Interferences with Diffusion Anemia Leukemia Hemophilia

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

Mature Normal Erythrocytes

Nutrients Needed for Erythrocytes

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)

Development of Blood Cells

Blood Components

What are the functions of blood components?

Functions of Blood

Drugs Affecting Hematologic Function

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

Anemia Clinical Manifestations

Interferences with Diffusion Anemias 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

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

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

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

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

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

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

Lab Study Findings in Anemias

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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 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

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

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

Sickle Cell Disease Manifestations

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

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

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% 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

Primary & Secondary Polycythemia

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 years Normal survival 8-10 days; ITP: 1-3 days TTP: Thrombotic Thrombocytopenic Purpura Uncommon syndrome; adults 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

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

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

Interferences with Diffusion Problems of Hemostasis Hemophilia Sex-linked recessive genetic disorder caused by defective or deficient coagulation Hemophilia A Factor VIIIRecessive – female carrier; Occurs primarily in male Hemophilia B Factor IXRecessive – female carrier; Occurs primarily in male Von Willebrand’s disease vWF and platelet dysfunction Autosomal dominant; seen in both sexes

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

Interferences with Diffusion Problems of Hemostasis Hemophilia TimeLife Expectancy Early 1900’s 11 years 1970’s 60 years 1980’slate 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

Coagulation Mechanism

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

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 VIIIa and Va in reactions requiring prot S and phospholipids as cofactors; Vit K dependent;Vit K - 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;heparin

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

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

Hemophilia Hematoma of the Ear

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

Acute Hemarthrosis – Right Knee

PAIR-SHARE What coagulation studies are affected by hemophilia?

Interferences with Diffusion Transfusion 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

Interferences with Diffusion Transfusion 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

Interferences with Diffusion Transfusion 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