Transcript Pediatric Malignancies_2010

The Child With a Malignancy
Jan Bazner-Chandler
CPNP,MSN, CNS, RN
Developmental and Biologic Variances
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Most childhood cancers arise for embryonic mesodermal
germ layer
Involves tissues of:
CNS, bone, muscle, endothelial tissue, connective tissue,
blood, lymph tissue
Grows in a short period of time
80% have metastasis at time of diagnosis
Assessment
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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
CBC with absolute neutrophil count
Urinalysis
Lumbar puncture
Urine catecholamines
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
Surgical Management
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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.
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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: Hematopoietic 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
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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
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
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 for 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.
Assessment / 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 – scarf - hat
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
Late Effects of Cancer Therapy
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Endocrine: sterility
Thyroid
Cardiovascular
Musculoskeletal
Vision
Hearing
Respiratory
Gastrointestinal
Genitourinary
Hematopoietic
Leukemia
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Most common malignancy
Cancer of blood or bone marrow characterized by an
abnormal proliferation of blood cells, usually white blood
cells (leukocytes)
Two types
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Acute lymphoblastic leukemia: 78%
Acute Myelogenous leukemia: 15 to 20%
High survival rate
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
Assessment
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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 6-mercaptopurine,
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.
Multidisciplinary 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 reocurrence of the disease.
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 – survival rate 73% to 80%.
2004 – survival rate 85%
2006 – survival rate 94%
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%
Assessment
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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.
Multidisciplinary Interventions
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Surgery if tumor accessible
Chemotherapy
Radiation = Reserved for patient older that 2-years 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
Assessment
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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
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.
Assessment
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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.
Multidisciplinary Interventions
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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
Assessment
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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
Wilm’s Tumor
Multidisciplinary Interventions
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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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Peak incidence between ages 10 and 20 years
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Genetic predisposition
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Approximately 20% have metastases at diagnosis
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High rate of metastasis to lungs
Assessment
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Pain in affected limb that increases with activity or weight
bearing
Refusal to walk and limited range of motion
Tenderness and edema
Diagnosis often made after traumatic injury
Diagnosis
Multidisciplinary Interventions
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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
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%
Prognosis
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Location
Extent of disease
Children with no detectible metastases at time of
diagnosis have better outcomes
Head and neck tumors have better prognosis
72% survival rate after 5 years
Multidisciplinary Interventions
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Biopsy of tumor mass
Imaging studies
Surgical removal
Chemo based on tissue biopsy
Radiation
Retinoblastoma
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Intraocular tumor composed of embryonal retinal cells
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1 in 16,000
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+ family history
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High incidence of malignancies
Assessment
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Child may initially present with strabismus
Impaired vision
Creamy white pupillary reflex
Painful eyes from inflammation
Retinoblastoma
Pupil reflex
“Cat Eyes”
Multidisciplinary Interventions
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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