Interferences with Diffusion

1. Interferences with Diffusion Anemia Leukemia Hemophilia

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 Willebrand’s Disease • Leukemias – • Acute myelogenous leukemia • Acute lymphocytic leukemia • Chronic myelogenous leukemia • Chronic lymphocytic leukemia • Lymphomas • Hodgkin’s Disease/ non-Hodgkin’s lymphomas • Multiple Myeloma

3. Mature Normal Erythrocytes

4. Nutrients Needed for Erythrocytes

5. Interferences with DiffusionHematologic 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)

6. Development of Blood Cells

7. Blood Components

8. What are the functions of blood components?

9. Functions of Blood

10. Drugs Affecting Hematologic Function

11. Interferences with DiffusionAnemias 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 body’s response to hypoxia • Mild (Hb 10 -14) no symptoms or minor changes • Moderate – (Hg 6 – 10) CV Changes: palpitations, dyspnea, diaphoresis • Severe – (Hg<6) multiple body system CV, Cerebral, Major Organs

12. Anemia Clinical Manifestations

13. Interferences with DiffusionAnemias Caused by Decreased Erythrocyte Production Iron-Deficiency Anemia • Common hematologic disorder • Etiology: Inadequate dietary intake, malabsorption, blood loss, or hemolysis • Clinical Manifestations: • Pallor • Glossitis – inflammation of the tongue • Cheilitis – inflammation of the lips • Headache, paresthesia, burning sensation of the tongue • Diagnostic Studies: Lab Studies Endoscopy to identify GI bleed • Treatment: Drug Therapy – oral Iron replacement • Iron absorbed best in duodenum • Ferrous sulfate – take about one hour prior to meal • Gastric side effects: nausea / constipation • Nursing Management – Diet & Medication Instruction

14. Interferences with DiffusionAnemias 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

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: GI—sore 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 injury—burns, trauma; pt compliance with replacement therapy

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: 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

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

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

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

20. Lab Study Findings in Anemias

21. PAIR-SHARE Name one thing you learned thus far

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

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

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 joints—hands & 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

25. Sickle Cell Disease Manifestations

26. PAIR-SHARE Priority Nursing Actions for the client in Sickle Cell Crisis

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

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 <HCT • Hydration • Myelosuppressive therapy: busulfan (Myleran); hydroxyurea (Hydrea) = inhibits bone marrow production • Gout – Allopurinol • Antiplatelet medication: Persantine, Plavix, ASA – prevent thrombotic complicatins

31. Primary & Secondary Polycythemia

32. Interferences with Diffusion Problems of Hemostasis Thrombocytopenia • Reduction of platelets below 150,000/ul • ITP: Immune Thrombocytopenic Purpura: autoimmune • platelets are coated with antibodies; destroyed in spleen • Women 20-40 years • Normal survival 8-10 days; ITP: 1-3 days • TTP: Thrombotic Thrombocytopenic Purpura • Uncommon syndrome; adults 20-50 years • Characterized by hemolytic anemia, thrombocytopenia, neurologic abnormalities, fever, renal abnormalities • HITT: Heparin-Induced Thrombocytopenia & Thrombosis Syndrome • Immune-mediated response to Heparin • Triggers platelet aggregation • Decreased platelets & increased thrombosis

33. Interferences with Diffusion Problems of Hemostasis Thrombocytopenia • Clinical Manifestations: • Bleeding: • Mucosal — epistaxis, gingival, large bullous hemorrhages on buccal mucosa • Skin -- superficial ecchymoses; petechiae — flat pin-pointed red brown microhemorrhages; purpura — numerous petechiae resulting reddish skin bruising • Prolonged bleeding – after injections & venipuncture • Internal bleeding – hemorrhage – any major organ -- orthostatic hypotension -- Cerebral hemorrhage fatal • Medical Management: • ITP: Corticosteroid; immunosuppressive therapy; splenectomy; platelet transfusion • TTP: Corticosteroids; plasma exchange; plasmapheresis; splenectomy • HITT: Discontinue Heparin – Protamine Sulfate

34. Interferences with Diffusion Anemia caused by Blood Loss or Erythrocyte Destruction Nursing Management • Assess: Assess oral cavity, skin, nasal cavity, urine, stool – occult and overt bleeding; Lab values—CBC, platelet count; vital signs; signs & symptoms for each blood dyscrasia • Nsg Action: Ice, packing, direct pressure to control bleeding; administer medications; No ASA or platelet-acting meds; oral hygiene, skin care, IV carefully—blood product transfusion care; Avoid IM/SQ meds; pad rails & firm surfaces; pain management as needed; post splenectomy care; • Pt Education: Interpret lab values; Rationale for no sharps – electric razor; disease process; medications; avoid valsalva; blow nose gently—one side at a time; signs & symptoms of bleeding; lab values; health promotion—rest, oxygenation, nutrition; pain management

35. Interferences with Diffusion Problems of Hemostasis Hemophilia Sex-linked recessive genetic disorder caused by defective or deficient coagulation

36. Interferences with Diffusion Problems of Hemostasis Hemophilia – Disease of Childhood • Clinical Manifestations: • Slow, persistent, prolonged bleeding from minor trauma and small cuts • Delayed bleeding after minor injuries • Uncontrollable hemorrhage after dental extractions • Epistaxis, especially after a blow to the face • GI bleed – ulcers & gastritis • Hematuria from GU trauma • Splenic rupture from abdominal trauma • Ecchymosis & subcutaneous hematomas • Neurologic signs – pain, anesthesia, & paralysis – from nerve compression caused by hematoma formation • Hemarthrosis – bleeding into joints – joint deformity – may cause crippling SYMPTOMS OFTEN LEAD TO THE DIAGNOSIS

37. Interferences with Diffusion Problems of Hemostasis Hemophilia Time Life Expectancy • Early 1900’s 11 years • 1970’s 60 years • 1980’s late 40’s • 90% of older people are HIV+ • Many are Hepatitis C+ • Improved blood product testing & screening has decreased the number of younger people with blood borne sequellae

38. Coagulation Mechanism

39. Clotting Cascade • XII + Surface: intrinsic path: heparin/ PT                   |                 XIIa                   |             VIIa + TF: extrinsic path: (warfarin/PT)                 XI---XIa <----/                       |                             IX-----IXa                                                     |                               VIIIa |                                 |                                 Thrombomodulin                       X----------Xa                 --------> Prot. C/S --                       /           | Va                                 |                     /             |                                     |       Antithrombin III ----->   II-------IIa ----------------------> VIIIa & Va                                     |                                     | Fibrinogen----Fibrin

40. Specific Clotting Factors fibrinogen (factor I); - prothrombin (factor II)     - converts finbrinogen to fibrin - activates V, VIII & XIII (when bound to thrombomodulin) ; - activates protein C; - Vit K dependent; - 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 dependen - 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; - factor X: - acts as Xa/Va phos complex that actives prothrombin; - Vit K dependent; - factor XII:   - protein C:     - when activated to Ca by thrombin bound to thrombomodulin, inhibits by proteolysis factors VIIIa and Va in reactions requiring prot S and phospholipids as cofactors; Vit K dependent;   - antithrombin III:     - is a plasma protease inhibitor that serves as a protease scavenger;     - any of the blood-clotting enzymes that move away from the growing clot rapidly form a complex, and their activities are neutralized     - formation of complexes is accelerated by heparin, forms of which are located in the microvasculature on the surfaces of endothelial cells;     - inhibitor of the enzymes thrombin, Xa, IXa; Is activated by   heparin;

41. Interferences with Diffusion Problems of Hemostasis Hemophilia • Diagnostic Studies: • Partial thromboplastin time: prolonged • Bleeding time: • Prolonged in von Willebrand’s because of structural defectiveplatelets; • Normal in Hemophilia A & B • Factor Assays: • Factor VIII – A • Factor IX – B • vWF – von Willebrand

42. Interferences with Diffusion Problems of Hemostasis Hemophilia • Medical Management Goals: • Preventive Care • Replacement Therapy during acute bleeding • May also be given prior to surgery or dental care • Factor VIII: Examples: Alpohanate, Bicolate, Koate • Factor IX: Examples: Alphanine, Benefix, Konyne • Mild Hemophilia A & von Willebrand’s: • Desmospressin acetate (DDAVP) – causes a release of vWF, which binds with factor VIII, increasing concentration • Short-lived; IV/SQ/Intranasal • Treatment of complications

43. HemophiliaHematoma of the Ear

44. Interferences with Diffusion Problems of Hemostasis Hemophilia • Nursing Management: • Health Promotion: • Acute Intervention: • Stop the topical bleeding – direct pressure, ice, Gelfoam or fibrin foam packing; topical hemostatic agents – thrombin • Administer the specific anticoagulant factor • Joint bleeding: rest; pack in ice; analgesia • Mobilization when bleeding subsides – P & AROM exercise • Manage life-threatening complications: • Airway management – Head and neck injuries • Neuro signs – Head trauma; spine trauma • Home Care: • Patient Education – Age-specific: how to live with illness: increased oral hygiene; injury prevention; Medic Alert; routine medical follow-up; non-contact sports; self-administration of replacement factors • Local chapters of National Hemophilia Society • Daily oral hygiene

45. Acute Hemarthrosis – Right Knee

46. PAIR-SHARE What coagulation studies are affected by hemophilia?

47. Interferences with DiffusionTransfusion Therapy - Indications • Packed red blood cells – anemia; Hgb < 6-9g/dL depending on symptoms • Washed red blood cells – hx allergic rx; bone marrow transplant patients • Platelets – thrombocytopenia; active bleeding with platelet count <80,000 • Fresh frozen plasma – deficiency in plasma coagulation factors • Cryoprecipitate – Hemophilia VII or von Willebrand’s disease • WBC’s – Sepsis, neutropenic infection

48. Interferences with DiffusionTransfusion Therapy • Responsibilities • Before Transfusion: assess lab values; verify order; assess pt’s VS, UO, hx of transfusion reactions; CONSENT; patent venous access; pt ID / Blood verification RN-RN - document. • During Transfusion: Appropriate tubing & filter; normal saline infusion; prescribed rate of administration; Remain with pt for the first 20 mins of transfusion; monitor VS and assess for transfusion rx. – document • After transfusion: Assess VS; discontinue infusion & dispose of bag & tubing according to policy; document; reassess blood work

49. Interferences with DiffusionTransfusion Therapy • Transfusion reactions • Hemolytic: blood type or Rh incompatibility – antigen-antibody reaction – fever, chills, DIC, circulatory collapse (back pain, tachypnea, tachycardiac, hypotension, chest pain, hemoglobinuria, apprehension • Allergic: Urticaria, bronchospasm, anaphylaxis – hx of allergy • Febrile: occurs in pts with anti-WBC antibodies – chills, tachycardiaa, fevere, hypotension, tachypnea • Bacterial: Rapid onset: occurs as a result of contamination – tachycardia; hypotension, fever, chills, shock • Circulatory Overload: Infused too quickly; Hx CHF – Hypertension, bounding pulse, distended jugular veins, dyspnea, restlessness, confusion