Practice nursing care for Clients
with Red Blood Cells Disorders
Disorders of the hematologic system can occur as a result of problems in the production, function, or normal destruction of any type of blood cell. The type and severity of the specific disorder determine the degree of threat to the client’s wellbeing. This chapter discusses hematologic conditions that may have only a minor impact on activities of daily living (ADLs), as well as those disorders that are potentially life threatening, such as sickle cell disease and leukemia.
RED BLOOD CELL DISORDERS
The major cellular population of the blood consists of red
blood cells (RBCs), or erythrocytes. Adequate tissue oxygenation depends on maintaining the circulating number of RBCs within the normal range for the person’s age and gender and ensuring that the cells can perform their normal functions. RBC disorders include problems in production, function, and destruction. These problems may result in an insufficient number or insufficient function of RBCs (anemia) or an excess of RBCs(polycythemia).
Anemia is a reduction in either the number of RBCs, the quantity of hemoglobin, or the hematocrit (percentage of packed RBCs per deciliter of blood). Anemia is a clinical sign, not a diagnosis, because it is a manifestation of a number of abnormal conditions. Anemia can result from dietary deficiencies, hereditary disorders, bone marrow disease, or bleeding. There are many types and causes of anemia. Some anemias are caused by a deficiency in one or more of the components needed to make fully functional RBCs. Such anemias can be caused by deficiencies of iron, vitamin B12, folic acid, or intrinsic factor. Additional causes include a decreased rate of erythrocyte production and increased destruction of RBCs. Table 40-1 lists common causes of various types of anemia.
Despite the many causes of anemia, the effects of anemia on the client (Chart 40-1) and the corresponding nursing care are similar for all types of anemia.
ANEMIA
In the human body, healthy red blood cells (RBCs) carry oxygen to tissues. A balance is maintained between the production of new RBCs and the disposal of old “worn-out” RBCs. Anemia occurs when something interferes with this balance or interferes with the maturation of cells.
Anemia is a state in which there are insufficient numbers of functioning RBCs, or a lack of hemoglobin, to meet the demands of the tissues for oxygen.
Etiology
There are three major classifications of anemia, according to cause:
• Anemia resulting from blood loss
• Anemia resulting from a failure in blood cell production
• Anemia associated with an excessive destruction of red cells
Rapid, severe bleeding leads to anemia from blood loss, hypovolemia (decreased volume of circulating blood), and, potentially, shock. A blood loss that leads to anemia may result from severe trauma to the blood vessels and massive hemorrhage or the blood loss may be more gradual, as from a small, bleeding peptic ulcer that causes a chronic blood loss.
The amount of blood loss that leads to hypovolemic shock varies, depending on the ability of the patient’s body to compensate for the lost fluid volume. A blood loss of even 500 mL in an adult who had normal circulating volume may cause hypovolemic shock
Table 17-1 shows the amount of blood loss and consequent clinical manifestations.
Anemia caused by a failure in cell production is the result of either a deficiency of certain substances necessary for the formation of RBCs, or results from the abnormal function of bone marrow. Examples of this type of anemia are:
• Nutritional anemia, in which there is an inadequate intake of foods containing proteins, folic acid, and iron
• Anemia resulting from bone marrow suppression caused by toxic substances
• Pernicious anemia, in which there is faulty absorption of specific nutrients, such as vitamin B12 Iron or folic acid may not be well absorbed in people who have an intestinal malabsorption syndrome.
Hemolytic anemias, in which red cells are destroyed prematurely in the body, have many causes. Hemolytic anemia can be a result of genetic defects that affect cell structure, causing the cells to disintegrate quickly. Some of the hemolytic anemias, such as thalassemia, are inherited, whereas others are acquired when erythrocytes are exposed to poisonous agents, such as chemicals or certain bacterial toxins.
Immune reactions can cause blood cell hemolysis (destruction of red cells). The presence of toxins in the blood, infections such as malaria, transfusion reactions, and changes in blood chemistry may cause red cell hemolysis. Blood incompatibility in the newborn (erythroblastosis fetalis) is another cause.
There is about a 20% incidence of anemia among the elderly, most often because of poor nutrition. Shock may develop with smaller blood loss in this group because of decreased vascular tone and impaired cardiac function.
Pathophysiology
Iron deficiency anemia occurs when total body iron is insufficient and erythropoiesis is diminished. The lack of iron impedes the formation of hemoglobin (Hb) (Concept Map 17-1). In pernicious anemia, an autoimmune disease, the intrinsic factor is missing from the gastric juices, and vitamin B12 is not absorbed without it. Vitamin B12 acts as a coenzyme in conjunction with folate metabolism and is important in the utilization of iron and protein for the manufacture of RBCs. The result of the missing intrinsic factor is that the red cell production is decreased, and those red cells that are produced are abnormal in their structure and function. To correct this condition, the physician will order the administration of vitamin B12. A folic acid deficiency also contributes to anemia (Gentili et al., 2009).
Hemolytic anemias associated with excessive destruction of RBCs are quite rare. When red cells are not normal, they break up easily or are destroyed by the body more quickly than are normal red cells.
This RBC destruction causes the anemia. Anemia occurs in end-stage renal disease patients when there is a deficiency of production of erythropoietin, a substance necessary to stimulate the production of RBCs in the bone marrow. This problem is usually corrected by the administration of epoetin alfa (Epogen), which stimulates red cell production (Singh, 2006). Oxygen transport depends on the number and condition of the red cells and the amount of hemoglobin they contain.
ANEMIAS RESULTING FROM INCREASED DESTRUCTION OF RED BLOOD CELLS
Sickle Cell Disease
OVERVIEW
Sickle cell disease is a condition in which chronic anemia is one of many problems causing pain, disability, increased risk for disease, and early death. Once considered a childhood disorder, clients with sickle cell disease who receive appropriate supportive care may live into their 30s and 40s. In addition, there is great variation among clients in the severity of the disease and the onset of complications.
Pathophysiology
The primary problem in this hereditary disorder is the formation of abnormal beta chains in the hemoglobin molecule. The hemoglobin molecule of adults is composed partially of the globin protein, consisting of two alpha chains and two beta chains of amino acids. This normal adult hemoglobin is called hemoglobin A (HbA).
The total hemoglobin of normal healthy adults is usually 98% to 99% HbA, with a small percentage of a fetal form of hemoglobin (HbF). In sickle cell disease, at least 40% of the total hemoglobin contains an abnormality of the beta chains, known as hemoglobin S (HbS). HbS is sensitive to changes in the oxygen content of the RBC. When RBCs containing large amounts of HbS are exposed to conditions of decreased oxygen, the abnormal beta chains contract and pile together within the cell, distorting the overall shape of the RBC. These cells assume a sickle shape, become rigid, clump together, and form clusters that block capillary blood flow (Figure 40-1).
Capillary obstruction leads to further tissue hypoxia (reduced oxygen supply) and more sickling, causing blood vessel obstructions and infarctions in the locally affected tissues.
Sickling of red cells occurs when tissue oxygen is low.
Situations that lead to sickling include hypoxia, dehydration, infections, vascular stasis, low environmental or body temperatures, acidosis, strenuous exercise, and anesthesia. Usually, sickled cells resume a normal shape when the precipitating condition is removed and proper oxygenation occurs. However, although the outward appearance of the RBCs is normal, at least some of the hemoglobin remains twisted, decreasing cell flexibility. The membranes of the cells become damaged over time, and cells become irreversibly sickled. In addition, the membranes of cells with HbS are more fragile and more easily destroyed in the spleen and in other organs that have long, twisted capillary pathways. The average life span of an RBC containing 40% or more of HbS is approximately 20 days, considerably less than the 120-day life span of RBCs containing only HbA.
This reduced life span is responsible for hemolytic (blood cell-destroying) anemia in clients with sickle cell disease. The client with sickle cell disease experiences periodic episodes of extensive cellular sickling, or crises. The crises have a sudden onset and can occur as frequently as weekly or as seldom as once a year. Many clients are in good health much of the time, with crises occurring sporadically in response to precipitating conditions that stimulate local or systemic hypoxemia (deficient oxygen in the blood). Repeated occlusions of progressively larger blood vessels have long-term negative effects on tissues and organs (Chart 40-2).
Most effects are thought to occur as a result of capillary and blood vessel occlusion leading to tissue hypoxia, anoxia, ischemia, and cell death. Tissues and organs begin to have small infarcted areas that eventually destroy all healthy cells and lead to organ failure. Tissues and organs most commonly affected in this way are the spleen, liver, heart, kidney, brain, bones, and retina.
Etiology
Sickle cell disease is a genetic disorder with an autosomal recessive pattern of inheritance. The formation of the beta chains of the hemoglobin molecule is dependent on a pair of genes. A mutation in these genes leads to the formation of HbS instead of HbA.
When the client inherits one abnormal gene of this pair, the condition is called sickle cell trait. The client can pass the condition on to his or her children, but the client has only mild manifestations of the disease under severe precipitating conditions because less than about 30% of the hemoglobin is abnormal.
When the client inherits two abnormal genes, the condition is called sickle cell disease (formerly sickle cell anemia), and the client has severe manifestations of the disease even under relatively mild precipitating conditions. In addition, if the client has children, each child will inherit one of the two abnormal genes and at least have sickle cell trait.
Incidence/Prevalence
Sickle cell trait and different forms of sickle cell disease occur in people of all races and ethnicities but less often among Caucasians.
COLLABORATIVE MANAGEMENT
Assessment
HISTORY
An adult with sickle cell disease has a long-standing diagnosis of the disorder. An adult who has sickle cell trait, however, may have had such mild clinical manifestations that he or she is unaware of the problem until it is diagnosed with an accompanying disorder or when anesthesia is administered. The nurse asks the client about previous crises, including precipitating events, severity, and usual treatments. Recent activities and situations are explored to determine the probable precipitating condition or event. The nurse also reviews all activities and events during the previous 24 hours, including food and fluid intake, exposure to temperature extremes, types of clothing worn, medications taken, exercise, trauma, stress, and ingestion of alcohol or other recreational drugs. This activity review provides important information about fatigue, activity tolerance, and participation in activities of daily living (ADLs). The client is asked about changes in sleep and rest patterns, ability to climb stairs, and any activity that induces shortness of breath. Obtaining a subjective baseline assessment of the client’s perceived energy level using a scale ranging from 0 to 10 (0 = not tired with plenty of energy; 10 = total exhaustion) can be useful in evaluating the degree of fatigue and the effectiveness of later treatments.
PHYSICAL ASSESSMENT/CLINICAL MANIFESTATIONS
Pain is the most common symptom experienced during sickle cell crisis. Jaundice may also be present as a result of increased red blood cell (RBC) destruction and release of bilirubin. Other clinical manifestations vary with the site of tissue damage.
CARDIOVASCULAR ASSESSMENT.
The nurse assesses the client’s cardiovascular and peripheral vascular 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 diminished or absent pulses. The heart rate may be rapid and the blood pressure low to average, with a decreased pulse pressure because breakage of RBCs leads to anemia.
INTEGUMENTARY ASSESSMENT.
The skin may be pale or cyanotic as a result of decreased perfusion and anemia. The nurse examines the lips, tongue, nail beds, conjunctivae, palms, and soles at regular intervals 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 manifestation associated with sickle cell disease is jaundice. Bilirubin, a major component of RBCs, is released when fragile cells are damaged, leading to jaundice.
The nurse assesses for jaundice in clients with darker skin by inspecting the oral mucosa, especially the hard palate, for yellow discoloration. Inspection of the conjunctivae and adjacent sclera may be misleading because of normal deposits of subconjunctival fat that produce a yellowish hue when seen in contrast to the dark periorbital skin. Therefore the nurse examines the sclera closest to the cornea to diagnose jaundice more accurately. Similarly, the palms and soles of darkskinned clients may appear yellow if callused and should not be mistaken for jaundice. Jaundice from excessive bilirubin may also cause intense itching. In spite of the anemia, clients with sickle cell disease usually are not deficient in iron. In fact, with increased RBC production and destruction, iron released from the cells may increase the pigmentation of the skin. As many as 75% of adult clients with sickle cell disease have stasis ulcers or pressure ulcers on the lower extremities. The ulcers usually occur on the lower portion of the legs (Rausch & Pollard, 1998). The outer sides and inner aspect of the ankle or the shin are common sites. The nurse inspects the legs and feet for open lesions or darkened areas that may indicate necrotic tissue. Infections of these lesions occur frequently.
ABDOMINAL ASSESSMENT.
Abdominal organs are usually the first to be damaged as a result of multiple episodes of hypoxia and ischemia. The nurse inspects the abdomen for asymmetry or bulging areas, gently palpating it. Affected organs, such as the liver or spleen, may be firm and enlarged with a nodular texture in later stages of the disease.
MUSCULOSKELETAL ASSESSMENT.
Extremities are a common site of vascular occlusion among clients who have sickle cell disease. In addition, joints may be damaged from frequent hypoxic episodes and undergo necrotic degeneration. The nurse inspects the extremities for symmetry and records any areas of swelling or color difference. Clients are asked to move all joints, and the nurse notes the range of motion and any accompanying pain.
CENTRAL NERVOUS SYSTEM ASSESSMENT.
Changes in central nervous system (CNS) function may occur directly or indirectly in sickle cell disease. During crises, clients may have a low-grade fever. If the CNS sustains infarcts or repeated episodes of hypoxia, they may have seizure activity or clinical manifestations of a stroke. Hand grasps are assessed bilaterally. The nurse assesses gait and coordination in those clients who are permitted to walk.
PSYCHOSOCIAL ASSESSMENT
Psychosocial assessment is important because behavioral changes may be the first observable clinical manifestations of cerebral hypoxia. The nurse observes the client and documents presenting behavior. Family members or significant others are questioned to determine whether the presenting behavior and mental status are typical. Sickle cell disease represents a chronic, painful, life-limiting disorder that can be passed on to one’s children. The nurse assesses the client’s psychosocial needs in terms of new factors, established support systems, previous and current coping patterns, and disease progression. The client is asked how he or she views the disease and what adjustments in lifestyle have been made to accommodate limitations.
LABORATORY ASSESSMENT
The primary laboratory finding associated with sickle cell disease is the large percentage of hemoglobin S (HbS) present on electrophoresis. A person who has sickle cell trait usually expresses less than 40% HbS, and the client with sickle cell disease may express 85% to 95% HbS. This percentage does not change during crises. Another indicator of sickle cell disease is the percentage of RBCs showing irreversible sickling. This value is less than 1% among people who do not have sickle cell disease, is 5% to 50% among people with sickle cell trait, and may exceed 90% among clients with sickle cell disease.
A variety of other laboratory tests reflect the problems associated with sickle cell disease, especially during crises. The hematocrit of clients with sickle cell disease is usually low (between 20% and 30%). This value decreases even more dramatically during vascular occlusive crises, or aplastic crises, when the bone marrow temporarily fails to produce cells during stressful periods (such as infection). The reticulocyte count is elevated, indicating anemia of long duration. Often the mean corpuscular hemoglobin concentration (MCHC) and total bilirubin level are elevated in the client who has sickle cell disease. The total white blood cell (WBC) count is usually above normal among clients who have sickle cell disease. It is thought that this elevation is related to chronic inflammation resulting from tissue hypoxia and ischemia.
RADIOGRAPHIC ASSESSMENT
Bone changes occur as a result of chronically stimulated marrow and hypoxic bone tissue. The skull may show radiographic changes resulting from chronic bone surface resorption and regeneration, giving the skull a “crew cut” appearance. Joint necrosis and degeneration also are obvious on x-ray examination.
OTHER DIAGNOSTIC ASSESSMENT
Electrocardiographic (ECG) changes document cardiac infarcts and tissue damage. Specific ECG changes are related to the area of the myocardium sustaining the damage. Ultrasonography, computed tomography (CT), positron emission tomography (PET), and magnetic resonance imaging (MRI) may reveal soft-tissue and organ degenerative changes resulting from inadequate oxygenation and chronic inflammation. Interventions The most common health problems for the client with sickle cell disease are pain, the potential complications of sepsis, and multiple organ dysfunction. Interventions are aimed at reducing or preventing these problems.
PAIN.
The most prominent clinical manifestation of sickle cell disease is pain. More than one fifth of clients experience frequent painful episodes and may have as many as 40 episodes per year (Rausch & Pollard, 1998). The pain associated with sickle cell crisis is the result of ischemic tissue injury caused by obstructed blood flow. The pain is often severe enough to require hospitalization and large doses of opioid analgesics. Pain is chronic with acute episodes and can occur anywhere in the body, often where circulation is impaired. It is sudden and is often described as gnawing or throbbing. Subjective reports of pain may be the only evidence, because the chronic nature of the pain may make physiologic changes less obvious. The subjective nature of the pain, racial prejudice, and concern for addiction often cause the client with sickle cell disease to be labeled as difficult (Gorman, 1999).
Health care providers must be aware of their own attitudes when caring for this population and must realize that lack of knowledge, in addition to concern for addiction, often prevents proper pain management of clients with sickle cell disease. Use of a pain rating scale by all nursing personnel can promote proper pain management. The nurse asks the client to rate pain on a scale ranging from 0 to 10 and evaluates the effectiveness of interventions based on the ratings. Use of contracts for pain control can also be helpful.
DRUG THERAPY.
Clients in acute sickle cell crisis often require at least 48 hours of parenteral analgesics. (Chart 40-3 highlights best practices for nursing care of the client in sickle cell crisis.)
Morphine and hydromorphone (Dilaudid) are the medications of choice (Gorman, 1999). For sickle cell crisis, these agents should be administered intravenously on a routine schedule. Once relief is obtained, the intravenous (IV) dose can be tapered and then administered orally (Rausch & Pollard, 1998). Meperidine (Demerol) is also used for sickle cell crisis, but long-term use of this agent can cause neurologic symptoms, including anxiety and seizures (Gorman, 1999). “As needed” (prn) schedules are discouraged because they do not provide adequate relief, and intramuscular (IM) injections are avoided because frequent injections lead to sclerosing of tissue (and absorption may be impaired by poor circulation). Moderate pain may be treated with oral doses of codeine, morphine sulfate, or nonsteroidal anti-inflammatory drugs (NSAIDs).
COMPLEMENTARY AND ALTERNATIVE THERAPIES.
Complementary therapies and other nonpharmacologic measures, such as keeping the room warm, using distraction and relaxation techniques, proper positioning with support for painful areas, aroma therapy, therapeutic touch, and warm soaks or compresses, have all been useful in decreasing pain. The nurse must not assume, however, that these methods alone will provide adequate pain relief. Analgesics are required to treat sickle cell pain.
POTENTIAL FOR SEPSIS.
The client with sickle cell disease is more susceptible to bloodborne infections and infection by encapsulated microorganisms, such as Streptococcus pneumoniae and Haemophilus influenzae, as a result of decreased spleen function. Interventions aim at preventing or halting the process of infection, controlling infection, and initiating early, effective treatment regimens for specific infections.
PREVENTION/EARLY DETECTION.
A major objective is to protect the client in sickle cell crisis from infection. Frequent, thorough handwashing is of the utmost importance. Any person with an upper respiratory tract infection who must enter the client’s room wears a mask. Strict aseptic technique is used for all invasive procedures. The nurse continually assesses the client for the presence of infection and monitors a daily complete blood count (CBC) with differential WBC count. The oral mucosa is inspected during every nursing shift for lesions indicating fungal or viral infection. The lungs are auscultated every 8 hours for crackles, wheezes, or diminished breath sounds. Each time the client voids, assistive nursing personnel inspect the urine for odor and cloudiness, and the client is asked about any sensation of urgency, burning, or pain during urination. Vital signs are taken at least every 4 hours to assess for fever.
DRUG THERAPY.
Drug therapy is a primary defense against the infections that develop in the client with sickle cell disease. Prophylactic therapy with twice-daily administration of oral penicillin in the penicillin-tolerant client has resulted in dramatic reductions in the number of pneumonia and other streptococcal infections. Yearly vaccination for influenza is advocated (Rausch & Pollard, 1998). Drug therapy for an actual infection can control infection and prevent complications associated with sepsis. Agents used depend on the sensitivity of the specific organism causing the infection, as well as the extent of the infection.
POTENTIAL FOR MULTIPLE ORGAN DYSFUNCTION.
The threat of multiple organ dysfunction arises from continued vascular occlusions after clumping of sickled cells. Management of sickle cell disease focuses on prevention of vascular occlusion and promotion of adequate oxygenation.
The client in sickle cell crisis is admitted to the acute care hospital. The nurse assesses for adequacy of circulation to all body areas. Restrictive clothing is removed, and the client is instructed to avoid keeping the hips or knees in a flexed position.
Dehydration perpetuates cell sickling and must be avoided. Nursing personnel assist the client in maintaining an adequate hydration status. The client in crisis requires an oral or parenteral intake of at least 200 mL/hr.
Oxygen is ordered, and the nurse ensures that oxygen therapy is delivered appropriately, including nebulization to prevent dehydration. Transfusion therapy has been used to decrease the incidence of organ dysfunction and stroke. RBC transfusions are therapeutic because levels of hemoglobin A (HbA) are sustained, whereas levels of hemoglobin S (HbS) are diluted. Transfusions also suppress erythropoiesis, thereby decreasing the production of sickle cells. Transfusions may be administered in either the acute care or clinic setting by a registered nurse. The nurse monitors the client closely for complications of transfusion therapy (discussed under Transfusion Reactions, p. 866).
In some treatment centers, bone marrow transplantation is being performed to correct abnormal hemoglobin permanently. Because bone marrow transplantation is expensive and may result in chronic and life-threatening complications, its risks and benefits need to be seriously considered for each client.
Community-Based Care
Sickle cell disease is a progressive disorder with periods of varying degrees of exacerbation. Rarely is there a true remission, although crisis episodes may be infrequent. Care focuses on prevention of complications, an ongoing daily necessity for the client with sickle cell disease.
The client with sickle cell disease may be cared for in a variety of settings, including acute care, subacute care, extended or assistive care, and home care. The client is taught to avoid specific activities that lead to hypoxia and hypoxemia. Recognition of the early signs and symptoms of crisis is emphasized, so that appropriate treatment can be initiated early to prevent undue pain, complications, and permanent tissue damage. The client is often given opioid analgesics for self-management of sickle cell crises at home; the nurse teaches clients and families about the correct administration. In addition, clients are counseled about the hereditary aspects of sickle cell disease, and information concerning prenatal diagnosis, birth control methods, and pregnancy options is offered.
Glucose-6-Phosphate Dehydrogenase Deficiency Anemia
OVERVIEW
Many forms of congenital hemolytic (blood cell-destroying) anemia result from defects or deficiencies of one or more enzymes within the red blood cell (RBC). More than 200 such disorders have been identified. Most of these enzymes are needed to complete some critical step in cellular energy production. The most common type of congenital hemolytic anemia is associated with a deficiency of the enzyme glucose-6- phosphate dehydrogenase (G6PD). This disease is inherited as an X-linked recessive disorder and affects about 10% of all African Americans (Cotran, Kumar, & Robbins, 1999).
G6PD stimulates critical reactions in the glycolytic pathway. RBCs contaio mitochondria (sites of high-efficiency production of the energy compoundadenosine triphosphate [ATP]), so active glycolysis is essential for energy metabolism. Newly produced RBCs from clients with G6PD deficiency have relatively sufficient quantities of G6PD; however, as the cells age, the concentration diminishes drastically. Cells that have reduced amounts of G6PD are more susceptible to breaking during exposure to specific drugs (e.g., phenacetin, sulfonamides, aspirin [acetylsalicylic acid], quinine derivatives, thiazide diuretics, and vitamin K derivatives) and toxins.
After exposure to any of these agents, clients experience acute intravascular hemolysis lasting from 7 to 12 days. During this acute phase, anemia and jaundice develop. The hemolytic reaction is self-limited because only older erythrocytes, containing less G6PD, are destroyed.
COLLABORATIVE MANAGEMENT
It is critical that the precipitating drug or the agent responsible for the hemolytic reaction be identified and totally removed. People should be screened for this deficiency before donating blood, because administration of cells deficient in G6PD can be hazardous for the recipient. During and immediately after an episode of hemolysis, adequate hydration is essential to prevent precipitation of cellular debris and hemoglobin in the kidney tubules, which can lead to acute tubular necrosis. Osmotic diuretics, such as mannitol (Osmitrol), may assist in preventing this complication. Transfusion therapy is indicated when anemia is present and kidney function is normal. Table 40-2 lists indications for transfusion with various types of blood components.
Immunohemolytic Anemia
OVERVIEW
Increased RBC destruction through hemolysis can occur in response to many situations, including trauma, infection (especially malarial infections), and autoimmune reactions. All increase the rate at which RBCs are destroyed by causing lysis (breakage) of the RBC membrane. The most common types of hemolytic anemias in industrialized countries are the immunohemolytic anemias, also referred to as autoimmune hemolytic anemias (Cotran, Kumar, & Robbins, 1999).
In immunohemolytic anemia, immune system components attack a person’s own RBCs. The exact mechanism that causes immune components to no longer recognize blood cells as self and to initiate destructive processes against RBCs is not known. Some hemolytic anemias are present with other autoimmune disorders (such as systemic lupus erythematosus) or lymphoproliferative disorders. Regardless of the cause, RBCs are viewed as non-self by the immune system and are destroyed.
There are two types of immunohemolytic anemia: warm antibody anemia and cold antibody anemia.
Warm antibody anemia is usually associated with immunoglobulin G (IgG) antibody excess. These antibodies are most active at 98° F (37° C) and may be stimulated by drugs, chemicals, or other autoimmune problems.
Cold antibody anemia is associated with fixation of complement proteins on immunoglobulin M (IgM) and occurs best at 86° F (30° C). This problem is commonly associated with a Raynaud-like response in which the arteries in the distal extremities constrict profoundly in response to cold temperatures or stress.
COLLABORATIVE MANAGEMENT
Treatment depends on clinical severity. Steroid therapy for mild to moderate immunosuppression is the first line of treatment and is temporarily effective in most clients. Splenectomy and more intensive immunosuppressive therapy with cyclophosphamide (Cytoxan, Procytox4) and azathioprine (Imuran) may be instituted if steroid therapy fails. Plasma exchange therapy to remove attacking antibodies is effective for clients who do not respond to immunosuppressive therapy.
ANEMIAS RESULTING FROM DECREASED PRODUCTION OF RED BLOOD CELLS
Anemias associated with decreased red blood cell (RBC) production can result from alterations in any of a variety of physiologic mechanisms. Some anemias are caused by failure or inability of the bone marrow to properly produce RBCs; others are caused by failure of the body to make or absorb a specific component necessary for RBC production.
Iron Deficiency Anemia
OVERVIEW
The adult body contains between 2 and 6 g of iron, depending on the size of the person and the amount of hemoglobin in the cells. Approximately two thirds of this iron is contained in hemoglobin; the other third is stored in the bone marrow, spleen, liver, and muscle. If a person has an iron deficiency, the iron stores are depleted first, followed by the hemoglobin stores.
As a result, RBCs are small (microcytic), and the client has relatively mild manifestations of anemia, including weakness and pallor.
Iron-deficiency Anemia, Peripheral Blood Smear
In iron deficiency anemia, serum ferritin values are less than 12 g/L. Iron deficiency anemia is the most common type of anemia and can result from blood loss, increased energy demands, gastrointestinal malabsorption, and dietary inadequacy. The basic problem of iron deficiency anemia is a decreased supply of iron for the developing RBC. Iron deficiency anemia can occur at any age but is more frequent in women, older adults, and people with poor diets.
COLLABORATIVE MANAGEMENT
The primary treatment of clients with iron deficiency anemia is to increase the oral intake of iron from common food sources (Table 40-3).
An adequate diet supplies a person with about 10 to 15 mg of iron per day, of which only 5% to 10% is absorbed in the stomach, duodenum, and upper jejunum (Worrall, Tompkins, & Rust, 1999). This amount is sufficient to meet the needs of healthy men and healthy women after childbearing age but is not sufficient to supply the greater needs of menstruating women and adolescents during growth spurts. Fortunately, if iron intake is inadequate, or if bleeding or pregnancy occurs, the gastrointestinal tract is capable of increasing the absorption of iron to about 20% to 30% of the total daily intake (Cotran, Kumar, & Robbins, 1999). When iron deficiency anemia is severe, iron preparations can be administered intramuscularly. Such preparations are administered using the Z-track best practice method outlined in Chart 40-4.
Thin, concave (spoon-shaped) nails with raised edges might interfere with iron absorption may be seen on people with iron deficiency anemia.
Vitamin B12 Deficiency Anemia
FIG.Vitamin B12 absorption and transport.
OVERVIEW
Proper production of RBCs depends on adequate deoxyribonucleic acid (DNA)synthesis in the precursor cells so that cell division and maturation into functional RBCs can occur. All DNA synthesis requires adequate amounts of folk acid (Mate) to ensure the availability of the nucleotide thymidine, which stimulates DNA synthesis.
One function of vitamin B12 is to serve as a cofactor to activate the enzyme system responsible for transporting folic acid into the cell, where DNA synthesis occurs. Thus a deficiency of vitamin B12 indirectly causes anemia by inhibiting folic acid transportation and limiting DNA synthesis in RBC precursor cells. These precursor cells then undergo improper DNA synthesis and increase in size. Only a few are released from the bone marrow.
This type of anemia is called megaloblastic (macrocytic) because of the large size of these abnormal cells. Vitamin B12 deficiency can result from inadequate intake (dietary deficiency). This can occur with strict vegetarian diets or diets lacking sufficient dairy products. Conditions such as small bowel resection, diverticula, tapeworm, or overgrowth of intestinal bacteria can lead to poor absorption of vitamin B12 from the intestinal tract (Worrall, Tompkins, & Rust, 1999).
Anemia caused by failure to absorb vitamin B12 (pernicious anemia) can also result from a deficiency of intrinsic factor (a substance normally secreted by the gastric mucosa), which is necessary for intestinal absorption of vitamin B12. Vitamin B12 deficiency anemia may be mild or severe, usually develops slowly, and produces few symptoms.
Clients usually have pallor and jaundice, as well as glossitis (a smooth, beefy-red tongue), fatigue, and weight loss. Because vitamin B12 also is necessary for normal nervous system functioning, especially of the peripheral nerves, clients with pernicious anemia may also have neurologic abnormalities, such asparesthesias (abnormal sensations) in the feet and hands and disturbances of balance and gait (Chart 40-5).
COLLABORATIVE MANAGEMENT
When anemia is caused by a dietary deficiency, the client must increase the intake of foods rich in vitamin B12 (animal proteins, eggs, dairy products). Vitamin supplements may be prescribed when anemia is severe. For clients who have anemia as a result of a deficiency of intrinsic factor, vitamin B12 must be administered parenterally on a regular schedule (usually weekly for initial treatment, then monthly for maintenance).
Folic Acid Deficiency Anemia
OVERVIEW
Primary folic acid deficiency can also cause megaloblastic anemia. Clinical manifestations are similar to those of vitamin B12 deficiency without the accompanying nervous system manifestations, because folic acid does not appear to affect nerve function. The absence of neurologic problems is an important diagnostic finding to differentiate folic acid deficiency from vitamin B12 deficiency. The disease develops slowly, and symptoms may be attributed to other problems or diseases. The three common causes of folic acid deficiency are poor nutrition, malabsorption, and drugs. Poor nutrition, especially a diet lacking green leafy vegetables, liver, yeast, citrus fruits, dried beans, and nuts, is the most common cause. Chronic alcohol abuse and parenteral alimentation without folic acid supplementation are other dietary causes. Malabsorption syndromes, such as Crohn’s disease, are the second most common cause. Specific drugs impede the absorption and conversion of folic acid to its active form and can also lead to folic acid deficiency and anemia. Such drugs include methotrexate, some anticonvulsants, and oral contraceptives.
Image: Folic Acid Deficiency
COLLABORATIVE MANAGEMENT
Prevention of folic acid deficiency anemia is aimed at identifying high-risk clients, such as older, debilitated clients with alcoholism; clients prone to malnutrition; and those with increased folic acid requirements. A diet high in folic acid and vitamin B12 prevents a deficiency (see Table 40-3). By routinely including assessment of dietary habits in a health history, the nurse can determine which clients are at risk for dietinduced anemias and provide appropriate follow-up. For the client diagnosed with this type of anemia, management includes oral folic acid 1 mg daily or intramuscular administration of folic acid for clients with absorption problems (Worrall, Tompkins, & Rust, 1999).
Aplastic Anemia
OVERVIEW
Aplastic anemia is a deficiency of circulating erythrocytes resulting from arrested development of RBCs within the bone marrow. It is caused by an injury to the hematopoietic precursor cell, the pluripotent stem cell.
Although aplastic anemia sometimes occurs alone, it is usually accompanied by agranulocytopenia (a reduction in leukocytes) and thrombocytopenia (a reduction in platelets). These three problems occur at the same time because the bone marrow produces not only RBCs but also white blood cells (WBCs) and platelets. Consequently, if the bone marrow is abnormal for any reason or if it has been exposed to a toxic substance that can damage bone marrow cells, production of erythrocytes, leukocytes, and thrombocytes slows greatly.
Pancytopenia (a deficiency of all three cell types) is common in aplastic anemia. The onset of aplastic anemia may be insidious or rapid. The development of aplastic anemia, although relatively rare, is associated with chronic exposure to several toxic agents (see Table 39-4). In about 50% of cases, the cause of aplastic anemia is unknown. Aplastic anemia may occur as an aftermath of viral infection (Cotran, Kumar, & Robbins, 1999), but the mechanism of bone marrow damage is unknown.
Symptoms of Aplastic Anemia
COLLABORATIVE MANAGEMENT
Blood transfusions are the mainstay of treatment for clients with aplastic anemia. Transfusion is indicated only when the anemia causes real disability or when bleeding is life threatening because of thrombocytopenia. Unnecessary transfusion, however, increases the opportunity for the development of immune reactions to platelets, shortens the life span of the transfused cell, and may increase the rate of rejection of transplanted marrow cells. Thus transfusions are discontinued as soon as the bone marrow begins to produce RBCs. Because clients with some types of aplastic anemia have a disease course similar to that of autoimmune problems, immunosuppressive therapy may be helpful.
Agents that selectively suppress lymphocyte activity, such as antilymphocyte globulin (ALG), antithymocyte globulin (ATG), and cyclosporine (Sandimmune), have brought about partial or complete remissions. In more severe cases, general immunosuppressive agents, such as prednisone and cyclophosphamide (Cytoxan, Procytox”*1), have been effective.
Splenectomy (removal of the spleen) is considered in clients with an enlarged spleen that is either destroying normal RBCs or suppressing their development. Bone marrow transplantation, which replaces defective stem cells, has also resulted in a cure for some clients (Cotran, Kumar, & Robbins, 1999). Cost, availability, and complications limit this technique for treatment of aplastic anemia, however.
POLYCYTHEMIA
In polycythemia, the number of red blood cells (RBCs) in whole blood is greater thaormal. The blood of a client with polycythemia is hyperviscous (thicker thaormal blood). The problem may be temporary (occurring as a result of other conditions) or chronic. One type of polycythemia, polycythemia vera, is fatal if left untreated.
Polycythemia Vera
OVERVIEW
Polycythemia vera (PV) is characterized by a sustained increase in blood hemoglobin concentration to 18 g/dL, an RBC count of 6 million/mm3, or a hematocrit increase to 55% or greater. PV is a cancer of the RBCs with three major hall marks: continuous production of massive numbers of RBCs, excessive leukocyte production, and overproduction of thrombocytes. As described in Chart 40-6, extreme hypercellularity (cell excess) of the peripheral blood occurs in people with PV.
The skin, especially facial, and mucous membranes have a dark, flushed (plethoric) appearance. These areas may appear purplish or cyanotic because the blood in these tissues is incompletely oxygenated. Most clients experience intense itching related to vasodilation and variation in tissue oxygenation. Blood viscosity is also greatly increased, causing a corresponding increase in peripheral resistance. Superficial veins are visibly distended. Blood moves more slowly through all tissues and thus places increased demands on the pumping action of the heart, resulting in hypertension. In some highly vascular areas, blood flow may become so slow that vascular stasis occurs. Vascular stasis causes thrombosis (clot formation) within the smaller vessels to the extent that the vessels are occluded and the surrounding tissues experience hypoxia, progressing to anoxia and further to infarction and necrosis. Tissues most prone to this complication are the heart, spleen, and kidneys, although infarction with loss of tissue and organ function can occur in any organ or tissue.
Because the actual number of cells in the blood is greatly increased and the cells are not completely normal, individual cell life spans are shorter. The shorter life spans, coupled with increased cell production, result in a rapid turnover of peripheral blood cells. This rapid turnover increases the amount of intracellular products (released when cells die) in the blood, adding to the general “sludging” of the blood. These products include uric acid and potassium, which cause the symptoms of gout and hyperkalemia (elevated serum potassium level). Later clinical manifestations of PV are related to abnormal blood cells. Even though the number of circulating erythrocytes is greatly increased, their oxygen-carrying capacity is impaired, and clients experience severe generalized hypoxia. In spite of the RBC excess, most clients with PV are susceptible to bleeding problems because of an associated platelet dysfunction (Cotran, Kumar, & Robbins, 1999).
COLLABORATIVE MANAGEMENT
Polycythemia vera is a malignant disease that progresses in severity over time. If left untreated, few people with PV live longer than 2 years. Conservative management with repeated phlebotomies (two to five times per week) can prolong life for 5 to 10 years.(Phlebotomy is the collection of the client’s RBCs to decrease the number of RBCs and diminish blood viscosity.) Maintaining adequate hydration and promoting venous return are essential to prevent thrombus formation. Therapy aims to prevent clot formation and includes the use of anticoagulants. Chart 40-7 lists preventive tips for clients with PV.
As the disease progresses, clients need more intensive therapies that suppress bone marrow activity, including oral alkylating agents and/or irradiation with injections of radioactive phosphorus. Bone marrow transplantation, an experimental treatment, is promising, but the results are too limited to determine its application to PV.
PLATELET DISORDERS
Platelets play a vital role in hemostasis.
For both the intrinsic and the extrinsic pathways, blood clotting starts with platelet adhesion and the formation of a platelet plug. Any condition that either reduces the number of platelets or interferes with their ability to adhere (to one another, blood vessel walls, collagen, or fibrin threads) can be manifested as increased bleeding. Platelet disorders can be inherited, acquired, or temporarily induced by the ingestion of substances that limit platelet production or inhibit aggregation. A drop in the number of platelets below the level needed for normal coagulation is called thrombocytopenia.
Thrombocytopenia may occur as a result of other conditions or treatments that suppress general bone marrow activity. It also can occur through processes that specifically limit platelet formation or increase the rate of platelet destruction. The two thrombocytopenic conditions affecting adults are autoimmune thrombocytopenic purpura and thrombotic thrombocytopenic purpura.
Autoimmune Thrombocytopenic Purpura
OVERVIEW
Before the underlying cause of autoimmune thrombocytopenic purpura was identified, this condition was known as idiopathic thrombocytopenic purpura (ITP). Although the cause is now thought to be an autoimmune reaction, the condition is still commonly known as ITP. The total number of circulating platelets is greatly reduced in ITP, even though platelet production in the bone marrow is normal. Clients with idiopathic thrombocytopenic purpura make an antibody directed against the surface of their own platelets (an antiplatelet antibody). This antibody coats the surface of the platelets, making them more susceptible to attraction and destruction by phagocytic leukocytes, especially macrophages. Because the spleen contains a large concentration of macrophages and because the blood vessels of the spleen are long and twisted, antibody-coated platelets are destroyed primarily in the spleen. When the rate of platelet destruction exceeds that of production, the number of circulating platelets decreases and blood clotting slows. Although the cause of this disorder appears to be autoimmune, the exact mechanism initiating the production of autoantibodies is unknown. ITP is most common among women between the ages of 20 and 40 and among people with a preexisting autoimmune condition, such as systemic lupus erythematosus (Cotran, Kumar, & Robbins, 1999).
Fig. Purpura
Fig. Tthrombocytopenic purpura
Fig. Ecchymoses of the hand from thrombocytopenia
COLLABORATIVE MANAGEMENT
Assessment
Clinical manifestations associated with ITP are generally limited to the skin and mucous membranes: large ecchymoses (bruises) on the arms, legs, upper chest, and neck or a petechial rash. Mucosal bleeding occurs easily. If the client has experienced significant blood loss, signs of anemia may also be present. A rare complication is an intracranial bleeding-induced stroke. The nurse assesses for neurologic function and mental status. Family members or significant others are asked if the client’s behavior and responses to the mental status examination are typical or represent a change from usual reactions. Idiopathic thrombocytopenic purpura is diagnosed by a decreased platelet count and large numbers of megakaryocytes in the bone marrow. Antiplatelet antibodies may be present in detectable levels in peripheral blood. If the client experiences any episodes of bleeding, hematocrit and hemoglobin levels also are low.
Thrombocytopenia
Interventions
NONSURGICAL MANAGEMENT.
As a result of the decreased platelet count, the client is at great risk for bleeding. Interventions include therapy for the underlying condition, as well as protection from trauma-induced bleeding episodes.
DRUG THERAPY.
Agents used to control ITP include drugs that suppress immune function to some degree. The premise for the use of agents such as corticosteroids and azathioprine (Imuran) is to inhibit immune system synthesis of antiplatelet autoantibodies. More aggressive therapy can include low doses of chemotherapeutic agents, such as the antimitotic agents and cyclophosphamide.
BLOOD REPLACEMENT THERAPY.
For the client with a platelet count of less than 20,000/mm3 who is experiencing an acute life-threatening bleeding episode, a platelet transfusion may be required. Platelet transfusions are not performed routinely, because the donated platelets are just as rapidly destroyed by the spleen as the client’s own platelets (see later discussion under Platelet Transfusions, p. 865).
MAINTAINING A SAFE ENVIRONMENT.
The nurse’s major objectives are to protect the client from situations that can lead to bleeding and to closely monitor the amount of bleeding that is occurring. (For nursing care actions, see Risk for Injury [Leukemia], p. 856.)
SURGICAL MANAGEMENT.
For the client who does not respond to drug therapy, splenectomy may be the treatment of choice. Because the leukocytes in the spleen perform many different immunodefensive functions, the client who has undergone a splenectomy is at increased risk for infection.
Thrombotic Thrombocytopenic Purpura
OVERVIEW
Thrombotic thrombocytopenic purpura (TTP) is a rare disorder in which platelets clump together inappropriately in the microcirculation and insufficient platelets remain in the systemic circulation. The client experiences inappropriate clotting, yet the blood fails to clot properly when trauma occurs. The underlying cause of TTP appears to be an autoimmune reaction in blood vessel cells (endothelial cells) that makes platelets clump together in very small blood vessels. As a result, tissues become ischemic. Common manifestations include renal failure, myocardial infarction, and stroke. If left untreated, this condition is fatal within 3 months in 90% of clients (McBrien, 1997).
COLLABORATIVE MANAGEMENT
Treatment for the client with TTP focuses on inhibiting the inappropriate platelet aggregation and disrupting the underlying autoimmune process. Primary treatment consists of plasma pheresis with the infusion of fresh frozen plasma. This treatment provides the necessary platelet aggregate inhibitors (McBrien, 1997). Drugs that inhibit platelet clumping, such as aspirin, alprostadil (Prostin), and plicamycin, also may be helpful. Immunosuppressive therapy reduces the intensity of this disorder.
CLOTTING FACTOR DISORDERS
Coagulation or bleeding disorders can result from a clotting factor defect, including the inability to produce a specific clotting factor, production of insufficient quantities, or a less active form of clotting factor. Most clotting factor disorders are congenitally transmitted gene abnormalities of one clotting factor. The few acquired clotting factor disorders are related to an inability to synthesize many clotting factors at the same time as a result of liver damage or an insufficiency of clotting cofactors and precursor products. Common congenital disorders that result in defects at the clotting factor level include hemophilias A and B and von Willebrand’s disease. Disseminated intravascular coagulation (DIC) may be considered an acquired clotting disorder but is more closely associated with septic shock.
Hemophilia
OVERVIEW
Hemophilia comprises two hereditary bleeding disorders resulting from deficiencies of specific clotting factors.
Hemophilia A (classic hemophilia) results from a deficiency of factor VIII and accounts for 80% of cases of hemophilia.
Hemophilia B (Christmas disease) is a deficiency of factor IX and accounts for 20% of cases. The incidence of both is 1 in 10,000.
Hemophilia is an Xlinked recessive trait.
Female carriers have a 50% chance of transmitting the gene for hemophilia to their daughters (who then are carriers) and to their sons (who will have overt hemophilia).
Hemophilia A is, with rare exceptions, a disease affecting males, none of whose sons will have the gene for hemophilia and all of whose daughters will be carriers (able to pass on the gene without actually having the disorder). In about 30% of clients with hemophilia, there is no family history, and it is presumed that their disease is the result of a new mutation (Cotran, Kumar, & Robbins, 1999).
The bleeding disorder associated with hemophilia A is so severe that before blood transfusions were available, children with hemophilia rarely survived past age 3 years. With the availability of blood transfusion and factor VIII therapy, mean survival time has increased so greatly that hemophilia now is commonly seen among adult clients. The clinical pictures of hemophilia A and B are identical.
The client has abnormal bleeding in response to any trauma because of an absence or deficiency of the specific clotting factor. Clients with hemophilia do not bleed more frequently or even more rapidly that those without the disease, but they do bleed for a longer period (Ligda, 1998). Hemophiliacs form platelet plugs at the bleeding site, but the clotting factor deficiency impairs the hemostatic response and the capacity to form a stable fibrin clot. This produces abnormal bleeding, which may be mild, moderate, or severe, depending on the degree of factor deficiency.
COLLABORATIVE MANAGEMENT
Assessment
Assessment of the client with hemophilia reveals the following:
Ø Excessive hemorrhage from minor cuts or abrasions caused by abnormal platelet function
Ø Joint and muscle hemorrhages that lead to disabling long-term sequelae
Ø A tendency to bruise easily
Ø Prolonged and potentially fatal postoperative hemorrhage
The laboratory test results for a client with hemophilia demonstrate a prolonged partial thromboplastin time (PTT), a normal bleeding time, and a normal prothrombin time (PT) (Cotran, Kumar, & Robbins, 1999). The most common health problem associated with hemophilia is degenerating joint function resulting from chronic bleeding into the joints, especially at the hip and knee.
Interventions
The bleeding problems of hemophilia A can be well managed by either regularly scheduled IV administration of factor VIII cryoprecipitate or intermittent administration as needed, depending on the individual’s activity level and injury probability (see Cryoprecipitate, p. 865). However, the cost of cryoprecipitate is prohibitive for many people with hemophilia. In addition, because the precipitated clotting factors are derived from pooled human serum, a risk of viral contamination remains, even with the use of heat-inactivated serum (Ligda, 1998). Major complications of hemophilia therapy during the 1980s were infection with hepatitis B virus, cytomegalovirus, and human immunodeficiency virus (HIV). Although heat-inactivated serum and the elimination of HIV-positive donors have reduced these risks, they have not yet been eliminated.
TRANSFUSION THERAPY
Two nurses must check the label on the blood product bag, the blood administration form of the blood bank, and the patient’s armband and blood bracelet.
Any blood component may be removed from a donor and transfused to benefit a recipient. Components may be transfused individually or collectively, with varying degrees of benefit to the recipient.
PRETRANSFUSION RESPONSIBILITIES
Nursing actions during transfusions aim largely at prevention or early recognition of adverse transfusion reactions. Preparation of the client for transfusion therapy is imperative, and in stitutional blood product administration procedures should be carefully followed. Before administering any blood product, the nurse reviews the agency’s policies and procedures.
Chart 40-15 presents best practices for transfusion therapy.
Legally, a physician’s order is needed to administer blood or its components. The order specifies the type of component to be delivered, the volume to be transfused, and any special conditions the physician judges to be important. The nurse verifies the order for accuracy and completeness. The nurse also evaluates the need for transfusion, considering both the client’s clinical condition and the laboratory values. In many hospitals a separate consent form must be obtained for the administration of blood products before a transfusion is performed.
A blood specimen is obtained for crossmatching (testing of the donor’s blood and the recipient’s blood for compatibility). The procedure and responsibility for obtaining this specimen are specified by hospital policy. The laboratory requires at least 45 minutes to complete the crossmatch testing. In most hospitals a new crossmatching specimen is required at least every 48 hours (Dreger & Tremback, 1998).
Because of the viscosity of blood components, a 19-gauge needle or larger is used, whenever possible, for venous access. Both Y-tubing and straight tubing sets are available for blood component administration. A blood filter (approximately 170 xm) to remove aggregates from the stored blood products is included with component administration equipment and must be used to transfuse all blood products. In massive transfusion, a microaggregate filter (20 to 40 jam) may be used.
Normal saline is the solution of choice for administration. Ringer’s lactate and dextrose in water are contraindicated for administration with blood or blood products because they cause clotting or hemolysis of blood cells.
Medications are never added to blood products.
Before the transfusion is initiated, it is essential to determine that the blood component delivered is correct. Two registered nurses simultaneously check the physician’s order, the client’s identity, and whether the hospital identification band name and number are identical to those on the blood component tag. The blood bag label, the attached tag, and the requisition slip are examined to ensure that the ABO and Rh types are compatible. The expiration date is also checked, and the product is inspected for discoloration, gas bubbles, or cloudiness—indicators of bacterial growth or hemolysis.
TRANSFUSION RESPONSIBILITIES
The nurse takes the vital signs, including temperature, immediately before initiating the transfusion. Infusion begins slowly. A nurse remains with the client for the first 15 to 30 minutes. Any severe reaction usually occurs with administration of the first 50 mL of blood. The nurse assesses vital signs 15 minutes after initiation of the transfusion to detect signs of a reaction. If there are none, the infusion rate can be increased to transfuse 1 unit in about 2 hours (depending on the client’s cardiovascular status). The nurse takes the vital signs every hour throughout the transfusion or as specified by agency policy. Blood components without large amounts of red blood cells (RBCs) can be infused more quickly. The identification checks are the same as for RBC transfusions. Physiologic changes in older clients may necessitate that blood products be transfused at a slower rate.
Best practices related to the nursing care needs of older clients undergoing transfusion therapy are provided in Chart 40-16.
TYPES OF TRANSFUSIONS
Red Blood Cell Transfusions
RBCs are administered to replace erythrocytes lost as a result of trauma or surgical interventions. Clients with clinical conditions that result in the destruction or abnormal maturation of RBCs may also benefit from RBC transfusions. Packed RBCs, supplied in 250-mL bags, are a concentrated source of RBCs and are the most common component administered to RBCdeficient clients (Dreger & Tremback, 1998). Packed RBCs are administered to individuals with a hemoglobin concentration less than 6 g/dL (or a hemoglobin value of 6 to 10 g/dL if clinical symptoms are present) (Kennedy, 1999). Blood transfusions are actually transplantations of tissue from one person to another. The donor and recipient blood must thus be carefully checked for compatibility to prevent potentially lethal reactions (Table 40-7).
Compatibility is determined by two different types of antigen systems (cell surface proteins): the ABO system antigens and the Rh antigen, present on the membrane surface of RBCs (Dreger & Tremback, 1998). RBC antigens are inherited.
For the ABO antigen system, a person inherits one of the following:
Ø A antigen (type A blood)
Ø B antigen (type B blood)
Ø Both A and B antigens (type AB blood)
Ø No antigens (type O blood)
Within the first few years of a child’s life, circulating antibodies develop against the blood type antigens that were not inherited. For example, a child with type A blood will form antigens against type B blood. A child with type O blood has not inherited either A or B antigens and will form antibodies against RBCs that contain either A or B antigens. If erythrocytes that contain a foreign antigen are infused into a recipient, the donated tissue can be recognized by the immune system of the recipient as non-self, and the client may have a reaction to the transfused products.
The mechanism of the Rh antigen system is slightly different. An Rh-negative person is born without the antigen and does not form antibodies unless he or she is specifically sensitized to it. Sensitization can occur with RBC transfusions from an Rh-positive person or from exposure during pregnancy and birth. Once an Rh-negative person has been sensitized and antibody development has occurred, any exposure to Rh-positive blood can cause a transfusion reaction. Antibody development can be prevented by administration of Rh-immune globulin as soon as exposure to the Rh antigen is suspected. People who have Rh-positive blood can receive an RBC transfusion from an Rh-negative donor, but Rh-negative people must never receive Rh-positive blood.
Platelet Transfusions
Platelets are administered to clients with platelet counts below 20,000 mm3 and to clients with thrombocytopenia who are actively bleeding or are scheduled for an invasive procedure (Dreger, & Tremback, 1998). Platelet transfusions are usually pooled from as many as 10 donors and do not have to be of the same blood type as the client.
For clients who are candidates for bone marrow transplantation (BMT) or who require multiple platelet transfusions, single-donor platelets may be ordered. Single-donor platelets are obtained from one person and decrease the amount of antigen exposure to the recipient, helping prevent the formation of platelet antibodies. The chances of allergic transfusion reactions to future platelet transfusions are thus reduced.
Platelet infusion bags usually contain 300 mL for pooled platelets and 200 mL for single-donor platelets. Because the platelet is a fragile cell, platelet transfusions are administered rapidly after being brought to the client’s room, usually over a 15- to 30-minute period. A special transfusion set with a smaller filter and shorter tubing is used. Standard transfusion sets are not used with platelets because the filter traps the platelets, and the longer tubing increases platelet adherence to the lumen. Additional platelet filters help remove white blood cells (WBCs) in the platelet concentrate. These filters are connected directly to the platelet transfusion set and are used for clients who have a history of febrile reactions or who will require multiple platelet transfusions.
The nurse takes the vital signs before the infusion, 15 minutes after the infusion is initiated, and at its completion. The client may be premedicated with meperidine (Demerol) or hydrocortisone to minimize the chances of a reaction. He or she can become febrile and experience rigors (severe chills) during transfusion, but these symptoms are not considered a true transfusion reaction. IV administration of amphotericin B (Amphotec, Fungizone), an antifungal agent given to many clients with leukemia, is discontinued during platelet transfusion and not resumed for at least 1 hour after transfusion. Amphotericin B can cause severe allergic reactions that are difficult to distinguish from transfusion reactions.
Plasma Transfusions
Historically, plasma infusions have been administered to replace blood volume, and they are occasionally still used for this purpose. It is more common for plasma to be frozen immediately after donation. Freezing preserves the clotting factors, and the plasma can then be used for clients with clotting disorders (Kennedy, 1999).
Fresh frozen plasma (FFP) is infused immediately after thawing while the clotting factors are still viable. Clients who are actively bleeding with a prothrombin time (PT) or partial thromboplastin time (FIT) greater than 1.5 times normal are candidates for an FFP infusion (Harrahill & DeLoughery, 1998). ABO compatibility is required for transfusion of plasma products. The volume of the infusion bag is approximately 200 mL. The infusion takes place as rapidly as the client can tolerate, generally over a 30- to 60-minute period, through a regular Y-set or straight-filtered tubing.
Fresh frozen plasma or cryodepleted plasma can be used for plasma exchange
Cryoprecipitate is a product derived from plasma. Clotting factors VIII and XIII, von Willebrand’s factor, fibronectin, and fibrinogen are precipitated from pooled plasma to produce cryoprecipitate. Clients with a fibrinogen level less than 100 mg/dL are candidates for a cryoprecipitate infusion (Harrahill & DeLoughery, 1998). This highly concentrated blood product is administered to clients with clotting factor disorders at a volume of 10 to 15 mL/unit. Although cryoprecipitate can be infused, it is usually given by IV push within 3 minutes. Dosages are individualized, and it is best if the cryoprecipitate is ABO compatible.
Granulocyte Transfusions
At some centers, neutropenic clients with infections receive granulocyte transfusions for WBC replacement. However, this practice is highly controversial because the potential benefit to the client must be weighed against the potential severe reactions that often accompany granulocyte transfusions. The surfaces of granulocytes contaiumerous antigens that can cause severe antibody-antigen reactions when infused into a recipient whose immune system recognizes these antigens as non-self. In addition, transfused granulocytes have a very short life span and are probably of minimal benefit to the client (see Chapter 20). There is some evidence that treatment with antibiotics alone results in better survival rates. Granulocytes are suspended in 400 mL of plasma and should be transfused over a 45- to 60- minute period. Institutional policies often require more stringent monitoring of clients receiving granulocytes. A physician may need to be present in the hospital unit, and vital signs may need to be taken every 15 minutes throughout the transfusion. Administration of amphotericin B and granulocyte transfusions should be separated by 4 to 6 hours.
TRANSFUSION REACTIONS
Clients can experience any of the following transfusion reactions: hemolytic, allergic, febrile, or bacterial reactions; circulatory overload; or transfusion-associated graft-versus-host disease (GVHD). The nurse is vigilant to prevent serious complications through early detection and initiation of appropriate treatment.
Hemolytic Transfusion Reactions
Hemolytic transfusion reactions are caused by blood type or Rh incompatibility. When blood containing antibodies against the recipient’s blood is infused, antigen-antibody complexes are formed and released into the circulation. These complexes can destroy the transfused cells and initiate inflammatory responses in the recipient’s blood vessel walls and organs. The ensuing reaction may be mild, with fever and chills, or life threatening, with disseminated intravascular coagulation (DIC) and circulatory collapse (Robb, 1999).
Other clinical signs include the following:
Ø Apprehension
Ø Headache
Ø Chest pain
Ø Low back pain
Ø Tachycardia
Ø Tachypnea.
Ø Hypotension
Ø Hemoglobinuria
Ø A sense of impending doom
The onset of a hemolytic transfusion reaction may be immediate or may not occur until subsequent units have been transfused.
Allergic Transfusion Reactions
Allergic transfusion reactions are most often seen in clients with a history of allergy. They may have urticaria, itching, bronchospasm, or occasionally anaphylaxis. Onset of this type of reaction usually occurs during or up to 24 hours after the transfusion. Clients with a history of allergy can be given buffy coat-poor or washed red blood cells (RBCs) in which the white blood cells (WBCs) and plasma have been removed. This procedure minimizes the possibility of an allergic reaction.
Febrile Transfusion Reactions
Febrile transfusion reactions occur most commonly in the client with anti-WBC antibodies, a situation seen after multiple transfusions. The recipient experiences the following: Sensations of cold • Tachycardia • Fever • Hypotension • Tachypnea Again, the physician can order buffy coat-poor RBCs or single-donor HLA-matched platelets. Leukocyte filters may also be used to trap WBCs and prevent their transfusion into the client.
Figure. Febrile transfusion reaction algorithm.
BP = blood pressure;
CXR = chest x-ray;
DAT = direct antiglobulin test;
Dx = diagnosis;
SOB = shortness of breath;
TRALI = transfusion-related acute lung injury
Bacterial Transfusion Reactions
Bacterial transfusion reactions are seen after transfusion of contaminated blood products. Usually a gram-negative organism is the source because these bacteria grow rapidly in blood stored under refrigeration.
Symptoms include the following:
• Tachycardia
• Hypotension
• Fever
• Chills
• Shock
The onset of a bacterial transfusion reaction is rapid.
Circulatory Overload
Circulatory overload can occur when a blood product is administered too quickly (Goldy, 1998). This complication is most common with whole-blood transfusions or when the client requires multiple transfusions. Older adults are most at risk for this condition (see Chart 40-16). Symptoms include the following: • Hypertension • Bounding pulse • Distended jugular veins • Dyspnea • Restlessness • Confusion The nurse can both manage and prevent this complication by monitoring intake and output, transfusing blood products more slowly, and administering diuretics.
Transfusion-Associated Graft-Versus-Host Disease
Transfusion-associated graft-versus-host disease (TA-GVHD) is an infrequent but life-threatening complication that can occur in both immunosuppressed and immunocompetent clients. Its cause in immunosuppressed clients is similar to that of GVHD associated with allogeneic bone marrow transplantation (BMT), discussed on p. 855, in which donor T-cell lymphocytes attack host tissues. The cause of TA-GVHD in immunocompetent hosts is uncertain. Reactions are more common when the host and donor share similar human leukocyte antigens (HLAs), such as in first-degree relatives or individuals with a similar ethnic background. Symptoms typically occur within 1 to 2 weeks and include thrombocytopenia, anorexia, nausea, vomiting, chronic hepatitis, weight loss, and recurrent infection. TA-GVHD has a 90% mortality rate but can be prevented by using irradiated blood products, which destroy T-cells and their cytokine products (Dreger & Tremback, 1998).
AUTOLOGOUS BLOOD TRANSFUSIONS
Autologous blood transfusions involve collection and transfusion of the client’s own blood. Advantages of this type of transfusion are guaranteed compatibility and elimination of the risk of transmitting diseases such as hepatitis or HIV. The four types of autologous blood transfusions are preoperative autologous blood donation, acute normovolemic hemodilution, intraoperative autologous transfusion, and postoperative blood salvage. Preoperative autologous blood donation, the most common type of autologous blood transfusion, involves collection of whole blood from the client, division into components, and then storage for later use (such as after a scheduled surgical procedure).
As long as hematocrit and hemoglobin levels are within a safe range, the client can donate blood on a weekly basis until the prescribed amount of blood is obtained. Fresh packed RBCs may be stored for 42 days. For individuals with rare blood types, blood may be frozen for up to 10 years. Platelets and plasma may be collected via pheresis. Some cardiovascular problems and bacteremia are contraindications for autologous blood donation. Acute normovolemic hemodilution involves withdrawal of a client’s RBCs and volume replacement just before a surgical procedure. The goal is to decrease RBC loss during surgery. The blood is stored at room temperature for up to 6 hours and reinfused after surgery. This type of autologous transfusion is appropriate for healthy clients but is contraindicated for individuals who are anemic or who have poor renal function. Intraoperative autologous transfusion and postoperative blood salvage involve the recovery and reinfusion of a client’s own blood, collected either from an operative field or postoperatively from a wound.
Several commercial products are available that collect, filter, and drain the blood into a transfusion bag. This autologous blood is often used for trauma or surgical clients with severe blood loss and must be reinfused within 6 hours. The nurse transfuses autologous blood products using the guidelines previously described. Although the client receiving autologous blood is not at risk for most types of transfusion reactions, the nurse must still assess for circulatory overload or bacterial transfusion reactions that can occur as a result of contamination.
