NU 428 Pediatric Oncology

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1

What are the characteristics of leukemia?

LEUKEMIA

  • Most common type of childhood cancer. Leukemia: cancer of the blood and blood forming tissues. What are blood forming tissues? Bone marrow & lymphatics.
  • Two types: Acute Lymphoblastic Leukemia – ALL (other synonyms on pg 1380) and Acute Myelogenous Leukemia – AML or also known as Acute nonlymphoid Leukemia – ANLL
  • ALL is the most common cancer found in children.
  • Peak incidence is age 2-5 years.
  • Most common in white young males.
2

What are the characteristics of acute lymphocytic leukemia?

  • Results from over production of immature lymphocytes
  • Lymphocytes are a type of white blood cell.
  • These immature cells overcrowd the bone marrow – which prevents marrow from producing healthy WBC, RBC, and platelets. These immature cells also compete for nutrients w/ the healthy cells.
  • Blasts: immature cells.
3

What are the symptoms of ALL?

You often see pancytopenia (everything is decreased): neutropenia, anemia, thrombocytopenia – all a result of bone marrow dysfunction.

Symptoms usually appear1-6 weeks before dx.

Most common presentation: fever, bone pain, and pallor.

If WBCs are decreased, you will see: infection and fever.

If RBCs are decreased, you will see: fatigue, SOB, pallor, anemia sx.

If platelets are decreased, you will see: bruising, abnormal bleeding, petechia (any little strain makes capillaries rupture

4

What symptoms correlate with specific organ involvement in ALL?

CNS: you will see cranial nerve sx like facial weakness

Liver, spleen, lymph nodes: you will see enlargement of organ affected.

Bone marrow: you will see bone pain and easy spontaneous fractures.

5

How is ALL diagnosed?

History: Be a good interviewer, ask a lot of questions. What s/s are present that parents are concerned about?

Physical Exam: See sx and labs

Peripheral Smear: If CBC is out of whack. Look at a smear of blood. Immature lymphocytes will be present if they have leukemia. Don’t want to see that.

Bone Marrow Biopsy: Further evaluation. How you really make a definitive diagnosis. Has to show greater than 25% blasts (immature cells).

Lumbar puncture: Just to see if there’s CNS involvement.

6

What the management recommendations for ALL?

Determined by typing and staging; there are 4 phases to treatment in ALL.

Typing determines the course of therapy; staging determines the prognosis.

4 phases of treatment:

  • Induction phase: 4-6 wks – goal: complete remission or less than 5% blasts. Remission is defined as absence of clinical sx and the presence of less than 5% blast cells in the bone marrow.
  • CNS prophylactic therapy: intrathecal meds – given to help prevent CNS involvement- helps to prevent leukemic cells from invading the tissues.
  • Intensification therapy (consolidation phase): gives high doses of chemo - eradicates residual leukemic cells.
  • Maintenance Phase: goal is to preserve remission and further reduce the number of leukemic cells – weekly or monthly blood counts done.
    • Remember complete remission is: less than 5% blast cells and absence of sx.

Relapse: when leukemic cell reappear in the bone marrow – most commonly occurs during first year off therapy.

7

What are the characteristics and risk factors of acute myelogenous leukemia?

Less common than ALL.

Worse prognosis than ALL.

AML affects a group of WBC’s called myeloid cells.

Risk Factors: 1) genetic predisposition (specific marker for AML), 2) Down’s Syndrome, 3) previous exposure to chemo or radiation for another type of cancer.

With AML, malignant myeloid blast cells crowd out normal WBCs, RBCs, and platelets.

8

What are the symptoms of AML? How is it diagnosed?

Symptoms

  • Flu like symptoms or severe symptoms
  • Bleeding associated with thrombocytopenia.
  • Gingival hypertrophy due to infiltration of the gums.
  • Chloromas: localized collections of malignant cells in the skin or SQ tissue.
  • You may see signs of increased ICP – AML does have higher incidence of CNS involvement.
  • May also see enlargement of liver and spleen (hepato/splenomegaly).

Diagnosis

  • Again, bone marrow aspirate is key to dx – must show > greater than 25% malignant myeloid blasts.
  • All other diagnostics (labs, hx, PE, etc) are same as ALL.
9

What is the treatment for AML?

Similar to ALL; typically involves systemic chemo.

Hemopoietic stem cell transplantation – those receiving stem cell transplant have best prognosis. Same thing as a bone marrow transplant. Terms used interchangeably.

Overall prognosis: long term survival is <50%.

10

What are the characteristics of a bone marrow transplant as treatment for leukemia?

More common for AML.

Used in ALL after second relapse. Not generally used with ALL during the first remission bc of excellent results with chemotherapy.

It is the replacement of a person’s hemopoietic stem cells after his own bone marrow has been destroyed. Also referred to as hemopoietic stem cell transplant.

Three types

  • Allogenic: histo-compatible donor: a suitable donor is one whose tissue closely matches the tissue of the client. Genetic markers on the surface of the WBC called leukocyte antigens (HLA) define a person’s tissue type. HLA markers are inherited, so siblings are more likely to be compatible donors.
  • Autologous: The pt’s own bone marrow is collected from disease free tissue, then frozen and treated to remove any malignant cells. Then it is given back once the diseased bone marrow has been destroyed.
  • Umbilical cord blood

3 phases of HSCT:

  • Pre-transplant phase: involves eval and testing of child and donor.
  • Transplant Phase: large doses of chemo and radiation given to rid the body of the cancer cells and suppress the child’s immune system to prevent rejection of transplanted cells. Transplanted cells are given via IV.
  • Post-Transplant phase: you will see pancytopenia secondary to BM suppression or myelosupression. What is pancytopenia? Decreased WBC, RBC, platelets.
  • Possible complication is graft vs host: this is an immune response to the presence of foreign HLA. Donor cells attack the child’s cells. Organs affected include: Skin, GI tract, and liver. Will see severe rash, diarrhea, jaundice, and infection. Can be life threatening.
11

What are the characteristics of lymphoma?

A neoplastic disease of the immune system cells - arises from the lymphoid and hemopoietic systems.

2 Types: Hodgkin’s L and Non-HL

In HL, the malignant cell is more defined than NHL and the pattern of infiltration is more specific.

NHL is more prevalent in children younger than 14. HL is more prevalent in adolescents and young adults ages 15-19.

12

What are the characteristics of Hodgkin's Lymphoma?

Mostly adolescent onset

Originates in lymphoid tissue.

Metastasizes to spleen, liver, bone marrow, lungs, and mediastinum.

Staging – accurate staging is basis for treatment; staging determines extent of disease; stages 1-4 w/4 most severe.

Ann Arbor staging system – assigns a stage based on the number of sites of 1) lymph node involvement, 2) presence of extra-nodal disease, and the 3) hx of any sx. Classified as “A” is asymptomatic and “B” is symptomatic.

13

What are the symptoms of HL?

Painless enlargement of lymph node(s).

Most common presentation: sentinel node- a lymph node that is enlarged, firm, non-tender, moveable in the supraclavicular (above the clavicle) or cervical (neck) area. This common presentation would be classified as “A” using the Ann Arbor system bc there are no other s/s.

“B” Symptoms are associated with a poorer prognosis. These include: fever, anorexia, n/v, weight loss, night sweats, pruritis.

14

How is HL diagnosed?

H&P: document any s/s including fever, night sweats, enlarged liver/spleen, enlarged lymph node(s).

CT and xray – often CT of neck, chest, abdomen, and pelvis

Blood tests

Lymph node biopsy is essential to establish histologic diagnosis and staging. *Reed Sternberg cell is characteristic of HL.

They may also have bone marrow biopsy.

15

How is HL treated?

Depends on staging

Radiation commonly used; Multi-agent chemo is another treatment.

Goal: cure with minimal toxicities. You may see chemo given before radiation to reduce bulky disease so lower levels of radiation can be used.

HL is associated with secondary malignancies: leukemia, NHL, lung, breast, GI, and thyroid.

If relapse, may require bone marrow transplant.

16

What nursing considerations should a nurse be aware of when caring for a patient with HL?

Remember that this is commonly a disease of adolescent clients when looking only a ped. onc. Adolescents appreciate psychological needs.

May have a high risk for infertility from chemo and radiation.

Appearance of secondary sexual characteristics and menstruation may be delayed. Especially distressful for adolescent clients. This can be stressful for the child.

17

What are the characteristics of NHL?

Usually diffuse (no single focus area); cell type poorly differentiated; dissemination (spread) more rapidly.

CNS and mediastinal involvement common.

Rapid onset

Most common in white males.

18

What are the symptoms of NHL? How is it diagnosed?

Symptoms:

Pain or swelling with acute onset and progression.

May be similar to HL.

If metastasis to bone marrow, will see s/s of leukemia.

Diagnosis:

Surgical biopsy

Burkitt’s lymphoma: usually involves jaw and abdomen.

19

What is the treatment and prognosis for NHL?

Radiation and Chemo – 4 phases like leukemia

Aggressive multi-agent chemo is used ASAP

Prognosis is good with children with localized disease.

Relapse after 2 yrs is rare.

20

What are the nursing considerations a nurse should be aware of when caring for a patient with NHL?

Tumor lysis syndrome: a life threatening condition – a complication that may be seen after initiation of cancer treatment. Rapid release of purines from destroyed lymphoblasts cause increase in uric acid production which can lead to acute renal failure.

Interventions: strict I&O, daily weights, liberal fluids, dialysis if ordered, focus on hydration, administration of allopurinol. Allopurinol: reduces uric acid synthesis and decreased hyperuricemia – frequently used in gout.

21

What are the characteristics of brain tumors?

Tumors of the CNS. Classified according to the cell type and location. May be benign or malignant. Astrocytomas are the most common glial tumor. Glial cells: supporting structures of the brain.

22

What are the symptoms of brain tumors?

Largely related to location, size of tumor, and age of child

Headache: especially worrisome if upon awakening.

Vomiting: Want to know if its random, not associated with any illness. Bad if it’s not related to eating

Neuromuscular changes: loss of balance, falling more than normal

Behavioral changes: irritability, moodiness, decreased appetite

Cranial nerve neuropathy: ex. Visual defect or any other sx that involve cranial nerves.

VS disturbances: dec. pulse and RR; inc. BP; temp. change

Other symptoms: seizures, increased head circumference, bulging fontanel, papilledema: which is edema of the optic nerve

23

How are brain tumors diagnosed? How are they treated?

Diagnosis:

  • Hx and physical
  • Head CT w or w/o contrast
  • MRI
  • Definitive dx: tissue biopsy

Treatment

  • Radiation and chemo to shrink tumor
  • May have surgery to remove – often see them try to remove as much of the tumor as possible while still preserving brain function.
  • IV steroids: reduce brain edema
  • Radiation to the brain is avoided in children under age 3.
24

What is a neuroblastoma? Where are common sites of origin?

Tumor that originates from embryonic neural crest cell. Most common malignant tumor of infancy.

Primary site: abdomen especially in the adrenal glands. Other sites include brain and mediastinum.

25

What are the symptoms of a neuroblastoma?

Depends on location and stage; unfortunately dx is usually made after metastasis has occurred bc no obvious s/s at first.

If in abdomen: firm non-tender irregular mass that crosses the midline

26

How are neuroblastomas diagnosed and what is their prognosis?

Diagnosis

  • Hx and physical: goal is to locate the primary site and areas of metastasis. May see periorbital ecchymosis or bluish cutaneous nodules in infants.
  • Tumor biopsy: will see neuroblasts in the tissue
  • CT: location of solid tumor
  • May see bone scan or Bone marrow aspiration performed.

Prognosis: Depends on age of child and stage of disease when diagnosed. Best prognosis if child is <1. Prognosis usually poor bc metastasis often occurs before dx made.

27

What is osetogenic sarcoma? Where are common sites? What are the risk factors?

Most common bone cancer in children. Peak incidence: 10-25 yrs, but may be seen at any age

Primary tumor sites: femur, end of any long bone (humerus, tibia, fibula)

Risk factors: Being a teenager, being a tall male, prior chemo and radiation treatment.

28

What are the symptoms of osteogenic sarcoma?

Most commonly localized pain in affected site: dull aching and constant.

Limping

Swelling

Palpable mass

Fracture

29

How is osteogenic sarcoma diagnosed and treated?

Diagnosis:

  • H&P: helps rule out other causes like trauma or infection
  • Plain xray and CT or MRI usually done
  • Biopsy to confirm dx.
  • CXR bc lung metastasis common.

Treatment:

  • Likely see surgery: Biopsy followed by limb salvage or amputation depending on size/location
  • Chemotherapy: chemo before and after surgery to reduce tumor size and decrease micro-metastatic disease
  • OS is radiant resistant: typically only see chemo or surgery
  • Limb salvage: take out tumor and replace bone with cadaver bone.
  • May see phantom limb pain: sensation such as tingling, itching, pain, burning, etc in amputated limb. Medicate it like real pain
30

What is Ewing sarcoma? Where are the most common sites?

Forms in the marrow spaces of the bone. Not the bone itself.

Most common sites: Long bone, may see in trunk bones. If it’s in the trunk, it may be harder to diagnose.

31

What are the symptoms of Ewing sarcoma? How is it treated?

Symptoms: intermittent pain which can lead to delay in dx. May have palpable mass or swelling but this may not be evident if centrally located.

Treatment:

  • Radiation and Chemo
  • Amputation not routinely recommended bc it is in the marrow, not the bone itself.
  • Long term follow up is crucial bc high rate of secondary metastasis.
32

What are Wilm's tumors?

Also known as nephroblastoma.

Often associated with undescended testes, hypospadias (urethral opening is low), and other genitourinary anomalies.

Most common renal & intra-abdominal tumor in childhood. Often diagnosed before age 5.

33

What are the symptoms of Wilm's tumors? How are they treated? What nursing considerations must be taken into account when caring for a patient with a Wilm's tumor?

Symptoms

  • Most Common symptom: swelling or a mass that’s firm and non-tender and contained to one side of the abdomen.
  • Other symptoms: hematuria, anemia, weight loss, fever, hypertension.

Treatment: based on staging

  • Surgery is preferred – removal of kidney if only one sided (only 10% of cases involve both kidneys)
  • If both kidneys involved, often use radiation to shrink tumor.

Nursing Considerations: DO NOT manipulate the abdomen of child with Wilm’s tumor. The tumor is encapsulated – great care is taken to keep the encapsulated tumor intact - rupture can seed cancer cells throughout the abdomen, lymph channel, and blood stream. AVOID trauma to remaining kidney.

34

What is a retinoblastoma? What are the symptoms? How is it treated?

Intraocular malignancy. Strong hereditary component.

Symptoms

  • Most common: red, painful eye
  • Strabismus is 2nd most common
  • Blindness or decreased vision
  • Red/painful eye, cat’s eye reflex: don’t get normal red reflex when looking with an ophthalmoscope. Might look white in a picture.

Treatment: Depends on staging – good prognosis – 90% 10 year survival rate.

  • Goal is to preserve useful vision in affected eye and eradicate tumor.
  • May use radiation or plaque brachytherapy, photocoagulation, lasers/cryotherapy to destroy blood vessels that feed tumor, or some spontaneously regress.
35

What are some general nursing care essentials when caring for the child with cancer?

Relieve pain

  • Assess pain w/ each VS and more often if needed.
  • Opioids are adjusted for the child’s needs and administered around the clock for optimal pain relief.
  • Non-pharmacological measures should be implemented as needed but not substituted for pain medication.

Prepare child and family for procedures and treatments: Answer questions and be as honest as possible.

Prevent complications from myelosuppression. What is myelosuppression? Bone marrow suppression, low counts.

What is it caused by? Leukemia or chemo.

Prevent infection: private room, restrict visitors, strict hand washing, strict aseptic technique on anything that’s supposed to be sterile, monitor VS especially temperature, provide good nutrition, DO NOT give live virus vaccines, DO give inactivated, not live vaccines, assess for s/s of infection, administer antibiotics as ordered, give granulocyte colony-stimulating factor (G-CSF) as ordered. What vaccines should be avoided? Nasal flu (Flumist), MMR, Varicella, oral rotavirus vaccine. Nobody in the house gets a live virus vaccine.

Prevent bleeding episodes: provide meticulous mouth care. Avoid rectal temps to prevent trauma. Avoid skin puncture if possible (ex. Im injections if can be given through existing IV).

36

What are some hemopoietic side effects of chemo a nurse should prepare for? What interventions are indicated for these side effects?

Hemopoietic SE: myelosuppression, anemia, neutropenia, thrombocytopenia, and immunosuppression

Interventions:

Assess for s/s of: anemia, infection, and bleeding

NO rectal temps or suppositories if possible.

Hold pressure for 10 min over injection sites.

Stress injury prevention, especially if platelets are low.

Do not give NSAIDS or ASA if possible.

Strict hand washing due to the neutropenia.

Administer antibiotics within 30min of scheduled time if possible.

Septic work up for all children with fever and neutropenia. Avoid invasive procedures when possible.

NO live virus vaccines

37

What are some GI side effects of chemo a nurse should prepare for? What interventions are indicated for these side effects?

GI SE: mucositis/stomatitis, N/V

  • Mucositis: inflammation anywhere along the GI tract. Stomatitis: another word used often interchangeably referring to inflammation and ulcerations in mouth.

Interventions:

N/V: give antiemetics 30 min to 1 hr prior to chemo or as ordered; may also need PRN as ordered (or regular scheduled).

Monitor liver function tests due to possibility of liver damage.

Diet: Soft, bland, and moist.

Assess for S/S of dehydration.

Good oral care is VIP! Use swabs or toothettes if platelet count low.

Sitz baths and occlusive ointments for rectal ulcers.

38

What are some renal side effects of chemo a nurse should prepare for? What interventions are indicated for these side effects?

Renal SE: chemo is renal toxic

Interventions:

Monitor BUN and creatinine.

Hemorrhagic cystitis: chemical irritation of the bladder from chemo and radiation. Can occur months after treatment so need routine UA.

Ensure liberal oral or IV fluids. Encourage frequent voiding.

Monitor I & O.

39

What are some integumentary side effects of chemo a nurse should prepare for?

Integumentary SE:

  • Alopecia: can be a result of some types of chemo as well as cranial radiation. Hair loss can be permanent but regrowth should begin in 3-6 mo after treatment.
  • Chemo is a vesicant (eats tissue if it infiltrates through peripheral IV and should be used with caution especially in peripheral IVs.
  • Steroids: often required – may develop cushingoid appearance – altered body image.
40

What are some side effects of radiation a nurse should prepare for?

Malaise: most common SE

Blood dyscrasia from radiation to long bones

Xerostomia: dryness of the mouth from dec. saliva production.

Skin changes: Dryness, redness, pruritis

Radiation pneumonitis: acute reaction – swelling and sloughing of endothelial cells of small vessels in lungs. Leads to respiratory distress!

Somnolence syndrome: 5-8 wks after CNS radiation – somnolence, fever, anorexia, N/V, HA.

Alopecia from cranial radiation may be permanent.

41

What are some long-term effects from radiation and chemo?

Radiation: Secondary malignancies most common. Children who receive cranial radiation at 5 or younger are more susceptible to developing brain tumors. Radiation can cause growth retardation and cognitive impairment especially in child under 5. Radiation to chest can cause heart damage. Radiation to neck can cause hypothyroidism.

Chemotherapy: Can cause sterility – may harvest eggs/sperm if able prior to treatment. Certain meds can cause heart damage. Cranial and intrathecal chemo can also be associated with cognitive and neuropsychologic deficits.