Chapter 30 Copyright © 2017, Elsevier Inc. All Rights Reserved. • A deficiency in •Number of erythrocytes (RBCs) •Quantity of hemoglobin •Volume of packed RBCs (hematocrit) Copyright © 2017, Elsevier Inc. All Rights Reserved. Copyright © 2017, Elsevier Inc. All Rights Reserved. • RBC function •Transport oxygen (O2) from lungs to systemic tissues •Carry carbon dioxide from tissues to lungs Copyright © 2017, Elsevier Inc. All Rights Reserved. • Not a specific disease • Manifestation of a pathologic process • Diagnosed based on •Complete blood count (CBC) •Reticulocyte count •Peripheral blood smear Copyright © 2017, Elsevier Inc. All Rights Reserved. • Classified as •Morphologic • Cellular characteristics • Descriptive, objective laboratory information •Etiologic • Underlying cause Copyright © 2017, Elsevier Inc. All Rights Reserved. • Caused by body’s response to tissue hypoxia •Manifestations vary based on rate of development, severity of anemia, presence of co-existing disease • Hemoglobin (Hgb) levels are used to determine severity of anemia Copyright © 2017, Elsevier Inc. All Rights Reserved. 7 • Pallor •↓ Hemoglobin •↓ Blood flow to the skin • Jaundice •↑ Concentration of serum bilirubin • Pruritus •↑ Serum and skin bile salt concentrations Copyright © 2017, Elsevier Inc. All Rights Reserved. • Result from additional attempts by heart and lungs to provide adequate O2 to tissues • Cardiac output maintained by increasing heart rate and stroke volume Copyright © 2017, Elsevier Inc. All Rights Reserved. • Subjective Data •Important health information • Past health history • Medications • Surgery or other treatments • Dietary history •Functional health patterns Copyright © 2017, Elsevier Inc. All Rights Reserved. • Objective Data •General •Integumentary •Respiratory •Cardiovascular •Gastrointestinal •Neurologic •Diagnostic findings Copyright © 2017, Elsevier Inc. All Rights Reserved. • Fatigue • Imbalanced nutrition: Less than body requirements • Ineffective health management •Assume normal activities of daily living •Maintain adequate nutrition •Develop no complications related to anemia Copyright © 2017, Elsevier Inc. All Rights Reserved. • Patients with fatigue •Alternate rest and activity •Prioritize activities • Accommodate energy levels • Maximize O2 supply for vital functions •Provide assistance to minimize risk for injury •Evaluate nutritional needs Copyright © 2017, Elsevier Inc. All Rights Reserved. • Nursing care varies •Numerous causes of anemia •Patient specific needs Copyright © 2017, Elsevier Inc. All Rights Reserved. (©Thinkstock/Comstock/Thinkstock) • K.L. is a 24-year-old female with complaints of ↑ lethargy, inability to pay attention at work, and headache. • She has a pale, beefy tongue and inflamed lip. • She tells you she is breastfeeding her 4month old. • What should you do? Copyright © 2017, Elsevier Inc. All Rights Reserved. • Anemia is not normal •More common in the 70s and beyond •Often related to an underlying cause • Signs and symptoms may go unrecognized •Other health issues •May be mistaken for normal aging Copyright © 2017, Elsevier Inc. All Rights Reserved. • Erythropoietin (EPO) is a glycoprotein primarily produced in the kidneys (10% in liver) •↑ Number of stem cells committed to RBC production •Shortens time to mature RBCs Copyright © 2017, Elsevier Inc. All Rights Reserved. • Life span of an RBC is 120 days • Three alterations in erythropoiesis may decrease RBC production: •Decreased hemoglobin synthesis •Defective DNA synthesis in RBCs •Diminished availability of erythrocyte precursors Copyright © 2017, Elsevier Inc. All Rights Reserved. • Most common nutritional disorder in the world • Most susceptible •Very young •Poor diet •Women in reproductive years Copyright © 2017, Elsevier Inc. All Rights Reserved. • Inadequate dietary intake •5% to 10% of ingested iron is absorbed • Malabsorption •Iron absorption occurs in the duodenum •Diseases or surgery that alter, destroy, or remove absorption surface of this area of intestine cause anemia Copyright © 2017, Elsevier Inc. All Rights Reserved. • Blood loss •2 mL whole blood contain 1 mg iron •Major cause of iron deficiency in adults •Chronic blood loss most commonly through GI and GU systems • Hemolysis • Pregnancy contributes to this condition Copyright © 2017, Elsevier Inc. All Rights Reserved. • General manifestations of anemia •Pallor is most common •Glossitis is second • Inflammation of tongue •Cheilitis is also found • Inflammation of lips Copyright © 2017, Elsevier Inc. All Rights Reserved. • Laboratory findings •Hgb, Hct, MCV, MCH, MCHC, reticulocytes, serum iron, TIBC, bilirubin, platelets • Stool occult blood test • Endoscopy • Colonoscopy Copyright © 2017, Elsevier Inc. All Rights Reserved. • Goal •Treat underlying disease causing reduced intake or absorption of iron • Replace iron •Nutritional therapy •Oral iron supplements •Transfusion of packed RBCs Copyright © 2017, Elsevier Inc. All Rights Reserved. (©Thinkstock/Comstock/Thinkstock) • K.L. has lab work done. Her results are back, and her lab values show •↓ Hct, Hgb, MCV, iron, ferritin, and transferrin •↑ TIBC • What is the likely cause of her anemia? Copyright © 2017, Elsevier Inc. All Rights Reserved. • Oral iron •Inexpensive •Convenient •Factors to consider • Enteric-coated or sustained-release capsules are counterproductive • Daily dose is 150 to 200 mg Copyright © 2017, Elsevier Inc. All Rights Reserved. • Oral iron •Factors to consider • Best absorbed as ferrous sulfate in an acidic environment • Liquid iron should be diluted and ingested through a straw • Side effects • Heartburn, constipation, diarrhea Copyright © 2017, Elsevier Inc. All Rights Reserved. • Parenteral iron •Indicated for malabsorption, oral iron intolerance, need for iron beyond normal limits, poor patient compliance •Can be given IM or IV •IM may stain skin • Z-track Copyright © 2017, Elsevier Inc. All Rights Reserved. • At-risk groups •Premenopausal women •Pregnant women •Persons from low socioeconomic backgrounds •Older adults •Individuals experiencing blood loss Copyright © 2017, Elsevier Inc. All Rights Reserved. • Diet teaching • Supplemental iron • Discuss diagnostic studies • Emphasize compliance • Iron therapy for 2 to 3 months after Hgb levels return to normal Copyright © 2017, Elsevier Inc. All Rights Reserved. (©Thinkstock/Comstock/Thinkstock) • How should K.L.’s anemia be treated? Copyright © 2017, Elsevier Inc. All Rights Reserved. (©Thinkstock/Comstock/Thinkstock) • K.L. tells you she can’t afford medicine. • She has 3 children and her husband is out of work. • Her income is barely keeping the household going. Copyright © 2017, Elsevier Inc. All Rights Reserved. (©Thinkstock/Comstock/Thinkstock) • Anything that costs money is out of the question. • What can you teach K.L. about effects of anemia if left untreated? Copyright © 2017, Elsevier Inc. All Rights Reserved. • A group of diseases involving inadequate production of normal Hgb • Results in decreased erythrocyte production Copyright © 2017, Elsevier Inc. All Rights Reserved. • Problem with globulin protein •Abnormal Hgb synthesis • Hemolysis occurs Copyright © 2017, Elsevier Inc. All Rights Reserved. • Autosomal recessive genetic basis • One thalassemic gene •Thalassemia minor • Two thalassemic genes •Thalassemia major Copyright © 2017, Elsevier Inc. All Rights Reserved. • Thalassemia minor •Frequently asymptomatic • Moderate anemia • Microcytosis • Hypochromia •Body adapts to reduction of Hgb – thus no treatment is indicated Copyright © 2017, Elsevier Inc. All Rights Reserved. • Thalassemia major •Life-threatening •Physical and mental growth often retarded •Pale and jaundiced •Splenomegaly, hepatomegaly, and cardiomyopathy •Symptoms develop in childhood Copyright © 2017, Elsevier Inc. All Rights Reserved. • Thalassemia major •As the bone marrow responds to the deficit of O2 -carrying capacity of the blood, RBC production is stimulated, and marrow becomes packed with immature erythroid precursors that die •Chronic bone marrow hyperplasia •Hepatitis C Copyright © 2017, Elsevier Inc. All Rights Reserved. • No specific drug or diet is effective in treating thalassemia • Thalassemia major •Blood transfusions or exchange transfusions with chelating agents that bind to iron to reduce iron overloading •Splenectomy Copyright © 2017, Elsevier Inc. All Rights Reserved. • Group of disorders •Caused by impaired DNA synthesis •Presence of megaloblasts • Majority result from deficiency in •Cobalamin (vitamin B12) •Folic acid Copyright © 2017, Elsevier Inc. All Rights Reserved. • Intrinsic factor (IF) •Protein secreted by parietal cells of gastric mucosa • IF is required for cobalamin absorption in the distal ileum •If IF is not secreted, cobalamin will not be absorbed Copyright © 2017, Elsevier Inc. All Rights Reserved. • Most commonly caused by pernicious anemia •Which is caused by an absence of IF •Insidious onset •Begins in middle age or later •Predominant in Scandinavians and African Americans Copyright © 2017, Elsevier Inc. All Rights Reserved. • Can also occur: •GI surgery •Chronic diseases of the GI tract •Excessive alcohol or hot tea ingestion •Smoking •Long-term users of H2-histamine receptor blockers and proton pump inhibitors •Strict vegetarians Copyright © 2017, Elsevier Inc. All Rights Reserved. • General manifestations of anemia develop slowly due to tissue hypoxia •GI manifestations: • Sore tongue, anorexia, nausea, vomiting, and abdominal pain •Neuromuscular manifestations: • Weakness, paresthesias of feet and hands, ↓ vibratory and position senses, ataxia, muscle weakness, and impaired thought processes Copyright © 2017, Elsevier Inc. All Rights Reserved. • Macrocytic RBCs have abnormal shapes and fragile cell membranes • Serum cobalamin levels are decreased • Normal serum folate levels and low cobalamin levels suggest megaloblastic anemia is due to cobalamin deficiency • Upper GI endoscopy with biopsy of gastric mucosa Copyright © 2017, Elsevier Inc. All Rights Reserved. • Parenteral or intranasal administration of cobalamin is treatment of choice •Patients will die in 1-3 years without treatment •This anemia can be reversed with ongoing treatment but long-standing neuromuscular complications may not be reversible Copyright © 2017, Elsevier Inc. All Rights Reserved. • Also a cause of megaloblastic anemia • Folic acid is required for DNA synthesis •RBC formation and maturation • Clinical manifestations are similar to those of cobalamin deficiency, but absence of neurologic problems differentiates them Copyright © 2017, Elsevier Inc. All Rights Reserved. • Common causes •Dietary deficiency •Malabsorption syndromes •Drugs •Increased requirement •Alcohol abuse and anorexia •Loss during hemodialysis Copyright © 2017, Elsevier Inc. All Rights Reserved. • Serum folate level is low •Normal is 3 to 16 mg/mL (7 to 36 mol/L) • Serum cobalamin level is normal • Treated by replacement therapy •Usual dose is 1 mg per day by mouth • Encourage patient to eat foods with large amounts of folic acid Copyright © 2017, Elsevier Inc. All Rights Reserved. • Early detection and treatment • Ensure safety •Diminished sensations to heat and pain from neurologic impairment • Focus on compliance with treatment • Regular screening for gastric cancer Copyright © 2017, Elsevier Inc. All Rights Reserved. At an outpatient clinic, K.L.’s 78-year-old grandma is found to have a Hgb of 8.7 g/dL (87 g/L) and a Hct of 35%. Based on the most common cause of these findings in the older adult, the nurse collects information regarding a. a history of jaundice and black tarry stools. b. a 3-day diet recall of the foods the patient has eaten. c. any drugs that have depressed the function of the bone marrow. d. a history of any chronic diseases such as cancer or renal disease. Copyright © 2017, Elsevier Inc. All Rights Reserved. • Caused by •Chronic inflammation •Autoimmune and infectious disorders • HIV, hepatitis, malaria •Heart failure •Malignant diseases •Bleeding episodes Copyright © 2017, Elsevier Inc. All Rights Reserved. • Associated with •Underproduction of RBCs •Mild shortening of RBC survival • Normocytic, normochromic, and hypoproliferative RBCs • Usually a mild anemia but can become severe if the underlying disorder is untreated Copyright © 2017, Elsevier Inc. All Rights Reserved. Copyright © 2017, Elsevier Inc. All Rights Reserved. • Anemia of chronic disease findings •↑ Serum ferritin •↑ Iron stores •Normal folate and cobalamin levels • Treating underlying cause is best •Rarely blood transfusions •Conservative use of erythropoietin therapy Copyright © 2017, Elsevier Inc. All Rights Reserved. • Pancytopenia •Decrease in all blood cell types • Red blood cells (RBCs) • White blood cells (WBCs) • Platelets • Hypocellular bone marrow • Ranges from chronic to critical Copyright © 2017, Elsevier Inc. All Rights Reserved. • Rare •Annual rate of 2-5 new cases/million/year • Usually acquired •Idiopathic •An autoimmune basis is presumed Copyright © 2017, Elsevier Inc. All Rights Reserved. • Abrupt or gradual development • Symptoms caused by suppression of any or all bone marrow elements • General manifestations of anemia •Fatigue, dyspnea •Cardiovascular and cerebral responses •Neutropenia Copyright © 2017, Elsevier Inc. All Rights Reserved. • Diagnosis confirmed by laboratory studies •Low Hgb, WBC, and platelet values •Low reticulocyte count •Elevated serum iron and TIBC •Hypocellular bone marrow with increased fat content (yellow marrow) Copyright © 2017, Elsevier Inc. All Rights Reserved. • Identify and remove causative agent (when possible) • Provide supportive care until pancytopenia reverses • Prevent complications from infection • Prevent hemorrhage Copyright © 2017, Elsevier Inc. All Rights Reserved. • Prognosis of severe untreated aplastic anemia is poor • Advances in treatment options have significantly improved outcomes •Immune therapies and bone marrow transplantation can be curative Copyright © 2017, Elsevier Inc. All Rights Reserved. • Anemia resulting from blood loss may be caused by either acute or chronic problems •Acute blood loss occurs as a result of sudden hemorrhage •Sources of chronic blood loss are similar to those of iron-deficiency anemia Copyright © 2017, Elsevier Inc. All Rights Reserved. • Causes of sudden hemorrhage •Trauma •Complications of surgery •Conditions or diseases that disrupt vascular integrity • Hypovolemic shock • Compensatory increased plasma volume with diminished O2 -carrying RBCs Copyright © 2017, Elsevier Inc. All Rights Reserved. • Caused by body’s attempts to maintain adequate blood volume and meet oxygen requirements • Clinical signs and symptoms are more important than laboratory values Copyright © 2017, Elsevier Inc. All Rights Reserved. • Pain •Internal hemorrhage • Tissue distention, organ displacement, nerve compression •Retroperitoneal bleeding • Numbness • Pain in lower extremities • Shock is major complication Copyright © 2017, Elsevier Inc. All Rights Reserved. • With sudden blood volume loss, values may seem normal or high for 2 to 3 days • Once plasma volume is replaced, low RBC concentrations become evident •Low RBC, Hgb, and Hct levels show up and reflect actual blood loss Copyright © 2017, Elsevier Inc. All Rights Reserved. • • • • Replacing blood volume to prevent shock Identifying source of hemorrhage and stopping blood loss Correcting RBC loss Providing supplemental iron Copyright © 2017, Elsevier Inc. All Rights Reserved. • May be impossible to prevent if caused by trauma • Postoperative patients •Monitor blood loss •Administer blood products for anemia • No need for long-term treatment Copyright © 2017, Elsevier Inc. All Rights Reserved. • Sources of chronic blood loss: •Bleeding ulcer •Hemorrhoids •Menstrual and postmenopausal blood loss • Management involves •Identifying the source •Stopping the bleeding •Providing supplemental iron as needed Copyright © 2017, Elsevier Inc. All Rights Reserved. • Destruction or hemolysis of RBCs at a rate that exceeds production •Caused by problems intrinsic or extrinsic to the RBCs • Intrinsic forms are usually hereditary and result from defects in RBCs themselves • RBCs are normal in acquired forms that are more common. Damage is caused by external factors. Copyright © 2017, Elsevier Inc. All Rights Reserved. Copyright © 2017, Elsevier Inc. All Rights Reserved. • General manifestations of anemia • Specific manifestations including •Jaundice •Enlargement of the spleen and liver • Maintenance of renal function is a major focus of treatment Copyright © 2017, Elsevier Inc. All Rights Reserved. (©Thinkstock/Comstock/Thinkstock) • C.P. is an African American woman with complaints of severe joint and abdominal pain, frequent urination during past 2 nights, and knee swelling. Copyright © 2017, Elsevier Inc. All Rights Reserved. (©Thinkstock/Comstock/Thinkstock) • She reports a history of feeling fatigued but attributed it to her active lifestyle. • What should you do? Copyright © 2017, Elsevier Inc. All Rights Reserved. • Group of inherited, autosomal recessive disorders •Characterized by presence of an abnormal form of Hgb in RBC •Genetic disorder usually identified during routine neonatal screening •Incurable and often fatal by middle age Copyright © 2017, Elsevier Inc. All Rights Reserved. • Abnormal hemoglobin, HgbS, causes the RBC to stiffen and elongate •Erythrocytes take on a sickle shape in response to ↓ O2 levels •Substitution of valine for glutamic acid on the β-globin chain of hemoglobin Copyright © 2017, Elsevier Inc. All Rights Reserved. • Substitution of valine for glutamic acid on the β-globin chain of hemoglobin Copyright © 2017, Elsevier Inc. All Rights Reserved. • Types of SCD •Sickle cell anemia • Most severe • Homozygous for hemoglobin S (HgbSS) •Sickle cell thalassemia •Sickle cell HgbC disease •Sickle cell trait (HgbAS) Copyright © 2017, Elsevier Inc. All Rights Reserved. Copyright © 2017, Elsevier Inc. All Rights Reserved. • The major pathophysiologic event of this disease •Triggered by low O2 tension in blood •Infection is most common precipitating factor •Initially, sickling is reversible with reoxygenation Copyright © 2017, Elsevier Inc. All Rights Reserved. • Severe, painful, acute exacerbation of sickling causes a vaso-occlusive crisis • Severe capillary hypoxia eventually leads to tissue necrosis • Life-threatening shock is a result of severe O2 depletion of the tissues and a reduction of the circulating fluid volume Copyright © 2017, Elsevier Inc. All Rights Reserved. (©Thinkstock/Comstock/Thinkstock) • In taking a more complete history, C.P. tells you she had one similar episode of symptoms (severe joint and abdominal pain, frequent urination during past 2 nights, fatigue, and knee swelling) when she had an acute attack of pneumonia 2 months ago. Copyright © 2017, Elsevier Inc. All Rights Reserved. (©Thinkstock/Comstock/Thinkstock) • What is unusual about her episode of pneumonia? Copyright © 2017, Elsevier Inc. All Rights Reserved. • Typical patient is asymptomatic except during sickling episodes • Symptoms may include •Pain from tissue hypoxia and damage •Pallor of mucous membranes •Jaundice from hemolysis • Prone to gallstones (cholelithiasis) Copyright © 2017, Elsevier Inc. All Rights Reserved. Copyright © 2017, Elsevier Inc. All Rights Reserved. • Infection is a major cause of morbidity and mortality •Function of spleen becomes compromised from sickled RBCs • Autosplenectomy is a result of scarring •Pneumococcal pneumonia most common •Severe infections can cause aplastic crisis • Can lead to shutdown of RBC production Copyright © 2017, Elsevier Inc. All Rights Reserved. • Acute chest syndrome •Pulmonary complications that include pneumonia, tissue infarction, and fat embolism •Characterized by fever, chest pain, cough, pulmonary infiltrates, and dyspnea •Leads to multiple serious complications Copyright © 2017, Elsevier Inc. All Rights Reserved. • Peripheral blood smear • Sickling test • Electrophoresis of hemoglobin • Skeletal x-rays • Magnetic resonance imaging (MRI) • Doppler studies • X-rays Copyright © 2017, Elsevier Inc. All Rights Reserved. (©Thinkstock/Comstock/Thinkstock) • C.P. tells you that 2 days ago she went hiking in the mountains. • She is sure her swollen knee is from the hike. • Physical examination showed an enlarged spleen and an enlarged, inflamed knee joint. • What should be done next? Copyright © 2017, Elsevier Inc. All Rights Reserved. • Care is directed toward •Prevention of sequelae from disease •Alleviating manifestations from complications •Minimizing end-organ damage •Promptly treating serious sequelae Copyright © 2017, Elsevier Inc. All Rights Reserved. • Hospitalized patients in sickle cell crisis •O2 for hypoxia and to control sickling •Vigilance for respiratory failure •Rest with DVT prophylaxis •Administration of fluids and electrolytes •Transfusion therapy • Chelation therapy with repeat exacerbations Copyright © 2017, Elsevier Inc. All Rights Reserved. • Under-treatment is a major problem • Pain management • Often pain medication tolerant • Require continuous and breakthrough analgesia with morphine and hydromorphone • Multimodal and interdisciplinary approach involving emotional and adjunctive measures Copyright © 2017, Elsevier Inc. All Rights Reserved. • Treat infections • Administer folic acid • Hydrea is only antisickling agent shown to be clinically beneficial • Hematopoietic stem cell transplantation (HSCT) is only available cure Copyright © 2017, Elsevier Inc. All Rights Reserved. • Patient and caregiver teaching and support are important •How to avoid crises •Importance of prompt medical attention •Pain control Copyright © 2017, Elsevier Inc. All Rights Reserved. (©Thinkstock/Comstock/Thinkstock) • C.P.’s lab values show •Hematocrit 30% •Hemoglobin 10 g/dL •WBC count 20,000/µL •Bilirubin 2.8 mg/dL •Urinalysis normal •Ferritin normal • X-ray of her knee is normal Copyright © 2017, Elsevier Inc. All Rights Reserved. (©Thinkstock/Comstock/Thinkstock) • What problem do C.P.’s history and symptoms suggest? Copyright © 2017, Elsevier Inc. All Rights Reserved. • Results from hemolysis of RBCs from extrinsic factors •Physical destruction •Antibody reactions •Infectious agents and toxins Copyright © 2017, Elsevier Inc. All Rights Reserved. • Physical destruction of RBCs results from exertion of extreme force on cells •Hemodialysis •Extracorporeal circulation used in cardiopulmonary bypass •Prosthetic heart valves • Abnormal vessels Copyright © 2017, Elsevier Inc. All Rights Reserved. • RBCs can be fragmented and destroyed as they try to pass through abnormal arterial or venous microcirculation •Excessive platelet aggregation and/or fibrin polymer formation • Seen in thrombotic thrombocytopenic purpura (TTP) and disseminated intravascular coagulopathy (DIC) Copyright © 2017, Elsevier Inc. All Rights Reserved. • Antibodies may destroy RBCs by mechanisms involved in antigenantibody reactions Copyright © 2017, Elsevier Inc. All Rights Reserved. • Cause hemolysis in 3 ways: •Invade the RBC and destroy its contents • Parasites such as in malaria •Release hemolytic substances • Clostridium perfringens •Generate an antigen-antibody reaction • Mycoplasma pneumoniae Copyright © 2017, Elsevier Inc. All Rights Reserved. • Supportive care until the causative agent can be eliminated or made less injurious • Emergency preparedness is essential for potential hemolytic crises • Hydration, electrolyte replacement, corticosteroids, blood products, splenectomy • Folic acid replacement and immunosuppressive agents for chronic conditions Copyright © 2017, Elsevier Inc. All Rights Reserved. • Iron overload disorder •Primarily caused by a genetic defect •May occur secondary to other diseases • Genetic link •Increased intestinal iron absorption •Increased tissue iron deposition Copyright © 2017, Elsevier Inc. All Rights Reserved. (©Thinkstock/Comstock/Thinkstock) • What patient teaching is essential for you to discuss with C.P.? Copyright © 2017, Elsevier Inc. All Rights Reserved.
