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    NCM 202 NCM of Clients with Hematologic Disorders Ms. Jean Javie

    General Objectives

    At the end of 18 hours the student will be able to acquire adequate knowledge, skills and desirable La

    Sallian values and attitudes in the care of clients with hematological disorders.

    Specific Objectives

    After 18 hours of lecture discussion, the student will be able to:

    1. Discuss briefly the Anatomy and Physiology of the hematologic system.2. Explain briefly the pathophysiology of each hematologic disorder.3. Perform accurate physical assessment of a sick client.4. Differentiate specific Diagnostic exam for each disorder.5. Distinguish specific medical management.6. Formulate at least 5 priority nursing diagnosis.7. Plan safe and quality cursing care based on the formulated nursing diagnosis.8. Analyze the different rationale behind the different nursing action.9. Identify the role of significant others in home teaching activities.10. Integrate appropriate La Sallian core values and attitude in everyday activities.

    Course Content

    A. Overview of the Anatomy and Physiology of the Hematologic System1. Bone Marrow2. Reticuloendothelial System3. Blood

    a. Plasmab. Blood Cells (Cellular Elements)

    i. RBCii. WBC

    iii. PlateletB. Blood Group Classification

    1. AVO System2. Rh System

    C. Disorders Affecting RBC Production1. Decrease RBC Production

    a. Anemiai.

    IDAii. Folic Acid Deficiency Anemia

    iii. Pernicious Anemiaiv. Aplastic Anemiav. Thalasemia

    2. Increase RBC Productiona. Polycythemia

    D. Disorder Affecting WBC Productiona. Agranulocytosisb. Leukemia

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    E. Disorder Affecting Platelet Production (Coagulation Disorders)a. Thrombocytopeniab. Hypoprothrombinemiac. Disseminated Intravascular Coagulationd. Hemophilia

    Grading System

    Quizzes 50% (60% passing)

    Exam 40%

    Class Participation 5%

    Research/Readings 5% (Comprehensiveness2. 5% Accuracy 2% Punctuality .5%)

    References: Black and Hawks, Ignatovicious, Daniels, Lippincott

    A. BoneMarrow- Soft materials that fill the central core of the bones where hematopoiesis takes place.

    a. Red Marrow aggregates of hematopoietic cells interspaced with sinusoidal capillariesb. Yellow Marrow adipose cells

    - Before Puberty (produced in the flat bones of the skull, clavicle, sternum, ribs, vertebrae, pelvis)- After Puberty (sternum , ribs, vertebrae, iliac region, femur, humerus)- Site for the maintenance of a self renewing pluripotent stem cell (renewed)- Storage for RBC,WBC, Neutrophils, Platelets- Antibody production (Ig)

    - T Lymphocytes (Surveillance system) recognizes foreign materials as harmful to our body- B lymphocytes (Mature to plasma cells secrete Ig) Humoral Immunity (Antibody Moderated

    Immunity)

    - Natural Killer Cells (NK Cells) secretes perforin to perforate the cells

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    B. Reticuloendothelial System- Composed of many organs- Liver, Lungs, Lymph Nodes, Spleen- Functions as tissue macrophages- Comes from monocytes (within 24hourse goes to the sinusoidal capillaries of the liver, lungs, lymph

    nodes and spleen

    - Kuppfer Cells are specialized macrophages located in the liver lining the walls of the sinusoids thatform part of the reticuloendothelial system

    - Spleen LUQ; behind and below the stomach A major source of hematopoiesis in fetal life Absorbs ghost cells (d/t RBC hemolysis) Conditioning of Reticulocytes to form mature RBC

    - Hematopoiesis occurs in the bone marrow- Liver and spleen compensate if bone marrow is diseased (Extra Medullary Hematopoiesis)- Liver produces Erythropoietin in lesser amounts, production of clotting factors- Lymph nodes should be less than 5mm in diameter greater in number in the thoracic and abdominal

    area; can also be found superficially in cervical and inguinal area

    - Alveolar cells in the lungs keep it sterileC. Blood

    Functions

    1. Transportation (CO2, hormones, enzymes, nutrients, metabolic waste)2. Regulation (regulation of temperature and body processes)3. Protection (from infection, bleeding)

    - Plasma 55% 92% H2O 7% protein (Se Albumin, Gamma globulin, fibrinogen) 1% electrolytes, metabolic wastes, gasses, enzymes, nutrients, inorganic salts, other clotting

    factor

    Se Albumin maintains colloidal osmotic pressure Gamma globulin immunity, antibodies

    o Ig GMAED Fibrinogen clotting factor

    - Female = 4-5L of blood Male = 5-6L of blood; the more fat, the lesser the blood one has- Blood Cells 45% RBC, WBC, Platelets- Poietins

    a. RBC Production (Erythropoiesis)- Kidneys produces erythropoietin bone marrow- Biconcave discs, flexible (contains cholesterol), can easily be compressed by fingers,

    membranes are thin

    - Life span is 80-120 days- Normocytic 7.5-8micrometers- Macrocytic >9micrometers- Microcytic

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    - Normochromic normal in color- Hyperchromic bright red in color; greater amount of hemoglobin- Hypochromic pale- RBC NV: F = 4.4-5M/mm3 M = 4.8-5.5M/mm3- Hgb NV: F = 13-15g/dL M = 14.5-16g/dL- Hct N: F = 40-45% M = 43-50%- RBC has glutathione- 200-300 molecules of hemoglobin per RBC- Heme Fe and Porphyrin- Carbonic anhydrase enzyme that combines H2O and CO2 carbonic acid and dissociates

    to form bicarbonate and hydrogen ions

    - Hemolysis every 80-120 days- RBC Debris eaten by macrophages and brought to the spleen

    b. Leukocytes (WBC) 7-10 days- Leukocytosis- Myelocytosis- 5000-10000/mm3- Lasts from few hours to 3 days- Granulocytes

    Basophils (allergies contains histamine, heparin anticoagulant)

    Eosinophils (counteracts histamine, modulates IgE mediated allergic response) Neutrophils acute inflammatory condition polymorphonuclear cells (bands,

    stabs, segmenter

    o 5% in circulationo 5% stays in the endothelial liningo 90% stored in the bone marrow

    - Agranulocytes Monocytes protozoal, fungal, malarial infection (function as a macrophage) Lymphocytes B, T, NK; increase in chronic inflammatory conditions

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    c. Platelets/Thrombocytes (Thrombopoiesis/Megakaryopoiesis)- NV 150000-450000/mm3- Life span 7-10 days- 5 days process of production

    System of Blood Classification

    Antigen (Agglutinogen) proteins that stimulate body to produce antibodies

    Antibody (Agglutinin) proteins that destroy/render harmless the specific antigen; may cause agglutination of

    blood cells causing hemolysis

    ABO System

    - Causes spontaneous reactions- It is best to give the same type of blood to the client

    Types Antigen in RBC Antibody in Plasma Donor/Donors

    A A Anti B O, A

    B B Anti A O, B

    AB AB None A,B, AB, O

    O none Anti AB O

    Rh System

    - Considered as the D antigen- If present. Rh+, of none Rh-- Reaction is non spontaneous- Coombs test, to check for the presence of Rh incompatibility- RhoGAM within 24h

    Anemia

    1. Mild- 10-12g/dL, usually asymptomatic occur only following strenuous exertion- Palpitations, dyspnea, diaphoresis

    2. Moderate- 6-10g/dL- Palpitations, dyspnea, diaphoresis

    3. Severe-

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    c. Mouth glossitis, smooth tongue, (others have sore beefy red tongues polycythemia vera)

    d. Cardiovasuclar tachycardia, angina, increase pulse pressure, congestive heart failure associated

    with tissue hypoxia

    e. Pulmonary tachypnea, orthopnea, shortness of breath

    f. Neurological headache, vertigo irritability, depression, impaired thought processes, tingling

    sensation

    g. GIT N and V, anorexia, hepatomegaly, spleenomegaly

    h. Skeletal system bone pain (d/t erythropoiesis)

    i. Reproductive system

    amenorrheaj. General

    sensitivity to cold, weight loss, lethargy, brittle hair and nails

    Heme Sources animal products

    Non-Heme plants sources

    Fe Daily Requirement: F = 15mg M = 10mg

    - 5-10% of iron is absorbed in the proximal small intestine (.6mg-1.5mg)- In cases of disease process, GIT can compensate absorption to 20-30%- Less than 1mg of iron everyday through urine, feces, sweat, breathing, desquamated skin, bile- 15mg of Fe/Month for women- An entire pregnancy loses about 500mg of Fe- Fe is stored in the form of Ferritin in the reticuloendothelial system- Ferric iron combines with Apoferritin in proximal small intestine transported by transferrin to the

    storage sit in the reticuloendothelial system spleen, liver bone marrow

    - 2-4cc blood loss about 1mg of Fe will be loss

    A. Iron Deficiency Anemia- Chronic microcytic Anemia caused by a deficiency in iron; hypochromic anemia- Causes

    1. Inadequate dietary intake Poverty Lifestyle Those having dental problem (poor dentition) Stress

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    Anorexia Nervosa Food preferences

    2. Blood Loss In cases of injury Coagulation disorders Gum bleeding, epistaxis Any forms of ulceration

    3. Impaired absorption of Fe GIT disorders (Malabsorption syndrome) Alcohol drinks inhibit non-heme iron absorption Carbonated drinks inhibit non-heme iron absorption Coffee and tea (tannates can inhibit non-heme iron absorption) Antacidsmagnesium trisilicate Pica for clay, uncooked rice, starch, non edible things, coats intestinal mucosa Chelating agent EDTA (Ehthylenadramine tetraacetic acid); Chelation process whereby a

    chemical compound forms complexes by binding with metal ion (Fe in this case) thereby

    causing excretion of the drug

    Parasitism parasite will utilize iron

    4. Excessive demands for the production of Fe (Menstruation, Pregnancy, Infancy, Childhood,Puberty), those who are more than 65 y/o, chronic blood donors

    - Signs and Symptomsi. General S/Sx

    Pallor, dyspnea, dizziness, weakness, easy fatiguability, palpitations,diaphoresis, shortness of breathing

    ii.

    Plummer Vinson Syndrome (Triad Symptoms) SAD Stomatitis, Atrophic Glossitis, Dysphagia)

    iii. Koilonychia - Spoon shaped or concave finger nailsiv. Angular cheilosis/Cheilitis inflammation of the corners of the lipsv. Brittle hair and fingernailsvi. Tinnitus

    vii. Picaviii.

    - PathophysiologyPoverty inadequate dietary intake lesser ferric iron less storage of Ferritin in RES depletion

    of iron stores ultimately depletes the iron in the hemoglobin [1. Causing dec oxygen carryingcapacity of the RBC 2. Dec the RBC massmicrocytic cell] General S/Sx of Anemia

    Excessive intake of coffee, carbonated and alcoholic drinks GIT ulceration Self medication of

    antacid (Mg trisilicate) Coats GIT mucosa impaired absorption of FE lesser ferric iron less

    storage of Ferritin in RES depletion of iron stores ultimately depletes the iron in the hemoglobin

    [1. Causing dec oxygen carrying capacity of the RBC 2. Dec the RBC mass microcytic cell]

    General S/Sx of Anemia

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    NCM 202 NCM of Clients with Hematologic Disorders Ms. Jean Javie

    - Diagnostic Testi. CBC

    Microcytic Hypochromic Dec RBC Dec Hgb Dec Hct

    ii. Se Ferritin Level decreased NV: Male = 15-200micrograms/mL Female = 11-200micrograms/mL

    iii. Se Iron Level Decreased to 10mcg/dL NV: 50-150mcg/dL

    iv. Total Fe Binding Capacity Increased in patient Inc to 350-500 mcg/dL NV: 250-350mcg/dL

    - Medical Managementi. Iron therapy (Ferrous sulfate, ferrous Gluconate, Ferrous fumarate)ii. Vitamin C

    iii. O2 therapyiv. Restv. Blood transfusionvi. Erythropoietin injection

    vii. Treatment of the underlying condition-

    Nursing Diagnosis Imbalanced nutrition less than body requirements (Goal: Attain and maintain adequate

    nutrition)

    o Give small frequent feedings with the appropriate time for the client to finish the mealo Eat foods rich in Fe and inc Vit C intakeo Weigh client dailyo Refer to dietitian or nutritionisto Avoid spicy foodo Give a well balanced dieto Avoid hot foodo Feed exhausted cliento Encourage or provide pleasant environment for eating

    Activity intolerance related to fatigabilityo Assist in ADLo Adequate rest

    Risk for injury related to dizziness Impaired gas exchange related to decreased O2 carrying capacity

    o Pulse oximetry, capillary refillo Refer palloro MIO

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    o Adequate resto Deep breathing, skin discoloration

    Impaired skin integrityo Cocoa butter lotion

    - Patient education and home care: How to take Fe Supplements1. Take Fe on an empty stomach (1h before meals and 2 hours after meals)2. Antacids or dairy products should not be taken with Fe3. If Fe causes gastric upset, the following schedule may work better

    Start with only 1 tablet/day for a few days then increase to 2 tablets per day. This gradually

    permits the body to adjust to the iron.

    4. Inc Vit C5. High fiber to decrease constipation6. If liquid form of Fe is tolerated, use straw or spoon at the back of the mouth. Rinse thoroughly.

    Fe salts can discolor or stain the teeth.

    B. Folic Acid Deficiency AnemiaFolic Acid

    - Needed of r DNA synthesis leading to RBC formation and maturation- Sources are GLV, grains, liver, legumes, citrus fruits, yeast products, nuts- Best absorbed in the proximal small intestineCauses

    1. Inadequate intake2. Impaired digestion and utilization of Folic Acid (as in Malabsorption Syndrome, Celiac Dse, Crohns

    Dse, Steatorrhea)

    3. Anorexia Nervosa (Malnutrition)4. Chronic Alcoholism (blocks erythropoiesis, by blocking response of the bone marrow)5. A bacterium that competes need for Folic Acid.6. TPN7. Hemodialysis8. Overcooking9. Excessive demand for Folic Acid (Pregnancy, Childhood, Adolescence)10. Infants have limited storage capacity11.Drugs that impedes or hinders Folic Acid absorption.

    Chemotherapeutic drugsPurine Analogues Azathioprine (Immunosuppressants)

    Pyrimidine Analogues 5 FU(Fluorouracil)

    Ribonucleic Acid Inhibitor Hydroxyurea

    Anticonvulsant Drugs Phenytoin, Phenobarbital

    Oral Contraceptive Drugs

    Hypoglycemic Agents (Metformin)

    Antibiotics - Neomycin

    Anti-inflammatory Drugs Coldricine

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    NCM 202 NCM of Clients with Hematologic Disorders Ms. Jean Javie

    Signs and Symptoms

    Assessment Data

    1. Anemia Reduced RBC count less than 3M/mm3. Impairs O2 carrying capacity S/Sx

    Weakness

    Pallor

    Dyspnea

    Palpitations (fatigue)

    2. Gastrointestinal S/Sx sore mouth Smooth beefy red tongue Constipation Diarrhea Weight loss Indigestion

    3. Jaundice Rupture and hemolysis of abnormally large RBC as they pass through capillaries

    Pathophysiology

    Inadequate Dietary Intake Less folic Acid Decrease DNA synthesis Dec RBC Formation and

    maturation Macrocytic RBC (Jaundice, Retinal Hemorrhage, Pruritus) dec RBC count decrease O2

    carrying capacity GIT S/Sx Anemia (pallor, dyspnea, palpitations, easy fatigability

    Dx Test

    1. Se Folate

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    3. Vitamin C can be given4. Multivitamins5. BT in cases of sever anemia

    Nursing Management

    1. Altered bowel elimination: constipation2. Impaired gas exchange3. Altered nutrition less than body requirements4. Impaired body image5. Activity intolerance6. Risk for impaired skin integrity7. Anxiety

    C. Pernicious Anemia- Vit. B12 Deficiency- B12 contains cobalt necessary for RBC division and maturation, contains enzyme that moves folic acid

    into the cell where DNA synthesis occurs; necessary for normal system functioning- RBC are misshaped (oval, macrocytic, membranes are thin, lesser O2 carrying capacity, prone to

    hemolysis)

    - B12 need presence of intrinsic factor secreted by parietal cells of the stomachs glandular mucosa- B12 is absorbed in the distal i leum

    Sources are liver, red meat, fish, egg, milk and dairy products- Autoimmune disease, macrocytic, caused by a deficiency of B12 d/t lack of intrinsic factorCauses

    1. Lack of Intrinsic Factora. Atrophy of the stomachs glandular mucosa

    o Hereditary d/t Human Leukocyte Antigen A (usually in Type A blood)b. Prolonged Fe Deficiencyc. Auto-immune 90% of patients have antibodies react specifically against parietal cells; 60%

    have anti-intrinsic factor antibodies

    d. Gastric SurgeryS/Sx

    Same with folic acid deficiency anemia; client has involvement of the stomach

    Decrease gastric acid secretions hypochlorhydria, achlorhydria

    Gastric lesions involving atrophy of the gastric mucosa and reduced HCl (needed for digestion) as well as

    the intrinsic factor

    Neurologic: Degeneration of the dorsal and lateral column of the spinal cord, peripheral nerves and brain

    (S/Sx: Paresthesia, paralysis, irritability, depression, psychotic behavior, ataxia)

    Macrocytic, hypochlorhydria, achlorhydria, fatal outcome without lifelong B12 injections

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    NCM 202 NCM of Clients with Hematologic Disorders Ms. Jean Javie

    Dx Test

    1. CBC dec RBC2. Total Fe Binding Capacity inc3. Se Fe4. Se Ferritin Level5. Gastric analysis6. Neurological Exams (Romberg Test)7. Peripheral Blood Smear8. Bone Marrow Exam9. Most Definitive Test

    Schilling Test: NPO for 12h; administration of Radioactive B12 0.2-2mcg; 2h after, IM B12, in order

    to aid in Renal Excretion of Radioactive B12 through 24h urine collection.

    NV: 7-8 to 10% Excreted Radioactive B12

    MD will repeat test if result is pathologic, Radioactive B12+IF, after 2h, IM B12, 24 hour urine

    collection.

    Medical Management

    1. Lifelong B12 Therapy, IM should be understood by the patient (cyanocobalamin andhydroxycobalamin)

    Reticulocytes will then increase.

    2. Patient will be given with Fe and Vit C3. Blood Transfusion4. Multivitamins5. Digestants to enhance metabolism (HCl) 4-10mL well diluted in H2O tid with meals during 1st week

    of B12 therapy

    6. Vit C, Fe, Folic Acid

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    NCM 202 NCM of Clients with Hematologic Disorders Ms. Jean Javie

    Nursing Diagnosis

    1. Risk for injury related to neurologic S/Sx AEB numbness of the hands and feet2. Self care deficit

    Nursing Management

    1. Risk for injuryRaise side rails, assist in ambulation, hand rails, assistive devices (walker, wheel chair, well lighted

    room), and avoid exposure to extreme temperature

    2. Impaired Skin integrity cocoa butter lotion, turn q1-2h, ROM, massage bony prominences3. Assist in bowel elimination, catheter care, bladder training, explain chronicity of the client, have

    regular check up because the client is prone to develop gastric CA

    D. Aplastic AnemiaE.