Sickle Cell Anemia

Sickle Cell Anemia

Vivarian Moulton, Summer 2014, Upper Division
University of South Florida

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Sickle Cell Anemia

Crescents or donuts without holes? That is the dilemma of those with sickle cell disease.
270 million Americans are affected by it. It is the most common inherited disorder of the blood
(Baker, 2011). Alterations of cells in the blood occur because of an insufficient circulation of
functional red blood cells (RBC) (Huether, 2012). The purpose of this paper is to utilize recent
research and nursing practices to evaluate the standard of patient care using evidence based
nursing (EBN). For this evidence based paper, the effects of sickle cell anemia (SCA) on the
normal physiological process, the medical interventions suggested by current research and
evaluation of the effectiveness of the actual applied treatment.
Pathophysiology of Disease
Regardless of the type of anemia, the fundamental cause is a reduced oxygen capacity of
the blood that results in tissue hypoxia. SCA is an abnormal hemoglobin making, abnormal cell
shape which increases the possibility of lysis, damage and consuming by phagocytes; the
primary cause of this is a congenital dysfunction of hemoglobin synthesis. (Huether, 2012)
Mechanics of Disease
Sickle cell disease is an abnormal hemoglobin S (Hb S- sickle hemoglobin), an inherited
variant of the normal hemoglobin A, within erythrocytes. Hemoglobin S is a genetic mutation in
which the amino acid, valine replaces glutamic acid. When HbS is hypoxemic and dehydrated it
solidifies and stretches the normal RBC into an elongated sickled shape (Huether, 2012). Oxygen
tensions of 40 mm Hg triggers the sickling of cells in sickle cell disease as opposed to 15 mm Hg

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in a carrier (Hb S from only one parent). Sickled RBCs block blood vessels and increase the
viscosity of the blood which slows the circulation that contributes to the pain, vascular occlusion
and organ infarction that occurs during a crisis. In patients with sickle cell anemia, 75%-95% of
their hemoglobin is HbS, hence, when the cells are sickling and destroyed in the spleen there is a
shortage of blood supply which further decreases oxygen supply and causes anemia.
Erythropoiesis is then triggered. After oxygenation and rehydration, the RBCs can return to their
normal shape.
Risk Factors
Risk factors for sickle cell are prevalent in persons with origins in countries around the
equator. In the United States, the disease is most common in African Americans and Hispanics.
The HbS trait must be in the family linage for it to be passed onto children who will be carriers
and combined with another HbS trait for them to inherit the disease. (Huether, 2012)
Diagnosing a patient
A patient can be diagnosed with sickle cell disease if their symptoms include sickle cell
induced pallor, fatigue, jaundice and irritability are usually in a vaso-occlusive, sequestration,
aplastic or hyperhemolytic crisis. In order to be diagnosed with sickle cell disease the parents
hematologic history, clinical manifestations confirmed by the patients own hematologic tests are
necessary. The sickle solubility tests confirms the HbS in peripheral blood while the hemoglobin
electrophoresis provides the amount of HbS in RBCs. Prenatal diagnosing can be made from 8
week from the chorionic villus and at 15 weeks from amniotic fluid. Newborns should
automatically be screened per state mandate (Huether, 2012).

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Treatment
Treatment of sickle cell disease is aimed at supportive care that will prevent the
consequences of anemia and avoiding crises. Genetic counseling and psychologic support are
also available. Hydroxyurea is a medication that inhibits the deoxyribonucleic acid synthesis but
increases the hemoglobin F; it is used in children with severe sickle cell to increase hemoglobin
levels and decrease vaso-occlusive crises and hospitalization. The most common side effect of
hydroxyurea is myelosuppression. (Huether, 2012)
Prognosis
Prognosis for sickle cell patients vary, however, clinical manifestations do not occur until
around 6 months old. Sepsis and meningitis occur in up to 10% of children with SCA during the
first 5 years of life with a mortality rate of 25%. Survival time of those with SCA is
unpredictable but there is a trend of individuals to die in their twenties. (Huether, 2012)
Genetic Factors
A person is predisposed to sickle cell anemia if both parents carry HbS. It is an inherited,
homozygous, autosomal recessive disorder; either a patient is a carrier with little to no symptoms
or they have the full disorder. Patients with sickle cell anemia have some protection against
malaria.
Medical Interventions and Care Guidelines
A major contributing factor of the sickling of cells is dehydration and lowered
oxygenation. Interventions for these goals include monitoring hydration status, administering
intravenous fluids at room temperature, teaching the patient the identification of early signs of

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hypovolemia and the late signs to include cyanosis, cold clammy skin, weak pulses and
confusion. Evidenced based nursing maintains that a systematic review confirmations that
hypotension and tachycardia and occasionally fever are clinical signs of dehydration. (Ackley,
2010)
Proper management of fluid intake will increase hydration and allow the cells to return to
their normal donut shape; will decrease the clogging effect of the sickled crescent cells and the
overall pain it causes. The best interventions for sickle cell pain include rapidly identifying
appropriate treatment of the pain. Parenteral corticosteroids accelerate the resolution of acute
pain and decrease hospital stay while patients receive chronic oral opioids for the treatment of
the chronic pain outside of the hospital (Baker, 2011). Consulting with a dietician for dietary
suggestions will increase the knowledge base in regards to adequate at home nutrition.
Clinical Application
In a given scenario, the patient presents to the emergency department with uncontrollable
pain, low oxygen saturation, decreased skin elasticity and an increased acute pain concentration
in the right arm, leg and back that gets even more painful with movement. The patient refuses
blood transfusions unless her bodys hemoglobin levels fall below 6. The patient also refuses
hydroxyurea because the side effects outweigh the benefits for her. Upon admission to the
hospital the patient was placed on intravenous fluids to increase her hydration and given alternate
doses of Percocet and Dilaudid with Zophran to decrease her pain levels and nausea. The
patients vitals were monitored and hematology labs were monitored.
Evaluation of Clinical Application

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The course taken by the hospital was limited by the beliefs of the patient. The administration
of the pain and nausea medications helped to alleviate the acute pain of the patient and follows
EBN, however, aside from hydroxyurea, no pain management was offered to the patient for
home care which should not have been the case. Research finds that relaxation techniques and
exercises seem the most beneficial to patients practicing contemporary alternative medicine
(CAM). When used in conjunction with at home medications, pain is managed even more. The
usage of CAM can be included in care plans to better manage chronic pain (Thompson, 2014).
Conclusion
Sickle cell anemia is still undergoing extensive research. The pathophysiology of the
disease can affect multiple organs and functions of the body. The medical interventions are
aimed at management of pain and prevention of crises. The application of interventions vary
depending on what the patient consents to but overall, interventions are aimed at managing pain
levels of the patient. By involving the various beliefs of the patient into the care plans, there
would be increased pain management and compliance outside of the hospital.

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References
Ackley, B. J., & Ladwig, G. B. (2010). Nursing diagnosis handbook: an evidence-based guide to
planning care (9th ed.). Maryland Heights, Mo.: Mosby.
Baker, M., & Hafner, J. (2012). What Is the Best Pharmacologic Treatment for Sickle Cell
Disease Pain Crises . Annals of Emergency Medicine, 59(6), 515-516. Retrieved July 9,
2014, from the University of South Florida Library database
Huether, S. E., & McCance, K. L. (2012). Understanding pathophysiology (5th ed.). St. Louis,
Mo: Elsevier.
Thompson, W. E., & Eriator, I. (2014). Pain Control in Sickle Cell Disease Patients: Use of
Complementary and Alternative Medicine. Pain Medicine, 15(2), 241-246. Retrieved
July 9, 2014, from the University of South Florida Libraries database.