Running Head: SICKLE CELL ANEMIA 1

Sickle Cell Anemia: A Review of the Literature

Kennedi L. Slayton

Hampton University, VA
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Abstract

Sickle Cell Anemia, the most overlooked disease in the world that is effecting millions

of lives world-wide. Sickle cell is due to genetic mutations and Iron deficiency in the

blood. The red blood cells in our bodies are not getting enough oxygen as needed to

survive. Thus, they begin to sickle(shed) and become a crescent shape. This makes

things even more complicated because the shape is now not able to fit and go along

with blood flow. So, it is clogging passage way for other materials and nutrients causing

organ failures.
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Sickle Cell Anemia: Literature Review

Sickle Cell Anemia is a commonly overlooked genetically inherited blood disorder

that can be life threatening. So why arent more people aware of this deadly disorder

then? This question is the main purpose behind this research paper. But to break down

this question even further, the four questions that can help gain a better understanding

about sickle cell are:

The Background of Sickle Cell Anemia: What is it?

Percentage wise, who are all being affected by this disorder?
Can it be Managed? Is there a cure?
How does having Sickle Cell Trait Differ?

If more people were aware of Sickle Cell Anemia, it would benefit and help those

going through it. By creating room for charity work and endorsement funds aided

towards the care of patients and medical bills. Also, it can help catch the attention of

those with the trait because it may possibly be there child in the future going through

this.

The Background of Sickle Cell Anemia: What is it?

As stated above, Sickle cell Anemia is blood disorder caused by an inherited

abnormal hemoglobin. This affects the red blood cells by stopping them from getting the

right nutrients and oxygen they need for survive. Thus, the cells start to sickle and are

crescent in shape. Due to the shape, it becomes very crucial for the body stopping the
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flow and transport of other materials to other organs. Which is why patients with this

disease are in excruciating pain.

Discovered in 1910, and the first analyzed case was by Dr. Ernest Irons. He noticed

the shape of the cells and from then on sickle cell became a mystery. Scientist couldnt

wrap their minds around the concept as to why these blood cells are lacking oxygen and

saw this as an unknown disease with no knowledge as to how it comes about.

Generation after generation are affected by those who are carriers of the trait and as the

generations progress more are likely to inherit the full-blown disease (As shown in figure

1).

Green-normal
Red-sickle cell trait
Blue-Sickle cell
(Figure 1)

The symptoms that arise from having sickle cell are fatigue, excruciating pain

episodes, arthritis and swollen joints, lung and heart injury, and leg ulcers. So many

complications are due to the bodys organs being damaged. Now this is just by having

the disease in general but by having the trait not all these symptoms are present.

Currently medicine hasnt been able to determine if patients with the trait experience

any side effects. But from prior experience and being one of those patients with the trait,

I find that strenuous exercise and environments make me extremely fatigue and in pain.
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What Population is Affected by this Blood Disorder?

Throughout the process of this research turns out that more people are affected by

this more than you know mainly in the African American community. Whether by

contracting the disease or by just having the trait. 1 in four children are at risk of having

the full-blown disorder and in the UK, there are 20,000 with sickle cell disease and trait

meaning that for future generations 2 million people could manifest the full

blown(Barton,1). Sickle cell is something not often talked about but it is spreading world-

wide and it is important to consider when starting a family if the significant other is a

carrier or not.

Is Sickle Cell Anemia Manageable? Is there a Cure?

So far there has been no found cure for sickle cell and in previous encounters

doctors managed it through gene therapy, bone marrow transplant, and blood

transfusions. But as of March,2017 there has been said to be a cure after all through the

process of gene therapy. Dr. Marina Cavazanna, senior author of the study and head of

the biotherapy department at Necker Childrens Hospital, said that all biological tests

we perform lead us to think he is cured, according to The Chicago Tribune (Spenlow,

Doc). Although the results are looking good right now, she and the rest of her

department can only be sure by more follow ups. Looking for any more changes and

possible upgrades to the gene therapy treatments.

There are not much stories about sickle cell patients in the news. With confidential

files of results and deaths, sickle cell is progressing annually. It is surprising researching
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how many people are living with this disorder. Famous actors and football players like

Tiki Barber and Larenz Tate from, are managing and coping with it and it is hard to

distinguish who has it.

How does Sickle Cell Trait Differ?

Having the sickle cell trait is not as life threatening as having the actual disorder but you

should be mindful as to the precautions. There have been cases where people even

with the sickle cell trait has passed away or experienced certain side effects.

Thousands are affected by just carrying the trait and following them, generations may

obtain the disease. This will become a continuous cycle and knowing that it runs in

bloodline can help prevent further diagnosiss.

Review of Literature

For my primary search a survey was conducted and majority of the people who

answered the questions had an idea as to what sickle cell was. They all heard it from

either a teacher, friend, or doctor. But the main consistency recognized was when the

question how many people do you think are affected by this annually? was asked and

they had no clue and were surprised by the results. For the secondary research, it was

hard trying to find patients who have had the trait and suffered from it. One prime

example was Ryan Clark, a NFL player for the Washington Redskins (figure 2). In his

case, it appears that by him being a carrier of the trait has turned into the full-blown

thing of Sickle cell anemia. This is due to his extreme workout condition and strenuous

heat climate changes. Gene mutations occur naturally and unnaturally. In the case of
sickle cell, which is the change of one amino acid in the DNA can be effected by

extreme weather and diets (unhealthy lifestyle).

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(Figure 2, Ryan Clark shares his story,

http://www.sbnation.com/nfl/2014/12/10/7361549/ryan-clark-pedicure-washington-football)

The past patients who have died due to having the trait of sickle are predominantly

football players. Or any other active participants who work out continuously in extreme

weather conditions that require a lot of oxygen. Both my primary and secondary

research tie into each other when it comes to how many people are aware? The lives

lost probably were not aware. Doctors usually dont see having the trait as a problem

until it becomes one. They dont warn the, of the precautions to take. As Ryan Clark,

vaguely states in his article in Figure 2.

In conclusion, the results from the survey proved that people are aware of this

disorder but dont see it as a big deal. They know very little about the topic but they do

know some of the important aspects of it. Sickle Cell Anemia is still undergoing

investigation to see if there a permanent cure. But for now, the word should be spread

and meantime try to lower the number of people affected annually.

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Reference
Barton, P. (n.d.). Help for Sickle Cell Disease. How many people suffer with sickle cell.
Retrieved March 19, 2017, from https://sugars4life.wordpress.com/2007/08/21/how-
many-people-suffer-with-sickle-cell/
Kogog, S. (2014, December 10). Sports News, Scores and Fan Opinion Powered by
320 Sports Blogs. Ryan Clark gets a pedicure and talks about the time he almost died -
SBNation.com. Retrieved March 19, 2017, from
http://www.sbnation.com/nfl/2014/12/10/7361549/ryan-clark-pedicure-washington-
football
Spenlow, D. (2017, March 7). The Marshalltown. Doctors discover a cure for sickle cell
anemia. Retrieved March 19, 2017, from http://www.themarshalltown.com/doctors-
discover-a-cure-for-the-sickle-cell-anemia/25989
William, S. (2016, September 7). SCD. Sickle cell anemia. Retrieved March 19, 2017,
from http://www.medicinenet.com/sickle_cell/article.htm
Winter, W.P. (n.d.). Sicklecell.howard.edu. A Brief History of Sickle Cell Disease.
Retrieved March 19, 2017, from http
://www.sicklecell.howard.edu/ABriefHistoryofSickleCellDisease.htm