Transcript Pediatric Malignancies

Pediatric Malignancies
Jan Bazner-Chandler
CPNP,MSN, CNS, RN
Pediatric Malignancies
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1% of all cancers
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Involves tissues of:
CNS, bone, muscle, endothelial tissue
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Grows in a short period of time
Causes
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Genetic alteration
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Environmental influences
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No know prevention
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Metastasic disease seen in 80%
Response to Treatment
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Very responsive to chemotherapy
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More than 60% cure rate
Classification of Tumors
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Embryonal tumor arises from embryonic tissue
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Lymphomas = lymphatic tissue
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Leukemias = blood
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Sarcoma = seen in bone, cartilage, nerve and fat
Cardinal Signs of Cancer
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Unusual mass or swelling
Unexplained paleness and loss of energy
Spontaneous bruising
Prolonged, unexplained fever
Headaches in morning
Sudden eye or vision changes
Excessive – rapid weight loss.
Diagnostic Tests
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X-ray
Skeletal survey
CT scan
Ultrasound
MRI
Bone marrow aspiration
Biopsy
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Identify cell to determine type of treatment
Treatment Modalities
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Determined by:
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Type of cancer
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Location
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Extent of disease
Surgery
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The oldest form of cancer treatment
Surgery plays important role in initial
diagnosis: biopsy of primary tumor.
Excision of tumor when possible
Facilitating treatment: insertion of catheters
for long-term treatment
Radiation Therapy
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The use of ionizing radiation to break apart bonds
within a cell causing cell damage and death.
External beam therapy accounts for the majority of
radiation treatments in children.
Problems: radiation beams cannot distinguish
between malignant cells and healthy cells.
Chemotherapy
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Primary treatment modality used to cure
many pediatric cancers.
Chemotherapy is the use of drugs to destroy
cancer cells.
The destruction is accomplished by inhibiting
cells within the body to divide, which
eventually leads to cell death.
Chemotherapy
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Can be given in addition to another form of
therapy such as radiation or surgery.
Drugs may be administered before surgery to
reduce size of tumor.
Adjuvant chemotherapy is used after surgery
or radiation therapy to prevent relapse.
Chemotherapy
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Combination chemotherapy is the use of
more than one class of drug.
Administering different classes of chemo
drugs ensures a greater chance of achieving
complete cancer cell destruction and
achieving remission.
Administration
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Chemotherapy can be given by mouth,
subcutaneous or intramuscular injections,
intravenously, or intrathecally.
 Oral route used if drug is well absorbed and non
irritating to the GI tract
 Sub-q or IM: Slow systemic release
 IV push, piggyback or intravenous infusion
Goals of Chemotherapy
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Reducing the primary tumor size
Destroying cancer cells
Preventing metastases and microscopic
spread of the disease
Chemotherapy Drugs
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Alkylating drug: attack DNA
Antimetabolites: interfere with DNA production
Antitumor antibiotics: interferes with DNA production
Plant alkaloids: prevent cells from dividing
Steroid hormones: slow growth of some cancers
Bone Marrow Transplant
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HSCT: Hematopoictic Stem Cell Transplant:
CHLA has one of the largest program.
The option of HSCT depends on the patients
disease, disease status, and general physical
condition.
Involves:
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Umbilical cord blood
Parent’s stem cells
Gene Therapy
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Use of gene therapy in the treatment of
childhood cancer is promising yet complex
and still in early phases of clinical application.
Management of Cancer
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Patient / family education
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Begins at time of diagnosis
Continues through treatment phases
Maintained in post-survival years
Support if death of child
Emotional aspects of leukemia
 http://leukemia.org/pages/413.html
Pain Management
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Pain caused by disease
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Pain with procedures and treatments
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Pain associated with side effects of treatment
Pain Management
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Pharmacologic
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Non-Pharmacologic
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Sedation or anesthetic medications
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EMLA cream
Conscious sedation
Pain Control
http://pedspain.nursing.uiowa.edu/
Immunosuppression and Infection
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Children with cancer become immune impaired
from a number of causes:
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Lymphocyte production is altered
Splenic dysfunction can prevent maturation of blood
cells and alteration is inflammatory response.
Cancer therapy can decrease immunoglobulin
concentrations.
Neutropenia
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Significant neutropenia can develop during
chemotherapy creating an increased risk of
infection in the child with cancer.
Neutropenia occurs when the absolute
neutrophil count decreases below 500.
Treatment of Neutropenia
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Granulocyte colony stimulating factor
decreases the duration of neutropenia by
stimulating the proliferation of the progenitor
cells of the granulocytes, specifically the
neutophils.
G-CSF: 5mcg/kg/day given subcutaneous
Varicella
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If an immunosuppressed child with no history
of varicella infection or varicella immunization
has direct contact with an individual with
chickenpox or shingles, varicella zoster
immune globulin should be administered.
Acyclovir IV is used in some cases.
Varicella Immunizations
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Three months after chemotherapy
Off prednisone
Many will have already had the immunization
as a toddler since it is now a required
immunization.
Central Venous Access Devices
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Two decades ago, CVAD were introduced as
an integral part of the pediatric oncology
patient’s treatment plan.
Used to deliver chemotherapy, blood
components, antibiotics, fluids, TPN,
medications and blood sampling.
CVAD Infection Prevention
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Teach family to report signs of catheter
infections: fever, chills, swelling, pain,
drainage, or erythema.
Aseptic technique for dressing changes and
heparin flushing.
Avoid trauma to device
Observe for catheter occlusion
Chemotherapy Side Effect
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Drugs affect not only the cancer cells but also
healthy cells.
Cells most affected are rapidly growing cells
such as hair follicles, reproductive system,
bone marrow and gastrointestinal tract.
Management of Side Effects
Malnutrition
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Occurs in 8 to 32% of the pediatric oncology
population
Nutritional goals focus on maintaining normal
growth and development as well as
preventing nutritional deficiencies.
Nutrition Interventions
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Initial nutritional assessment
History of child’s eating habits, food allergies,
use of nutritional supplements, base line
weight and height measurements.
Enteral feedings at night: preserve intestinal
mucosa by keeping it functional
Nausea and Vomiting
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Most common side effect of cancer treatment.
Chemotherapy-associated vomiting is a reflex
controlled by chemoreceptor trigger zone that
stimulates the vomiting center in the brain.
Tumor location
Radiation therapy
Anticipatory nausea
Interventions
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Antiemetics such as Phenothiazines: (Trilafon),
(Phenergan)and (Thorazine) block dopamine
receptors from stimulating the chemoreceptor trigger
zones.
Serotonin-receptor antagonist such as Granisetron
(Kytril) and Ondansetron (Zofran) are very effective.
(>3 years)
Antihistamines: benadryl
Administer before chemotherapy
Mucositis
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Progressive, inflammatory, ulcerative condition of the oral
and gastric mucosa.
Occurs due to the interruption of cell renewal process of
the epithelium leading the mucosal atrophy and ulceration
Thrombocytopenia or physical trauma may lead to bleeding
and further mucosal damage.
Neutropenia and poor dental hygiene predisposes the oral
mucosa to secondary infection.
Interventions
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Baseline assessment including the oral
cavity, teeth, and gingival mucosa.
History of dental exam and use of orthodontic
appliances
Meticulous oral care
Mouth rinses
Monitor hydration status
Constipation
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Assess normal bowel habits
Increase fiber and fluids in diet
Stool softeners / colace
Physical activity
Avoid digital manipulation
Diarrhea
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Assess for signs of dehydration
Record stool patterns
IV fluids as needed
Low-residue or lactose-free diet
Good hand washing
Hair Loss
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More important in the older child.
Most patients will experience hair loss within
10 days of induction chemotherapy
Prepare patient for hair loss
Males: shave hair
Females: short hair style – pick out wig
Psychosocial Support
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Support groups
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Open communication
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Daily contact with oncology team
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Trusting relationship with nurse
Growth and Development
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Promote normal G & D
Allow decision making
Establish daily routines
Play therapy
Friends
School attendance or tutor
Leukemia
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Most common malignancy
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4 in 100,000
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Increase in chromosome disorders
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High survival rate
Leukemia
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Unrestricted proliferation of immature WBC’s
in the blood forming tissues of the body.
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The cells look different from normal cells and
do not function properly.
Prognosis
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Initial WBC most significant
The higher the count the poorer the
outcomes
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Greater than 100,000 WBC count = poor outcome
Children under 2 years and older than 10
Girls do better than boys
Diagnosis
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Peripheral blood smear
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Bone marrow analysis
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Lumbar puncture
Peripheral Blood Smear
Red circles or RBC; large blue are WBC; blue dots are platelets
http://www.fghi.com/careers/html/body_smear.html
Bone Marrow
Normal
Acute Lymphoid Leukemia
http://alice.ucdavis.edu/imd/420a/dib/acute/index.htm
Clinical Manifestations
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Pallor and fever
Lethargy
Anorexia
Weight loss
Hemorrhage / petechiae
Hepatomegly / splenomegaly
3 Phase Treatment
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Induction: goal is to achieve remission last
about a month
Consolidation: most intensive phase of
chemotherapy lasts 4 to 8 months
Maintenance: last two to three years
If leukemia cells are detected in bone marrow
process is started all over again.
Induction Therapy
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Goal of therapy is to achieve remission
Leukemia cells are no longer found in the bone
marrow samples, the normal cells return and blood
counts become normal.
Drugs used: L-asparaginase, vincristine and a
steroid (dexamethasone), for high-risk children a
fourth drug (daunorbucin) is often used
Consolidation Phase
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Several drugs are used in combination to
prevent remaining leukemia cells from
developing resistance.
Drugs include: methotrexate and 6mercaptopurine, vincristine and prednisone
Maintenance
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If leukemia continues to be in remission
maintenance therapy can be started.
Two drugs: vincristine and steroids over a
brief period every 4 to 8 weeks.
Duration of total therapy 2 to 3 years.
CNS Therapy
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CNS prophylaxis is initiated at diagnosis and is used
to reduce the risk for CNS disease.
Preventive CNS is based on the premise that the
CNS provides a sanctuary site for leukemic cells
that are undetected at diagnosis and reside
protected from the action of systemic therapy by the
brain blood barrier.
Nursing Interventions
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Assess for infection
Monitor blood values
I & O / nutrition
Complications of chemotherapy
Good hand washing
Aseptic technique for blood draws
Leukemia Time Line
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1962 cure rate for pediatric cancer is 4 %.
1971 – A combination of chemotherapy and cranial
irradiation proves it can cure at least half of all
children with ALL.
1975 – A new combination of chemo drugs helps
patients with reccurrence of the disease.
Time Line
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1991 Long-term survival rate increases to
73% with intensive induction therapy followed
by two years of treatment with eight anti
cancer drugs used on a rotating basis.
Time Line
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1997 – Bone marrow transplants from
unrelated, genetically matched donors are
effective against many childhood leukemia's.
1998 – Study reveals the cure rate for All has
increased from 73% to 80%.
CNS Tumors
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2nd most common malignancy
65% have 5 year survival rate
Most common tumors:
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Astrocytomas 50%
Medulloblastomas 25%
Brain stem gliomas 10%
Clinical Manifestations
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Classic signs and symptoms are indicative of
increased intracranial pressure.
Pressure is due to tumor mass compressing vital
structure, blockage of cerebrospinal fluid flow or
tumor associated edema.
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Gait changes / ataxia
Headache with or without vomiting
Blurred vision, or diplopia
Forceful vomiting upon rising in the morning or
papilledema.
Management
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Surgery if tumor accessible
Chemotherapy
Radiation = Reserved for patient older that 2years of age
Survival rate based on location
Chemotherapy
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After surgery to prevent tumor from coming
back
Shrink tumor that cannot be operated on
Shrink tumor so it can be operated on
Chemotherapy
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Blood brain barrier – natural filter within the body
that allows certain substances through from the
blood to the brain tissues.
Drugs used are: temozolamide, procarbazine or
lomustine
Methotrexate is injected intra-thecal
Implantable wafers: drug is fixed with gel wafer –
drug is slowly released into brain over 2 to 3 weeks
Brain Tumors
Astrocytoma
Large right frontal lobe
neoplasm with small
area of necrosis
Hodgkin's Disease
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3rd most common malignancy
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15 to 30 years
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Three times higher in males
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Excellent cure rates
Clinical Manifestations
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Night sweats
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Weight loss
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Malaise
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Painless, firm nodes
Treatment
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Radiation to nodes
Chemotherapy
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Combination therapy for six months
Prednisone
Stem cell transplant
Long Term Side Effects
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Infertility: drugs can damage ovaries or testicles
Second cancers: small risk for leukemia in future
Heart disease: some drugs can cause heart
problems or radiation to middle of chest
Lung damage: pneumonitis from bleomycin
Neuroblastoma
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Approximately 600 new cases a year.
Embryonic tumor
Average age of diagnosis is 2 years.
Poorest survival rate
50 to 60% have metastases at time of
diagnosis.
Clinical Manifestations
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Depends on site of tumor
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Diagnosis
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CT scan
Bone scan
95% secrete catecholamines in the urine.
Treatment
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Determined by the stage of disease and age
of child.
Children who have localized disease and
complete response to treatment are more
likely to achieve a disease free state and
long-term survival.
Neuroblastoma
Wilm’s Tumor
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Most common type of renal tumor in children
Approximately 460 new cases each year.
Children with hypospadius or cryptorchidism have a
slightly higher incidence.
African American and Females at highest risk
Clinical Manifestations
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Firm non-tender, painless mass in abdomen
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Hematuria
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Hypertension
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Do not palpate the abdomen
CT Scan Wilm’s Tumor
http://www.uhrad.com/pedsarc/peds048.htm
Wilm’s Tumor
Treatment
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Surgery
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Nephrectomy
Prevent rupture of capsule
Sample for pathology
Chemotherapy and radiation are given based
on the stage of the disease.
Osteogenic Sarcoma
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Malignant tumor of bone
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400 new cases each year
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Peak incidence is in the second decade of life, when adolescents
are gaining vertical height rapidly.
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Approximately 20% have metastases at diagnosis
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High rate of metastasis to lungs
Diagnosis
Osteosarcoma Tumor
Femur has a large
mass involving
the metaphysis of
bone. Tumor has
destroyed the cortex.
Anderson’s Pathology
Treatment
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Limb salvage
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Amputation
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Chemotherapy
Limb Salvage
http://www.clevelandclinic.org/ortho/tumor/limbSalvageLg.htm
Ewing Sarcoma
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Tumor of flat bones
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Pelvis, chest, vertebrae
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Rare in children under 5 years
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75% diagnosed by age 20
Ewing Sarcoma
Ewing Sarcoma Tumor
Ewing Sarcoma at
distal end of the tibia.
tumor extends into
the soft tissue.
Anderson’s Pathology
Rhabdomyosarcoma
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Most common soft bone tissue tumor
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Head and neck 40%
GU 20%
Extremities 20%
Trunk 15%
Rhabdomyosarcoma
http://www.brisbio.ac.uk/ROADS/subject-listing/face.html
Treatment
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Surgical removal
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Chemo based on tissue biopsy
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Radiation
Retinoblastoma
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Intraocular / Embryonic tumor
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1 in 16,000
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+ family history
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High incidence of malignancies
Retinoblastoma
Pupil reflex
“Cat Eyes”
http://www.djo.harvard.edu/meei/PI/RB/RB.html
Retinoblastoma
http://homepage.idx.com.au/muznsam/
Retinoblastoma
Treatment
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Surgical enucleation of eye
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Genetic counseling
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Follow-up care up to 18 Years