Kristine Ruggiero CPNP, MSN, RN

1. Kristine Ruggiero CPNP, MSN, RN Chapter 29 Alterations in Cellular Growth

2. Differences between Pediatric and Adult presentation of Cancer • Incidence of Cancer in Childhood: • Approximately 9,000 children diagnosed in United States in 2003 • Cancer is the leading cause of death for children under age 15 • In 2003, about 1,500 children died of cancer, 1/3rd from leukemia • Types of tumors vary by age and affect survival rate

3. FIGURE 29–1Percentage of primary tumors by site of origin for different age groups. Notice that in the early years of life, in addition to leukemia, cancers that derive from embryonic cells such as sympathetic nervous system (neuroblastoma) and eye (retinoblastoma) are common. As the child grows, lymphoma becomes more common in school years, and germ cell cancers of ovary and testes emerge as more common causes in teens. Jane W. Ball and Ruth C. BindlerChild Health Nursing: Partnering with Children & Families © 2006 by Pearson Education, Inc.Upper Saddle River, New Jersey 07458All rights reserved.

4. Anatomy and Physiology of Pediatric Differences • Immune system more immature in children • This affects how well body can defend itself • Nonspecific and specific cellular responses are immature in infants and premies • Children are still growing and developing • As a result, some cancers grow and progress more rapidly • Apoptosis (programmed-cell death) not well-developed in young children • Children more commonly present with metastases at time of dx than adults d/t the difficulty in recognizing s/sx of cancer (usually mistaken for childhood illness)

5. Differences between adults and children with cancer • Childhood cancers respond better to chemotherapy • Children tolerate chemotherapy better than adults • Childhood survivors of cancer need to be monitored for late-effects and long-term side effects of cancer treatment • “Late-effects” clinics

6. Etiology and Pathophysiology • Alterations in cellular growth occur in response to external and internal stimuli • Neoplasms are caused by one or a combination of three factors; • External stimuli that cause genetic mutations • Innate immune system and gene abnormalities • Chromosomal abnormalities

7. FIGURE 29–2 A protooncogene normally regulates cellular growth and development. When altered by a virus or other external cause, it can change to an oncogene, which allows unregulated genetic activity and tumor growth. Tumor-suppressor genes regulate the effects of oncogenes to decrease wildly proliferating cellular growth. Jane W. Ball and Ruth C. BindlerChild Health Nursing: Partnering with Children & Families © 2006 by Pearson Education, Inc.Upper Saddle River, New Jersey 07458All rights reserved.

8. Clinical Manifestations • Vary by type and location • Pain • Cachexia • (syndrome characterized by anorexia, weight loss, anemia, asthenia (weakness) and early satiety) • Anemia • Infections • Bruising • Neurologic symptoms • Palpable mass

9. CBC Bone marrow aspiration Bone marrow biopsy Lumbar puncture CXR Radiographic examination MRI CTscan US Biopsy of Tumor (See Table 29-2) Diagnostic Tests

10. Important blood work • CBC w/ diff • Hgb and Hct • RBC incices • Mean corpuscular volume (MCV), mean corpuscular hgb concentration (MCHC) and mean corpusuclar hgb (MCH) • WBC indices • Basophils, eosinophils, monocytes, lymphocytes, neutrophils (segmented and banded) • ANC • (neutrophils x WBC count)= infection-fighting capacity (ANC > 1,000 = nml; ANC< 500= risk for infection) • Chem 7 • Others: BUN/ creat, Liver function tests, tumor markers

11. Clinical Therapy • Child managed by Pediatric Oncology Team • Therapy may be singular or combination • Surgery • Chemotherapy (protocol-action plan for chemo) • Radiation • Biotherapy (antibodies developed to target tumor cells for apoptosis; cancer vaccines) • HSCT (hematopoietic stem cell transplant) • Complementary therapies • Palliative care (presence of palliative care team)

12. FIGURE 29–4Chemotherapy drugs either act at specific parts of the cell cycle or are nonspecific for action (act throughout all cell phases). Jane W. Ball and Ruth C. BindlerChild Health Nursing: Partnering with Children & Families © 2006 by Pearson Education, Inc.Upper Saddle River, New Jersey 07458All rights reserved.

13. FIGURE 29–5Chemotherapy protocol. A protocol is a map or plan of action that directs therapy by identifying the drug and its accompanying treatment. Jane W. Ball and Ruth C. BindlerChild Health Nursing: Partnering with Children & Families © 2006 by Pearson Education, Inc.Upper Saddle River, New Jersey 07458All rights reserved.

14. Chemotherapy Side Effects • Nausea/ Vomiting • Alopecia • Malaise • Bone Marrow Depression • Infection • Bleeding • Anemia • Stomatitis

15. Nursing Care Plan • Based on type of cancer and therapy • Infection control • Pain • Nutrition • Growth and Development • Emotional • Spiritual

16. Three Types of Oncological Emergencies • Oncologic emergencies result from the cancer itself or as a side effect of treatment. • Most common emergencies are tumor lysis syndrome, septic shock, brain herniation, spinal cord compression, and superior vena cava compression. • 3 Types of Oncological Emergencies: • Metabolic • Hematologic • Space-occupying lesions

17. Types of Oncological Emergencies • Metabolic • Tumor lysis syndrome: • Metabolic emergency results from lysis of tumor cells. This cell destruction releases high levels of uric acid, K+, and phosphates into the blood. Low levels of Na and Ca occur and metabolic acidosis results. • Most commonly seen in Burkit’s lymphoma and ALL • Tx: see table on pg 1049

18. Types of Oncologic Emergencies • Metabolic • Septic Shock • During period of immune suppression the child is vulnerable to overwhelming infection, resulting in circulatory failure, hypo/hyperthermia, tachypnea, mental changes, inadequate tissue perfusion, and hypotension • Hypercalcemia • Elevated calcium in serum. • Occurs when large amounts of bone are destroyed by treatment • Most common in children w/ ALL and rhabdomyosarcoma • Tx includes hydration and adequate intake of oral phosphate

19. Types of Oncologic Emergencies • Hematologic: • Results from bone marrow suppression or infiltration of brain and respiratory tissue w/ high numbers of leukemic blast cells (hyperleukocytosis) • Bone marrow suppression results in anemia and thrombocytopenia • This leads to coagulation problems and hemorrhage.

20. Types of Oncologic Emergencies • Space-occupying lesions • Tumors w/ extensive growth that may result in life-threatening situations (ie spinal cord compression, increased intracranial pressure, brain herniation, respiratory complications, etc.)

21. Nursing Management for a child with Cancer • Nursing interventions focus on preventive teaching for all families about risk factors for cancer • Health promotion and health maintenance of the child undergoing cancer treatment • Carrying out treatment interventions • Managing health problems r/t both cancer and the side effects of tx • Partnering w/ families to manage the challenging psychosocial needs that emerge when cancer is diagnosed.

22. FIGURE 29–11 A vascular access device allows chemotherapeutic agents to be administered without the need for repeated “sticks” to the child. Jane W. Ball and Ruth C. BindlerChild Health Nursing: Partnering with Children & Families © 2006 by Pearson Education, Inc.Upper Saddle River, New Jersey 07458All rights reserved.

23. FIGURE 29–6 (continued)A, The child with cancer depends on parents and family members to provide support. B, A special relationship often develops between the nurse and the child receiving treatment. B Jane W. Ball and Ruth C. BindlerChild Health Nursing: Partnering with Children & Families © 2006 by Pearson Education, Inc.Upper Saddle River, New Jersey 07458All rights reserved.

24. Nursing Assessment and Diagnosis • Obtain a thorough history. Including: • Family hx of cancer • Hx of exposure to known carcinogens • Does parent work w/ chemicals/ asbestos • Was child tx’d w/ radiation/ chemo for cancer previously • Does the child have any known conditions such as Down’s syndrome • Any congenital anomalies

25. Nursing Assessment • Physiologic Assessment • Includes possible s/sx of cancer or thorough physical assessment if cancer already identified • Psychosocial Assessment • Stress and coping • Knowledge • Support systems • Body Image

26. FIGURE 29–7 One of the most common threats to a child’s body image at any age is hair loss induced by chemotherapy. Use of hats can improve self-concept. Jane W. Ball and Ruth C. BindlerChild Health Nursing: Partnering with Children & Families © 2006 by Pearson Education, Inc.Upper Saddle River, New Jersey 07458All rights reserved.

27. Nursing Assessment • Developmental Assessment • Children under 6 should be regularly screened for developmental surveillance • If changes in development are noted, or regression in milestones occurs during tx, refer to specialist • Assessment for Impact of Cancer Survival • 1 in 1,000 young adults is survivor of childhood cancer • Ongoing care is essential: long-term follow-up clinics • Help families manage long-term effects of cancer tx

28. Nursing Care of the hospitalized child with Cancer • (Review Nursing care plans on pp 1057-1061) • Planning and Implementation: • Ensure optimal nutritional intake • 30% of children w/ cancer are malnourished • High metabolic rate of cancer depletes nutritional stores • 24 hour dietary recall to assess nutritional intake • Antiemetic drugs to decrease N/V from chemo • w/ parenteral nutrition, weekly labs should be performed including: chem 7, liver function studies and glucose • Oral hygiene

29. Nursing care of the hospitalized child w/ cancer • Planning and Implementation: • Administer Medication • Most chemo drugs calculated based on child’s weight (dose/meter squared) • Polypharmacy • Manage Treatment side effects • Myelosuppression (suppression of blood cell prod) • Neutropenia (ANC < 500) • Broad spectrum abx, G-CSF, isolation

30. Nursing Care of the Hospitalized Child with Cancer • Planning and Implementation: • Ensure adequate hydration • Strict I’s and O’s • Adequate hydration • IVFs • Prevent and treat infection • Ensure standard precautions • Proper care of central lines • Teach parents infection control • Immunizations utd

31. Nursing Care of the Hospitalized Child with Cancer • Planning and Implementation: • Manage Pain *** • From disease and medical interventions • Incorporate Pain management team when possible • Conscious sedation for some procedures (ie LPs) • Topical anesthetics (EMLA creams) for IV access • Group painful procedures during sedation when possible (ie LP and Port access) • Include parents/family, when possible to comfort child

32. FIGURE 29–10 A child in a pediatric oncology clinic giving injections to a doll. This type of play therapy helps the child deal with fear, thus lowering his or her stress level. Jane W. Ball and Ruth C. BindlerChild Health Nursing: Partnering with Children & Families © 2006 by Pearson Education, Inc.Upper Saddle River, New Jersey 07458All rights reserved.

33. Nursing care of the Hospitalized Child with Cancer • Planning and Implementation: • Provide Psychosocial Support • To child and family • Care in the Community • Assist family in preparation for home therapy • Health Promotion/ Health Maintenance • Treatment is often a long process (2-3 years) • Still important to have health maintenance visits during this time

34. Leukemia • Most commonly diagnosed malignancy in children under 14 • Cancer of the blood or bone marrow, characterized by an abnormal proliferation of blood cells, usually wbc’s.

35. Childhood Leukemia • Acute Lymphocytic Leukemia (ALL) 75% of all leukemias in children • Peak age of onset is 2-4 years • 90-95% of children w/ ALL will achieve a 1st remission • Almost 80% will live 5 years • Rapid proliferation of immature blood cells which makes the bone marrow unable to produce healthy blood cells • Acute nonlymphocytic (ANLL) or Acute Myelomonocytic leukemia • Most common in children under 2 y.o and adolescents • 17% of all leukemias; 50-70% of adolescents achieve a 1st remission • 40% live 5 years

36. Leukemia • The cause… • Exact cause of most leukemias remains unknown • Increasing evidence suggests a combination of contributing factors • Predisposing factors • Familial tendency • Monozygotic twin w/ leukemia • Congenital disorders, such as Down’s, AT • Viruses • Ionizing radiation • Exposure to the chemical benzene and cytotoxins such as alkylating agents

37. Pathophysiology of Leukemia • WBCs are produced so rapidly that immature cells (blast cells) are released into circulation • Blast cells multiply continuously w/o respect to the body’s needs • Blast cells may be as high as 95% in the bone marrow (nml= <5%) • Increased proliferation of WBCs robs healthy cells of nutrition • Bone marrow undergoes hypertrophy (can lead to fxs) • Bone marrow then undergoes atrophy, leading to anemia, bleeding disorders, and immunosuppression

38. Leukemia • Clinical Manifestations: • High fever • Thrombocytopenia • Abdominal or bone pain • Pallor, chills, and recurrent infections • Petechiae and ecchymosis • Abnormal bleeding, (nosebleeds, wounds, and oral lesions • Confusion, lethargy, and HA if the blood-brain barrier has been crossed • Fatigue • Painless lumps in neck, underarm, stomach and groin

39. Diagnostic Test findings in Leukemia • Bone marrow biopsy confirms dx: • aspirate showing a proliferation of immature WBCs (>25% blasts) • Blood counts show thrombocytopenia, neutropenia, and anemia • Differential leukocyte count determines cell type • LP detects meningeal involvement (if CNS involved) • Cytogenetic analysis shows a “Philadelphia chromosome” in ALL

40. Treatment of Leukemia • Systemic chemo aims to eradicate leukemic cells and induce remission • Intrathecal chemo to prevent or tx CNS infiltration • Abx, antifungal and antiviral drugs and granulocyte injections to control infection • Transfusions of plts to prevent bleeding and of RBCs to prevent anemia • Bone marrow transplant

41. Nursing Interventions • Prevent infection (private room, oral hygiene, screen visitors for infections) • Inspect skin frequently (avoid rectal meds, temps) • Give increased fluids to flush chemo through kidneys • Provide pain relief • Monitor CNS for involvement • Group nursing interventions • Help child adjust to changes in body image

42. Soft Tissue Tumors • Hodgkin’s Disease • Disorder of the lymphoid system • Usually arises from a single or group of lymph nodes • Incidence: 3/100,000; peaks in adolescent boys • Clinical Manifestations include: main sx= a nontender, firm lymphadenopathy, fever, night sweats, weight loss • Prognosis excellent: Tx based on staging: 5 year survival 90%

43. FIGURE 29–15Lymph nodes and organs affected in Hodgkin disease in children. Jane W. Ball and Ruth C. BindlerChild Health Nursing: Partnering with Children & Families © 2006 by Pearson Education, Inc.Upper Saddle River, New Jersey 07458All rights reserved.

44. Soft tissue tumors • Non-Hodgkin’s Lymphoma • 3 types: • Lymphoblastic Lymphoma (30-40%) • Small noncleaved cell (Burkit’s) Lymphoma (40-50%) • Large cell Lymphoma (15%) • Malignant tumors of lymphoreticular (internal framework of the lymph sx) origin • Peak incidence b/t 7-11 y.o; 3X more common in boys

45. Soft Tissue Tumors • Non-Hodgkin’s Lymphoma • Clinical Manifestations include: enlarged lymph glands usually in the axillary, cervical, inguinal and femoral nodes. • Tx based on type and staging

46. Any Questions….Let’s do a case study!! A Jane W. Ball and Ruth C. BindlerChild Health Nursing: Partnering with Children & Families © 2006 by Pearson Education, Inc.Upper Saddle River, New Jersey 07458All rights reserved.