A Draft of methods used in research (the topic about SICKLE CELL ANEMIA)

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LITERATURE REVIEW ABOUT SICKLE CELL ANEMIA 1

Literature Review about Sickle Cell Anemia

Student’s Name :Abdulmohsen Almohammedali

Professor’s Name :Ahmed Abdulhadi

Courses : CLS604

Date :11.10.2020

LITERATURE REVIEW ABOUT SICKLE CELL ANEMIA 2

The literature review as the research method in the research is substantially beneficial as

it renders the research to investigate the current literature about the topic under investigation.

The situation helps them gain a vital overview that induces the different gaps that need to be

filled from further research. Additional researcher articles have been articulated to determine and

research the various problems in sickle cell disease

According to the World Health Organization data estimates that reflect on the SCD, there

are about 3500 children born with sickle cell disease annually. From the WHO data, around 20%

of the children will not attain the age of five years of age due to the complication associated with

sickle cell diseases. The student's situation can only change if the children are diagnosed at the

appropriate time, and the proper treatment enhanced effectively. The data obtained from the

APAE –Salvador from the reposted data of 2000 upon the start of the neonatal screening that was

conducted for the hemoglobin diseases, identified that there are about 1655 lives of the newborn

that are associated with sickle cell anemia and the single child with sickle cell traces for every

17th birth. Thus based on the effective analysis of the studies carried in the health organization,

there is an indication that the magnitude of the sickle cell diseases. Most of the children with

CSD have various complications of the variables and squabble that bring about hearing

impairment in the children and the adults. In Kenya, the retrospective examination was

conducted with a sample group of 360 patients who had the age group of 7-21years. Who had the

trait of sickle cell disease from the assessment? After the neurological assessment, the squeal's

sample was found to be 67% of the total sample population. Around 33% of the population had a

stroke, visual disorder, and inability to hear. There was a need for longitudinal audiology and the

effective neurological flow that should be conducted to the patient with sickle cell anemia from

LITERATURE REVIEW ABOUT SICKLE CELL ANEMIA 3

the analyses. Around 21.1 % of the sensorineural hearing impairment is caused by sickle cell

disease. Cooperatively of the 3.6% from the control group without the sickle cell Anemia

Wilson & Nelson (2015) conducted the study that its primary purpose was to determine

sickle cell Disease pain management, especially in Adolescence. From the investigation, that

notes that around 98000 individuals were experiencing the Sickle cell disease threat. The study

indicated that the disease is mostly characterized by the prevailing genetic blood disorder that

affects human blood cells. Acute severe pain is caused by the ischemia tissue caused by the

existence of the blood vessel occlusion, which hinders the progress of the blood flow in the body.

The authors dissipated that nursing knowledge is essential, and it facilitates the patient from

reducing the stigma and provides the effective management of the condition.

The author obtained the qualitative and quantitative data from the patient and the hospital

database record, which included MEDLINE, PubMed, and other significant nursing sources to

get the data. The author narrowed the information they obtained by retaining the date of the

publication. The author noted that there was insufficient concrete evidence that connected the

real indicator that was used to improve the pain management for the patient with sickle cell

disease. From the article, there was an indication that there were many delays associated with the

government among the patient. Thus, the result indicated the need for more advancement that

will facilitate the change in the management procedure. Notably, the author noted that there

should be better nursing and health work guidelines that can embrace the better management of

the health conditions. In this case, the authors stated the requisite need to understand the

patient's pain better. The research advocated for understanding the evidence-based practices that

should be developed to provide consistency among the health workers, especially the nurses who

were providing the nursing care for the patient. The research concluded that the need for protocol

LITERATURE REVIEW ABOUT SICKLE CELL ANEMIA 4

change would ensure that the patient's caring with the sickle Cell Diseases well managed to

prevent the VOC pain that occurs by diagnosing the acute problem related to the disease. The

study recommended adopting the pain algorithm management that will ensure that there is the

control of rapid pain control for the patient, thus initiating proper treatment.

Wang et al. (2011) used the comprehensible Delphi technique to obtain the various set of

final quality care indicators for the patient with the Sickle Cell Disease complication. In this

case, the Delphi technique was the combination of anonymous ratings conducted by the multiple

experts who induced the ideas through face-face discussion. The expert used a method to

indicate the best appropriate and effective quality of the indicator that portrayed the patient's best

care with the Sickle Cell Disease complication. The author noted that most of the children

diagnosed with Sickle Cell Disease were at a relatively higher risk for acute and long-term

impediments that can result in a higher mortality rate. From the analysis, the author noted that

there was a deficiency in the efforts that are considered to improve the excellent access to quality

care for the patient with the Sickle Cell Disease conditions. Comparatively, the rate of

management was relatively lower as compared to the other patient with chronic conditions.

In conclusion, there is a need to increase the clinical evidence that will provide the

information that provides the best ways to induce a better quality of care indicator that will

change the system of the treatment. Consequently, Haywood et al. (2013) researched that the

main aim was to determine the rate of time waiting that the patient with the Sickle Cell Disease

complication takes before their cases are solved compared to other patients with other aligning

medical conditions. The study obtained qualitative and quantitative data from the nursing

database and administered the questionnaire to the hospital's different stakeholders. The authors

employed the cross-sectional comparative tabulation of the data from the various conducted

LITERATURE REVIEW ABOUT SICKLE CELL ANEMIA 5

surveys ranging from 2003-2008. The research used the generalized linear model technique that

was used to examine the ratios among the patients who were having the Sickle Cell Disease

complication wait time in the ER before the initiation of the treatment procedure. Haywood et al.

(2013) indicated that more than 200000 patients with the complication problems of the Sickle

Cell Disease are seen regularly in the ER for the treatment therapies.

The research reported that most of the patients with Sickle Cell Disease are seen aligned

in the ER. Most of them are unhappy with the kind of services they were equipped with within

the treatment procedure. More so, the patient was subject to delay, and they were taking

substantial extra time before they receive the treatment, where some of them wait for more than

four hours. Before they obtain the initial dose of the pain medication. In this case, Haywood et

al.'s (2013) study indicated that the SCD related visits are around 171989 patients. However, the

study showed that most patients with SCD wait longer in the general waiting rooms than

available patients. The study concludes that there was the minimal provision of care for the

patient with SCD, thus limiting the quality of the care for the treatment of the condition.

Dampier et al. (2013) used the randomized technique controlled trial that was used to

give a comparison of two patient-controlled analgesia. Dosing strategies for the children and the

adolescent individual with Acute SDC pain. The study examined the various experiences related

to the protocol, especially which was inducing the development and the implementation that

aimed to progressively analyze the value that the patient experience significantly associated with

the pain before the initiation of the treatment of the problem. For the sufficient balance of the

analysis, the research investigated 31 clinical sites to improve better the Pain management and

outcome with the various strategies aimed at the trial. In this case, the investigation employed the

randomized, single-blind two arms inpatient analgesic that imposed the clinical trial

LITERATURE REVIEW ABOUT SICKLE CELL ANEMIA 6

randomization. In the case study, the total number of 33 adults who were above 18 years was

selected. More so, the study established 12 pediatric patients that were below 18 years. The two

groups of the sample population were induced with the PCA dosing strategies. In the case study,

the patient's PCA was morphine with lower doses demands using the lower constant infusion.

During the investigation, the goal of the opioids uses was to enhance and provide a practical

hypothetical ideal that had the relation with the patient with the SCD complication. During the

study's examination, Dampier et al. (2013) employed the use of the analog visual scale that was

used to keep the measurement, especially during the duration of the patient hospitalization, using

the different pain measures as the advanced secondary values.

In their argument, Dampier et al. (2013) implicated the compelling need to optimize the

amount of the PCA dosing strategies for the different patients with SCD based on the numerous

hazardous effects associated with the chronic anemia on an opioid. Dampier et al. (2013)

observed that chronic anemia in the patients increases the level of the cardiac output, which

induces the increases in the rate of the hepatic and renal flow. Notably, several; analgesics

analyses indicate that they use the morphine metabolic pathways that cause the hepatic and renal

excretions, thus inducing the rate of the plasma clearance to the different patients with the

problem of the SCD complication.

Furthermore, Dampier et al. (2013) implicated the use of the conventional drug dosing to

the patient with the SCD health complication can result in the sub-therapeutic drug level,

especially whenever providing the continuous infusion, Thus causing the continuity of the

pharmacokinetic abnormalities to the patient population. The examination recommended for the

effective maintenance of the prudential drug level can be effectively maintained by inducing the

demand dose PCA strategy. Dampier et al. (2013) argued that the PCA dosing is used to ensure

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the better delivery of the parental opioids analgesics that induce better relieving of the pain

fenced by the SCD patient in the communities. Dampier et al. (2013) had an indictment that

only implicates the generation of the few studies related to the PCA optimal dosing strategies

that treat the postoperative setting.

Among the conducted analysis most of them indicated that numerous physicians use the

PCA in the maintenance of the pain for the individual with the SCD complication. . Dampier et

al. (2013) concluded by advocating for the effective care of the frequency demand dose of the

PCA strategies for both adults and the pediatric patients. The study results indicated that adults

had positive responses that demonstrate the improvement compared to the pediatric participant

who suggested having the gain associated with pain control. The gap related to the reduction in

the pediatric patient's control measure induces the gap that requires to be examined in future

studies to facilitate the effective use of the knowledge to improve the advancement that can bring

the solution to the SCD complicated pediatric patient.

Vijenthira et al.'s (2012) was the examination that planned to investigate the VOC and the

SCD. The review aimed to facilitate the significant benchmark that induces the outcome of the

pain and the clinical development that is experienced by the patients with SCD. Vijenthira et al.'s

(2012) examined that the management of the acute pain in the different patients with SCD does

not effectively reflect the translation induced in the daily practices. In the study, Vijenthira et

al.'s (2012) employed the retrospective cohort from the electronic patient chart that associates the

patient with the SCD who had an influential position to meet the research criteria. In the

examination process, Vijenthira et al.'s (2012) took a sample of 50 individuals for admission for

the SCD and VOC. The data obtained were analyzed using the SPSS software Version 17. From

the analysis, the result indicated that 51% of the patient had pain experiences on site. The

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number of individual patients who had pain in various places was 41%. More so, the number of

patients who had problems in the legs was 37%. The number of patients who had pain in the

chest was 33%. The total number of patients who had pain in the abdomen was 12. %. The total

number of patients who had the problem that was reported in the arm was 12%. Notably, for the

overall patient presented in the ER, 43 % of the patient had not taken the pain nursing and

medication drugs before arriving at the ER. More so, of the total population, 27% of the

population had taken some form of the analgesic.

The other remaining population of around 57% had taken the two combinations of the

opioids and the acetaminophen before arrival. In this case scenario, Vijenthira et al.'s (2012)

implicated that the current states of the current VOC practice need to induce to be advanced

improved and that the benchmark that is required whenever evaluating the children with the

complication of the SCD for pain management. Vijenthira et al.'s (2012) noted that the

significant employment of the pain assessment and documentation strategy and the significant

clinical results could provide an avenue for identifying and delivering the practical clinical

setting for the hospitalized children with the complication of SCD. Vijenthira et al.'s (2012), in

his examination, revealed that there is a need for the advancement of the study that can facilitate

the adequate understanding of the various practices and on the numerous effect that induces the

improvement of the pain outcome in the patient with the SCD. Vijenthira et al.'s (2012)

examination indicated that the big problem of the SCD complication is managing the pain, which

remains untreated throughout the patient hospitalizations. Thus the author in the research finding

articulated the necessity that can induce the better management of the vast-occlusive pain,

especially in the children. Hence Vijenthira et al.'s (2012) in his examination suggested the effect

of better control of the VOC and improvement of the clinical outcome that can reduce the pain

LITERATURE REVIEW ABOUT SICKLE CELL ANEMIA 9

intensity for the significant improvement of the patient. In his finding, Vijenthira et al.'s (2012)

indicated that around 24% of the patient with the SCD pain reflected in the ER has no adequate

documentation after being triaged. More so, in the pediatric patient's case, around 55.5% of the

group had no documentation of the pain score. Vijenthira et al.'s (2012) concluded that for the

better prevention and compartments of the pain for the adult and the children with SCD, practical

pain assessment and good documentation are efe4ctive to reduce episodes. Therefore, the study

implication indicated that there is insufficient relief associated with the replacement of sickle cell

pain for the patients.

Swanson, Grosse, & Kulkarni (2011) conducted the study examination to investigate the

disability among individuals with sickle cell diseases complication. From the investigation, the

researcher articulated that chronic conditions can induce disability due to the primary impairment

and the advancement of secondary complications. Swanson, Grosse, & Kulkarni (2011) obtained

the nursing database data, especially the Pubmed search, where they examined the SCD

combinations. The article the investigation was narrowed to 2005 to facilitate the significant

gathering of the information. The study implicated that most of the children and the adult who

have the complication of the SCD have a higher probability of developing the impairments.

Swanson, Grosse, & Kulkarni (2011) noted that most of the patients who have the impairments

implication due to the SCD had numerous restrictions that limited their participation. Swanson,

Grosse, & Kulkarni (2011) used the clinical setting using the medical model of disability that

mostly induces recognizing the different accumulative injury to the multiple other organs

situated in the red blood cell, thus causing pain. The researcher was able to incorporate the

medical model's psychological approach that facilitated individual isolation from the social

context.

LITERATURE REVIEW ABOUT SICKLE CELL ANEMIA 10

The researcher's use of the social model of the disability was so effective as it was

drawing the various functional outcomes that were inducing the product and the interaction

within the context of the social and physical environment. Swanson, Grosse, & Kulkarni (2011)

examined that pain is the individual's everyday experience with SCD. Swanson, Grosse, &

Kulkarni (2011) abducted the target population of around 232 adults with the SCD complication

in the prospective cohort study. The study noted that approximately 50% of the individual under

investigation reported pain more than half of the daily record. More so in the U.S clinical study

from the sample population of 41 adults with the complication associated with the SCD indicated

that pain occurred regularly for the individual with difficulty. Swanson, Grosse, & Kulkarni

(2011) noted that the longitudinal data implies a decrease in the rate of cognitive adaptability and

functioning among the different kids who had the SCD. The examination indicated that the

scenario was the implication of the function of the cumulative neurological impairment

associated with the individual with the cerebrovascular infarcts.

Swanson, Grosse, & Kulkarni (2011) from the study that analyzed the CSSCS data for

the sample population of around 223 children followed for nine months indicated that there was

an average reduction in the rate of the intelligent quotient score of approximately 2.3 points,

especially for the children with the retained normal MRI status. Comparatively, the children who

had the silent infarcts issues were losing the intelligent quotient with around 6.4 points.

Swanson, Grosse, & Kulkarni (2011) examined that most of the children born with the

complication of the SCD have the required right to expect to live their independent lives to

enhance community building's full participation. Swanson, Grosse, & Kulkarni (2011), in their

investigation, identified that there was a limited number of the study that were articulating the

assessment of the disability and the effective functioning of the individual with the of SCD

LITERATURE REVIEW ABOUT SICKLE CELL ANEMIA 11

complication. Most of the studies have a short duration followed up, thus undermining the

progressive assessment of the impairment and the resulting impact on the individual with the

SCD. Swanson, Grosse, & Kulkarni (2011) examined the need to use the population-based

longitudinal data that trespass all over the life of the SCD to facilitate the effective correlation

functional outcome with the various intervention and the environmental factors. Swanson,

Grosse, & Kulkarni (2011) concluded that there is a need to better the understanding of the

multiple factors that result to the limiting in the numerous participation of the people with SCD.

And identify the best intervention that can hole the better promises to improve the outcome that

can reduce the pain to facilitate social and emotional development.

Sarri, Grammati, et al. (2018) examined the study to investigate the patient-reported

outcome instrument's assessment in Sickle cell diseases. In the investigation, the author noted

that SCD is a chronic condition associated with mortality and morbidity. The author articulated

that the disorders are characterized by various clinical symptoms, mostly the painful vaso-

0cclusive crises that have negative impairment related to quality health care progress. Sarri,

Grammati, et al. (2018) in the study applied the systematic literature that was performed Medline

and the Embase. The study confined the article from the year 1995-2017 publication that

reported the proper PRO tools and instruments used to control the trial procedure and the real-

world setting. Sarri, Grammati, et al. (2018) conducted the SLR using the transparent and

reproducible technique recommended by the preferred and better systematic review.

According to Sarri, Grammati et al. (2018), the 21 studies adopted in the SLR report a

total of around 24 PRO instrument tools was developed that validated the total sample population

with the individual with the SCD in the United States. The sample that represented the adult

from the total sampling population was nine. Moreover, the sample population induced for the

LITERATURE REVIEW ABOUT SICKLE CELL ANEMIA 12

children under the age of 17 was from the 12 publication. Sarri, Grammati, et al. (2018) indicated

that most of the instruments were few, and there was no validation data from the study conducted

in the SCD development cohort. Sarri, Grammati, et al. (2018), from the analysis, there was

insufficient information reported in the assessment and the validation of the study. The author

examined in the finding that two of the instrument used in the survey process accessing pain had

good content validity, thus indicating that was better and sufficient reflection that was

constructed to facilitate the measurement of the variable. From the analysis, the SCD represents

the significant issues that most of the patient and the families' faces in the nursing and health care

profession. Sarri, Grammati, et al. (2018) recommended the numerous PRO research that can

facilitate better management of the SCD population. The author suggests that there should be

validation that enhances the generic choices based on the PRO instrument and, too, facilitates

better evaluation. In the investigation, the research should validate the information that induces

the adults' practical psychological assessment and induce the specific instrument that can

validate the young children.

There is still insufficient information in the study, thus creating the gap in the

information required in the PRO instruments that are developed by an individual with the SCD.

Therefore further studies should be designed and validated the PRO instrumental that is effective

for assessing the results and the impact of the SCD on the adult and the children to facilitate the

best caregiving that can reduce and manage the pain that individuals with SCD experience.

Sarri, Grammati, et al. (2018) proposed the additional information that can guide and provide

insight from the SLR that can help provide the information that will be needed in the future

clinical test for the patient with the SCD complication.

LITERATURE REVIEW ABOUT SICKLE CELL ANEMIA 13

Elkins et al. (2013) conducted the randomized that facilitated the adequate controlled trial

that determined the efficacy that induces the treatment of the chronic pain inpatient with cancer

and the chest pain associated with the SCD. Elkins et al. (2013) used the reflection from the

various clinical techniques that surround cognitive hypnotherapy that facilitates the reflective

management of the pain with the SCD. According to the insight of Elkins et al. (2013),

hypnotherapy involves the process that enables the significant hypnotic induction that facilitates

the changes in the perception of the different behavior that induces the effective mechanism that

facilitates better pain management. Hypnotherapy is mostly used to reduce the severe pain

resulting from the post-session from the easy come back to the more comfortable conditions.

Elkins et al. (2013) primarily ensure that it provides the patient with adequate analgesia that

reduces the pain related to stress and anxiety.

The conditions boost the increase of sleep and strengthen the patient's mood, reducing the

need for opioids, especially when the patient is undergoing a painful medical procedure. Elkins

et al. (2013) articulated the need to incorporate both clinical practices and the cogitative behavior

that will facilitate better intervention hence managing the pain of the patient more effectively. In

conclusion, health workers should ensure that they determine the patient's content with the pain

experience to facilitate a better treatment plan. In this case, the psychological factor and social

factor should be effectively assessed in the treatment procedure.

Campbell et al., 2010 conducted a study that its primary purpose was to explore the

different adult experiences of the health services related to the SCD. Campbell et al., 2010

indicated that African –American adolescent experience SDC described various issues associated

with a problematic relationship that that face in most healthcare professionals. From the

observation, the author noted that painful crises were reported to be neglected and

LITERATURE REVIEW ABOUT SICKLE CELL ANEMIA 14

underestimated by the nursing profession when delivering and facilitating the patient's acute care

setting with SCD. Most of the adolescents felt stressed to actively advocate for themselves in

healthcare to gain effective medical care that most of the health officers in the region lack

empathy and the requisite knowledge and skills to deal with a better understanding of the SCD.

The research used the quantitative and qualitative data obtained from the different articles in the

nursing database. From the studies, most Adolescence faces a lot of challenges for them to quire

the medication that is related to SCD. Alternatively, there are still numerous gaps that require to

be closed to understand better the altitude that the Adolescence has towards the SCD

complication and medication access. The strength of the examination integrated the multiple

reviews and the methodology that effectively adopted the better broad perspective that induced

the quantitative, qualitative, and mixed-method to facilitate the prudential that provided the

quality analytical depth synthesis for practical analyses of the assessment the study.

Granados and Jacob (2009) evaluated the pain most of the adolescent experiences when

hospitalized—using the multidimensional pain evaluation tool and the content of the relief they

receive in terms of medication. The examination indicated that most adolescents experiencing a

lot of pain episodes were excluded from the drug. The sample population that was sampled

included eight men and 11 women with SCD conditions. Granados and Jacob (2009) obtained

the research data for around one year. The different clients in the sample population were

interrogated about their pain until they are discharged from the hospital. The patient's pain

condition was described using the numeric rating scale that consisted of 0-10 before and after

analgesic treatment. The patient was also asked to deploy the diagram of their body whenever

they experience the pain condition.

LITERATURE REVIEW ABOUT SICKLE CELL ANEMIA 15

Moreover, all the medication prescribed for the patient was recorded every day until the final day

of discharge. The study applied descriptive statistics that analyzed different variables to explain

the patient's pain location, the magnitude of the pain quality, the intensity of the pain, and the

pain relief's effectiveness after the medication. Granados and Jacob (2009) noted that the

problem was ultimately relieved from the patient in all the analgesics patients.

LITERATURE REVIEW ABOUT SICKLE CELL ANEMIA 16

Reference

Campbell-Hall, V., Petersen, I., Bhana, A., Mjadu, S., Hosegood, V., Flisher, A. J., & MHaPP

Research Programme Consortium. (2010). Collaboration between traditional practitioners

and primary health care staff in South Africa: developing a workable partnership for

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Dampier, C. D., Smith, W. R., Wager, C. G., Kim, H. Y., Bell, M. C., Miller, S. T., ... &

Eckman, J. R. (2013). IMPROVE trial: a randomized controlled trial of patient-controlled

analgesia for sickle cell painful episodes: rationale, design challenges, initial experience,

and recommendations for future studies. Clinical Trials, 10(2), 319-331.

Elkins, G. R., Fisher, W. I., Johnson, A. K., Carpenter, J. S., & Keith, T. Z. (2013). Clinical

hypnosis in the treatment of post-menopausal hot flashes: a randomized controlled trial.

Menopause (New York, NY), 20(3).

Haywood Jr, C., Tanabe, P., Naik, R., Beach, M. C., & Lanzkron, S. (2013). The impact of race

and disease on sickle cell patient wait times in the emergency department. The American

journal of emergency medicine, 31(4), 651-656.

Nguyen, M., Crimlisk, J. T., Johnstone, D. J., Sanchez, G. M., Granados, R., Jacob, E., ... &

Kuchinski, A. M. (2009). 147 Nurse’s Asssessment of Caregiver Burden. Medsurg

Nursing, 18(3), 139.

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Sarri, G., Bhor, M., Abogunrin, S., Farmer, C., Nandal, S., Halloway, R., & Revicki, D. A.

(2018). Systematic literature review and assessment of patient-reported outcome

instruments in sickle cell disease. Health and quality of life outcomes, 16(1), 99.

Swanson, M. E., Grosse, S. D., & Kulkarni, R. (2011). Disability among individuals with sickle

cell disease: literature review from a public health perspective. American journal of

preventive Medicine, 41(6), S390-S397.

Wang, C. J., Kavanagh, P. L., Little, A. A., Holliman, J. B., & Sprinz, P. G. (2011). Quality-of-

care indicators for children with sickle cell disease. Pediatrics, 128(3), 484-493.

Wilson, B. H., & Nelson, J. (2015). Sickle cell disease pain management in adolescents: a li

terature review. Pain Management Nursing, 16(2), 146-151.

Wilson, B. H., & Nelson, J. (2015). Sickle cell disease pain management in adolescents: a

literature review. Pain Management Nursing, 16(2), 146-151.

World Health Organization. (2018). WHO meeting on ethical, legal, human rights and social

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