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Sickle Cell Disease Concept


Sickle cell disease (SCD) is a major health concern in the United States. According to Akingbola et al. (2016), the introduction of hydroxyurea (HU) was a major relief to patients as it has been proven to alleviate symptoms of the disease. Adewoyin, Oghuvwu, and Awodu (2017) argue that this mode of managing SCD is yet to be universally embraced by medical practitioners in the country. Some still believe in other forms of medication. Research has proven that this form of managing SCD helps in prolonging the life of a patient by protecting vital organs and reducing pain (Sandhu & Cohen, 2015).

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Background of the Problem

Sickle cell anemia is an inherited health problem that medical researchers have been working tirelessly to find ways of managing. The first case of a sickle cell anemia patient was recorded in 1910 (Sandhu & Cohen, 2015). Walter Noel, a dental student in Chicago, went to the hospital complaining of episodic pains and signs of anemia. Dr. Ernest Irons examined the blood and noticed that his red blood cells had the shape of a sickle (Adewoyin et al., 2017). Working closely with Dr. James Herrick, they were able to examine the condition and discovered that it was a complex health problem, which medical experts had not encountered (Sandhu & Cohen, 2015). Since then, a lot of research and tests have been conducted to find the best way of dealing with the problem.

Theoretical Foundations

Theoretical models may help in explaining the relevance of HU among SCD patients and the manner in which both practitioners and patients can be convinced to embrace it. The Health Belief Model (HBM) is one of the most appropriate theories that can be used in this project. Adewoyin et al. (2017) define HBM as “a psychological model that attempts to explain and predict health behaviors” (p. 258). This theory is based on three main concepts.

The first concept is that a person is likely to take a health-related action if he or she believes that doing so will avoid negative health consequences. The second concept is that one is likely to take appropriate action if the person has a positive expectation that when a recommended action is taken, a negative health condition would be avoided (Sandhu & Cohen, 2015). The last concept is that one will be motivated to take a health-related action if he or she has the right knowledge on how to take a specific action.

The theory will be relevant in this project because it outlines strong reasons why it is necessary to popularize the use of HU when dealing with SCD patient. As explained above, the perception that people have towards a given mode of therapy defines whether they will embrace it. When a patient is convinced that this method will alleviate the symptoms and reduce pain, he or she will demand it. The pressure from patients will push doctors to embrace this mode of medication. The theory also emphasizes the importance of understanding how a given treatment method words for it to be acceptable. Doctors can only embrace the new method if they understand the manner in which it works and the possible consequences. Sandhu and Cohen (2015) explain that in many cases, doctors are keen on avoiding methods that they have not used before for fear of making mistakes.

Review of the Literature

Reviewing relevant literature helps in identifying the main themes that will be the primary focus of the study. In this section, the researcher has identified three main themes that will be discussed further when conducting the study.

Patients’ Perceptions of HU Efficacy

The perception of patients towards HU efficiency determines whether they will request for it or not. The following factors have a direct impact on patients’ perception towards HU.

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Information from medical experts

According to Sandhu and Cohen (2015), the information that patients receive from experts has a direct impact on their beliefs. If experts popularize this form of medication as being effective, patients will trust it.

Information from colleagues

Information that patients receive from colleagues suffering from similar complications affects their trust. When these colleagues attest to the effectiveness of the drug, these patients will be motivated to embrace it

Patient’s experiences

Patients who have received the medication before are likely to form an opinion based on their experience (Keikhaei, Yousefi, & Bahadoram, 2016). If the medication alleviated the symptoms before, they would support its use.

HU’s Impact on Organ Damage

According to Sandhu and Cohen (2015), proper use of HU reduces organ damage among patients suffering from sickle cell disease. The following are some of the ways in which this medication helps to protect these organs.

Ability to increase fetal hemoglobin levels

A study by Lebensburger, Grosse, Altice, Thierry, and Ivankova (2015) revealed that HU could increase fetal hemoglobin. In so doing, the blood can transport necessary nutrients to sensitive body organs, ensuring that they remain healthy.

Decrease the severity and frequency of SCD exacerbations

SCD exacerbations have a direct negative impact on the heart of patients suffering from sickle cell disease. However, this medication could reduce the severity and frequency of SCD exacerbations, which in turn helps in protecting the heart (Tafrali et al., 2013).

Motivating regular check-ups

Patients who embrace HU are often motivated to conduct regular tests to ensure that they remain healthy when taking the medication. Regular tests make it possible to identify if any of the vital organs is affected. Early diagnosis makes it easy to address the problem (Fitzhugh et al., 2015).

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Increase in Patients’ Life Expectancy

The primary goal of these new methods of managing sickle cell disease is to ensure that patients’ life expectancy is prolonged. Eliminating pain associated with symptoms of this disease is not enough (Milosevic, Levy, Alcolado, & Radomsky, 2015). The following are some of the ways in which HU can help prolong lives of patients suffering from SCD.

Decrease the frequency of pain episodes in patients

Some of the symptoms of SCD are often accompanied by severe pain. HU is known to eliminate such pain, making life more meaningful. Matt and Matthew (2013) argue that some patients often consider taking their own lives because of such cases of extreme pain. Eliminating the pain prolongs the life of the patient.

Prevent various life-threatening complications

As explained in the section above, HU helps in protecting vital body organs such as the heart. Eliminating these life-threatening complications such as heart failure prolongs the life of a patient suffering from SCD (Wang et al., 2013).

Regular medication

Patients who are enrolled in this HU programs are often encouraged to take regular medication as a way of combating their condition. Maintaining regular medication helps in suppressing symptoms of SCD (Tafrali et al., 2013). Such a patient is more likely to lead a near-normal lifestyle than their colleagues who are not maintaining regular medication.

Problem Statement

It is not known by many medical practitioners and patients that Hydroxyurea is an effective method of managing sickle cell disease. Although studies have revealed the effectiveness of this form of therapy, it is yet to be widely embraced as the most appropriate medication for patients with sickle cell disease. It is necessary to undertake this project because it focuses on the promotion of this form of medication when managing SCD.

It will address some of the doubts that some of the practitioners have about the usefulness of HU. If it is embraced, this new method will benefit the entire population of those with SCD not only in the United States but also in other parts of the world. The project will help in solving the problem of uncertainty among practitioners when it comes to using HU to manage SCD.

Purpose of the Project

The purpose of this project is to investigate if taking hydroxyurea by adults aged 18-30 who are diagnosed with sickle cell disease decreases sickle cell crisis (SCC) severity. The study was to be conducted within a period of six weeks. To achieve this goal, the researcher intends to conduct quantitative research with a retrospective chart review. Of interest will be to determine the number of respondents who believe that using HU can significantly improve the condition of an SCD patient as opposed to embracing the other existing methods.

The chosen design will make it possible to present the findings made using relevant graphs and charts for clarity. It will be easy to determine the benefits of this medication by statistically comparing the level of improvement made by those who take HU and those who do not. The outcome will help clarify the significance of this method of managing sickle cell disease. The project will specifically affect patients with sickle cell disease and all practitioners who are responsible for offering them medical services.

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To the patients, the project will help in determining a better medication that can prolong their lives and eliminate adverse effects of SCD. To the medical practitioners, the report will provide a better way of dealing with SCD in a way that reduces cases of hospital readmissions. The project will make an important contribution in this field by providing evidence of the effectiveness of a new method of managing SCD.

Clinical Questions and Variables

According to Tafrali et al. (2013), developing an effective research question is critical when planning to conduct research. The question defines the data that is to be collected to meet goals and objectives of the study. A properly structured question should capture the primary aim of research. As such, it was necessary to develop an appropriate question to be used in this project. The researcher developed the following PICOT question to help in conducting the investigations.

In adults (aged 18-30) diagnosed with Sickle Cell Disease (SCD), does taking Hydroxyurea compared to those not taking Hydroxyurea decrease Sickle Cell Crisis (SCC) severity over a six- week period?

The affected population (P), as shown in the question above, is patients diagnosed with sickle cell anemia aged 18-30 years. These young, energetic adults are expected to play leading roles in the country’s economic growth. When they are affected by this problem, their productivity is significantly reduced. Taking Hydroxyurea is the intervention (I) plan. It is believed that taking this medication may help improve the condition of a patient with SCD. The intervention does not only eliminate the pain but also protects vital organs within the body, increasing the patient’s life expectancy.

The comparison (C) is not taking HU among patients suffering from sickle cell disease. The analysis will try to compare the condition of a patient who takes this medication with that of a patient who does not. The comparison will provide the needed proof that taking HU is significantly important for patients with SCD. Decreasing the severity of sickle cell crisis if the outcome (O). It is expected that this intervention will have a positive impact on the affected group as discussed above.

The medication should be scientifically proven to offer the expected therapeutic relief to the patient. Finally, the time (T) set for the outcome to be evident is six months. The period is long enough to enable the medication to have the desired impact on the patient while at the same time being short enough to make it possible to complete the project within the set time. The outcome of the study will inform the practice by determining the relevance of HU among SCD patients.

Significance of the Project

This project fits with other similar studies related to the introduction of new forms of medication. According to Wang et al. (2013), change is always a difficult concept to introduce in any setting. In most of the cases, people tend to fear shifting from one popular method to another. The same is the problem when it comes to managing sickle cell disease. Most doctors are reluctant when it comes to using HU because they are accustomed to other traditional approaches.

The project will help them overcome the fear of change so that they can embrace HU as an appropriate and more effective way of managing SCD than other traditional methods. The findings made in this study will introduce new knowledge in the overall research within this field. The document will be of great value to cardiologists and physicians who frequently handle patients with SCD.

Rationale for Methodology

The chosen method for the project was quantitative analysis. It was considered best fit for the PICOT question because it facilitates statistical analysis of data. As shown above, the PICOT question focuses on conducting a comparison between those who use HU and those who do not. The goal is to ascertain if those who use this new method of medication experience little pain and improved quality of life than those who do not use it.

The nature of the question makes it necessary to use quantitative data to compare the two groups. It will be possible to prove, based on the statistical findings that this method of medication is effective and should be given priority by cardiologists and other medical specialists. Another factor that made it necessary to use this method is the use of charts to present the outcome of the analysis. When charts are used, it is easy for the audience to understand the significance of HU in managing sickle cell disease among adults aged 18 to 30 years.

Nature of the Project Design

The chosen research design for this project is a retrospective chart review (RCR) also known as a medical record review. Matt and Matthew (2013) define RCR as “a type of research design in which pre-recorded, patient-centered data are used to answer one or more research questions” (p. 78). When using this design, data may be presented in various forms such as electronic databases, notes from healthcare providers, and results from diagnostic tests among others (Badawy et al., 2017).

The method has gained massive popularity among researchers conducting studies in disciplines related to healthcare. The design was specifically appropriate in answering the research question stated above. The fact that the design is currently popular among medical researcher further attests to its effectiveness in facilitating a thorough investigation of a given concept in this field.

Instrumentation or Sources of Data

Data will be collected from two main sources. The first source is secondary data that will be obtained from books and reliable journal articles. According to Araujo et al. (2016), it is advisable for a researcher to review what other scholars have found out in a given field. It not only eliminates duplication of already existing information but also provides a background for further investigation. It identifies research gaps that need focus to expand the body of knowledge in the specific field.

The second source was primary information obtained from sampled respondents. The researcher will conduct a survey to facilitate primary data collected. A mixed survey was considered appropriate in ensuring that both quantitative and qualitative data would be collected. The survey will be conducted with the help of a questionnaire. The questionnaire will be developed to ensure that interviews are conducted using standardized data.

It was important to ensure that both sources of data are reliable before using them to make a conclusion. Journal articles used had to be peer-reviewed from reliable websites. It was also necessary to ascertain the academic credentials of the authors. The use of numerous secondary sources also enhanced the reliability of data. The validity of primary data will be enhanced by eliminating influencers. Personal opinion of the researcher will not influence the nature of primary data collected.

Data Collection Procedures

Primary data will have to be collected from a sample of respondents. Primary data collection will be done in different steps. The researcher will start by identifying healthcare institutions that will take part in the study. The next step will be to obtain informed consent from the management of these institutions. A letter will be written to the administrators explaining the relevance of the study and the role of the institutions. The third step will be to select a sample of participants (SCD patients).

A set of two participants, those who will take HU and those who will note. A sample of 60 respondents (30 in each set) will be needed. The participants will be recruited from the participating healthcare institutions. Data will be collected from both sets by determining if HU has any positive impact on SCD patients.

Data Analysis Procedures

The demographic data collected from participants will be analyzed using descriptive statistics. As explained above, participants will be patients suffering from sickle cell disease aged between 19-30 years. When conducting primary data analysis, the focus will be to answer the following PICOT question below in great details.

In adults (aged 18-30) diagnosed with Sickle Cell Disease (SCD), does taking Hydroxyurea compared to those not taking Hydroxyurea decrease Sickle Cell Crisis (SCC) severity over a six- week period?

It is important to note that the question focuses on comparing how sickle cell crisis severity is reduced between two groups, one using HU and the other is not. Kruskal–Wallis one-way analysis of variance will be the most appropriate inferential statistics that will facilitate comparing data from the two groups. This analysis will make it possible to compare results obtained from the two groups identified above. To ensure that the data meet the assumptions for the selected inferential statistics, care will be taken to ensure that the two groups are statistically asymmetrical. The priori alpha level of 0.05 will be used. It means that the confidence level in the study will be 95%.

Ethical Considerations

When conducting this research, it will be necessary to observe ethical concerns as advised by Curtis, Danda, Etzion, Cohen, and Billett (2015). The chosen methodology makes it necessary to collected primary data from respondents. Unlike the randomized controlled trials, the chosen design will require the researcher to conduct interviews with sampled respondents. One of the ethical considerations that will be observed is to ensure that participants remain anonymous.

This will be done by assigning participants numerical names instead of using their real names to enhance privacy and confidentiality. Personal information collected from them will not be shared with third parties as a way of enhancing the confidentiality. When sampling, the researcher will not use coercion. Participation will be on a voluntary basis. Informed consent will be obtained whenever necessary to ensure that institutional rules and policies are respected.

Any potential conflict of interest noticed will be avoided as much as possible. The researcher will adhere to the Belmont report key principles of respect, justice, and beneficence when interacting with the participants. As required by this principle, views and interest of participants will be respected. Any action that may have a direct negative consequence on the participants or other parties will be avoided when conducting this study.


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