Presented by Marlene Meador RN, MSN, CNE Hematologic System Adult Pedi Life cycle of RBC- 120 days Life cycle of RBC- 100 days Cell production- marrow and spleen RBC’s= 4.1 to 4.9 million/ml Hemoglobin= Hematocrit= (neonate) Cell production- red bone marrow (infant) #RBC’s= 5million/ml at birth Hemoglobin= 17-18 g Hematocrit= 45-50% 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? 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 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 Three Types of Sickle Cell Crisis Vaso-occlusive Acute sequestration Aplastic 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 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) 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? Diagnosis & Treatment Cord blood testing if one parent is known to carry trait Blood transfusions Complications Nursing interventions before/during/after Treatment Patient/family teaching Medications Immunizations- why important? 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? Hemophilia (p 1291) X-linked trait What factor is missing or defective? Who is the carrier, and who is effected by this disorder? Diagnosis & Treatment When does diagnosis most commonly occur? What specific laboratory tests and values? What are signs & symptoms? 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) 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 Complications of hemophilia Hemarthrosis- assess child for joint pain, edema, or permanent deformity. Where most common? At risk for hemorrhage Death 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) 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 Childhood Cancer & Treatment Chemotherapy Surgery Radiation Stem Cell Transplantation Steroid Therapy Biologic Agents Complementary and Alternative Medical (CAM) 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 Chemotherapy: side effects/ nursing interventions Bone Marrow Suppression Neutropenia Anemia Thrombocytopenia GI and GU Nausea/vomiting Stomatitis Anorexia Renal damage Integumentary Changes Hair loss Hyperpigmentation of skin Hypersensitivity to sunlight 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 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? 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 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? 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 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 Complementary /Alternative Medical Therapies (CAM) Risks-vs- benefits Qualification of practitioner Delay or interfere with conventional treatment Costs Contraindications 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 Lab values for a diagnosis of leukemia: examination of CBC with at least 25% blasts confirm the diagnosis Normal Leukocytes < 10,000 Leukemia Leukocytes> 10,000 Platelets 20-100,000 Hemoglobin 7-11 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? Treatment and Plan of Care: (p 1274-1280) Chemotherapy: three phases Induction phase Consolidation Delayed intensification Remission and maintenance Nursing Care for a Child Undergoing Chemotherapy: review Myelosupression- protect from injury Infection/sepsis (neutropenia)- protect from infection Renal damage GI disturbances Metabolic emergencies Intrathecal Medication Chemotherapy instilled into spinal canal Assess and monitor for placement of intrathecal catheter and assess neuro checks Cranial Radiation Head and neck tumors are more sensitive to radiation than chemotherapy. When would chemotherapy become an adjunct to radiation therapy? Tumor Lysis Syndrome: What causes tumor lysis syndrome? What are signs and symptoms of this complications What nursing interventions apply to treatment? 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 Nursing Management Assess by observation and inspection palpation) Document bowel and bladder function Record height & weight, observe gait Chemotherapy, radiation, surgery Teach parents S&S of infection. Why? (not 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 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 Rhabdomyosarcoma Pathophysiology and Manifestations Most common soft tissue malignancy Divided by young (<10 yrs) and older (adolescents) in location 60% have positive prognosis Soft to hard, nontender mass (depends on location) In pelvic tumors, may disrupt organ function Diagnosis and Treatment CT, BM aspiration and biopsy Renal function and liver function tests Treated with chemo, surgery and radiation Nephroblastoma- Wilm’s Tumor Soft renal tumor - one or both kidneys (p 1291) Metastasis or seeding spread by palpation Nephrectomy treatment of Wilm’s tumor 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 Retinoblastoma (p1292) Retinoblastoma- rare malignant tumor of the neural retina “cat’s eye” reflex seen as a white light in the pupil is the most common “leukocoria” May have strabismus of involved eye Red painful eye is late symptom Staging based on extent of disease Nursing care of the child/family with a malignant disease: (p905-915) Initial focus on support of family members Nurses facilitate the educational process to allay fears of unknown Encourage family members to verbalize fears and questions Postoperative care if indicated Community resources (through the discharge planner, case worker) 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 Nursing Care 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 Nursing Care for the Nurse Providing physical and psychosocial support for the patient and family places additional stressors on the staff and nurses Caring for dying children and their families is emotionally demanding. Grief counseling plays an important role for both family and staff For questions or concerns please contact Marlene Meador RN, MSN, CNE mmeador@austincc.edu References: McKinney, James, Murray, & Ashwill. Maternal- Child Nursing forth ed.(2015). Saunders.