Knowledge Of Sickle Cell Disease Research Articles

Evaluating the Impact of Film on The Knowledge of Sickle Cell Disease amongst Residents of Obio-Akpor Local Government Area

Evaluating the Impact of Film on The Knowledge of Sickle Cell Disease amongst Residents of Obio-Akpor Local Government Area

Health education to promote knowledge about sickle cell disease and newborn screening in pregnant women: a community-based pilot study using the healthy beginning initiative

BackgroundPregnancy presents a critical period for any maternal and child health intervention that may impact the health of the newborn. With low antenatal care attendance by pregnant women in health facilities in Nigeria, community-based programs could enable increased reach for health education about sickle cell disease (SCD) and newborn screening (NBS) among pregnant women. This pilot study aimed to assess the effect of education on the knowledge about SCD and NBS among pregnant women using the Healthy Beginning Initiative, a community-based framework.MethodsA pre-post study design was used to evaluate knowledge of SCD and NBS in a convenience sample of 89 consenting pregnant women from three communities. Participants were given surveys prior to and following completion of a health education session. McNemar’s test was used to compare the proportion of participants with correct responses. The level of significance was taken as p < 0.05.ResultsCompared to pre-test values, post-test values showed that participants understood that SCD is hereditary (93.3% vs. 69.7%), both parents must have at least one gene for someone to have SCD (98.9% vs. 77.5) and blood test is the right way to know if one has SCD (98.8% vs. 78.7%). Also, a large proportion of participants (post-test ~ 89.9%; compared to pre-test ~ 23.6%) understood that the chance of conceiving a child with SCD was 25% for a couple with the sickle cell trait (SCT). Knowledge of the possibility of diagnosing SCD shortly after birth was highly increased in the post test phase of the study when compared to the pre-test phase (93.3% vs. 43.9%, respectively). Concerning the overall knowledge scores, those with high level of knowledge significantly increase from 12.6% pretest to 87.4% posttest (p = 0.015).ConclusionThe health education intervention was associated with significant improvement on almost all measures of SCD knowledge. Focused health education for pregnant women using community structures can improve knowledge of SCD and NBS.

Knowledge and practices of sickle cell disease among healthcare providers in Kinshasa, Democratic Republic of the Congo.

In Kinshasa, Democratic Republic of Congo, there is a low evocation of the diagnosis of sickle cell disease (SCD) by first-level healthcare providers (HCPs), most likely because of poor knowledge of the disease. To assess the levels of knowledge and practices of SCD and to identify determinants of the practices among primary HCPs. Healthcare facilities in Selembao health zone in Kinshasa, Democratic Republic of the Congo. A cross-sectional study of HCPs randomly selected through a two-stage sampling design. Data were collected using a pre-tested interviewer-administered questionnaire. Univariate and bivariate analysis were done to describe the levels of knowledge and practices of SCD. Factors associated with better practices on SCD were determined using multiple linear regression. The threshold for statistical significance wasp˂ 0.05. A total of 318 HCPs, which included 80 physicians and 238 nurses, participated in the study. The participants showed different scores on the components of the knowledge. All the participants showed poor practices on SCD. Multiple linear regression retained overall knowledge of SCD as a significant predictor of better practice for physicians. Knowledge of SCD and duration of work experience were significant predictors of better practices among nurses. The practices of healthcare providers on SCD were far from optimal. These practices were significantly associated with knowledge and experience of healthcare providers.Contribution:This study highlighted the need for continuing professional education to enhance the management of SCD in the setting.

Knowledge, Awareness and Opinion Related to Sickle Cell Disease Among the Professional College Students of Amravati, Maharashtra

The aim of this study is to investigate the awareness, clinical manifestations, treatment and populations at risk of Sickle Cell Disease (SCD) amongst the professional college students of Amravati district. This study was also focus on knowledge about carriers (HbAA) and sufferers (HbSS). The total 842 students from technical streams such as engineering, pharmacy and biotech were enrolled. All participants were assessed on their general knowledge of the disease. The structured questionnaire containing the questions on origin, prevalence, incidences, life expectancy, painful crisis, counseling methods, treatments, management, attitude and behavior towards SCD were provided to all participants. The results had shown that, only 144(17.1%) subjects were found to be known about SCD. Out of them, 56(38.8%) individuals had appreciable knowledge, 80(55.5%) had limited knowledge and 8(5.5%) had marginal knowledge of SCD. The 30.5%(n=44) respondents were known about need of blood transfusion and 18.75%(n=27) participants had knowledge of interrelation between sickle cell anemia and the disease jaundice be an important clinical complaint in SCD. 25.69% (n=37) respondents known to have about intimate relationship between sickle cell disease and pain crisis. Majority of respondents (512/842) was believed that, people should have knowledge and awareness about the genetic diseases that helps to restrict its further spread. Keywords: Sickle cell disease, Professional college, Amravati, Maharashtra

Knowledge of Sickle Cell Disease Among Medical Students at Al-Baha University, Saudi Arabia: A Cross-Sectional Study.

Sickle cell disease (SCD) presents a major health challenge in Saudi Arabia due to its high prevalence. The important role of medical students as future healthcare leaders necessitates high awareness and knowledge about the disease. To assess SCD awareness among Al-Baha University medical students, and to evaluate its relation to gender and academic level. A cross-sectional study was conducted, including 105 medical students from the first to sixth year at Al-Baha University. Data collection utilized an online self-administered questionnaire, covering demographic characteristics and assessing SCD knowledge. Fisher's exact and Pearson Chi-squared tests were employed to analyze associations between gender, academic level, and SCD awareness. The majority of male participants 52 (89.6%)and all females 47 (100%) demonstrated awareness of SCD. Clinical year enrollment (68.6%) correlated with heightened awareness across various aspects of SCD compared to the preclinical year. Most participants were in clinical years (68.6%), and 94.3% of them had knowledge about SCD. Approximately, 75 (71%) of participants correctly identified features of sickle cell crisis, 83 (79%) reported the accurate cause of SCD, and 75 (71%) cited the appropriate preventive measures. Only 15 (14%) demonstrated knowledge of correct management of SCD. Contrarily, 84 (80%) were aware of SCD complications, 66 (63%) recognized different SCD types, 67 (64%) felt adequately informed about SCD, and 34 (32%) were involved in SCD counseling. Male participants exhibited greater awareness of SCD features than females. Notably, involvement in SCD counseling was more prevalent among students of clinical years. This study underscores the need for targeted educational initiatives, particularly among preclinical year students to enhance SCD awareness among students. We also emphasize the role of clinical education in fostering a comprehensive understanding of SCD, with increased participation in counseling programs.

Impact of Sickle Cell Awareness Day on online health information seeking in Africa using Google Trends.

The United Nations Council Assembly recognized sickle cell disease (SCD) as a global public health problem due to its increasing burden, particularly in sub-Saharan Africa. To raise awareness, a resolution was adopted, designating June 19th as SCD awareness day. However, the impact of this awareness day on online health information seeking behaviour (OHISB) in African countries is not well understood, especially in Nigeria, Ghana and Uganda where SCD prevalence is high. To assess the impact, the study used Google Trends data as a measure of OHISB for SCD. The analysis covered the 60 days before the awareness day, the awareness day itself, and the 60 days afterward. Time series analysis was conducted using joinpoint regression to identify significant changes in OHISB trends. The results indicated that the impact of the Sickle Cell Awareness Day on OHISB varied across African countries and did not consistently inspire significant changes in information seeking behaviour. This suggests the need for more targeted awareness campaigns to improve public knowledge of SCD in Africa. It also highlights the importance of revising the current awareness day or creating alternative health awareness initiatives that adopt a long-term approach and address the specific health needs of the African population. Furthermore, due to limitations in using Google Trends data in some African countries with insufficient data, future research should explore other sources of internet data or conduct surveys to gain a more comprehensive understanding of the impact of the Sickle Cell Awareness Day on OHISB in Africa.

Motivators and Barriers for People with Sickle Cell Disease Participating in Clinical Trials: Global Findings from the LISTEN Survey

Background: The success of clinical trials of new therapies for sickle cell disease (SCD) will depend on the recruitment and retention of a large and diverse group of people with SCD (PwS). Qualitative studies have reported barriers to participation in clinical trials, including the potential impact on health, unmanageable study demands, limited knowledge of trials, and lack of trust in healthcare(Patterson CA et al. J Pediatr Hematol Oncol 2015; Lee LH et al. Blood Adv 2021). The Learning and Insights into Sickle Cell Trial Experiences (LISTEN) Survey was developed to provide a robust and comprehensive understanding of the global barriers and motivators to participation in clinical trials for PwS. Aim: To present interim findings from the LISTEN Survey regarding participation and experiences of PwS in clinical trials, barriers and motivators to participation, and differences in responses from PwS compared with perspectives of healthcare professionals (HCPs) treating PwS. Methods: Between October 6, 2022 and June 22, 2023, a broad range of adults (≥18 years) with SCD and HCPs involved in the treatment and/or clinical research of SCD in 17 countries were asked to complete corresponding quantitative surveys. PwS were asked whether they had participated in a clinical trial and, if so, whether their experience had met their expectations. PwS were asked to rate on a 7-point scale (from not at all to extremely important) and rank (from most to least important) the importance of specified factors (grouped into five categories) when deciding whether to participate in a clinical trial for SCD. HCPs provided their perspectives on the importance of these factors to PwS. The results presented here include the total proportion of respondents who rated factors as extremely or very important and the proportion who ranked factors first or second. Results: Overall, 1028 PwS (57% female) with a median age (interquartile range) of 30 years (24-37) from 16 of the 17 countries and 361 HCPs (67% hematologists and/or SCD specialists) from 17 countries completed the survey. In total, 35% of PwS had been invited to participate in a clinical trial for SCD. In PwS who had taken part in a clinical trial (24%; n=249/1028), compared with their expectations, 36% reported a better experience, 36% the same as expected experience, 9% a worse experience, and 20% did not know what to expect. Extremely or very important factors that motivated PwS to participate in a clinical trial included the potential to better manage their symptoms (52%), the opportunity to try a new treatment that might work better (51%), and to increase their knowledge of SCD (51%; Figure 1). An important barrier to participation was the potential to experience different side effects (51%). In the trial information category, PwS ranked (first or second of five) safety measures (57%) and how the treatment works (49%) as the most important factors; these were considered to be significantly more important than who is leading (27%) or funding (24%) the trial; p&amp;lt;0.001 for all combinations. In the further considerations category, the majority of PwS ranked (first or second of five) speaking to other PwS involved in the trial (51%) and experts running the trial (50%) as the most important factors. HCPs overstated the potential practical barriers for PwS compared with responses from PwS, including missing school/work (50% vs 42%; p=0.009), the effort required to start the trial treatment (45% vs 32%; p&amp;lt;0.001), and travel requirements (52% vs 36%; p&amp;lt;0.001). HCPs understated the importance of wider clinical trial outcomes as motivators for PwS compared with responses from PwS, including supporting new treatment developments that may benefit them (41% vs 51%; p=0.001) or other PwS (38% vs 49%; p&amp;lt;0.001; Figure 2). Conclusions: Improving access and recruitment into clinical trials in SCD will require clear communication of the potential benefits to individuals and the wider SCD community, as well as potential safety and side effects. Findings from the LISTEN Survey highlight that those directly involved in the trial should deliver these messages, including other PwS who are involved in the trial. Given the disconnect between PwS and HCPs, shared decision-making may also improve understanding and increase participation in clinical trials. Further analyses of the survey results will be important to identify the differences in responses between regions, age groups, and other subpopulations.

Advancing Care of Patients with Sickle Cell Disease through a Transformative Quality Improvement Initiative

Background Managing sickle cell disease (SCD) and its associated complications requires specialized knowledge often limited in primary and emergency care settings. Despite the availability of guideline recommendations and an expanding therapeutic landscape, hematologists experience challenges in managing sickle cell pain and implementing multidisciplinary care coordination that improves morbidity, overall disease management, and quality of life. To improve SCD care, we conducted a quality improvement (QI) study, bringing together healthcare professionals (HCPs) from established SCD centers to assess current practice patterns and develop strategies to overcome barriers to acute pain management and transitions of care for patients with SCD. Methods This QI initiative is comprised of baseline surveys (n = 115), pre- and post-surveys (n = 71) from the small-group, team-based audit-feedback (AF) sessions (Table 1), and 90-day follow-up surveys of HCPs from the four enrolled centers. Survey questions were designed to assess knowledge, confidence, and experiences managing acute pain, transitioning care, and keeping up with the latest clinical evidence and guideline recommendations for SCD. Care teams from each clinic, along with a nationally renowned expert SCD physician, participated in audit and feedback sessions to (a) assess system-specific practice gaps identified via the provider surveys, (b) prioritize areas for improvement, and (c) develop action plans for addressing root causes. Results Baseline surveys results revealed providers' top challenge encountered in managing patients with SCD as managing acute complications (40%) (Figure 1). Gaps were observed in clinicians reporting high/very high confidence in their ability to adequately manage SCD patients' acute pain episodes (51%), align clinical practices with evidence-based SCD guidelines (44%), and coordinate transitions of care (33%). Furthermore, baseline surveys showed only 19% of providers reported they are very/extremely satisfied with their current process for transitioning patients, noting top challenges in patient's difficulty navigating the adult healthcare system (47%) and deficiency of providers caring for patients with SCD near me (26%). Following participation in the AF sessions, clinicians indicated gains in high/very high confidence in their ability to adequately manage an acute pain episode for a patient with SCD (52% to 69%) and ability to coordinate transitions of care for patients with SCD (44% to 56%). During the AF sessions, Care teams set goals to improve provider education, appropriate acute care management of SCD and patient engagement. Action plans to achieve these goals include sharing the action plan with additional clinical team members (47%), collaborating among clinical teams to improve pain management plans for patients with SCD (39%) and improving patient education about SCD (31%). In 90-day follow up surveys, 75% of clinics reported improvements in acute pain management and transitions of care for patients with SCD. Furthermore, the top 3 areas of most improvement at the time of follow-up were enhancing sickle cell education provided to patients (75%), supporting teenage and young adult patients in independently managing their own care (50%), and coordinating meetings with local adult sickle cell providers and teenage/young adult patients and families (50%). Conclusions Through this QI initiative, clinical teams identified barriers to optimal SCD care, creating and implementing specific action plans to increasing knowledge of SCD and evidence-based therapies, optimizing transitions of care and improving acute pain management. While meaningful confidence and action plan gains were demonstrated, these data underscore clinical practice gaps to address in future initiatives to support safe, effective, and evidence-based processes for managing SCD to prevent complications and optimize outcomes and quality of life. Study Sponsor Statement The study reported in this abstract was funded by educational grants from Vertex Pharmaceuticals Incorporated and Agios Pharmaceuticals, Inc. The grantors had no role in the study design, execution, analysis, or reporting.

Understanding Gaps in Current Practices of Newborn Screening Follow-up for Sickle Cell Disease in the United States

Introduction: Newborn screening (NBS) for sickle cell disease (SCD) has significantly improved childhood survival. However, gaps remain in follow-up after NBS as demonstrated by poor implementation of penicillin prophylaxis and stroke risk (TCD) screening. It is unknown how time to initial engagement with SCD specialist may impact long-term patient engagement. Currently, there are no standardized follow-up requirements for NBS across the United States; each state can decide how they inform families of diagnoses and initiate follow-up. The average time to the initial follow-up with a SCD specialist is not currently evaluated as a measure of quality assurance. Semi-structured interviews were conducted with 19 participants across 11 states to explore NBS processes. Participants included health care providers (n = 7), and stakeholders (NBS health department employees and community based SCD organization representatives (CBO); (n = 12). Data were analyzed using directed content analysis, informed by the SCD care continuum model, which outlines how newborns with SCD should proceed from diagnosis to initial and ongoing care engagement with a SCD provider. We examined how families were informed of abnormal results, how newly-diagnosed children were referred to SCD specialists, and when cases were closed. Results Representatives from 11 states including AL, AK, CA, GA, LA, MI, MS, MO, NY, SC, TN were included in this study (Table 1). Initial Notification: In all 11 states, the physician of record (on the NBS bloodspot card) is notified of abnormal results and expected to communicate results to families. This physician of record is not necessarily the child's actual pediatrician. In 2 states, a letter is sent to families at the same time as physicians are notified, but the letter does not convey screening results. In 1 state, the CBO NBS coordinator is responsible for communicating results to the family. Referral to a SCD specialist: There was substantial variability in referral practices. In most states, infants with positive SCD screens were referred to a SCD specialist by the pediatrician. In some states, the public health department refers infants with positive screens directly to the SCD center. In 2 states, the decision to refer an infant to a SCD center is left to the family, NBS coordinator or CBO. Timing of first visit: Most states intend infants with positive SCD screens to follow-up with the SCD clinic in &amp;lt; 2 months; however, some states do not require the initial visit until 6 months. Notably, most states do not track whether children with a positive SCD screen ever see a SCD specialist, Instead, cases are closed once the physician of record is informed. Facilitators and Barriers: The most common facilitators for communicating NBS results were having the correct physician of record on the blood spot card, the physician's knowledge of SCD, or the use of state-based immunization databases to track children with missing demographic information. Key facilitators for linkage to care included partnerships with SCD CBOs and/or having designated NBS follow up coordinators that offer ongoing family education and appointment reminders. Other facilitators included families with extended support and those who already had a child with SCD. The most common barriers to communicating NBS results were difficulty locating or reaching families, families moving out of state, insufficient contact information, and/or having the wrong physician of record. The main barriers to linkage to care included lack of insurance, lack of transportation, families who have difficulty accepting the SCD diagnosis, immigrant or refugee families with language barriers and other conflicting social/life demands. Conclusion The results of our study demonstrate wide variability in NBS follow-up for SCD with limited quality assurance to ensure newborns see a SCD specialist. These findings indicate a large multi-level intervention trial is needed to identify best practices for systematic improvement in NBS follow up process and ensure all infants are seen by a SCD specialist.

Sickle Cell Disease Management Practices across Nigeria: A Cross-Sectional Analysis

Introduction Of 300,000 children born with sickle cell disease (SCD) in the world, approximately 75% of these are born in Sub-Saharan Africa. Owing to the frequent painful vaso-occlusive and hemolytic episodes associated with this disease, irreversible multi-organ damage and premature mortality are common. For instance, in Nigeria, 490 deaths per 1000 births among children under 5 have been attributed to SCD; this is four times higher compared to mortality in children born without SCD. On the other hand, those with SCD who survive beyond childhood are faced with an estimated life expectancy of 21 years. To address these challenges, guidelines for SCD management were developed by the Nigerian Federal Health Ministry, National Heart, Lung, and Blood Institute (NHLBI), and the American Society of Hematology but their dissemination and implementation has been difficult. To better understand current management practices, we present a preliminary, descriptive analysis of hydroxyurea and blood transfusion practices for sickle cell disease management across Nigeria. Methods Using a cross-sectional design, we collected demographic and practice-related information from eligible clinicians that provide SCD care in Nigeria. The “SCD Knowledge, Attitudes, and Practices - Nigeria” survey was disseminated to pediatric and adult consultant physicians, resident doctors, and nurses through their respective professional communication platforms. SCD-related practices questions were based on the NHLBI recommendations for SCD management which informed the Nigerian Federal Ministry's National SCD Guideline. A 5-point Likert scale was used to answer each question on hydroxyurea use and blood transfusion. Descriptive analyses of current practices were used to characterize these results and bivariate analyses were conducted using Chi-square or Fisher's test as appropriate. Data analysis was performed using SAS 9.4 (SAS Institute; Cary, NC). Ethical approval was obtained from Duke University Institutional Review Board and the University of Nigeria, College of Medicine Health Research Ethics Committee. Results Analysis of SCD-related practices from the first 73 participants who have completed the survey, revealed most respondents to be female (62%), between 35-65 years old (77%), and adult hematologists or nurses (37% and 40% respectively). This group of clinicians provide care mainly to adults (47%), but 45% admitted providing care to both adults and children. Nearly half (45%) of this group provides care for 0-5 individuals with SCD weekly and 57 participants (78%) work in tertiary-level hospitals. Only 22 of those working in tertiary hospitals had a comprehensive SCD center in their hospital compared to those without an SCD center (39% vs. 61%, p=0.0148). SCD experts were also found in tertiary-level hospitals (75% vs. 25%, p&amp;lt;0.0001). General hematologist expertise was also common in tertiary care (88% vs. 12%, p=0.2424). Figures 1 and 2 report HU and blood transfusion practices. Although 73% confirmed hydroxyurea (HU) prescribing and 90% endorsed the use of simple blood transfusions, HU and blood transfusion practices varied greatly. Respondents indicated most educated patients about HU (frequently/always = 74%) and started HU for patients with &amp;gt;3 VOCs in past year (frequently/always = 62%), however, only 31% frequently/always begin at age 9 months (Figure 1). Most respondents reported prophylactic blood transfusions to reduce HbS below 30% (frequently/always = 71%) and 73% frequently/always performed transfusions for acute SCD complications, yet, 60% indicated they never/rarely match for RBC antigens. Conclusion The data highlights SCD-related expertise and specialty care are found more in tertiary-level hospitals. HU practices described may be attributed to the more conservative approach suggested by the Nigerian SCD Management Guideline compared to the NHLBI recommendations, however there is an important opportunity to expand HU prescribing. Blood transfusion, which has historically been considered difficult in this differently resourced setting, was common. However, chelation, leukoreduction/white blood cell filtration, and red blood cell antigen testing were not common. These preliminary findings represent an opportunity for a larger study and future studies to investigate strategies to increase uptake of existing SCD management recommendations.

Quality of Care Among Emerging Adults with Sickle Cell Disease Using the Ascq-ME Qoc Measure: Baseline ST3P-up Study Data Results

ASCQ-Me ® ( Adult Sickle Cell Quality of Life Measurement Information System) is a disease specific patient-reported outcome measurement system to evaluate and track the physical, mental, and social well-being of adults living with sickle cell disease (SCD). It includes a mixed format ASCQ-Me Quality of Care (QOC) measure which aims to quantitatively, and qualitatively, understand the way adults living with SCD experience healthcare. The ASCQ-Me QOC measure follows the Donabedian's model that evaluates health services research around structure, process, and outcomes of healthcare system interactions.1 Prior and ongoing research report that adults with SCD avoid going to the emergency department (ED) for acute symptoms.2,3 Poor provider knowledge and understanding of SCD feeds a misperception as drug seeking and heightens the structural inequities and racial stigma of living with the disease.4 This is further compounded by poor adherence to acute triage guidelines, disbelief in patient reports of pain and of their usual care plan, severe delays in receiving acute care, poor provider patient communication and distrust. The Sickle Cell Trevor Thompson Transition Project (ST3P-UP), a multicenter, comparative effectiveness study of a structured education-based transition program +/- peer mentoring to improve acute care utilization for SCD ages 16-26y was conducted at 14 SCD clinical sites.5 Each site was required to engage a community-based organization (CBO) and had established community advisory boards (CABs) focused on collaborative partnerships to improve access to and experience of care. The study captured baseline and longitudinal reports of patients' experiences with health care using the ASCQ-Me QOC measure on all enrolled participants with annual rescoring as part of routine care. ST3P-UP study demographics: N=291, mean age 18.8y (SD1.5); 52% F; 96% Black; 3.6 acute care visits/yr. at baseline (range 0-31, SD4.9). Over 84% of participants reported being usually or always satisfied with the QOC when care was needed right away. Overall health care was rated between 7-10 (10 = best care possible) by 83%; 73% reported they usually or always were able to access care right away when needed while 78% usually or always waited &amp;lt;2h to receive treatment for their acute pain. These reports were equivalent to that reported when SCD experts care for SCD patients. QOC scores for ED care were higher than previously published and also better than anecdotal reports. Specifically, patients reported that they felt the doctors (84% of the time) and nurses (83% of the time) usually or always seemed to really care about them and that when they needed care right away, they were treated with courtesy and respect by clerks and receptionists usually or always 87% of the time. Only 11% of participants reported that providers were able to only help their pain “a little or not at all” and that providers “ did not” or “only slightly” believed their report of pain, respectively. Participants were usually or always satisfied with the care received in the ED 94.1% of the time. However, a bad experience continues to drive the decision to avoid going to the ED for care in 63% of participants. A previous report by Kanter et al cited only 51% of adults with SCD were usually or always satisfied with the ED care received and 52% indicated a bad experience in the ED led them to decide to avoid seeking care.6 These results demonstrate a surprisingly higher satisfaction with the quality, process and outcomes of ED care delivered and less discrepancy between ambulatory vs. ED care than expected compared to published and anecdotal reports. This could reflect the bias of ST3P-UP study enrollment and a younger patient population with ~25% having &amp;lt;1 baseline acute visits /yr. ST3P-UP sites had existing strong partnerships with providers, a local CBO and established CABs thus care may not be generalizable to the broader SCD care ecosystem. While longitudinal survey results will be important as comparison, our data highlights the value of objectively quantifying ambulatory and ED experiences soon after a visit to gain quantitative insights into these experiences. Our results do not obviate the need for subjective reports on specific experiences however both types of data can guide local and systemic improvement efforts with attention on identifying subpopulations differently affected and interventions that lower the risk for poor QOC experiences.

Knowledge, Beliefs and Attitude towards Sickle Cell Disease among Students of Kampala International University Jinja Teaching Site Uganda

Sickle cell disease (SCD) is one of the most widespread hereditary disorders among the African lineage. SCD is associated with recurrent severe pain, increased morbidity and mortality yet has received less recognition in the public domain. Evidence of the need to increase awareness to reduce the disease incidence is increasing due to the rising incidence of this preventable disease. This study aimed to describe the knowledge, beliefs and attitude towards SCD of students at Kampala international university. A descriptive cross-sectional study design was employed. A total of 384 university students participated in the study, and using a semi-structured questionnaire, information was collected from participants on demographic characteristics, general knowledge of SCD, and beliefs and attitudes of students towards SCD. Nearly all the students were aware of SCD (99.2%). Knowledge level of respondents on SCD based on scores revealed a mean score of 9.7± 4.5 with 65%, 30%, and 5% for poor, moderate and excellent respectively. Most of the respondents strongly agreed that they feel worried (52.9%) and sympathetic (51.3%) for people affected with SCD. Participants had the belief that it is an inherited disease acquired from parents (48.2%) but not a punishment from God (76.3%). In general, there was a limited understanding and inadequate knowledge of SCD among the students particularly on the pattern of inheritance. Results from the study highlights the need for effective public health education on SCT/SCD in trusted sources such as schools, media (radio/Television), health centres and churches. This is necessary to address misconceptions and increase knowledge level as well as an understanding of the risks of having a child with SCD to influence personal reproductive options. Keywords: Sickle cell disease, Parents, Students, Child, Preventable disease.

Disease knowledge and health literacy in parents of children with sickle cell disease.

We aimed to (1) describe sickle cell disease (SCD) knowledge and health literacy levels in parents of children with SCD, (2) examine associations with socio-demographic factors and (3) analyse the association with hospital admissions andfrequency of occurrence of painful episodes. Parents who presented with their child at routine hospital consultation at the National Sickle Cell Disease Centre in Benin were administered a questionnaire assessing SCD knowledge, health literacy (newest vital sign [NVS]) and socio-demographic and clinical characteristics. In total, 117 parents participated (108, 92.3% females). The predominant SCD genotype was HbSS (79.5%). The average SCD knowledge score was 13.6 (standard deviation [SD]=2.0). Only 34 (29.1%) participants correctly answered ≥70% of the questions, indicating good knowledge. Health literacy was relatively low (mean NVS score=3.3; SD=1.1). SCD knowledge was higher in parents with older children (p=0.001) and higher education levels (primary, p=0.010; tertiary, p=0.036 compared to participants with no formal education). Hospital admissions were more frequent when parents had lower SCD knowledge (p=0.034) and in parents with younger children (p=0.039). No associations were found between health literacy and hospital admissions (p=0.940) and frequency of occurrence of painful episodes (p=0.224). Continuous disease-specific education for parents of children with SCD may help them better identify and prevent the occurrence of symptoms and decrease the number of hospital admissions.

Sickle Cell Disease Knowledge and Reproductive Decisions: A Saudi Cross-Sectional Study.

Sickle cell disease (SCD) is an autosomal recessive disorder. Decisions following premarital screening results might be influenced by several factors. Thus, this study aims to assess the knowledge and beliefs toward SCD and reproductive decisions. A cross-sectional study was conducted among adults in the eastern province of Saudi Arabia. Data was collected using a web-based questionnaire. Participants were divided into two groups based on their knowledge scores. A total of 390 participants were recruited with a mean age of 28.6 years. The majority (90.5%) of participants had general knowledge about the disease mode of inheritance however, 66.9% had poor knowledge about the disease overall. Regarding knowledge level, there was no statistically significant difference between the non-healthy group (diseased and carrier) and healthy participants (P=0.304). Moreover, the participants' decisions about choosing future partners were more likely to be affected among those with prior knowledge about the disease (p=0.008). However, 152 (91.6%) male participants with prior knowledge would change their decision about selecting a future partner compared to 225 (92.4%) female participants. Regarding reproduction, only 38.5% of participants were aware of in vitro fertilization (IVF) as an assistive reproductive technology. Furthermore, female participants believed that IVF is a way to have healthy babies and would consider it despite the cost more than male participants (p=0.0001, p=0.007 respectively). SCD is an inherited disease with economic, physical, and psychological burdens. However, curative options are costly, and hence, prevention is key. Therefore, healthcare decision-makers should consider implementing policies to minimize the financial burden that may still affect society despite the availability of free medical care. This study warrants extensive community-based education programs that may contribute toward cost savings. It also highlights the importance of premarital counselling for disease and carrier people including alternative reproduction options.

Strategies to improve healthcare services for patients with sickle cell disease in Nigeria: The perspectives of stakeholders.

Background: Sickle cell disease (SCD) continues to pose physical and psychosocial burdens to patients, caregivers and health workers. Stakeholder engagement in the processes of policy making and implementation is increasingly becoming the cornerstone of best practices in healthcare. Aim and Objectives: To engage stakeholders with a view to assessing the knowledge of SCD; ascertain the challenges associated with accessibility and affordability of healthcare services; improve the quality of care, and thereby effect behavioral change through increasing attendance and follow-up of patients in the clinics. Methodology: A Stakeholders' Engagement meeting organized by the Sickle Pan Africa Research Consortium Nigeria Network (SPARC-NEt) was attended by patients, caregivers and members of patient support groups, healthcare providers and management/policymakers. The engagement was through PowerPoint presentations, structured questionnaires and an interactive session. The structured questionnaire assessed the knowledge of stakeholders about SCD; the quality of healthcare services; challenges with access and affordability; and SCD-related government policies. Results: Three hundred and twelve stakeholders attended the engagement meeting. Of the 133 that participated in the study, medical workers were the most represented. The majority had good knowledge of what causes SCD (96.2%) and the best place to get help during SCD crisis (98.5%). However, knowledge of the specific preventive measures of SCD and its crisis was not optimal. In terms of the role of community engagement and education, only about one-quarter of the study participants, 34 (25.6%) knew about their positive role in reducing the prevalence of SCD and alleviating SCD crises. Challenges identified include inadequate healthcare personnel and facilities, delay in obtaining laboratory results, long waiting time in the clinic, poor communication, absence of holistic consultation, uncoordinated healthcare services, high cost of care, ignorance, non-prioritization of SCD by government, lack of multisectoral collaboration and partnership with NGOs and international organizations. Strategies proffered to improve healthcare services include, community/stakeholder engagement and health education, sickle cell daycare services, access to a willing and dedicated multidisciplinary workforce, collaboration with support groups and government policies and programs. Conclusion: There is need for regular stakeholder engagement to improve access to healthcare services for SCD patients in Nigeria.

Assessing knowledge of sickle cell disease and health beliefs on premarital genetic screening among healthcare trainees at a tertiary institution: A cross-sectional study.

The uptake of sickle cell trait (SCT) test is challenged by several factors. A community of healthcare professionals educating the public to undergo screening is critical in reducing the disease burden. We investigated knowledge and attitude towards premarital SCT screening among healthcare trainee students who are the next generation of healthcare practitioners. A cross-sectional design was employed, and quantitative data were collected from 451 female students pursuing healthcare programs at a tertiary institution in Ghana. Descriptive, bivariate, and multivariate logistic regression analysis was performed. More than half of the participants were 20-24 years (54.55%) and had good knowledge (71.18%) about sickle cell disease (SCD). Age and school or social media as sources of information were significantly associated with good knowledge about SCD. Students between the age 20-24 (adjusted odds ratio [AOR] = 2.54, confidence interval [CI] = 1.30-4.97) and knowledge (AOR = 2.19, CI = 1.41-3.39) were 3 times and 2 times more likely to have a positive perception about SCD severity. Students who have SCT (AOR = 5.16, CI = 2.46-10.82), whose source of information was family member/friends (AOR = 2.83, CI = 1.44-5.59) and social media (AOR = 4.59, CI = 2.09-10.12) were 5 times, 2 times and 5 times likely to have a positive perception about the susceptibility of SCD. Students whose source of information is school (AOR = 2.06, CI = 1.11-3.81) and who have good knowledge of SCD (AOR = 2.25, CI = 1.44-3.52) were 2 times more likely to have a positive perception about the benefits of testing. Students with SCT (AOR = 2.64, CI = 1.36-5.13) and source of information was social media (AOR = 3.01, CI = 1.36-6.64) were about 3 times more likely to have a positive perception about the barriers to testing. Our data shows that high level of SCD knowledge influences positive perceptions about the severity of SCD, the benefits and relatively low barriers to SCT or SCD testing and genetic counseling. Dissemination of SCT, SCD and premarital genetic counseling education should be intensified especially in schools.

Caregivers’ Perspective on the Psychological Burden of Living with Children Affected by Sickle Cell Disease in Kinshasa, the Democratic Republic of Congo

There is limited information on knowledge, perceptions, and management of sickle cell disease (SCD) in Africa in general and in the Democratic Republic of the Congo (DRC) in particular. This study explored knowledge, perceptions, and burden of 26 parents/caregivers of children with SCD in three selected hospitals in Kinshasa, DRC. We conducted a focus group with in-depth interviews with parents/caregivers of children affected with SCD. Four themes were discussed, including knowledge and perceptions, diagnosis and management, society's perceptions, and the psychosocial burden and the quality of life of the family affected by SCD. The majority of participants/caregivers felt that society, in general, had negative perceptions of, attitudes toward, and knowledge about SCD. They reported that children with sickle cell are often marginalized, ignored, and excluded from society or school. They face a number of challenges related to care, management, financial difficulties, and a lack of psychological support. The results suggest the need to promote measures and strategies to improve knowledge and management of SCD in Kinshasa, DRC.

The efficacy of maternal health education and maternal screening on knowledge and the uptake of infant screening for sickle cell disease in Dar-Es-Salaam, Tanzania; a quasi experimental study

BackgroundGlobally, Sickle cell disease (SCD) is one of the most common genetic disease with high childhood mortality. Early identification of babies with SCD through newborn screening (NBS) and linking them to care are among the recommended interventions. The purpose of this study was to assess the efficacy of maternal health education and maternal screening for SCD on knowledge and the uptake of infant screening for SCD among mother-infant pairs attending antenatal clinics at Government health facilities in Dar-es-salaam, Tanzania. MethodsThis study was a pre-test post-test, quasi-experimental which involved pregnant women attending antenatal clinics at three hospitals; Mbagala hospital, Sinza hospital and Buguruni health center in Dar Es Salaam. A structured questionnaire was used in data collection. Knowledge on SCD was assessed for all participants before and after two sessions of health education. Participants in Mbagala and Buguruni were also screened for SCD using Sickle SCAN point-of-care test (BioMedomics Inc, USA). The efficacy for health education intervention was computed as the post-intervention minus baseline knowledge score. For proportions, a two-sample z-test was used. Univariate and multivariate logistic regression were used to analyze the efficacy of health education intervention and also predictors of infant diagnosis. ResultsFor two sessions of health education intervention, a total of 467 pregnant women completed the sessions. During antenatal visits, a total of 218 were screened for SCD. The proportion of participants with good knowledge of SCD had significantly increased to 85.9% from 12.4% at baseline following the education intervention. In multivariate analysis, sharing the received education on SCD was an independent predictor of the efficacy of health education intervention. Maternal occupation, maternal SCD status as well as sharing the received education on SCD were independent predictors of the uptake of SCD infant diagnosis.ConclusionThis study has demonstrated that maternal health education and maternal screening for SCD are feasible and efficacious interventions in raising knowledge and improving the uptake of infant diagnosis for SCD. These interventions are strongly recommended to be included in the comprehensive care package for pregnant women attending antenatal clinics, particularly in areas with a high burden of SCD.

Awareness of Sickle Cell Disease Among Adolescents and Young Adults

Background: Sickle hemoglobinopathy is the most common genetic disorder identified through the state mandated newborn screening program, occurring in 1:2,647 births and may pose an enormous health burden. While sickle cell disease is associated with significant morbidity, sickle cell trait is a benign condition. Due to this reason, knowledge of their sickle cell trait for an individual may be lacking while the condition may have been identified at birth. There were 2904 cases of sickle cell traits identified through the NJ newborn screening program in 2020. While other countries have programs to increase awareness of sickle hemoglobinopathy status to include premarital genetic screening, the implication of sickle cell trait on procreation and family planning are not highly emphasized in the US. Assessing awareness of sickle cell disease among adolescents is important given high rates of teenage pregnancies among this population. Through this study, we set out to assess awareness of sickle cell disease among adolescents. Methods: We performed a cross sectional study of 180 adolescents who sought medical care at one of the outpatient clinics or emergency room at Children's Hospital of New Jersey in Newark, NJ and administered an anonymous questionnaire to volunteers 12-21 years of age from January 2020-June 2022. The study was approved by our local IRB and an informed consent and/or assent was obtained from each enrolled subject. Questionnaires included a variety of questions including demographics and several questions to assess their knowledge of sickle cell disease. Questionnaires that were incomplete were not included in analysis. Results: Of the 180 adolescents, most were teenagers ages 14-18 years old (72%), majority identified as females (59%) and 92% were African Americans or Hispanic. More than 30% of subjects' parents were high school graduates, 25% were college graduates and the remainder had some other lower level of education. Out of 180,136 children (76%) had heard of sickle cell disease and only 78 children (44%) reported that they knew about sickle cell disease. However, when asked questions regarding sickle cell disease, only 12% were able to correctly identify that it is a genetic disease that may cause pain crises. One hundred and eighteen patients (66%) were unaware of their sickle cell status. Of those with sexual partners, 53% did not think that knowing their partner's sickle cell status would influence their choice and yet, 59% wouldn't consider having a child with someone who has sickle cell disease. Only 5 individuals (2.78%) were able to correctly identify the inheritance pattern of sickle cell disease. Of the 4 individuals with children, only 1 (25%) reported being aware of their child's sickle cell status. Ninety-two percent were interested in learning about sickle cell disease, and the majority identified their doctor, internet and school as primary forms of obtaining health information. Conclusion: Sickle cell disease is associated with significant health burden. While we cannot prevent sickle cell disease, as health care providers, we should make every effort to educate youth about this important condition. One of the ways is to review newborn screening hemoglobinopathy results during adolescent well visits, especially for those with sickle cell trait. Appropriate education and genetic counseling must be provided to these young individuals especially since many may engage in sexual activity at a young age.

"Here's What I Wish You Knew": A Qualitative Narrative Analysis of the Adolescent Sickle Cell Pain Experience

Background Bias, stigmatizing language, and social isolation are significant barriers to the optimal care of pain for adolescents and young adults (AYA) with sickle cell disease (SCD). Qualitative studies that include first-person narratives of SCD pain are underrepresented in outcomes research but provide valuable information for clinical education and community care models. Here, we present the interim analysis of an ongoing thematic research study that analyzes narrative experiences of AYA patients with SCD and their journeys- both in and out of the healthcare system- with chronic pain. Methods In this IRB-approved study, a single semi-structured interview is administered to patients ages 13-21 with sickle cell disease (Hemoglobin SS, SC, and SBeta0Thal genotypes) living in a mid-sized metropolitan center and receiving care at an academic teaching hospital. Patients are offered the opportunity to participate from inpatient units, ambulatory continuity clinics, and community advocacy groups. Interviews are conducted either in-person or remotely, recorded, transcribed, and independently checked for accuracy. The interview includes questions about coping with pain in medical and non-medical settings, interactions with the healthcare team, impacts of pain on social and community life, and opportunities to describe what patients wish family members, teachers, friends, and healthcare providers understood about living with SCD pain. Transcripts are analyzed for thematic coding by 2 independent coders using a deductive approach with latent textual analysis (Braun & Clarke, 2013). Codes are translated into themes by key-words-in-context and indigenous categorization methods (Ryan, 2011). Thematic coders are not members of the patients' clinical care teams, and patients do not provide interviews during acute pain crises. Results At the time of submission, 8 interviews were completed and analyzed. The median patient age was 17 years. Concordant coding revealed five primary themes: the relationship between acute and chronic pain, coping with rituals and cycles of care, the unpredictable nature of pain, navigating a biased healthcare system, and social isolation. The majority of patients detailed their experiences with intense, coexistent physical and emotional pain (6/8) that creates a pervasive sense of uncertainty and foreboding (4/8) in their lives. They described navigating a healthcare system that is difficult to understand, while experiencing doubts and dismissals of their pain from healthcare providers, particularly when making the transition to adult care (4/8). Lack of education and awareness about SCD in academic settings (5/8) and "missing out" on social milestones and norms (6/8) were cited as sources of isolation and emotional fatigue. Having a family member or community-based care advocate was viewed as a critical source of support within both the health system and community (4/8). Discussion and Future Directions This ongoing qualitative research study in adolescent and young adults with SCD provides narrative data about the lived experience of sickle cell pain from the patient perspective. Interim analysis suggests several locations in the care trajectory where reports of pain may be particularly vulnerable to bias: in healthcare settings for management of acute-onset pain crisis, in schools and other academic support settings where knowledge of SCD may be incomplete, and in the transition of care from pediatric to adult care models. Particularly during acute pain management and transition care experiences, patients describe the use of stigmatizing language- in conversation and in the electronic health record- that contribute to feelings of isolation, frustration, and emotional suffering. Results of this study are being used to inform community, pastoral, and school-based sickle cell education programs as well as medical education curricula aimed at reducing stigmatizing language. Results are also being used to develop standard criteria for sickle cell patient advocates, and to improve infrastructure for the integration of patient advocates into sickle cell care teams. Future research will measure the impact of patient participation in narrative research on clinical outcomes and attitudes toward engaging the healthcare system, both for routine care and potential SCD clinical research. Figure 1View largeDownload PPTFigure 1View largeDownload PPT Close modal