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Red blood cells (RBCs), also known as erythrocytes, are the major cell in the blood. RBC disorders include problems in production, function, and destruction. Problems may result in poor function of RBCs, decreased numbers of RBCs (anemia), or an excess of RBCs (polycythemia).
Anemia is a reduction in either the number of RBCs, the amount of hemoglobin, or the hematocrit (percentage of packed RBCs per deciliter of blood). Anemia can result from dietary problems, genetic disorders, bone marrow disease, or excessive bleeding. GI bleeding is the most common reason.
Anemia is a reduction in either the number of RBCs, the amount of hemoglobin, or the hematocrit (percentage of packed RBCs per deciliter of blood). Anemia can result from dietary problems, genetic disorders, bone marrow disease, or excessive bleeding. GI bleeding is the most common reason.
Integumentary Manifestations
• Pallor, especially of the ears, the nail beds, the palmar creases, the conjunctivae, and around the mouth
• Cool to the touch
• Intolerance of cold temperatures
• Nails become brittle and become concave over time
Cardiovascular Manifestations
• Tachycardia at basal activity levels, increasing with activity and during and immediately after meals
• Murmurs and gallops heard on auscultation when anemia is severe
• Orthostatic hypotension
Respiratory Manifestations
• Dyspnea on exertion
• Decreased oxygen saturation levels
Neurologic Manifestations
• Increased somnolence and fatigue
• Headache
• Pallor, especially of the ears, the nail beds, the palmar creases, the conjunctivae, and around the mouth
• Cool to the touch
• Intolerance of cold temperatures
• Nails become brittle and become concave over time
Cardiovascular Manifestations
• Tachycardia at basal activity levels, increasing with activity and during and immediately after meals
• Murmurs and gallops heard on auscultation when anemia is severe
• Orthostatic hypotension
Respiratory Manifestations
• Dyspnea on exertion
• Decreased oxygen saturation levels
Neurologic Manifestations
• Increased somnolence and fatigue
• Headache
Sickle cell disease (SCD) is a genetic disorder that results in chronic anemia, pain, disability, organ damage, increased risk for INFECTION, and early death. 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).
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.
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 (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 and ischemia. Cause progressive organ damage from anoxia and infarction. 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. Usually sickled cells go back to normal shape when the precipitating condition is removed, the blood oxygen level is normalized, and proper tissue PERFUSION resumes. Some of the hemoglobin remains twisted.
The average life span of an RBC containing 40% or more of HbS is about 10 to 20 days compared to 120. Reduced RBC life span causes hemolytic (blood cell-destroying) anemia.
CRISES have a sudden onset, which are periodic episodes of extensive cellular sickling. Damage results from tissue hypoxia, anoxia, ischemia, and cell death. Organs begin to have small infarcted areas and scar tissue formation, and eventually organ failure results. Tissues most often affected are the spleen, liver, heart, kidney, brain, joints, bones, and retina.
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.
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 (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 and ischemia. Cause progressive organ damage from anoxia and infarction. 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. Usually sickled cells go back to normal shape when the precipitating condition is removed, the blood oxygen level is normalized, and proper tissue PERFUSION resumes. Some of the hemoglobin remains twisted.
The average life span of an RBC containing 40% or more of HbS is about 10 to 20 days compared to 120. Reduced RBC life span causes hemolytic (blood cell-destroying) anemia.
CRISES have a sudden onset, which are periodic episodes of extensive cellular sickling. Damage results from tissue hypoxia, anoxia, ischemia, and cell death. Organs begin to have small infarcted areas and scar tissue formation, and eventually organ failure results. Tissues most often affected are the spleen, liver, heart, kidney, brain, joints, bones, and retina.
Sickle cell disease (SCD) is a genetic disorder with an autosomal recessive pattern of inheritance. In sickle cell disease, the patient has two HbS gene alleles, one inherited from each parent, usually resulting in 80% to100% of the hemoglobin being HbS. Because both hemoglobin alleles are S, sickle cell disease is sometimes abbreviated "SS."
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 half of the hemoglobin chains are abnormal. Sickle cell trait is abbreviated "AS."
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 half of the hemoglobin chains are abnormal. Sickle cell trait is abbreviated "AS."
Those with sickle cell trait usually have no manifestations or abnormal laboratory findings other than the presence of hemoglobin S. This person may be unaware that he or she has 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.
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
Ask about previous crises, what led to the crises, severity, and usual management.
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
Pain is the most common manifestation of SCD crisis.
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. Compare 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. Heart rate may be rapid and blood pressure may be low to average.
Priapism is a prolonged erection that can occur in men who have SCD. The cause is excessive vascular engorgement in erectile tissue. Urinary retention!
Skin changes include pallor or cyanosis because of poor GAS EXCHANGE from decreased PERFUSION. Examine the lips, tongue, nail beds, conjunctivae, palms, and soles of the feet for color. With cyanosis, the lips and tongue are gray and the palms, soles, conjunctivae, and nail beds have a bluish tinge. 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. Jaundice equals itching. Inspect the legs and feet for ulcers or darkened areas that may indicate necrotic tissue.
Abdominal changes include damage to the spleen and liver. In crisis, abdominal pain is diffuse and steady, involving the back and legs. The liver or spleen may feel firm and enlarged with a nodular or "lumpy" texture in late stage.
Kidney and urinary changes are common. Chronic kidney disease occurs as a result of anoxic damage to the kidney nephrons. The urine contains protein, and the patient may not concentrate urine. Eventually, the kidneys fail, resulting in little or no urine.
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.
Central nervous system (CNS) changes may occur. With crises, patients may have a low-grade fever. If the CNS has infarcts or repeated episodes of hypoxia, patients may have seizures or manifestations of a stroke. Assess for the presence of "pronator drift," bilateral hand grasp strength, gait, and coordination.
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. Compare 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. Heart rate may be rapid and blood pressure may be low to average.
Priapism is a prolonged erection that can occur in men who have SCD. The cause is excessive vascular engorgement in erectile tissue. Urinary retention!
Skin changes include pallor or cyanosis because of poor GAS EXCHANGE from decreased PERFUSION. Examine the lips, tongue, nail beds, conjunctivae, palms, and soles of the feet for color. With cyanosis, the lips and tongue are gray and the palms, soles, conjunctivae, and nail beds have a bluish tinge. 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. Jaundice equals itching. Inspect the legs and feet for ulcers or darkened areas that may indicate necrotic tissue.
Abdominal changes include damage to the spleen and liver. In crisis, abdominal pain is diffuse and steady, involving the back and legs. The liver or spleen may feel firm and enlarged with a nodular or "lumpy" texture in late stage.
Kidney and urinary changes are common. Chronic kidney disease occurs as a result of anoxic damage to the kidney nephrons. The urine contains protein, and the patient may not concentrate urine. Eventually, the kidneys fail, resulting in little or no urine.
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.
Central nervous system (CNS) changes may occur. With crises, patients may have a low-grade fever. If the CNS has infarcts or repeated episodes of hypoxia, patients may have seizures or manifestations of a stroke. Assess for the presence of "pronator drift," bilateral hand grasp strength, gait, and coordination.
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, is 5% to 50% among people with AS, and may be 90% among patients with SCD.
The hematocrit of patients with SCD is low (between 20% and 30%) because of RBC shortened life span/destruction.
This value decreases even more during crises or during 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.
The hematocrit of patients with SCD is low (between 20% and 30%) because of RBC shortened life span/destruction.
This value decreases even more during crises or during 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.
The skull may show changes on x-ray as a result of bone surface cell destruction and new growth, giving the skull a "crew cut" appearance on x-ray. X-rays of joints may show necrosis and destruction. Ultrasonography, CT, positron emission tomography (PET), and MRI may show soft-tissue and organ changes from poor PERFUSION and chronic inflammation.
OTHER: Electrocardiographic (ECG) changes document cardiac infarcts and tissue damage. Specific ECG changes are related to the area of the heart damaged. Echocardiograms may show cardiomyopathy and decreased cardiac output (low ejection fraction).
OTHER: Electrocardiographic (ECG) changes document cardiac infarcts and tissue damage. Specific ECG changes are related to the area of the heart damaged. Echocardiograms may show cardiomyopathy and decreased cardiac output (low ejection fraction).
The pain with sickle cell crisis is the result of tissue injury. 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 opioids.
Opioid addiction is rare, occurring in only 2% to 5% of patients with SCD. 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. Morphine and hydromorphone (Dilaudid) are given IV on a routine or PCA. Avoid "as needed" (PRN) schedules because they do not provide adequate relief. Moderate pain may be managed with oral opioids/NSAIDs.
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 . This drug is associated with an increased incidence of leukemia. Hydroxyurea also suppresses bone marrow function including IMMUNITY, and require follow-up to monitor CBCs.
Hydration by the oral or IV route helps reduce the duration of pain episodes. 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 down to the normal range of 270 to 300 mOsm, the IV rate is reduced to 125 mL/hr if more needed.
Opioid addiction is rare, occurring in only 2% to 5% of patients with SCD. 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. Morphine and hydromorphone (Dilaudid) are given IV on a routine or PCA. Avoid "as needed" (PRN) schedules because they do not provide adequate relief. Moderate pain may be managed with oral opioids/NSAIDs.
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 . This drug is associated with an increased incidence of leukemia. Hydroxyurea also suppresses bone marrow function including IMMUNITY, and require follow-up to monitor CBCs.
Hydration by the oral or IV route helps reduce the duration of pain episodes. 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 down to the normal range of 270 to 300 mOsm, the IV rate is reduced to 125 mL/hr if more needed.
Action AlertHydroxyurea is teratogenic (can cause birth defects). Teach sexually active women of childbearing age using this drug to adhere to strict contraceptive measures while taking hydroxyurea and for 1 month after the drug is discontinued.Chart 40-2
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).
• Avoid taking blood pressure with external cuff.
• Check circulation in extremities every hour:
▪ Pulse oximetry of fingers and toes
▪ Capillary refill
▪ Peripheral pulses
▪ Toe temperatureSickle Cell Disease: Preventing Sepsis, Multiple Organ Dysfunction, and Death 1SCD is at greater risk for bacterial INFECTION because of decreased spleen function. The patient with a fever should have diagnostic testing for sepsis including CBC with differential, blood cultures, reticulocyte count, urine culture, and a chest x-ray. Usually these patients are started on prophylactic antibiotics.
Handwashing is of the utmost importance. Any person with an upper respiratory tract infection who enters the patient's room must wear a mask. Use strict aseptic technique for all invasive procedures.
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. Take vital signs at least every 4 hours to assess for fever.
Drug therapy by prophylaxis with twice-daily oral penicillin reduces the number of pneumonia and other streptococcal. Yearly influenza vaccinations and to receive the pneumonia vaccine. Drug therapy for an actual INFECTION depends on the sensitivity of the specific organism.Sickle Cell Disease: Preventing Sepsis, Multiple Organ Dysfunction, and Death 2Continued 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. Preventing heart and lung damage is a priority.
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. 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.
Transfusion with RBCs can be helpful to increase HbA levels and dilute HbS levels, although they must be prescribed cautiously to prevent iron overload.
Hematopoietic stem cell transplantation (HSCT) is performed to correct abnormal hemoglobin permanently. Because HSCT is expensive and may result in life-threatening complications, its risks and benefits need to be considered.Sickle Cell Disease: Community-Based CareTeach the patient to avoid specific activities that lead to hypoxia and hypoxemia. Stress the recognition of the early manifestations of crisis so that interventions can be started early.
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. The use of hormone-based contraceptives is controversial, because these drugs may increase clot formation, especially among smokers, predisposing them to crises. Urge women using hormone-based contraceptives to not smoke.Chart 40-3
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 health care provider at the first sign of illness or infection.
• Be sure to get a "flu shot" every year.
• Ask your 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 like Denver and Santa Fe).
• Ensure that any health care professional who takes care of you knows 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.Glucose-6-Phosphate Dehydrogenase Deficiency AnemiaThe most common type of inherited hemolytic anemia is the deficiency of the enzyme glucose-6-phosphate dehydrogenase (G6PD). This disease is inherited as an X-linked recessive disorder with more severe expression in males.
G6PD stimulates reactions in glucose metabolism important for energy in RBCs because they contain no other way to produce adenosine triphosphate (ATP). Cells with reduced amounts of G6PD break more easily during exposure to some drugs (e.g., sulfonamides, aspirin, quinine derivatives, chloramphenicol, dapsone, high doses of vitamin C, and thiazide diuretics) and exposure to benzene and other toxins.
New RBCs have some G6PD, but the enzyme diminishes as the cells age. The patient usually does not have manifestations until exposed to triggering agents or until a severe INFECTION develops. After exposure to a precipitating cause, acute RBC breakage begins and lasts 7 to 12 days. During this acute phase, anemia and jaundice develop. The hemolytic reaction is limited because only older RBCs, containing less G6PD, are destroyed.Glucose-6-Phosphate Dehydrogenase Deficiency Anemia: Patient-Centered Collaborative CarePrevention is the most important therapeutic measure. Men who belong to the high-risk groups should be tested for this problem before being given drugs.
Hydration is important during an episode of hemolysis to prevent debris and hemoglobin from collecting in the kidney tubules, which can lead to acute kidney injury (AKI). Osmotic diuretics, such as mannitol (Osmitrol), may help prevent this complication. Transfusions are needed when anemia is present and kidney function is normalImmunohemolytic AnemiaThe pathophysiology is abnormal IMMUNITY that results in the excessive destruction of red blood cell membranes (lysis) followed by accelerated erythropoiesis. Acquired hemolytic syndromes result from increased RBC destruction occurring from trauma, viral infection, malaria, exposure to certain chemicals or drugs, and autoimmune reactions.
Immune system products (e.g., antibodies) attack a person's own RBCs for unknown reasons.
Warm antibody anemia occurs with immunoglobulin G (IgG) antibody excess. These antibodies are most active at 98.6° F (37° C) and may be triggered by drugs, chemicals, or other autoimmune problems. Cold antibody anemia has complement protein fixation on immunoglobulin M (IgM) and occurs most at 86° F (30° C). This problem often occurs with a Raynaud's-like response in which the arteries in the hands and feet constrict profoundly in response to cold temperatures or stress.
Management depends on disease severity. Steroid therapy to suppress IMMUNITY is temporarily effective in most patients. Splenectomy and more intense immunosuppressive therapy with chemotherapy drugs may be used if steroid therapy fails. Plasma exchange therapy with antibody removal is effective for patients who do not respond to chemotherapy.Considerations for Older AdultsOlder patients often have restricted diets and may be unable to eat meat because of tooth loss or economic reasons and thus are at risk for iron deficiency anemia. Ask about a family history of anemia. B12 deficiency anemia often occurs in patients 50 to 80 years of age and may result from an inherited genetic mutation.Iron deficiency anemiaIt can result from blood loss, poor GI absorption of iron, and an inadequate diet. The problem is a decreased iron supply for the developing RBC.
Adults usually have between 2 and 6 g of iron, depending. With chronic iron deficiency, RBCs are small (microcytic) and the patient has mild symptoms of anemia, including weakness and pallor. Other manifestations include fatigue, reduced exercise tolerance, and fissures at the corners of the mouth. Serum ferritin values are less than 10 ng/mL (normal range is 12 to 300 ng/mL).
Any adult with iron deficiency should be evaluated for abnormal bleeding, look at GI. Management of iron deficiency anemia involves increasing the oral intake of iron from food sources (e.g., red meat, organ meat, egg yolks, kidney beans, leafy green vegetables, and raisins). If iron losses are mild, oral iron supplements, such as ferrous sulfate, are started until the hemoglobin levels return. Instruct patients to take the iron supplement between meals for better absorption and to reduce GI distress. When iron deficiency anemia is severe, iron solutions (iron dextran [Dexferrum, INFeD, Pri-Dextra]; ferumoxytol [Feraheme]) can be given parenterally.Vitamin B12 deficiency anemiaVitamin B12 deficiency anemia results in failure to activate the enzyme that moves folic acid into precursor RBC cells so that cell division and growth into functional RBCs can occur. This type of anemia is called megaloblastic or macrocytic anemia because of the large size.
Causes of vitamin B12 deficiency include vegan diets or diets lacking dairy products, small bowel resection, chronic diarrhea, diverticula, tapeworm, or overgrowth of intestinal bacteria. Anemia resulting from failure to absorb vitamin B12 (pernicious anemia) is caused by a deficiency of intrinsic factor (a substance normally secreted by the gastric mucosa), which is needed for intestinal absorption of vitamin B12.
Manifestations include pallor and jaundice, glossitis (a smooth, beefy-red tongue) (Fig. 40-3), fatigue, and weight loss. Patients with pernicious anemia may also have paresthesias (abnormal sensations) in the feet and hands and poor balance.
Focus of management is to increase the intake of foods rich in vitamin B12 (animal proteins, fish, eggs, nuts, dairy products, dried beans, citrus fruit, and leafy green vegetables). Vitamin supplements may be prescribed. Patients who have pernicious anemia are given vitamin B12 injections weekly at first and then monthly for the rest of their lives. Oral B12 preparations and nasal spray or sublingual forms of cobalamin may be used to maintain vitamin levels after the patient's deficiency has first been correctedFolic acid deficiency anemiaWith manifestations similar to those of vitamin B12 deficiency. However, nervous system functions remain normal because folic acid deficiency does not affect nerve function.
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. Malabsorption syndromes, such as Crohn's disease, are the second most 805common cause. Anticonvulsants and oral contraceptives can contribute to folic acid deficiency and anemia.
Prevention begins by identifying high-risk patients, such as older, debilitated patients with alcoholism; patients at risk for malnutrition; and those with increased folic acid requirements. A diet rich in foods containing folic acid and vitamin B12 prevents a deficiency. This type of anemia is managed with scheduled folic acid replacement therapy.Aplastic anemiaA deficiency of circulating red blood cells (RBCs) because of failure of the bone marrow to produce these cells. It is caused by an injury to the immature precursor cell for red blood cells. Although aplastic anemia sometimes occurs alone, it usually occurs with leukopenia (a reduction in white blood cells [WBCs]) and thrombocytopenia (a reduction in platelets), a condition known as pancytopenia.
The most common type of the disease is caused by long-term exposure to toxic agents, drugs, ionizing radiation, infection, or idiopathic.
Common hereditary form of the disease is Fanconi's anemia.
The patient has manifestations of severe anemia. A complete blood count (CBC) shows severe macrocytic anemia, leukopenia, and thrombocytopenia. A bone marrow biopsy may show replacement of cell-forming marrow with fat. Infection is common.
Blood transfusions are used only when the anemia causes disability or when bleeding is life threatening. Unnecessary transfusion increases the chances for developing immune reactions to platelets. Discontinued as soon as the bone marrow begins to produce RBCs.
Hematopoietic stem cell transplantation with donor cells is the most successful method of treatment for aplastic anemia that does not respond to other therapies.
Immunosuppressive therapy helps patients who have the types of aplastic anemia with a disease course similar to that of autoimmune problems. Drugs such as prednisone, antithymocyte globulin (ATG), and cyclosporine A (Sandimmune) have resulted in partial or complete remissions. For moderate aplastic anemia, daclizumab (Zenapax) has improved both blood counts and transfusion requirements. Splenectomy may be needed for patients with an enlarged spleen that is either destroying normal RBCs or suppressing their development.Polycythemia VeraIn polycythemia, the number of red blood cells (RBCs) in the blood is greater than normal. The blood of a patient with polycythemia is hyperviscous (thicker than normal blood).
Polycythemia vera (PV) is a disease with a sustained increase in blood hemoglobin levels to 18 g/dL, an RBC count of 6 million/mm3, or a hematocrit of 55% or greater. PV is a cancer of the RBCs with three major hallmarks: massive production of RBCs, excessive leukocyte production, and excessive production of platelets. More than 90% of patients with PV show a mutation of the JAK2 kinase gene. Extreme hypercellularity (cell excess) of the peripheral blood occurs.
Facial skin and mucous membranes have a dark, purple or cyanotic, flushed (plethoric) appearance with distended veins. Intense itching caused by dilated blood vessels, and poor PERFUSION. Thick blood moves more slowly and places increased demands on the heart, resulting in hypertension. Vascular stasis causes thrombosis (CLOTTING) within the smaller vessels, occluding them, which leads to tissue hypoxia, anoxia and, later, to infarction and necrosis. Tissues most at risk for this problem are the heart, spleen, and kidneys.
Cell life spans are shorter. The shorter life spans and increased cell production cause a rapid turnover of circulating blood cells. This rapid turnover increases the amount of cell debris (released when cells die) in the blood, adding to the general "sludging" of the blood. This debris includes uric acid and potassium, which cause the manifestations of gout and hyperkalemia (elevated serum potassium level).
Oxygen-carrying capacity is impaired from abnormal RBCs, and patients have poor GAS EXCHANGE with severe hypoxia. Bleeding problems are common because of platelet impairment.Polycythemia Vera: Patient-Centered Collaborative CareIf left untreated, few people with PV live longer than 2 years after diagnosis. With management by repeated phlebotomy with apheresis (2 to 5 times per week), the patient may live 10 to 15 years or longer. (Apheresis is the withdrawal of whole blood and removal of some of the patient's blood component, in this case RBCs. The plasma is then reinfused back into the patient.) Increasing hydration and promoting venous return help prevent clot formation. Therapy for PV also includes the use of anticoagulants.
Aggressive IV chemotherapy is no longer recommended because of its increased risk for inducing leukemia. Aspirin therapy may be used to decrease clot formation but increases the risk for GI bleeding. Hydroxyurea, an oral chemotherapy drug, may be prescribed for severe manifestations of the disease. Interferon-alfa therapy has also shown some benefit in controlling RBC production.Chart 40-4
Polycythemia Vera• Drink at least 3 liters of liquids each day.
• Avoid tight or constrictive clothing, especially garters and girdles.
• Wear gloves when outdoors in temperatures lower than 50° F (10° C).
• Keep all health care-related appointments.
• Contact your health care provider at the first sign of infection.
• Take anticoagulants as prescribed.
• Wear support hose or stockings while you are awake and up.
• Elevate your feet whenever you are seated.
• Exercise slowly and only on the advice of your physician.
• Stop activity at the first sign of chest pain.
• Use an electric shaver.
• Use a soft-bristled toothbrush to brush your teeth.
• Do not floss between your teeth.Hereditary HemochromatosisHereditary hemochromatosis is an autosomal recessive disorder in which a mutation in both alleles of the HFE gene cause increased intestinal absorption of dietary iron. Excess iron is deposited in a variety of tissues and organs, including the liver, spleen, heart, joints, skin, and pancreas. The iron deposits can damage the organs. More common in men and manifestations appear in men during their 40s.
Clinical manifestations are abdominal pain, liver enlargement, hyperglycemia, and a gradual darkening of the skin. Later problems include diabetes, liver cirrhosis, endocrine gland failure, heart disease, and death.
Diagnosed on the basis of clinical manifestations and altered iron levels.
Phlebotomy and removal of 500 mL of blood at a time, occurring as often as twice weekly at first, is performed to reduce the overall iron load of the blood. The desired outcome is to reduce blood ferritin levels to less than 9 to 50 micrograms per liter. Once this level has been achieved, phlebotomy frequency can be reduced to once every 2 to 4 months for maintenance.Myelodysplastic SyndromesMyelodysplastic syndromes (MDS) are a group of disorders caused by the formation of abnormal cells in the bone marrow. These abnormal cells are usually destroyed shortly after they are released into the blood. As a result, patients with MDS have a decrease in all blood cell types. Anemia is the most common problem with MDS, although neutropenia (low white blood cell count [WBC]) and thrombocytopenia (low platelets) are also often present. MDS has cancer-like features and is considered to be a precancerous state. About 30% of all patients with MDS do eventually develop acute leukemia.
Patients are categorized into risk groups (i.e., low, intermediate [1 and 2], high) based on the severity of pancytopenia (low counts of all blood cell types), cytogenetic abnormalities, and numbers of blast cells (immature WBC cells).
Risk factors include normal physiologic changes associated with aging, chemical exposures (pesticides, benzene), tobacco smoke, and exposure to radiation or chemotherapy drugs. Diagnosis is made by examination of the chromosomes and the genes within the chromosomes (cytogenetic testing) of the bone marrow cells. Peripheral blood smears are used to assess the level of cell maturation and the proportion of abnormal cells.Myelodysplastic Syndromes: Patient-Centered Collaborative CarePotentially curative treatment for MDS is an allogeneic hematopoietic stem cell transplantation.
For low-risk and intermediate-1-risk MDS, the antitumor immunomodulatory agent lenalidomide (Revlimid) is approved for patients whose dysplastic cells have the chromosome abnormality of a deleted 5q. Two other agents approved for intermediate-2-risk and high-risk MDS are azacitidine (Vidaza) and decitabine (Dacogen) (Kurtin, 2012). These drugs often require at least 3 to 6 months to achieve a clinical response; therefore supportive care is necessary. Supportive care includes blood transfusions for anemia and platelet transfusions when platelet levels are very low. Erythropoiesis-stimulating agents (ESAs), such as epoetin alfa (Epogen, Procrit) or darbepoetin alfa (Aranesp), may be given in addition to transfusions.LeukemiaLeukemia is cancer with uncontrolled production of immature WBCs ("blast" cells) in the bone marrow. As a result, the bone marrow becomes overcrowded with immature, nonfunctional cells. Leukemia may be acute, with a sudden onset, or chronic.
Leukemic cells coming from the lymphoid pathways are typed as lymphocytic or lymphoblastic. Leukemic cells coming from the myeloid pathways are typed as myelocytic or myelogenous.
M3 is a subtype (referred to as acute promyelocytic leukemia [APL]) that has a specific treatment different. Biphenotypic leukemia is acute leukemia that shows both lymphocytic and myelocytic features.
Cancer most often occurs in the stem cells or early precursor leukocyte cells, causing excessive growth of a specific type of immature leukocyte.
Bone marrow stops normal bone marrow production, leading to anemia, thrombocytopenia, and leukopenia. Without treatment, the patient will die of INFECTION or hemorrhage. For patients with acute leukemia, these changes occur rapidly and, without intervention, progress to death.Leukemia: Etiology and Genetic RiskPossible risk factors for the development of leukemia include ionizing radiation, viral infection, exposure to chemicals and drugs, disorders such as myelodysplastic syndrome or Fanconi's anemia, genetic factors, immunologic factors, environmental factors, and combination.
Radiation therapy for cancer treatment or heavy accidental exposures increase the risk for leukemia development, particularly acute myelogenous leukemia (AML).
Immune deficiencies may promote the development of leukemia. Chronic lymphocytic leukemia appears to have a familial or genetic predisposition.TABLE 40-2
Classification of Leukemia TypesPG 807 IGGYLeukemia: HistoryAge is important because the risk for adult-onset leukemia increases with age. Occupation and hobbies may reveal exposure to agents that increase the risk for leukemia. Previous illnesses and the medical history may reveal exposure to ionizing radiation or drugs.
Changes in IMMUNITY increase the risk for INFECTION in the patient with leukemia. Even when the blood count shows a normal or high level of WBCs, these cells are immature and cannot protect the patient.
Platelet function is reduced with leukemia, interfering with CLOTTING. Ask about any excessive bleeding episodes, such as:
• A tendency to bruise easily or longer after minor trauma
• Nosebleeds
• Increased menstrual flow
• Bleeding from the gums
• Rectal bleeding
• Hematuria (blood in the urine)
The patient with leukemia often has weakness and fatigue from anemia and from the increased metabolism of the leukemic cells. Ask whether any of these problems have occurred:
• Headaches
• Behavior changes
• Increased somnolence; decreased alertness; fatigue
• Decreased attention span
• Muscle weakness
• Loss of appetite
• Weight loss
A 24-hour activity history may reveal activity intolerance, changes in behavior, and unexplained fatigue.Chart 40-5
Key Features
Acute LeukemiaPG 808 IGGYLeukemia: Physical Assessment/Clinical ManifestationsCardiovascular changes often are related to adjustments needed when PERFUSION and GAS EXCHANGE are reduced. The heart rate is increased, and blood pressure is decreased. Murmurs (abnormal blood flow sounds in the heart) and bruits (abnormal blood flow sounds over arteries) may be heard. Capillary refill is slow. When the WBC count is greatly elevated and blood is highly viscous, blood pressure is elevated with a bounding pulse.
Respiratory changes are related to reduced GAS EXCHANGE from anemia and to INFECTION. Respiratory rate increases as anemia becomes more severe. If a respiratory infection is present, the patient may have coughing and dyspnea. Abnormal breath sounds are heard.
Skin changes include pallor and coolness to the touch as a result of reduced PERFUSION from anemia. Pallor is most evident on the face, around the mouth, and in the nail beds. The conjunctiva of the eye also is pale, as are the creases on the palm. Petechiae may be present on any area of skin surface, especially the legs and feet. Inspect for skin infections or injured areas that have failed to heal. Inspect the mouth for gum bleeding and any sore or lesion.
Intestinal changes may be related to an increased bleeding tendency and to fatigue. Weight loss, nausea, and anorexia are common. Examine the rectal area for fissures, and test stool for occult blood. Many patients with leukemia have reduced bowel sounds and are constipated because reduced perfusion. Enlargement of the liver and spleen and abdominal tenderness also may be present.
Central nervous system (CNS) changes include cranial nerve problems, headache, and papilledema from leukemic invasion of the CNS. Seizures and coma also may occur.
Miscellaneous changes can include bone and joint tenderness as the marrow is damaged and the bone reabsorbs. Leukemic cells invade lymph nodes, causing enlargement.Leukemia: Laboratory AssessmentThe patient with acute leukemia usually has decreased hemoglobin and hematocrit levels, a low platelet count, and an abnormal white blood cell (WBC) count. The WBC count may be low, normal, or elevated.
The definitive test for leukemia is an examination of cells obtained from bone marrow aspiration and biopsy. The bone marrow is full of leukemic blast phase cells (immature cells that are dividing). The proteins (antigens) on the surfaces of the leukemic cells are "markers" that help diagnose the type of leukemia and may indicate prognosis. These include the T11 protein, terminal deoxynucleotidyl transferase (TDT), the common acute lymphoblastic leukemia antigen (CALLA), and the CD33 antigen.
Blood CLOTTING times and factors are usually abnormal with acute leukemia. Reduced levels of fibrinogen and other clotting factors are common. Whole-blood clotting time (Lee-White clotting test) is prolonged, as is the activated partial thromboplastin time (aPTT).
Chromosome analysis (cytogenetic studies) of the leukemic cells may identify marker chromosomes to help diagnose the type of leukemia.Leukemia: Imaging AssessmentSpecific manifestations determine the need for specific tests. In a patient with dyspnea, a chest x-ray is needed to determine whether leukemic infiltrates are present in the lung. Skeletal x-rays may help determine whether loss of bone minerals and bone density is present.Leukemia: Preventing Infection-InterventionsINFECTION is a major cause of death in the patient with leukemia. Sepsis is a common complication. Infection occurs through both auto-contamination (normal flora overgrows and penetrates the internal environment) and cross-contamination (organisms from another person or the environment are transmitted to the patient).
Gram-negative bacteria are the most common cause.Chart 40-6
Focused Assessment
Patients at Risk for InfectionGeneral Condition
• Age
• History of allergies
• History of chemotherapy, radiation therapy, or other immunosuppressive therapies, such as steroid use
• Chronic diseases
• History of febrile neutropenia and associated symptoms
• Nutrition status
• Functional status—problems with immobility
• Tobacco use—cigarettes, pipe, cigars, oral
• Recreational drug use
• Alcohol use
• Prescribed and over-the-counter drug use
• Baseline and ongoing vital signs—blood pressure, heart rate, respiratory rate, and temperature
Skin and Mucous Membranes
• Thorough inspection of all skin surfaces with attention to axillae, anorectal area, and under breasts; inspection of skin for color, vascularity, bleeding, lesions, edema, moist areas, excoriation, irritation, erythema; general condition of hair and nails, pressure areas, swelling, pain, tenderness, biopsy or surgical sites, wounds, enlarged lymph nodes, catheters, or other devices
• Inspection of oral cavity, including lips, tongue, mucous membranes, gingiva, teeth, and throat—color, moisture, bleeding, ulcerations, lesions, exudate, mucositis, stomatitis, plaque, swelling, pain, tenderness, taste changes, amount and character of saliva, ability to swallow, changes in voice, dental caries, patient's oral hygiene routine
• History of current skin or mucous membrane problems
Head, Eyes, Ears, Nose
• Pain, tenderness, exudate, crusting, enlarged lymph nodes
Cardiopulmonary
• Respiratory rate and pattern, breath sounds (presence/absence, adventitious sounds), quantity and characteristics of sputum, shortness of breath, use of accessory muscles, dysphagia, diminished gag reflex, tachycardia, blood pressure
Gastrointestinal
• Pain, diarrhea, bowel sounds, character and frequency of bowel movements, constipation, rectal bleeding, hemorrhoids, change in bowel habits, sexual practices, erythema, ulceration
Genitourinary
• Dysuria, frequency, urgency, hematuria, pruritus, pain, vaginal or penile discharge, vaginal bleeding, burning, lesions, ulcerations, characteristics of urine
Central Nervous System
• Cognition, level of consciousness, personality, behavior
Musculoskeletal
• Tenderness, pain, loss of functionLeukemia: Drug Therapy for Acute LeukemiaInduction therapy is intense and consists of combination chemotherapy started at the time of diagnosis. The purpose of this therapy is to achieve a rapid, complete remission of all manifestations of disease. One example of aggressive induction therapy is continuous IV cytosine arabinoside for 7 days together with an anthracycline for the first 3 days, sometimes referred to as a "7 plus 3" regimen. This therapy results in severe bone marrow suppression with neutropenia. For acute promyelocytic leukemia, the agent tretinoin (Vesanoid) is added.
Recovery of bone marrow function requires at least 2 to 3 weeks, during which the patient must be protected from life-threatening INFECTIONS. Other side effects of drugs used for induction therapy include nausea, vomiting, diarrhea, alopecia (hair loss), stomatitis (mouth sores), kidney toxicity, liver toxicity, and cardiac toxicity.
Consolidation therapy consists of another course of either the same drugs used for induction at a different dosage or a different combination. This treatment occurs early in remission, and its intent is to cure.
Maintenance therapy may be prescribed for months to years after successful induction and consolidation therapies for acute lymphocytic leukemia (ALL) and acute promyelocytic leukemia (APL). The purpose is to maintain the remission achieved through induction and consolidation.Action AlertAPL places patient at greater risk for sepsis with disseminated intravascular coagulation (DIC) during induction therapy than are patients with other subtypes of AML.Leukemia: Drug Therapy for Chronic LeukemiaImatinib mesylate (Gleevec) is a common first-line drug therapy for CML that is Philadelphia chromosome-positive. Other drugs approved for first-line therapy or for patients whose disease is resistant or intolerant to imatinib are dasatinib (Sprycel) or nilotinib (Tasigna). Other drugs used to treat CML include interferon-alfa, which reduces the growth of leukemic cells, but its use is limited because of side effects, such as flu-like manifestations and fevers.
Chronic lymphocytic leukemia (CLL) is the most prevalent form of leukemia in adults.
Treatment of CLL with standard chemotherapy can cause remissions but does not cure the disease.
Rituximab (Rituxan) is often combined with standard chemotherapy drugs or used as a single agent. Another drug approved for CLL is bendamustine (Treanda), which may be used alone or along with rituximab. Other monoclonal antibodies approved for CLL are ofatumumab (Arzerra) and alemtuzumab (Campath). Current investigational therapies for CLL include ibrutinib, oblimersen (Genasense), flavopiridol (Alvocidib), and lenalidomide (Revlimid).
Hematopoietic stem cell transplantation in patients with CLL is an option that offers curative potential or prolonged disease-free survival.Leukemia: Drug Therapy for InfectionDrug therapy is the main defense against INFECTIONS that develop in patients undergoing therapy for AML. Drugs used depend on the sensitivity of the organism causing the infection, as well as infection severity.Leukemia: Infection ProtectionAll personnel must use extreme care during all nursing procedures. Frequent, thorough handwashing is of the utmost importance. Anyone with an upper respiratory tract infection who enters the patient's room must wear a mask. Observe strict asepsis.
Ensure that the patient is in a private room to reduce cross-contamination. Do not allow standing water in vases, denture cups, or humidifiers in the patient's room, because they are breed grounds.
Some facilities place a high-efficiency particulate air (HEPA) filtration or laminar airflow system.
The patient with leukopenia may have a severe infection without pus and with only a low-grade fever.
Monitor the patient's daily CBC with differential WBC count and absolute neutrophil count (ANC). Inspect the mouth during every shift. Assess the lungs every 8 hours for crackles, wheezes, and reduced breath sounds. Assess urine for odor and cloudiness. Ask about any urgency, burning, or pain. Assess VS for fever.
Obtain blood for bacterial and fungal cultures from IV sites. After the specimens are obtained, the patient begins IV antibiotics.
Skin care is important for preventing INFECTION in the patient with leukemia because the skin may be the only intact defense. Teach him or her about hygiene, and urge daily bathing. If the patient is immobile, turn him or her every hour and apply skin lubricants.
Perform pulmonary hygiene every 2 to 4 hours. Listen to the lungs for crackles, wheezes, and reduced breath sounds. Urge the patient to cough and deep breathe or to perform sustained maximal inhalations every hour.Critical RescueA temperature elevation of even 1° F (or 0.5° C) above baseline is significant for a patient with leukopenia and indicates INFECTION until it has been proven otherwise. Report this finding to the health care provider at once.Leukemia: Hematopoietic Stem Cell TransplantationHematopoietic stem cell transplantation (HSCT), sometimes called bone marrow transplantation (BMT), is standard treatment for the patient with leukemia who has a closely matched donor and who is in temporary remission after induction therapy. It is used also for lymphoma, multiple myeloma, aplastic anemia, sickle cell disease.
Before an HSCT, additional chemotherapy with or without total body irradiation is given to purge (condition or clean) the marrow of leukemic cells. These treatments are lethal to the bone marrow, and without replacement of stem cells by transplantation, the patient would die of INFECTION or hemorrhage.
After conditioning, new healthy stem cells are given to the patient. The new cells go to the marrow and then begin the process of hematopoiesis.
HSCT started with the use of allogeneic bone marrow transplantation (transplantation of bone marrow from a sibling or matched unrelated donor) and has advanced to the use of human leukocyte antigen (HLA)-matched stem cells from the umbilical cords of non-relatives.
Stem cells for transplantation may be obtained by bone marrow harvest, peripheral stem cell apheresis, or umbilical cord blood stem cell banking. Transplantation has five phases: stem cell obtainment, conditioning regimen, transplantation, engraftment, and post-transplantation recovery.TABLE 40-3
Classification of TransplantsPG 811 IGGYLeukemia: Obtaining the Stem CellsTaken either from the patient directly (autologous stem cells), an HLA-identical twin (syngeneic stem cells), or from an HLA-matched person (allogeneic stem cells).
Bone marrow harvesting occurs after a suitable donor is identified. Marrow is removed through multiple aspirations from the iliac crests. About 500 to 1000 mL of marrow is aspirated, and the donor's marrow regrows. The marrow is then filtered and, if autologous, is treated to rid the marrow of any remaining cancer cells. Allogeneic marrow is transfused into the recipient immediately. Autologous marrow is frozen for later use.
Monitor the donor for fluid loss, assess for complications of anesthesia, and manage pain. Donors are hydrated with saline infusions before and immediately after. Assess the harvest site/dressing.
Peripheral blood stem cell (PBSC) harvesting requires three phases: mobilization, collection by apheresis, and reinfusion. PBSCs are stem cells that have been released from the bone marrow and circulate within the blood. During the mobilization phase, chemotherapy or hematopoietic growth factors are given to the patient for an autologous collection, and hematopoietic growth factors alone are given to the donor for an allogeneic or syngeneic collection. These agents increase the numbers of stem cells and WBCs in the peripheral blood. Drug used is plerixafor (Mozobil).
Stem cells are then collected by apheresis (withdrawing whole blood, filtering out the cells, and returning the plasma to the patient).
During apheresis, complications include catheter clotting and hypocalcemia (caused by anticoagulants). Low calcium levels may cause numbness or tingling in the fingers and toes, abdominal or muscle cramping, or chest pain.
Cord blood harvesting involves obtaining stem cells from umbilical cord blood of newborns.Leukemia: Conditioning RegimenThe conditioning regimen serves two purposes: (1) to "wipe out" the patient's own bone marrow, thus preparing him or her for optimal graft take; and (2) to give higher-than-normal doses of chemotherapy and/or radiotherapy to rid the person of cancer cells (myeloablation). Regimen usually includes high-dose chemotherapy and, less commonly, total-body irradiation (TBI).
A non-myeloablative approach may be used instead. Non-myeloablative regimens use lower doses of chemotherapy and/or lower dose of TBI that allow for recovery of a recipient's own immune system. The use of non-myeloablative conditioning regimens decreases the chemotherapy side effects but relies on the development of graft-versus-host disease (GVHD) for the control of the cancer.
Myeloablative conditioning regimens use high doses of chemotherapy with or without radiation therapy to completely destroy a recipient's bone marrow, allowing for replacement by a new immune system.
During conditioning, bone marrow and normal tissues respond immediately to the chemotherapy and radiation. The patient has all of the expected side effects associated with both therapies. When chemotherapy is given in high doses, these side effects are more intense than those seen with standard doses.
Late effects from the conditioning regimen may occur as late as 3 to 10 years after transplantation. These problems include veno-occlusive disease (VOD), skin toxicities, cataracts, lung fibrosis, second cancers, cardiomyopathy, endocrine complications, and neurologic complications.Leukemia: TransplantationFrozen marrow, PBSCs, or umbilical cord blood cells are thawed and then infused through the patient's central catheter like an ordinary blood transfusion. The patient may have fever and hypertension in response to the preservative used in stem cell storage. To prevent these reactions, acetaminophen (Tylenol), hydrocortisone, and diphenhydramine (Benadryl) are given before the infusion. Antihypertensives or diuretics may be needed to treat fluid volume changes.Action AlertDo not use blood administration tubing to infuse stems cells because the cells could get caught in the filter, resulting in the patient receiving fewer stem cells.Leukemia: EngraftmentTransfused PBSCs and marrow cells circulate briefly in the peripheral blood. The stems cells find their way to the marrow-forming sites of the patient's bones and establish residency.
Engraftment, the successful "take" of the transplanted cells in the patient's bone marrow, is key to the whole transplantation. Stem cells must survive and grow in the patient's bone marrow sites.
To aid engraftment, growth factors, such as granulocyte colony-stimulating factor or granulocyte-macrophage colony-stimulating factor, may be given. When engraftment occurs, the patient's WBC, RBC, and platelet counts begin to rise. Engraftment syndrome (ES) with fever and weight gain may occur.
Monitoring of engraftment involves checking the patient's blood for "chimerism," which is the presence of blood cells that show a different genetic profile or marker from those of the patient. Mixed chimerism is the presence of both the patient's cells and those from the donor. Progressive chimerism with increasing percentages of donor cells indicates engraftment. Regressive chimerism with increasing percentages of the patient's cells indicates graft failure. When engraftment is successful, only the donor's cells are present.Leukemia: Prevention of ComplicationsINFECTION and poor CLOTTING with bleeding are severe problems because recovery period. Care for this patient is the same as for the patient during induction therapy.
In addition to the problems related to the period of pancytopenia (too few circulating blood cells), other complications of HSCT include failure to engraft, development of graft-versus-host disease (GVHD), and veno-occlusive disease (VOD).
Failure to engraft occurs when the donated stem cells fail to grow in the bone marrow and function properly. The causes include too few cells transplanted, attack or rejection of donor cells by the recipient's remaining immune system cells, infection of transplanted cells, and unknown biologic factors. If the transplanted cells fail to engraft, the patient will die unless another transplant with stem cells is successful.
Graft-versus-host disease (GVHD) is when immunocompetent cells of the donated marrow recognize the patient's (recipient) cells, tissues, and organs as foreign and start an immunologic attack against them. The graft is actually trying to attack the host tissues and cells. The tissues usually damaged are the skin, eyes, intestinal tract, liver, female genitalia, lungs, immune system, and musculoskeletal system. Fig. 40-6 shows the typical skin appearance of GVHD. GVHD indicates successful engraftment.
Management of GVHD involves limiting the activity of donor T-cells by using drugs to suppress IMMUNITY such as cyclosporine, tacrolimus, methotrexate, corticosteroids, mycophenolate mofetil (Cellcept, MMF), and antithymocyte globulin (ATG).
Veno-occlusive disease (VOD) is the blockage of liver blood vessels by CLOTTING and inflammation (phlebitis). Patients who received high-dose chemotherapy, especially with alkylating agents, are at risk for life-threatening liver complications. Manifestations include jaundice, pain in the right upper quadrant, ascites, weight gain, and liver enlargement.
Because there is no way of opening the liver vessels, treatment is supportive. Early detection improves the chance for survival. Fluid management is also crucial. Assess the patient 814daily for weight gain, fluid retention, increases in abdominal girth, and hepatomegaly.Leukemia: Minimizing Injury-InterventionsDuring the period of greatest bone marrow suppression (the nadir), the platelet count may be less than 10,000/mm3. The patient is at extreme risk for bleeding once the platelet count falls below 50,000/mm3, and spontaneous bleeding may occur when the count is lower than 20,000/mm3.
Bleeding Precautions are used to protect the patient at increased risk for injury from bleeding (Chart 40-7). Assess at least every 4 hours for evidence of bleeding: oozing, enlarging bruises, petechiae, or purpura. Inspect all stools, urine, drainage, and vomit for blood, and test for occult blood. Measure any blood loss as accurately as possible, and measure the abdominal girth daily. Increases in abdominal girth can indicate internal hemorrhage.Chart 40-7
The Patient with Thrombocytopenia• Handle the patient gently.
• Use a lift sheet when moving and positioning in bed.
• Avoid IM injections and venipunctures.
• When injections or venipunctures are necessary, use the smallest-gauge needle for the task.
• Apply firm pressure to the needle stick site for 10 minutes or until the site no longer oozes blood.
• Apply ice to areas of trauma.
• Test all urine and stool for the presence of occult blood.
• Observe IV sites every 2 hours for bleeding.
• Avoid trauma to rectal tissues:
▪ Do not give enemas.
▪ Administer well-lubricated suppositories with caution.
▪ Advise patient not to have anal intercourse.
• Measure abdominal girth daily.
• Advise the patient to use an electric shaver.
• Teach the patient to avoid mouth trauma:
▪ Use soft-bristled toothbrush or tooth sponges.
▪ Do not floss between teeth.
▪ Avoid dental work, especially extractions.
▪ Avoid hard foods.
▪ Make sure that dentures fit and do not rub.
• Encourage the patient not to blow the nose or insert objects into the nose.
• Advise the patient to avoid contact sports.
• Teach the patient to wear shoes with firm soles when ambulating.Leukemia: Conserving Energy-InterventionsNutrition therapy is needed to assist the patient to eat enough calories to meet at least basal energy requirements. Provide small, frequent meals high in protein and carbohydrates.
Blood transfusions are sometimes indicated for the patient with fatigue. Transfusions with packed RBCs increase the blood's oxygen-carrying capacity and replace missing RBCs.
Drug therapy with colony-stimulating growth factors may reduce the severity and duration of anemia and neutropenia after intensive chemotherapy. For anemia, erythropoiesis-stimulating agents (ESAs) that boost production of RBCs may be used. These agents now carry a warning for causing hypertension and increasing the risk for myocardial infarction. Not used unless the hemoglobin level is lower than 10 mg/dL and are stopped when this level is reached. Assess for side effects such as hypertension, headaches, fever, myalgia (muscle aches), and rashes.
Activity management helps conserve the patient's energy (Chart 40-8).Chart 40-8
Conserving Energy• Reassure the patient that fatigue is temporary and energy levels will improve over a period of weeks to months. Stress that a return to previous energy levels may take as long as a year.
• Teach the patient that shortness of breath and palpitations are symptoms of over-activity.
• Instruct the patient to stop activity when shortness of breath or palpitations are present.
• Space care activities at least an hour apart, and avoid the time right before or right after meals.
• Schedule care activities at times when the patient has more energy (e.g., immediately after naps).
• Perform complete bed bath only every other day. In between complete baths, ensure cleansing of face, hands, axillae, and perineum.
• In collaboration with other members of the health care team, cancel or reschedule non-essential tests and activities.
• Provide four to six small, easy-to-eat meals instead of three larger ones.
• Urge the patient to drink small amounts of protein shakes or other nutritional supplements.
• During periods of extreme fatigue, encourage the patient to allow others to perform personal care.
• Help the patient identify one or two lead visitors (those designated as allowed to visit at any time and who do not disturb the patient).
• Selectively limit non-lead visitors when the patient is resting or sleeping.
• Remind families that, although independence is important, independence in ADLs during extreme fatigue can be detrimental to the patient's health.
• Monitor oxygen saturation and respiratory rate during any activity to determine patient responses and activity tolerance.Leukemia: Self-Management EducationChart 40-9 lists guidelines for central venous catheter care at home.
Teach about proper hygiene and the need to avoid crowds or others with infections. Neither the patient nor any household member should receive live virus immunization (poliomyelitis, measles, or rubella) for 2 years after transplantation. Stress to the patient that he or she should immediately notify the physician if a fever or any other indications of infection develop. Chart 40-10 lists guidelines for infection.
Reinforce safety and bleeding precautions, and emphasize that these precautions must be followed until the platelet count remains above 50,000/mm3. Teach the patient and family to assess for petechiae, avoid trauma and sharp objects, apply pressure to wounds for 10 minutes, and report blood in the stool or urine or headache that does not respond to acetaminophen. Chart 40-11 lists guidelines for patients at risk for bleeding.Chart 40-9
Home Care of the Central Venous Catheter• To maintain patency, flush the catheter briskly with saline once a day and after completing infusions.
• Change the Luer-Lok cap on each catheter lumen weekly.
• Change the dressing as often as prescribed:
▪ Use clean technique with thorough handwashing.
▪ Clean the exit site with alcohol and povidone-iodine (Betadine) or with chlorhexidine.
▪ Apply antibacterial ointment to the site, if prescribed.
▪ Cover the site with dry sterile gauze dressing, taped securely, or with transparent adherent dressing.
• To prevent tension, always tape the catheter to yourself.
• Look for and report any signs of infection (redness, swelling, or drainage at the exit site).
• In case of a break or puncture in the catheter lumen, immediately clamp the catheter between yourself and the opening. Notify your physician immediately.Chart 40-10
Prevention of Infection• Avoid crowds and other gatherings of people who might be ill.
• Do not share personal toilet articles, such as toothbrushes, toothpaste, washcloths, or deodorant sticks, with others.
• If possible, bathe daily.
• Wash the armpits, groin, genitals, and anal area at least twice a day with an antimicrobial soap.
• Clean your toothbrush daily by either running it through the dishwasher or rinsing it in liquid laundry bleach and then rinsing it with running water.
• Wash your hands thoroughly with an antimicrobial soap before you eat or drink, after touching a pet, after shaking hands with anyone, as soon as you come home from any outing, and after using the toilet.
• Eat a low-bacteria diet, and avoid salads, raw fruits and vegetables, and undercooked meat.
• Wash dishes between uses with hot, sudsy water, or use a dishwasher.
• Do not drink water that has been standing for longer than 15 minutes.
• Do not reuse cups and glasses without washing.
• Avoid changing pet litter boxes. If unavoidable, use gloves or wash hands immediately.
• Avoid keeping turtles and reptiles as pets.
• Do not feed pets raw or undercooked meat.
• Take your temperature at least twice a day.
• Report any of these manifestations of infection to your physician immediately:
▪ Temperature greater than 100° F (38° C)
▪ Persistent cough (with or without sputum)
▪ Pus or foul-smelling drainage from any open skin area or normal body opening
▪ Presence of a boil or abscess
▪ Urine that is cloudy or foul smelling, or burning on urination
• Take all drugs as prescribed.
• Do not dig in the garden or work with houseplants.
• Avoid travel to areas of the world with poor sanitation or inadequate health care facilities.Chart 40-11
The Patient at Risk for Bleeding• Use an electric shaver.
• Use a soft-bristled toothbrush, and do not floss.
• Do not have dental work done without consulting your doctor.
• Do not take aspirin or any aspirin-containing products. Read the label to be sure the products do not contain aspirin or salicylates.
• Wear shoes or slippers with a sole to avoid foot injury.
• Do not participate in contact sports or any activity likely to result in your being bumped, scratched, or scraped.
• If you are bumped, apply ice to the site for at least 1 hour.
• Notify your physician if you:
▪ Experience an injury and persistent bleeding results
▪ Have excessive menstrual bleeding
▪ See blood in your urine or bowel movement
▪ Have a headache that does not respond to acetaminophen
• Avoid anal intercourse.
• Take a stool softener to prevent straining during a bowel movement.
• Do not use enemas or rectal suppositories.
• Avoid bending over at the waist.
• Do not wear clothing or shoes that are tight or that rub.
• Avoid blowing your nose or placing objects in your nose. If you must blow your nose, do so gently without blocking either nasal passage.Hodgkin's LymphomaPeak risk in two different age-groups: (1) teens and young adults, and (2) adults in their 50s and 60s.
Possible causes of HL include viral infections (i.e. Epstein-Barr virus [EBV], human T-cell leukemia/lymphoma virus [HTLV], and human immune deficiency virus [HIV]) and exposure to chemicals. Most cases of the disease, however, occur in people without known risk factors.
This cancer usually starts in a single lymph node or a single chain of nodes. These nodes contain a specific cancer cell type, the Reed-Sternberg cell, a marker for HL. HL often spreads predictably from one group of lymph nodes to the next, unlike non-Hodgkin's lymphoma.Hodgkin's Lymphoma: AssessmentCommon assessment finding is a large but painless lymph node or nodes. The patient may also have constitutional manifestations ("B symptoms") that include: fevers (>101.5° F [>38.6° C]); heavy night sweats; and unplanned weight loss (>10% of normal body weight).
Diagnosis and subtype are established when biopsy reveals Reed-Sternberg cells.
Staging is performed to determine the extent of disease. Staging usually includes a history and physical examination, CBC, electrolyte panel, kidney and liver function tests, erythrocyte sedimentation rate (ESR), bone marrow aspiration and biopsy, and computed tomography (CT) of the neck, chest, abdomen, and pelvis. Positron emission tomography (PET) may be used to assess for disease not detected by CT. PET scans are helpful after treatment to assess disease response.
After staging procedures are complete, the stage of the disease is determined by the Ann Arbor Staging Criteria (Table 40-4).TABLE 40-4
Ann Arbor Staging Criteria for Hodgkin's LymphomaPG 817 IGGYHodgkin's Lymphoma: InterventionsFor stages I and II disease, the treatment is external radiation of involved lymph node regions. With more extensive disease, radiation and combination chemotherapy are used to achieve remission.
Nursing management of the patient undergoing treatment for HL focuses on the acute side effects of therapy, especially:
• Drug-induced pancytopenia with increased risk for INFECTION, anemia, and bleeding
• Severe nausea and vomiting
• Skin problems at the site of radiation
• Constipation or diarrhea
• Permanent sterility for male patients receiving radiation to the lower abdomen or pelvic region in combination with specific chemotherapy drugs (The patient is informed and given the option to store sperm in a sperm bank before treatment.)
• Secondary cancer development and the need for long-term follow-upNon-Hodgkin's LymphomaNon-Hodgkin's lymphoma (NHL) includes all lymphoid cancers that do not have the Reed-Sternberg cell. NHL generally spreads through the lymphatic system in a less orderly fashion than HL.
Incidence is higher among patients with solid organ transplantation, immunosuppressive drug therapy, and HIV disease. Chronic infection from Helicobacter pylori is associated with a type of lymphoma called mucosa-associated lymphoid tissue (MALT) lymphoma, and Epstein-Barr viral infection has been associated with Burkitt's lymphoma. There is an increased incidence of NHL among people exposed to pesticides, insecticides, and dust.
Patients usually have swollen lymph nodes (lymphadenopathy) or tumor spread to other organs (e.g., GI tract, skin, bone marrow, sinuses, thyroid, central nervous system). Enlarged lymph nodes may be the only manifestation of lymphoma. Painless swelling of the cervical, axillary, inguinal, and femoral nodes is most often seen. The diagnosis of NHL is made only after the biopsy of an involved lymph node. NHLs are broadly classified as B-cell or T-cell lymphomas, depending on the lymphocyte type that gave rise to the cancer.
Lactate dehydrogenase (LDH) levels and beta-2 microglobulin levels are also evaluated to measure tumor growth rates and calculate prognosis. (High LDH levels and high beta-2 microglobulin levels are associated with a poorer prognosis.) Cerebrospinal fluid is evaluated when lymphoma is present in the CNS, around the spinal column, brain, or testes, and when HIV-related lymphoma is diagnosed.
Patients with indolent (slow-growing) lymphomas usually have painless lymph node swelling at diagnosis. Those with more aggressive B-cell lymphomas may have large masses at diagnosis and manifestations. Constitutional manifestations ("B symptoms"), as seen in Hodgkin's lymphoma, occur in about one third of patients with aggressive lymphomas and rarely in indolent lymphomas. Bone marrow involvement in indolent lymphomas is common.Non-Hodgkin's Lymphoma: Patient-Centered Collaborative CareTherapies include combinations of chemotherapy drugs alone or in combination with monoclonal antibodies (e.g., rituximab and alemtuzumab), localized radiation therapy, radiolabeled antibodies (131 I tositumomab and 90Y ibritumomab tiuxetan), hematopoietic stem cell transplantation, and investigational agents.
Nursing care needs are similar to those for patients with HL, with additional organ-specific problems if the disease is widespread. With the use of biotherapy for NHL, close monitoring for infusion-related reactions is needed during and after the delivery of monoclonal antibodies.Multiple MyelomaMultiple myeloma is a white blood cell (WBC) cancer that involves a mature B-lymphocyte called a plasma cell, which secretes antibodies. These cells are overgrown in the bone marrow. When these cells become cancerous, they produce excessive antibodies (gamma globulins). Thus the disorder is called a "gammopathy." When myeloma cells are overproduced, fewer red blood cells (RBCs), WBCs, and platelets are produced, leading to anemia and increased risk for INFECTION and bleeding.
Multiple myeloma cells also produce excess cytokines that increase cancer cell growth and destroy bone. The excess antibodies are in the blood, increasing the serum protein levels and clogging blood vessels in the kidney and other organs. Without treatment, the disease causes progressive bone destruction, bleeding problems, kidney failure, immunosuppression, and death.
Possible risk factors include radiation exposure, chemical exposure, and INFECTION with human herpes virus-8 (HHV-8).
When the specifically altered immunoglobulin is present in a high enough quantity, the type can be recognized as a unique "spike" pattern on a serum electrophoresis test of plasma proteins. Because one clone of cells develops into cancer cells, the abnormal immunoglobulin produced by these cells is a monoclonal paraprotein.Multiple Myeloma: AssessmentAn elevation of serum total protein or a detection of a monoclonal protein (also known as paraprotein) in the blood or urine may be the only finding. Other common manifestations include fatigue, anemia, bone pain, pathologic fractures, recurrent bacterial INFECTIONS, and kidney dysfunction. A positive finding of a serum monoclonal protein is not sufficient to make a diagnosis. Multiple myeloma is distinguished from MGUS by having more than 10% of the bone marrow infiltrated with plasma cells, the presence of a monoclonal protein in the serum or urine, and the presence of osteolytic bone lesions.
Stages and prognostic groups based on the serum beta-2 microglobulin and albumin levels. Other factors that help determine prognosis include age, performance status, serum creatinine, serum albumin, serum calcium, lactate dehydrogenase (LDH) level, C-reactive protein, hemoglobin level, platelet count, quantitative immunoglobulins, beta-2 microglobulin, serum free light chains, serum protein electrophoresis (SPEP) with immunofixation, 24-hour urine for SPEP, and cytogenetic abnormalities found in the bone marrow biopsy.
The patient usually first notices fatigue, easy bruising, and bone pain. Bone fractures, hypertension, INFECTION, hypercalcemia, and fluid imbalance may occur as the disease progresses. Diagnosis is made by x-ray findings of bone thinning with areas of bone loss that resemble Swiss cheese, high immunoglobulin and plasma protein levels, and the presence of Bence-Jones protein (protein composed of incomplete antibodies) in the urine. A bone marrow biopsy is performed to diagnose the diseasemonoclonal gammopathy of undetermined significance or MGUSPositive finding of a serum monoclonal protein is not sufficient to make a diagnosis of multiple myeloma. About 1% of the population produce a monoclonal protein in the blood but do not have multiple myeloma. Considered a premalignant condition.Multiple Myeloma: InterventionsWatchful waiting may be an option instead of chemotherapy. Standard treatment for multiple myeloma is the use of proteasome inhibitors, such as bortezomib (Velcade) or carfilzomib (Kyprolis), and immunomodulating drugs, such as thalidomide (Thalomid) or lenalidomide (Revlimid). All these agents, which are types of targeted cancer therapy, may be used alone or in combination with steroids, such as dexamethasone (Decadron).
Drug therapy is used to reduce tumor burden before transplantation. For patients who are not eligible for an autologous stem cell transplantation, standard chemotherapy drugs such as melphalan, prednisone, vincristine, cyclophosphamide, doxorubicin, and carmustine are usually effective in controlling but not curing the disease.
Myelosuppression is an expected side effect of many myeloma therapies. A nursing priority is to teach the patient about the manifestations. The risk for thromboembolic events is increased with the use of thalidomide and lenalidomide. Peripheral neuropathy can be challenging, causing pain and poor quality of life. GI side effects, such as nausea, vomiting, diarrhea, and constipation, are severe and can be life threatening.
Despite therapy, multiple myeloma remains largely incurable. Best outcomes are seen with autologous hematopoietic stem cell transplantation.
Most patients with multiple myeloma have bone pain, analgesics and alternative approaches for pain management.
The bone disease of multiple myeloma is treated with bisphosphonates (pamidronate [Aredia], zoledronic acid [Zometa], denosumab [Xgeva]), which inhibit bone resorption and can help reduce the skeletal complications.Autoimmune Thrombocytopenic PurpuraAutoimmune thrombocytopenic purpura is also called idiopathic thrombocytopenic purpura (ITP). The number of circulating platelets is greatly reduced in ITP, even though platelet production is normal.
Patients with this disorder make an antibody against the surface of their own platelets (an antiplatelet antibody). This antibody coats the platelet surfaces, making destruction by macrophages easier. This and spleen involvement increase destruction of antibody-coated platelets.
Viral INFECTION is suspected cause.Autoimmune Thrombocytopenic Purpura: AssessmentLarge ecchymoses (bruises) or a petechial rash on the arms, legs, upper chest, and neck; mucosal bleeding occurs easily. If the patient has had significant blood loss, anemia may also be present.
A rare complication is intracranial bleeding-induced stroke. Assess for neurologic function and mental status.
ITP is diagnosed by a low platelet count and increased megakaryocytes in the bone marrow. Antiplatelet antibodies may be detected in the blood. If the patient has any episodes of bleeding, hematocrit and hemoglobin levels may be low.Autoimmune Thrombocytopenic Purpura: InterventionsDrug therapy to control ITP includes drugs that suppress immune function. Drugs such as corticosteroids, azathioprine (Imuran), eltrombopag, rituximab (Rituxan), and romiplostim are used to inhibit production of antiplatelet autoantibodies. IV immunoglobulin and IV anti-Rho can help prevent the destruction of antibody-coated platelets, although anti-Rho carries an increased risk for intravascular hemolysis and death. Aggressive therapy involves low doses of chemotherapy.
Platelet transfusions are used when platelet counts are less than 10,000/mm3 or the patient has an acute life-threatening bleeding episode. Transfusions are not performed routinely because the donated platelets are just as rapidly destroyed by the spleen.
Maintaining a safe environment helps protect the patient from bleeding.
Surgical management with a splenectomy may be needed for the patient who does not respond to drug therapy. After splenectomy, the patient is at increased risk for INFECTION because the spleen performs many protective immune functions, especially antibody generation. For this reason, vaccinations against pneumococcal, meningococcal, and Haemophilus influenzae are recommended either 2 weeks before a planned splenectomy or 2 weeks after the surgery. Teaching patients about their increased risk for infection, avoiding crowds and people who are ill.Thrombotic Thrombocytopenic PurpuraIn thrombotic thrombocytopenic purpura (TTP), platelets clump together abnormally in the capillaries and too few platelets remain in circulation. The patient has inappropriate CLOTTING, yet the blood fails to clot when trauma occurs. The cause of TTP appears to be an autoimmune reaction in small blood vessel cells (endothelial cells).
Tissues become ischemic, leading to kidney failure, myocardial infarction, and stroke. Untreated, this disorder is often fatal within 3 months.
Management of the patient with TTP focuses on preventing platelet clumping and stopping the autoimmune process. Plasma removal and the infusion of fresh frozen plasma reduce the clumping caused by elements of the patient's blood. Drugs that inhibit platelet clumping, such as aspirin, alprostadil (Prostin), and plicamycin, also may be helpful. Immunosuppressive therapy reduces the intensity of this disorder.HemophiliaHemophilia is a hereditary bleeding disorder with two forms. Hemophilia A (classic hemophilia) is a deficiency of factor VIII and accounts for 80% of cases. Hemophilia B (Christmas disease) is a deficiency of factor IX and accounts for 20%.
The patient has abnormal bleeding in response to any trauma because of a deficiency of the specific CLOTTING factor. Hemophiliacs form platelet plugs at the bleeding site, but the clotting factor deficiency impairs the formation of stable fibrin clots. This allows excessive bleeding, which may be mild, moderate, or severeGenetic/Genomic ConsiderationsHemophilia is an X-linked recessive trait. Women who are carriers (can pass on the gene without expressing bleeding problems) have a 50% chance of passing the hemophilia gene to their daughters (who then are carriers) and to their sons (who then have hemophilia). Hemophilia A affects mostly males, none of whose sons will have the gene for hemophilia and all of whose daughters will be carriers. About 30% of patients with hemophilia have no family history and their disease may be the result of a new gene mutation (Beery & Workman, 2012). Ensure that the family is referred to the appropriate level of genetic counseling.Hemophilia: Patient-Centered Collaborative CareAssessment of the patient with hemophilia shows:
• Excessive bleeding from minor cuts, bruises, or abrasions (from abnormal platelet function)
• Joint and muscle hemorrhages that lead to disabling long-term problems and may require joint replacement
• A tendency to bruise easily
• Prolonged and potentially fatal hemorrhage after surgery
The laboratory test results for a patient with hemophilia show a prolonged activated partial thromboplastin time (aPTT), a normal bleeding time, and a normal prothrombin time (PT). The most common problem that occurs with hemophilia is degenerating joint function as a result of chronic bleeding into the joints.
The bleeding problems of hemophilia A are managed by either regularly scheduled infusions of synthetic factor VIII or the infusion of this substance only when injury or bleeding occurs. The source of factor VIII varies (Table 40-5), and the traditional sources, derived from pooled human serum, are no longer recommended because of the risk for infection.TABLE 40-5
Antihemophilic DrugsPG 821 IGGYHeparin-Induced ThrombocytopeniaHeparin-induced thrombocytopenia (HIT) is a serious IMMUNITY-mediated CLOTTING disorder with an unexplained drop in platelet count after heparin treatment. HIT is an immune-mediated drug reaction that is caused by heparin-dependent platelet-activating immunoglobulin G (IgG) antibodies in which heparin binds with platelet factor 4 (PF4). This drug binding leads to the development of a highly reactive immune complex that activates the platelets. Once activated, platelets release procoagulants and PF4, which neutralizes heparin and increases thrombin generation from prothrombin.
HIT can occur in patients receiving any type of heparin(unfractionated more common). Incidence is higher among patients with risk factors of (1) duration of heparin use longer than 1 week, (2) exposure to unfractionated heparin, (3) postsurgical thromboprophylaxis, and (4) being female. Manifestations of HIT include venous thromboembolism (VTE) such as deep vein thrombosis and pulmonary embolism. The diagnosis is based on the patient's exposure to heparin. Thrombocytopenia after heparin exposure is the hallmark sign of HIT.
Once HIT is diagnosed, anticoagulation therapy is started. Drug management for HIT management is with a direct thrombin inhibitor such as argatroban (Argatroban), lepirudin (Refludan), and bivalirudin (Angiomax).TABLE 40-6
Indications for Treatment with Blood ComponentsPG 821 IGGYChart 40-12
Transfusion TherapyPG 821 IGGYPretransfusion ResponsibilitiesChart 40-12 lists best practices for transfusion therapy.
A health care provider's prescription is needed to administer blood components. The prescription specifies the type of component, the volume, and any special conditions. Verify the prescription for accuracy and completeness.
A separate consent form obtained from the patient before a transfusion according to agency.
A blood specimen is obtained for type and crossmatch. Usually a new type-and-crossmatch specimen is required at least every 72 hours.
Both Y-tubing and straight tubing sets are used for blood component infusion (Fig. 40-7). A blood filter (about 170 µm) to remove sediment from the stored blood products is included with blood administration sets and must be used to transfuse most, but not all, blood products.
Use normal saline ONLY as the solution to administer with blood products.
Priority action is to determine that the blood component delivered is correct and that identification of the patient is correct. Check the physician's prescription together with another registered nurse to determine the patient's identity and whether the hospital identification band name and number are identical to those on the blood component tag.
Examine the blood bag label, the attached tag, and the requisition slip to ensure that the ABO and Rh types are compatible with those of the patient. Check the expiration date, and inspect the product for discoloration, gas bubbles, or cloudiness, which are all indicators of bacterial growth or hemolysis.Action AlertNever add to or infuse other drugs with blood products because they may clot the blood during transfusion.Action AlertThe nurse who will be actually administering the blood products must be one of the two professionals comparing the patient's identification with the information on the blood component bag.Transfusion ResponsibilitiesAssess vital signs and temperature immediately before starting the infusion. Begin the infusion slowly. Remain with the patient for the first 15 to 30 minutes. Any severe reaction usually occurs with infusion of the first 50 mL of blood. Ask the patient to report unusual sensations such as chills, shortness of breath, hives, or itching. Assess vital signs 15 minutes after starting the infusion for indications of a reaction. If there are none, the rate can be increased to transfuse 1 unit in 2 hours (depending). Take vital signs every hour during the transfusion. Blood components without large amounts of RBCs can be infused more quickly.
May be necessary to infuse blood products at a slower rate for older patients. Best practices related to the nursing care needs of older patients during transfusion therapy are listed in Chart 40-13.
During transfusions, some cells are damaged, releasing potassium and raising the patient's serum potassium level above normal (hyperkalemia). This problem is more likely when the blood being transfused has been frozen or is several weeks old.Chart 40-13
The Older Adult Receiving a Transfusion• Assess the patient's circulatory, kidney, and fluid status before initiating the transfusion.
• Use no larger than a 19-gauge needle.
• Try to use blood that is less than 1 week old. (Older blood cell membranes are more fragile, break easily, and release potassium into the circulation.)
• Take vital signs (especially pulse, blood pressure, and respiratory rate) every 15 minutes throughout the transfusion. Changes in these parameters can indicate fluid overload and may also be the only indicators of adverse transfusion reactions.
▪ Overload
1. Rapid bounding pulse
2. Hypertension
3. Swollen superficial veins
▪ Transfusion Reaction
1. Rapid thready pulse
2. Hypotension
3. Increased pallor, cyanosis
• Administer blood slowly, taking 2 to 4 hours for each unit of whole blood, packed red blood cells, or plasma.
• Avoid concurrent fluid administration into any other IV site.
• If possible, allow 2 full hours after the administration of 1 unit of blood before administering the next unit.Red Blood Cell TransfusionsRBCs are given to replace cells lost from trauma or surgery. Patients with problems that destroy RBCs or impair RBC maturation also may receive RBC transfusions. Packed RBCs, supplied in 250-mL bags, are a concentrated source of RBCs.
Blood transfusions are transplantations of tissue!! Donor and recipient blood must be carefully checked for compatibility. Compatibility is determined by two different antigen systems (cell surface proteins): the ABO system antigens and the Rh antigen.
RBC ANTIGENS are inherited. For the ABO system, a person inherits one of these:
• A antigen (type A blood)
• B antigen (type B blood)
• Both A and B antigens (type AB blood)
• Neither A nor B antigens (type O blood)
A person with type A blood forms antibodies against type B blood. A person with type O blood has not inherited either A or B antigens and will form antibodies against RBCs with either A or B antigens. If RBCs that have an antigen are infused into a recipient who does not share that antigen, the infused blood is recognized by the recipient's antibodies as non-self and the recipient then has a reaction to the transfused products.
An Rh-negative person is born without the Rh-antigen on his or her RBCs and does not form antibodies unless 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 antibodies develop, any exposure to Rh-positive blood can cause a transfusion reaction. Antibody development can be prevented by giving anti-Rh-immunoglobulin (RHoGAM) 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 should not receive Rh-positive blood.Platelet TransfusionsPlatelets are given to patients with platelet counts below 10,000/mm3 and to patients with thrombocytopenia who are actively bleeding or are scheduled for an invasive procedure. Do not have to be of the same blood type as the patient has. For patients who are having a hematopoietic stem cell transplantation (HSCT) or who need multiple platelet transfusions, platelets from a single donor may be prescribed.
Platelet infusion bags usually contain 300 mL for pooled platelets and 200 mL for single-donor platelets. Platelets are fragile and must be infused immediately after being brought to the patient's room, usually over a 15- to 30-minute period. A special transfusion set with a smaller filter and shorter tubing is used. Additional platelet filters help remove white blood cells (WBCs) from the platelets for patients who have a history of febrile reactions or who need multiple platelet transfusions.
Take the vital signs before the infusion, 15 minutes after the infusion starts, and at its completion. A patient who has had a transfusion reaction in the past may be given diphenhydramine (Benadryl) and acetaminophen (Tylenol) before the transfusion to reduce the fever and severe chills (rigors).Action AlertWhen infusing platelets, do not use the standard blood administration set because the filter traps the platelets and the longer tubing increases platelet adherence to the lumen.Plasma TransfusionsPlasma infusions may be given fresh to replace blood volume and CLOTTING factors. More often, plasma is frozen immediately after donation, forming fresh frozen plasma (FFP). Infuse FFP immediately after thawing while the clotting factors are still active.
ABO compatibility is required for transfusion of plasma products because the plasma contains the donor's ABO antibodies that could react with the recipient's RBC antigens. The infusion bag contains about 200 mL. Infuse FFP as rapidly as the patient can tolerate, generally over a 30- to 60-minute period, through a regular Y set or straight filtered tubing.Granulocyte (White Blood Cell) TransfusionsWBC surfaces have many antigens that can cause severe reactions when infused into a patient whose immune system recognizes these antigens as non-self.
WBCs are suspended in 400 mL of plasma and should be infused slowly, usually over a 45- to 60-minute period.
Often require stricter monitoring of patients receiving WBCs because reactions are more common. 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. Amphotericin B infusion should be separated from WBC transfusions by 4 to 6 hours.Acute Transfusion ReactionsTransfusion reactions: febrile, hemolytic, allergic, or bacterial reactions; circulatory overload; or transfusion-associated graft-versus-host disease (GVHD).Febrile transfusion reactionsOccur most often in the patient with anti-WBC antibodies, which can develop after multiple transfusions, white blood cell transfusions, and platelet transfusions. The patient develops chills, tachycardia, fever, hypotension, and tachypnea. Giving leukocyte-reduced blood or single-donor HLA-matched platelets reduces the risk. WBC filters may be used.Hemolytic transfusion reactionsCaused by blood type or Rh incompatibility. When blood containing antigens different from the patient's own antigens is infused, antigen-antibody complexes are formed in his or her blood. These complexes destroy the transfused cells and start inflammatory responses in the blood vessel walls and organs. The reaction may be mild, with fever and chills, or life threatening, with disseminated intravascular coagulation (DIC) and circulatory collapse. Other manifestations include:
• Apprehension
• Headache
• Chest pain
• Low back pain
• Tachycardia
• Tachypnea
• Hypotension
• Hemoglobinuria
• A sense of impending doomAllergic transfusion reactions(anaphylactic transfusion reactions) are most often seen in patients with other allergies. They may have urticaria, itching, bronchospasm, or anaphylaxis. With an allergy history can be given leukocyte-reduced or washed RBCs in which the WBCs, plasma, and immunoglobulin A have been removed, reducing the possibility of an allergic reaction.Bacterial transfusion reactionsOccur from infusion of contaminated blood products, especially those contaminated with a gram-negative organism. Manifestations include tachycardia, hypotension, fever, chills, and shock. The onset of a bacterial transfusion reaction is rapid.Circulatory overloadOccur when a blood product is infused too quickly, especially in an older adult. This is most common with whole-blood transfusions or when the patient receives multiple packed RBC transfusions. Manifestations include:
• Hypertension
• Bounding pulse
• Distended jugular veins
• Dyspnea
• Restlessness
• Confusion
You can both manage and prevent this complication by monitoring intake and output, infusing blood products more slowly, and giving diuretics.Transfusion-associated graft-versus-host disease (TA-GVHD)A rare but life-threatening problem that occurs more often in an immunosuppressed patient. Its cause in immunosuppressed patients is similar to that of GVHD that occurs with allogeneic stem cell transplantation, in which donor T-cell lymphocytes attack host tissues.
Manifestations usually occur within 1 to 2 weeks and include thrombocytopenia, anorexia, nausea, vomiting, chronic hepatitis, weight loss, and recurrent INFECTION. TA-GVHD has an 80% to 90% mortality rate but can be prevented by using irradiated blood products. Irradiation destroys T-cells and their cytokines.Transfusion-related acute lung injury (TRALI)A life-threatening event that occurs most often when donor blood contains antibodies against the recipient's neutrophil antigens, HLA, or both. Common manifestations are a rapid onset of dyspnea and hypoxia within 6 hours of the transfusion.Acute pain transfusion reaction or APTRA rare event that can occur during or shortly after transfusion of any blood product. Its actual pathophysiology has not yet been elucidated. The manifestations are severe chest pain, back pain, joint pain, hypertension, and redness of the head and neck. Most patients also have anxiety. The reaction does not appear to be life threatening, and most patients respond well with drugs for pain and rigors. Although the manifestations are general, diagnosis can be supported with a positive direct antibody test (DAT), indicating that some degree of hemolysis has occurred but is not widespread. APTR management focuses on patient support and drugs to control or reduce manifestations.Interventions for Transfusion ReactionsInterventions for transfusion reactions occurring during transfusion (hemolytic reactions, allergic reactions, and bacterial reactions) begin with stopping the transfusion and removing the blood tubing. (For hemolytic and suspected bacterial reactions, return the component bag, labels, and all tubing to the blood bank or laboratory.) Notify the Rapid Response Team. If the patient has no other IV access, keep the access and flush with normal saline. Do not flush the contents of the blood transfusion tubing, which would allow more of the reaction-causing blood to enter the patient. Usually oxygen is applied and diphenhydramine (Benadryl) is administered by IV push. If manifestations of shock are present, fluid resuscitation and hemodynamic monitoring are needed. Blood pressure support with vasopressors may be needed. Other drug therapy is supportive, such as antipyretics for fever, antibiotics for suspected bacterial contamination, and meperidine for rigors.Autologous Blood TransfusionsAutologous blood transfusions involve collection and infusion of the patient's own blood. Four types of autologous blood transfusions are preoperative autologous blood donation, acute normovolemic hemodilution, intraoperative autologous transfusion, and postoperative blood salvage.
Autologous blood donation before surgery is the most common type of autologous blood transfusion. It involves collecting whole blood from the patient (who must meet certain criteria), dividing it into components, and storing it for later use. As long as hematocrit and hemoglobin levels are within a safe range, the patient can donate blood on a weekly basis until the prescribed amount of blood is obtained. Fresh packed RBCs 826may be stored for 40 days.
Acute normovolemic hemodilution involves withdrawal of a patient'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 not used with anemic patients or those with poor kidney function.
Intraoperative autologous transfusion and blood salvage after surgery are the recovery and reinfusion of a patient's own blood from an operative field or from a bleeding wound. Special devices collect, filter, and drain the blood into a transfusion bag. This blood is used for trauma or surgical patients with severe blood loss. The salvaged blood must be reinfused within 6 hours.
Transfuse autologous blood products using the guidelines previously described.What might you NOTICE if the patient is experiencing inadequate GAS EXCHANGE and PERFUSION as a result of hematologic problems?• Skin cyanosis or pallor (in patients with light skin)
• Cyanosis or pallor of the lips and oral mucous membranes
• Tachycardia
• Tachypnea and dyspnea
• Slow capillary refill
• Cool to cold extremities
• Change in cognition, acute confusion
• Decreased oxygen saturation
• Decreased urine output
• Presence of bruises or petechiae
• Bleeding of gums, at IV sites, at injection sitesWhat should you INTERPRET and how should you RESPOND to this patient experiencing inadequate GAS EXCHANGE and PERFUSION as a result of a hematologic problem?Perform and interpret physical assessment, including:
• Taking vital signs
• Monitoring oxygen saturation by pulse oximetry
• Checking for blood in stool, urine, emesis
• Checking for bleeding in the mouth, around IV sites, drains, urinary catheters
• Checking most recent laboratory values for hematocrit and hemoglobin levels and platelet and RBC counts
• Assessing cognition
Respond by:
• Applying oxygen
• Keeping the patient's head elevated to about 30 degrees
• Handling the patient gently
• Keeping the patient warm (blankets)
• Applying firm pressure to areas actively bleeding
• Notifying physician or Rapid Response Team
• Instituting Bleeding Precautions
• Maintaining or initiating IV therapy
• Preparing to administer blood or blood products
• Prioritizing and pacing activities to prevent fatigueKey Points: Safe and Effective Care Environment• Use aseptic technique during all central line dressing changes or any invasive procedure. Safety image
• Use good handwashing techniques before providing any care to a patient who is either immunocompromised or immune deficient.
• Modify the environment to protect patients who have thrombocytopenia.
• Use Bleeding Precautions for any patient with thrombocytopenia or pancytopenia (see Chart 40-7).
• Ensure informed consent is obtained before any invasive procedure or transfusion.
• Verify with another registered nurse prescriptions for transfusion of blood products.
• Use at least two forms of identification for the patient who is to receive a blood product transfusion (e.g., name, birthdate, identification number).Key Points: Health Promotion and Maintenance• Teach patients with sickle cell disease to avoid conditions that are known to trigger crises.
• Teach people to avoid unnecessary contact with environmental chemicals or toxins. If contact cannot be avoided, teach people to use safety precautions.
• Identify patients at high risk for INFECTION because of disease or therapy.
• Teach the patient and family about the manifestations of INFECTION and when to seek medical advice.
• Instruct patients who have anemia as a result of dietary deficiency which foods are good sources of iron, folic acid, and vitamin B12.
• Teach precautions to take to avoid injury (see Chart 40-11) to patients at risk for poor CLOTTING and increased bleeding.Key Points: Psychosocial Integrity• Allow the patient the opportunity to express his or her feelings regarding the diagnosis of leukemia or lymphoma or the treatment regimen.
• Explain all procedures, restrictions, drugs, and follow-up care to the patient and family.
• Offer alternative therapies for relaxation, pain reduction, and distraction, such as massage, music therapy, and guided imagery.
• Reassure patients having pain that using opioid analgesics for needed pain relief is not drug abuse.Key Points: Physiological Integrity• Pace nonurgent health care activities to reduce the risk for fatigue among patients with anemia or pancytopenia.
• Assess patients in the induction phase of chemotherapy, those after HSCT, and anyone with neutropenia every 8 hours for manifestations of INFECTION.
• Assess the skin integrity of the perianal region of a patient with leukemia or profound neutropenia after every bowel movement.
• Administer analgesics on a schedule rather than PRN.
• Use normal saline as the solution infusing with blood products.
• Transfuse blood products more slowly to older patients or those who have a cardiac problem.
• Remain with the patient during the first 15 minutes of infusion of any blood product.
• Do not administer any drugs with infusing blood products.
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