Hematologic and Breast Assessment PDF

CHAPT ER 39 1571 Assessment of the Hematologic System M. Linda Workman PRIORITY AND INTERRELATED CONCEPTS The priority concepts for this chapter are: CLOTTING PERFUSION LEARNING OUTCOMES Safe and Effective Care Environment 1. Collaborate with the interprof...

CHAPT ER 39 1571 Assessment of the Hematologic System M. Linda Workman PRIORITY AND INTERRELATED CONCEPTS The priority concepts for this chapter are: CLOTTING PERFUSION LEARNING OUTCOMES Safe and Effective Care Environment 1. Collaborate with the interprofessional team to perform a complete hematologic assessment, including CLOTTING and PERFUSION. Health Promotion and Maintenance 2. Explain how physiologic aging changes the hematologic functions of CLOTTING and PERFUSION and how these changes affect the associated care of older adults. 3. Teach all adults how to protect the hematologic system. Psychosocial Integrity 4. Implement patient-centered nursing interventions to help the patient and family cope with the psychosocial impact of a possible hematologic health problem. Physiological Integrity 5. Apply knowledge of anatomy and physiology, including information about genetic risk, to perform an evidence-based assessment for the patient with a possible hematologic problem. 6. Interpret assessment findings for the patient undergoing hematologic assessment. 7. Teach the patient and caregivers about diagnostic procedures associated with hematologic assessment. 8. Explain the effects of anticoagulants, fibrinolytics, and inhibitors of platelet activity on CLOTTING and PERFUSION. 9. Prioritize nursing care for the patient after bone marrow aspiration or biopsy. The blood, blood cells, lymph, and organs involved with blood formation or blood storage compose the hematologic system. Perfusion is the total arterial blood flow through the tissues (peripheral perfusion) and blood that is pumped by the heart (central perfusion). All systems depend on the blood for oxygen perfusion, and any problem of the hematologic system affects total body health. Clotting is a complex, multi-step process by which blood forms a protein-based structure (clot) in an appropriate area of tissue injury to prevent excessive bleeding while maintaining whole-body blood flow (perfusion). 1572 FIG. 39-1 Role of the hematologic system in gas exchange and tissue PERFUSION. RBCs, Red blood cells. 1573  Anatomy and Physiology Review Bone Marrow Bone marrow is responsible for blood formation by producing red blood cells (RBCs, erythrocytes), white blood cells (WBCs, leukocytes), and platelets. Bone marrow also is involved in the immune responses (see Chapter 17). Each day the bone marrow normally releases about 2.5 billion RBCs, 2.5 billion platelets, and 1 billion WBCs per kilogram of body weight. In adults, cell-producing marrow is present only in flat bones (sternum, skull, pelvic and shoulder girdles) and the ends of long bones. With aging, fatty tissue replaces active bone marrow, and only a small portion of the remaining marrow continues to produce blood in older adults (Touhy & Jett, 2016). The bone marrow first produces blood stem cells, which are immature, unspecialized (undifferentiated) cells that are capable of becoming any type of blood cell, depending on the body's needs (Fig. 39-2) (McCance et al., 2014). FIG. 39-2 Bone marrow cell growth and blood cell differentiation pathways. The next stage in blood cell production is the committed stem cell (or precursor cell). A committed stem cell enters one growth pathway and can at that point specialize (differentiate) into only one cell type. Committed stem cells actively divide but require the presence of a specific growth factor for specialization. Blood Components Blood is composed of plasma and cells. Plasma is an extracellular fluid similar to the interstitial fluid found between tissue cells, but containing much more protein. The three major types of plasma proteins are albumin, globulins, and fibrinogen. Albumin maintains the osmotic pressure of the blood, preventing the plasma from leaking into the tissues (see Chapter 11). Globulins have many functions, such as transporting other substances and, as antibodies, protecting the body against infection. Fibrinogen is activated to form fibrin, which is critical in the blood CLOTTING process. The blood cells include RBCs, WBCs, and platelets. These cells differ in structure, site of maturation, and function. 1574  Red blood cells (erythrocytes) are the largest proportion of blood cells. The number of RBCs an adult has varies with gender, age, and general health, but the normal range is from 4,200,000 to 6,100,000/mm3 (4.2 to 6.1 × 1012/L). As shown in Figs. 39-2 and 39-3, RBCs start out as stem cells, enter the myeloid pathway, and progress in stages to mature erythrocytes. Healthy, mature, circulating RBCs have a life span of about 120 days. As RBCs age, their membranes become more fragile. These old cells are trapped and destroyed in the tissues, spleen, and liver. Some parts of destroyed RBCs (e.g., iron, hemoglobin) are recycled and used to make new RBCs. FIG. 39-3 Erythrocyte (red blood cell) growth pathway. The RBCs produce hemoglobin (Hgb). Each normal mature RBC contains hundreds of thousands of hemoglobin molecules. Each hemoglobin molecule needs iron to be able to transport up to four molecules of oxygen. Therefore iron is an essential part of hemoglobin. Hemoglobin also carries carbon dioxide. RBCs also help maintain acid-base balance. The most important feature of hemoglobin is its ability to combine loosely with oxygen. Only a small drop in tissue oxygen levels increases the transfer of oxygen from hemoglobin to tissues, known as oxygen dissociation. RBC production or erythropoiesis (selective growth of stem cells into mature erythrocytes) must be properly balanced with RBC destruction or loss. The trigger for RBC production is an increase in the tissue need for oxygen. The kidney produces the RBC growth factor erythropoietin at the same rate as RBC destruction or loss occurs to maintain a constant normal level of circulating RBCs. When tissue oxygen is less than normal (hypoxia), the kidney releases more erythropoietin, which then increases RBC production in the bone marrow. 1575  Many substances are needed to form hemoglobin and RBCs, including iron, vitamin B12, folic acid, copper, pyridoxine, cobalt, and nickel. A lack of any of these substances can lead to anemia, which results in unmet tissue oxygen needs because of a reduction in the number or function of RBCs. White blood cells (WBCs, leukocytes) also are formed in the bone marrow. The many types of WBCs all have specialized functions that provide protection through inflammation and immunity TABLE 39-1 Functions of Specific Leukocytes LEUKOCYTE FUNCTION Inflammation Neutrophil Nonspecific ingestion and phagocytosis of microorganisms and foreign protein Macrophage Nonspecific recognition of foreign proteins and microorganisms; ingestion and Phagocytosis Monocyte Destruction of bacteria and cellular debris; matures into macrophage Eosinophil Weak phagocytic action; releases vasoactive amines during allergic reactions Basophil Releases histamine and heparin in areas of tissue damage Antibody-Mediated Immunity B-lymphocyte Becomes sensitized to foreign cells and proteins Plasma cell Secretes immunoglobulins in response to the presence of a specific antigen Memory cell Remains sensitized to a specific antigen and can secrete increased amounts of immunoglobulins specific to the antigen on re-exposure Cell-Mediated Immunity T-lymphocyte Enhances immune activity through the secretion of various factors, cytokines, and helper/inducer T- Lymphokines Cell Cytotoxic-cytolytic Selectively attacks and destroys non-self cells, including virally infected cells, grafts, T-cell and transplanted organs Natural killer cell Nonselectively attacks non-self cells, especially body cells that have undergone mutation and become malignant; also attacks grafts and transplanted organs Platelets are the third type of blood cells. They are the smallest blood cells, formed in the bone marrow from megakaryocyte precursor cells. When activated, platelets stick to injured blood vessel walls and form platelet plugs that can stop the flow of blood at the injured site. They also produce substances important to blood CLOTTING and aggregate (clump together) to perform most of their functions. Platelets help keep small blood vessels intact by initiating repair after damage. Production of platelets is controlled by the growth factor thrombopoietin. After platelets leave the bone marrow, they are stored in the spleen and then released slowly to meet the body's needs. Normally 80% of platelets circulate and 20% are stored in the spleen. 1576 Accessory Organs of Blood Formation The spleen and liver are important accessory organs for blood production. They help regulate the growth of blood cells and form factors that ensure proper CLOTTING. The spleen contains three types of tissue: white pulp, red pulp, and marginal pulp. These tissues help balance blood cell production with blood cell destruction and assist with immunity. White pulp is filled with white blood cells (WBCs) and is a major site of antibody production. As whole blood filters through the white pulp, bacteria and old RBCs are removed. Red pulp is the storage site for RBCs and platelets. Marginal pulp contains the ends of many blood vessels. The spleen destroys old or imperfect RBCs, breaks down the hemoglobin released from these destroyed cells, stores platelets, and filters antigens. Anyone who has had a splenectomy has reduced immune functions and an increased risk for infection and sepsis. The liver produces prothrombin and other blood CLOTTING factors. Also, proper liver function is important in forming vitamin K in the intestinal tract. (Vitamin K is needed to produce clotting factors VII, IX, and X and prothrombin.) Large amounts of whole blood and blood cells can be stored in the liver. The liver also stores extra iron within the protein ferritin. Hemostasis and Blood Clotting Hemostasis is the multi-stepped process of controlled blood CLOTTING. It results in localized blood clotting in damaged blood vessels to prevent excessive blood loss while continuing blood PERFUSION to all other areas. This complex function balances blood clotting actions with anti-clotting actions. When injury occurs, hemostasis starts the formation of a platelet plug and continues with a series of steps that eventually cause the formation of a fibrin clot. Three sequential processes result in blood clotting: platelet aggregation with platelet plug formation, the blood clotting cascade, and the formation of a complete fibrin clot. Platelet aggregation begins forming a platelet plug by having platelets clump together, a process essential for blood CLOTTING. Platelets normally circulate as individual small cells that do not clump together until activated. Activation causes platelet membranes to become sticky, allowing them to clump together. When platelets clump, they form large, semi-solid plugs in blood vessels, disrupting local blood flow. These platelet plugs are not clots and last only a few hours. Thus they cannot provide complete hemostasis but only start the hemostatic process. Substances that activate platelets and cause clumping include adenosine diphosphate (ADP), calcium, thromboxane A2 (TXA2), and collagen. Platelets secrete some of these substances, and other activating substances are external to the platelet. Platelet plugs start the cascade action that ends with local blood CLOTTING and are important at most steps within the cascade. When too few platelets are present, clotting is impaired, increasing the risk for excessive bleeding. Blood clotting is a cascade triggered by the formation of a platelet plug, which then rapidly amplifies the cascade. The final result is much larger than the triggering event. Thus the cascade works like a landslide—a few stones rolling down a steep hill can eventually dislodge large rocks, trees, and soil, causing an enormous movement of earth. Just like landslides, cascade reactions are hard to stop once set into motion. Intrinsic factors are conditions, such as circulating debris or venous stasis, within the blood itself that can activate platelets and trigger the blood CLOTTING cascade (Fig. 39-4). Continuing the cascade to blood clotting requires sufficient amounts of all the clotting factors and cofactors (Table 39-2). FIG. 39-4 Summary of the blood clotting cascade. 1577  TABLE 39-2 - The Clotting Factors FACTOR ACTION I: Fibrinogen Factor I is converted to fibrin by the enzyme thrombin. Individual fibrin molecules form fibrin threads, which are the mesh for clot formation and wound healing. II: Prothrombin Factor II is the inactive thrombin. Prothrombin is activated to thrombin by clotting factor X. Activated thrombin converts fibrinogen (clotting factor I) into fibrin and activates factors V and VIII. Synthesis is vitamin K–dependent. III: Tissue Factor III interacts with factor VII to initiate the extrinsic clotting cascade. thromboplastin IV: Calcium Calcium (Ca2+), a divalent cation, is a cofactor for most of the enzyme- activated processes required in blood clotting. Calcium enhances platelet aggregation and makes red blood cells clump together. V: Proaccelerin Factor V is a cofactor for activated factor X, which is essential for converting prothrombin to thrombin. VI: Is an artifact No factor VI is involved in blood clotting. VII: Proconvertin Factor VII activates factors IX and X, which are essential in converting prothrombin to thrombin. Synthesis is vitamin K– dependent. VIII: Antihemophilic Factor VIII together with activated factor IX activates factor X. Factor VIII combines factor with von Willebrand's factor to help platelets adhere to capillary walls in areas of tissue injury. A lack of factor VIII results in classic hemophilia (hemophilia A). IX: Plasma Factor IX, when activated, activates factor X to convert thromboplastin prothrombin to thrombin. A lack of factor IX causes component (Christmas hemophilia B. factor) Synthesis is vitamin K–dependent. X: Stuart-Prower factor Factor X, when activated, converts prothrombin into thrombin. Synthesis is vitamin K–dependent. XI: Plasma Factor XI, when activated, assists in the activation of factor IX. However, a similar thromboplastin factor must exist in tissues. People who are deficient in factor XI have mild bleeding antecedent problems. XII: Hageman factor Factor XII is critically important in the intrinsic pathway for the activation of factor XI. XIII: Fibrin-stabilizing Factor XIII assists in forming cross-links among the fibrin threads to form a strong factor fibrin clot. Extrinsic factors outside of the blood can also activate platelets. The most common extrinsic event is trauma that damages blood vessels and exposes the collagen in vessel walls. Collagen then activates platelets to form a platelet plug within seconds. The blood clotting cascade is started sooner by this pathway because some intrinsic pathway steps are bypassed. Other blood vessel changes that can activate platelets include inflammation, bacterial toxins, or foreign proteins. Whether the platelet plugs are formed because of abnormal blood (intrinsic factors) or by exposure to inflamed or damaged blood vessels (extrinsic factors), the end result of the cascade is the same: formation of a fibrin clot and local blood CLOTTING (coagulation). The cascade, from the formation of a platelet plug to the formation of a fibrin clot, depends on the presence of specific clotting factors, calcium, and more platelets at every step. Fibrin clot formation is the last phase of blood CLOTTING. Fibrinogen is an inactive protein made in the liver. The activated enzyme thrombin removes the end portions of fibrinogen, converting it to active fibrin that can link together to form fibrin threads. Fibrin threads make a meshlike base to form a blood clot. After the fibrin mesh is formed, clotting factor XIII tightens up the mesh, making it more dense and stable. 1578 Anti-Clotting Forces Because blood CLOTTING occurs through a rapid cascade process, in theory it keeps forming fibrin clots whenever the cascade is set into motion until all blood throughout the entire body has coagulated and PERFUSION stops. Therefore, whenever the clotting cascade is started, anti-clotting forces are also started to limit clot formation only to damaged areas so normal perfusion is maintained everywhere else. When blood clotting and anti-clotting actions are balanced, clotting occurs only where it is needed, and normal perfusion is maintained. The anti-clotting forces both ensure that activated clotting factors are present only in limited amounts and also cause fibrinolysis to prevent over enlargement of the fibrin clot. Fibrinolysis is the process that dissolves fibrin clot edges with special enzymes (Fig. 39-5). The process starts by activating plasminogen to plasmin. Plasmin, an active enzyme, then digests fibrin, fibrinogen, and prothrombin, controlling the size of the fibrin clot (McCance et al., 2014).  FIG. 39-5 The process of fibrinolysis. When the blood clotting cascade is activated, certain additional anti-clotting substances are also activated, such as protein C, protein S, and antithrombin III. Protein C and protein S increase the breakdown of clotting factors V and VIII. Antithrombin III inactivates thrombin and clotting factors IX and X. These actions prevent clots from becoming too large or forming in an area where CLOTTING is not needed. Deficiency of any anti-clotting factor increases the risk for pulmonary embolism, myocardial infarction, and strokes. Hematologic Changes Associated With Aging Aging changes the blood components (Touhy & Jett, 2016). The older adult has a decreased blood volume with lower levels of plasma proteins. The lower plasma protein level may be related to a low dietary intake of proteins and to reduced protein production by the older liver. Chart 39-1 lists assessment tips for older adults. Chart 39-1 Nursing Focus on the Older-Adult Hematologic Assessment NORMAL CHANGES IN THE FINDINGS IN HEMATOLOGIC DISORDERS SIGNIFICANCE/ALTERNATIVES OLDER ADULT Nail Beds (for Capillary Refill) Pallor or cyanosis may indicate a hematologic Thickened or discolored nails make Use another body area, such as the lip, to assess central capillary refill. disorder. viewing color of nail beds impossible. Hair Distribution Thin or absent hair on the trunk or extremities Progressive loss of body hair is a A relatively even pattern of hair loss that has occurred over an extended period is not may indicate poor PERFUSION to a particular normal facet of aging. significant. Older adults also have decreased pubic hair as a result of age-related hormone area. changes. Skin Moisture Skin dryness may indicate any of a number of Skin dryness is a normal result of Skin moisture is not usually a reliable indicator of an underlying pathologic condition in the hematologic disorders. aging. older adult. Skin Color Skin color changes, especially pallor and jaundice, Pigment loss and skin yellowing are Pallor in an older adult may not be a reliable indicator of anemia. Laboratory testing is are associated with some hematologic disorders. common changes associated with required. aging. Yellow-tinged skin in an older adult may not be a reliable indicator of increased serum bilirubin levels. Laboratory testing is required. As bone marrow ages, it produces fewer blood cells. Total red blood cell (RBC) and white blood cell (WBC) counts are lower among older adults, although platelet counts do not change. Lymphocytes become less reactive to antigens and lose immune function. Antibody levels and responses are lower and slower in older adults. The WBC count does not rise as high in response to infection in older adults as it does in younger adults. Hemoglobin levels in men and women fall after middle age. Iron-deficient diets may play a role in this reduction. NCLEX Examination Challenge 39-1 Safe and Effective Care Environment With which client will the nurse apply pressure to an injection site for 5 minutes because of an increased risk for bleeding? A. 28-year-old who has had type 1 diabetes for 15 years B. 42-year-old newly diagnosed with type 2 diabetes 1579 C. 58-year-old with chronic hypertension and heart failure D. 62-year-old with extensive liver damage from cirrhosis 1580 Assessment: Noticing and Interpreting Patient History Age and gender are important to consider when assessing the patient's hematologic status. Bone marrow function and immune activity decrease with age. Gender Health Considerations Patient-Centered Care At all ages, women have lower red blood cell counts than do men. This difference is greater during menstrual years because menstrual blood loss may occur faster than blood cell production. This difference also is related to blood dilution caused by fluid retention from female hormones. Always assess for RBC adequacy in a woman hospitalized for any reason. Liver function, the presence of known immunologic or hematologic disorders, current drug use, dietary patterns, and socioeconomic status are important to assess. Because the liver makes CLOTTING factors, ask about symptoms that may indicate liver problems, such as jaundice, anemia, and gallstones. Previous radiation therapy for cancer may impair hematologic function if marrow- forming bones were in the radiation path. Ask about the patient's occupation and hobbies and whether the home is located near an industrial setting. This information may identify exposure to agents that affect bone marrow and hematologic function. Check all drugs that the patient is using or has used in the past 3 weeks. Ask about the use of drugs listed in Table 39-3 that are known to change hematologic function. Check a drug handbook to determine whether other drugs the patient takes can affect hematologic function. TABLE 39-3 Drugs Impairing the Hematologic System DRUGS CAUSING BONE MARROW SUPPRESSION DRUGS CAUSING DRUGS DISRUPTING PLATELET ACTION HEMOLYSIS Altretamine Acetohydroxamic acid Aspirin Amphotericin B Amoxicillin Carbenicillin Azathioprine Chlorpropamide Carindacillin Chemotherapeutic agents Doxapram Dipyridamole Chloramphenicol Glyburide Ibuprofen Chromic phosphate Mefenamic acid Meloxicam Colchicine Menadiol diphosphate Naproxen Didanosine Methyldopa Oxaprozin Eflornithine Nitrofurantoin Pentoxifylline Foscarnet sodium Penicillin G benzathine Sulfinpyrazone Ganciclovir Penicillin V Ticarcillin Interferon alfa Primaquine Ticlopidine Pentamidine Procainamide hydrochloride Valproic acid Sodium iodide Quinidine polygalacturonate Zalcitabine Quinine Zidovudine Sulfonamides Tolbutamide Vitamin K Ask the patient about use of blood “thinners” and NSAIDs, which change blood CLOTTING activity. Such drugs include anticoagulants and platelet inhibitors. Many patients refer to these drugs as blood thinners, although they do not change blood thickness (viscosity). Fig. 39-4 shows where in the blood clotting cascade these agents work. Anticoagulant drugs work by interfering with one or more steps involved in the blood CLOTTING cascade. Thus these agents prevent new clots from forming and limit or prevent extension of formed clots. Anticoagulants do not break down existing clots. These drugs are classified as direct thrombin inhibitors, indirect thrombin inhibitors, and vitamin K antagonists. Direct thrombin inhibitors (DTIs) can be given by the parenteral route and orally. The parenteral drugs include lepirudin (Refludan), desirudin (Iprivask), bivalirudin (Angiomax), and argatroban (ARGATROBAN, Novastin ). Oral agents include apixaban (Eliquis), dabigatran (Pradaxa, Pradax ), edoxaban (Savaysa), and rivaroxaban (Xarelto). These drugs prevent the conversion of prothrombin (factor X) to its active form, thrombin (factor Xa). Less thrombin disrupts the CLOTTING cascade by reducing the amount of fibrinogen that is converted to active fibrin (Burchum & 1581 Rosenthal, 2016). A clinical problem with the DTIs has been a lack of an antidote to administer when excessive bleeding is present as a result of DTI therapy. A recently approved IV antidote for dabigatran is idarucizumab (Praxbind), which is a monoclonal antibody that specifically binds to the structure of dabigatran (O'Malley, 2015; Siegal, et al., 2015). It is not effective against other thrombin inhibitors. Two other drugs under current study for reversal of bleeding related to other thrombin inhibitors are andexanet alfa and ciraparantag (Aripazine). Indirect thrombin inhibitors include the heparins and heparinoids. These drugs include enoxaparin (Lovenox), dalteparin (Fragmin), tinzaparin (Innohep), and fondaparinux (Arixtra). All are given parenterally. Lower molecular weight drugs are preferred for home use. The drugs cause anticoagulation by binding to and increasing the activity of antithrombin III (AT III). By activating ATIII, coagulation factor Xa (thrombin) is indirectly inhibited. Vitamin K antagonists (VKAs) decrease vitamin K synthesis in the intestinal tract, which then reduces the production of vitamin K–dependent CLOTTING factors II, VII, IX, and X. When clotting factor synthesis is reduced, anticoagulation results. The most commonly used VKA is warfarin (Coumadin, Jantoven), an oral agent. Fibrinolytic drugs (also known as thrombolytic drugs or “clot busters”) selectively break down fibrin threads present in formed blood clots. The mechanism starts with activation of the inactive tissue protein plasminogen to its active form, plasmin. Plasmin directly attacks and degrades the fibrin molecule. Fibrinolytic drugs include alteplase (Activase), reteplase (Retavase), tenecteplase (TNKase), and urokinase (Abbokinase, Kinlytic). All are IV agents. Urokinase is approved for use only in patients who have a massive pulmonary embolism. The use of fibrinolytic drugs results in the best clot breakdown with less disruption of blood CLOTTING. These drugs are the first-line therapy for problems caused by small, localized formed clots such as myocardial infarction (MI), limited arterial thrombosis, and thrombotic strokes. For some problems such as MI, these drugs are usually given only within the first 6 hours after the onset of symptoms. This time limitation is not related to drug activity because fibrinolytic agents can break down clots older than 6 hours. Rather, the tissue that has been anoxic for more than 6 hours as a result of an acute event is not likely to benefit from this therapy, making the risks to the patient greater than the advantages. Platelet inhibitors or antiplatelet drugs prevent either platelet activation or aggregation (clumping). The most widely used drug for this effect is aspirin, which irreversibly inhibits the production of substances that activate platelets, such as thromboxane. Other drugs change the platelet membrane, reducing its “stickiness,” or prevent activators from binding to platelet receptors by inhibiting a variety of enzymes important to platelet activation. These drugs include cilostazol (Pletal), clopidogrel (Plavix), dipyridamole (Persantine), prasugrel (Effient), ticagrelor (Brilinta), and ticlopidine (Ticlid). Another group of drugs that inhibits platelets by binding to certain membrane proteins includes abciximab (ReoPro), eptifibatide (Integrilin), and tirofiban (Aggrastat), which are all administered parenterally. The complementary therapy agents St. John's wort and Ginkgo biloba also inhibit platelet activity. Nutrition Status Diet can alter cell quality and affect CLOTTING. Ask patients to recall what they have eaten during the past week. Use this information to assess possible iron, protein, mineral, or vitamin deficiencies. Diets high in fat and carbohydrates and low in protein, iron, and vitamins can cause many types of anemia and decrease the functions of all blood cells. Diets high in vitamin K, found in leafy green vegetables, may increase the rate of blood clotting. Assess the amount of salads and other raw vegetables that the patient eats and whether supplemental vitamins and calcium are used. Ask about alcohol consumption because chronic alcoholism causes nutrition deficiencies and impairs the liver, both of which reduce blood CLOTTING. Ask about personal resources, such as finances and social support. An adult with a low income may have a diet deficient in iron and protein because foods containing these substances are more expensive. Family History and Genetic Risk Assess family history because many disorders affecting blood and blood CLOTTING are inherited. 1582 Ask whether anyone in the family has had hemophilia, frequent nosebleeds, postpartum hemorrhages, excessive bleeding after tooth extractions, or heavy bruising after mild trauma. Ask whether any family member has sickle cell disease or sickle cell trait. Although sickle cell disease is seen most often among African Americans, anyone can have the trait. Current Health Problems Ask about lymph nodes swelling, excessive bruising or bleeding, and whether the bleeding was spontaneous or induced by trauma. Ask about the amount and duration of bleeding after routine dental work. Ask women to estimate the number of pads or tampons used during the most recent menstrual cycle and whether this amount represents a change from the usual pattern of flow. Ask whether clots are present in menstrual blood. If menstrual clots occur, ask women to estimate clot size using coins or fruit for comparison. Assess and record whether the patient has shortness of breath on exertion, palpitations, frequent infections, fevers, recent weight loss, headaches, or paresthesias. Any or all of these symptoms may occur with hematologic disease. The most common symptom of anemia is fatigue as a result of decreased oxygen delivery to cells. Cells use oxygen to produce the high-energy chemical adenosine triphosphate (ATP) needed to perform most cellular work. When oxygen delivery to cells is reduced, cellular work decreases, and fatigue increases. Ask patients about feeling tired, needing more rest, or losing endurance during normal activities. Ask them to compare their activities during the past month with those of the same month a year ago. Determine whether other symptoms of anemia, such as vertigo, tinnitus, and a sore tongue, are present. Physical Assessment Assess the whole body because blood problems may reduce oxygen delivery and tissue PERFUSION to all systems (Jarvis, 2016). Some assessment findings associated with hematologic problems are less reliable when seen in the older adult (see Chart 39-1). Equipment needed for hematologic assessment includes gloves, a stethoscope, a blood pressure cuff, and a penlight. Remember to gently handle the patient suspected of having a hematologic problem or reduced CLOTTING to avoid causing bruising, petechiae, or excessive bleeding. Skin Assessment Inspect the skin and mucous membranes for pallor or jaundice. Assess nail beds for pallor or cyanosis. Pallor of the gums, conjunctivae, and palmar creases (when the palm is stretched) indicates decreased hemoglobin levels and poor tissue oxygenation. Assess the gums for active bleeding in response to light pressure or brushing the teeth with a soft-bristled brush and assess any lesions or draining areas. Inspect for petechiae and large bruises (ecchymoses). Petechiae are pinpoint hemorrhagic lesions in the skin. Bruises may cluster together. For hospitalized patients, determine whether there is bleeding around nasogastric tubes, endotracheal tubes, central lines, peripheral IV sites, or Foley catheters. Check the skin turgor and ask about itching because dry skin from poor perfusion itches. Assess body hair patterns. Areas with poor circulation, especially the lower legs and toes, may have sparse or absent hair, although this may be a normal finding in an older adult. Cultural/Spiritual Considerations Patient-Centered Care Pallor and cyanosis are more easily detected in adults with darker skin by examining the oral mucous membranes and the conjunctiva of the eye. Jaundice can be seen more easily on the roof of the mouth. Petechiae may be visible only on the palms of the hands or the soles of the feet. Bruises can be seen as darker areas of skin and palpated as slight swellings or irregular skin surfaces. Ask the patient about pain when skin surfaces are touched lightly or palpated. (Chapter 24 provides tips for assessing darker skin.) 1583 Head and Neck Assessment Check for pallor or ulceration of the oral mucosa. The tongue is smooth in pernicious anemia and iron deficiency anemia or smooth and beefy red in other nutrition deficiencies. These symptoms may occur with fissures at the corners of the mouth. Assess for scleral jaundice. Inspect and palpate all lymph node areas. Document any lymph node enlargement, including whether palpation of the enlarged node causes pain and whether the enlarged node moves or remains fixed with palpation. Respiratory Assessment When blood problems reduce oxygen delivery, the lungs work harder to maintain tissue PERFUSION. Assess the rate and depth of respiration while the patient is at rest and during and after mild physical activity (e.g., walking 20 steps in 10 seconds). Note whether the patient can complete a 10- word sentence without stopping for a breath. Assess whether he or she is fatigued easily, has shortness of breath at rest or on exertion, or needs extra pillows to breathe well at night. Anemia can cause these problems as a result of respiratory changes made as adjustments to the reduced tissue oxygen levels. Cardiovascular Assessment When blood problems reduce oxygen delivery, the heart works harder to help maintain tissue PERFUSION. Pulses may become weak and thready. Observe for distended neck veins, edema, or indications of phlebitis. Use a stethoscope to listen for abnormal heart sounds and irregular rhythms. Assess blood pressure (BP). Systolic BP tends to be lower than normal in patients with anemia and higher than normal when the patient has excessive red blood cells. Kidney and Urinary Assessment The kidneys have many blood vessels, and bleeding problems may cause hematuria (blood in the urine). Inspect urine for color. Hematuria may be seen as grossly bloody red or dark-brownish gold urine. Test the urine for proteins with a urine test dipstick because blood contains protein and blood in the urine increases its protein content. Keep in mind that the adult with chronic kidney disease (CKD) produces less natural erythropoietin and often is anemic. Musculoskeletal Assessment Rib or sternal tenderness may occur with leukemia (blood cancer) when the bone marrow overproduces cells, increasing the pressure in the bones. Examine the skin over superficial bones, including the ribs and sternum, by applying firm pressure with the fingertips. Assess the range of joint motion and document any swelling or joint pain. Abdominal Assessment The normal adult spleen is usually not palpable, but an enlarged spleen occurs with many hematologic problems. An enlarged spleen may be detected by palpation, but this is usually performed by the primary health care provider because an enlarged spleen is tender and ruptures easily. Nursing Safety Priority Action Alert Do not palpate the splenic area of the abdomen for any patient with a suspected hematologic problem. An enlarged spleen ruptures easily and can lead to hemorrhage and death. Palpating the edge of the liver in the right upper quadrant of the abdomen can detect enlargement, which often occurs with hematologic problems. The normal liver may be palpable as much as 4 to 5 cm below the right costal margin but is usually not palpable in the epigastrium. A common cause of anemia among older adults is a chronically bleeding GI ulcer or intestinal polyp. If the ulcer is located in the stomach or the small intestine, obvious blood may not be visible 1584 in the stool, or such a small amount is passed each day that the patient is not aware of it. Obtain a stool specimen for occult blood testing. Central Nervous System Assessment Assessing cranial nerves and testing neurologic function are important in hematologic assessment because some problems cause specific changes. Vitamin B12 deficiency impairs nerve function, and severe chronic deficiency may cause permanent neurologic degeneration. Many neurologic problems can develop in patients who have leukemia because leukemia can cause bleeding, infection, or tumor spread within the brain. When the patient with a suspected bleeding disorder has any head trauma, expand the assessment to include frequent neurologic checks and checks of cognitive function (see Chapter 41). NCLEX Examination Challenge 39-2 Safe and Effective Care Environment The nurse performing a hematologic assessment on an older-adult client identifies the following findings. Which ones does the nurse associate with age-related changes rather than a specific hematologic problem? Select all that apply. A. Bleeding gums B. Dry skin on distal extremities C. Pale lips D. Smooth tongue E. Sparse pubic hair F. Bright yellow-tinged sclera Psychosocial Assessment Regardless of the type of hematologic problem, each patient brings his or her own coping style to the illness. Develop a rapport with the patient and learn which coping mechanisms he or she has used successfully in the past. Ask the patient and family members about social support networks and financial resources. A problem in these areas can interfere with the patient's adherence to therapy. Diagnostic Assessment Laboratory Tests Laboratory test results provide definitive information about hematologic problems. Chart 39-2 lists laboratory data used to assess hematologic function. When a venipuncture is necessary, apply pressure to the site for at least 5 minutes on a patient suspected of having a hematologic problem to prevent bleeding and hematoma formation. Chart 39-2 Laboratory Profile Hematologic Assessment TEST REFERENCE RANGE CANADIAN REFERENCE SIGNIFICANCE OF ABNORMAL FINDINGS UNITS Red blood cell (RBC) count Females: 4.2-5.4 million/ 4.2-5.4 × 1012 cells/L Decreased levels indicate possible anemia or hemorrhage. µL Increased levels indicate possible chronic hypoxia or polycythemia vera. Males: 4.7-6.1 4.7-6.1 × 1012 cells/L million/ µL 1585 Hemoglobin (Hgb) Females: 12-16 g/dL 120-160 g/L Same as for RBC. Males: 14-18 g/dL 140-180 g/L Hematocrit (Hct) Females: 37%-47% 0.37-0.47 volume fraction Same as for RBC. Males: 42%-52% 0.42-0.52 volume fraction Mean corpuscularvolume (MCV) 80-95 fL Same as referencerange Increased levels indicate macrocytic cells, possible anemia. Decreased levels indicate microcytic cells, possible iron deficiency anemia. Mean corpuscularhemoglobin (MCH) 27-31 pg Same as referencerange Same as for MCV. Mean corpuscular 32-36 g/dL or Same as referencerange Increased levels may indicate spherocytosis or anemia. hemoglobin concentration 32%-36% Decreased levels may indicate iron deficiency anemia or a hemoglobinopathy. (MCHC) White blood cell (WBC) count 5000-10,000/mm3 5.0-10.0 × 109 cells/L Increased levels are associated with infection, inflammation, autoimmune disorders, and leukemia. Decreased levels may indicate prolonged infection or bone marrow suppression. Reticulocyte count 0.5%-2.0% of RBCs Same as referencerange Increased levels may indicate chronic blood loss. Decreased levels indicate possible inadequate RBC production. Total iron-bindingcapacity(TIBC) 250-460 mcg/dL 45-82 mcmol/L Increased levels indicate iron deficiency. Decreased levels may indicate anemia, hemorrhage, hemolysis. Iron (Fe) Females: 60-160 mcg/dL 11-29 mcmol/L Increased levels indicate iron excess, liver disorders, hemochromatosis, megaloblastic Males: 80-180 mcg/dL 14-32 mcmol/L anemia. Decreased levels indicate possible iron deficiency anemia, hemorrhage. Serum ferritin Females: 10-150 ng/mL 10-150 mcg/L Same as for iron. Males: 12-300 ng/mL 12-300 mcg/L Platelet count 150,000-400,000/mm3 150-400 × 109/L Increased levels may indicate polycythemia vera or malignancy. Decreased levels may indicate bone marrow suppression, autoimmune disease, hypersplenism. Hemoglobin electrophoresis Hgb A1: 95%-98% Same as referencerange Variations indicate hemoglobinopathies. Hgb A2: 2%-3% Hgb F: 0.8%-2% Hgb S: 0% Hgb C: 0% Hgb E: 0% Direct and indirect Coombs'test Negative Negative Positive findings indicate antibodies to RBCs. International normalized ratio (INR) 0.8-1.1 times the control Same as referencerange Increased values indicate longer clotting times. This is desirable for anticoagulation value therapy with warfarin. Decreased values indicate hypercoagulation and increased risk for venous thromboembolic events. Prothrombin time (PT) 11-12.5 sec Same as reference range Increased time indicates possible deficiency of clotting factors V and VII. 85%-100% Decreased time may indicate vitamin K excess. fL, Femtoliter; pg, picograms. Data from Pagana, K., Pagana, T., & Pike-MacDonald, S. (2013). Mosby's Canadian manual of diagnostic and laboratory tests. St. Louis: Mosby; Pagana, K., Pagana, T. J., & Pagana, T. N. (2017). Mosby's diagnostic and laboratory test reference (13th ed.). St. Louis: Mosby. Tests of Cell Number and Function. A peripheral blood smear is made by taking a drop of blood and spreading it over a slide. It can be read by an automated calculator or a technologist with a microscope. This rapid test provides information on the sizes, shapes, and proportions of different blood cell types within the peripheral blood. A complete blood count (CBC) includes a number of studies: red blood cell (RBC) count, white blood cell (WBC) count, hematocrit, and hemoglobin level. The RBC count measures circulating RBCs in 1 mm3 (or 1 L) of blood. The WBC count measures all leukocytes present in 1 mm3 (or 1 L) of blood. To determine the percentages of different types of leukocytes circulating in the blood, a WBC count with differential leukocyte count is performed (see Chapter 17). The hematocrit (Hct) is the percentage of RBCs in the total blood volume (also known as volume fraction). The hemoglobin (Hgb) level is the total amount of hemoglobin in blood and is measured as g/dL (or g/L). The CBC can measure other features of the RBCs. The mean corpuscular volume (MCV) measures the average volume or size of individual RBCs and is useful for classifying anemias. When the MCV is elevated, the cell is larger than normal (macrocytic), as seen in megaloblastic anemias. When the MCV is decreased, the cell is smaller than normal (microcytic), as seen in iron deficiency anemia. The mean corpuscular hemoglobin (MCH) is the average amount of hemoglobin by weight in a single RBC. The mean corpuscular hemoglobin concentration (MCHC) measures the average amount of hemoglobin by percentage in a single RBC. When the MCHC is decreased, the cell has a hemoglobin deficiency and is hypochromic (a lighter color), as in iron deficiency anemia. These three tests can help determine possible causes of low RBC counts that are not related to blood loss. Reticulocyte count is helpful in determining bone marrow function. A reticulocyte is an immature RBC that still has its nucleus. An elevated reticulocyte count indicates that RBCs are being produced and released by the bone marrow before they mature. Normally only about 2% of circulating RBCs are reticulocytes. An elevated reticulocyte count is desirable in an anemic patient or after hemorrhage because this indicates that the bone marrow is responding to a decrease in the total RBC level. An elevated reticulocyte count without a precipitating cause usually indicates health problems, such as polycythemia vera (a malignant condition in which the bone marrow overproduces RBCs). A platelet count, also known as a thrombocyte count, reflects the number of platelets in circulation. The normal range is 150,000 to 400,000/mm 3 (150 to 400 × 109/L). When this value is low (thrombocytopenia), the patient is at greater risk for bleeding because platelets are critical for blood clotting. Patients who have values between 40,000/mm 3 (40 × 109/L) and 80,000/mm 3 (800 × 109/L) 1586 may have prolonged bleeding from trauma, dental work, and surgery. With platelet values below 20,000/mm3 (20 × 109/L), the patient may have spontaneous bleeding that is very difficult to stop. Hemoglobin electrophoresis detects abnormal forms of hemoglobin, such as hemoglobin S in sickle cell disease. Hemoglobin A is the major type of hemoglobin in an adult. Leukocyte alkaline phosphatase (LAP) is an enzyme produced by normal mature neutrophils. Elevated LAP levels occur during episodes of infection or stress. An elevated neutrophil count without an elevation in LAP level occurs with some types of leukemia. Coombs' tests, both direct and indirect, are used for blood typing. The direct test detects antibodies against RBCs that may be attached to a patient's RBCs. Although healthy adults can make these antibodies, in certain diseases (e.g., systemic lupus erythematosus, mononucleosis) these antibodies are directed against the patient's own RBCs. Excessive amounts of these antibodies can cause hemolytic anemia (Pagana et al., 2017). The indirect Coombs' test detects the presence of circulating antiglobulins. The test is used to determine whether the patient has serum antibodies to the type of RBCs that he or she is about to receive by blood transfusion (Pagana et al., 2017). Serum ferritin, transferrin, and the total iron-binding capacity (TIBC) tests measure iron levels. Abnormal levels of iron and TIBC occur with problems such as iron deficiency anemia. The serum ferritin test measures the amount of free iron present in the plasma, which represents 1% of the total body iron stores. Therefore the serum ferritin level provides a means to assess total iron stores. Adults with serum ferritin levels at least 10 ng/100 mL have adequate iron stores; adults with levels less than 10 ng/100 mL have inadequate iron stores and have difficulty recovering from any blood loss. Transferrin is a protein that transports dietary iron from the intestines to cell storage sites. Measuring the amount of iron that can be bound to serum transferrin indirectly determines whether an adequate amount of transferrin is present. This test is the total iron-binding capacity (TIBC) test. Normally only about 30% of the transferrin is bound to iron in the blood. TIBC increases when a patient is deficient in serum iron and stored iron levels. Such a value indicates that an adequate amount of transferrin is present but less than 30% of it is bound to serum iron. Tests Measuring Bleeding and Coagulation. Tests that measure bleeding and coagulation provide information that reflects the effectiveness of different aspects of blood CLOTTING. These tests are used to diagnose specific hematologic health problems, determine drug therapy effectiveness, and identify risk for excessive bleeding or clotting. Prothrombin time (PT) measures how long blood takes to clot, reflecting the level of clotting factors II, V, VII, and X and how well they are functioning. When enough of these clotting factors are present and functioning, the PT shows blood CLOTTING between 11 and 12.5 seconds or within 85% to 100% of the time needed for a control sample of blood to clot. PT is prolonged when one or more of these clotting factors are deficient. The PT test is now used less often to assess how fast blood clots, because control blood is taken from different adults and may not be the same even in one laboratory from one day to the next. To reduce PT errors as a result of control blood variation or in some of the chemicals used in the test, the international normalized ratio is used to assess clotting time. International normalized ratio (INR) measures the same process as the PT by establishing a normal mean or standard for PT. The INR is calculated by dividing the patient's PT by the established standard PT. A normal INR ranges between 0.8 and 1.1 (Pagana et al., 2013; Pagana et al., 2017). When using the INR to monitor warfarin therapy, the desired outcome is usually to maintain the patient's INR between 2.0 and 3.0, regardless of the actual PT in seconds. However, the desired INR range for any patient is individualized for specific patient factors and medical conditions. The partial thromboplastin time (PTT) assesses the intrinsic CLOTTING cascade and the action of factors II, V, VIII, IX, XI, and XII. PTT is prolonged whenever any of these factors is deficient, such as in hemophilia or disseminated intravascular coagulation (DIC). Because factors II, IX, and X are vitamin K–dependent and are produced in the liver, liver disease can prolong the PTT. Desired therapeutic ranges for anticoagulation are usually between 1.5 and 2.0 times normal values but can be greater depending on the reason the adult is receiving anticoagulation therapy. The anti-factor Xa test measures the amount of anti-activated factor X (anti-Xa) in blood, which is affected by heparin. It is used mainly to monitor heparin levels in patients treated with either standard unfractionated heparin or low-molecular-weight heparin. For adults not receiving heparin 1587 in any form, the reference range is less than 0.1 IU/mL. The usual therapeutic range for patients receiving standard heparin is 0.5 to 1.0 IU/mL, and the usual therapeutic range for patients receiving low-molecular-weight heparin is 0.3 to 0.7 IU/mL. Test results are affected by age, gender, health history, and the specific laboratory technique used for the test. Platelet aggregation, or the ability to clump, is tested by mixing the patient's plasma with an agonist substance that should cause clumping. The degree of clumping is noted. Aggregation can be impaired in von Willebrand's disease and during the use of drugs such as aspirin, anti- inflammatory agents, psychotropic agents, and platelet inhibitors. NCLEX Examination Challenge 39-3 Health Promotion and Maintenance What is the most important precaution for the nurse to teach a client whose platelet counts usually range between 50,000 to 60,000/mm3 (50 × 109/L to 60 × 109/L)? A. “Drink at least 3 liters of fluid daily.” B. “Take a multiple vitamin that contains iron.” C. “Avoid aspirin and aspirin-containing drugs.” D. “Increase your intake of dark green, leafy vegetables.” Imaging Assessment Assessment of the patient with a suspected hematologic problem can include radioisotopic imaging. Isotopes are used to evaluate the bone marrow for sites of active blood cell formation and iron storage. Radioactive colloids are used to determine organ size and liver and spleen function. The patient is given an IV radioactive isotope by about 3 hours before the procedure. Once in the nuclear medicine department, he or she must lie still for about an hour during the scan. No special patient preparation or follow-up care is needed for these tests. Standard x-rays may be used to diagnose some hematologic problems. For example, multiple myeloma causes classic bone destruction, with a “Swiss cheese” appearance on x-ray. Bone Marrow Aspiration and Biopsy Bone marrow aspiration and biopsy, which are similar invasive procedures, help evaluate the patient's hematologic status when other tests show abnormal findings that indicate a possible problem in blood cell production or maturation. Results provide information about bone marrow function, including the production of all blood cells and platelets. In a bone marrow aspiration, cells and fluids are suctioned from the bone marrow. In a bone marrow biopsy, solid tissue and cells are obtained by coring out an area of bone marrow with a large-bore needle. A hematologic health care provider's prescription and a signed informed consent are obtained before either procedure is performed. Bone marrow aspiration may be performed by a physician, an advanced practice nurse, or a physician assistant, depending on the agency's policy and regional law. The procedure may be performed at the patient's bedside, in an examination room, or in a laboratory. After learning which specific tests will be performed on the marrow, check with the hematology laboratory to determine how to handle the specimen. Some tests require that heparin or other solutions be added to the specimen. Patient Preparation. Most patients are anxious before a bone marrow aspiration, even those who have had one in the past. You can help reduce anxiety and allay fears by providing accurate information and emotional support. Some patients like to have their hand held during the procedure. Explain the procedure and reassure the patient that you will stay during the entire procedure. Tell the patient that the local anesthetic injection will feel like a stinging or burning sensation. Tell him or her to expect a heavy sensation of pressure and pushing while the needle is being inserted. 1588 Sometimes a crunching sound can be heard or scraping sensation felt as the needle punctures the bone. Explain that a brief sensation of painful pulling will be experienced as the marrow is being aspirated by mild suction in the syringe. If a biopsy is performed, the patient may feel more discomfort as the needle is rotated into the bone. Assist the patient onto an examining table and expose the site (usually the iliac crest). If this site is not available or if more marrow is needed, the sternum may be used. If the iliac crest is the site, place the patient in the prone or side-lying position. Depending on the tests to be performed on the specimen, a laboratory technician may also be present to ensure its proper handling. Procedure. The procedure usually lasts from 5 to 15 minutes. The type and amount of anesthesia or sedation depend on the clinician's preference, the patient's preference and previous experience with bone marrow aspiration and biopsy, and the setting. A local anesthetic agent is injected into the skin around the site. The patient may also receive a mild tranquilizer or a rapid-acting sedative, such as midazolam (Versed), lorazepam (Ativan, Apo- Lorazepam , Novo-Lorazem ), or etomidate (Amidate). Some patients do well with guided imagery or autohypnosis. Nursing Safety Priority Action Alert Aspiration or biopsy procedures are invasive, and sterile technique must be observed. The skin over the site is cleaned. For an aspiration, the needle is inserted with a twisting motion, and the marrow is aspirated by pulling back on the plunger of the syringe. When sufficient marrow has been aspirated to ensure accurate analysis, the needle is withdrawn rapidly while the tissues are supported. For a biopsy, a small skin incision is made, and the biopsy needle is inserted. Pressure and several twisting motions are needed to ensure coring and loosening of an adequate amount of marrow tissue. Apply external pressure to the site until hemostasis is ensured. A pressure dressing or sandbags may be applied to reduce bleeding at the site. Follow-Up Care. The nursing priority after a bone marrow aspiration or biopsy is prevention of excessive bleeding. Cover the site with a dressing after bleeding is controlled, and closely observe it for 24 hours for signs of bleeding and infection. A mild analgesic (aspirin-free) may be given for discomfort, and ice packs can be placed over the site to limit bruising. If the patient goes home the same day as the procedure, instruct him or her to inspect the site every 2 hours for the first 24 hours to assess for active bleeding or bruising. Advise the patient to avoid any activity that might result in trauma to the site for 48 hours. Information obtained from bone marrow aspiration or biopsy reflects the degree and quality of bone marrow activity present. The counts made on a marrow specimen can indicate whether different cell types are present in the expected quantities and proportions. In addition, bone marrow aspiration or biopsy can confirm the spread of cancer cells from other tumor sites. Clinical Judgment Challenge 39-1 Ethics, Patient-Centered Care, Teamwork and Collaboration The patient is Joe, a 28-year-old man with Down syndrome who lives at home with his parents. His blood cell counts are all abnormal, and the next diagnostic test scheduled is a bone marrow aspiration. Joe can read at a fourth grade level and is very friendly; however, he is afraid of needles and had to be restrained during the venipuncture for blood testing. 1. From whom should informed consent be obtained for the procedure, Joe or his parents? 1589 2. Should anyone explain to Joe what the procedure entails? Why or why not? 3. Who is responsible for obtaining the informed consent? 4. If Joe says he does not want the test but his parents insist that he have it, what if any, ethical principles may be violated? (If necessary, review the ethical principles in Chapter 1.) 5. What members of the interprofessional team could provide guidance in this situation? 1590 Get Ready for the NCLEX® Examination! Key Points Review the following Key Points for each NCLEX Examination Client Needs Category. Safe and Effective Care Environment Verify that a patient having a bone marrow aspiration or biopsy has signed an informed consent statement. QSEN: Safety Handle patients with suspected hematologic problems gently to avoid bleeding or bruising. QSEN: Safety Do not palpate the splenic area of any patient suspected of having a hematologic problem. QSEN: Safety Maintain pressure over a venipuncture site for at least 5 minutes to prevent excessive bleeding. QSEN: Safety Health Promotion and Maintenance Teach adults to avoid unnecessary contact with environmental chemicals or toxins. If contact cannot be avoided, teach them to use safety precautions. Instruct patients about the importance of eating a diet with adequate amounts of foods that are good sources of iron, folic acid, and vitamin B12. QSEN: Patient-Centered Care Psychosocial Integrity Support the patient during a bone marrow aspiration or biopsy. QSEN: Patient-Centered Care Physiological Integrity Interpret blood cell counts and clotting tests to assess hematologic status. QSEN: Evidence-Based Practice Be aware that: Tissue oxygenation and perfusion rely on normal hematologic function for oxygen delivery. The most common symptom of a hematologic problem is fatigue. A platelet plug and a fibrin clot are not the same. Both clotting forces and anticlotting forces are needed to maintain adequate perfusion. Use the lip rather than nail beds to assess capillary refill on older adults. QSEN: Evidence-Based Practice Rely on laboratory tests rather than skin color changes in older adults to assess anemia or jaundice. QSEN: Evidence-Based Practice Assess the patient's endurance in performing ADLs. Teach patients and family members about what to expect during procedures to assess hematologic function, including restrictions, drugs, and follow-up care. QSEN: Patient-Centered Care Ask patients about their activity level and whether they are satisfied with the energy they have for activities. QSEN: Patient-Centered Care Apply an ice pack to the needle site after a bone marrow aspiration or biopsy. QSEN: Patient- Centered Care Check the needle insertion site at least every 2 hours after a bone marrow aspiration or biopsy. If the patient is going home, teach the patient and family how to assess the site for bleeding and when to seek help. QSEN: Patient-Centered Care 1591  Instruct patients to avoid activities that may traumatize the site after a bone marrow aspiration or biopsy. QSEN: Evidence-Based Practice 1592 Selected Bibliography Burchum J, Rosenthal L. Lehne's pharmacology for nursing care. 9th ed. Elsevier: St. Louis; 2016. Drug News. Edoxaban compares well to warfarin. Nursing 2014. 2014;44(2):10. Jarvis C. Physical examination & health assessment. 7th ed. Saunders: St. Louis; 2016. McCance K, Huether S, Brashers V, Rote N. Pathophysiology: The biologic basis for disease in adults and children. 7th ed. Mosby: St. Louis; 2014. O'Malley P. Waiting for the antidote. Clinical Nurse Specialist. 2015;29(5):262–264. Pagana K, Pagana TJ, Pagana TN. Mosby's diagnostic and laboratory test reference. 13th ed. Mosby: St. Louis; 2017. Pagana K, Pagana T, Pike-MacDonald S. Mosby's Canadian manual of diagnostic and laboratory tests. Mosby: St. Louis; 2013. Rauen C. Beyond the bloody mess: Hematologic assessment. Critical Care Nurse. 2012;32(5):42– 46. Sendir M, Buyukylmaz F, Celik Z, Taskopru I. Comparison of 3 methods to prevent pain and bruising after subcutaneous heparin administration. Clinical Nurse Specialist. 2015;29(3):174– 180. Siegal D, Curnutte J, Connolly S, Lu G, Conley P, Wiens B, et al. Adexanel alfa for reversal of factor Xa inhibitor activity. New England Journal of Medicine. 2015;373(25):2413–2414. Straznitskas A, Giarratano M. Emergent reversal of oral anticoagulation: Review of current treatment strategies. AACN Advanced Clinical Care. 2014;25(1):5–12. Touhy T, Jett K. Ebersole and Hess' toward healthy aging. 9th ed. Mosby: St. Louis; 2016. 1593 CHAPT ER 40 1594 Care of Patients With Hematologic Problems Katherine L Byar PRIORITY AND INTERRELATED CONCEPTS The priority concepts for this chapter are: PERFUSION IMMUNITY The PERFUSION concept exemplar for this chapter is Sickle Cell Disease, below. The IMMUNITY concept exemplar for this chapter is Leukemia, p. 817. The interrelated concepts for this chapter are: CELLULAR REGULATION GAS EXCHANGE CLOTTING LEARNING OUTCOMES Safe and Effective Care Environment 1. Collaborate with the interprofessional team to coordinate high-quality care to patients who have a hematologic problem affecting CLOTTING, IMMUNITY, PERFUSION, or GAS EXCHANGE. 2. Teach the patient and caregiver(s) about home safety related to impaired IMMUNITY, impaired CLOTTING, and other changes caused by hematologic problems or their management. Health Promotion and Maintenance 3. Identify community resources for patients with a chronic hematologic problem. 4. Teach adults undergoing therapy for a hematologic problem how to reduce the risk for infection and bleeding related to impaired IMMUNITY and impaired CLOTTING. Psychosocial Integrity 5. Implement nursing interventions to help the patient and family cope with the psychosocial impact caused by chronic or life-threatening hematologic problems and their therapies. Physiological Integrity 6. Apply knowledge of pathophysiology to assess patients with common complications caused by hematologic problems and their therapies. 7. Teach the patient and caregiver(s) about common drugs used as therapy for hematologic problems and their complications, including pain, impaired IMMUNITY, and impaired CLOTTING. 8. Prioritize nursing responsibilities during transfusion therapy. The hematologic system is responsible for the production and function of blood cells, which are critical for PERFUSION, IMMUNITY, CLOTTING, and GAS EXCHANGE. These vital activities can be impaired by any hematologic problem that interferes with the production, function, and maintenance of 1595 blood cells. The type and severity of the problem determine the impact on health. This chapter discusses mild hematologic disorders and those that are potentially life threatening, such as sickle cell disease and hematologic malignancies. 1596  Perfusion Concept Exemplar Sickle Cell Disease Pathophysiology Sickle cell disease (SCD), formerly called sickle cell anemia, is one of several related genetic hemoglobin disorders that result in chronic anemia, pain, disability, organ damage, increased risk for infection, and early death as a result of poor blood perfusion. Other similar disorders in this category include hemoglobin C disease and the thalassemias. SCD overall is more severe than the other hemoglobin disorders, although there is great variation in disease severity and when complications start. Perfusion is adequate arterial blood flow through the tissues (peripheral perfusion) and blood that is pumped by the heart (central perfusion) to oxygenate body tissues. Chapter 2 provides a summary discussion of issues about the concept. SCD results in the formation of abnormal hemoglobin chains. In healthy adults, the normal hemoglobin (hemoglobin A [HbA]) molecule has two alpha chains and two beta chains of amino acids. Normal adult red blood cells usually contain 98% to 99% HbA, with a small percentage of a fetal form of hemoglobin (HbF). In SCD, at least 40% (and often much more) of the total hemoglobin is composed of an abnormal beta chain (hemoglobin S [(HbS]). HbS is sensitive to low oxygen content of the red blood cells (RBCs). When RBCs with large amounts of HbS are exposed to decreased oxygen conditions, the abnormal beta chains contract and pile together within the cell, distorting the cell into a sickle shape. Sickled cells become rigid and clump together, causing the RBCs to become “sticky” and fragile. The clumped masses of sickled RBCs block blood flow and PERFUSION (Fig. 40-1), known as a vaso-occlusive event (VOE). VOE leads to further tissue hypoxia (reduced oxygen supply) and more sickle-shaped cells, which then leads to more blood vessel obstruction, inadequate perfusion, and ischemia in the affected tissues. Conditions that cause sickling include hypoxia, dehydration, infection, venous stasis, pregnancy, alcohol consumption, high altitudes, low or high environmental or body temperatures, acidosis, strenuous exercise, emotional stress, and anesthesia. FIG. 40-1 Red blood cell actions under conditions of low tissue oxygenation. (HbA, Hemoglobin A; HbS, hemoglobin S.) Usually sickled cells go back to normal shape when the precipitating condition is removed and the blood oxygen level is normalized, which allows tissue PERFUSION to resume. Although the cells then appear normal, some of the hemoglobin remains twisted, decreasing cell flexibility. The cell membranes are damaged over time, and cells are permanently sickled. The membranes of cells with HbS are more fragile and more easily broken. The average life span of an RBC containing 40% or more of HbS is about 10 to 20 days, much less than the 120-day life span of normal RBCs (McCance et al., 2014). This reduced RBC life span causes hemolytic (blood cell–destroying) anemia in patients with SCD. The patient with SCD has periodic episodes of extensive cellular sickling, called crises. The crises have a sudden onset and can occur as often as weekly or as seldom as once a year. Many patients are in good health much of the time, with crises occurring only in response to conditions that cause local or systemic hypoxemia (deficient oxygen in the blood). 1597  Repeated VOEs and impaired PERFUSION in large blood vessels cause long-term damage to tissues and organs. Most damage results from tissue hypoxia, anoxia, ischemia, and cell death. Organs develop small infarcted areas and scar tissue formation, and eventually organ failure results. The spleen, liver, heart, kidney, brain, joints, bones, and retina are affected most often. Etiology and Genetic Risk Sickle cell disease (SCD) is a genetic disorder with an autosomal-recessive pattern of inheritance (see Chapter 5). A specific mutation in the hemoglobin gene alleles on chromosome 11 leads to the formation of HbS instead of HbA. In SCD, the patient has two HbS gene alleles, one inherited from each parent, usually resulting in 80% to 100% of the hemoglobin being HbS. Because both hemoglobin alleles are S, SCD is sometimes abbreviated “SS.” Patients with SCD often have severe symptoms and greatly impaired PERFUSION, even when triggering conditions are mild. If a patient with SCD has children, each child will inherit one of the two abnormal gene alleles and at least have sickle cell trait. Sickle cell trait occurs when one normal gene allele and one abnormal gene allele for hemoglobin are inherited and only about half of the hemoglobin chains are abnormal. Sickle cell trait is abbreviated “AS.” The patient is a carrier of the HbS gene allele (Fig. 40-2) and can pass the trait on to his or her children. However, the patient has only mild symptoms of the disease when precipitating conditions are present because less hemoglobin is abnormal. FIG. 40-2 Possible transmission of sickle cell disease and sickle cell trait when both parents are carriers. (HbA, Hemoglobin A; HbS, hemoglobin S.) Incidence and Prevalence Sickle cell trait and different forms of SCD occur in people of all races and ethnicities but is most common among African Americans in the United States. About 90,000 to 100,000 people have SCD, occurring in 1 in 500 African Americans. About 1 in 12 to 1 in 15 (8%) African Americans are carriers of one sickle cell gene allele and have AS (Centers for Disease Control and Prevention [CDC], 2015). Interprofessional Collaborative Care Sickle cell disease (SCD) is a chronic disease that reduces PERFUSION. Patients must self-manage continually at home or other residential settings. When crises or other acute complications occur, patients are cared for in an acute care. Assessment: Noticing History. 1598 Those with sickle cell trait usually have no symptoms or abnormal laboratory findings other than the presence of hemoglobin S. Patients with sickle cell trait may be unaware that they have a hematologic problem until an acute illness is present or when anesthesia is administered. Ask about previous crises, what led to the crises, severity, and usual management. Explore recent contact with ill people and activities to determine what caused the current crisis. Ask about signs and symptoms of infection. Review all activities and events during the past 24 hours, including food and fluid intake, exposure to temperature extremes, drugs taken, exercise, trauma, stress, recent airplane travel, and ingestion of alcohol or other recreational drugs. Ask about changes in sleep and rest patterns, ability to climb stairs, and any activity that induces shortness of breath. Determine the patient's perceived energy level using a scale ranging from 0 to 10 (0 = not tired with plenty of energy; 10 = total exhaustion) to assess the degree of fatigue. Physical Assessment/Signs and Symptoms. Pain is the most common symptom of SCD crisis (Matthie & Jenerette, 2015). Others vary with the site of reduced PERFUSION and the tissue damaged. Cardiovascular changes, including the risk for high-output heart failure, occur because of the anemia. Assess the patient for shortness of breath and general fatigue or weakness. Other problems may include murmurs, the presence of an S3 heart sound, and increased jugular-venous pulsation or distention. Assess the cardiovascular status by comparing peripheral pulses, temperature, and capillary refill in all extremities. Extremities distal to blood vessel occlusion are cool to the touch with slow capillary refill and may have reduced or absent pulses, which indicate reduced PERFUSION. Heart rate may be rapid, and blood pressure may be low to normal with anemia. Respiratory system changes occur over time. Pulmonary hypertension, recurrent pneumonia. Further assessment with pulmonary function testing. Acute chest syndrome is a common reason for hospitalization and is the most common cause of death (USDHHS, 2014). This life-threatening condition is usually associated with respiratory infection and can also be caused by fat embolism and pulmonary debris from sickled cells. Symptoms are similar to pneumonia with cough, shortness of breath, abnormal breath sounds, and an infiltrate on chest x-ray. Fever may or may not be present. Without intervention, this complication can lead to respiratory failure and failure of all other organ systems. Priapism is a prolonged penile erection that can occur in men who have SCD. The cause is excessive vascular engorgement in erectile tissue. The condition is very painful and can last for hours. During the priapism episode, the patient usually cannot urinate. Skin changes include pallor or cyanosis because of poor GAS EXCHANGE from decreased PERFUSION and anemia. Examine the lips, tongue, nail beds, conjunctivae, palms, and soles of the feet at least every 8 hours for subtle color changes. With cyanosis, the lips and tongue are gray; and the palms, soles, conjunctivae, and nail beds have a bluish tinge. Another skin sign of SCD is jaundice. Jaundice results from RBC destruction and release of bilirubin. To assess for jaundice in patients with darker skin, inspect the roof of the mouth for a yellow appearance. Examine the sclera closest to the cornea to assess jaundice more accurately. Jaundice often causes intense itching. Many adults with SCD have ulcers on the lower legs that are caused by poor PERFUSION, especially on the outer sides and inner aspect of the ankle or the shin. These lesions often become necrotic or infected, requiring débridement and antibiotic therapy. Inspect the legs and feet for ulcers or darkened areas that may indicate necrotic tissue. Abdominal changes include damage to the spleen and liver, which often occurs early from many episodes of hypoxia and ischemia. In crisis, abdominal pain from reduced PERFUSION is diffuse and steady, also involving the back and legs. The liver or spleen may feel firm and enlarged with a nodular or “lumpy” texture in later stages of the disease. Kidney and urinary changes are common as a result of poor PERFUSION and decreased tissue GAS EXCHANGE. Chronic kidney disease occurs as a result of anoxic damage to the kidney nephrons (USDHHS, 2014). Early damage makes the kidneys less effective at filtration and reabsorption. The 1599 urine contains protein, and the patient may not concentrate urine. Eventually the kidneys fail, resulting in little or no urine output. Musculoskeletal changes occur because arms and legs are often sites of blood vessel occlusion. Joints may be damaged from hypoxic episodes and have necrotic degeneration. Inspect the arms and legs and record any areas of swelling, temperature, or color difference. Ask patients to move all joints. Record the range of motion and any pain with movement. Central nervous system (CNS) changes may occur in SCD. During crises, patients may have a low- grade fever. Long-term effects of reduced PERFUSION to the CNS may result in infarcts with repeated episodes of hypoxia, causing the patient to have seizures or symptoms of a stroke (USDHHS, 2014). Assess for the presence of “pronator drift,” bilateral hand grasp strength, gait, and coordination. Psychosocial Assessment. Often cognitive and behavioral changes are early indications of cerebral hypoxia from poor PERFUSION. Assess the patient and document mental status examination results. Ask family members whether the current behavior and mental status are usual for the patient. Assess the patient and family for knowledge and understanding of SCD and how to live with the disease to the highest level of wellness possible. SCD is a painful, life-limiting disorder that can be passed on to one's children. When assessing psychosocial needs, keep in mind new factors that might contribute to a crisis. Also assess established support systems, use of coping patterns, disease progression, and the impact that all of these have on the patient and family. Laboratory Assessment. The diagnosis of SCD is based on the percentage of hemoglobin S (HbS) on electrophoresis. A person who has AS usually has less than 40% HbS, and the patient with SCD may have 80% to 100% HbS. This percentage does not change during crises. Another indicator of SCD is the number of RBCs with permanent sickling. This value is less than 1% among people with no hemoglobin disease, 5% to 50% among people with AS, and up to 90% among patients with SCD. Other laboratory tests can indicate complications of the disease, especially during crises. The hematocrit of patients with SCD is low (between 20% and 30% [0.2 and 0.3 volume fraction]) because of RBC shortened life span and destruction. This value decreases even more during crises or stress (aplastic crisis). The reticulocyte count is high, indicating anemia of long duration. The total bilirubin level may be high because damaged RBCs release iron and bilirubin. The total white blood cell (WBC) count is usually high in patients with SCD. This elevation is related to chronic inflammation caused by tissue hypoxia and ischemia. Imaging Assessment. Bone changes occur as a result of chronically stimulated marrow and low bone oxygen levels. The skull may show changes on x-ray. Ultrasonography, CT, positron emission tomography (PET), and MRI may show soft- tissue and organ changes from poor PERFUSION and chronic inflammation. Other Diagnostic Assessment. ECG changes document cardiac infarcts and tissue damage. Echocardiograms may show cardiomyopathy and decreased cardiac output (low ejection fraction). Analysis: Interpreting The priority collaborative problems for the patient with sickle cell disease include: 1. Pain due to poor tissue oxygenation and joint destruction 2. Potential for infection, sepsis, multiple organ dysfunction, and death 1600  Planning and Implementation: Responding Managing Pain Planning: Expected Outcomes. The pain associated with sickle cell disease may be acute during crises and chronic as a result of complications (Matthie & Jenerette, 2015). Acute pain episodes have a sudden onset, usually involving the chest, back, abdomen, and extremities. Complications of SCD can cause severe, chronic pain, requiring large doses of opioid analgesics. Regardless of the pain type, expected outcomes include that the patient's pain is controlled to a level acceptable to him or her (e.g., a 3 or less on a pain intensity rating scale of 0 to 10) and can participate in self-care or other activities to the degree he or she wishes (Lentz & Krautz, 2017). Interventions. The pain with sickle cell crisis is the result of tissue injury caused by poor PERFUSION and tissue GAS EXCHANGE from obstructed blood flow. Mild pain can be managed at home. However, pain is often severe enough to require hospitalization and opioid analgesics. Ask whether the pain is typical of past pain episodes. If not, other pain causes or disease complications must be explored. Ask the patient to rate pain on a scale ranging from 0 to 10 and evaluate the effectiveness of interventions based on the ratings. Concerns about substance abuse can lead to inadequate pain treatment in these patients. Opioid addiction is rare in patients with SCD (Matthie & Jenerette, 2015). Because the pain of crisis has no objective signs, pain management is based on past pain history, previous drug use, disease complications, and current pain assessment. Many patients have had negative interactions with nurses and other members of the interprofessional team who suggest that the pain is not a problem and that patients with SCD may be “drug seekers” (O'Connor et al., 2014). Health care professionals need to be aware of their own attitudes when caring for this population. If substance abuse occurs, management of addiction is incorporated into the overall treatment plan. Addicted patients in acute pain crisis still need opioids. Drug therapy for patients in acute sickle cell crisis often starts with at least 48 hours of IV analgesics. (Chart 40-1 lists best practices for nursing care of the patient in sickle cell crisis.) Morphine and hydromorphone (Dilaudid) are given IV on a routine schedule or by infusion pump using patient-controlled analgesia (PCA). Once relief is obtained, the IV dose can be tapered and the drug given orally. Avoid “as needed” (PRN) schedules because they do not provide adequate relief. Moderate pain may be managed with oral doses of opioids or NSAIDs. Chart 40-1 Best Practice for Patient Safety & Quality Care Care of the Patient in Sickle Cell Crisis Administer oxygen. Administer prescribed pain medication. Hydrate the patient with normal saline IV and with beverages of choice (without caffeine) orally. Remove any constrictive clothing. Encourage the patient to keep extremities extended to promote venous return. Do not raise the knee position of the bed. Elevate the head of the bed no more than 30 degrees. Keep room temperature at or above 72° F (22.2° C). 1601  Avoid taking blood pressure with external cuff. Check circulation in extremities every hour: Pulse oximetry of fingers and toes Capillary refill Peripheral pulses Toe temperature Hydroxyurea (Droxia) may reduce the number of sickling and pain episodes by stimulating fetal hemoglobin (HbF) production. Increasing the level of HbF reduces sickling of red blood cells in some but not all patients with sickle cell disease (Vacce & Blank, 2017). However, this drug increases the risk for leukemia. Long-term complications should be discussed with the patient before this therapy is started. Hydroxyurea also suppresses bone marrow function, including IMMUNITY, and regular follow-up to monitor complete blood counts (CBCs) for drug toxicity is important. Nursing Safety Priority Action Alert Hydroxyurea is teratogenic (can cause birth defects). Teach sexually active women of childbearing age using hydroxyurea to adhere to strict contraceptive measures while taking it and for 1 month after it is discontinued. Hydration by the oral or IV route helps reduce the duration of pain episodes. Urge the patient to drink water or juices. Because the patient is often dehydrated and his or her blood is hypertonic, hypotonic fluids are usually infused at 250 mL/hr for 4 hours. Once the patient's blood osmolarity is reduced to the normal range of 270 to 300 mOsm, the IV rate is reduced to 125 mL/hr if more hydration is needed. Complementary and integrative therapies and other measures, such as keeping the room warm, using distraction and relaxation techniques, positioning with support for painful areas, aroma therapy, therapeutic touch, and warm soaks or compresses, all help reduce pain perception. NCLEX Examination Challenge 40-1 Physiological Integrity Which change in laboratory test results of a client with sickle cell disease who was started on therapy with hydroxyurea 4 weeks ago indicates to the nurse that the therapy is effective? A. Increased HbF from 2% to 10% B. Decreased HbA from 3% to 2.5% C. Increased platelets from 250,000/mm3 (250 × 109/L) to 300,000/mm3 (300 × 109/L) D. Decreased white blood cells from 8200/mm3 (8.2 × 109/L) to 7700/mm3 (7 × 109/L) 1602  Preventing Sepsis, Multiple Organ Dysfunction Syndrome, and Death Planning: Expected Outcomes. The patient with SCD is expected to remain free from infection and sepsis. Indicators include: Absence of fever and foul-smelling or purulent drainage Absence of cough, chest pain, and dyspnea Absence of pain, burning on urination Interventions. The patient with SCD is at greater risk for bacterial infection because of reduced IMMUNITY from anoxic damage to the spleen. Interventions focus on preventing infection, controlling infection, and starting drug therapy early when infection is present. The patient with a fever should have diagnostic testing for sepsis, including complete blood count (CBC) with differential, blood cultures, reticulocyte count, urine culture, and a chest x-ray. Usually these patients are started on prophylactic antibiotics. Prevention and early detection strategies : Frequent, thorough handwashing is of the utmost importance. Wear a mask. Strict aseptic technique is used for all invasive procedures. Continually assess the patient for infection and monitor the daily CBC with differential WBC count. Inspect the mouth every 8 hours for lesions indicating fungal or viral infection. Listen to the lungs every 8 hours for crackles, wheezes, or reduced breath sounds. Inspect voided urine for odor and cloudiness and ask about urgency, burning, or pain on urination. Take vital signs at least every 4 hours to assess for fever or supervise this action when performed by others. Drug therapy by prophylaxis with twice-daily oral penicillin reduces the number of pneumonia and other streptococcal infections. Urge the patient to receive a pneumonia vaccination and annual influenza vaccinations. Drug therapy for an actual infection depends on the sensitivity of the specific organism and the extent of the infection. Continued blood vessel occlusion by clumping of sickled cells increases the risk for multiple organ dysfunction. Acute chest syndrome, in which a vaso-occlusive event (VOE) causes infiltration and damage to the pulmonary system, is a major cause of death in adults with SCD. Thus preventing heart and lung damage is a priority. Management focuses on prevention of VOEs and promotion of PERFUSION. Assess the patient admitted in sickle cell crisis for adequate PERFUSION to all body areas. Remove restrictive clothing and instruct the patient to avoid flexing the knees and hips. Hydration is needed because dehydration increases cell sickling and must be avoided. Help the patient maintain adequate hydration. The patient in acute crisis needs an oral or IV fluid intake of at least 200 mL/hr. Oxygen is given during crises because lack of oxygen is the main cause of sickling. Ensure that oxygen therapy is nebulized to prevent dehydration. Monitor oxygen saturation. If saturation is low, evaluation of arterial blood gases (ABGs) and a chest x-ray may be needed. Transfusion with RBCs can be helpful to increase HbA levels and dilute HbS levels, although they must be prescribed cautiously to prevent iron overload from repeated transfusions (Martin & Haines, 2016). Transfusion therapy in some centers is a mainstay of SCD management to reduce the risk for stroke. Monitor the patient for transfusion complications (discussed in the Acute Transfusion Reactions section). Hematopoietic stem cell transplantation (HSCT) may correct abnormal hemoglobin permanently during childhood. Care Coordination and Transition Management Care focuses on teaching the patient and family how to prevent crises and complications (Chart 40- 2). The patient with SCD may receive care in acute care, subacute care, extended or assistive care, and home care settings. 1603  Chart 40-2 Patient and Family Education: Preparing for Self- Management Prevention of Sickle Cell Crisis Drink at least 3 to 4 liters of liquids every day. Avoid alcoholic beverages. Avoid smoking cigarettes or using tobacco in any form. Contact your primary health care provider at the first sign of illness or infection. Be sure to get a “flu shot” every year. Ask your primary health care provider about taking the pneumonia vaccine. Avoid temperature extremes of hot or cold. Be sure to wear socks and gloves when going outside on cold days. Avoid planes with unpressurized passenger cabins. Avoid travel to high altitudes (e.g., cities such as Denver and Santa Fe). Ensure that any health care professional who takes care of you knows that you have sickle cell disease, especially the anesthesia provider and radiologist. Consider genetic counseling. Avoid strenuous physical activities. Engage in mild, low-impact exercise at least 3 times a week when you are not in crisis. Self-Management Education. Having the patient and family be partners in the life-long management of SCD is critical for improved outcomes. Thus self-management education is extensive and should be reinforced at every health care encounter with a patient who has SCD. Both income level and education level have a positive correlation with the patient's ability to be successful in the performance of self-care (Matthie et al., 2015b). See the Evidence-Based Practice box for a discussion of the role of self-care in SCD management. Evidence-Based Practice Which Factors Positively Influence Self-Care in Young Adults With Sickle Cell Disease? Matthie, N., Jenerette, C., & McMillan, S. (2015). Role of self-care in sickle cell disease. Pain Management Nursing, 16(3), 257–266. Sickle cell disease (SCD) is a serious, lifelong condition with complications that impair physical function and shorten life span. Consistent practice of recommended self-care activities can result in fewer or less severe complications and a longer life span. Once considered a childhood problem, more patients with SCD are living to adulthood and transitioning from a pediatric hematologic or SCD management setting to adult hematologic management. As young adults, these patients are expected to assume more responsibility for self-care in the management of SCD. The study reported here, which was a descriptive cross-sectional design using secondary analysis of existing data, sought to identify the personal and sociodemographic factors that positively influenced self-care in an SCD population. Self-care was defined as “One's perceived ability to participate in general therapeutic activities aimed at improving health status and quality 1604 of life, as well as the actual performance of those activities.” Self-care actions included maintaining adequate hydration, avoiding temperature extremes, eating a healthy diet, obtaining regular checkups, and ensuring adequate rest. Data from 103 adults with SCD ranging in age from 18 to 30 years were examined for demographic information, SCD self-care efficacy, perceived social support, self-care, and the annual number of hospital visits for pain crises. Several factors demonstrated importance in promoting self-care management. Patients who had more education and more social support had overall higher scores for SCD self-efficacy, which included self-care management, perceived self-care ability, and participation in self-care actions. Social support in particular had the most significant effect on self-care. There was a negative association between income level and number of hospital visits for pain crises. The interpretation of this finding was that adult SCD patients with health insurance and/or higher incomes were able to access and obtain primary care, which reduce the number of pain crises experienced. Level of Evidence: 3 The study reanalyzed data from a much larger, multisite study and had adequate power to generate statistically significant results for the guiding research hypotheses. The instruments used to measure the concepts of SCD self-efficacy, perceived social support, perceived self-care ability, and self-care actions were all well established with appropriate validity and reliability. Commentary: Implications for Practice and Research The finding that social support was most positively associated with SCD self-care is consistent with the findings of other studies regarding self-care in chronic disease management. Nurses can have an impact in promoting social support by working to help SCD patients, family, and friends have an adequate understanding of the disease, complication-avoiding activities, and performance of self-care activities. Although the availability of such information and professional support is a critical component of pediatric SCD management centers, it may not be nearly as well developed in the adult hematologic care setting. This lack becomes more significant as the SCD population ages and more adults with the disease are managed in nonpediatric settings. The authors conclude that more knowledge on the part of students and practicing nurses in the adult setting about the care and education needs of SCD patients and families could have a positive effect on increasing social support. Teach the patient to avoid specific activities that lead to reduced GAS EXCHANGE from hypoxia and hypoxemia. Stress the recognition of the early symptoms of crisis or infection so interventions can be started early to prevent pain, complications, and permanent tissue damage. Teach the patient and family the correct use of opioid analgesics at home. Health Care Resources. Some adults are unfamiliar with the hereditary aspects of SCD. For those who need it, refer patients to genetic counselors. These professionals can provide information about birth control methods and pregnancy options. Many patients and family members can be helped by local support groups. Provide information about the closest local chapter of the Sickle Cell Foundation. Often local children's hospitals have sickle cell support groups that include adults with the disease. Gender Health Considerations Patient-Centered Care Pregnancy in women with SCD may be life threatening. Barrier methods of contraception (cervical cap, diaphragm, or condoms with or without spermicides) are often recommended for women with SCD who are sexually active. The use of combination hormone drugs for contraception may