainal radiation
Hodgkins
Non Hodgkins
Males>Females
Late adolescent-20’s
Single origin of cervical
gland
Good Prognosis
Males > females
Ages 5-15
No single origin widespread involvement
Aggressive treatmentmay have poorer
progrosis
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Malignant tumor of retina
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Immature retinal cells become malignant
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Affects children 6 weeks of age to
preschool age
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May be unilateral or bilateral
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Absent red reflex
Whitish glow to
pupil
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Strabismus
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Eye pain
 Small
retinoblastoma
◦ Cryosurgery
◦ Partial vision
 Large
retinoblastoma and/or
metastases brain/ 2nd eye
◦ Chemo
◦ Radiation
◦ Enucleation
◦ Eye prosthesis
Neuroblastoma
Nephroblastoma
Osteoscaroma
Ewing’s Sarcoma
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Solid tumor usually in abdomen
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Affects infants to pre-school age children
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Cancer cells arise from sympathetic
nervous system called crest cells
◦ Embryologic cells of adrenal glands
◦ Etiology: unknown
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Depend on
◦ extent of disease
◦ location of tumor
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65% of neuroblastomas
◦ protuberant, firm, irregular abdominal mass that
crosses midline
 impaired
ROM and mobility
 pain & limping
 respiratory symptoms
 Management
◦ depends on the presence and extent
of metastasis
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Malignant tumor of the kidneys
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Peak age 3-4 years
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Girls > boys
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Cause is unknown
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Parents usually notice a large, mobile
abdominal mass while bathing or the diaper
doesn’t fit anymore
Grows extremely quickly, in a matter of days
 Hematuria
 Hypertension
 Abdominal
 Fatigue
 Anemia
 Fever
pain
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tumor confined to the kidney and completely removed
surgically
tumor extending beyond the kidney but completely
removed surgically
regional spread of disease beyond the kidney with
residual abdominal disease postoperatively
metastases to lung (primary site), liver, bone, distant
lymph nodes
bilateral disease
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DO NOT PALPATE ABDOMEN
◦ can rupture the tumor and cause spreading of
cancerous cells
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State 1 and 2
◦ Nephrectomy
◦ Chemotherapy
Stage 3-5
◦ Nephrectomy
◦ Radiation
◦ Chemotherapy
 Bone
cancer from osteoblasts
 Affects
adolescents
 Attributed
to extremity injury or
growth spurt
 40-50%
knee
occur at distal femur and
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Progressive pain at site of tumor
Palpable mass & swelling
Limping
Limited range of motion
Pathological fractures
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Remove tumor, prevent spread of disease
Combination of surgery & chemo
Amputation my be necessary
Limb salvage operation
 Comfort
 Infection
 Potential
hemorrhage
 Phantom limb pain
 Prosthesis
 Changes in body image and
functioning
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Highly malignant
tumor in bone
marrow
Can present in any
bone
Spreads
longitudinally
through bone
Affects older children
and young
adolescents
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Intermittent pain attributed to injury
Swelling at tumor site
Pain becomes constant
Progresses into
◦ Weight loss
◦ Fever
◦ Increased sed rate
Metastases is usually present at time of dx (lungs, bone,
CNS, lymph nodes)
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Surgery
Multi agent chemo
◦ Risk for tumor lysis syndrome
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Radiation
Osteogenic scaroma
Ewing’s Sarcoma
Affects long bones
Older adolescents
Intermittent pain Palpable
mass & swelling
Limping, progressive
limited range of motion
Pathological fractures
Metastases not as likely
Surgery and chemo
Affects any bone
School-age and adolescents
Intermittent pain becomes
constant
Swelling at tumor site
Progresses into systemic
symptoms
Metastases likely
Aggressive treatment
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Fear
Death Anxiety
Anticipatory Grieving
Hopelessness
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Goals for the child
◦ Achieve and maintain normalization
◦ Obtain the highest level of health and function
possible
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Goals for the family
◦ Remain intact
◦ Achieve and maintain normalization
◦ Maximize function throughout
the illness
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Attend to the needs of the family system
Revise goals frequently to meet the child’s
changing developmental needs
Listen carefully to the child's perception of
the condition
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Be available to assist both child and family
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Avoid imposing personal beliefs and expectations
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Provide time and attention to the dying child
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Recognize the need to talk about illness
and death
Provide adequate pain control, oral care, privacy,
and information about the signs of imminent death
After death, allow family members as much time as
they desire with the child
A child is being admitted to the unit with
thalassemia major (Cooley’s anemia). In
preparing client assignments, the charge
nurse wants to assign a nurse to this child
who can:
1.
2.
3.
4.
Teach dietary sources of iron
Administer blood infusions
Work with a dying child
Monitor the child for bleeding tendencies
A 14-year-old boy with sickle cell anemia
is admitted with severe pain in his
abdomen and legs. He asks why the
doctor ordered oxygen when he is not
having any breathing problems. The nurse
states the therapeutic action of O2 is:
1.
2.
3.
4.
Prevent further sickling
Prevent respiratory complications
Increase O2 capacity of RBCs
Decrease the potential for infection
A 10-year old in the ER has a CBC results
that include a Hgb of 8, and Hct of 24. The
nurse determines that based on the lab
results which nursing action has a high
priority?
1.
2.
3.
4.
Promotion of skin integrity
Promotion of hydration
Promotion of nutrition
Conserving energy
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A 17-year old is being admitted for an
amputation related to a bone tumor. The
nurse is developing a nursing care plan
and determines the most appropriate age
related diagnosis is:
1. Risk
for disuse syndrome
2. Disturbed body image
3. Self-care deficit
4. Activity related intolerance
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WBC 7,200
RBC3.01
Hgb 9.1
Hct 29.3
Platelets 371,000
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Iron 64
Ferritin 70
Transferrin 250
• Bilirubin 18.2
• PTT 45 seconds
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After analyzing the results, the nurse
suspects the child may have:
1.
2.
3.
4.
Fe Deficiency Anemia
Cooley’s Anemia
Sickle Cell Anemia
Aplastic Anemia
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The nurse is admitting a child for a swollen
elbow. The history indicated multiple
bruising. Which of the following laboratory
results heightens the nurses suspicion for
Hemophilia?
1. Hbg 12,000
2. WBC 9,000
3. Platelets 356,000
4. PTT 73 seconds