1. Sickle Cell Anemia – Case Study
Posted by: Daisy Jane, RN, MN
October 16, 2010 · Comments (0)
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Definition
Sickle cell anemia is an inherited disorder on the beta chain of the hemoglobin resulting
to abnormally shaped red blood cells. RBC’s assume a crescent or C-shape that
decreases the cell’s life span and ability to function thereby, producing various
complications. In some cases, these sickled cells may block the flow causing pain and
organ damage.
Incidence
 Equal male-female ratio
 Increase incidence in African Americans
Etiology
Autosomal recessive disorder
Review of Related Anatomy and Physiology
Red blood cells or erythrocytes carry oxygen to the different parts of the body. Different
from other cells, RBC’s do not contain a nucleus (anucleated). These small cells are
circular and flattened with depressed centers on both sides resembling to that of a
doughnut when viewed under a microscope. Their size and shape provides a large
surface area for carrying oxygen in relation to their volume. The normal RBC count is 4-
6 million/mm3. RBC’s has the life span of 120 days.
Hemoglobin is a form of protein that contains iron which is responsible for transporting
oxygen that is carried in blood. Adult hemoglobin contains a beta chain (HBB) while a

2. fetus’ hemoglobin has a gamma chain. Hemoglobin is comprised of four protein (amino
acid) components. It has two beta-globin and two alpha-globin. The subunit beta-globin
is located inside the RBCs. These amino acids carry an iron-bearing molecule called
heme. Heme molecules, which are only found in mature RBC’s, enables the
erythrocytes to pick oxygen from the lungs and transport it throughout the body. Once
oxygen attaches to hemoglobin it gives the blood its bright red pigment.
The more hemoglobin molecules the RBC contain, a higher amount of oxygen will they
be able to carry. If the hemoglobin is defective, the erythrocyte will also malfunction. A
red blood cell is just a vessel; the one that performs the oxygen transportation is the
hemoglobin. Normal hemoglobin is 13-18 grams/100 ml of blood in males and 12-16
grams in females.
Pathophysiology
Erythrocytes in sickle cell anemia contain abnormal hemoglobin that affects the beta-
chain producing hemoglobin S or HbS. In this disorder, the beta-chains (beta-globins)
are replaced by Hemoglobin S. Valine (an amino acid) takes the place of the normally
appearing glutamic acid in beta-chains. Replacement of glutamic acid with valine
causes the polymerization of HbS components to cohere forming long and insoluble
particles. These distort the red blood cells, which assumes an inflexible crescent or
sickle shape.
The abnormally shaped cells become sharp and spiky when the RBCs are discharging
oxygen molecules and in cases where the oxygen content in blood is low such as
performing vigorous exercise and being in high altitude areas. Typically, a sickled cell’s
lifespan is only 20 days. The deformed erythrocytes also rupture easily and they tend to
be trapped in the microcirculation, obstructing blood flow and oxygen transport that
might lead to painful episodes of ischemic injury.
Sickle cell crisis refers to episodes of acute and severe sickling that blocks the
circulation posing a threat of extensive organ damage. Severe pain is noted during
these incidents caused by occluded vessels in the bone possibly resulting to bone
necrosis. The crisis is triggered by hypoxemia, acidosis, or other conditions such as
dehydration, infection, vigorous exercise, pregnancy or cold weather.
A condition called a sickle cell trait is identified by the presence of a single defective
gene, instead of two. Individuals with this trait are essentially normal however, they are
carriers. Meaning two sickle cell trait carrier parents can contribute a defective gene to a
child that will carry the sickle cell disorder.
Diagnosis
Prenatal:
 Chorionic Villi sampling
 Amniocentesis (blood from the cord)
At birth:
 Newborn Screening
 Hemoglobin electrophoresis
Signs and Symptoms
The following manifestations are observed in children with a sickle cell disorder, at
about 6 months of age (because fetal hemoglobin contains gamma, not a beta chain):
 Fever
 Anemia

3.  Swelling of the hands and feet (hand-foot syndrome) – caused by blood stasis and
infarction
 Protruding abdomen – due to enlarged spleen and liver secondary to trapping of
sickled cells in microcirculation and obstruction of blood flow
 Icteric sclera – caused by bilirubin release during hemolysis (sickle cell destruction)
 Priaprism (males) – due to pooling of abnormally shaped erythrocytes in the blood
vessels of the penis.
 Chest syndrome – symptoms same with pneumonia that is the major cause of death
in sickle-cell patients
Management
Conservative Management
1. Pain relief with Acetaminophen (Tylenol) or IV of morphine to reduce metabolic
demand of oxygen thereby, terminating cell sickling.
2. Adequate hydration – IV fluids and electrolyte replacement
3. Oxygenation
4. Antibiotics – if the cause of sickling is infection.
5. Blood transfusion with packed RBC’s
6. Hydroxyurea – antineoplastic agent that increases production of fetal hemoglobin in
children.
7. Exchange transfusion – replacing sickled with normal cells
If the patient does not respond to the usual therapies STEM CELL
TRANSPLANTATION is done.
Nursing Management
1. Monitor vital signs. Assess for pain.
2. Obtain blood and urine culture, chest x-ray and CBC results if infection is the cause of
sickling.
3. Monitor child’s nutritional intake with hydroxyurea. If taken orally, this drug can cause
anorexia.
4. Assess for kidney function by noting if the child has urinated or not. (Kidney infarction
may occur)
5. Do not administer potassium if the kidney function is not verified. Potassium if not
excreted by the kidney may cause arrhythmia.
Possible nursing diagnosis
1. Ineffective tissue perfusion R/T decreased hemoglobin concentration in blood
2. Acute pain R/T impaired blood flow due to obstruction of sickled cells
image from elev8.com, daviddarling.info
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About Daisy Jane, RN, MN
Nursing Care Plan – Anemia
Posted by: Admin
July 6, 2008 · Comments (7)
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Anemia is the reduction in red blood cells (erythrocytes) thus
decreasing the oxygen carrying capacity of the blood due to the following
(1) excessive blood loss (2) deficiencies and abnormalities of RBC
production (3) Excessive destruction of RBC.
*Common Symptoms of Anemia
These symptoms appear in most types of anemia:
 pale skin
 dizziness
 fatigue
 headaches
 irritability
 low body temperature
 numb/cold hands or feet
 rapid heartbeat
 shortness of breath
 weakness
 chest pain
*Types of Anemia
Different types of anemia have different causes and symptoms.
Iron Deficiency Anemia: Iron deficiency is one of the most common causes of anemia, especially in women. In the U.S. alone, twenty million women
suffer from iron deficiency anemia. Iron deficiency causes insufficient hemoglobin production which, in turn, causes anemia and anemia symptoms.
Sickle Cell Anemia: Sickle cell anemia, or sickle cell disease, is one of the hereditary causes of anemia. Most often seen in people of African descent,
sickle cell anemia is characterized by the production of rigid, sickle-shaped red blood cells. These abnormal sickle cells break down faster then normal
red blood cells, resulting in a chronic shortage of red blood cells and anemia symptoms.
Sickle cell anemia is a form of hemolytic anemia, which describes types of anemia caused by the rapid destruction of red blood cells.
Pernicious Anemia: Certain types of anemia are referred to as megaloblastic, or vitamin deficiency anemia. Pernicious anemia is caused by an
inability of the intestines to absorb sufficient amounts of vitamin B12, which is required in the production of red blood cells.
Pernicious anemia symptoms include vision problems, memory loss, difficulty walking and numb hands and feet, as well as the more common anemia
symptoms.
Risk factors for pernicious anemia include a family history of pernicious anemia, Scandinavian or European ancestry or a history of autoimmune
endocrine disorders.
Vitamin B12 injections are the standard pernicious anemia treatment. Oral vitamin B12 supplements may also be used as pernicious anemia treatment,
but they’re not as efficient as B12 injections.
Folic Acid Deficiency Anemia: Folic acid deficiency anemia, like pernicious anemia, is a megaloblastic anemia. Folic acid is essential for healthy
blood and a healthy nervous system. A folic acid deficiency during pregnancy can cause birth defects.

5. Folic acid supplements form the basis of folic acid anemia treatment. Anemia treatment also includes eating a diet high in folic acid, including such
foods as:
 beans and legumes
 citrus fruits and juices
 dark green leafy vegetables
 pork
 poultry
 shellfish
 wheat bran
Thalassemia: Causes of anemia often include genetic mutations. Thalassemia is one of the genetically inherited types of anemia. Thalassemia occurs
when the body’s genes, which are responsible for proper hemoglobin production, become damaged or mutated. Like sickle cell anemia, thalassemia is
one of the hemolytic anemia types.
Many possible gene variations can cause thalassemia, and the disease has both mild and severe varieties. Cooley’s anemia is the most common
severe thalassemia in the U.S. The disease tends to be present at birth and its symptoms include:
 dark colored urine
 deformed facial bones
 fatigue
 jaundice
 poor growth rates
 a swollen abdomen caused by an enlarged spleen and liver
 weakness
A number of ethnic groups are at higher risk of developing thalassemia, including people of African, Chinese, Filipino, Indian, Italian, Greek and Middle
Eastern ethnicity.
Treatment for thalassemia depends on the severity of the disease. Mild types of thalassemia may be treated with regular blood transfusions. Severe
cases require multiple blood transfusions, iron chelation therapy to remove excess iron from the blood and bone marrow transplants. Without
treatment, severe thalassemia often proves fatal in early childhood.
Aplastic Anemia: Aplastic anemia is one of the rarer types of anemia, occurring when the body makes insufficient red and white blood cells. Causes
of aplastic anemia include:
 radiation therapy
 chemotherapy
 toxic chemicals
 some medications
 bone marrow infections