Assessment of Hematologic Function and Treatment Modalities PDF

Assessment of Hematologic Function and Treatment Modalities Prepared by: Judd Wilson T. Pasculado RN Lecturer: Anthony C. Barrera, RN ANATOMIC AND PHYSIOLOGIC OVERVIEW ANATOMIC AND PHYSIOLOGIC OVERVIEW The hematologic system consists of the blood and the sites where blood is produ...

Assessment of Hematologic Function and Treatment Modalities Prepared by: Judd Wilson T. Pasculado RN Lecturer: Anthony C. Barrera, RN ANATOMIC AND PHYSIOLOGIC OVERVIEW ANATOMIC AND PHYSIOLOGIC OVERVIEW The hematologic system consists of the blood and the sites where blood is produced, including the bone marrow and the reticuloendothelial system (RES) Plasma is the fluid portion of blood contains various proteins, as albumin, globulin, fibrinogen, and other factors necessary for clotting, as well as electrolytes, waste products, and nutrients 55% of blood volume is plasma STRUCTURE AND FUNCTION OF THE HEMATOLOGIC SYSTEM Blood Cellular component of blood consists of three primary cell types: 1. erythrocytes (red blood cells [RBCs], red cells) 2. Leukocytes (white blood cells [WBCs]) 3. thrombocytes (platelets) 40% to 45% of the blood volume The primary site for hematopoiesis is the bone marrow STRUCTURE AND FUNCTION OF THE HEMATOLOGIC SYSTEM STRUCTURE AND FUNCTION OF THE HEMATOLOGIC SYSTEM Bone Marrow Site of hematopoiesis, or blood cell formation Marrow is one of the largest organs of the body, making up 4% to 5% of total body weight Within it are primitive cells called Stem Cells These stem cells are committed to produce specific types of blood cells Lymphoid stem cells produce either T or B lymphocytes Myeloid stem cells differentiate into three broad cell types: erythrocytes, leukocytes, and platelets STRUCTURE AND FUNCTION OF THE HEMATOLOGIC SYSTEM Blood Cells Erythrocytes (Red Blood Cells) Consist primarily of hemoglobin Contains iron and makes up 95% of the cell mass Marrow releases slightly immature forms of erythrocytes, called reticulocytes Important property of heme is its ability to bind to oxygen loosely and reversibly Oxygen readily binds to hemoglobin in the lungs and is carried as oxyhemoglobin Lifespan of a normal circulating erythrocyte is 120 days STRUCTURE AND FUNCTION OF THE HEMATOLOGIC SYSTEM Blood Cells Leukocytes (White Blood Cells) Leukocytes are divided into two general categories: 1. Granulocytes 2. Lymphocytes Total leukocyte count is 4000 to 11,000 cells/mm3 STRUCTURE AND FUNCTION OF THE HEMATOLOGIC SYSTEM Blood Cells Leukocytes (White Blood Cells) Granulocytes presence of granules in the cytoplasm of the cell eosinophils, basophils, and neutrophils Agranulocytes Monocytes - remove debris as Macrophages Lymphocytes – Natural killer (NK) cells STRUCTURE AND FUNCTION OF THE HEMATOLOGIC SYSTEM Blood Cells Platelets (Thrombocytes) Platelets play an essential role in the control of bleeding They adhere to the site of injury and to each other, forming a platelet plug that temporarily stops bleeding Substances released from platelet granules activate coagulation factors in the blood plasma and initiate the formation of a stable clot composed of fibrin STRUCTURE AND FUNCTION OF THE HEMATOLOGIC SYSTEM Plasma and Plasma Proteins Liquid portion of the blood is called plasma 90% of plasma is water. The remainder consists primarily of plasma proteins; -clotting factors(particularly fibrinogen); nutrients, enzymes, waste products, and gases Plasma proteins consist primarily of albumin and globulins 1. Globulins carry various substances in bound form in the Circulation 2. Albumin is particularly important for the maintenance of fluid balance within the vascular system STRUCTURE AND FUNCTION OF THE HEMATOLOGIC SYSTEM Reticuloendothelial System (RES) Composed of special tissue called macrophages Defend the body against foreign invaders (i.e., bacteria and other pathogens) via phagocytosis Stimulates the inflammatory process and present antigens to the immune system The spleen is the site of activity for most macrophages STRUCTURE AND FUNCTION OF THE HEMATOLOGIC SYSTEM Hemostasis process of preventing blood loss from intact vessels and of stopping bleeding from a severed vessel, which requires adequate numbers of functional platelets severed blood vessel constricts → Circulating platelets aggregate at the site → adhere to the vessel and to one another → unstable hemostatic plug is formed →coagulation process → formation of fibrin ASSESSMENT of the HEMATOLOGIC SYSTEM ASSESSMENT of the HEMATOLOGIC SYSTEM HEALTH HISTORY Family History Assessment Specific to Hematologic Disorders 1. Collect family history information on maternal and paternal relatives from three generations of the family. 2. Assess family history for other family members with histories of blood disorders or episodes of abnormal bleeding. 3. If a family history or personal risk is suspected, the person should be carefully screened for bleeding disorders prior to surgical procedures. ASSESSMENT of the HEMATOLOGIC SYSTEM HEALTH HISTORY Patient Assessment Specific to Hematologic Disorders Assess for specific symptoms of hematologic diseases: 1. Extreme fatigue (the most common symptom of hematologic disorders) 2. Delayed clotting of blood 3. Easy or deep bruising 4. Abnormal bleeding (e.g., frequent nosebleeds) 5. Abdominal pain (hemochromatosis) or joint pain (sickle cell disease) 6. Review blood cell counts for abnormalities. 7. Assess for presence of illness despite low risk for the illness (e.g., a young adult with a blood clot) ASSESSMENT of the HEMATOLOGIC SYSTEM HEALTH HISTORY ASSESSMENT of the HEMATOLOGIC SYSTEM PHYSICAL ASSESSMENT The physical assessment should be comprehensive and include careful attention to the skin, oral cavity, lymph nodes, and spleen ASSESSMENT of the HEMATOLOGIC SYSTEM PHYSICAL ASSESSMENT ASSESSMENT of the HEMATOLOGIC SYSTEM PHYSICAL ASSESSMENT ASSESSMENT of the HEMATOLOGIC SYSTEM PHYSICAL ASSESSMENT ASSESSMENT of the HEMATOLOGIC SYSTEM PHYSICAL ASSESSMENT ASSESSMENT of the HEMATOLOGIC SYSTEM BONE MARROW ASPIRATION AND BIOPSY Bone marrow aspiration procedure. The posterior superior iliac crest is the preferred site for bone marrow aspiration and biopsy because no vital organs or vessels are nearby. The patient is placed either in the lateral position with one leg flexed or in the prone position. The anterior iliac crest or sternum may also be used Therapeutic Approaches to Hematologic Disorders THERAPEUTIC APPROACHES TO HEMATOLOGIC DISORDERS SPLENECTOMY The surgical removal of the spleen Enlarged spleen may be the site of excessive destruction of blood cells Enlarged spleens develop severe thrombocytopenia THERAPEUTIC APPROACHES TO HEMATOLOGIC DISORDERS THERAPEUTIC APHERESIS Apheresis is a Greek word meaning “separation.” Blood is taken from the patient and passed through a centrifuge, where a specific component is separated from the blood and removed THERAPEUTIC APPROACHES TO HEMATOLOGIC DISORDERS HEMATOPOIETIC STEM CELL TRANSPLANTATION Hematopoietic stem cells may be transplanted from either allogeneic or autologous donors For most hematologic diseases, allogeneic transplant is more effective here, stem cells are obtained from a donor whose cells match those of the patient. Patient’s own stem cells are harvested and then used in autologous transplant THERAPEUTIC APPROACHES TO HEMATOLOGIC DISORDERS THERAPEUTIC PHLEBOTOMY Therapeutic phlebotomy is the removal of a certain amount of blood under controlled conditions. Patients with elevated hematocrits or excessive iron absorption can usually be managed by periodically removing 1 unit (about 500 mL) of whole blood. Over time, this process can produce iron deficiency, leaving the patient unable to produce as many erythrocytes. THERAPEUTIC APPROACHES TO HEMATOLOGIC DISORDERS BLOOD COMPONENT THERAPY THERAPEUTIC APPROACHES TO HEMATOLOGIC DISORDERS BLOOD COMPONENT THERAPY THERAPEUTIC APPROACHES TO HEMATOLOGIC DISORDERS BLOOD COMPONENT THERAPY THERAPEUTIC APPROACHES TO HEMATOLOGIC DISORDERS TRANSFUSION Administration of blood and blood components requires knowledge of correct administration techniques and possible complications. Refer to your LECLAB on Blood Transfusions for the procedure!!! THERAPEUTIC APPROACHES TO HEMATOLOGIC DISORDERS TRANSFUSION POSTPROCEDURE 1. Obtain vital signs and auscultate breath sounds; compare with baseline measurements. If signs of increased fluid overload present, consider obtaining prescription for diuretic, as warranted. 2. Dispose of used materials properly. 3. Document procedure in patient’s medical record, including patient assessment findings and tolerance to procedure. 4. Monitor patient for response to and effectiveness of procedure. A CBC may be ordered 1-6 hours after transfusion to facilitate this evaluation. 5. If patient is at risk for transfusion-associated circulatory overload (TACO), monitor closely for 6 hours after transfusion if possible. THERAPEUTIC APPROACHES TO HEMATOLOGIC DISORDERS TRANSFUSION COMPLICATIONS 1. Febrile Nonhemolytic Reaction 2. Acute Hemolytic Reaction 3. Allergic Reaction 4. Transfusion-Associated Circulatory Overload (TACO) 5. Bacterial Contamination 6. Transfusion-Related Acute Lung Injury (TRALI) 7. Delayed Hemolytic Reaction 8. Disease Acquisition THERAPEUTIC APPROACHES TO HEMATOLOGIC DISORDERS Common Complications Resulting from Long-Term Packed Red Blood Cell Transfusion Therapy THERAPEUTIC APPROACHES TO HEMATOLOGIC DISORDERS Nursing Management for Transfusion Reactions Stop the transfusion. Maintain the IV line with normal saline solution through new IV tubing, given at a slow rate. Assess the patient carefully. Compare the vital signs with baseline, including oxygen saturation. Assess the patient’s respiratory status carefully. Note the presence of adventitious breath sounds; the use of accessory muscles; extent of dyspnea; and changes in mental status, including anxiety and confusion. Note any chills, diaphoresis, jugular vein distention, and reports of back pain or urticaria. Notify the primary provider of the assessment findings, and implement any treatments prescribed. Continue to monitor the patient’s vital signs and respiratory, cardiovascular, and renal status. Notify the blood bank that a suspected transfusion reaction has occurred. Send the blood container and tubing to the blood bank for repeat typing and culture. The patient’s identity and blood component identifying tags and numbers are verified. THERAPEUTIC APPROACHES TO HEMATOLOGIC DISORDERS Nursing Management for Transfusion Reactions If a hemolytic transfusion reaction or bacterial infection is suspected, the nurse does the following: 1. Obtains appropriate blood specimens from the patient 2. Collects a urine sample as soon as possible to detect hemoglobin in the urine 3. Documents the reaction according to the institution’s policy THERAPEUTIC APPROACHES TO HEMATOLOGIC DISORDERS DISEASES POTENTIALLY TRANSMITTED BY BLOOD TRANSFUSION Hepatitis (Viral Hepatitis B, C) There is greater risk from pooled blood products and blood of paid donors than from volunteer donors. A screening test detects most hepatitis B and C. Transmittal risk for Hepatitis B is estimated at 1:350,000 donated units. THERAPEUTIC APPROACHES TO HEMATOLOGIC DISORDERS DISEASES POTENTIALLY TRANSMITTED BY BLOOD TRANSFUSION AIDS (HIV and HTLV) Donated blood is screened for antibodies to HIV. Transmittal risk is estimated at 1:1.5 million donated units. People with high-risk behaviors (multiple sex partners, anal sex, IV/injection drug use) and people with signs and symptoms that suggest AIDS should not donate blood. THERAPEUTIC APPROACHES TO HEMATOLOGIC DISORDERS DISEASES POTENTIALLY TRANSMITTED BY BLOOD TRANSFUSION Cytomegalovirus (CMV) Transmittal risk is greater for premature newborns with CMV antibody–negative mothers and for immunocompromised recipients who are CMV negative (e.g., those with acute leukemia, organ or tissue transplant recipients). Blood products rendered “leukocyte reduced” help reduce transmission of virus. THERAPEUTIC APPROACHES TO HEMATOLOGIC DISORDERS DISEASES POTENTIALLY TRANSMITTED BY BLOOD TRANSFUSION Graft-Versus-Host Disease (GVHD) GVHD occurs only in severely immunocompromised recipients (e.g., Hodgkin disease, bone marrow transplantation). Transfused lymphocytes engraft in recipient and attack host lymphocytes or body tissues; signs and symptoms are fever, diffuse reddened skin rash, nausea, vomiting, and diarrhea. Preventive measures include irradiating blood products to inactivate donor lymphocytes (no known radiation risks to transfusion recipient) and processing donor blood with leukocyte reduction filters. THERAPEUTIC APPROACHES TO HEMATOLOGIC DISORDERS DISEASES POTENTIALLY TRANSMITTED BY BLOOD TRANSFUSION Creutzfeldt-Jakob Disease (CJD) CJD is a rare, fatal disease that causes irreversible brain damage. There is no evidence of transmittal by transfusion. All blood donors must be screened for positive family history of CJD. Potential donors who spent a cumulative time of 5 years or more (January 1980 to present) in certain areas of Europe cannot donate blood; blood products from a donor who develops CJD are recalled. THERAPEUTIC APPROACHES TO HEMATOLOGIC DISORDERS PHARMACOLOGIC ALTERNATIVES TO BLOOD TRANSFUSIONS Growth Factors Recombinant technology has provided a means to produce hematopoietic growth factors necessary for the production of blood cells within the bone marrow Erythropoietin Effective alternative treatment for patients with chronic anemia secondary to diminished levels of erythropoietin, as in chronic renal disease Granulocyte Colony-Stimulating Factor (G-CSF) Cytokine that stimulates the proliferation and differentiation of myeloid stem cells; a rapid increase in neutrophils is seen within the circulation THERAPEUTIC APPROACHES TO HEMATOLOGIC DISORDERS PHARMACOLOGIC ALTERNATIVES TO BLOOD TRANSFUSIONS Granulocyte-Macrophage Colony-Stimulating Factor (GM-CSF) Cytokine that is naturally produced by a variety of cells, including monocytes and endothelial cells Thrombopoietin Cytokine that is necessary for the proliferation of megakaryocytes and subsequent platelet formation Management of Patients With Nonmalignant Hematologic Disorders ANEMIA ANEMIA Hemoglobin concentration is lower than normal Reflects the presence of fewer than the normal number of erythrocytes (i.e., red blood cells [RBCs]) within the circulation Amount of oxygen delivered to body tissues is also diminished Not a specific disease state but a sign of an underlying disorder Most common hematologic condition ANEMIA Classification of Anemias ANEMIA Classification of Anemias HYPOPROLIFERATIVE ANEMIAS Iron Deficiency Anemia - intake of dietary iron is inadequate Anemias in Renal Disease - lack of erythropeitin in the kidneys Anemia of Inflammation- chronic diseases of inflammation, infection, and malignancy as causes for this type of anemia Aplastic Anemia - decrease in or damage to marrow stem cells Megaloblastic Anemias - caused by deficiencies of vitamin B12 or folic acid ANEMIA Classification of Anemias HEMOLYTIC ANEMIAS Sickle Cell Disease - inheritance of the sickle hemoglobin (HbS) gene, which causes the hemoglobin molecule to be defective Thalassemias - group of hereditary anemias characterized by hypochromia (an abnormal decrease in the hemoglobin content of erythrocytes), extreme microcytosis (smaller-than-normal erythrocytes), hemolysis, and variable degrees of anemia. Glucose-6-Phosphate Dehydrogenase Deficiency - The G-6-PD gene is the source of the abnormality in this disorder; this gene produces an enzyme within the erythrocyte that is essential for membrane stability Immune Hemolytic Anemias - anemias can result from exposure of the erythrocyte to antibodies ANEMIA CLINICAL MANIFESTATIONS Symptoms may vary on the rapidity, duration(i.e., its chronicity), the metabolic requirements of the patient, other concurrent disorders or disabilities (e.g., cardiac or pulmonary disease), and complications or concomitant features of the condition that produced the anemia More rapidly an anemia develops, the more severe its symptoms A person who has become gradually anemic, with hemoglobin levels between 9 and 11 g/dL, usually has fewer or no symptoms other than slight tachycardia on exertion and possibly fatigue. Patients with hypothyroidism with decreased oxygen needs may be completely asymptomatic Patients with coexistent cardiac, vascular, or pulmonary disease may develop more pronounced symptoms(e.g., dyspnea, chest pain, muscle pain or cramping) ANEMIA ASSESSMENT AND DIAGNOSTIC FINDINGS Hematologic studies Bone marrow aspiration Diagnostic studies may be performed to determine the presence of underlying chronic illness, such as malignancy, or the source of any blood loss, such as polyps or ulcers within the gastrointestinal (GI) tract ANEMIA COMPLICATIONS Heart failure Paresthesias Delirium Patients with underlying heart disease are far more likely to have angina or symptoms of heart failure ANEMIA MEDICAL MANAGEMENT Directed toward correcting or controlling the cause of the anemia May be replaced with a transfusion of packed red blood cells NURSING PROCESS (ANEMIA) ASSESSMENT Physical Assessment Weakness, fatigue, and general malaise are common, as are pallor of the skin and mucous membranes, jaundice; angular cheilosis (ulcerated corners of the mouth); and brittle, ridged, concave nails may be present in patients with megaloblastic anemia (characterized by the presence of abnormally large, nucleated RBCs) or hemolytic anemia, tongue may be beefy red and sore in megaloblastic anemia, or smooth and red in iron deficiency anemia Patients with iron deficiency anemia may infrequently crave ice, starch, or dirt; this craving is known as pica Health History Should include a medication history, history of alcohol intake, Family history, athletic endeavors, nutritional assessment Cardiac status should be carefully assessed. GI system Neurologic examination NURSING PROCESS (ANEMIA) NURSING DIAGNOSES Based on the assessment data, major nursing diagnoses may include: Fatigue related to decreased hemoglobin and diminished oxygen carrying capacity of the blood Imbalanced nutrition, less than body requirements, related to inadequate intake of essential nutrients Activity intolerance related to inadequate hemoglobin and hematocrit Noncompliance with prescribed therapy NURSING PROCESS (ANEMIA) Collaborative Problems/Potential Complications Potential complications may include the following: Heart failure Angina Paresthesias Confusion Injury related to falls Depressed mood NURSING PROCESS (ANEMIA) Planning and Goals The major goals for the patient may include decreased fatigue, attainment or maintenance of adequate nutrition, maintenance of adequate tissue perfusion, compliance with prescribed therapy, and absence of complications. NURSING PROCESS (ANEMIA) NURSING INTERVENTIONS 1. Managing fatigue 2. Maintaining adequate nutrition 3. Maintaining adequate nutrition 4. Promoting adherence with prescribed therapy 5. Monitoring and managing potential complications NURSING PROCESS (ANEMIA) EVALUATION 1. Reports less fatigue a. Follows a progressive plan of rest, activity, and exercise b. Prioritizes activities c. Paces activities according to energy level 2. Attains and maintains adequate nutrition a. Eats a healthy diet b. Develops a meal plan that promotes optimal nutrition c. Maintains adequate amounts of iron, vitamins, and protein from diet or supplements d. Adheres to nutritional supplement therapy when prescribed e. Verbalizes understanding of rationale for using recommended nutritional supplements f. Verbalizes understanding of rationale for avoiding nonrecommended nutritional supplements NURSING PROCESS (ANEMIA) EVALUATION 3. Maintains adequate activity level a. Has vital signs within baseline for patient b. Has pulse oximetry (arterial oxygenation) value within normal limits 4. Absence of complications a. Avoids or limits activities that trigger dyspnea, palpitations, dizziness, or tachycardia b. Uses rest and comfort measures to alleviate dyspnea c. Has vital signs within baseline for patient d. Has no signs of increasing fluid retention e. Remains oriented to time, place, and situation f. Remains engaged in social situations, exhibits no signs of depression g. Ambulates safely, using assistive devices as necessary h. Remains free of injury i. Verbalizes understanding of importance of serial CBC measurements j. Maintains safe home environment; obtains assistance as necessary POLYCYTHEMIA POLYCYTHEMIA Refers to an increased volume of RBCs Hematocrit is elevated (more than 55% in males, more than 50% in females) Dehydration (decreased volume of plasma) can cause an elevated hematocrit but not typically to the level to be considered polycythemia. Polycythemia is classified as either primary or secondary. Primary polycythemia, also called polycythemia vera, is a proliferative disorder POLYCYTHEMIA SECONDARY POLYCYTHEMIA Caused by excessive production of erythropoietin. Occur in response to a reduced amount of oxygen, which acts as a hypoxic stimulus, as in heavy cigarette smoking, obstructive sleep apnea (OSA), chronic obstructive pulmonary disease (COPD), or cyanotic heart disease, or with conditions such as living at a high altitude or exposure to low levels of carbon monoxide. It can also result from certain hemoglobinopathies (e.g., hemoglobin Chesapeake), in which the hemoglobin has an abnormally high affinity for oxygen or from genetic mutations. Secondary polycythemia can also occur from neoplasms (e.g., renal cell carcinoma) that stimulate erythropoietin production, excess erythropoietin-stimulating agent use, or androgen use. POLYCYTHEMIA MEDICAL MANAGEMENT Mild, treatment may not be necessary When treatment is necessary, it involves treating the primary condition. Therapeutic phlebotomy may be necessary in symptomatic patients to reduce blood viscosity and volume as well as when the hematocrit is significantly elevated. Therapeutic phlebotomy should not be used when the cause for an elevated RBC level is an appropriate, compensatory response to tissue hypoxia POLYCYTHEMIA MEDICAL MANAGEMENT Mild, treatment may not be necessary When treatment is necessary, it involves treating the primary condition. Therapeutic phlebotomy may be necessary in symptomatic patients to reduce blood viscosity and volume as well as when the hematocrit is significantly elevated. Therapeutic phlebotomy should not be used when the cause for an elevated RBC level is an appropriate, compensatory response to tissue hypoxia NEUTROPENIA NEUTROPENIA A neutrophil count of less than 2000/mm3 results from decreased production of neutrophils or increased destruction of these cells Neutrophils are essential in preventing and limiting bacterial infection. A patient with neutropenia is at increased risk for infection from both exogenous and endogenous sources NEUTROPENIA CAUSES OF NEUTROPENIA DECREASED PRODUCTION OF NEUTROPHILS Aplastic anemia, due to medications or toxins Chemotherapy Metastatic cancer, lymphoma, leukemia Myelodysplastic syndromes Radiation therapy INEFFECTIVE GRANULOCYTOPOIESIS Megaloblastic anemia INCREASED DESTRUCTION OF NEUTROPHILS Bacterial infections Hypersplenism Immunologic disorders (e.g., systemic lupus erythematosus) Medication induceda Viral disease (e.g., infectious hepatitis, mononucleosis) NEUTROPENIA CLINICAL MANIFESTATIONS There are no definite symptoms of neutropenia until the patient develops an infection. A routine CBC with differential, as obtained after chemotherapy treatment, can reveal neutropenia before the onset of infection. NEUTROPENIA MEDICAL MANAGEMENT Treatment of the neutropenia varies depending on its cause. medication induced, the offending agent is stopped immediately, if possible. Corticosteroids may be used if the cause is an immunologic disorder. growth factors such as granulocyte colony-stimulating factor or granulocyte-macrophage colonystimulating factor can be effective in increasing neutrophil production Withholding or reducing the dose of chemotherapy or radiation therapy may be required NEUTROPENIA NURSING MANAGEMENT Nurses in all settings have a crucial role in assessing the severity of neutropenia and in preventing and managing complications, which most often include infections. NEUTROPENIA The Patient at Risk for Infection At the completion of education, the patient and/or caregiver will be able to: State the impact of alterations in neutrophils, lymphocytes, immunoglobulins on physiologic functioning, ADLs, IADLs, roles, relationships, and spirituality. State changes in lifestyle (e.g., diet, activity) or home environment necessary to decrease risk for infection. Maintain good hand hygiene technique, oral hygiene, total body hygiene, and skin integrity. Avoid cleaning birdcages and litter boxes; consider avoiding garden work (soil) and fresh flowers in stagnant water. Maintain a high-calorie, high-protein diet, with fluid intake of 3000 mL daily (unless fluids are restricted). NEUTROPENIA The Patient at Risk for Infection At the completion of education, the patient and/or caregiver will be able to: Avoid people with infections and crowds. Perform deep breathing; use incentive spirometer every 4 hours while awake if mobility is restricted. Provide adequate lubrication with gentle vaginal manipulation during sexual intercourse; avoid anal intercourse. Identify signs and symptoms of infection Demonstrate how to monitor for signs of infection. Describe to whom, how, and when to report signs of infection. Describe appropriate actions to take should infection occur NEUTROPENIA The Patient at Risk for Infection At the completion of education, the patient and/or caregiver will be able to: Avoid people with infections and crowds. Perform deep breathing; use incentive spirometer every 4 hours while awake if mobility is restricted. Provide adequate lubrication with gentle vaginal manipulation during sexual intercourse; avoid anal intercourse. Identify signs and symptoms of infection Demonstrate how to monitor for signs of infection. Describe to whom, how, and when to report signs of infection. Describe appropriate actions to take should infection occur LYMPHOPENIA LYMPHOPENIA Lymphocyte count less than 1500/mm3 Result from ionizing radiation, long-term use of corticosteroids, uremia, infections (particularly viral infections), some neoplasms (e.g., breast and lung cancers, advanced Hodgkin disease), and some protein-losing enteropathies (in which the lymphocytes within the intestines are lost) Mild, it is often without sequelae; Severe, it can result in bacterial infections (due to low B lymphocytes) or in opportunistic infections (due to low T lymphocytes). Tcell lymphocyte depletion is most common, typically due to viral infection, such as the human immune deficiency virus (HIV). BLEEDING DISORDERS BLEEDING DISORDERS Failure of normal hemostatic mechanisms can result in bleeding, Bleeding is commonly provoked by trauma; however, in certain circumstances, it can occur spontaneously. When the cause is platelet or coagulation factor abnormalities, the site of bleeding can be anywhere in the body. When the source is vascular abnormalities, the site of bleeding may be more localized. Some patients have simultaneous defects in more than one hemostatic mechanism. BLEEDING DISORDERS The bone marrow may be stimulated to increase platelet production (thrombopoiesis) as a reactive response to bleeding Sometimes, the increase in platelets does not result from increased production but from a loss in platelet pooling within the spleen. BLEEDING DISORDERS CLINICAL MANIFESTATIONS Signs and symptoms of bleeding disorders vary according to the type of defect. Abnormalities of the vascular system give rise to local bleeding, usually into the skin. Patients with platelet defects develop petechiae, often in clusters; Bleeding from platelet disorders can be severe. In contrast, coagulation factor defects do not tend to cause superficial bleeding, because the primary hemostatic mechanisms are still intact. Instead, bleeding occurs deeper within the body (e.g., subcutaneous or intramuscular hematomas, hemorrhage into joint spaces). BLEEDING DISORDERS CLINICAL MANIFESTATIONS BLEEDING DISORDERS MEDICAL MANAGEMENT Management varies based on the underlying cause of the bleeding disorder. transfusions of blood products may be indicated. Hemostatic agents such as aminocaproic acid (Amicar) can be used to inhibit this process. A patient scheduled for an invasive procedure, including a dental extraction, may need a transfusion prior to the procedure to minimize the risk of excessive bleeding. BLEEDING DISORDERS NURSING MANAGEMENT Patients must be educated to observe themselves carefully and frequently for signs of bleeding They need to understand the importance of avoiding activities that increase the risk of bleeding, such as contact sports. Examine the skin for petechiae and ecchymoses (bruises) and the nose and gums for bleeding. If severe, patients who are hospitalized are monitored for bleeding by testing all drainage and excreta (feces, urine, emesis, and gastric drainage) for occult blood as well as obvious blood. BLEEDING DISORDERS NURSING MANAGEMENT Patients must be educated to observe themselves carefully and frequently for signs of bleeding They need to understand the importance of avoiding activities that increase the risk of bleeding, such as contact sports. Examine the skin for petechiae and ecchymoses (bruises) and the nose and gums for bleeding. If severe, patients who are hospitalized are monitored for bleeding by testing all drainage and excreta (feces, urine, emesis, and gastric drainage) for occult blood as well as obvious blood. SECONDARY THROMBOCYTOSIS Increased platelet production is the primary mechanism of secondary, or reactive, thrombocytosis. The platelet count is above normal, an increase of more than 1 million/mm3 Platelet function is normal; survival time is normal or decreased. disorders or conditions can cause a reactive increase in platelets, including infection, iron deficiency, chronic inflammatory disorders, malignant disease, acute hemorrhage, and splenectomy. Treatment is aimed at the underlying disorder. With successful management, the platelet count usually returns to normal. THROMBOCYTOPENIA Thrombocytopenia (low platelet level) can result from various factors: 1. Decreased production of platelets within the bone marrow 2. Increased destruction of platelets 3. Increased consumption of platelets (e.g., the use of platelets in clot formation). THROMBOCYTOPENIA CLINICAL MANIFESTATIONS Bleeding and petechiae usually do not occur with platelet counts greater than 50,000/mm3 When the platelet count drops to less than 20,000/mm3, petechiae can appear, along with nasal and gingival bleeding, excessive menstrual bleeding, and excessive bleeding after surgery or dental extractions. When the platelet count is less than 5000/mm3, spontaneous, potentially fatal central nervous system or GI hemorrhage can occur. If the platelets are dysfunctional as a result of disease (e.g., MDS) or medications (e.g., aspirin), the risk of bleeding may be much greater even when the actual platelet count is not significantly reduced, because the function of the platelets is altered. THROMBOCYTOPENIA MEDICAL MANAGEMENT The management of secondary thrombocytopenia is usually treatment of the underlying disease. If platelet production is impaired, platelet transfusions may increase the platelet count and stop bleeding or prevent spontaneous hemorrhage. In some instances, splenectomy can be a useful therapeutic intervention, but often it is not an option. THROMBOCYTOPENIA NURSING MANAGEMENT the nurse considers the cause of the thrombocytopenia, the likely duration, and the overall condition of the patient. Education is important, as are interventions to promote patient,safety, particularly fall prevention in the older adult or patient who is frail. The interventions for a patient with thrombocytopenia are the same as those for a patient with cancer who is at risk for bleeding THROMBOCYTOPENIA NURSING MANAGEMENT the nurse considers the cause of the thrombocytopenia, the likely duration, and the overall condition of the patient. Education is important, as are interventions to promote patient,safety, particularly fall prevention in the older adult or patient who is frail. The interventions for a patient with thrombocytopenia are the same as those for a patient with cancer who is at risk for bleeding IMMUNE THROMBOCYTOPENIC PURPURA More common among children and young women. Also called idiopathic thrombocytopenic purpura and immune thrombocytopenia. Primary ITP occurs in isolation Secondary ITP often results from autoimmune diseases (e.g., antiphospholipid antibody syndrome), viral infections (e.g., hepatitis C, HIV), and various drugs (e.g., sulfa drugs). Primary ITP is defined as a platelet count less than 100 × 109/L with an inexplicable absence of a cause for thrombocytopenia IMMUNE THROMBOCYTOPENIC PURPURA PATHOPHYSIOLOGY Antiplatelet antibodies develop in the blood and bind to the patient’s platelets → ingested and destroyed by the reticuloendothelial system (RES) or tissue macrophages → body attempts to compensate for this destruction by increasing platelet production within the marrow → platelet production also be impaired as the antibodies may also induce cell death (via apoptosis) of the megakaryocytes → inhibit platelet production within the bone marrow IMMUNE THROMBOCYTOPENIC PURPURA CLINICAL MANIFESTATIONS Many patients have no symptoms, and the low platelet count is an incidental finding (often less than 30,000/mm3; less than 5000/mm3 is not uncommon). Common physical manifestations are easy bruising, heavy menses, and petechiae on the extremities or trunk Simple bruising or petechiae (“dry purpura”) tend to have fewer complications from bleeding than those with bleeding from mucosal surfaces, such as the GI tract (including the mouth) and pulmonary system (e.g., hemoptysis), which is termed wet purpura. Severe thrombocytopenia (platelet count less than 20,000/mm3) IMMUNE THROMBOCYTOPENIC PURPURA ASSESSMENT AND DIAGNOSTIC FINDINGS A careful history and physical assessment must be obtained to exclude causes of the thrombocytopenia and identify evidence of bleeding. Patients should be tested for hepatitis C and HIV, if not previously done to rule out these potential causes. If a bone marrow aspirate is performed, an increase in megakaryocytes may be seen. The severity of the thrombocytopenia is highly variable, but a platelet count that is less than 20,000/mm3 is a common finding. IMMUNE THROMBOCYTOPENIC PURPURA MEDICAL MANAGEMENT The primary goal of treatment is a “safe” platelet Count exceeds 30,000/mm3 to 50,000/mm3 may be carefully observed without additional intervention. less than 30,000/mm3 or if bleeding occurs, the goal is to improve the patient’s platelet count rather than to cure the disease. basis is not of the patient’s platelet count but on the severity of bleeding Aminocaproic acid—a fibrinolytic enzyme inhibitor that slows the dissolution of clots—may be useful for patients with significant mucosal bleeding refractory to other treatments The mainstay of short-term therapy is the use of immunosuppressive agents. IVIG is also commonly used to treat ITP IMMUNE THROMBOCYTOPENIC PURPURA NURSING MANAGEMENT Nursing care includes an assessment of the patient’s lifestyle to determine the risk of bleeding from activity. A careful medication history is also obtained, including use of over-the-counter (OTC) medications, herbs, and nutritional supplements. The nurse must be alert for sulfa-containing medications and others that alter platelet function (e.g., aspirin-based or other NSAIDs). The nurse assesses for any history of recent viral illness and reports of headache or visual disturbances, which could be initial symptoms of intracranial bleeding. Patients who are admitted to the hospital with wet purpura and low platelet counts should have a neurologic assessment incorporated into their routine vital sign measurements. All injections or rectal medications should be avoided, and rectal temperature measurements should not be performed, because they can stimulate bleeding. PLATELET DEFECTS Qualitative defects, the number of platelets may be normal but the platelets do not function normally. The morphology of platelets is often hypogranular and pale, and may be larger than normal. Aspirin may induce a platelet disorder. NSAIDs can also inhibit platelet function, but the effect is not as prolonged as with aspirin ( Other causes of platelet dysfunction include end-stage renal disease, possibly from metabolic products affecting platelet function; MDS; multiple myeloma (due to abnormal protein interfering with platelet function); cardiopulmonary bypass; herbal therapy; and other medications PLATELET DEFECTS CLINICAL MANIFESTATIONS Bleeding may be mild or severe. elevated PT in the setting of a normal aPTT and platelet count may suggest factor VII deficiency, whereas an elevated partial thromboplastin time (PTT) in the setting of a normal PT and platelet count may suggest hemophilia or von Willebrand disease (vWD). Ecchymoses are common, particularly on the extremities. Patients with platelet dysfunction may be at risk for significant bleeding after trauma or invasive procedures PLATELET DEFECTS MEDICAL MANAGEMENT If the platelet dysfunction is caused by medication, its use should be stopped, if possible If platelet dysfunction is marked, bleeding can often be prevented by transfusion of normal platelets before invasive procedures. Antifibrinolytic agents (e.g., aminocaproic acid) may be required to prevent significant bleeding after such procedures; Desmopressin (DDAVP) can decrease the duration of bleeding in some situations and improve hemostasis (Levi et al., 2016). PLATELET DEFECTS MEDICAL MANAGEMENT If the platelet dysfunction is caused by medication, its use should be stopped, if possible If platelet dysfunction is marked, bleeding can often be prevented by transfusion of normal platelets before invasive procedures. Antifibrinolytic agents (e.g., aminocaproic acid) may be required to prevent significant bleeding after such procedures; Desmopressin (DDAVP) can decrease the duration of bleeding in some situations and improve hemostasis PLATELET DEFECTS NURSING MANAGEMENT Instruct to avoid substances that can diminish platelet function, such as certain OTC medications, some herbal therapies, nutritional supplements, and alcohol. inform their health care providers (including dentists) of the underlying condition before any invasive procedure is performed so that appropriate steps can be initiated to diminish the risk of bleeding. Maintaining good oral hygiene is very important so that gingival bleeding can be minimized. HEMOPHILIA Hemophilia A is caused by a genetic defect that results in deficient or defective factor VIII. Hemophilia B (also called Christmas disease) stems from a genetic defect that causes deficient or defective factor IX Both types of hemophilia are inherited as X-linked traits, so most affected people are males; females can be carriers but are almost always asymptomatic Hemophilia is recognized in early childhood, usually in the toddler age group. However, patients with mild hemophilia may not be diagnosed until they experience severe trauma (e.g., a high school football injury) or surgery. HEMOPHILIA CLINICAL MANIFESTATIONS Hemorrhages into various parts of the body Severe and can occur even after minimal trauma. The frequency and severity of the bleeding depend on the degree of factor deficiency as well as the intensity of the precipitating trauma. About 75% of all bleeding in patients with hemophilia occurs into joints. The most commonly affected joints are the knees, elbows, ankles, shoulders, wrists, and hips. HEMOPHILIA CLINICAL MANIFESTATIONS HEMOPHILIA CLINICAL MANIFESTATIONS Bleeding is commonly associated with dental extraction Spontaneous hematuria and GI bleeding can also occur Surgical procedures typically result in excessive bleeding at the surgical site Falls carry significant risk for morbidity in adults HEMOPHILIA MEDICAL MANAGEMENT Recombinant forms of factor VIII and X concentrates are available and decrease the need for using factor concentrates, or, more infrequently, fresh-frozen plasma. Other therapeutic options include administering recombinant factor VIIa (Novoseven, Novo Nordixk) or activated prothrombin complex concentrates (IaPCC, FEIBA) Aminocaproic acid inhibits fibrinolysis and therefore stabilizes the clot HEMOPHILIA NURSING MANAGEMENT Diagnosed as children, hey often require assistance in coping with the condition because it is chronic, places restrictions on their lives, and is an inherited disorder that can be passed to future generations Patients need extensive education about activity restrictions and self-care measures to diminish the chance of hemorrhage and complications of bleeding Patients and family members are instructed how to administer the factor concentrate at home at the first sign of bleeding so that bleeding is minimized and complications avoided During hemorrhagic episodes, the extent of bleeding must be assessed Carefully. Close observation and systematic assessment for emergent complications Warm baths promote relaxation, improve mobility, and lessen pain Genetic testing and counseling regarding having children VON WILLEBRAND DISEASE (VWD) Inherited as a dominant trait, vWD is a common bleeding disorder that affects males and females equally Deficiency of vWF, which is necessary for factor VIII activity Type 1, the most common, is characterized by decreases in structurally normal vWF. Type 2 shows variable qualitative defects based on the specific vWF subtype involved. Type 3 is very rare (less than 5% of cases) and is characterized by a severe vWF deficiency as well as significant deficiency of factor VIII VON WILLEBRAND DISEASE (VWD) CLINICAL MANIFESTATIONS Bleeding tends to be mucosal. Recurrent nosebleeds, easy bruising, heavy menses, prolonged bleeding from cuts, and postoperative bleeding. Massive soft tissue or joint hemorrhages are not often seen, unless the patient has severe type 3 vWD. As the laboratory values fluctuates, so does the bleeding. VON WILLEBRAND DISEASE (VWD) Assessment and Diagnostic Findings The diagnosis is established by the patient meeting all of these criteria: 1. A history of bleeding since childhood 2. Reduced vWF activity in plasma 3. History of bleeding within the family Laboratory test results show a normal platelet count but a prolonged bleeding time and a slightly prolonged aPTT. Ristocetin cofactor, or vWF collagen binding assay, which measures vWF activity vWF antigen, factor VIII, and, for patients with suspected type 2 defects, vWF multimers, which measure specific subtypes of vWF. VON WILLEBRAND DISEASE (VWD) MEDICAL MANAGEMENT The goal of treatment is to replace the deficient protein (e.g., vWF or factor VIII) at the time of spontaneous bleeding or prior to an invasive procedure to prevent subsequent bleeding. Desmopressin, a synthetic vasopressin analogue, can be used to prevent bleeding associated with dental or surgical procedures Replacement products include Humate-P and Alphanate, which are commercial concentrates of vWF and factor VIII Aminocaproic acid is useful in managing mild forms of mucosal bleeding Estrogen–progesterone compounds may diminish the extent of menses. Platelet transfusions are useful when there is significant bleeding. While rich in vWF and Factor VIII, cryoprecipitate is typically used only in emergent situations ACQUIRED COAGULATION DISORDERS LIVER DISEASE Hepatic dysfunction (due to cirrhosis, tumor, or hepatitis) can result in diminished amounts of the factors needed to maintain coagulation and hemostasis. Prolongation of the PT, unless it is caused by vitamin K deficiency, may indicate severe hepatic dysfunction. Although bleeding is usually minor (e.g., ecchymoses), these patients are also at risk for significant bleeding, related especially to trauma or surgery. Transfusion of fresh-frozen plasma may be required to replace clotting factors and to prevent or stop bleeding. Patients may also have life-threatening hemorrhage from peptic ulcers or esophageal varices. In these cases, replacement with fresh-frozen plasma, PRBCs, and platelets is usually required. VITAMIN K DEFICIENCY The synthesis of many coagulation factors depends on vitamin K. Vitamin K deficiency is common in malnourished patients. Prolonged use of some antibiotics decreases the intestinal flora that produces vitamin K Administration of vitamin K (phytonadione [Mephyton]), either orally or as a subcutaneous injection, can correct the deficiency quickly; adequate synthesis of coagulation factors is reflected by normalization of the PT. DISSEMINATED INTRAVASCULAR COAGULATION Disseminated intravascular coagulation (DIC) is not an actual disease but a sign of an underlying condition. DIC may be triggered by sepsis, trauma, cancer, shock, abruptio placentae, toxins, allergic reactions, and other conditions the vast majority (two thirds) of cases of DIC are initiated by an infection or a malignancy The severity of DIC is variable, but it is potentially life-threatening. DISSEMINATED INTRAVASCULAR COAGULATION PATHOPHYSIOLOGY DISSEMINATED INTRAVASCULAR COAGULATION CLINICAL MANIFESTATIONS DISSEMINATED INTRAVASCULAR COAGULATION ASSESSMENT AND DIAGNOSTIC FINDINGS DISSEMINATED INTRAVASCULAR COAGULATION Medical Management The most important factor in managing DIC is aggressively treating the underlying cause Correcting the secondary effects of tissue ischemia by improving oxygenation, replacing fluids, correcting electrolyte imbalances, and administering vasopressor medications is also important. If serious hemorrhage occurs, the depleted coagulation factors and platelets may be replaced to reestablish the potential for normal hemostasis and thereby diminish bleeding. Cryoprecipitate is given to replace fibrinogen and factors V and VII. Administering fresh frozen plasma replaces coagulation factors. Platelets are transfused to correct severely low platelet levels, control bleeding All management strategies must be individualized to the specific patient, underlying cause of DIC, and response to interventions. DISSEMINATED INTRAVASCULAR COAGULATION NURSING MANAGEMENT Nurses need to be aware of which patients are at risk for DIC Patients need to be assessed thoroughly and frequently for signs and symptoms of thrombi and bleeding and monitored for any progression of these signs Lab values must be monitored frequently, not only for the actual result but to note trends over time as well as the rate of change in values. Assessment and interventions should target potential sites of end-organ damage DISSEMINATED INTRAVASCULAR COAGULATION NURSING MANAGEMENT Nurses need to be aware of which patients are at risk for DIC Patients need to be assessed thoroughly and frequently for signs and symptoms of thrombi and bleeding and monitored for any progression of these signs Lab values must be monitored frequently, not only for the actual result but to note trends over time as well as the rate of change in values. Assessment and interventions should target potential sites of end-organ damage THROMBOTIC DISORDERS Altered balance within the normal hemostasis process, causing excessive thrombosis that may be arterial (due to platelet aggregation) or venous (composed of platelets, red cells, and thrombin). Decreased clotting inhibitors within the circulation, altered hepatic, lack of fibrinolytic enzymes, and tortuous or atherosclerotic vessels Thrombosis may occur as an initial manifestation of an occult malignancy or as a complication from a pre- existing cancer. Hyperhomocysteinemia, antithrombin (AT) deficiency, protein C deficiency, protein S deficiency, activated protein C (APC) resistance, and factor V Leiden deficiency - hypercoagulable states THROMBOTIC DISORDERS THROMBOTIC DISORDERS A recent study found that taking aspirin after completing standard anticoagulation therapy for treating VTE reduced the risk of recurrent thrombosis With some conditions, or with repeated thrombosis, lifelong anticoagulant therapy is necessary. HYPERHOMOCYSTEINEMIA Homocysteine can promote platelet aggregation. Increased plasma levels of homocysteine are a significant risk factor for VTE (e.g., deep vein thrombosis [DVT], pulmonary embolism [PE]), recurrent VTE, and arterial thrombosis (e.g., ischemic stroke, ACS) Hyperhomocysteinemia can be hereditary, or it can result from a nutritional deficiency of folate and, to a lesser extent, of vitamins B12 and B6, because these vitamins are cofactors in homocysteine metabolism. In hyperhomocysteinemia, the endothelial lining of the vessel walls is denuded, which can precipitate thrombus formation. Smoking causes low levels of vitamin B6 and B12 and folate ANTITHROMBIN DEFICIENCY AT is a protein that inhibits thrombin and certain coagulation factors, and it may also play a role in diminishing inflammation within the endothelium of blood vessels. Most commonly a hereditary condition that can cause venous thrombosis common sites for thrombosis are the deep veins of the leg and the mesentery. Patients tend to exhibit heparin resistance; require greater amounts of heparin AT deficiency can also be acquired by four mechanisms: 1. Accelerated consumption of AT (as in DIC) 2. Reduced synthesis of AT (as in hepaticdysfunction) 3. Increased excretion of AT (as in nephrotic syndrome) 4. Medication induced (e.g., estrogens, L-asparaginase) PROTEIN C DEFICIENCY Protein C is a vitamin K–dependent enzyme synthesized in the liver; when activated, it inhibits coagulation. Deficient, the risk of thrombosis increases, and thrombosis can often occur spontaneously. Individuals with Protein C deficiency are at higher risk for recurrent PE Complication of anticoagulation management is warfarin-induced skin necrosis. Treatment with purified protein C concentrate is sometimes indicated. PROTEIN S DEFICIENCY Protein S is another natural anticoagulant normally produced by the liver. Like patients with protein C deficiency, those with protein S deficiency have a greater risk of recurrent venous thrombosis early in life, and also with recurrent PE Thromboses most commonly occur in the axillary, mesenteric, and cerebral veins. Warfarin-induced skin necrosis is possible. Acquired protein S deficiency can also occur. Pregnancy, DIC, liver disease, nephritic syndrome, HIV infection, and the use of L-asparaginase have all been associated with reduced protein S levels. ACTIVATED PROTEIN C RESISTANCE AND FACTOR V LEIDEN MUTATION APC resistance is a common condition that can occur with other hypercoagulable states. APC is an anticoagulant, and resistance to APC increases the risk of venous thrombosis. Factor V Leiden mutation is the most common cause of inherited hypercoagulability in Caucasians, Factor V Leiden mutation synergistically increases the risk of thrombosis in patients with other risk factors (e.g., the use of oral contraceptives, hyperhomocysteinemia, increased age). The duration of anticoagulation is based upon the coexistence of other risk factors for thrombi formation ACTIVATED PROTEIN C RESISTANCE AND FACTOR V LEIDEN MUTATION MEDICAL MANAGEMENT The primary method of treating thrombotic disorders is anticoagulation. However, in thrombophilic conditions, when to treat (prophylaxis or not) and how long to treat (lifelong or not) can be controversial. Anticoagulation therapy is not without risks; the most significant risk is bleeding. ACTIVATED PROTEIN C RESISTANCE AND FACTOR V LEIDEN MUTATION PHARMACOLOGIC THERAPY 1. Heparin 2. Warfarin (Coumadin) 3. Thrombin and Factor Xa Inhibitors 4. Aspirin ACTIVATED PROTEIN C RESISTANCE AND FACTOR V LEIDEN MUTATION NURSING MANAGEMENT Patients with thrombotic disorders should avoid activities that lead to circulatory stasis (e.g., immobility, crossing the legs). Exercise, especially ambulation, should be performed frequently throughout the day, particularly during long trips by car or plane. Anti-embolism stockings are often prescribed Surgery further increases the risk of thrombosis. Medications that alter platelet aggregation, such as low-dose aspirin or clopidogrel (Plavix), may be prescribed. Anticoagulants such as warfarin ACTIVATED PROTEIN C RESISTANCE AND FACTOR V LEIDEN MUTATION NURSING MANAGEMENT Assessed for concurrent risk factors for thrombosis and should avoid them if possible. Tobacco and nicotine products should be avoided In many instances, younger patients with thrombophilia may not require prophylactic anticoagulation; however, with concomitant risk factors (e.g., pregnancy), increasing age, or subsequent thrombotic events,prophylactic or long-term anticoagulation therapy may be required. Accurate health history can be extremely useful and can help guide the selection of appropriate therapeutic interventions. Patients need to understand risk factors for thrombosis and what they can do to diminish or reduce them, such asavoiding smoking, using alternative forms of contraception, increasing mobility, and maintaining a healthy weight. ACTIVATED PROTEIN C RESISTANCE AND FACTOR V LEIDEN MUTATION NURSING MANAGEMENT Assessed for concurrent risk factors for thrombosis and should avoid them if possible. Tobacco and nicotine products should be avoided In many instances, younger patients with thrombophilia may not require prophylactic anticoagulation; however, with concomitant risk factors (e.g., pregnancy), increasing age, or subsequent thrombotic events,prophylactic or long-term anticoagulation therapy may be required. Accurate health history can be extremely useful and can help guide the selection of appropriate therapeutic interventions. Patients need to understand risk factors for thrombosis and what they can do to diminish or reduce them, such asavoiding smoking, using alternative forms of contraception, increasing mobility, and maintaining a healthy weight. ACTIVATED PROTEIN C RESISTANCE AND FACTOR V LEIDEN MUTATION NURSING MANAGEMENT When a patient with a thrombotic disorder is hospitalized, frequent assessments should be performed for signs and symptoms of beginning thrombus formation, particularly in the legs (DVT) and lungs (PE). Ambulation or range-of-motion exercises as well as the use of antiembolism stockings Properly fitted graduated compression stockings may reduce pain and edema associated with the acute stage of DVT Thank You for Listening!