Download Macrocytic/ Microcytic and Sickcle Cell Anemia

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