Interferences with Diffusion Anemia Leukemia Hemophilia

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Interferences with Diffusion Anemia Leukemia Hemophilia Slide 2 Interferences with Diffusion Describe clinical manifestations, causes, therapeutic interventions, & nursing management of patients with the following Hematologic Problems: Anemias Decreased Erthrocyte Production Iron Deficiency Anemia; Thalassemia; Megaloblastic Anemias: Cobalamin deficiency, Folic Acid Deficiency; Aplastic Anemia Anemia Caused by Blood Loss Anemia Caused by Erythrocyte Destruction Sickle Cell Disease Acquired Hemolytic Anemia Hemochromatosis Polycythemia Problems of Hemostasis: Thrombocytopenia Hemophilia and Von Willebrands Disease Leukemias Acute myelogenous leukemia Acute lymphocytic leukemia Chronic myelogenous leukemia Chronic lymphocytic leukemia Lymphomas Hodgkins Disease / non-Hodgkins lymphomas Multiple Myeloma Slide 3 Mature Normal Erythrocytes Slide 4 Nutrients Needed for Erythrocytes Slide 5 Interferences with Diffusion Hematologic System Review Complete Blood Count Studies Hgb Hct Total RBC Count Red Cell Indices MCV mean corpuscular volume (size of RBC) MCH mean corpuscular hemoglobin (weight of Hb/RBC) MCHC mean corpuscular hemoglobin concentration (saturation of RBC with Hb) WBC WBC Differential Platelet Count Erythrocyte Sedimentation Rate (ESR or Sed Rate) Slide 6 Development of Blood Cells Slide 7 Blood Components Slide 8 What are the functions of blood components? Slide 9 Functions of Blood Slide 10 Drugs Affecting Hematologic Function Slide 11 Interferences with Diffusion Anemias Caused by Decreased Erythrocyte Production Anemia Deficiency in the number of erythrocytes (RBCs) The quantity of hemoglobin Volume of packed RBCs (hematocrit) Clinical Manifestations: caused by the bodys response to hypoxia Mild (Hb 10 -14) no symptoms or minor changes Moderate (Hg 6 10) CV Changes: palpitations, dyspnea, diaphoresis Severe (Hg Interferences with Diffusion Anemias Caused by Decreased Erythrocyte Production Thalassemia Autosomal recessive genetic disorder of inadequate production of normal hemoglobin Hemolysis occurs Abnormal Hb synthesis Ethnic groups of Mediterranean Sea & near equatorial regions of Asia and Africa Clinical Manifestation: mild moderate anemia with hypochromia (pale cells) or microcytosis (small cells) Minor: one thalassemic gene mild Major: two thalassemic genes severe physical & mental growth retarded - cardiac failure is fatal Medical Management: Medication: Chelation Therapy IV deferoxamine (Desferal) iron binding agent to reduce iron overload Transfusions to maintain Hg >10g/dl Nursing Management: Supportive Slide 15 Interferences with Diffusion Anemias Caused by Decreased Erythrocyte Production Megaloblastic Anemias Caused by impaired DNA synthesis & characterized by the presence of large RBCs Cobalamin Deficiency (Vitamin B12) Intrinsic factor (IF) is secreted by the parietal cells of the gastric mucosa Cobalamin is not absorbed if IF is not present Causes: Pernicious anemia, nutritional deficiency; heredity enzyme defect Clinical Manifestations: GIsore tongue, anorexia, N&V, abdominal pain; muscle weakness, paresthesias of feet and hands; confusion Diagnostic Testing: Serum cobalamin levels; gastroscopy; Schilling Test assesses parietal cell function Medical Management: Parenteral administration of cobalamin daily for 2 weeks, then weekly until >HCT, then monthly for life; intranasal form Nursing Management: Health Promotion; protection from sensory injuryburns, trauma; pt compliance with replacement therapy Slide 16 Interferences with Diffusion Anemias Caused by Decreased Erythrocyte Production Megaloblastic Anemias Folic Acid Deficiency Folic Acid is required for DNA synthesis leading to RBC formation & maturation Causes: Poor nutrition green leafy vegetables, citrus fruits, & beans, nuts, grains; malabsorption syndromes; drugs that impede absorption (Dilantin); Alcohol abuse; anorexia; hemodialysis patients Clinical Manifestations Clinical Manifestations: similar to cobalamin deficiency dyspepsia, smooth, beefy red tongue; absence of neurologic problems Diagnostic Testing: < Folate Level (norm: 3-25mg/ml) Medical Management: Replacement Therapy Folic Acid 1mg/day Nursing Management: Medication & dietary compliance Slide 17 Interferences with Diffusion Anemias Caused by Decreased Erythrocyte Production Anemia of Chronic Disease Associated with underproduction of RBCs and decreased RBC survival Causes: Renal failure; advanced liver cirrhosis; chronic inflammation; malignancy; immunosuppression Medical Management: Correct underlying disorder Erythropoietin Therapy Epogen, Procrit Nursing Management: Care of the debilitated patient dietary & medication compliance Slide 18 Interferences with Diffusion Anemias Caused by Decreased Erythrocyte Production Aplastic Anemia Pancytopenia decrease of all blood cell types --- RBCs, WBCs, platelets & hypocellular bone marrow Congenital Acquired exposure to radiation & chemicals, post viral & bacterial infections Idiopathic 70% Medical Management: Immunosuppressive therapy Bone Marrow Transplantation Slide 19 Interferences with Diffusion Anemia Caused by Blood Loss Acute Blood Loss Hemorrhage Decreased oxygen-carrying capacity Chronic Blood Loss Body maintains its blood volume by slowly increasing plasma volume < RBCs Clinical Manifestations: Range from fatigue with melena to orthostatic BP changes to shock Medical Management: Treat underlying cause Blood replacement packed RBCs Supplemental Iron Slide 20 Lab Study Findings in Anemias Slide 21 PAIR-SHARE Name one thing you learned thus far Slide 22 Interferences with Diffusion Anemia Caused by Increased Erythrocyte Destruction Sickle Cell Disease Group of inherited autosomal recessive disorders characterized by the presence of abnormal Hgb in the erythrocyte Causes the erythrocyte to stiffen & elongate Sickle shape in response to lack of oxygen Occurrence: 50,000 Americans 1 in 350-500 African Americans; Mediterranean, So Am; East Indian, Arabian ancestry Types: Sickle Cell Anemia: most severe inherited homozygous for hemoglobin S (HbSS) from both parents Sickle Cell Trait: mild - inherited from one parent + one normal Slide 23 Interferences with Diffusion Anemia Caused by Increased Erythrocyte Destruction Sickle Cell Disease Sickling Episodes: Hypoxemia triggered by stress, surgery, blood loss, viral or bacterial infection*(most common), dehydration, acidosis Low oxygen tension in the blood Sickled cells cannot easily pass through capillaries Hemolyzed in the spleen Initially reversible then becomes irreversible due to chronic sickling Slide 24 Interferences with Diffusion Anemia Caused by Increased Erythrocyte Destruction Sickle Cell Crisis Severe, painful, acute exacerbation of RBC sickling causing a vasoocclusive crisis Impaired blood flow, vasospasm, severe capillary hypoxia RBC Cell membrane permeability changes plasma loss, & thrombi; tissue ischemia & infarction Sudden & persists for days Clinical Manifestation: PAIN tissue ischemia Aching jointshands & feet Organs that have a high need for oxygen are most affected: heart, lungs, eyes, kidneys, brain Spleen scarring & small auto splenectomy Bones osteoporosis Chronic leg ulcers Prone to infection pneumococcal pneumonia Medical Management: Hospitalization--Oxygen, rest, fluids & electrolytes, treat infection, transfusion therapy, Chelation therapy, pain management Bone Marrow Transplant & Gene therapy technology Slide 25 Sickle Cell Disease Manifestations Slide 26 PAIR-SHARE Priority Nursing Actions for the client in Sickle Cell Crisis Slide 27 Interferences with Diffusion Anemia Caused by Increased Erythrocyte Destruction Acquired Hemolytic Anemia Extrinsic causes of hemolysis: Physical trauma renal dialysis; CP bypass Autoimmune Reactions medications; systemic lupus erythematosus; leukemia; lymphoma Infectious agents and toxins parasites; antigen- antibody reactions; splenomegaly Medical Management: Corticosteroids; Blood product administration; splenectomy Slide 28 Slide 29 Slide 30 Interferences with Diffusion Anemia Caused by Increased Erythrocyte Destruction Polycythemia Production o& presence of increased number of RBCs Increased blood viscosity hyperviscosity Increased circulating volume hypervolemia Types: Primary polycythemia vera / chromosomal mutation insidious -- >50 years of age Secondary chronic hypoxia stimulates erythropoietin production in the kidney > erythrocyte production High altitude, COPD, CV disease Diagnosis: Elevated RBC, WBC, Platelets; bone marrow aspiration hypercellularity of RBCs; splenomegaly Medical Management: Phlebotomy to maintain HCT 45-48% 300-500 ml removed every other day until Clotting Cascade XII + Surface: intrinsic path: heparin/ PT | XIIa | VIIa + TF: extrinsic path: (warfarin/PT) XI---XIa Prot. C/S -- / | Va | / | | Antithrombin III -----> II-------IIa ----------------------> VIIIa & Va | | Fibrinogen----FibrinheparinwarfarinFibrinogen Slide 40 Specific Clotting Factors fibrinogen (factor I);fibrinogen - prothrombin (factor II) - converts finbrinogen to fibrin - activates V, VIII & XIII (when bound to thrombomodulin) ; - activates protein C; - Vit K dependent;Vit K - factor V: when activated, serves as enzyme co-factor - factor Xa: part of Xa/Va complex which activates prothrombin; - factor VII: - part of factor VII/tissue factor complex-activates factor X & IX; - is activated by Xa; - Vit K dependenVit K - factor VIII: serves as enzyme cofactor to help activate factor X; - factor IX: - acts w/ IXa/VIIIa/phos complex that activates factor X; - Vit K dependent;Vit K - factor X: - acts as Xa/Va phos complex that actives prothrombin; - Vit K dependent;Vit K - factor XII: - protein C: - when activated to Ca by thrombin bound to thrombomodulin, inhibits by proteolysis factors V