University of Northern Iowa Sickle Cell Disease Paper Attached is a 8-pages research paper. Follow all the comments and instructions. 1
Research Paper
3/31/2020
Sickle Cell Disease
According to the World Health Organization , approximately 240,000 people are dying
every year because of Sickle Cell Disease in Africa (WHO, 2016). However, have you ever
thought that living with Sickle Cell Disease doesnt necessarily mean one is approaching death?
and that its possible for you, me, and the society as a whole to make life comfortable for those
who are suffering from Sickle Cell Disease? As Im a health education and promotion major, I
tend to be interested in health-related topics in general. Moreover, I get more interested when it
comes to those subjects that Im personally concerned about oftentimes and very familiar with.
Out of all topics in the public health field, I chose to talk about Sickle Cell Diseases, also
known as Sickle Cell Anemia. Sickle Cell is the most commonly known blood disorder and its a
genetic blood disorder that causes the red blood cells to have sickled, abnormal shapes. Sickle
Cell Anemia is a lifelong disease and is still under study as theres no official treatment for it and
only its symptoms can be cured or at least mitigated. Sickle Cell Disease is one of those diseases
that can be diagnosed as soon as 5 months in infants.
Since I was a baby, I was diagnosed and confirmed to be a Sickle Cell Disease carrier of
the trait. Having this condition doesnt mean that Im considered sick or anemic, it just means
that I have this trait inherited from one of my parents, which in this case is my mother, and its a
gene that I could also pass to my children. In addition, a few of my siblings have this trait along
with other blood traits that are also inherited from my parents, such as G6PD and Thalassemia.
This research is conducted for the purpose of informing people around me about Sickle
Cell Disease. The goal is to raise awareness by describing what the disease is and how can we do
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a better job at handling it. In addition, another goal and argument thats going to be brought up
for this research is to expose and reveal the secrets of why Sickle Cell isnt sufficiently studied
and research like other diseases are. The main points discussed in this research are the general
background of the disease, the evolution of the disease treatments, and the main argument. The
main argument for this research is discovering the reasons why Sickle Cell isnt given the
attention it needs as a threatening disease that kills thousands of adults and children worldwide.
Background and history of the disease:
The first point is aimed towards informing and raising awareness by defining the disease
and where it originated from, differences between Sickle Cell Disease and Sickle Cell trait, The
symptoms and risk factors, and marriage and pregnancy in cases of Sickle Cell. First, Sickle Cell
Disease was first witnessed in Africa where it had been present for at least six thousand years
and has been known by many names in many tribal languages. For years, people didnt really
understand the symptoms accompanying the disease until the year 1910. In 1910, Sickle Cell
Disease was first identified and discovered in the United States by Dr. James Herrick, a
cardiologist.
The story begins where a dental student from Chicago came to Dr. Herrick complaining
about a series of constant pain, which now known as a crisis, which happens to him every now
and then. A blood test was done and his blood cells were examined under the microscope and
here comes the surprise. Some of his red blood cells didnt have that normal shape which is
round but, instead, they had a sickle shape. As weeks and months went by, more cases started to
surface with the same scenarios and the same symptoms. As much as it was mysterious, it was
obvious and clear that all cases were of those from African descent and that led to the idea of the
disease being able to be genetically inherited for sure. Finally, in 1951, the idea and structure of
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the disease started to become even clearer and it was the first among other similar blood diseases
to be discovered. It was fully understood that its a genetic blood disorder that can only be passed
by genes and thats the reason behind the disease being most relevant among those from African
origins.
The fact that its extremely common among Africans doesnt mean its exclusive to them
and the proof to this is that nowadays, Sickle Cell can be detected in few other regions of the
world such as the Middle East. After this discovery, it was also understood that seeing sickleshaped cells under the microscope isnt an effective way to detect Sickle Cell Disease. The
reason behind this is that individuals with Sickle Cell Trait also have a small percentage of
sickle-shaped cells in their blood. So, as long as they also have cells that are sickled, what really
is the difference between the disease and the trait?
There are a few differences between a sickler, a person who has Sickle Cell Disease, and
a carrier of the trait. First, the most obvious distinguishment is that the percentage of sickleshaped cells are very low compared to the percentage in sicklers because they only have one
copy of the gene instead of two genes just like the sicklers. Second, as a result of the low
percentage of sickle cells, carriers of Sickle Cell Trait generally don’t experience any symptoms
and I was able to observe this from my personal experience as Ive never experienced any type
of symptoms. Third, unlike carriers of the trait, sicklers have a higher probability and tendency
of having medical complications, especially during surgeries.
Symptoms, complications, and the management of the disease:
Speaking of complications, one of the risks that sicklers may face could include strokes,
infections, eye damage, acute chest pain, skin ulcers, blindness, and hypertension, fever,
coughing, and trouble breathing. Another common complication thats more serious than those
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previously mentioned is organ damage which could include the damage of bones, brain, lungs,
liver, heart, and kidneys. Organ damage is proven to be the most threatening complications for
people affected by Sickle Cell Disease.
When it comes to symptoms, there are a few of them that make it easy to distinguish
Sickle Cell Anemia. First, sicklers experience severe fatigue and irritability, yellowing of the
eyes and skin, bedwetting from associated kidney problems, and swelling of hands and feet, bone
infarcts due to failure of blood supply. From my personal life, I was able to see one of my friends
whos been diagnosed with Sickle Cell Anemia being impacted by bone infarcts in one of her
legs which resulted in one leg being slightly shorter than the other.
To avoid or mitigate these symptoms and complications, individuals with Sickle Cell
Disease should take precautions and avoid several risk factors. As mentioned earlier, adults and
children affected by Sickle Cell Disease experience something called a crisis, when pain
suddenly begins and is persistent for a couple of hours or even days. The factors that increase the
likelihood of having a crisis are alcohol consumption, tobacco consumption, excessive physical
activities, being pregnant, exposure to cold weather conditions or any sudden change in
temperature, dehydration, pre-existing medical conditions such as diabetes, etc.
However, theres more to it than avoiding risk factors as there are some factors that are
unavoidable for the disease itself. According to Claster , there are some risk factors that may
increase the probability of having Sickle Cell Disease in general (Claster, Susan, & Vichinsky,
2003). As I previously explained, the only possible way of getting Sickle Cell Disease itself is by
inheriting two defective genes from either one gene from each parent or two genes from one
parent. Moreover, the following determinants cause a baby to be more likely to be born with
Sickle Cell Disease. For instance, genetic factors is the number one factor that should be
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considered and thats when genetic counseling comes in handy. In addition, ones ethnic
background should always be looked at when trying to figure out the chances of getting Sickle
Cell Disease. For me personally, Im from Saudi Arabia where the prevalence of the disease is
very high compared to other countries surrounding me. But also, people from African, Middle
Eastern, and Mediterranean origins should also be informed and educated about this matter.
Whether were talking about the anemia or the trait, Sickle Cell would always get in the
way and be an issue as a result of not being able to marry or have babies with anyone randomly
without a pre-test. I grew up in Saudi Arabia where the prevalence rates of Sickle Cell are very
high for both Sickle Cell Disease and Sickle Cell Trait. I truly believe that its important to
inform people around me about this subject as many Americans have no clue what Sickle Cell is,
especially those who are planning a family. I believe this lack of knowledge on this topic
happened as a result of the low prevalence rates here in the United States compared to other parts
of the world, such as the Middle East and some parts of Africa.
Revolution of the treatment:
The second point thats going to be researched is the treatment of Sickle Cell Disease and
the revolution of medicines throughout the years. Just like any other disease, it starts with
treatments that are rudimentary and basic. At first, there was a medicine called Hydroxyurea
thats known to be the treatment of several types of cancer. After that, in 1997, Hydroxyurea was
approved by the FDA as the first therapy of Sickle Cell Disease. There are other medicines that
could be treatments for other diseases but also used to treat Sickle Cell Disease such as Statins,
which is known as the treatment of high cholesterol. Other treatments might include blood
transfusion and gene therapy.
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Theres another way to treat Sickle Cell Disease especially in severe cases which is
transplanting bone marrow. The pro of this procedure is that it could treat Sickle Cell Disease
forever with a high success rate. However, the success rate for this procedure is 85% but theres
also a fatality rate of %% which makes people skeptical. Another con for this therapy is that you
have to have a person that matches your bone marrow, which is hard to find.
In my personal opinion, the most effective treatment or method to mitigate Sickle Cell
Disease symptoms and to manage a crisis is by attempting a monthly blood transfusions. When
blood transfusions are done on a regular basis can definitely help raise red blood cell counts and
as a result, it may reduce sickling and blood clots that causes health complications.
So are people saved from this?
After defining the disease, its symptoms, and all the possible treatments for it, people
would think that everyone should be able to overcome the disease and be able to manage it.
However, people are still dying from this deadly disease and most of the treatments are only
acting almost like pain relievers, which are not enough. As discussed in the section about
treatment, we tend to see a trend of using medicines that are already known as treatments for
other diseases and trying them on patients with Sickle Cell Disease and if it works, then it works.
Why theres no official cure for Sickle Cell Disease yet? A lot of individuals may think or
consider bone marrow transplant to be the official cure but it cant be. Bone marrow treatment is
very limited as its hard to find a match and a suitable donor. So, what makes the medical field
and professionals procrastinate when it comes to finding the cure?
Dr. David Williams asked the White House research team to allocate only $5 million out
of $755 million thats assigned for research to study the nature of Sickle Cell Disease more.
White House research team already spends billions of dollars annually on cancer research.
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Unfortunately, he waited so long for their response and yet he was disappointed by their
rejection. They justified their rejection by saying that doing research on kids with cancer is more
important. Of course, he wasnt convinced by their reasoning and there are a few theories around
this issue. One of the reasons why scientists usually disregard the effort to research Sickle Cell
Disease is because the research for it is always underfunded and never prioritized. We need a
more concerted work and effort to overcome this problem. Not only we need financial support,
but we also need intellectual resources.
Another theory and reason why this disease isnt given the attention it needs is that its
mostly observed and diagnosed in African-Americans and this group of ethnicity already face
and struggle with discrimination and racism. As sensitive as this topic really is, a lot of
individuals including those who are affected as well as scientists think that this group of people
tends to be disregarded. Africans are already treated badly in a lot of cases and setting so why
not in the medical field? This is not different from what they have to deal with on a regular basis
in hospitals and in other places as well. Sickle Cell treatment here is a microcosm of how issues
of race, ethnicity, and identity come into conflict with issues of health care, said Keith Wailoo,
a professor at Princeton University. I found this quote really interesting and pretty much sums up
the idea of Sickle Cell neglect.
Theres one case of an African woman named NeDina Brocks-Capla who has Sickle Cell
Disease and her son also was diagnosed with the disease. She lost her 36 years old son, Kareem
Jones, because of Sickle Cell Disease. He suffered all his life from this disease but his mom
explained how poor the medical care really was. Moreover, she claims that if her son was given
the proper care he should be getting, he could have survived. The case of Jones isnt the first of
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its kind and if we paid more attention, we would hear a lot of cases and incidence of
discrimination inside the medical field not just against Africans but also against other minorities.
When it comes to my personal opinion and theory, I think that whenever it comes to a
disease where not a lot of people are affected and its also not a communicable and contagious
disease, it tends to be underestimated and scientists are not really interested. The drastic lack of
studies and research might be because most developed countries arent really affected by Sickle
Cell Disease. This type of ignorance is causing a lack of knowledge across the globe, even inside
the medical field. In most countries including the U.S, ones with Sickle Cell Disease can
probably be treated only by a specialized doctor whereas in other countries such as Saudi Arabia,
any physician can help Sickle Cell patients and he doesnt have to be a specialized
hematologist.
In conclusion, the purpose of this research is to give advice on how to recognize signs in
a crisis and how to help prevent any further symptoms. But most importantly, the intention of
this paper is to shed light on why Sickle Cell Disease isnt sufficiently studied and looked at and
what may be the motives of that. As a possible solution to this issue, I personally think all
countries including the U.S should have the capacity to train all physicians and doctors to treat
patients with Sickle Cell Disease so that they can be ready for such cases. As a society, we
definitely can do a better job and we can take some steps in order to improve our knowledge and
as a result, have a higher quality performance at dealing and coping with Sickle Cell patients.
Works Cited
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Anie, Kofi A., and John Green. Psychological Therapies for Sickle Cell Disease and Pain.
Cochrane Database of Systematic Reviews, no. 5, 2015,
doi:10.1002/14651858.CD001916.pub3.
Bloom, Pacific, et al. Probabilities of Sickle Cell Disease. The Mathematics Teacher, vol. 113,
no. 2, 2020, pp. 152155. JSTOR, www.jstor.org/stable/10.5951/mtlt.2019.0062.
Accessed 21 Mar. 2020.
Claster, Susan, and Elliott P. Vichinsky. Managing Sickle Cell Disease. BMJ: British Medical
Journal, vol. 327, no. 7424, 2003, pp. 11511155. JSTOR,
www.jstor.org/stable/25457778. Accessed 21 Mar. 2020.
George, Stephanie, et al. Sickle Cell Disease: Relating Community Health and Heredity.
Science Scope, vol. 38, no. 4, 2014, pp. 3338. JSTOR, www.jstor.org/stable/43691212.
Accessed 21 Mar. 2020.
Hydroxyurea. AHFS Consumer Medication Information, Feb. 2020, p. 1. EBSCOhost,
search.ebscohost.com/login.aspx?direct=true&db=hxh&AN=78170489&site=ehost-live.
Lehmann, H. Treatment Of Sickle-Cell Anaemia. The British Medical Journal, vol. 1, no. 5338,
1963, pp. 11581159. JSTOR, www.jstor.org/stable/20380556. Accessed 22 Mar. 2020
Rees, David C., Thomas N. Williams, and Mark T. Gladwin. “Sickle-cell disease.” The Lancet
376.9757 (2010): 2018-2031.
Serjeant, Graham R. “The geography of sickle cell disease: opportunities for understanding its
diversity.”
Taylor, Lou Ella V., et al. “A biopsychosocial-spiritual model of chronic pain in adults with sickle
cell disease.” Pain management nursing 14.4 (2013): 287-301.
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Wailoo, Keith. Dying in the City of the Blues : Sickle Cell Anemia and the Politics of Race and
Health. University of North Carolina Press, 2001.
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