1. 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.edummeador@austincc.edu