Study guide Flashcards
ALL stands for?
Acute Lymphoblastic Leukemia
Factors that determine the prognosis of ALL:
1) WBC count
2) age
3) gender
1) > 50K
2) < 2 and > 10 at time of diagnosis
3) female have better prognosis than males
Pathophysiology of ALL
- extreme proliferation of immature lymphocytes called Blast Cells
- cells fill the bone marrow, impairing hematopoiesis
ALL originates in which type of cell?
Blast cells
ALL originates in which type of cells?
B and T lymphoid cells or lymphoblasts
Which type of ALL is more prevalent?
B-lymphoblastic leukemia
S/S of ALL?
- Anemia
- neutropenia
- thrombocytopenia
- extramedullary disease
The systemic spread of ALL outside the bone marrow and blood?
- etramedullary disease
- can present at time of diagnosis or recurrence
What are the most common sites of extramedullary spread?
- CNS, testes, liver, kidneys, and spleen
What are the first diagnostic procedures used to confirm leukemia (AML & ALL)?
CBC, lumbar puncture, bone marrow aspiration and biopsy
What is the first step and goal of treament for children with ALL?
to induce remission with combination chemo
Steps of treatment in leukemia?
1) combination chemo
2) further intensive chemo after remission is achieved
3) maintenance chemo over 2-3 yrs
How long is the maintenance chemo period for leukemia?
2 -3 yrs
Why are males treated for a longer period than females?
concern with testes as a sanctuary site for cancer cells
What are possible sanctuary sites for leukemia?
CSF and testes
Care during chemotherapy includes?
supportive care to prevent acute bleeding and infectious complications
Methods of reducing risks of infection for children during chemo?
wash hands, limit visitors and exposure to germs, prophylactic antibiotics
Clinical S/S of leukemia?
Anemia: fatigue, malaise, pallor Thrombocytopenia: gingival,cutaneous or nasal bleeding Neutropenia: fever, recurrent infections Hepatosplenomegaly, abd pain, N/V Bone pain Lymphadenopathy Weight loss, anorexia,
Gastrointestinal side effects of chemo tx
N/V, diarrhea, constipation, impaired liver function, pancreatitis, anorexia, & electrolyte imbalances
Skin/mucosal side effects of chemo tx
mucositis, stomatitis, rashes, discoloration, increased sensitivity to sunlight, alopecia
Hematologic side effects of chemo tx
anemia, neutropenia, thrombocytopenia
Other side effects of CTX
immunosuppression, pulmonary, renal, neurological, cardiac damage, sterility
Why are children with leukemia diagnosed weeks after appreance of symptoms?
the care givers think they have the flu or other childhood illness
Wilms has a higher incidence in __________ and in familial cases, is an _____________ trait?
1) siblings
2) autosomal dominant
Wilms primary affects which organ?
kidney (nephroblastoma, which is the most common intraabdominal and kidney tumor in children)
Congenital anomalies associated with Wilm’s tumor?
Aniridia, , hemihypertrophy Genitourinary abnormalities, mental retardation
Stage I and V of Wilms Tumor?
Stage I: only 1 kidney involved, can be removed
Stage V: both kidneys affected
Does Wilm’s tumor a rapid or slow growing tumor?
rapid
What is the most common sign of Wilm’s tumor if child is asymptomatic?
non-tender abdominal mass, firm, confined to one side, deep within the flank
S/S of Wilm’s Tumor?
non-tender abdominal mass, abd pain, hematuria, HTN, weight loss, fever
the presence of distended abd veins may indicate what in a child diagnosed with Wilm’s Tumor?
occlusion of the inferion vena cava
Treatment of Wilm’s tumor?
surgical removal even if tumor has spread , followed by chemo
Wilm’s Tumor preop concerns?
do not palpate the abd mass (may cause spread)
Wilm’s Tumor post-op care?
monitor GI activitiy (BS, BM, distention and vomiting)
observe for s/s of infection (esp. during chemo)
A retinoblastoma is composed of which type of cells?
embryonal retinal cells
Most retinoblastomas are detected by what age?
2 y/o
What is the most common cause of death with a retinoblastoma diagnosis?
intracranial spread of the tumor
Where does Retinoblastoma usually develop?
the posterior portion of the retina
Malignancy of a retinoblastoma may appear as a signle tumor in a ___________, or have multiple centers of activity.
rosette formation
How does a retinoblastoma metastasize locally?
usually occurs within the intraocular space, then invades the globes
How does a retinoblastoma metastasize to the body?
tumor cells grow along the optice nerve,enter the subarachnoid space and spread to the CNS
Why does necrosis occur in a retinoblastoma?
the tumor outgrows its blood supply
What is an important diagnostic sign seen in patients with retinoblastomas?
Calcification in the necrotic areas of the tumor
What is the difference in hereditatry vs non-hereditary retinoblastoma?
Hereditary: bilateral, increased risk of other malignancies
Non-hereditary: unilateral, no increased risk of other malignancies
What classification system is used to stage a retinoblastoma and predicts what?
1) Reese-Ellsworth staging classification
2) tumor control and vision preservation
S/S of retinoblastoma (small tumor)?
strabismus secondary to impaired vision
S/S of retinoblastoma (large tumor)?
leukocoria or cat’s eye, tumor may be visualized easily
Late S/S of retinoblastoma?
red, painful eyes, resulting from inflammation, uveitis, or vetreous hemorrhage
a complete diagnosis of retinoblastoma is usually made following which exam?
a complete funduscopic exam
retinoblastomas color?
creamy pink or white
Treatment of retinoblastoma?
surgery, radiotherapy, phototherapy and cryotherapy
enucleation
surgical removal of the eye
a creamy white pupillary reflec caused by an enlarging retinoblastoma?
leukocoria
When does a child get fitted for an ocular prosthesis following enucleation?
3 weeks, unless complications occur
How to clean an ocular prosthesis?
place in water that is slightly warmer than room therapy and soak for several minutes
Why are cancer cells difficult to treat when they are in the G0 phase?
because they are not actively dividing
G1 phase?
- first “gap phase”, lasts 8-48 hrs
- DNA synthesis begins
- active RNA and protein synthesis
What happens during the S phase of cellular reproduction?
- phase where most DNA synthesis occurs (doubles)
- lasts 10-30 hrs
G2 phase?
- RNA and protein synthesis
- lasts 1-12 hrs
Steps of Mitosis?
Prophase, Metaphase, Anaphase, Telophase
Phases of Chemo?
1) induction
2) consolidation
3) maintenance
4) observation
What happens if a child relapses during the maintenance phase of chemo?
the tx protocol is changed and tx begins, starting in the induction phase
Nursing assessments to be done prior to chemo?
- need baseline organ function
- liver
- kidney: creatinine clearance
- Heart: ECG
- Lungs: pulmonary function test
- Neuro: deep tendon and sensory
Sigs of chemo toxicity?
Increased creatinine levels, presence of proteins, or hematuria, changes from baseline organ function
What are the problems manifested in the Tetralog of Fallot?
1) large ventricular septal defect
2) right ventricular outflow obstruction or pulmonary stenosis
3) an overriding aorta (dextroposition of the aorta)
4) right ventricular hypertrophy
Signs of anemia
malaise, fatigue, pallor
signs of thrombocytopenia
gingival, cutaneous, or nasal bleeding
signs of neutropenia
fever, recurring infections
CNS manifestations of leukemia?
Increase ICP, HA, vomiting & visual disturbances
Non-physical signs of abuse in children?
- extreme efforts to please
- withdrawal
- anxiety,
- are wary of adults
- cringing with sudden movements
- apathy
- excessive daydreaming
- depression,
- not turning to the parent for support,
- poor hygiene and unusual dress
Common sites of intentional injury in children?
- nose, groin, upper thigh, upper back, butt, back of knees and upper legs
Common sites of unintentional injury in children?
Forehead, knees, shins, elbows
Suspicious bruising on children?
- patterns (iron, shoe)
- bruises in different stages of healing
- on the trunk and ear, genitals
- bruises don’t match the hx
- bruising on an infant
Normal pathological conditions that mimic abuse in children?
- Henoch-Schonlein Purpura (face, neck, upper chest)
- Nevus Simplex (perm blanching at base of neck)
- Mongolian Spots (asian/black, perm discoloration)
- Bullous Impetigo (look like cigaretter burn)
- Epidermolysis Bollusa
Water temp should be set to?
120 deg F
What to assess for if bilateral black eyes are present?
- Consider basilar skull fracture
- Neuroblastoma
- Injury to forehead
- Look for injury to surrounding tissue
Signs of forced submersion burns?
uniform demarcation
What is the most common location for inflicted burns in children?
dorsal aspect of the hand
Signs of an accidental burn?
irregular, varying degrees of severity, splash marks
Short falls (< 3ft) may cause what in children?
bruising, swelling, small skull fracture, SMALL subdural bleed
Death trajectories?
- Sudden, unexpected death
- Death from potentially curable disease (ALL)
- Lethal congenital anomaly (anencephaly)
- Death from progressive conditions with intermittent crises (muscular dystrophy, get pneumonia, never recover)
What does the acronym CHILD stand for in relation to child end of life treatment?
Consider Honesty Include Listen Do (repeat)
Term for the mental work following a loss? (e.g. death of loved one)
bereavement
What are the stages of Grief?
Denial Anger Bargaining Depression Acceptance
Ductus venosus
shunts oxygenated blood from umbilical vein to inferior vena cava
Foramen ovale
shunts oxygenated blood from R. atrium to L. atrium
What is Ductus arteriosus?
shunts blood from pulmonary artery to aorta
Infant presents with “machinery” type murmur upon auscultation, what do you suspect?
PDA
Child presents with activity intolerance that has gotten worse as he has aged. What do you suspect?
ASD
Infant presents with a systolic murmur along the left sternal border upon auscultation, what do you suspect?
VSD
Amplatzer Duct occludor is used to treat?
PDA