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Running head: ANEMIA 1 Macrocytic/ Microcytic and Sickle Cell Anemia Ronald Otwori and Melisa Dervisevic State University of New York Institute of Technology ANEMIA 2 Abstract Anemia is when the body doesn’t have enough healthy red blood cells in the body. It is measured by hemoglobin and hematocrit in a complete blood count (CBC). It can be caused by various different health conditions. There are many different types of anemia, such as macrocytic, microcytic, and sickle cell anemia. The signs and symptoms of the patient depends on the type of anemia they are presenting with. Also, the treatment regimen and diagnosis is different for each type of anemia. This paper will discuss the different types of anemia, presentation of the patient with that type of anemia, how the diagnosis is made, treatment, and patient education. Keywords: anemia, hemoglobin, hematocrit, macrocytic, microcytic, sickle cell ANEMIA 3 Macrocytic/ Microcytic and Sickle Cell Anemia Anemia is a blood disorder that results in low levels of circulating red blood cells, its measurements are obtained as part of complete blood count (CBC) (Schrier, Mentzer, & Landaw, 2014). The count involves the hemoglobin concentration, hematocrit and red blood cells count; there are many types of anemia with different etiologies, the symptoms may vary widely depending on the cause of the anemia (Schrier, Mentzer, & Landaw, 2014). Nurse Practitioners (NP) who work as primary care providers are probably the first to come into contact with patients who initially present with symptoms of anemia. According to Poggial, De Amias & Motta (2014) early diagnosis and treatment of specific kinds of anemia have good outcomes. What are the roles of nurse practitioners in early identification and management of anemia? Due to the nonspecific initial presenting symptoms of various types of anemia, it is important for the NP to take a complete health history and physical exam to identify the problem. The purpose is to identify what kind of anemia the patient has and initiate the right treatments early or make a referral to a specialist. The literature review was conducted using terms anemia, chronic anemia, sickle cell anemia, microcytic anemia and macrocytic, diagnosis and treatment of anemia, courses and symptoms of anemia. The reviews were done using State University of New York Institute of Technology Cayan library electronic database and review articles were obtained from CINAHL, science direct; Up-to-date website and Google scholar search engine. The peer-reviewed articles obtained, have similarities in diagnosis and treatments of common anemia that are caused by blood loss and iron poor diet. The articles however provide varied treatments for genetically acquired anemia and provide an ongoing research for better ANEMIA 4 treatments. According to Karlson et al. (2012) the treatment for genetically acquired anemia need prompt diagnosis and treatment to reduce the severity of the disease. The review articles provide detailed discussion on microcytic, macrocytic and sickle cell anemia. Anemia is a general word and is used to describe a decrease in red blood cells production, increased red blood cells destruction and blood loss (Schrier et al.2014). The causes of anemia can be infection, lack of iron, vitamin B12 or folate deficiency, iron deficient diet, malabsorption, blood loss, bone marrow disorders, bone marrow suppression, low levels of trophic hormones such as erythropoietin, hypothyroidism, hypogonadism, inflammation, and malignant disorders (Schrier et al. 2014 and Vinchinsky & Mahoney, 2014). Macrocytic anemia is caused by lack of vitamin B12 and folic acid, a condition which occurs in malnutrition, poor diet, alcohol intake, liver disease, hypothyroidism, gastrointestinal surgery and malabsorption syndrome, medications, bone marrow dysplasia and reticulocytosis. (Parker-Williams, 2013 and Shilpa & Deepak, 2009). Physiological changes to the body for example pregnancy, or pathological causes such as chronic hemolytic anemia and myelofibrosis may cause macrocytic anemia (Goonewardene, Shehata, & Hamad, 2012). The diagnosis of macrocytic anemia is based on various clinical presentation, which may include the following; stomatitis, glossitis, nausea, bilateral peripheral neuropathy, impaired memory, malaise and diarrhea (Parker-Williams, 2013 & Poggiali, De Amicis, & Motta, 2013). Additional diagnostic tests include the following; peripheral blood smear, alanine transaminase (ALT), aspartate aminotransferase (AST), bilirubin, lactate dehydrogenase, thrombocytopenia and urobililinogen. Reticulocyte count, vitamin B12 serum level, comprehensive metabolic panel (CMP), thyroid stimulating hormone (TSH), methylmalonic acid and homocysteine levels. (Schrier et al., 2014; Poggiali et al., 2013; Parker-Williams, 2013 and Domino, 2013). ANEMIA 5 Treatment of macrocytic anemia includes; oral folic acid supplementation and correction of underlying causes of folic acid deficiency. Patient education on continued folic acid supplementation is necessary and dietary modifications (Schrier et al., 2014). Nutritional education and dietary modifications should include foods that are rich in folate such as green leafy vegetables, legumes and fortified cereals. Patients should be encouraged to avoid or reduce alcohol intake as alcohol can cause alcohol related folate deficiency (Koike et al.2012). Additional diet modification includes foods that are rich in vitamin B complex and intake of adequate Vitamin C which will increase the absorption of iron. Parenteral therapy should be initiated if malabsorption exists and the hemoglobin level is less than 6 gm/dl (Schrier et al. 2014; Dunphy, Winland-Brown, Porter, & Thomas, 2011 &Koike et al. 2012). Patient education on macrocytic anemia includes; the need to increase folic acid and vitamin B12 in their diet, increase dark-green vegetables in one or two meals daily and continued education on basic signs and symptoms identified above on macrocytic anemia so they can selfmonitor (Dunphy et al., 2011). Microcytic anemia is defined as a low mean corpuscular volume (MCV) (less than 80fL) and red blood cells that are smaller than normal, the MCV in microcytic anemia is involved in causing a disturbance in iron metabolism (Parker-Williams, 2013). The signs and symptoms of microcytic anemia are based on clinical presentation and a detailed physical exam. A patient may present with the following symptoms; excessive sleepiness, tiredness, activity intolerance, syncope, orthopnea and shortness of breath (Shilpa & Deepak, 2009). The causes of microcytic anemia are; iron deficiency, bleeding, lead poisoning, chronic inflammation, copper deficiency and atransferrinemia, sideroblastosis and thalassemia (Shilpa & Deepak, 2009 Parker-Williams, 2013 & Fellows & Fellows, 2011). According to Shilpa & ANEMIA Deepak (2009) race and ethnicity, diet, cow’s milk, chronic blood loss can all lead to poor absorption of iron. The United States Preventive Task Force recommends screening for iron deficiency in children ages 9 to 12 months, 15 to 18 months and 2 to 5 years. The diagnosis of microcytic anemia is based on lab tests and clinical presentations. The clinical and physical findings are pallor, ulcers to the lower extremities, frontal bossing, changes in texture of nails (they become fragile and break easily), chest pain, decreased appetite, dizziness, glossitis, stomatitis, tachycardia, splenomegaly and guaiac positive stools (Shilpa & Deepak, 2009). The lab tests that are done to determine specific causes of iron deficiency include serum ferritin, serum iron, total binding capacity, transferrin saturation, bone marrow iron stores, stool for occult blood, sedimentation rate, blood lead (Rogers, Mahoney, & Hoppin, 2014;Shilpa & Deepak, 2009; Schrier et al., 2014 & Vinchinsky & Mahoney, 2014). The treatment of microcytic anemia is only successful if the cause is established before treatment is initiated, if there is no response to therapy the diagnosis should be reviewed and compliance questioned (Parker-Williams, 2013). The treatments of choice include vitamin B12/folate supplements; the treatment of underlying conditions for example hypothyroidism, if treatment fails with oral supplementation; then start parenteral iron therapy if indicated. It is recommended that 500 mg of ascorbic acid be given two-three times per day to improve the absorption iron (Shilpa & Deepak, 2009). Patient education should be focused on self-care and primary care management of the underlying cause. In iron-deficiency anemia, patients should take ferrous sulfate on an empty stomach, eat foods that are rich in iron, vitamin C, and B-complex vitamins, remain active, rest when needed, and self-monitor for any fatigue, shortness of breath, pale-colored stools, or tachycardia (Shilpa & Deepak, 2009). 6 ANEMIA 7 Sickle cell anemia is an inherited disorder characterized by presence of hemoglobin S from either the mutation of better globin chain or from heterozygosity of a sickle beta globin mutation (Vinchinsky & Mahoney, 2014). According to Dunphy (2011) sickle cell anemia results in a marked hemoglobinopathy which forms abnormal polymers that lead to sickled red blood cells in hypoxic conditions. The polymers that are formed are elongated in rope-like structures that align with other fibers, resulting in alteration of the red blood cells into the curved or sickle shape. Due to this deformity there is a marked decrease in red cell movement in blood vessels (Rogers et al., 2014. & Vinchinsky & Mahoney, 2014). Sickle cell anemia is predominant in people from West African descent, European and Middle Eastern ancestry (Dunphy et al., 2011). The disorder may be seen after a few months of life or later in life. In the United States, all 50 states have a universal neonatal screening for hemoglobinopathies (Vinchinsky & Mahoney, 2014). According to Vinchinsky & Mahoney (2014). Prenatal testing and counseling should be done to at risk couples early in the pregnancy due to the chronic and debilitating process of the disease. The screening of newborns is done early in infancy stage to determine or detect presence of disease with the goal of early detection and intervention to reduce morbidity and mortality (Karlson et al., 2012). Infants are usually asymptomatic until about 4 months old; the disease process starts by causing increased red cell adhesion, increased leukocyte adhesion, hyper coagulability and reperfusion injury to the blood vessels (Mousa et al., 2010). By the age of 6-12 months, the condition starts to get worse with sickle cell crisis and pain (Karlson et al., 2012). The presenting clinical symptoms depends on the severity of the disease and the presence of precipitating factors such as hypoxia, infection, fever, acidosis, dehydration, pregnancy and exposure to extreme temperatures (Mousa et al., 2010 & Vinchinsky & Mahoney, 2014) . In ANEMIA 8 children the swelling of the hands and feet, known as hand-foot syndrome or dactylitis, is often the first presentation of sickle cell anemia (Rogers et al., 2014). The initial diagnosis is based on a CBC and blood smears, the CBC will reveal sickled cells, increased reticulocyte count and elevated white blood cells (Vinchinsky & Mahoney, 2014). A physical examination will include; fever, pale skin, and mild jaundice (Domino, 2013). The treatment of sickle cell anemia is based on the initial presentation and ongoing symptoms, since the severity of the condition varies so does the treatment options. (Domino, 2013; Mousa et al., 2010 &Vinchinsky & Mahoney, 2014). Some of the treatment options include oxygen supplementation, nonnarcotic and narcotic pain management, hydroxyurea, and inhaled nitric oxides (Domino, 2013; Mousa et al., 2010 &Vinchinsky & Mahoney, 2014). In children prevention of infection is important and a prophylactic antibiotics should be initiated between 2 months and 5 years to prevent serious infection such as pneumonia (Vinchinsky & Mahoney, 2014). Blood transfusion may be needed during acute symptomatic and exacerbations of sickle cell anemia; it is important to maintain adequate fluid and electrolyte balance in acute stages and exacerbations (Vinchinsky & Mahoney, 2014). Non-pharmacological management of sickle cell anemia include ; transcutaneous electrical nerve stimulation (TENS), warm compresses , physical therapy, hypnotherapy relaxation therapy, avoidance of extreme temperatures, increased fluid intake , avoid constipation, nutritional supplements and diets that are rich in vitamin B and vitamin C complex, adequate intake of proteins, folic acid and iron (Poggiali et al., 2013 & Rogers et al., 2014). According to Karlson et al. (2012) and Rogers et al. (2014) many complications may arise with sickle cell anemia. It is estimated that 24% of individuals with sickle cell anemia will experience a stroke by age 45. In childhood, 25% have silent ischemic lesions that may impair ANEMIA 9 their neurocognitive functions (Vinchinsky & Mahoney, 2014). The risk for intracranial hemorrhage exists and is 2-3 times more likely to develop epilepsy (Rogers et al., 2014). Psychosocial issues of low self-esteem associated with the illness, social isolation, poor family relationships, withdrawal, anxiety, depression, and inappropriate pain coping strategies are among the many factors that lead to poor outcomes (Karlson et al., 2012). Additional complications include the following ; bone infarction and necrosis due to accelerated hematopoiesis, increased cardiac output, acute myocardial infarction (MI), vasoocclusion in the skin leading to ulcers, hepatic dysfunction caused by acute hepatic ischemia, benign cholestasis, transfusion iron overload, cholelithiasis, renal failure, retinal artery occlusion, retinal detachment, hemorrhage, prolonged and painful priapism, acute severe anemia, aplastic crisis, and hyperhemolytic crisis (Dunphy et al., 2011;Domino, 2013 and Vinchinsky & Mahoney, 2014). Patient education is the key to effective management of the sickle cell anemia. Parents should be given information about sickle cell disease as early as possible with the importance of regularly scheduled health maintenance visits, penicillin (PCN) prophylaxis and immunizations, including pneumococcal vaccination (Karlson et al., 2012 and Rogers et al., 2014). Information should be given regarding home management of pain, importance of adequate hydration, rest and avoidance extreme heat/cold (Vinchinsky & Mahoney, 2014). The parents should be taught how to recognize the signs and symptoms that require medical attention such as enlarged spleen, fever, pallor of skin, respiratory symptoms, pain and inability to move extremities (Karlson et al., 2012 and Rogers et al., 2014). ANEMIA 10 Appendix 1 Studies Focus Subjects Age Method Findings Case Populatio n Across Parker- Manageme 1month Longitudin Early Williams(201 nt study life span > al study diagnosis and 3) of anemia Koike (2010) Foliate Clinical deficiency case folate study deficiency treatment Female 33 Focus Mousa et al. Manageme case Across Not (2010) nt of sickle study life span identifie associated d with sickle cell anemia Focus Treatment of Severe pain cell anemia Goonewarden Anemia in Literatur e pregnant e review (2012) women Female 15-49 Focus yrs Treatment of anemia in pregnant women Shilapa (2009) Microcytic anemia in Literatur Pediatrics e review pediatrics 6 Focus Treatment of months- microcytic 18 years anemia Fellow & Anemia and Case Across None Fellows GI life span identifie microcytic d anemia study (2011) Nagy et al Macrocytic Clinical Across (2012) anemia case life span study 98 Focus Treatment of Genome Polymorphis study m in catalase microcytic anemia ANEMIA 11 References Domino, F. J. (2013). The 5- minute clinical consult (21 ed.). New York, NY: Wolters Kluwer Lippincott Williams & Wilkins. Dunphy, L. M., Winland-Brown, J. E., Porter, B. O., & Thomas, D. J. (Eds.). (2011). Primary care: The art and science of advanced practice nursing (3rd ed.). Philadelphia, PA: Davis. Fellows, H., & Fellows, I. (2011,). Anemia and the gastrointestinal (GI) tract. Medicine, 79-83. Retrieved from http://www.sciencedirect.com.ezproxy.sunyit.edu/ Goonewardene, M., Shehata, M., & Hamad, A. (2012). Anemia and pregnancy. Best Practice & Research Clinical Obstetrics & Gynaecology, 26(1), 3-24. http://dx.doi.org/10.1016/j.bpobgyn.2011.10.010 Karlson, C. W., Haynes, S. L., Smith, M., Faith, M. N., Elkin, D., & Megason, G. (2012). Examination of risk and resiliency in a pediatric sickle cell disease population using the psychosocial assessment tool 2.0. Journal of pediatric psychology, 37(9), 1031-1040. http://dx.doi.org/10.1093/jpepsy/jsso87 Koike, H., Hama, T., Kawagashira, Y., Hashimoto, R., Tomita, M., Lijima, M., & Sobue, G. (2012). The significance of folate deficiency in alcoholic and nutritional neuropathies: Analysis of a case. Nutrition, 28, 821-824. http://dx.doi.org/doi:10.1016/j.nut.2011.11.022 Mousa, S. A., Momen, A. A., Sayegh, F. A., Jaouni, S. A., Nasrullar, Z., Saeed, H. A., ... Sayegh, M. A. (2010). Management of painful vaso-occlusive crisis of sickle-cell anemia: consensus opinion. Clinical and Applied Thrombosis/Hemostasis, 16, 365-373. http://dx.doi.org/10.1177/1076029609352661 ANEMIA 12 Nagy, T., Csodas, M., Kosa, Z., & Goth, L. (2012). A simple method for examination of polymorphisms of catalase exon 9: rs769217 in Hungarian microcytic anemia and betathalassemia patients. Archives of Biochemistry and Biophysics, 525(2), 201-206. http://dx.doi.org/http://dx.doi.org/10.1016/j.abb.2012.01.004 Parker-Williams, E. J. (2013,). Investigation and management of anaemia. Medicine, 14(4), 212218. Retrieved from http://www.sciencedirect.com.ezproxy.sunyit.edu/ Poggiali, E., De Amicis, M. M., & Motta, I. (2013). Anemia of chronic disease: A unique defect of iron recycling for many different chronic diseases. European Journal of Internal Medicine, 25(), 12-17. http://dx.doi.org/10.1016/j.ejim2013.07.011 Rogers, Z. R., Mahoney, D. H., & Hoppin, A. G. (2014). Routine comprehensive care for children with sickle cell disease. UpToDate. Retrieved from www.uptodate.com Schrier, S. L., Mentzer, W. C., & Landaw, S. A. (2014). Approach to the adult patients with anemia. UpToDate. Retrieved from www.uptodate.com Schrier, S. L., Mentzer, W. C., & Landaw, S. A. (2014). Approach to the adult with anemia. UpToDate. Retrieved from www.uptodate.com Shilpa, J., & Deepak, K. (2009). Evaluation of microcytic anemia. Clinical Pediatrics, 48(1), 713. http://dx.doi.org/10.1177/0009922808323115 Vinchinsky, E. P., & Mahoney, D. H. (2014). Diagnosis of sickle cell anemia. UpToDate. Retrieved from www.uptodate.com