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Providing Patient Centered Care for the Child with a Hematologic Disorder

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  1. Providing Patient Centered Care for the Child with a Hematologic Disorder 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

  4. 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?

  5. What Parents Want to Know:(p 1243) • Specific foods: (based on age of child) • Cream of wheat or iron fortified cereal • Apricots, prunes, raisins and other dried fruits • Egg yolks • Dark green leafy vegetables • Administration of Iron Supplements: • Give with vitamin C –rich fluids • Prevent staining from liquid iron supplements • Changes in stool patterns • Avoid mixing supplement with food/drink containing calcium

  6. Sickle Cell Disease

  7. Sickle Cell disease • Sickle cell trait- genetic disorders characterized by production of elongated, crescent shaped erythrocyte in the place of normal Hbg • Precipitating factors (p 1248-Home Care of the Child with Sickly Cell Disease) • Signs and symptoms

  8. Three Types of Sickle Cell Crisis • Vaso-occlusive • Acute sequestration • Aplastic

  9. 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

  10. 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)

  11. Quick Review: • What is most common reason for admission to the ED 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?

  12. Diagnosis & Treatment • Cord blood testing if one parent is known to carry trait • Blood transfusions • Complications • Nursing interventions before/during/after

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

  14. Clinical Judgment: • Why are blood transfusions ordered for the patient in sickle cell crisis? • Can a neonate have a diagnosis of sickle cell disease? • What ethical issues relate to this diagnosis?

  15. Hemophilia

  16. Hemophilia (p 1291) • X-linked trait • What factor is missing or defective? • Who is the carrier, and who is effected by this disorder?

  17. Diagnosis & Treatment • When does diagnosis most commonly occur? • What specific laboratory tests and values? • What are signs & symptoms?

  18. 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)

  19. 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

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

  21. The Child with Cancer

  22. Childhood Cancers “…communication promotes understanding and clarity; with understanding, fear diminishes; in the absence of fear, hope emerges; and in the presence of hope, anything is possible” (Stovall, 1995)

  23. 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

  24. Childhood Cancer & Treatment • Chemotherapy • Surgery • Radiation • Stem Cell Transplantation • Steroid Therapy • Biologic Agents • Complementary and Alternative Medical (CAM)

  25. Chemotherapy: Antinoeplastic agents • Titrated to specific formula- closely correlated with cardiac output and blood flow to kidneys and liver • Highly specialized nurses- The Association of Pediatric Hematology/Oncology Nurses • Administration routes: • Oral • Intravenously (large bore catheter) • Intramuscularly • Subcutaneously • Intrathecally

  26. Chemotherapy: side effects/ nursing interventions

  27. Chemotherapy Nursing Interventions: • Protection of the patient: • Isolation- what specific type? • Exposure to sun, chemicals, skin irritants • Nutritional needs: • Prevent nausea & vomiting • Types of foods to meet metabolic requirements • Temperatures/textures/acid-base • Fluid balance • Intake and output • Integumentary • Self image

  28. Clinical Judgment: • Why does the nurse increase the amount of water/liquid intake for a patient on chemotherapy? • What specific interventions does the nurse need to follow with relation to the patient’s output? • Why are stool softeners very important? • What are the best foods for a patient on chemotherapy? Why? • What do parents and family need to understand about the patient’s psycho-social needs?

  29. Radiation Therapy • Purpose • Palliative- prevent growth, reduce tumor size, pain relief • Eradicate or kill a tumor • Side effects similar to chemotherapy: Sub-acute & Late • Somulence syndrome • Fever • Irritability • Ataxia • Anorexia • Dysphasia

  30. Surgical treatment for cancer • Purpose • Confirm diagnosis • Debulking or resecting • Removal of tumor as adjunct to chemotherapy/radiation • Nursing Interventions • How does the nurse prepare the patient for surgery? • What interventions apply specifically to the family?

  31. Hematopoietic Stem Cell and Bone Marrow Transplantation : • Conditioning- eradicate disease with high-dose chemo/radiation therapy • Infusion- implantation of stem cells or bone marrow • Increase in patient’s WBC, RBC, and platelets signal success! • Complications • GI disturbances • Graft-Versus-Host Disease (GVHD) • Maintain patient on anti-rejection medications • Prednisone • Cyclosporine • Tacrolimus

  32. Graft-Versus-Host Disease (GVHD) Potentially lethal immunologic response of donor T cells against the tissue of the recipient. Prevention: Careful tissue typing, irradiation of blood products to inactivate mature T lymphocytes. • Signs & Symptoms • rash, malaise, high fever, diarrhea, liver and spleen enlargement • Treatment • Maintain patient on anti-rejection medications • Prednisone • Cyclosporine • Tacrolimus

  33. Complementary /Alternative Medical Therapies (CAM) • Risks-vs- benefits • Qualification of practitioner • Delay or interfere with conventional treatment • Costs • Contraindications

  34. Acute Lymphatic Leukemia- ALL

  35. 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

  36. 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

  37. 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?

  38. Treatment and Plan of Care: (p 1274-1280) • Chemotherapy: three phases • Induction phase • Consolidation • Delayed intensification • Remission and maintenance

  39. Nursing Care for a Child Undergoing Chemotherapy: review • Myelosupression- protect from injury • Infection/sepsis (neutropenia)- protect from infection • Renal damage • GI disturbances • Metabolic emergencies

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

  41. Treatment of Tumor Causing Cancers

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

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

  44. Other Cancers of Childhood

  45. Clinical manifestations of Neuroblastoma(p 1286) • Smooth, hard, non-tender along sympathetic nervous system • Frequent location is abdomen • Neck and facial edema from vena cava syndrome • Increased ICP • Limp if metastasis to bone • Pancytopenia

  46. 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?

  47. Osteosarcoma

  48. Osteosarcoma- most common primary bone malignancy in children • Goal of treatment- remove tumor and prevent spread of disease • Biopsy Chemo Surgery Chemo (radiation=palliative pain control) • Promote self esteem • Side effects of chemotherapy • Amputation of extremity • Separation from friends and family

  49. Ewing Sarcoma- second most common bone tumor associated with children • Pain, soft tissue swelling • Anorexia, fever, malaise with metastasis • Diagnosis same as osteosarcoma • Management • Chemo • Surgery (decrease tumor bulk) • Radiation

  50. Rhabdomyosarcoma