NSG 4052 Exam #4 Study Guide / NSG4052 Study Guide Exam #4 (Latest-2020): South University

NSG 4052 Exam #4 Study Guide / NSG4052 Study Guide Exam #4 General Assessment of patients with anemia: (3-5) signs & symptoms, nursing management, labs It is a prevalent condition with many diverse causes, such as blood loss, impaired production of erythrocytes, or increased destruction of erythrocytes. Anemia is diagnosed based on a complete blood count (CBC), reticulocyte count, and peripheral blood smear. Clinical Manifestations The clinical manifestations of anemia are caused by the body's response to tissue hypoxia.Mild states of anemia (Hgb 10 to 12 g/dL [100 to 120 g/L]) may exist without causing symptoms. Symptoms include palpitations, dyspnea, and mild fatigue. In moderate anemia (Hgb 6 to 10 g/dL [60 to 100 g/L]) the above cardiopulmonary symptoms are increased. The patient may experience them while resting, as well as with activity. Increased palpitations with “bounding pulse,” dyspnea, “roaring in the ears” and Fatigue In severe anemia (Hgb less than 6 g/dL [60 g/L]) the patient has many clinical manifestations involving multiple body system Body System SEVERITY OF ANEMIA Severe (Hgb 6 g/dL [60 g/L]) Integument Pallor, jaundice,* pruritus* Eyes Icteric conjunctiva and sclera,* retinal hemorrhage, blurred vision Mouth Glossitis, smooth tongue Cardiovascular Tachycardia, increased pulse pressure, systolic murmurs and bruits, intermittent claudication, angina, heart failure, myocardial infarction ( if myocardial O2 not met). Pulmonary Tachypnea, orthopnea, dyspnea at rest Neurologic Headache, vertigo, irritability, depression, impaired thought processes Gastrointestinal Anorexia, hepatomegaly, splenomegaly, difficulty swallowing, sore mouth Musculoskeletal Bone pain General Sensitivity to cold, weight loss, lethargy Table 30-5 Nutritional Therapy Nutrients for Erythropoiesis ________________________________________ Role in Erythropoiesis Food Sources Cobalamin (Vitamin B12) RBC maturation Red meats, especially liver, eggs, enriched grain products, milk and dairy foods, fish Folic Acid RBC maturation Green leafy vegetables, liver, meat, fish, legumes, whole grains, orange juice, peanuts, avocado Iron Hemoglobin synthesis Liver and muscle meats, dried fruits, legumes, dark green leafy vegetables, whole-grain and enriched bread and cereals, beans In addition to the skin, the sclera of the eyes and mucous membranes should be evaluated for jaundice because they reflect the integumentary changes more accurately, especially in a dark-skinned individual. Benign prostatic hyperplasia: (3) know etiology/signs & symptoms; nursing management, priorities of care, complications; routine/priority of care for post-op urinary conduits Benign prostatic hyperplasia (BPH) is a condition in which the prostate gland increases in size, leading to disruption of the outflow of urine from the bladder through the urethra. Almost half of the men with BPH will have bothersome lower urinary tract symptoms (LUTS), such as difficulty starting a urine stream, a decreased flow of urine, or urinary frequency. Clinical Manifestations ● Symptoms can be divided into two groups: irritative and obstructive. ● Irritative symptoms include nocturia, urinary frequency, urgency, dysuria, bladder pain, and incontinence. These symptoms are associated with inflammation or infection. Nocturia is often the first symptom that the patient notices. ● Obstructive symptoms, caused by prostate enlargement, include a decrease in the caliber and force of the urinary stream, difficulty in initiating a stream, intermittency (stopping and starting stream several times while voiding), and dribbling at the end of urination. ● These symptoms are due to the increased effort of the bladder as it tries to empty through the decreased diameter of the urethra. As a group, both irritative and obstructive symptoms are considered lower urinary tract symptoms (LUTS). Complications ● Some men may experience acute urinary retention. This complication is manifested by the sudden and painful inability to urinate. ○ Treatment involves the insertion of a catheter to drain the bladder. Surgery may be indicated in severe situations. ● Urinary tract infection (UTI) can also be a complication of BPH. Since the bladder is unable to empty completely, bacteria can grow and cause infection within the urinary tract. In more severe cases, sepsis may also develop. ● Bladder calculi (stones) may develop in the bladder because of the alkalinization of the residual urine. Although bladder stones are more common in men with BPH, the risk of kidney stones is not significantly increased. ● Additional complications include renal failure caused by hydronephrosis (distention of pelvis and calyces of the kidney by urine that cannot flow through the ureter to the bladder), pyelonephritis, and bladder damage if treatment for acute urinary retention is delayed. Diagnostic Studies ● A digital rectal examination (DRE) is done to estimate the prostate size, symmetry, and consistency. In BPH, the prostate is symmetrically enlarged, firm, and smooth. ● Other diagnostic testing may include a urinalysis with culture to look for bacteria, white blood cells (WBCs), or microscopic hematuria, which indicate infection or inflammation. ● A prostate-specific antigen (PSA) blood test may be done to screen for prostate cancer. However, PSA levels may be slightly elevated in patients with BPH. ● Serum creatinine levels may be ordered to rule out renal insufficiency. Because symptoms of BPH are similar to those of a neurogenic bladder, a neurologic examination may also be performed. ● In patients with an abnormal DRE and elevated PSA, a transrectal ultrasound (TRUS) is typically ordered. ○ This examination allows for accurate assessment of prostate size and can help to differentiate BPH from prostate cancer. Biopsies can be taken during the ultrasound procedure. Interprofessional Care ● The goals of interprofessional care are to (1) restore bladder drainage, (2) relieve the patient's symptoms, and (3) prevent or treat the complications of BPH. ● The most conservative treatment that may be recommended for some patients with BPH is referred to as active surveillance, or watchful waiting. When the patient has mild symptoms (AUA symptom scores of 0 to 7), a wait-and-see approach is taken. ● Making dietary changes (decreasing intake of caffeine, artificial sweeteners, and spicy or acidic foods), avoiding drugs such as decongestants and anticholinergics, and restricting evening fluid intake may improve symptoms. ● In addition, a timed voiding schedule may reduce or eliminate symptoms, thus eliminating the need for further intervention. Drug Therapy. ● Two classes of drugs that are used to treat BPH include 5α-reductase inhibitors and α-adrenergic receptor blockers. 5α-Reductase Inhibitors. ● 5α-Reductase inhibitors work by reducing the size of the prostate gland. ● Finasteride (Proscar) blocks the enzyme 5α-reductase, which is necessary for the conversion of testosterone to DHT (principal intraprostatic androgen). Prostate size is directly related to the amount of DHT. By blocking DHT, overly enlarged prostates can decrease in size. This class of medication is more effective for men with larger prostates who experience bothersome symptoms. ○ Take up to 6 months to be effective. These drugs must be taken on a regular basis to have an effect. ● Serum PSA levels may be decreased by almost 50% when taking finasteride. Therefore to “correct” for the decrease caused by the finasteride, the HCP should double the PSA result. ● Dutasteride (Avodart) too. The combination of a 5α-reductase inhibitor (dutasteride) and an α-adrenergic receptor blocker (tamsulosin) is available in a single oral medication (Jalyn). ● In addition to decreasing the symptoms of BPH, finasteride, dutasteride, and Jalyn (finasteride plus tamsulosin) may also lower the risk of prostate cancer. However, the use of these drugs in the prevention of prostate cancer has not been recommended. ○ Patients with an increased PSA level while taking these medications should be referred to their HCP. Also encourage the patient to discuss the need for prostate cancer screening with the HCP. Drug Alert Finasteride (Proscar)________________________________________ • Women who may be or are pregnant should not handle tablets due to potential risk to male fetus (anomaly). α-Adrenergic Receptor Blockers. ● α-Adrenergic receptor blockers are another drug treatment option for BPH. These medications selectively block α1-adrenergic receptors, which are abundant in the prostate, and are increased in hyperplastic prostate tissue. Although nonspecific α-adrenergic blockers are more commonly used for the treatment of hypertension, these drugs promote smooth muscle relaxation in the prostate, thus facilitating urinary flow through the urethra. ● Several α-adrenergic blockers are used, including silodosin (Rapaflo), alfuzosin (Uroxatral), doxazosin (Cardura), prazosin (Minipress), terazosin, and tamsulosin (Flomax). Erectogenic Drugs. Tadalafil (Cialis) has been used in men who have symptoms of BPH alone or in combination with erectile dysfunction (ED). The drug has shown to be effective in reducing symptoms for both of these conditions. Nursing management ● Intermittent or continuous bladder irrigation (CBI) is usually required after invasive prostate surgery to prevent bladder obstruction by clots or mucus. Surgery management ● The main complications after surgery are hemorrhage, bladder spasms, urinary incontinence, and infection. ● After surgery the patient will have a standard catheter or a triple-lumen catheter. Bladder irrigation is typically done to remove clotted blood from the bladder and ensure drainage of urine. The bladder is irrigated either manually on an intermittent basis or more commonly as continuous bladder irrigation (CBI) with sterile normal saline solution or another prescribed solution. If the bladder is manually irrigated (if ordered), instill 50 mL of irrigating solution and then withdraw with a syringe to remove clots that may be in the bladder and catheter. Painful bladder spasms often occur as a result of manual irrigation. ● Continuously monitor the inflow and outflow of the irrigant. If outflow is less than inflow, assess the catheter patency for kinks or clots. Urinary Diversions: (5-8): know etiology/signs & symptoms; nursing management, priorities of care, complications; routine/priority of care for post-op urinary conduits Urinary Diversions: Performed to treat cancer of the bladder, neurogenic bladder, or trauma to the bladder ● TYPE OF DIVERSION DEPENDS ON PT. AGE, CONDITION OF BLADDER, DEGREE OF OBESITY, RENAL FUNCTION, BODY BUILD, & PT. WILLINGNESS TO LEARN & PARTICIPATE IN POSTOP CARE REQUIRED. ● DEPENDS ON THE LOCATION OF THE DIVERSION, IF A SEAL CAN BE MAINTAINED TO PREVENT LEAKAGE, & THEIR ABILITY TO MANAGE THE APPARATUS. Bladder: ● PERFORMED TO: DIVERT URINE FROM BLADDER TO A NEW SITE, THROUGH AN OPENING CREATED IN THE SKIN. **2 TYPES: Ileal conduit & continent diversions -ileal conduit –urine drains through an opening in the abd. wall & skin -it is the oldest method & standard -A piece of the ileum is used to make the passageway for urine and bowel is resected. -Stents are usually used to prevent occulsion d/t edema postop & left in 12-21 days. -Jackson pratts normally inserted into the pelvic area where bladder was to prevent accumulation of fluid in the space. Complications: ● INFECTION ● WOUND DEHISCENCE ● URINARY LEAKAGE ● URETERAL OBSTRUCTION ● SMALL BOWEL OBSTRUCTION, ILEUS ● STOMAL GANGRENE ● STENOSIS OF STOMA ● ↓ RENAL FUNCTION D/T REFLUX, CALCULI Goal of Care: Ileal Conduit 1. Maintaining peristomal skin integrity 2. Increase self-esteem 3. Develop appropriate coping mechanisms 4. ↑ knowledge of management of urinary function 5. Prevent complications 6. Prevent dislodgement of bag. Continent Urinary Diversions MOST COMMON – INDIANA POUCH: CREATED WHEN BLADDER REMOVED OR CAN NO LONGER FUNCTION (NEUROGENIC BLADDER). MUST INSERT A CATH Q 2-4 HRS TO REMOVE URINE FROM POUCH. CONTINENT URINARY DIVERSION- an intra abdominal urinary reservoir that can be catheterized or that has an outlet controlled by the anal sphincter. These are internal pouches created similarly to the ileal conduit. ● PORTION OF THE INTESTINES IS USED TO CREATE A NEW RESERVOIR FOR URINE. Postoperative Nursing Care ● Continent ileal urinary pouch is similar to nursing care of the patient with an ileal conduit. ● Usually have additional drainage tubes (cecostomy catheter from the pouch, stoma catheter exiting from the stoma, ureteral stents, penrose drain, urethral catheter) ● Must irrigate cecostomy tube to maintain patency w/ NS at least 2-3 x/day. ● Patient needs to self-catheterize q 4-6 hrs, but does not need external attachments for drainage. Sickle cell anemia: (6-10) know etiology/signs & symptoms, medical treatment, nursing management, priorities of care, complications-e.g. splenectomy, priapism, splenic crisis Sickle cell disease (SCD) is a group of inherited, autosomal recessive disorders characterized by an abnormal form of hemoglobin in the RBC. Because this is a genetic disorder, SCD is usually identified during routine neonatal screening. It is an incurable disease that is often fatal by middle age because of renal failure, infection, pulmonary failure, and/or stroke. Etiology and Pathophysiology Genetic Link. ● In SCD the abnormal hemoglobin, hemoglobin S (Hgb S), results from the substitution of valine for glutamic acid on the β-globin chain of hemoglobin. Hgb S causes the erythrocyte to stiffen and elongate, taking on a sickle shape in response to low O2 levels. ● Types of SCD disorders include sickle cell anemia, sickle cell–thalassemia, sickle cell Hgb C disease, and sickle cell trait. Sickle cell anemia, the most severe of the SCD syndromes, occurs when a person is homozygous for hemoglobin S (Hgb SS); the person has inherited Hgb S from both parents. Sickle cell trait occurs when a person is heterozygous for hemoglobin S (Hgb AS); the person has inherited hemoglobin S from one parent and normal hemoglobin (Hgb A) from the other parent. Sickle cell trait is typically a mild to asymptomatic condition. Sickling Episodes. ● The major pathophysiologic event of SCD is the sickling of RBCs. ● Sickling episodes are most commonly triggered by low O2 tension in the blood. ● Hypoxia or deoxygenation of the RBCs can be caused by viral or bacterial infection, high altitude, emotional or physical stress, surgery, and blood loss. ● Infection is the most common precipitating factor. ● Other events that can trigger or sustain a sickling episode include dehydration, increased hydrogen ion concentration (acidosis), increased plasma osmolality, decreased plasma volume, and low body temperature. ● Sickled RBCs become rigid and take on an elongated, crescent shape. Sickled cells cannot easily pass through capillaries or other small vessels and can cause vascular occlusion, leading to acute or chronic tissue injury. ● The resulting hemostasis promotes a self-perpetuating cycle of local hypoxia, deoxygenation of more erythrocytes, and more sickling. Circulating sickled cells are hemolyzed by the spleen, leading to anemia. ● Initially the sickling of cells is reversible with reoxygenation, but it eventually becomes irreversible because of cell membrane damage from recurrent sickling. Thus vaso-occlusive phenomena and hemolysis are the clinical hallmarks of SCD. ● Sickle cell crisis is a severe, painful, acute exacerbation of RBC sickling, causing a vaso-occlusive crisis. As blood flow is impaired by sickled cells, vasospasm occurs, further restricting blood flow. ● Severe capillary hypoxia causes changes in membrane permeability, leading to plasma loss, hemoconcentration, thrombi, and further circulatory stagnation. Tissue ischemia, infarction, and necrosis eventually occur from lack of O2. ● Shock is a possibly life-threatening consequence of sickle cell crisis because of severe O2 depletion of the tissues and a reduction of the circulating fluid volume. Sickle cell crisis can begin suddenly and persist for days to weeks. ● The frequency, extent, and severity of sickling episodes are highly variable and unpredictable but largely depend on the percentage of Hgb S present. Individuals with sickle cell anemia have the most severe form because the erythrocytes contain a high percentage of Hgb S. Clinical Manifestations ● The effects of SCD vary greatly from person to person, the severity of which may be due to genetic polymorphisms. They may have chronic health problems and pain because of organ tissue hypoxia and damage (e.g., involving the kidneys or liver). ● The typical patient is anemic but asymptomatic except during sickling episodes. Because most individuals with sickle cell anemia have dark skin, pallor is more readily detected by examining the mucous membranes. ● The skin may have a grayish cast. Because of the hemolysis, jaundice is common and patients are prone to gallstones (cholelithiasis). ● The primary symptom associated with sickling is pain. The pain severity can range from trivial to excruciating. During sickle cell crisis the pain is severe because of ischemia of tissue. ● The episodes can affect any area of the body or several sites simultaneously, with the back, chest, extremities, and abdomen being most commonly affected. Pain episodes are often accompanied by clinical manifestations such as fever, swelling, tenderness, tachypnea, hypertension, nausea, and vomiting. Complications ● With repeated episodes of sickling, there is gradual involvement of all body systems, especially the spleen, lungs, kidneys, and brain. Organs that have a need for large amounts of O2 are most often affected and form the basis for many of the complications of SCD (Fig. 30-4). ● Infection is a major cause of morbidity and mortality in patients with SCD. One reason for this is the failure of the spleen to phagocytize foreign substances as it becomes infarcted and dysfunctional (usually by 2 to 4 years of age) from the sickled RBCs. The spleen becomes small because of repeated scarring, a phenomenon termed autosplenectomy. ● Pneumonia is the most common infection and often is of pneumococcal origin. ● Acute chest syndrome is a term used to describe acute pulmonary complications that include pneumonia, tissue infarction, and fat embolism. ○ It is characterized by fever, chest pain, cough, pulmonary infiltrates, and dyspnea. ● Pulmonary infarctions may cause pulmonary hypertension, MI, HF, and ultimately cor pulmonale. The heart may become ischemic and enlarged, leading to HF. ● Retinal vessel obstruction may result in hemorrhage, scarring, retinal detachment, and blindness. ● The kidneys may be injured from the increased blood viscosity and the lack of O2, and renal failure may occur. ● Pulmonary embolism or stroke can result from thrombosis and infarction of cerebral blood vessels. ● Bone changes may include osteoporosis and osteosclerosis after infarction. ● Chronic leg ulcers can result from the hypoxia and are especially prevalent around the ankles. ● Priapism (persistent penile erection) may occur if penile veins become occluded. Diagnostic Studies ● A peripheral blood smear may reveal sickled cells and abnormal reticulocytes. The presence of Hgb S can be diagnosed by the sickling test, which uses RBCs (in vitro) and exposes them to a deoxygenation agent. ● As a result of the accelerated RBC breakdown, the patient has characteristic clinical findings of hemolysis (jaundice, elevated serum bilirubin levels) and abnormal laboratory test results. Hemoglobin electrophoresis may be done to determine the amount of hemoglobin S. Skeletal x-rays demonstrate bone and joint deformities and flattening. ● MRI may be used to diagnose a stroke caused by blocked cerebral vessels from sickled cells. Doppler studies may be used to assess for deep vein thromboses. Nursing and Interprofessional Management: Sickle Cell Disease ● Interprofessional care for a patient with SCD is directed toward (1) prevention of sequelae from the disease; (2) alleviating the manifestations from the complications of the disease; (3) minimizing end-organ damage; and (4) promptly treating serious sequelae, such as acute chest syndrome, that can lead to immediate death. ● Teach patients with SCD to avoid high altitudes, maintain adequate fluid intake, and treat infections promptly. ● Screening for retinopathy should begin at age 10. ● Immunizations such as pneumococcal, Haemophilus influenzae, influenza, and hepatitis should be administered. ● Sickle cell crises may require hospitalization. O2 may be administered to treat hypoxia and control sickling. Because respiratory failure is a most common cause of death, vigilantly assess for any changes in respiratory status. ● Rest may be instituted to reduce metabolic requirements, and deep vein thrombosis prophylaxis (using anticoagulants) should be prescribed. ● Fluids and electrolytes are administered to reduce blood viscosity and maintain renal function. ● Priapism is managed with pain medication, fluids, and nifedipine (Procardia). If it does not resolve within a few hours, a urologist can be called to inject the corpus cavernosum with a dilute solution of epinephrine to preserve penile function. ● Transfusion therapy is indicated when an aplastic crisis occurs. Aggressive total RBC exchange transfusion programs may be implemented for patients who have frequent crises or serious complications, such as acute chest syndrome. These patients, like those with thalassemia major, may require iron chelation therapy to reduce transfusion-produced iron overload. ● Undertreatment of sickle cell pain is a major problem. Lack of understanding can lead HCPs to underestimate how much pain these patients suffer. Patients, because of their prior opioid treatment, may be tolerant, and thus large doses may be needed to reduce the pain to a tolerable level. Morphine and hydromorphone are the drugs of choice. ● Although pain is the most common symptom of patients with SCD, infection is a frequent complication and must be treated. ● Infections, such as chronic leg ulcers, may be treated with rest, antibiotics, warm saline soaks, mechanical or enzyme debridement, and grafting if necessary. ● Patients with acute chest syndrome are treated with broad-spectrum antibiotics, O2 therapy, fluid therapy, and possibly exchange transfusion. Blood transfusions have little if any role in the treatment between crises, since patients develop antibodies to RBCs and iron overload. However, because chronic hemolysis results in increased use of folic acid stores, routine oral folic acid should be taken. ● Although many antisickling agents have been tried, hydroxyurea (Hydrea) is the only one that has been shown to be clinically beneficial. This drug increases the production of hemoglobin F (fetal hemoglobin) and alters the adhesion of sickled RBCs to the endothelium. The increase in Hgb F is accompanied by a reduction in hemolysis, an increase in hemoglobin concentration, and a decrease in sickled cells and painful crises.14 ● HSCT is the only available treatment that can cure some patients with SCD. ● The patient and caregiver must understand the basis of the disease and the reasons for supportive care and ongoing pertinent screening tailored to SCD manifestations, such as eye examinations. ● Teach the patient ways to avoid crises, including taking steps to avoid dehydration and hypoxia, such as avoiding high altitudes and seeking medical attention quickly to counteract problems such as upper respiratory tract infections. Polycythemia: (3-5) signs & symptoms, medical treatment, nursing management, priorities of care, complications Polycythemia is the production and presence of increased numbers of RBCs. The increase in RBCs can be so great that blood circulation is impaired as a result of the increased blood viscosity (hyperviscosity) and volume (hypervolemia). Primary Polycythemia. (Decreased EPO or Normal) ● Polycythemia vera is a chronic myeloproliferative disorder. Therefore not only RBCs are involved but also WBCs and platelets, leading to increased production of each of these blood cells. The disease develops insidiously and follows a chronic, vacillating course. ● In polycythemia vera there is enhanced blood viscosity and blood volume and congestion of organs and tissues with blood. Splenomegaly (which occurs in 90% of patients) and hepatomegaly are common. These patients have hypercoagulopathies that predispose them to clotting. Genetic Link. ● Polycythemia vera is associated with mutations in the Janus kinase-2 (JAK2) gene. The JAK2 gene provides instructions for making a protein that promotes proliferation of cells, especially blood cells from hematopoietic stem cells. Secondary Polycythemia.(Increased EPO) ● Secondary polycythemia can be either hypoxia driven or hypoxia independent. In hypoxia-driven secondary polycythemia, hypoxia stimulates erythropoietin (EPO) production in the kidney, which in turn stimulates RBC production. The need for O2 may result from high altitude, pulmonary disease, cardiovascular disease, alveolar hypoventilation, defective O2 transport, or tissue hypoxia. ● EPO levels may return to normal once the hemoglobin is stabilized at a higher level. In this situation, secondary polycythemia is a physiologic response in which the body tries to compensate for a problem, rather than a pathologic response. (Hypoxia-driven secondary polycythemia is discussed in the section on chronic obstructive pulmonary disease in Chapter 28.) ● In hypoxia-independent secondary polycythemia, EPO is produced by a malignant or benign tumor tissue. Serum EPO levels often remain elevated in these situations. Splenomegaly does not accompany secondary polycythemia. Clinical Manifestations and Complications ● Circulatory manifestations of polycythemia vera occur because of the hypertension caused by hypervolemia and hyperviscosity. ● They are often the first manifestations and include subjective complaints of headache, vertigo, dizziness, tinnitus, and visual disturbances. ● Generalized pruritus (often exacerbated by a hot bath) may be a striking symptom and is related to histamine release from an increased number of basophils. ● Paresthesias and erythromelalgia (painful burning and redness of the hands and feet) may also be present. ● In addition, the patient may experience angina, HF, intermittent claudication, and thrombophlebitis, which may be complicated by embolization. These manifestations are caused by blood vessel distention, impaired blood flow, circulatory stasis, thrombosis, and tissue hypoxia caused by the hypervolemia and hyperviscosity. ● The most common serious acute complication is stroke secondary to thrombosis. ● Hemorrhagic phenomena caused by either vessel rupture from overdistention or inadequate platelet function may result in petechiae, ecchymoses, epistaxis, or GI bleeding. Hemorrhage can be acute and catastrophic. ● Hepatomegaly and splenomegaly from organ engorgement may contribute to patient complaints of satiety and fullness. ● The patient may also experience pain from peptic ulcer caused either by increased gastric secretions or by liver and spleen engorgement. ● Plethora (ruddy complexion) may also be present. ● Hyperuricemia is caused by the increase in RBC destruction that accompanies excessive RBC production. Uric acid is one of the products of cell destruction. As RBC destruction increases, uric acid production also increases, thus leading to hyperuricemia and possible gout. ● Although the incidence is low, myelofibrosis and leukemia develop in some patients with polycythemia vera. These disorders may be caused by the chemotherapeutic drugs used to treat the disease, or they may be secondary to a disorder in the stem cells that progresses to erythroleukemia. ● The major cause of morbidity and mortality from polycythemia vera is related to thrombosis (e.g., stroke). Diagnostic Studies ● The following laboratory manifestations are seen in a patient with polycythemia vera: (1) elevated hemoglobin and RBC count with microcytosis; (2) low to normal EPO level (secondary polycythemia has a high level); (3) elevated WBC count with basophilia; (4) elevated platelet count (thrombocytosis) and platelet dysfunction; (5) elevated leukocyte alkaline phosphatase, uric acid, and cobalamin levels; and (6) elevated histamine levels. ● Bone marrow examination in polycythemia vera shows hypercellularity of RBCs, WBCs, and platelets. Interprofessional Care ● Treatment is directed toward reducing blood volume and viscosity and bone marrow activity. ● Phlebotomy is the mainstay of treatment. The aim of phlebotomy is to reduce the hematocrit and keep it less than 45% in men and 42% in women. ● Generally, at the time of diagnosis 300 to 500 mL of blood may be removed every other day until the hematocrit is reduced to normal levels. ● An individual managed with repeated phlebotomies eventually becomes deficient in iron, although this effect is rarely symptomatic. Avoid iron supplementation. Hydration therapy is used to reduce the blood's viscosity. ● Myelosuppressive agents such as hydroxyurea (Hydrea), busulfan (Myleran), and chlor­ambucil (Leukeran) are used. ● Ruxolitinib (Jakafi), which inhibits the expression of the JAK2 mutation, is used for patients who have not responded to hydroxyurea. ● Low-dose aspirin is used to prevent clotting. α-Interferon (α-IFN) is of particular use in women of childbearing age or those with intractable pruritus. ● Anagrelide (Agrylin) may be used to reduce the platelet count and inhibit platelet aggregation. ● Allopurinol (Zyloprim) may reduce the number of acute gouty attacks.(Gout may occur because of increased uric acid levels from the destruction of cells.) Nursing Management: Polycythemia Vera ● Primary polycythemia (polycythemia vera) is not preventable. When acute exacerbations of polycythemia vera develop, you have several responsibilities. ● Depending on the institution's policies, you may either assist with or perform the phlebotomy. ● Evaluate fluid intake and output during hydration therapy to avoid fluid overload (which further complicates the circulatory congestion) and underhydration (which can make the blood even more viscous). ● Assess the patient's nutritional status, since inadequate food intake can result from GI symptoms of fullness, pain, and dyspepsia. ● Begin activities and/or medications to decrease thrombus formation. Initiate active or passive leg exercises and ambulation when possible. ● Because of its chronic nature, polycythemia vera requires ongoing evaluation. ● Phlebotomy may be needed every 2 to 3 months, reducing the blood volume by about 500 mL each time. Evaluate the patient for complications. Megaloblastic Anemias ● Megaloblastic anemias are a group of disorders caused by impaired DNA synthesis and characterized by the presence of large RBCs. Macrocytic RBCs are easily destroyed because they have fragile cell membranes. Although the overwhelming majority of megaloblastic anemias result from cobalamin and folic acid deficiencies. Table 30-8 Classification of Megaloblastic Anemia Cobalamin (Vitamin B12) Deficiency • Dietary deficiency • Deficiency of gastric intrinsic factor • Pernicious anemia • Gastrectomy • Gastric bypass • Celiac disease • Helicobacter pylori • Intestinal malabsorption • Increased requirement (pregnancy) • Chronic alcoholism Folic Acid Deficiency • Dietary deficiency (e.g., leafy green vegetables, citrus fruits) • Malabsorption syndromes • Celiac disease • Small bowel resection • Drugs interfering with absorption or use of folic acid • Methotrexate • Antiseizure drugs (e.g., phenobarbital, phenytoin [Dilantin]) • Increased requirement (pregnancy) • Chronic alcoholism Vitamin B 12 Deficiency: (3-5) signs & symptoms, medical treatment, nursing management, priorities of care, complications; nutrition-types of foods; labs Megaloblastic Anemia: Cobalamin (Vitamin B12) Deficiency ● Normally, a protein termed intrinsic factor (IF) is secreted by the parietal cells of the gastric mucosa. IF is required for cobalamin (extrinsic factor) absorption. (Cobalamin is normally absorbed in the distal ileum.) Therefore if IF is not secreted, cobalamin will not be absorbed. Etiology Pernicious Anemia. ● The most common cause of cobalamin deficiency is pernicious anemia, which is caused by an absence of IF. ● In pernicious anemia the gastric mucosa is not secreting IF because of either gastric mucosal atrophy or autoimmune destruction of parietal cells. In the autoimmune process antibodies are directed against the gastric parietal cells and/or IF itself. Because parietal cells also secrete hydrochloric (HCl) acid, in pernicious anemia there is a decrease in HCl in the stomach. An acid environment in the stomach is required for the secretion of IF. ● Pernicious anemia is a disease of insidious onset that begins in middle age or later (usually after age 40), with 60 years being the most common age at diagnosis. Pernicious anemia occurs frequently in persons of Northern European ancestry (particularly Scandinavians) and African Americans. Women. Other Causes of Cobalamin Deficiency. ● Cobalamin deficiency can also occur in patients who have had GI surgery (e.g., gastrectomy, gastric bypass); patients who have had a small bowel resection involving the ileum; and patients with Crohn's disease, ileitis, celiac disease, diverticula of the small intestine, or chronic atrophic gastritis (Table 30-8). ● In these cases, cobalamin deficiency results from the loss of IF-secreting gastric mucosal cells or impaired absorption of cobalamin in the distal ileum. ● Cobalamin deficiency is also found in excessive alcohol or hot tea ingestion, smoking, long-term users of H2-histamine receptor blockers and proton pump inhibitors, and those who are strict vegetarians. Clinical Manifestations ● General manifestations of anemia related to cobalamin deficiency develop because of tissue hypoxia (Table 30-3). ● GI manifestations include a sore, red, beefy, and shiny tongue; anorexia, nausea, and vomiting; and abdominal pain. ● Typical neuromuscular manifestations include weakness, paresthesias of the feet and hands, reduced vibratory and position senses, ataxia, muscle weakness, and impaired thought processes ranging from confusion to dementia. ● Because cobalamin deficiency–related anemia has an insidious onset, it may take several months for these manifestations to develop. Diagnostic Studies ● Laboratory data reflective of cobalamin deficiency anemia are presented in Table 30-6. The RBCs appear large (macrocytic) and have abnormal shapes. This structure contributes to erythrocyte destruction because the cell membrane is fragile. Serum cobalamin levels are reduced. ● Serum folate levels are also obtained. If they are normal and cobalamin levels are low, it suggests that megaloblastic anemia is due to a cobalamin deficiency. A serum test for anti-IF antibodies may be done that is specific for pernicious anemia. Because the potential for gastric cancer is increased in patients with pernicious anemia, an upper GI endoscopy and biopsy of the gastric mucosa may also be done. ● Testing of serum methylmalonic acid (MMA) (elevated mainly in cobalamin deficiency) and serum homocysteine (elevated in both cobalamin and folic acid deficiencies) helps determine the cause of this type of anemia. Interprofessional Care ● Regardless of how much is ingested, the patient is not able to absorb cobalamin if IF is lacking or if absorption in the ileum is impaired. For this reason, increasing dietary cobalamin does not correct this anemia. However, instruct the patient on adequate dietary intake to maintain good nutrition (Table 30-5). ● Parenteral vitamin B12 (cyanocobalamin or hydroxocobalamin) or intranasal cyanocobalamin (Nascobal, CaloMist) is the treatment of choice. Without cobalamin administration, these individuals will die in 1 to 3 years. A typical treatment schedule consists of 1000 mcg/day of cobalamin IM for 2 weeks and then weekly until the hemoglobin is normal, and then monthly for life. High-dose oral cobalamin and sublingual cobalamin are also available for those in whom GI absorption is intact. As long as supplemental cobalamin is used, the anemia can be reversed. However, if the person has had long-standing neuromuscular complications that may not be reversible, physical therapy may be warranted. Folic Acid deficiency: (3-5) signs & symptoms, medical treatment, nursing management, priorities of care, complications; nutrition-types of foods; labs Megaloblastic Anemia: Folic Acid Deficiency ● Folic acid (folate) deficiency also causes megaloblastic anemia. Folic acid is required for DNA synthesis leading to RBC formation and maturation. Common causes of folic acid deficiency are listed in Table 30-8. ● The clinical manifestations of folic acid deficiency are similar to those of cobalamin deficiency. The disease develops insidiously, and the patient's symptoms may be attributed to other coexisting problems (e.g., cirrhosis, esophageal varices). ● GI disturbances include dyspepsia and a smooth, beefy red tongue. The absence of neurologic problems is an important diagnostic finding and differentiates folic acid deficiency from cobalamin deficiency. ● The diagnostic findings for folic acid deficiency are presented in Table 30-6. In addition, the serum folate level is low (normal is 3 to 16 ng/mL [7 to 36 nmol/L]) and the serum cobalamin level is normal. ● Folic acid deficiency is treated by replacement therapy. ○ The usual dosage is 1 mg/day by mouth. In malabsorption states or with chronic alcoholism, up to 5 mg/day may be required. The duration of treatment depends on the reason for the deficiency. Encourage the patient to eat foods containing large amounts of folic acid (Table 30-5). Nursing Management: Megaloblastic Anemia ● Because of the familial predisposition for pernicious anemia, the most common type of cobalamin deficiency, patients who have a positive family history of pernicious anemia should be evaluated for symptoms. Although the disease cannot be prevented, early detection and treatment can lead to reversal of symptoms. Also consider signs and symptoms of other possible megaloblastic anemias and bring them to the attention of the HCP. ● Ongoing care focuses on ensuring good patient adherence with treatment. Carefully assess for neurologic difficulties that were not fully corrected by replacement therapy. Because the potential for gastric cancer may be increased in patients with atrophic gastritis-related pernicious anemia, the patient should have frequent and careful appropriate screenings. ● The nursing measures presented in eNursing Care Plan 30-1 for the patient with anemia are appropriate for the patient with cobalamin or folic acid deficiency anemia. In addition to these measures, ensure that injuries are not sustained because of the diminished sensitivity to heat and pain resulting from the neurologic impairment. Protect the patient from falling, burns, and trauma. If heat therapy is required, evaluate the patient's skin at frequent intervals to detect redness. Iron deficiency anemia: (3-5) signs & symptoms, medical treatment, nursing management, priorities of care, complications; lab; nutrition-types of foods Etiology ● Iron-deficiency anemia may develop as a result of inadequate dietary intake, malabsorption, blood loss, or hemolysis. ● Normal dietary iron intake is usually sufficient to meet the needs of men and older women, but it may be inadequate for individuals with higher iron needs (e.g., menstruating or pregnant women). ● Malabsorption of iron may occur after certain types of gastrointestinal (GI) surgery and in malabsorption syndromes.As iron absorption occurs in the duodenum, malabsorption syndromes may involve disease of the duodenum in which the absorption surface is altered or destroyed. Clinical Manifestations ● In the early course of iron-deficiency anemia, the patient may not have any symptoms. As the disease becomes chronic, any of the general manifestations of anemia may develop (Table 30-3). Specific clinical manifestations may occur related to iron-deficiency anemia. ● Pallor is the most common finding, and glossitis (inflammation of the tongue) is the second most common. ● Another finding is cheilitis (inflammation of the lips). ● In addition, the patient may report headache, paresthesias, and a burning sensation of the tongue, all of which are caused by lack of iron in the tissues. Diagnostic Studies ● Laboratory abnormalities characteristic of iron-deficiency anemia are presented in Table 30-6. ● Other diagnostic studies (e.g., stool occult blood test) are done to determine the cause of the iron deficiency. ● Endoscopy and colonoscopy may be used to detect GI bleeding. A bone marrow biopsy, if other tests are inconclusive. Interprofessional Care The main goal is to treat the underlying disease that is causing reduced intake (e.g., malnutrition, alcoholism) or absorption of iron Drug Therapy. Oral iron should be used whenever possible because it is inexpensive and convenient. Many iron preparations are available. When administering iron, consider the following five factors: 1. Iron is absorbed best from the duodenum and proximal jejunum. Therefore enteric-coated or sustained-release capsules are counterproductive and expensive. 2. The daily dose should provide 150 to 200 mg of elemental iron. This can be ingested in three or four daily doses, with each tablet or capsule of the iron preparation containing between 50 and 100 mg of iron (e.g., a 300-mg tablet of ferrous sulfate contains 60 mg of elemental iron). 3. Iron is best absorbed as ferrous sulfate (Fe2 ) in an acidic environment. For this reason and to avoid binding the iron with food, iron should be taken about an hour before meals, when the duodenal mucosa is most acidic. Taking iron with vitamin C (ascorbic acid) or orange juice, which contains ascorbic acid, enhances iron absorption. Gastric side effects, however, may necessitate ingesting iron with meals. 4. Undiluted liquid iron may stain the patient's teeth. Therefore it should be diluted and ingested through a straw. 5. GI side effects of iron administration may occur, including heartburn, constipation, and diarrhea. If side effects develop, the dose and type of iron supplement may be adjusted. For example, many individuals who need supplemental iron cannot tolerate ferrous sulfate because of the effects of the sulfate base. However, ferrous gluconate may be an acceptable substitute. Constipation is common, and the patient should be started on stool softeners and laxatives, if needed, when started on iron. Drug Alert Iron ________________________________________ • Some preparations of IV iron have a risk of an allergic reaction, and the patient should be monitored accordingly. • Oral iron should be taken about 1 hr before meals. • Vitamin C (ascorbic acid) enhances iron absorption. ● In some situations it may be necessary to administer iron parenterally. Parenteral use of iron is indicated for malabsorption, intolerance of oral iron, a need for iron beyond oral limits, or poor patient adherence in taking the oral preparations of iron. ● Parenteral iron can be given IM or IV. An iron-dextran complex (INFeD) contains 50 mg/mL of elemental iron available in 2-mL single-dose vials. ● Sodium ferrous gluconate and iron sucrose are alternatives and may carry less risk of life-threatening anaphylaxis.8 ● Because IM iron solutions may stain the skin, separate needles should be used for withdrawing the solution and for injecting the medication. Use a Z-track injection technique. Thrombocytopenia: (3-5) heparin-induced thrombocytopenia (HIT)/ thrombocytopenic purpura (ITP): signs & symptoms, medical treatment, nursing management, priorities of care, complications; labs Thrombocytopenia Etiology and Pathophysiology ● Thrombocytopenia is a reduction of platelets below 150,000/µL (150 × 109/L). Acute, severe, or prolonged decreases from this normal range can result in abnormal hemostasis that manifests as prolonged bleeding from minor trauma or spontaneous bleeding without injury. ● Although some agents are directly myelosuppressive (e.g., chemotherapy, ganciclovir [Cytovene]), the usual mechanism of thrombocytopenia caused by drugs is accelerated platelet destruction caused by antibodies. Antibodies attack the platelets when the offending agent binds to the platelet surface. Table 30-11 Causes of Acquired Thrombocytopenia • Immune thrombocytopenic purpura (ITP) • Thrombotic thrombocytopenic purpura (TTP) • Heparin-induced thrombocytopenia (HIT) • Disseminated intravascular coagulation (DIC) • Drugs • Chemotherapy drugs • Aspirin • Quinine • Infections • Viral infection (hepatitis C virus, HIV, cytomegalovirus) • Bacterial infection • Hematologic malignancy (leukemia, lymphoma, myeloma) Immune Thrombocytopenic Purpura. (ITP) ● The most common acquired thrombocytopenia is a syndrome of abnormal destruction of circulating platelets termed immune thrombocytopenic purpura (ITP). In ITP, platelets are coated with antibodies. Although these platelets function normally, when they reach the spleen the antibody-coated platelets are recognized as foreign and are destroyed by macrophages. Decreased platelet production contributes to ITP. Sometimes infection, such as Helicobacter pylori or viral infection, contributes to this disorder. Thrombotic Thrombocytopenic Purpura. (TTP) ● Thrombotic thrombocytopenic purpura (TTP) is an uncommon syndrome characterized by hemolytic anemia, thrombocytopenia, neurologic abnormalities, fever (in the absence of infection), and renal abnormalities; not all features are present in all patients.The disease is associated with enhanced aggregation of platelets, which form microthrombi that deposit in arterioles and capillaries. ● In most cases the syndrome is caused by the deficiency of a plasma enzyme (ADAMTS13) that usually breaks down the von Willebrand clotting factor (vWF) into normal size. (vWF is the most important protein-mediating platelet adhesion to damaged endothelial cells.) Without the enzyme, unusually large amounts of vWF attach to activated platelets, thereby promoting platelet aggregation. ● TTP is a medical emergency because bleeding and clotting occur simultaneously. Heparin-Induced Thrombocytopenia (HTP). ● One of the risks associated with the broad and increasing use of heparin is the development of the life-threatening condition called heparin-induced thrombocytopenia (HIT), also called heparin-induced thrombocytopenia and thrombosis syndrome (HITTS). ● Although the major clinical problem of HIT is venous thrombosis, arterial thrombosis can also develop. Deep vein thromboses and pulmonary emboli most commonly result as a complication of the thromboses. ● In HIT, platelet destruction and vascular endothelial injury are the two major responses to an immune-mediated response to heparin. Clinical Manifestations ● Many patients with thrombocytopenia are asymptomatic. The most common symptom is bleeding, usually mucosal or cutaneous. ● Mucosal bleeding may manifest as epistaxis and gingival bleeding, and large bullous hemorrhages may appear on the buccal mucosa because of the lack of vessel protection by the submucosal tissue. ● Bleeding into the skin is manifested as petechiae, purpura, or superficial ecchymoses (Fig. 30-6). Petechiae are small, flat, pinpoint, red or reddish brown microhemorrhages. When the platelet count is low, RBCs may leak out of the blood vessels and into the skin to cause petechiae. When petechiae are numerous, the resulting reddish skin bruise is called purpura. Larger purplish lesions caused by hemorrhage are termed ecchymoses. Ecchymoses may be flat or raised; pain and tenderness sometimes are present. ● Prolonged bleeding after routine procedures such as venipuncture or IM injection may indicate thrombocytopenia. Because the bleeding may be internal, be aware of manifestations that reflect this type of blood loss, including weak­ness, fainting, dizziness, tachycardia, abdominal pain, and hypotension. ● The major complication of thrombocytopenia is hemorrhage. The hemorrhage may be insidious or acute, and internal or external. It may occur in any area of the body, including the joints, retina, and brain. Cerebral hemorrhage may be fatal. Insidious hemorrhage may first be detected by discovering the anemia that accompanies blood loss. ● Because thrombocytopenia can be accompanied by vascular thromboses, signs and symptoms of vascular ischemic problems can manifest (see Chapter 37). For example, subtle confusion, headache, or even serious manifestations such as seizures and coma due to TTP-related thrombosis may be seen. Because signs and symptoms may be subtle, astute and thorough assessment of the patient is essential. Diagnostic Studies ● The platelet count is decreased in thrombocytopenia. Any reduction below 150,000/µL (150 × 109/L) may be termed thrombocytopenia. However, prolonged bleeding from trauma or injury does not usually occur until platelet counts are less than 50,000/µL (50 × 109/L). When the count drops below 20,000/µL (20 × 109/L), spontaneous, life-threatening hemorrhages (e.g., intracranial bleeding) can occur. Platelet transfusions are generally not recommended until the count is below 10,000/µL (10 × 109/L), unless the patient is actively bleeding. Nursing Management: Thrombocytopenia Nursing Assessment Subjective and objective data that should be obtained from a patient with thrombocytopenia are presented in Table 30-13. Table 30-13 Nursing Assessment Thrombocytopenia ________________________________________ Objective Data General Fever, lethargy Integumentary Petechiae, ecchymoses, purpura Gastrointestinal Splenomegaly, abdominal distention; guaiac-positive stools Possible Diagnostic Findings Platelet count 150,000/µL (150 × 109/L), prolonged bleeding time, ↓ hemoglobin and hematocrit; normal or ↑ megakaryocytes in bone marrow examination Nursing Implementation Health Promotion. ● Discourage excessive use of over-the-counter (OTC) medications known to be possible causes of acquired thrombocytopenia. Many medications contain aspirin as an ingredient. Acute Care. ● The goal during acute episodes of thrombocytopenia is to prevent or control hemorrhage. Emphasize that a seemingly minor nosebleed or new petechiae may indicate potential hemorrhage and the HCP should be notified. ● Bleeding from the posterior nasopharynx may be difficult to detect because the blood may be swallowed. If a subcutaneous injection is unavoidable, the use of a small-gauge needle and application of direct pressure for at least 5 to 10 minutes after injection is indicated, or application of an ice pack may be helpful. Avoid IM injections. Help the patient understand the importance of adherence to self-care measures that reduce the risk of bleeding (Table 30-15). Table 30-15 Patient & Caregiver Teaching Thrombocytopenia ________________________________________ Include the following instructions when teaching a patient or caregiver the precautions to take when the platelet count is low. 1. Notify your HCP of any manifestations of bleeding. These include the following: • Black, tarry, or bloody bowel movements • Black or bloody vomit, sputum, or urine • Bruising or small red or purple spots on the skin • Bleeding from the mouth or anywhere in the body • Headache or changes in how well you can see • Difficulty talking, sudden weakness of an arm or leg, confusion 2. Ask your HCP regarding restrictions in your normal activities, such as vigorous exercise or lifting weights. Generally, walking can be done safely and should be done with sturdy shoes or slippers. If you are weak and at risk for falling, get help or supervision when getting out of bed or chair. 3. Do not blow your nose forcefully; gently pat it with a tissue if needed. For a nosebleed, keep your head up and apply firm pressure to the nostrils and bridge of your nose. If bleeding continues, place an ice bag over the bridge of your nose and the nape of your neck. If you are unable to stop a nosebleed after 10 min, call your HCP. 4. Do not bend down with your head lower than your waist. 5. Prevent constipation by drinking plenty of fluids. Do not strain when having a bowel movement. Your HCP may prescribe a stool softener. Do not use a suppository, an enema, or a rectal thermometer without the permission of your HCP. 6. Shave only with an electric razor. Do not use blades. 7. Do not tweeze your eyebrows or other body hair. 8. Do not puncture your skin, such as getting tattoos or body piercing. 9. Avoid using any medication that can prolong bleeding, such as aspirin. Other medications and herbs can have similar effects. If you are unsure about any medication, ask your HCP or pharmacist about it in relation to your thrombocytopenia. 10. Use a soft-bristle toothbrush to prevent injuring the gums. Flossing is usually safe if it is done gently using the thin tape floss. Do not use alcohol-based mouthwashes, since they can dry your gums and increase bleeding. 11. Women who are menstruating should keep track of the number of pads that are used per day. When you start using more pads per day than usual or bleed more days, notify your HCP. Do not use tampons; only use sanitary pads. 12. Ask your HCP before you have any invasive procedures done, such as a dental cleaning, manicure, or pedicure. ● In a woman with thrombocytopenia, menstrual blood loss may exceed the usual amount and duration. Counting sanitary napkins used during menses is another important intervention to detect excess blood loss. Blood loss of 50 mL will completely soak a sanitary napkin. ● Suppression of menses with hormonal agents may be indicated during predictable periods of thrombocytopenia (e.g., during chemotherapy and HSCT) to reduce blood loss from menses. Multiple Myeloma: (3) signs & symptoms, medical treatment, nursing management, priorities of care, complications; labs ● Multiple myeloma, or plasma cell myeloma, is a condition in which neoplastic plasma cells infiltrate the bone marrow and destroy bone. Etiology and Pathophysiology ● The cause of multiple myeloma is unknown. It is possible that exposure to radiation, organic chemicals (such as benzene), metals, herbicides, and insecticides may play a role. ● The disease process involves excessive production of plasma cells. Plasma cells are activated B cells, which produce immunoglobulins (antibodies) that normally protect the body. However, in multiple myeloma instead of a variety of plasma cells producing antibodies to fight different infections, myeloma tumors produce monoclonal antibodies. Monoclonal means they are all of one kind, making them ineffective and even harmful. Not only do they not fight infections, but they also infiltrate the bone marrow. ● Furthermore, plasma cell production of excessive and abnormal amounts of cytokines (interleukins [ILs]; IL-4, IL-5, and IL-6) also plays an important role in the pathologic process of bone destruction. As myeloma protein increases, normal plasma cells are reduced, which further compromises the body's normal immune response. Proliferation of malignant plasma cells and the overproduction of immunoglobulin and proteins result in the end-organ effects of myeloma on the bone marrow, bone, and kidneys and possibly the spleen, lymph nodes, liver, and even heart muscle. Clinical Manifestations ● Multiple myeloma develops slowly and insidiously. The patient often does not manifest symptoms until the disease is advanced, at which time skeletal pain is the major manifestation. ● Pain in the pelvis, spine, and ribs is particularly common and is triggered by movement. Diffuse osteoporosis develops as the myeloma protein destroys bone. Osteolytic lesions are seen in the skull, vertebrae, and ribs. Vertebral destruction can lead to collapse of vertebrae with ensuing compression of the spinal cord. Loss of bone integrity can lead to the development of pathologic fractures. ● Bony degeneration also causes calcium to be lost from bones, eventually causing hypercalcemia. ● Hypercalcemia may cause renal, GI, or neurologic manifestations such as polyuria, anorexia, confusion, and ultimately seizures, coma, and cardiac problems. ● A serum hyperviscosity syndrome leading to cerebral, pulmonary, renal, and other organ dysfunction can occur in some patients. Even without hyperviscosity, high protein levels caused by the myeloma protein can result in renal tubular obstruction, interstitial nephritis, and renal failure. ● The patient may also display manifestations of anemia, thrombocytopenia, neutropenia, and immune dysfunction, all of which are related to the replacement of normal bone marrow with plasma cells. Diagnostic Studies ● Evaluating multiple myeloma involves laboratory, radiologic, and bone marrow examination. M protein is found in the blood and urine. Possible findings include pancytopenia, hypercalcemia, Bence Jones protein in the urine, and an elevated serum creatinine. ● Skeletal bone surveys, MRI, and/or PET and CT scans show distinct lytic areas of bone erosions; generalized thinning of the bones; or fractures, especially in the vertebrae, ribs, pelvis, and bones of the thigh and upper arms. ● Bone marrow analysis shows significantly increased numbers of plasma cells in the bone marrow. ● The simplest measure of staging and prognosis in multiple myeloma is based on blood levels of two markers: β2-microglobulin and albumin. Interprofessional Care ● Interprofessional care involves managing both the disease and its symptoms. ● The current treatment options include corticosteroids, chemotherapy, immunotherapy, and targeted therapy, and HSCT. ● Ambulation and adequate hydration are used to treat hypercalcemia, dehydration, and potential renal damage. ● Weight bearing helps the bones resorb some calcium, and fluids dilute calcium and prevent protein precipitates from causing renal tubular obstruction. ● Control of pain and prevention of pathologic fractures are other goals of management. Analgesics, orthopedic supports, and localized radiation help reduce the skeletal pain. ● Kyphoplasty is sometimes used to control spine vertebral disease. Kyphoplasty is a minimally invasive procedure (usually done in an interventional radiology area) that injects cement to stabilize the vertebral compression. ● Bisphosphonates, such as pamidronate (Aredia) and zoledronic acid (Zometa), inhibit bone breakdown and are used for the treatment of skeletal pain and hypercalcemia. They inhibit bone resorption without inhibiting bone formation and mineralization. They are given monthly by IV infusion. ● Chemotherapy with corticosteroids is usually the first treatment recommended for multiple myeloma. It is used to reduce the number of plasma cells. ● Immunotherapy and targeted therapy are also used to treat multiple myeloma. Immunomodulator drugs (described in Chapter 15) include thalidomide (Thalidomide), lenalidomide (Revlimid), and pomalidomide (Pomalyst). Panobinostat (Farydak) is a drug that can affect which genes are active inside of cells. Daratumumab (Darzalex) is a monoclonal antibody against CD38, which is expressed on the surface of myeloma cells. ● Drugs may also be used to treat complications of multiple myeloma. For example, allopurinol may be given to reduce hyperuricemia, and IV furosemide (Lasix) promotes renal excretion of calcium. In some patients, the levels of plasma proteins are so high that it causes a hyperviscosity syndrome leading to neurologic changes, renal insufficiency, and other problems related to lack of blood flow. In this instance plasmapheresis is used. Nursing Management: Multiple Myeloma ● A major focus of care relates to the bone involvement and sequelae from bone breakdown. ● Maintaining adequate hydration is a primary nursing consideration to minimize problems from hypercalcemia. IV fluids may be administered to attain a urine output of 1.5 to 2 L/day if the patient does not already have renal compromise. ● Because of the potential for pathologic fractures, use caution when moving and ambulating the patient. A slight twist or strain in the wrong area (e.g., a weak area in the patient's bones) may be sufficient to cause a fracture. ● In addition, the development of peripheral neuropathy is common with several therapies for multiple myeloma and can contribute to discomfort, the inability to perform basic activities of daily living, and the risk of injury from falling. ● Pain medications and therapies can also lead to severe constipation. ● Analgesics, such as nonsteroidal antiinflammatory drugs, acetaminophen, or an acetaminophen/opioid combination, may be more effective than opioids alone in diminishing bone pain. Braces, especially for the spine, may also help control pain. ● Patients may also be at risk for deep vein thrombosis related to chemotherapy and immobility and should have appropriate preventive measures employed. Hemophilia: (3-5) signs & symptoms, medical treatment, nursing management, priorities of care, complications ● Hemophilia is an X-linked recessive genetic disorder caused by a defective or deficient coagulation factor. ○ The two major types of hemophilia, which can occur in mild to severe forms, are hemophilia A (classic hemophilia, factor VIII deficiency) and hemophilia B (Christmas disease, factor IX deficiency). von Willebrand disease is a related disorder involving a deficiency of the von Willebrand coagulation protein. Factor VIII is synthesized in the liver and circulates as a complex with von Willebrand factor (vWF). Clinical Manifestations and Complications ● All clinical manifestations relate to bleeding, and any bleeding episode in persons with hemophilia may lead to a life-threatening hemorrhage. ● Clinical manifestations and complications related to hemophilia include: ● (1) slow, persistent, prolonged bleeding from minor trauma and small cuts; ● (2) delayed bleeding after minor injuries (the delay may be several hours or days); ● (3)uncontrollable hemorrhage after dental extractions or irritation of the gingiva with a hard-bristle toothbrush; ● (4) epistaxis, especially after a blow to the face; ● (5) GI bleeding from ulcers and gastritis; ● (6) hematuria and potential renal failure from GU trauma and splenic rupture resulting from falls or abdominal trauma; ● (7) ecchymoses and subcutaneous hematomas (Fig. 30-7) and possible compartment syndrome; ● (8) neurologic signs, such as pain, anesthesia, and paralysis, which may develop from nerve compression caused by hematoma formation; and ● (9) hemarthrosis (bleeding into the joints) (Fig. 30-8), which may lead to joint injury and deformity severe enough to cause crippling (most commonly in knees, elbows, shoulders, hips, and ankles). Diagnostic Studies Laboratory studies are used to determine the type of hemophilia present. Any factor deficiency within the intrinsic system (factor VIII, IX, XI, or XII or vWF) will yield the laboratory results presented in Table 30-17. Table 30-17 Laboratory Results in Hemophilia Test Results Prothrombin time Normal. No involvement of extrinsic system Thrombin time Normal. No impairment of thrombin-fibrinogen reaction Platelet count Normal. Adequate platelet production Partial thromboplastin time Prolonged because of deficiency in intrinsic clotting system factor Bleeding time Prolonged in von Willebrand disease because of structurally defective platelets. Normal in hemophilia A and B because platelets not affected Factor assays Reductions of factor VIII in hemophilia A, factor IX in hemophilia B, vWF in von Willebrand disease Interprofessional Care ● Care for persons with hemophilia or von Willebrand disease requires ○ (1) preventive care ○ (2) the use of replacement therapy during acute bleeding episodes and as prophylaxis, and ○ (3) the treatment of the complications of the disease and its therapy. ○ Replacement of deficient clotting factors is the primary means of supporting a patient with hemophilia. In addition to treating acute crises, replacement therapy may be given before surgery and dental care as a prophylactic measure. Nursing Management: Hemophilia Nursing Implementation Acute Care. Interventions are related primarily to controlling bleeding and include the following: 1. Stop the topical bleeding as quickly as possible by applying