Interferences with Diffusion

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Interferences with Diffusion Anemia Leukemia Hemophilia

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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 - PowerPoint PPT Presentation

Transcript of Interferences with Diffusion

Page 1: Interferences  with  Diffusion

Interferences with

Diffusion

Anemia

Leukemia

Hemophilia

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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

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Mature Normal Erythrocytes

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Nutrients Needed for Erythrocytes

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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)

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Development of Blood Cells

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Blood Components

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What are the functions of blood components?

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Functions of Blood

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Drugs Affecting Hematologic Function

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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

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Anemia Clinical Manifestations

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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

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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

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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

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Interferences with Diffusion Anemias Caused by Decreased Erythrocyte Production

Megaloblastic AnemiasFolic 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 ManifestationsClinical 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

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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

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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

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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

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Lab Study Findings in Anemias

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PAIR-SHARE

Name one thing you learned thus far

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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 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

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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

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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

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Sickle Cell Disease Manifestations

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PAIR-SHARE

Priority Nursing Actions

for the client

in

Sickle Cell Crisis

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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

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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

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Primary & Secondary Polycythemia

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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

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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

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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

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Interferences with Diffusion Problems of Hemostasis

Hemophilia

Sex-linked recessive genetic disorder caused by defective or deficient coagulation

Hemophilia

A

Factor VIII Recessive – female carrier;

Occurs primarily in male

Hemophilia

B

Factor IX Recessive – female carrier;

Occurs primarily in male

Von Willebrand’s disease

vWF and platelet dysfunction

Autosomal dominant; seen in both sexes

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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

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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

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Coagulation Mechanism

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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

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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;

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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

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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

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HemophiliaHematoma of the Ear

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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

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Acute Hemarthrosis – Right Knee

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PAIR-SHARE

What coagulation studies

are affected by hemophilia?

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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

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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

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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