NCM 202 NCM of Clients with Hematologic Disorders Ms.

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

Course Content A. Overview of the Anatomy and Physiology of the Hematologic System 1. Bone Marrow 2. Reticuloendothelial System 3. Blood a. Plasma b. Blood Cells (Cellular Elements) i. RBC ii. WBC iii. Platelet B. Blood Group Classification 1. AVO System 2. Rh System C. Disorders Affecting RBC Production 1. Decrease RBC Production a. Anemia i. IDA ii. Folic Acid Deficiency Anemia iii. Pernicious Anemia iv. Aplastic Anemia v. Thalasemia 2. Increase RBC Production a. Polycythemia D. Disorder Affecting WBC Production a. Agranulocytosis b. Leukemia

NCM 202 NCM of Clients with Hematologic Disorders Ms. Jean Javier E. Disorder Affecting Platelet Production (Coagulation Disorders) a. Thrombocytopenia b. Hypoprothrombinemia c. Disseminated Intravascular Coagulation d. 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. Bone Marrow - Soft materials that fill the central core of the bones where hematopoiesis takes place. a. Red Marrow aggregates of hematopoietic cells interspaced with sinusoidal capillaries b. 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

NCM 202 NCM of Clients with Hematologic Disorders Ms. Jean Javier 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 that form 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 sterile C. 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 <6micrometers microcytic

NCM 202 NCM of Clients with Hematologic Disorders Ms. Jean Javier 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 circulation o 5% stays in the endothelial lining o 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

NCM 202 NCM of Clients with Hematologic Disorders Ms. Jean Javier 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 A B AB O Antigen in RBC A B AB none Antibody in Plasma Anti B Anti A None Anti AB Donor/Donors O, A O, B A,B, AB, O O

Rh System 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 - <6g/dL - Multisystem involvement a. Integumentary Pale, pruritus (because of hemolysis presence of bile salts) b. Eyes Icteric conjunctiva, sclera, retinal hemorrhage (RBC are macrocytic), blurred vision Considered as the D antigen If present. Rh+, of none RhReaction is non spontaneous Coombs test, to check for the presence of Rh incompatibility RhoGAM within 24h

NCM 202 NCM of Clients with Hematologic Disorders Ms. Jean Javier 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 amenorrhea j. 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

NCM 202 NCM of Clients with Hematologic Disorders Ms. Jean Javier 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) Antacids magnesium 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 Symptoms i. 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 nails iv. Angular cheilosis/Cheilitis inflammation of the corners of the lips v. Brittle hair and fingernails vi. Tinnitus vii. Pica viii. Pathophysiology Poverty 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 carrying capacity of the RBC 2. Dec the RBC mass microcytic 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

NCM 202 NCM of Clients with Hematologic Disorders Ms. Jean Javier Diagnostic Test i. 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 Management i. Iron therapy (Ferrous sulfate, ferrous Gluconate, Ferrous fumarate) ii. Vitamin C iii. O2 therapy iv. Rest v. Blood transfusion vi. 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 meal o Eat foods rich in Fe and inc Vit C intake o Weigh client daily o Refer to dietitian or nutritionist o Avoid spicy food o Give a well balanced diet o Avoid hot food o Feed exhausted client o Encourage or provide pleasant environment for eating Activity intolerance related to fatigability o Assist in ADL o Adequate rest Risk for injury related to dizziness Impaired gas exchange related to decreased O2 carrying capacity o Pulse oximetry, capillary refill o Refer pallor o MIO

NCM 202 NCM of Clients with Hematologic Disorders Ms. Jean Javier o Adequate rest o Deep breathing, skin discoloration Impaired skin integrity o Cocoa butter lotion - Patient education and home care: How to take Fe Supplements 1. Take Fe on an empty stomach (1h before meals and 2 hours after meals) 2. Antacids or dairy products should not be taken with Fe 3. 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 C 5. High fiber to decrease constipation 6. 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 Anemia Folic 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 intestine Causes 1. Inadequate intake 2. 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. TPN 7. Hemodialysis 8. Overcooking 9. Excessive demand for Folic Acid (Pregnancy, Childhood, Adolescence) 10. Infants have limited storage capacity 11. Drugs that impedes or hinders Folic Acid absorption. Chemotherapeutic drugs Purine 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

NCM 202 NCM of Clients with Hematologic Disorders Ms. Jean Javier 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 <4Ng/mL 1Ng 1 billionth of a gram NV: 7-20Ngm/mL 2. CBC Macrocytic Some can be hyperchromic and abnormally larger 3. Bone Marrow Exam (Dec RBC count) 4. Blood Smear Hyperchromic, macrocytic, dec Hct/Hgb\ 5. No neurologic S/Sx except if person has problems with B1, Calcium, Magnesium which can be associated with chronic alcoholism 6. Responds to therapeutic trial of 50-100mg of Folic Acid IM / 10 days= inc in Reticulocyte count of client. Gastric analysis to differentiate from Pernicious Anemia; client has HCl secretions Medical Management 1. Folic Acid therapy 0.1-5 mg daily (Folinic Acid/Leucovorin Ca) 2. Vitamin C can be given

NCM 202 NCM of Clients with Hematologic Disorders Ms. Jean Javier 3. Vitamin C can be given 4. Multivitamins 5. BT in cases of sever anemia Nursing Management 1. Altered bowel elimination: constipation 2. Impaired gas exchange 3. Altered nutrition less than body requirements 4. Impaired body image 5. Activity intolerance 6. Risk for impaired skin integrity 7. 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 ileum 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 factor Causes 1. Lack of Intrinsic Factor a. Atrophy of the stomachs glandular mucosa o Hereditary d/t Human Leukocyte Antigen A (usually in Type A blood) b. Prolonged Fe Deficiency c. Auto-immune 90% of patients have antibodies react specifically against parietal cells; 60% have anti-intrinsic factor antibodies d. Gastric Surgery S/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

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

Dx Test 1. 2. 3. 4. 5. 6. 7. 8. 9. CBC dec RBC Total Fe Binding Capacity inc Se Fe Se Ferritin Level Gastric analysis Neurological Exams (Romberg Test) Peripheral Blood Smear Bone Marrow Exam 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 and hydroxycobalamin) Reticulocytes will then increase. 2. Patient will be given with Fe and Vit C 3. Blood Transfusion 4. Multivitamins 5. 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

NCM 202 NCM of Clients with Hematologic Disorders Ms. Jean Javier Nursing Diagnosis 1. Risk for injury related to neurologic S/Sx AEB numbness of the hands and feet 2. Self care deficit Nursing Management 1. Risk for injury Raise 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 prominences 3. 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 Anemia E.