Pediatric Hematologic Disorders and Cancer

1. Pediatric Hematologic Disorders and Cancer Presented by Marlene Meador RN, MSN, CNE

2. Hematologic System Adult Pedi • Life cycle of RBC- 120 days • Cell production- marrow and spleen • RBC’s= 4.1 to 4.9 million/ml • Hemoglobin= • Hematocrit= • Life cycle of RBC- 100 days (neonate) • Cell production- red bone marrow (infant) • #RBC’s= 5million/ml at birth • Hemoglobin= 17-18 g • Hematocrit= 45-50%

3. Iron Deficiency Anemia • Cause • Signs and symptoms • Diagnostic tests • Nursing interventions • Oral supplements- What significant side effects does the nurse need to remember? • Dietary teaching- what specific foods?

4. Sickle Cell disease • Sickle cell trait- genetic disorders characterized by production of elongated, crescent shaped erythrocyte in the place of normal Hbg. p. 1494-1503 • Precipitating factors (p. 1496) • Signs and symptoms

5. Types of Sickle Cell Crisis • Vaso-occlusive- most common effects • Pain • Hand and foot syndrome (dactylitis) • CVA- hemiplegia, aphasia, seizures, LOC changes, vision changes, and headache • Acute chest syndrome- chest pain, fever cough (leading cause of death in SCD) • Priapism • Hepatomegaly • Hematuria

6. Types of Sickle Cell Crisis • Aplastic Crisis: • Decreased RBC production- S&S malaise, headache, pallor, lethargy, and fainting (precipitated by infection) • Splenic sequestration- life threatening S&S pallor, irritability, tachycardia, hypovolemic shock • Hyperhemolytic crisis- (not in text)- RBC’s destroyed more rapidly than usual (immature cells)

7. Quick Review: • What is most common reason for admission for a child with SCD? • What precipitates a sickle cell crisis? • How does sickling effect the life span of an RBC? • what organs experience complications as a result of chronic sickling crisis?

8. Diagnosis & Treatment • Cord blood testing if one parent is known to carry trait • Blood transfusions-

9. Treatment • Patient/family teaching- • Medications • Immunizations- why important?

10. Hemophilia • X-linked trait • What factor is missing or defective? Factor VIII • Who is the carrier, and who is effected by this disorder?

11. Diagnosis & Treatment • When does diagnosis most commonly occur? • What are signs & symptoms?

12. Nursing Care: • Factor VIII- when should the patient receive this medication? • What does the family need to know about factor VIII? • Human plasma • Vasopressin (DDAVP)

13. Nursing Care cont… • What is the primary nursing goal for a patient with hemophilia? • Prevent or stop bleeding • What are specific interventions to achieve this goal? • Administer Factor VIII • Apply local pressure for 10-15 minutes • Elevate the joint and immobilize • Apply cold compresses

14. Complications of hemophilia • Hemarthrosis- assess child for joint pain, edema, or permanent deformity. Where most common? • Death- at risk for hemorrhage

15. Childhood Cancer • C- continual unexplained weight loss, fatigue malaise • H- headaches with vomiting (early morning) • I- increased edema or pain in joints • L- lump or mass, persistent lymphadenopathy • D- development of whitish appearance in pupil of the eye • R- recurrent or persistent fevers, night sweats • E- excessive bruising or bleeding • N- noticeable pallor

16. What signs and symptoms would lead to the diagnosis of leukemia? • Fever • Pallor • Overt signs of bleeding • Lethargy or malaise • Anrexia • Large joint or bone pain • Petechiae, frank bleeding • Enlarged liver or spleen, changes in lymph nodes • Neurologic changes

17. Lab values for a diagnosis of leukemia: examination of CBC with at least 25% blasts confirm the diagnosis Normal Leukemia • Leukocytes < 10,000 • Leukocytes> 10,000 • Platelets 20-100,000 • Hemoglobin 7-11

18. Further diagnostic findings: • Bone marrow aspiration- iliac crest (why this site?) • How does the nurse prepare the child/family for this procedure? • What are the nurse responsibilities for this procedure?

19. Treatment and Plan of Care: • Chemotherapy: four phases • Induction phase • Consolidation • Delayed intensification • Remission and maintenance

20. Nursing Plan of Care: • Myelosupression- protect from injury • Infection/sepsis (neutropenia)- protect from infection • Renal damage • GI disturbances • Metabolic emergencies

21. Intrathecal Medication • Chemotherapy instilled into spinal canal Assess and monitor for placement of intrathecal catheter and assess neuro checks

22. Cranial Radiation • Head and neck tumors are more sensitive to radiation than chemotherapy. • When would chemotherapy become an adjunct to radiation therapy?

23. Tumor Lysis Syndrome: • What causes tumor lysis syndrome? • What are signs and symptoms of this complications • What nursing interventions apply to treatment?

24. Bone marrow and Stem Cell Transplantation • Used to treat leukemia, neuroblastomas and some noncancerous conditions-aplastic anemia • Goal to administer a lethal dose to kill the cancer, and resupply the body with stem cells from the child’s own bone marrow, or a compatible donor

25. Develop a plan of care for the child undergoing treatment for leukemia: • Risk for injury: • Soft tissue/mucous membranes • Generalized trauma • Risk for infection: • Risk for alteration in bowel elimination • Risk for GI distress • Fluid volume delicate

26. Wilm’s Tumor • Soft tumor frequently along sympathetic nervous system • (p.1552-3) Metastasis or seeding spread by palpation • Nephrectomy treatment of Wilm’s tumor

27. Nursing treatment of Wilms’ tumor: • Pain management • Frequent reposition • Noninvasive and pharmacologic pain interventions • Prevent circulatory overload • Weigh daily • I&O, urine for specific gravity • Prevent infection • Hand washing • Protective isolation • Homecare needs

28. Clinical manifestations of neuroblastoma: • P. 1549-1552 • Smooth, hard, non-tender along sympathetic nervous system • Frequent location is abdomen • Neck and facial edema from vena cava syndrome • Increased ICP • Limp if mets to bone • Pancytopenia

29. Nursing Management • Assess by observation and inspection (not palpation) • Document bowel and bladder function • Record height & weight, observe gait • Chemotherapy, radiation, surgery • Teach parents S&S of infection. Why?

30. Death and Dying: Understanding of death according to developmental age: • < 3 years- no understanding/concept of death • 3-5 years- afraid of separation from parents • 5-9 years- understand death is permanent, irreversible and sad. Concerns for fear of pain, being left alone and leaving parents and friends. • Age 10> have adult’s concept of death

31. Nursing and Grief • Child- encourage child to express feelings, allow choices, help maintain independence • Family- listen, answer questions, provide information, encourage expression of feelings and fears

32. For questions or concerns please contact Marlene Meador RN, MSN, CNE Email mmeador@austincc.edu