Introduction - Evidence Based Medicine

Evidence Based Medicine

Table of Contents
INTRODUCTION...........................................................................................................................1
LITERATURE REVIEW ...............................................................................................................1
Sickle Cell Anemia (SCA)...........................................................................................................1
Hydroxyurea treatment for sickle cell .........................................................................................2
Treatment efficacy and effectiveness ..........................................................................................3
Impact of prolong usage of hydroxyurea treatment and associated limitations ..........................5
CONCLUSION ...............................................................................................................................6
REFERENCES ...............................................................................................................................8

INTRODUCTION
Sickle cell anemia (SCA) is known as the blood disorder which causes shape deformation
of the red blood cells. The deformation of blood cells in the shape of sickle makes them fragile
and it clogs the blood vessels. Due to the blockage oxygen transportation through blood becomes
difficult and tissue damage cause severe pain. The pain events are called vaso-occlusive
incidence or pain crises (Jones, Howard and Nevitt, 2017). Usually these are treated with pain
relief medications, blood transfusion and vigorous hydration. However, Hydroxyurea is the best
known drug for the treatment of these SCA generated pain killers. This report will provide a
literature review on the efficacy of the hydroxyurea treatment in SCA.
LITERATURE REVIEW
Sickle Cell Anemia (SCA)
According to Ware and et.al., (2017) SCA is hemoglobin disorder and can be inherited
through genes. Its major symptoms results in the abnormal shape of red blood cells (RBC) which
hinders the easy and regular flow of blood through blood vessels. Hemoglobin is the protein
pigment present in the RBC which is responsible for carrying oxygen. The sickle shaped RBC as
the result of SCA are highly vulnerable to rupture due to their increase fragility. The rupture of
blood cells leads to significant reduction in the number of RBCs and thus anemia is resulted. The
irregular sickle shaped blood cells also cause extreme pain and break down of the tissues.
As per the view of Schumacker, (2018), vulnerability of SCA also depends upon
ethnicity. Like the disease is more common among Asian and African communities as compare
to the western regions. In healthy individuals RBC are round and flexible which allows them to
easily move through blood vessels. However due to SCA these cells becomes sticky and rigid
which hinders their free movement. Piel, Steinberg and Rees, (2017) stated that the most
common symptoms of this disorder are Anemia, severe pain episodes called pain crises, frequent
infections, visionary issues and painful swelling in foot and hands. Since the sickle cells can
easily break down the deficiency of RBC also causes fatigue or even death.
The intensity of the pain crises is also severe and can cause damage to joints or bones and
ulcers if not treated properly on time. In the same context Tshilolo and et.al., (2018) argued that
the genetic mutation is the only cause for SCA thus there are no effective treatment methods for
the disorder. However recent developments such as hydroxyurea have proven to be very
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effective in managing pain and SCA associated complications like stroke, cardiovascular issues,
leg ulcers and organ damage.
The most common treatment strategies for SCA and its complications are regular intake
of pain medications, blood transfusions, medications for preventing infections and folic acid and
bone marrow transplant. McGann and Ware, (2015)elucidated that since the bone marrow
transplant depends upon the availability of the donor the most common treatment intervention is
use of hydroxyurea in which medications are given to minimise the frequency of acute chest
syndrome and pain crises. It also reduces the frequent blood transfusion needs. However, there
has been several discussions regarding long term impact of hydroxyurea which must be
evaluated for the better health outcomes.
Hydroxyurea treatment for sickle cell
According to Ware, (2015) hydroxyurea therapy enhances the production of fetal
hemoglobin (HbF) which ameliorate the intensity and severity of SCA. Thus, the dosage of
hydroxyurea influences the yield of HbF and provides good response by reducing painful crises
(Savage and et.al., 2015). As compare to the other agents for the treatment of SCA hydroxyurea
has several advantages such as easy oral administration, prevention of vaso-occlusive events,
modest toxicity profile and clinical efficacy. It can also prevent from the organ dysfunction.
However, despite having several advantages the treatment is not preferably used for the younger
people especially for children. McGann and et.al., (2016) stated that even when hydroxyurea
treatment is available the health care professionals used to avoid its use for the children due to
unexplained long term impact of therapy on individuals.
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