Care Of Patients With Hematologic System Problems PDF

CARE OF PATIENTS WITH PROBLEMS OF THE PLASMA 55 % HEMATOLOGIC SYSTEM Proteins (7%) Albumins control/ hold...

CARE OF PATIENTS WITH PROBLEMS OF THE PLASMA 55 % HEMATOLOGIC SYSTEM Proteins (7%) Albumins control/ hold water inside so that (58 %) it will not go outside; to prevent Functions and Composition of Blood edema 1. Blood helps maintain homeostasis in several ways (↓albumins- edema) Transport of gases, nutrients, waste Globulins carries o2 products (38 %) Fibrinogen for blood clotting Transport of processed molecules (4 %) Sunlight !!! → Skin → 7 Dehydrocholesterol (converted Other solutes (2%) into) →Pre-vitamin D or D3 → Liver → Kidney → VITAMIN D (stored in small intestine; reason to take up calcium) 8. Blood is a sticky, opaque fluid with a metallic Transport of regulatory molecules taste Regulation of pH and osmosis 9. Color varies from scarlet to dark red Maintenance of body temperature 10. The pH of blood is 7.35–7.45 Protects against foreign substances such 11. Temperature is 38°C as microorganisms and toxins 12. Blood accounts for approximately 8% of body weight Blood clotting prevents fluid and cell loss 13. Average volume: 5–6 L (1.5 gallons) for males, and and is part of tissue repair 4–5 L for females 2. Blood is a connective tissue consisting of plasma and formed elements PLASMA 3. Blood is the body’s only fluid tissue 4. It is composed of liquid plasma and formed elements Pale yellow fluid containing over 100 solutes 5. Formed elements include: Mostly water (91%) Erythrocytes, or red blood cells (RBCs) Contains proteins (7%) Leukocytes, or white blood cells (WBCs) - Albumin (58% of the plasma proteins) Platelets Helps maintain osmotic pressure 6. Hematocrit: the percentage of RBCs out of the total Globulins (38% of the plasma proteins) blood volume - Immunity: antibodies and complement 7. Total blood volume is approximately 5 liters - Transport: bind to molecules such as hormones - Clotting Factors Fibrinogen (4% of the plasma proteins) - Converted to fibrin during clot formation Other substances (2%) Ions (electrolytes): sodium, potassium, calcium, chloride, bicarbonate Nutrients: glucose, carbohydrates, amino acids Waste products: lactic acid, urea, creatinine Respiratory gases: oxygen and carbon dioxide Composition of Plasma Water- Acts as a solvent and suspending medium for blood components Proteins- Maintain osmotic pressure (albumin), destroy foreign substances (antibodies and complement), transport molecules (albumin, globulins) and form clots (fibrinogen) Ions- Involved in osmotic pressure (sodium and chloride ions), membrane potentials (sodium and  potassium ions) and acid-based balance (hydrogen, hydroxide and bicarbonate ions) Nutrients- Source of energy and "building blocks" of more complex molecules (glucose, amino acids, triglycerides) Gases- Involves in aerobic respiration (oxygen and carbon dioxide) Waste Products- Break down products of protein metabolism (urea and ammonia salts), red blood cells (bilirubin) and aerobic respiration (lactic acid) Regulatory Substances- Catalyze chemical reactions (enzymes) and stimulate or inhibit many body functions (hormones) FORMED ELEMENTS Erythrocytes or red blood cells (RBCs) - About 95% of formed elements - RBCs have no nuclei or organelles RED BLOOD CELLS - Normal: 4.2- 6 Leukocytes or white blood cells (WBCs) Biconcave discs, anucleate, essentially no - Most of the remaining 5% of formed elements organelles - Only WBCs are complete cells RBCs are dedicated to respiratory gas transport - Five types of WBCs - Filled with hemoglobin (Hb), a protein that - Normal: 4,500- 12,000 functions in gas transport Platelets RBCs are an example of how structure fits function - Just cell fragments - Biconcave shape has a huge surface area - Most formed elements survive in the relative to volume bloodstream for only a few days  Structural characteristics contribute to - Normal: 150,000- 450,000 its gas transport function PRODUCTION OF FORMED ELEMENTS Biconcave shape also allows RBCs to bend or fold around their thin center Most blood cells do not divide but are renewed by  Gives erythrocytes their flexibility stem cells (hemocytoblasts) in bone marrow  Allow them to change shape as Hematopoiesis: blood cell production - necessary Occurs in different locations before and after birth Erythropoiesis is the production of RBCs Fetus- Liver, thymus, spleen, lymph nodes, and red bone marrow A hemocytoblast is transformed into a After birth- In the red bone marrow of the proerythroblast axial skeleton and girdles, epiphyses of the Proerythroblasts develop into early erythroblasts humerus and femur, some white blood cells The developmental pathway consists of three are produced in lymphatic tissues phases Hemocytoblasts give rise to all formed elements 1. Ribosome synthesis in early erythroblasts - Growth factors determine the type of formed 2. Hb accumulation in intermediate element derived from the stem cell erythroblasts and late erythroblasts 3. Ejection of the nucleus from late erythroblasts and formation of reticulocytes - Reticulocytes are released from the red bone marrow into the circulating blood, which contains ~1-3% reticulocytes Reticulocytes then become mature erythrocytes  Circulating erythrocytes: The number remains  Oxygen content determines blood constant and reflects a balance between RBC color » Oxygenated: bright red » production and destruction Deoxygenated: darker red - Too few RBCs leads to tissue hypoxia - Globin molecules transport carbon dioxide - Too many RBCs causes undesirable blood One RBC contains 250 million Hb groups thus it can viscosity carry 1 billion molecules of O2 Erythropoiesis is hormonally controlled and Normal: 12- 16 g/dl (*but it also depends on gender) depends on adequate supplies of iron, amino acids, and B vitamins (folate and B12) Erythropoietin (EPO) release by the kidneys is triggered by - Hypoxia due to decreased RBCs - Decreased oxygen availability - Increased tissue demand for oxygen Enhanced erythropoiesis increases the - RBC count in circulating blood - Oxygen carrying ability of the blood HEMOGLOBIN BREAKDOWN !!! Erythropoietin- stimulates Bone Marrow Bone Marrow- produces RBC RBC- ↑ O2 in the body *TO REMEMBER !!! Sickle Cell- 20 days life span Pancytopenia- Low RBC, WBC, and Platelet in Bone Marrow HEMOGLOBIN (HB) Accounts for about a third of the cell’s volume 1. The goblin chains of hemoglobin are broken down to Consists of: individual amino acids and are metabolized or used - The protein globin, made up of two alpha and to build new proteins two beta chains, each bound to a heme group 2. Iron is released from the heme of hemoglobin. The - Each heme group bears an atom of iron, which heme is converted into bilirubin can bind to one oxygen molecule 3. Iron is transported in the blood to the red bone - Heme molecules transport oxygen (Iron is marrow and used in the production of new required) hemoglobin 4. Bilirubin is transported in the blood to the liver  SEVERITY 5. Bilirubin is excreted as a part of the bile into the small Normal -12- 16 g/dl intestine Mild- Hb 11 g/dl 6. Bilirubin derivatives contribute the color of the feces → asymptomatic, mild tachycardia with increased or are reabsorbed from the intestine into the blood activity and excreted from kidneys in the urine Moderate- Hb 7- 10 g/dl → signs and symptoms become evident Important !!! Severe- 4-6 g/dl Hemoglobin- split into amino acid (building block of → decompensatory manifestations protein) Cardiovascular collapse- ≤ 3 g/dl Iron → Bone Marrow (for RBC production) Bilirubin → Liver → Bilirubin in BILE (not all CLINICAL MANIFESTATIONS bilirubin will go into bile, kidney or feces) → → Depends on the severity of tissue hypoxia and the Intestine → Kidney (*gallstones- excess; need to effectiveness of compensatory mechanisms remove) employed by the body Compensatory Mechanisms: ANEMIA → Tachycardia → Tachypnea It is not a disease but rather a term used to describe → The hemoglobin releases O2 to the tissue more a decreased O2-carrying capacity of the blood readily caused by an underlying disease or injury → Activation of the RAAS → sodium and water retention → increased blood volume and BP It is manifested by an abnormally low RBC count → Increased erythropoiesis or hemoglobin or both When compensatory mechanisms decline: !!! MECHANISMS → Pallor o Fatigue → CNS: dizziness/fainting, headache, light Increased RBC loss headedness, slow thought process, decreased Increased destruction attention span, restlessness, apathy Deficient production → Heart: palpitations, cardiac murmur, chest pain, CHF to susceptible individuals → Lungs: dyspnea progressing from exertion to rest MORPHOLOGY → Anorexia Normocytic- normal size → Poor tolerance to cold Microcytic – small erythrocytes Macrocytic – large size cells Normochromic- normal color Hypochromic – pale RBCs Megaloblastic- large RBC + protein in DNA synthesis *Vitamin B12 Deficiency/ Pernicious Anemia *Folic Acid Deficiency ETIOLOGY Hypo proliferative anemia decreased production Deficiency anemias Decreased number of erythrocyte precursor Examples: MANAGEMENT - Iron Deficiency Anemia Laboratory investigations - Megaloblastic Anemia (Folic Acid Treat the underlying cause Deficiency, Pernicious Anemia/ Vitamin Blood transfusions B12 Deficiency) Nutritional supplements - Aplastic Anemia (Pancytopenia) Hemolytic anemia → increased destruction → Examples: - Sickle Cell Anemia/ Crisis  NURSING DIAGNOSIS HYPO PROLIFERATIVE ANEMIA ACTIVITY INTOLERANCE Promote optimal activity and protect from injury – let patient participate in self care IRON DEFICIENCY ANEMIA Provide adequate rest period – replan activities that cause fatigue Avoid sudden movement A type of anemia marked by inadequate supply of DECREASED CARDIAC OUTPUT iron for optimal formation of hemoglobin → smaller Monitor: V/S and signs of heart failure, Edema, cells with less color neck vein distention, decreased urine output, Most common type of anemia palpitations, chest pain NURSING MANAGEMENT Increase O2 demand Adequate rest Quiet activities O2 supplement may be necessary Fowler’s position Support RBC production Nutritious diet Vitamin and iron supplement A type of anemia caused by LOW IRON Small frequent meals CAUSES: PAL !!! Prevention of infection → Poor Intake- (*low socioeconomic status) → Absorption Problem- (*ex. pregnancy, BLOOD LOSS ANEMIA lactation, rapid growth, infants↑ deman of Acute Iron) Hypovolemia → circulatory collapse → Losing Iron RBC indices are normal  Gastric resection or intestinal If there are sufficient Fe stores, normal RBC resection count returns to normal after 3 to four weeks  Chronic diarrhea Chronic  (*profuse bleeding, menstruation, Does not affect the blood volume but it will lead surgery) to Fe deficiency and low hemoglobin when Fe → BLOOD LOSS (major cause) stores are already depleted  Bleeding (2ml of whole blood No symptoms until Hb < 8g /dl contains 1 mg of iron) !!! RBC indices: microcytic, hypochromic  Major sources of chronic blood loss: GI and GU systems, menstrual cycle, parasitism SIGNS AND SYMPTOMS: LOW IRON !!! → Lethargic → Over exerted easily → Weird food cravings- (*Pica- nail biting, eating of clay or hair) → White face → Inflammation of the tongue- (*Glossitis or Vinson-Plummer’s Syndrome) → Increased HR → Reduced Hgb level → Observe changes in RBC → Nail changes - (*KOILONYCHIA- coarsely, spoon shaped, brittle nails) !! → Neuro changes- (*can’t concentrate, dizziness, headache, etc.)  NURSING MANAGEMENT: PERNICIOUS ANEMIA/ VITAMIN B12 DEFICIENCY → Monitoring of blood levels, signs and symptoms A form of B12 Deficiency Anemia that is an → Educate IRON supplement autoimmune (antibodies) condition where the body → Take on empty stomach (*absorbed does not produce INTRINSIC FACTOR which plays better) a vital role in GI SYSTEM= absorbing vitamin b12 → Take it with vitamin C (*enhances PARIETAL CELLS (*seen in small intestine) → absorption) INTRINSIC FACTOR (a protein responsible for the → Don’t take it with milk or antacids (after absorption of vitamin b12 in ILLEUM) 2 hours interval + it inhibits absorption) Very large and oval shaped RBC → Dark black stool or dark green CAUSES: → Liquid preparation (mix in drink, drink → Antibodies attack parietal cells with straw *to prevent staining, after- → Genetics- (*some do not have parietal gargle ) cells) → Side effect; constipation → Elderly- (*decrease acid in the body, → IM ex. INFERRON: can’t secrete parietal cells= ↑ need acid for  Give deep IM, Z-track do not parietal cells to produce IF) !!! massage (*to prevent muscle → Stomach surgery staining) → GI diseases- (*ex. Celiac disease-  O2 inhalation malabsorption, parietal cell malfunction)  BT as needed o Nutritional SIGNS AND SYMPTOMS: PERNICIOUS !!! supplements  Transfusion of packed RBCs → Pale Foods Rich In Iron: EAT LOTS OF IRON !!! → Energy gone → RED BEEFY TOUNGE (*pathognomonic Egg yolks pOtato sign) !!! Apricot Fish → Numbness/ tingling hands and feet Tofu → Intestinal issue Legumes, leafy vegetables Iron fortified cereal → Confusion Oyster Red meat, raisins → Increased sadness Tuna pOultry → lOss of appetite Sardines Nuts → Unsteady gait → Shortness of breath DIAGNOSTICS: AFFECTED SYSTEMS: → CBC- ↓Hb, hematocrit, RBCs ✓ → Nervous System → ↓MCV and MCH → Cardiovascular System (Heart) → Peripheral smear – microcytic and → Gastrointestinal Tract hypochromic RBCs ✓ DIAGNOSTICS → ↑ serum iron binding capacity → CBC- Low Hb, RBCs, Platelets, and WBCs → ↓ serum iron and ferritin levels → Peripheral blood smear- (*size and shape of RBC) MEGALOBLASTIC ANEMIA: Pernicious & Folic Acid → VIT B12 level- low serum levels of Vit. B12 → Intrinsic factor essay Caused by impaired DNA synthesis → defective → Bone marrow biopsy- (*increase or maturation of RBCs → large abnormal RBCs. decrease production of RBC) Because megaloblast are defective they are → Schilling’s test (radioactive, non- destroyed by the spleen radioactive, 24 hour urine collection) Folic acid and Vit. B12 are essential in the synthesis of DNA Other cells from the myeloid stem cell are also diminished because of altered DNA synthesis Vitamin B12 is also important for the integrity of the myelin sheath of nerve cells Stage 1: WBCs and platelets are also diminished because Radioactive Vitamin B12 (liquid intake) of altered DNA synthesis Non-radioactive Vitamin B12 IM, after 1hr Vitamin B12 is also essential for the integrity of the 24hr urine collection myelin sheath of nerve cells +” if no radioactive Vit. B12 is found in the urine Megaloblast have shorter life span; destroyed in the Stage 2 spleen in increased rate → slight jaundice Radioactive Fruit, dairy products, cereal, most abundant in - Intrinsic factor green leafy vegetables 24hr urine collection Stage 3 APLASTIC ANEMIA Radioactive - Antibiotic + B12 (*if bacteria is the cause) - 7 days prior before 24hr urine collection Any form of anemia caused by aplasia of the BONE Stage 4 MARROW Radioactive Aplasia – defective development of an organ or - Pancreatic Enzyme +B12 complete absence due to failure of development - 3 days prior (*check if there is a problem in There is depression or cessation of activity of all pancreas; enzyme could be the reason) blood-producing elements → PANCYTOPENIA Leukopenia – decreased number of WBC → high → Tubeless gastric analysis- risk for infection  *check hydrochloric acid in the body Thrombocytopenia – decreased number of  if not in color BLUE= negative/ problem platelets → bleeding tendency in parietal cell (*nursing management: assess bleeding & pt. should  Diagnex/Azuressin given PO) not be expose in communicable disease) NURSING MANAGEMENT Anemia – decreased number of RBC → Replace Vit B12 (IM weekly, monthly, for They die from hemorrhage and infection life *1mg HYDROCOBALAMIN) !!! + once DIAGNOSTICS: a month= for the rest of the life → CBC → Oral CYANOCOBALAMIN → Peripheral blood smear → Rich in IRON foods → Bone marrow transplant → Oral hygiene MANAGEMENT: → Vitamin B12 (poultry, organ meats, clams, → Removal of the causative agent beef, sardines, cereal, tuna, salmon, dairy → Can be cured by a bone marrow transplant products) (BMT) or peripheral blood stem transplant (PBSCT) FOLIC ACID DEFICIENCY → Immunosuppressive therapy – to prevent lymphocytes from destroying the stem cells COMMON CAUSES: ex. Cyclosporine, androgens, → Elderly antithymocytes globulin (ATG) → Poor nutrition → Corticosteroids as immunosuppressive agent – not very useful → Malabsorption syndromes and bowel disorders → Supportive therapy  PRBC transfusion → Cancer  Platelet transfusion → Anti-Convulsant Drugs that impede the NURSING MANAGEMENT: absorption of folic acid  Methotrexate → Careful assessment and management of  Oral contraceptives the complications of pancytopenia  Antiseizure agents  Assess carefully for signs of infection and bleeding → Alcohol abuse and anorexia → Prevention of Infection → Hemodialysis  Use of protective isolation With same clinical manifestations as that of Vit.  Meticulous hygiene B12 deficiency but without the neurologic  Oral care symptoms  Monitor invasive lines for signs of Absorbed in the JEJUNUM infection  Avoidance of catheterization   Instruction of handwashing HEMOLYTIC ANEMIA  Control visitor → Prevention of bleeding  Monitor invasive lines, feces, urine Premature destruction of RBCs: decreased life  Minimize venipuncture and IM span → fewer circulating erythrocytes → decreased injections available O2  Use of soft sponges for oral care CAUSES: → Care for anemic patient → INTRINSIC → Monitor for side effects of therapy  Usually inherited  Ex. hypersensitive reaction (ATG);  Defects of the cell membrane or of long-term effects of cyclosporine the hemoglobin including renal and liver failure (hemoglobinopathy sickle cell → Patient and family education about anemia, G6PD) disease and assisting in developing  Abnormal cells don’t function well positive coping strategies and are destroyed by the MPS GUIDELINES OF SAFE PRACTICE !!! → EXTRINSIC Infection Hemorrhage Fatigue  Drugs − Methyldopa (Aldomet) Good Observe for Take frequent  associated with production of handwashing signs such as rest between antibodies against RBCs technique bloody urine ADLs  Bacteria/toxins (sepsis) and stool,  Antibodies (incompatible blood Avoid crowds petechiae, and Avoid excessive transfusion, autoimmunity) − and persons report work load; ask Physical trauma (heart valve with infection immediately for assistance prosthesis) Extra vascularly - The spleen removes erythrocytes Avoid sharing Use of soft Increase time from the circulation at an accelerated rate utensils toothbrush; necessary for Intravascularly - The erythrocytes lyse and spill avoid use of routine care Skin care dental floss contents into the plasma Report signs of COMMON LABORATORY RESULTS: Avoid eating Keep mouth increased → Elevated unconjugated bilirubin raw foods clean fatigue → Elevated reticulocyte count Report immediately Avoid enemas SICKLE CELL ANEMIA early signs of or rectal infection insertions Abnormal hemoglobin shape in RBC Avoid picking Normal: Hemoglobin A or blowing nose Hemoglobin S- Sickle cell anemia (*sensitive to low forcibly oxygen= 3S or sickle shaped, stiff and sticky) !!! Weak- 20 days life span (*SPLEENOMEGALY !!!) +congested spleen, can’t fight infection (WBC) = prone to infection Autosomal recessive: 25 % chance (*carrier) DIAGNOSTICS: → Sickledex Test- a small blood sample; It looks at how many red blood cells have the sickle shape in the body but cannot say if it is carrier or already a disease. → Hemoglobin electrophoresis- small blood sample is taken and can say if it is carrier or not NO SIGNS AND SYMPTOMS; however, when exposed to dehydration, high altitudes, stress, illness, surgery, etc. or anything that increases oxygen demand in body= sickle shaped, stiff and sticky (SICKLE CELL CRISIS) SICKLE CELL CRISIS Significant blood loss, surgery, trauma, etc. Illness Climbing or flying to HIGH ALTITUDES Keeping continued STRESS Low fluid intake (DEHYDRATION) Elevated temperature ( fever, exercise) or COLD TYPES Vaso occlusive- impede blood flow to the different organs and tissues in the body Sequestration crisis- pulling of sickle cell; occlude, then malfunction of organ Aplastic SIGNS AND SYMPTOMS: PHEDSALGPS → Pain → Hyper hemolytic → Eye Problem → Dactylitis- (*common in infant with SCC) → Splenomegaly- (*infection) → Gallstones → Anemia → Leg Ulcer- (*elevate the affected area) → Pneumonia or PNA- chest syndrome, chest pain, cough, fever → Stroke MANAGEMENT: PHOB HSFV → Pain Management- (*opioids) → Hydration → Oxygen → Bedrest → Hydroxyurea- (*drug used in breast cancer, SCC side effect: increase fetal hemoglobin; prevent sickling) → Stem cell transplant → Folic acid, no iron → Vaccines THALASSEMIA MANAGEMENT: → Regular Transfusions  Hgb goal post transfusion of Inherited disorder of hemoglobin synthesis 10g/dl o needed approximately Primarily affects persons of Mediterranean origin, but every 4 weeks it also occurs in southeast Asians, Chinese, and → Chelation Africans  Desferoxamine (Desferal There is a decreased synthesis of one of the  SQ over 10-12 hours, 5-6 days/wk globin chains of hemoglobin (β chain is most  Avoid if < 3 years old because of affected) → decreased synthesis of hemoglobin and toxicity an accumulation of the α chain in the erythrocyte →  Side effects: ototoxicity with high hemolysis frequency hearing loss, retinal TYPES: changes, bone dysplasia/truncal → Thalassemia Minor shortening  The heterozygous state  Oral Chelator? So far, no safe  Mild, usually asymptomatic alternative  Usually presented with mild → Folate supplementation anemia  Splenectomy (for hypersplenism)  No therapy is required as indicated by: → Thalassemia Major  Dramatic increase in transfusion  The homozygous state requirements  Also called Cooley’s anemia  Massive size that interferes with  Characterized by severe anemia breathing and nutrition o severe  RBCs are hypochromic and pain microcytic  Avoid before 5 years if at all  Electrophoresis is the definite possible diagnostic test  Immunize with pneumovax and  Growth failure usually begins menigovax pre - splenectomy between ages 10 and 12  Post splenectomy will need  Death usually occurs during the penicillin prophylaxis for life young adult years (17 – 30) Stem cell transplantation Bone Marrow Transplant (cure) Educate family SPHEROCYTOSIS HEMOPHILIA Most common problem of alteration in erythrocyte Platelet Defects shape Type A- defective factor VIII Hereditary, rare – 1 in 5000 persons Type B- defective factor IX (Christmas Disease) Characterized by a membrane abnormality → Classification based on tendency to develop osmotic swelling →susceptibility to destruction in the bleeding spleen Its capability to carry O2 is maintained Normal- 50-100 % Diagnostics Mild- >5% Moderate- 1-5 % Red cell survival time Peripheral blood smear Severe-