Prevention Of Sickle Cell Disease Research Articles

Sickle cell disease awareness and perception among Christian religious leaders in Accra Metropolis: a qualitative study.

Sickle Cell Disease (SCD) is a severe hemoglobin gene mutation disorder inherited from both parents. 2% of Ghanaian newborns are affected by SCD; one in three Ghanaians has the hemoglobin S gene. Christian religious leaders may play a role in the prevention of SCD through the promotion of genetic counseling, genotype screening for premarital couples, and offering counseling to couples on prenatal screening and diagnosis for SCD. However, little is known about the awareness and perception of SCD among Christian religious leaders in Ghana, and this study aims to explore these. This study adopted a qualitative descriptive design to explore the awareness and perception of SCD among Christian religious leaders in the capital city of Ghana. A purposive sampling technique selected 16 participants from churches under the main Christian groups. The participants were chosen based on their roles and responsibilities within their respective churches. Data was collected using a semi-structured interview guide, which included open-ended questions to encourage participants to share their thoughts and experiences. The interviews were conducted in a private setting to ensure confidentiality. The data was then analyzed using a thematic analysis approach, which involved identifying recurring themes and patterns in the participants' responses. The study's findings are crucial. They reveal a high awareness of SCD among Christian religious leaders, but also some misconceptions. Most of the religious leaders knew SCD was a genetic disease, although a few associated SCD with superstitious beliefs, poor dietary intake, and lifestyle. Some also stated that SCD was a disease of the blood group instead of the defective haemoglobin gene. They perceived SCD to be burdensome, disruptive, and draining, and they associated the disease with burnout in Persons Living with SCD (PLWSCD) and their families. The religious leaders had a good social network with PLWSCD, including family, friends, colleagues, and congregants. These findings underscore the need for intense education about SCD, especially among Christian religious leaders. It is crucial to engage all stakeholders to intensify public awareness and education about SCD while improving the management and social support systems available to PLWSCD and families. This includes the religious institution's leadership, PLWSCD and families, the Ministry of Health, Ghana Health Service, and the Ghana Education Service. As active stakeholders, religious leaders can play a vital role in supporting PLWSCD if they are equipped with the necessary knowledge about the condition. .

Awareness of Sickle Cell Disease in the Eastern Region of Saudi Arabia: Study of Medical Specialty Students

Introduction: Sickle cell disease (SCD) is a major health challenge in Saudi Arabia due to its high prevalence, and healthcare providers play a key role in reducing the prevalence of this genetic disorder.Objective: To assess awareness and knowledge of SCD among medical specialty students in various medical programs and to explore its differences based on the sociodemographics of participants.Methods: The study included a cross-sectional survey of 302 medical students across different years of study at Medical College in the eastern region of Saudi Arabia. Participants completed a self-administered questionnaire developed based on a literature review. Descriptive analysis using SPSS V 26 and inferential analyses were utilized.Results: While 61.6% of participants felt they had sufficient SCD information, only 45% had previously received SCD education or awareness training. Only 6.6% reported experiencing college problems due to SCD. Most participants demonstrated good knowledge of SCD's cause, transmission, prevention, and diagnosis. However, fewer were aware of risk factors for children of carriers (6.6%), the disease's prevalence in Saudi Arabia (45%), or the possibility of curing SCD through stem cell transplant (49.7%). The study found married respondents were more aware of SCD than single or divorced students. Conclusion: While medical students grasp SCD basics, more targeted educational initiatives are needed, especially around risk factors and prevalence, to enhance awareness and control the high SCD rate in Saudi Arabia, particularly in the eastern region where it is most common. Expanding SCD education programs in medical institutions could improve future providers' knowledge

Beliefs negatively mediate adolescents’ and adults’ knowledge about sickle cell disease prevention and relationship choices; a one-center exploratory study in Central Region, Ghana

BackgroundSickle cell disease (SCD) is a common monogenic inheritable condition in sub-Saharan Africa. 30% of Ghanaians are estimated to be carriers creating a condition for stable SCD penetrance. Being inheritable, SCD is scientifically preventable through intentional spousal selection. This study therefore explored young adults’ knowledge, beliefs and prevention strategies regarding SCD.MethodsThis exploratory study employed a mixed-methods approach (semi-structured questionnaires and focus group discussion [FGD]) to explore the SCD knowledge and beliefs of participants (15–49 years). The data collection was intentionally sequential; initial administration of 386 questionnaires and then followed by the FGD (16 participants). FGD was thematically analysed whereas quantitative data was explored using structural equation modeling (SEM); p < 0.05 was considered statistically significant under two-tailed assumptions.ResultsWhereas 98.7% reported having heard about SCD, < 50% got the information through formal education. Overall, 49.7% knew their respective SCD status; the proportion increased with age (48.8% in < 30 years vs 64.4% in ≥ 30 years old), or higher degree status (48.8% in undergraduates vs 67.4% in postgraduates). Moreover, whereas nine-in-ten correctly identified that SCD is hereditable, three-in-ten believed that having a SCD child was a matter of fate. Our FGD revealed that whereas curses, and spiritual attack were misconceived as potential causes of SCD, stigmatization associated with SCD was a major concern. The SEM demonstrated that one’s knowledge about SCD prevention is significantly positively associated with relationship choices (b = 0.757, p < 0.05). Also, a participant’s knowledge about SCD preventive strategies was significantly associated with the individual’s beliefs about SCD (b = 0.335; p < 0.05). However, a participant’s SCD beliefs negatively mediated SCD preventive strategies-relationship choices association.ConclusionsSCD beliefs likely transform linear quantitative associations into a complex non-linear interaction; public health campaigns ought to unearth and address SCD beliefs to maximize achieving the intended targets.

Understanding Knowledge, Attitude, and Practices towards Sickle Cell Disease among Adult Outpatients: Insights from Lira Regional Referral Hospital, Northern Uganda

This study investigates the knowledge, attitude, and practices (KAP) regarding sickle cell disease (SCD) among adult outpatients at Lira Regional Referral Hospital (LRRH) in Northern Uganda. Employing a descriptive cross-sectional qualitative approach, data were collected from 100 adult patients aged 18 years and above attending the general outpatient department (OPD) at LRRH. Results revealed a substantial level of awareness of SCD among respondents, predominantly sourced from healthcare facilities and media outlets. While many recognized SCD as hereditary, misconceptions regarding its etiology and symptoms were evident. Notably, there was a knowledge gap regarding the treatability of SCD. Attitudes towards marrying individuals with SCD were predominantly negative, reflecting concerns about genetic transmission. However, there was a positive inclination towards premarital SCD testing. Regarding practices, reliance on medical facilities for SCD treatment was predominant, with minimal engagement with alternative therapies. Recommendations include sustained awareness campaigns, integration of SCD prevention and control initiatives, and further research to elucidate regional SCD prevalence determinants. Efforts to enhance male involvement in SCD advocacy and capacity building among healthcare providers are advocated to optimize SCD management and prevention strategies within the community. Keywords: Knowledge, Attitude, Practices, Sickle Cell Disease, Outpatients

Withdrawal: "Primary prevention of sickle cell disease using preimplantation genetic testing and in vitro fertilization is cost-effective." Christina N. Cordeiro Mitchell, Apoorva Pradhan, Bhuchitra Singh, Rakhi P. Naik, Valerie L. Baker, Sophie M. Lanzkron, Mindy S. Christianson, Lydia H. Pecker.

Christina N. Cordeiro Mitchell (2021) Primary prevention of sickle cell disease using preimplantation genetic testing and in vitro fertilization is cost-effective (https://doi.org/10.1002/ajh.25974). The above article, published online on 20 August 2020 in Wiley Online Library (wileyonlinelibrary.com) as an Accepted Article, has been withdrawn by agreement between the authors, the journal Editor in Chief, and John Wiley & Sons Ltd. The article has been withdrawn after the accepted article was posted online, the authors received independent feedback that has prompted them to review their analysis in the article to ensure that the original conclusions are accurate.

Genetic counseling in sickle cell disease: Insights from the Indian tribal population.

Sickle cell disease (SCD) is an inherited disorder of hemoglobin. With an overall prevalence of 4.3%, India is the second-largest hub of SCD after Africa. Genetic counseling (GC) is the most cost-effective intervention to reduce the burden of a genetic disease including SCD. Even though GC's role in reducing SCD birth prevalence is well established, it is still not incorporated into Indian national policy and is unavailable to most Indians approaching their marriageable age and childbirth. GC perception and efficacy have alsonot been explored yet among young adults, especially in Indian tribal communities. Counseling in these communities requires careful consideration of their socioeconomic, cultural, and ethical values. Community engagement with local tribes and healthcare infrastructure in a multitier approach is essential for an effective GC. This review aims to provide healthcare providers and genetic counselors with the essentials of GC in the prevention and management of SCD among tribal communities based on the author's counseling experience in South India.

Prenatal diagnosis and preimplantation genetic diagnosis for sickle cell disease in Africa

Sickle cell disease (SCD) is the most common genetic haematological disorder worldwide, and it is a major public health concern, especially in Sub-Saharan Africa. Prenatal diagnosis (PD) and preimplantation genetic diagnosis (PGD) are important reproductive options for the prevention of SCD. Despite the high prevalence of SCD in Nigeria and Sub-Saharan Africa, current trends in PD and PGD for the prevention of SCD are still slow compared to that in developed countries. Attitudes towards PD and PGD for the prevention of SCD in African are influenced by level of awareness, knowledge and educational status, and the main barriers to the uptake of PD and PGD for SCD in Africa are cost, religion, sociocultural, ethical and moral considerations. We reviewed available data on PD and PGD for SCD in Africa, using the PubMed, PubMed Central, Google Scholar and African Index Medicus search engines, through a combination of words and phrases relevant to the subject. This article reviewed the current trends in PD and PGD for the prevention of SCD and discussed the attitudes towards and the barriers to the uptake of PD and PGD for SCD in Africa.

Contribution of malaria and sickle cell disease to anaemia among children aged 6–59 months in Nigeria: a cross-sectional study using data from the 2018 Demographic and Health Survey

ObjectivesTo estimate the fraction of anaemia attributable to malaria and sickle cell disease (SCD) among children aged 6–59 months in Nigeria.DesignCross-sectional analysis of data from Nigeria’s 2018 Demographic and Health...

Sickle cell disease prevention: How prepared are the senior secondary school students in Surulere Local Government Area, Lagos, Nigeria?

BackgroundSickle cell disease (SCD), a common hereditary disease, can be prevented by preparing young people ahead of the conception of an affected foetus.AimTo assess the knowledge and attitude regarding SCD amongst senior secondary school students in Surulere Local Government Area (LGA), Lagos, Nigeria.SettingSenior secondary schools in Surulere LGA.MethodsThis was a descriptive cross-sectional study amongst 300 senior secondary school students. Data were collected using a self-administered questionnaire and analysed using Stata16. The Chi-square and Fisher’s exact tests were used to determine the association between categorical variables. The level of significance was predetermined at p < 0.05.ResultsThe mean age of the respondents was 15.2 (±1.3) years, with a male-to-female ratio of about 1:2. The majority (90.0%) of the respondents were aware of SCD, 63.0% had good knowledge, although less than half of them (46.3%) knew SCD to be a blood disorder, whilst about two-thirds (53.1%) knew that it was an inherited condition. About one fifth (24.4%) of them knew about prevention by genetic counselling. The majority (97.0%) of them had a positive attitude towards SCD. Over two-thirds (72.6%) were aware of their genotype. The prevalence of SCD was 2.0%, whilst 18.9% of them were carriers of the sickle cell trait. Knowing their SCD status but not necessarily their genotype was significantly associated with their attitude towards the disease (p = 0.014).ConclusionThe prevention of SCD was not known to the majority, and better attitudes were more likely when the SCD status was known. Therefore, routine screening and counselling could potentially aid SCD control.

Overview of current progress and challenges in diagnosis, and management of pediatric sickle cell disease in Democratic Republic of the Congo

ABSTRACT Objectives Sickle cell disease (SCD) encompasses health complications, primarily affecting the hematologic system and leading to high death rates in childhood. As a rule, the World Health Organisation (WHO) stepwise gold-standard about the strategies for prevention, diagnosis, and treatment of SCD must be multidimensional. This overview aimed to highlight current advances and challenges linked to strategic issues, diagnosis, the prevalence, and treatment of pediatric cases in Sub-Saharan Africa, particularly the Democratic Republic of the Congo. Methods We searched data on Google Scholar, Medline, PubMed, Science Direct, Scopus, and ResearchGate. Results The laboratory diagnosis of SCD has progressed from conventional electrophoresis to rapid point-of-care tests that allows early neonate screening. HemoTypeSCTM is an affordable test for neonatal screening in DRC. The pediatric SCD prevalence in Sub-Saharan Africa lay within 1–7.7% of homozygous(SS) and 15–40% of the heterozygous(AS) forms of SCD, depending on the method used and the ethnic population tested. Various supportive management protocols for comorbidities and complications exist, but they are not standardized in the Region. Conclusion Notwithstanding some progress accomplished, the disease is still challenging in Sub-Saharan Africa due to limited early diagnostic testing and a lack of specific medications. There is a need for harmonizing therapeutic protocols and conducting controlled valid clinical trials.

Safety and Tolerability of PF-04447943 across a Clinical Trial Program Including 277 Patients

▪Background: Current treatment options (eg, hydroxyurea, chronic blood infusions, and bone marrow or stem cell transplantation) for the prevention of sickle cell disease (SCD) complications vary in effectiveness and are associated with safety risks/tolerability issues. PF-04447943, a selective inhibitor of the cyclic guanosine monophosphate−specific phosphodiesterase-9A enzyme, is being developed for prophylactic therapy to reduce the incidence and/or severity of vaso-occlusive crises in patients with SCD. Herein, we report on the safety and tolerability of PF-04447943, based on a large data set from the clinical trial program.Methods: The PF-04447943 safety database includes data from 8 phase 1 studies in healthy adult and elderly subjects, a phase 2 study in patients with Alzheimer's disease (AD) from a previous development program, and a phase 1b study in stable SCD patients. Study durations ranged from 1 day to 3 months. Data are presented descriptively.Results: Safety data for 277 patients receiving PF-04447943 were available. Frequently reported treatment-emergent adverse events (TEAEs) were headache, diarrhea, and nausea, which were generally mild in severity. In the phase 1 thorough QTc (TQT) study (N=44; study duration, 28 days), at therapeutic and supratherapeutic doses, PF-04447943 was not associated with QTc interval prolongation that met the threshold of clinical concern, based on ICH E14 criteria. In the phase 2 study of the AD development program (PF-04447943, n=91; placebo, n=100; study duration 12 weeks), the incidence of TEAEs was 63.7% with PF-04447943 and 58.0% with placebo. Incidence of all-causality AEs in the gastrointestinal disorders class (including abdominal pain, diarrhea, dry mouth, dyspepsia, gastroesophageal reflux disease, intestinal obstruction, nausea, and upper gastrointestinal hemorrhage) was higher with PF-04447943 (19.8%) vs placebo (5.0%); no other notable differences were observed. In the phase 1b study in SCD patients (safety analysis set: PF-04447943, n=22; placebo, n=7; study duration, 29 days plus 28 days follow-up), nervous system disorder TEAEs (eg, headache) occurred more frequently with PF-04447943 (25 mg BID, 60.0%; 5 mg BID, 57.1%) vs placebo (28.69%). In total, 6 serious AEs (sickle cell anemia with crisis, n=4 [one patient had 3 crises, of which 2 occurred during the follow-up period]; pneumonia, n=1; biliary colic, n=1) occurred in 3 patients receiving PF-04447943; all were consistent with the course of SCD and were not considered to be treatment related.Conclusion: Review of the safety database for PF-04447943, which includes 277 patients who received the study drug, indicates that PF-04447943 is generally well tolerated, with no major safety signals. Planned long-term, phase 2 and 3 studies of PF-04447943 in patients with SCD will provide additional safety data in this population. DisclosuresCharnigo:Pfizer: Employment. Tan:Pfizer Inc: Employment. Rybin:Pfizer: Employment. Pittman:Pfizer: Employment. Sivamurthy:Pfizer Inc: Employment. Beidler:Pfizer: Employment. Clarke:Pfizer Inc: Employment.

Knowledge, Attitude, And Practice of Genotype Screening among Junior and Senior Secondary School Students in Gwagwalada Area Council, FCT Abuja

Background: The knowledge, attitude and practice of genotype screening is very important in the control and prevention of sickle cell disease (SCD). Methods of preventing this disease include preschool and premarital screening, genetic counseling, prenatal diagnosis, preconception diagnosis and implantation of normal embryos after in vitro fertilization and in vitro therapy using stem cell transplantation. The objective of this study is to determine the knowledge, attitude and the practice of genotype screening among junior and senior secondary school students in Gwagwalada Area Council, FCT, Abuja. Methodology: This was a cross-sectional descriptive study conducted among students aged 11-23 years in junior and senior secondary schools in Gwagwalada. A sample size of 264 was studied. A multi-stage sampling technique was used to select the schools, classes and participants. Data was collected by means of self-administered and semi-structured questionnaire. Demographic variables and associations were tested using Chi-square test with the level of statistical significance set at p <0.05, at 95% confidence interval. Results: This study showed that the students had good knowledge of SCD as 81.5% and 88.0% of the Junior Secondary School (JSS) and Senior Secondary School (SSS) students respectively knew the causes of SCD, 89.5% and 92.7% of the JSS and SSS students knew it could be transmitted from parents to offspring, and 83.3% and 88.0% of the JSS and SSS students knew that marriage of two AS partners could result in a sickle cell child. Also. this study revealed that the students had positive attitudes to SCD as 68.5% and 72.6% of the JSS and SSS students agreed that pre-school practice could prevent SCD while 79.8% and 80.7% of the JSS and SSS students also agreed pre-marital screening could prevent SCD. In addition, 50.9% and 48% of the JSS and SSS students agreed that newborn screening for SCD should be encouraged. The practice of genotype screening was however found to be poor as much as 45.7% and 24.6% of the JSS and SSS students respectively do not know their genotypes, p = 0.001. Conclusion: The students generally have good knowledge and positive attitudes towards genotype screening; however, the practice of genotype screening was poor. The school authorities should therefore ensure pre-school genotype screening and teaching on SCD should be incorporated early into the school curriculum to reduce the burden of the disease.

The Crisis of Sickle Cell Disease in Africa from Insights into Primary Prevention in Ghana and Nigeria: Notes from the Field.

Sickle cell disease (SCD), an inherited blood disorder, impacts 2% of newborns in Nigeria and Ghana. Despite devastating health consequences, SCD prevention is not a priority in either country. This article describes our U.S. research team's feasibility assessment for adapting CHOICES, a computer-based SCD education program, for use in Ghana and Nigeria. We identified indigenous collaborators by reviewing published research and investigating advocacy organizations online. This led to a fact-finding trip to Africa to discuss SCD prevention with local boards of advisors. Three major recommendations emerged from the group discussions: design a culturally appropriate intervention; enlist community healthcare workers to deliver the CHOICES program; and collaborate with religious and community leaders and elders in public awareness campaigns. Based on extensive advisor input, we will modify the content and delivery of the CHOICES intervention to meet the needs of those impacted by SCD in Ghana and Nigeria.

Knowledge, Attitude and Practice of Genotype Screening Among Undergraduate Students of the University of Abuja, Nigeria

The knowledge, attitude and practice of genotype screening for sickle cell are very important in the control and prevention of sickle cell disease (SCD). The objectives of this study therefore are to determine the knowledge, attitude and practice of genotype screening among undergraduate students of the University of Abuja, Nigeria. The study was a descriptive cross-sectional study. A sample size of 256 was studied and multi-stage sampling technique was used to select the faculties, departments and participants. Data was collected by using self-administered, semi-structured questionnaire. Associations between variables and proportions were ascertained using Chi-square tests at 5% level of statistical significance. Most of the respondents were males (51.1%) and majority belong to age group of 21-25 years. About 98% of the students have good knowledge of genotype screening for sickle cell. Also, 93.8% of the students had positive attitude to genotype screening and 76.6% of the participants had ever had a genotype screening with 52.7% having AA genotype, 18.4% AS and 5.5% with SS genotype. In addition, the study revealed a statistically significant association between good knowledge of genotype screening and the practice (98.0% vs. 58.2%; p value <0.003). Since the students have good knowledge and positive attitude to genotype screening, increased awareness on genotype screening should be encouraged so as to increase the uptake of screening and reduce the burden of sickle cell disease.

Cumulative outcome of pre-implantation genetic diagnosis for sickle cell disease: a 5-year review.

To review the cumulative outcome of pre-implantation genetic diagnosis (PGD) cycles performed for prevention of sickle cell disease (SCD). Couples referred for PGD for SCD between April 2012 and October 2017 were included. Ovarian stimulation was performed using a short gonadotrophin-releasing hormone (GnRH) antagonist protocol and follicle-stimulating hormone injections. The GnRH agonist was used to trigger oocyte maturation. Oocytes were fertilised using intracytoplasmic sperm injection. Trophectoderm biopsy was performed on day 5 or 6 followed by vitrification. Genetic testing was done using pre-implantation genetic haplotyping. A total of 60 couples started 70 fresh PGD cycles (mean 1·2cycles/couple) and underwent a total of 74 frozen-embryo-transfer (FET) cycles (mean 1·3FET/couple). The mean (SD) female age was 33(4·4)years and the mean (SD) anti-müllerian hormone level was 22·9(2·8)pmol/l. The cumulative live-birth rate was 54%/PGD cycle started and 63%/couple embarking on PGD. The rate of multiple births was 8%. The cumulative outcome of PGD treatment for prevention of SCD transmission is high and PGD treatment should be offered to all at-risk couples.

Distribution of haemoglobin genotypes, knowledge, attitude and practices towards sickle cell disease among unmarried youths in the Buea Health District, Cameroon.

Introductionsickle cell disease (SCD) is one of the commonest genetic causes of morbidity and mortality in the world. In resource-limited settings, SCD prevention through public education and screening could be a significant strategy to curb its prevalence. The study aimed at determining the distribution of haemoglobin genotypes among unmarried youths in Buea, Cameroon as well as their knowledge, attitude and practices towards SCD.Methodsa community-based, analytic, cross-sectional study was conducted within the city of Buea. Data was collected from 410 youths using self-administered questionnaires. Of the 410 youths, 100 were selected by purposive random sampling and their haemoglobin genotyping was done using haemoglobin electrophoresis. The data was analysed using the statistical software Epi Info Version 7.Resultsthe majority (51.5%) of the 410 respondents were females. The modal age range was 18- 21 years (46.8%) and 60.4% had tertiary education. Less than one quarter (20.5%) had good knowledge of SCD. Only 13.2% knew their genotype and 59.3% were willing to avoid carrier marriages. Out of the 100 participants for genotyping, 84.0% had normal haemoglobin (HbAA) and 16.0% had the sickle cell trait (HbAS).Conclusionmost of the respondents had moderate knowledge of SCD. Only a few knew their haemoglobin genotype and more than half were willing to avoid carrier marriages. The prevalence of sickle cell trait is high in Buea. The promotion of preventive methods like public education and genetic screening is recommended to reduce the burden of SCD in Cameroon.

Improving the Capacity of Health System and Community for Sickle Cell Disease Screening and Management Among Tribal Population in India: Protocol of an Intervention Study.

Sickle cell disease (SCD) is one of the major public health problems in the world. In India, the burden of SCD is comparatively high in socio-economically disadvantaged tribal communities. Though efficacious interventions are available to manage SCD, they are not reaching to these communities and no comprehensive programme is in place in the health care system. Therefore, the Indian Council of Medical Research has initiated a nation-wide study to develop an effective intervention model for SCD patients in tribal areas through the government health care system. This intervention includes increasing awareness and preparing the communities for accessing the government health care system for SCD care, and improving the capacity of the primary health care systems including the training of the health care providers on prevention and management of SCD. The study adopted a quasi-experimental design with pre-vs. post-intervention comparisons of outcome variables within the interventional groups and with the control group. The study will be implemented in 6 districts which are endemic for SCD, spread across different geographical zones of India. In each district, four primary health centre (PHC) areas which are predominantly inhabited by tribal population will be selected. Of these four PHC areas, two will be selected randomly for implementing the intervention and the remaining two will be the control area. Information necessary for development and implementation of the intervention will be gathered during formative research, by using both quantitative and qualitative research methods. Intervention with an inclusive partnership and community mobilization will be implemented. The major steps in the implementation of intervention are partnership building with various health and non-health partners including the community. Capacity building and strengthening is another important component to enable the primary health facilities to screen and manage SCD patients. Primarily, sub-health centres and primary healthcare centres will be equipped with appropriate SCD screening techniques. All doctors in the system will be trained in advanced treatment and management issues. To improve the community’s awareness and readiness, community mobilization activities will be conducted. An impact evaluation will be carried out at the end of the intervention by comparing the improvement of SCD management in intervention PHCs to that of the control PHCs. However, the process evaluation and necessary mid-term corrections will be made throughout the intervention period. Thus, an intervention model in terms of its suitability, replicability and sustainability for the tribal population will be developed and tested. The findings of this study are more suitable to use during advocacy and to replicate the model by the state health departments. This study develops and places an appropriate referral system for SCD patients at the PHC level. Improving the community’s access to health care, improving the quality of care in government health centres and raising awareness among tribal communities are crucial to achieving through innovation. Taken together, these innovations would significantly contribute to better access to health care and management of the SCD patients of underserved tribal population.

Rate of Sickle Hemoglobin Recovery in Sickle Cell Disease Patients Undergoing Red Blood Cell (RBC) Exchange Transfusion Is Associated with Age of Patients and Number of RBC Units Transfused

Rate of Sickle Hemoglobin Recovery in Sickle Cell Disease Patients Undergoing Red Blood Cell (RBC) Exchange Transfusion is Associated with Age of Patients and Number of RBC Units Transfused Introduction: Automated and manual red blood cell exchange (RBCX) transfusions are useful in the primary and secondary prevention of sickle cell disease (SCD) complications (Ware et al., 2012). The ability to consistently maintain sickle hemoglobin (HbS) below target (30% or 50% depending on indication) is quite variable (Kuo et al., 2012). With emerging indications such as silent cerebral infarction, it is imperative that effective means of chronic transfusion to maintain appropriate hematological and clinical targets be identified. We hypothesize the rate of HbS recovery is dependent on the individual's hemolytic and erythropoietic rate. The purpose of this study is to evaluate the effect of the rate of erythropoiesis and hemolysis on HbS recovery in SCD patients undergoing RBCX. Methods: Fifteen (15) patients were prospectively recruited from the adult SCD transfusion program (9 automated, 6 partial manual), from December 2018 to July 2019, and followed through one exchange cycle (4 weeks). Automated and partial manual exchange transfusion protocols have been previously described elsewhere (Canadian Haemoglobinopathy Association Consensus Statement on the Care of Patients with Sickle Cell Disease in Canada, Version 2.0, Ottawa; 2015). Exclusion criteria included active hydroxyurea or erythropoietic stimulating agents use, reported ill health in the preceding 4 weeks, co-morbid hemolytic condition or non-HbSS genotype. Hemoglobin, hematocrit, HbS, lactate dehydrogenase (LDH), reticulocyte count, indirect bilirubin, and serum erythropoietin level were determined for each patient: pre- and post- first exchange, weekly for 3 weeks and pre- second exchange (the 4th week). Descriptive variables were either expressed as means ± SD or median (IQR), based on normality, while linear regression was performed for continuous variables. Co-variates were included in multivariable analysis if P &lt; 0.10. Multivariable linear regression was conducted to examine the potential association between the change in HbS over one RBCX cycle and age of patients, pre-RBCX hematocrit, LDH, and number of RBC units transfused. Results: We identified 36 eligible patients from the Program database, after which 15 consented to participate in the study. Mean age was 32.9 ± 12.3 years, consisting of 7 males and 8 females. There was an association between the rate of change in HbS and age of patients (p=0.035), pre-RBCX hematocrit (p=0.030) and number of transfused RBC units (p=0.030). LDH showed a trend towards reduced rate of change in HbS (p=0.069). Rate of change in HbS was not associated with automated vs. partial RBCX (Figure), female vs. male patients, pre-RBCX HbS, erythropoietin, indirect bilirubin, reticulocyte and age of transfused RBCs. Age of patients (p&lt;0.001) and number of units transfused (p=0.010) were independently associated with the rate of change of HbS, after adjusting for hematocrit and LDH. For every decade increase in age, the rate of HbS recovery was 3% lower in one RBCX cycle. For each additional unit of RBC exchanged, the rate of HbS recovery was 0.78% higher in one RBCX cycle. Conclusion: This is the first study to evaluate the determinants of variability in the rate of HbS recovery in SCD patients on RBCX. Age of patients and number of RBC units transfused may underlie the significant variability in achieving HbS targets in SCD patients on RBCX. We failed to show a significant difference in the rate of change in HbS between automated and partial manual exchange transfusion. Reduction in HbS recovery with advancing age may be related to erythropoietic reserve in the patients' marrow. However, the influence of units of RBC recovery appeared paradoxical, and the finding may be spurious due to small sample size. It may therefore be possible to appropriately titrate the number of RBC units with advancing age of SCD patients with a view to achieving desired HbS targets. Disclosures Patriquin: Ra Pharma: Consultancy, Research Funding; Apellis: Consultancy, Honoraria, Research Funding; Alexion: Consultancy, Honoraria, Research Funding; Octapharma: Consultancy, Honoraria, Research Funding. Kuo:Agios: Consultancy; Alexion: Consultancy, Honoraria; Apellis: Consultancy; Bioverativ: Other: Data Safety Monitoring Board; Bluebird Bio: Consultancy; Celgene: Consultancy; Novartis: Consultancy, Honoraria; Pfizer: Consultancy.

Knowledge Deficit of Sickle Cell Trait Status: Can Nurses Help?

Sickle cell disease and sickle cell trait are identified through universal newborn screening, which has been implemented in all hospitals across the United States since the 1970s. Yet, studies report that only 16% of Americans with sickle cell trait know their status. Despite these striking statistics, there appears to be no standardized methods for reporting positive sickle cell trait results of newborn screening to doctors or families of affected persons. This article will demonstrate how current literature supports the gaps in knowledge of trait status and its implications as well as knowledge deficit of inheritance patterns of prospective parents. The article will also elucidate how knowledge deficit in this subject adversely affects primary prevention strategies including genetic counseling. Primary prevention of sickle cell disease is a public health area that can be championed by registered nurses who have the tools and experiences to effectively handle case management and patient education.

Beyond the Legislation for Sickle Cell Disease Prevention - Getting the Right Outcomes

This study aimed at highlighting benefits derivable from legislation for Sickle cell disease (SCD) prevention using the three levels of health legislative power (primary, secondary and tertiary). It was an evidence-based integrative review of 54 references related to SCD, epidemiology, management, and legislation for its control over the past three decades (1979-2018). Two keywords (SCD and Legislation/Policy) were used as search strategy to identify the problem, intervention, and outcome evaluation of the research questions. MeSH, Medline, CINAHL, PubMed, Cochrane Database Systematic Review, Google Scholar and African Journal Online were search databases reviewed. The expected outcomes were categorized into three levels of benefits. They were primary, secondary and tertiary categories of benefit. While the primary category would institutionalize pre-genetic counseling in the health institutions, the secondary and tertiary groups would establish screening (prenatal and newborn)/comprehensive care and curative interventions (i.e., investigational therapies and stem cell transplantation) respectively for people living with SCD. These interventions will enhance SCD surveillance, screening, auditing, health needs assessment, treatment, control, and prevention in the target audience. Conclusions The legislation for Sickle Cell Disease prevention in the target population would promote early disease detection, treatment, and prevention. We expect that with an increase in case ascertainment, the disability-adjusted life year (DALY) would reduce so also other complications. This legislation will diffuse new innovations in SCD management and research which will ultimately improve the health indices of people living with the disease globally.