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POLYCYTHEMIA
An increase in RBC and hemoglobin production It is a compensatory response to chronic hypoxia Hypoxia
Erythropietin production by the kidneys Stimulation of the bone marrow to increase RBC production
POLYCYTHEMIA VERA
Due to HYPERPLASIA of the bone marrow Increased RBC (erythrocytosis); increased WBC (leukocytosis); and increased platelets (thrombocytosis) Cause is unknown; associated with genetics
POLYCYTHEMIA VERA
Increased RBC,WBC and platelets
Blood viscosity organ infiltration capillary over distension
Thromboembolism
rupture
hemorrhage
hypovolemia
POLYCYTHEMIA VERA
Clinical Manifestations 1. Ruddy complexion 2. Headache 3. Dizziness 4. Blurred vision 5. Fatigue 6. Hepato-splenomegaly
POLYCYTHEMIA VERA
Management 1. Increase fluid intake 2. Monitor for signs and symptoms of bleeding 3. Monitor for signs and symptoms of thromboembolism (angina, claudication, thrombophlebitis, pruritus) 4. Administer analgesic as ordered 5. Therapeutic phlebotomy 6. Chemotherapy 7. Radiation therapy (Na phosphate/IV) 8. Avoid high altitude
ANEMIA
Decrease availability of oxygen to the tissues Types: a. Iron deficiency Anemia b. Folate Deficiency Anemia c. Aplastic Anemia d. Pernicious Anemia
ANEMIA
Causes: 1. Acute/Chronic blood loss 2. Inadequate dietary intake of vitamins and minerals needed for RBC production 3. Decreased RBC production by the bone marrow 4. Increased destruction of RBC 5. Increased demands of vitamins/ minerals needed for RBC production
ANEMIA
Common Clinical Manifestations 1. Pallor 2. Easy fatigability 3. weakness 4. Anorexia/weight loss 5. SOB 6. Headache/dizziness/syncope 7. Tachycardia/palpitations 8. brittle hair/nails 9. Paresthesia 10. Cold sensitivity
PERNICIOUS ANEMIA
Macrocytic, hyperchromic anemia Causes: gastric surgery; crohns disease
Decrease intrinsic factor production decrease vit B12 absorption Decrease RBC production Decrease DNA synthesis in maturing RBC (megaloblastic cells) Impairment of integrity of cells (mouth, stomach, anus, vagina, axon of neurons)
PERNICIOUS ANEMIA
1. Diagnostic tests: Tubeless Gastric Analysis Diagnex blue/Azuressin tablet Collect urine specimen
(+)blue
(-) blue
HCl is present
PERNICIOUS ANEMIA
2. Schillings Test Most definitive diagnostic test Oral radioactive Vit B12 followed by IM nonradioactive Vit. B12 Collect 24-hour specimen Decreased excretion of Vit. B12 in the urine (+) Pernicious anemia
PERNICIOUS ANEMIA
Clinical Manifestations 1. Beefy red inflamed tongue 2. Achlorhydria 3. Tingling, numbness 4. Lack of balance, uncoordinated movements 5. Confusion 6. Paralysis 7. Depression, psychosis 8. Jaundice (faulty erythropoiesis)
PERNICIOUS ANEMIA
Management: 1. Vit. B12 IM 7 days (daily) 10 weeks (weekly) lifetime (100mg/mo.) monthly 2. HCl p.o for 1 week 3. Iron therapy 4. BT as needed 5. PE every 6 months * Patient is at risk for Gastric cancer
APLASTIC ANEMIA
1. 2. 3. 4. 5. 6.
Hypoplasia of the bone marrow causing pancytopenia Fat replaces bone marrow Causes Congenital Acquired Idiopathic Infections Medications Heavy metals
APLASTIC ANEMIA
Diagnostic Tests: Bone marrow aspiration/biopsy Adults (post. Iliac crest); prone position; brief sharp pain Child (tibia) ; side lying position Medical Management Immunosuppressive therapy (ATG) central line for 7-10 days BT prn Stool softener: high fiber diet
APLASTIC ANEMIA
Nursing Interventions 1. Assess for signs and symptoms of infection, tissue hypoxia and bleeding 2. Frequent rest periods 3. Good oral care 4. Use soft-bristled toothbrush 5. Male clients use electric razor 6. Dont give ASA (aspirin) 7. Reverse/protective isolation 8. Practice asepsis 9. No fresh fruits/fresh flowers in the patients unit 10. Limit visitors 11. Dont allow persons with s/sx of infection to visit
LEUKEMIA
1. 2. 3. 4. 5. Chronic or acute neoplasm of blood-forming cells in the bone marrow and lymph nodes where there is proliferation of abnormal WBCs that do not mature beyond the blast phase In the bone marrow , blast cells crowd out healthy WBCs, RBCs, and platelets, leading to bone marrow depression. Blast cells also infiltrate other organs, most commonly the liver, spleen, kidneys and lymph tissues. Most common form of child cancer Causes: Idiopathic Viral Genetic Radiation Chemicals
LEUKEMIA
Types: 1. Acute Myelogenous Leukemia (AML) Malignant disorder that affects monocytes, granulocytes, erythrocytes and platelets Survival 1-3 years (with chemotherapy); 2-5 months (if untreated) 2. Chronic Myelogenous Leukemia (CML) Malignancy of the myeloid stem cells that leads to uncontrolled proliferation of granulocytes. Survival: 3-5 years (if without BMT)
LEUKEMIA
3. Acute Lymphocytic Leukemia (ALL) Malignant proliferation of lymphoblasts arising from a disorder of a single lymphoid stem cell. 80-85% of childhood leukemia Survival : 5 years (with chemotherapy and radiation therapy) 50% can be cured 4. Chronic Lymphocytic Leukemia (CLL) Caused by a proliferation of small, abnormal B lymphocytes Survival: 5-7 years
LEUKEMIA
Proliferation of Immature WBCs
Immunocompromised state Decreased production of RBCs and platelets Hypertrophy of the bone marrow Organ infiltration Bone pain, hepatosplenomegaly, renal insufficiency, hyperuricemia, increase ICP (meningeal irritation)
LEUKEMIA
Clinical Manifestations 1. Anemia due to decreased production of RBCs; weakness, pallor, dyspnea 2. Bleeding due to decreased platelet production, petechiae, spontaneous bleeding and ecchymosis 3. Infection due to decreased WBC production, fever, malaise 4. Enlarged lymph nodes 5. Enlarged spleen and liver 6. Abdominal pain with weight loss and anorexia 7. Bone pain due to expansion of marrow
LEUKEMIA
Management 1. Chemotherapy 2. BMT 3. BT Nursing Interventions 1. Protect from infection a. reverse/protective isolation b. Practice asepsis c. Limit visitors d. Dont allow people with injections to visit 2. Prevent trauma a. Minimize parenteral injxn b. Use small gauge needle c. apply pressure at injection site for 5 minutes d. Support/handle body parts gently e. Use soft-bristled toothbrush f. Use electric razor g. dont administer ASA
LEUKEMIA
3. Conserve energy/increase oxygen supply a. adequate rest b. O2 supplementation
HODGKINS LYMPHOMA
Malignant noplasm of lymph tissue, usually originating in localized group of lymph nodes; a proliferation of lymphocytes Metastasizes first to adjacent lymph nodes Cause is unknown Most prevalent in adolescents; accounts for 5% of all maliganancies Prognosis now greatly improved for these children; influenced by stage of disease and histologic type Long term effects include increased incidence of 2nd malignancy; especially leukemia and infertility
HODGKINS LYMPHOMA
Clinical manifestations 1. Major presenting symptom is enlarged nodes in low cervical region; nodes are nontender, firm and movable 2. Recurrent intermittent fever 3. Night sweats 4. Weight loss, malaise, lethargy 5. Pruritus 6. Diagnostic test: presence of Reed-Sternberg cells
HODGKINS DISEASE
Medical Management 1. Radiation therapy 2. Chemotherapy 3. Supportive care Nursing Intervention 1. Provide care for child receiving radiation therapy. 2. Administer chemotherapy as ordered and monitor or alleviate side effects. 3. Protect client from infection, especially if splenectomy is performed. 4. Provide support for child/parents; specific needs of adolescent client must be considered.
NON-HODGKINS LYMPHOMA
Tumor originating in lymphatic tissue Significantly different from Hodgkins lymphoma : a. control of primary tumor is difficult b. Disease is diffuse, cell type undifferentiated c. Tumor disseminates early d. Includes wide range of disease entities: lymphosarcoma, Burkitts lymphoma primary sites include GIT, ovaries, testes, bone, CNS, liver, breast. Affects all age groups
NON-HODGKINS LYMPHOMA
Clinical Manifestations: 1. Depend on anatomic site and extent of involvement 2. Rapid onset and progression 3. May have advanced disease at diagnosis
NON-HODGKINS LYMPHOMA
Medical Management: 1. Chemotherapy: multiagent regimens including cyclophosphamide (Cytoxan), vincristine, prednisone, procarbazine, doxorubicin, bleomycin 2. Radiation therapy: primary treatment in localized disease 3. Surgery for diagnosis and clinical staging
Nursing Interventions 1. Provide care for child receiving chemotherapy, radiation therapy, and surgery