Awareness Of Sickle Cell Disease Research Articles

Update on the practice of premarital screening for sickle cell traits in Africa: a systematic review and meta-analysis

BackgroundScreening for sickle cell traits before marriage or producing children is one of the outstanding preventive measures for sickle cell disease (SCD).The disease is a collection of inherited blood disorders that impact millions globally, with a predominant 75% occurrence in the sub-Saharan region. With increasing burden of SCD on the continent amidst a cost effective prevention method, no study has systematically reviewed or presented meta-analytic uptake or practice of premarital sickle cell trait screening.MethodsThis review systematically explored the uptake or practice of premarital genotype screening in Africa. We searched PubMed and Scopus databases for African studies on premarital screening for sickle cell traits.ResultsOur results indicate that the pooled uptake of premarital sickle cell trait screening in Africa is 47.82% (95% CI: [46.53–49.11]; I2: 98.95% [98.74–99.13]). Our review observed, a significant relationship between the awareness of sickle cell disease and the uptake of genotype screening; F(1, 13) = 12.04, p = 0.004). The model explained approximately 48.08% of the variation in genotype screening (R² = 0.4808) and predicted a 0.729 increase in the likelihood of genotype screening uptake for every unit rise in sickle cell disease awareness (β = 0.729, p = 0.004). Additionally, Pearson correlation (r = 0.6934) indicated a moderately strong positive correlation between the two variables.ConclusionWith over 75% of the global burden of sickle cell disease domiciled in Africa, the continent cannot overlook the cost of hemoglobinopathies. The uptake of sickle cell traits screening is suboptimal across the continent. To achieve the mandate of sustainable development goal number (3); to end preventable deaths of newborns and children under 5 years of age by 2030, there is need to intensify campaigns on premarital genetic screening through education and other health promotion tools.

IJCM_364A: A Cross Sectional Study on Sickle Cell Disease awareness among adolescents in a tribal district of Maharashtra, India

Background: Sickle Cell Disease (SCD) poses a substantial health concern in India, especially among tribal populations. The Ministry of Health and Family Welfare’s Tribal Health Expert Committee has identified SCD as one of the top 10 challenges in tribal health. This study aims to assess the knowledge gap about SCD among school-going adolescents in a tribal district of Maharashtra. Objective: 1. To assess awareness of sickle cell anaemia among adolescents aged 12-18 years in a tribal district of Maharashtra 2. To provide recommendations based on study findings. Methodology: A cross-sectional study was conducted in a tribal district of Maharashtra, involving 394 school-going adolescents. Participant’s knowledge of sickle cell anaemia was assessed through face-to-face interview after obtaining necessary permissions from concerned authorities and assent from the participants. Data collection included information on general awareness, understanding of disease transmission, familiarity with sickle cell crisis, and preventive measures, with strict maintenance of data confidentiality. Results: Out of the 394 participants surveyed, 212 (53.8%) were females, and 182 (46.2%) were males. Only 19 students (5%) demonstrated awareness of SCD, with 16 (7.5%) females and 3 (1.6%) males out of the total. Surprisingly, none (0%) had knowledge of its transmission or had undergone screening for SCD. Within the aware subset, only 3 (15%) recognized Sickle Cell Crises, and a lone respondent (5%) was aware of its preventive measures. Conclusion: The study highlights a notable lack of sickle cell disease awareness among school-going adolescents in Maharashtra’s tribal district, posing a potential public health challenge. Urgent, tailored interventions are essential to sensitize the future generation, aligning with SDG 3, which aims for improved health and well -being through enhanced understanding and awareness.

Awareness of sickle cell disease among nursing undergraduates in Farasan: Its interference with malaria.

The present study was conducted to generate data on awareness and incidence of sickle cell disease (SCD) and also to adduce the widespread myths peddled about SCD. Students studying in the Department of Nursing were recruited. A pretested, self-administered sickle cell assessment questionnaire was distributed electronically through WhatsApp group to collect necessary data. Participants were screened for malaria by thin blood smear analyses, and their hemoglobin (Hb) contents (g/dL) were determined by Sahli's haemoglobinometer. Statistical analyses were done using Origin (version 8.1, USA). A reliability study was performed for the validity of questionnaire data. Study participants had significantly high awareness regarding SCDs (89.9%, P < 0.001). Most participants (96.3%) were aware about government policy regarding premarital screening for genetic disorders and replied that the government has strict health policies backed by equally robust laboratory diagnostics. Moreover, none of the participants had SCDs, although their parents had a consanguineous marriage. Thin blood smear analyses of participants did not reveal any cases of Plasmodium falciparum. However, significant percentages (33.1%) were found to be anemic, probably due to their dietary habits and lifestyles, as has been reflected by questionnaire analyses. Furthermore, a very less number of students had knowledge about genetic variations that might occur in malaria-endemic regions after long exposure to offer protection from malaria. Knowledge about management practices was also lacking among study participants (29%). This research points to the necessity that the nursing study plan should focus on providing specific training on management skills and preventive measures for SCDs, which is of paramount importance.

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.

Exploring the Experiences of Pediatric Patients with Sickle Cell Disease and Their Families

Background: Sickle Cell Disease (SCD) is a common lifelong genetic disorder of red blood cells, which causes abnormal hemoglobin shape often resulting in pain crises and other serious health complications (Ware, et al., 2017). SCD disproportionally affects people of African descent, and several recent surveys have shown that patients with SCD experience discrimination in healthcare settings (Nelson and Hackman, 2013; Haywood, et al., 2014; Kanter, 2020; Oyedeji and Strouse, 2020). In pediatrics, parents of patients with SCD report higher levels of race-related discrimination in healthcare settings compared to families with other chronic illnesses, as well as decreased satisfaction in several areas of their care (Brousseau, 2009; Oyedeji, 2020; Kanter, et al., 2020). This qualitative project aimed to explore further specific, actionable areas of dissatisfaction and any areas of increased satisfaction among pediatric patients with SCD and their families. Methods: Semi-structured interviews were conducted with pediatric patients with SCD and/or their guardians at one academic institution. Any patient with SCD over the age of eight was eligible to participate in an interview, and parents of a child with SCD of any age were eligible to participate, as well. Interviews were audio-recorded and took place in person in clinic. Recordings were then transcribed verbatim. The interviews were qualitatively coded using standard multi-level semantic analysis, working directly from the transcriptions and audio-recordings. Seven (41%) interviews were independently double-coded, and discrepancies were resolved by consensus. Final codes were analyzed, and major themes were identified. Results: Eighteen pediatric patients with SCD and/or their families were asked to participate in the study, and seventeen (94%) agreed. There were twenty-six total participants. Ten family interviews were conducted, and another seven were conducted with parents only because the patients were younger than eight years old. Fifteen families identified as African American, one family identified as Hispanic, and one family identified as African. Patients and their families described five main areas leading to dissatisfaction in the healthcare setting: (1) Long wait times; (2) Neglect for SCD protocols within emergency departments; (3) Biases against patients with sickle cell disease; (4) Frustration with procedures, specifically with blood draws; (5) Lack of empathy and compassionate listening among healthcare providers. Positive experiences were almost all due to aspects of strong patient-provider relationships. Outside of the healthcare setting, patients and their families emphasized the need for improved education and awareness of SCD within the community. Conclusion: While this qualitative study explored perspectives from only a limited number of pediatric families, these conversations provided insight into specific areas of dissatisfaction among patients with SCD. This research provides actionable items that healthcare providers can apply to help improve the healthcare experiences and the everyday lives of children with SCD. Additional efforts and resources should be directed toward addressing social determinants of health and systemic racism to future improve healthcare experiences of patients with SCD.

Sickle Cell Illness Awareness among the General Public

Background: Lifelong ickle cell disease (SCD), a group of inherited blood disorders, afflicts millions of individuals. Sickle cell disease (SCD), with a global prevalence of 112 cases per 100,000 individuals, frequently gives rise to this condition. Sickle Cell Disease (SCD) exhibits a high prevalence in various regions, including Sub-Saharan Africa, Saudi Arabia, India, South and Central America, as well as the Mediterranean. We conducted a study in Tabuk, Saudi Arabia to assess the level of public knowledge and awareness of Sickle Cell Disease (SCD). Methods: The present study employed a cross-sectional observational design, encompassing a sample of 386 individuals residing in Tabuk, who were over the age of 18 and represented both genders and various nationalities. Demographic data and sickle cell disease awareness were obtained through the utilization of a structured questionnaire that was developed from previous research. Results: The present study included a total of 386 adults residing in Tabuk, Saudi Arabia, who satisfied the predetermined inclusion criteria. Among the participants, 47.4% fell between the age range of 18 to 25 years. The majority of participants had a satisfactory level of knowledge, with 24.1% of individuals aged 18-25, 10.1% of those aged 26-35, 7.3% and 6.55% of individuals aged 36-45, and a significant proportion of participants aged over 45. Conclusion: The survey participants demonstrated a satisfactory degree of understanding on the prevalence of sickle cell disease (SCD) in the Kingdom of Saudi Arabia (KSA).

Sickle cell disease: time to act on the most neglected global health problem

Despite being the first monogenic disease described in scientific literature over a century ago, sickle cell disease remains an illness with high morbidity and early mortality, particularly in sub-Saharan Africa and other low-income and middle-income countries (LMICs) in historically malaria-endemic regions of the world. 1 Piel FB Steinberg MH Rees DC Sickle cell disease. N Engl J Med. 2017; 376: 1561-1573 Crossref PubMed Scopus (635) Google Scholar Sickle cell disease is a chronic and progressive condition associated with frequent painful, vaso-occlusive episodes, multiple end-organ complications, and detrimental effects on the psychosocial wellbeing of those affected, their families, and communities. Significant advances in the care of individuals with sickle cell disease over the past several decades, including newborn screening (NBS), penicillin prophylaxis, expanded immunisation programmes, and hydroxyurea therapy, have substantially reduced mortality among children and adolescents living in higher income countries. 2 Quinn CT Rogers ZR McCavit TL Buchanan GR Improved survival of children and adolescents with sickle cell disease. Blood. 2010; 115: 3447-3452 Crossref PubMed Scopus (619) Google Scholar , 3 Telfer P Coen P Chakravorty S et al. Clinical outcomes in children with sickle cell disease living in England: a neonatal cohort in East London. Haematologica. 2007; 92: 905-912 Crossref PubMed Scopus (237) Google Scholar Unfortunately, premature mortality from sickle cell disease during early childhood persists in LMICs, 4 Makani J Cox SE Soka D et al. Mortality in sickle cell anemia in Africa: a prospective cohort study in Tanzania. PLoS One. 2011; 6e14699 Crossref PubMed Scopus (191) Google Scholar where a majority of the global sickle cell disease burden is found. The clinical framework in LMICs is decades behind that of higher income countries, with most affected infants dying in the first years of life due to scarce access to an early diagnosis and available prophylactic or disease-modifying interventions. Despite multiple statements and calls to action from WHO to recognise and address the global burden of sickle cell disease, 5 WHO Regional Committee for AfricaSickle-cell disease: a strategy for the WHO African Region. World Health Organization, Geneva2010 Google Scholar there remains a crucial paucity of data in individual countries or across the highest burdened regions of the world to accurately quantify this problem and drive resource allocation to improve the diagnosis, management, and awareness of sickle cell disease. Global, regional, and national prevalence and mortality burden of sickle cell disease, 2000–2021: a systematic analysis from the Global Burden of Disease Study 2021Our findings show a strikingly high contribution of sickle cell disease to all-cause mortality that is not apparent when each death is assigned to only a single cause. Sickle cell disease mortality burden is highest in children, especially in countries with the greatest under-5 mortality rates. Without comprehensive strategies to address morbidity and mortality associated with sickle cell disease, attainment of SDG 3.1, 3.2, and 3.4 is uncertain. Widespread data gaps and correspondingly high uncertainty in the estimates highlight the urgent need for routine and sustained surveillance efforts, further research to assess the contribution of conditions associated with sickle cell disease, and widespread deployment of evidence-based prevention and treatment for those with sickle cell disease. Full-Text PDF Open Access

“If Pain Could Speak, What Would It Say?” Re-lational Inter/Intra-rogation of sickle cell disease: A Poetic (Inquiry) Insight.

If pain could speak, what would it say? If it could have a form, what would it look like? &#x0D; I start with these questions to illustrate the line of inquiry I am reframing as insight. The insights come from a conversation with a therapist warrior battling sickle cell disease (SCD). I use the language of therapist warrior for multiple reasons. To protect the anonymity of my conversational partner; to obscure her name but not her identity or infinite resourcefulness; to work with an archetype in a way that is congruent with the model of engagement I use within our conversations - Internal Family Systems (IFS), (Schwartz, 2013).&#x0D; I work with this model in my therapy practice. I draw on it in this paper to highlight the transformational power of externalising the pain experienced by the therapist I am conversing with. I will offer an overview of the model and an overview of the new language I am introducing in relation to methodology.&#x0D; IFS offers a different lens through which to view pain. Pain can be externalised as a “part”, no longer within the individual. This helps to empower the individual and their experience of pain.&#x0D; In this paper, I seek to highlight the resilience of the therapist I am conversing with and raise awareness of sickle cell disease. The paper actively examines the oppression and racialisation experienced by individuals with SCD from within the health care system. Care is contested.&#x0D; I use “re-lational inter-rogation/intra-rogation”, a methodology I am developing, to examine this. This way of engaging with conversational material highlights the importance of intentionally exploring relational changes after every re-connection or newfound acceptance between and within a group and connecting those insights with wider political forces.&#x0D; I use my embodied reflexivity for witnessing the impact, experiences, and happenings that occur during and after our dialogue. I illuminate my conversational partner’s resilience within the wider discourse of racialisation and marginalisation.&#x0D; Poems are transcribed from the conversation to add to the quality and aesthetics of the paper for the readers, as well as a reflexive process for me during and after my conversation with the therapist warrior. Poetry has a long history as a counter-narrative to the status quo and can be viewed as a decolonial, political act (van Rooyen and d’Abdon, 2020).

Knowledge and awareness of sickle cell disease among general population in Tabuk, Saudi Arabia

Knowledge and awareness of sickle cell disease among general population in Tabuk, Saudi Arabia

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.

Knowledge and awareness of sickle cell disease: a cross sectional study amongst unmarried adults in Nigeria’s capital city

Sickle cell disease is a genetic disorder characterised by the tendency of haemoglobin to polymerise and deform red blood cells to a sickle or crescent shape; this consequently results in vaso-occlusive condition. A better knowledge and awareness about sickle cell disease amongst the population can help reduce its prevalence. This study aimed at assessing awareness and knowledge of unmarried adults in Nigeria’s capital. A cross sectional survey was undertaken amongst unmarried individuals residing in the Federal Capital Territory. Questionnaires were administered to participants using convenience sampling strategy. Data were analysed using Statistical Package for Social Sciences version 25. Descriptive and inferential statistical analyses were carried out. A total of 1423 questionnaires were completed and returned, response rate was 83.71%, male participants were in the majority as indicated by 52% of the sample, and the dominant age group was 21 to 30 years (47.90%). Almost all the study participants (92.50%), have heard about sickle cell disease. Knowledge about sickle cell disease was average, as mean score for all the participants was 9.01 ± 3.18, with a range of 0 to 17. Some misconceptions were observed, for instance some participants believed that bacterial or viral infections could cause sickle cell disease. Male participants had a higher knowledge score compared to females (p < 0.001), and older participants were more knowledgeable about sickle cell disease (p < 0.001). This study identified that knowledge gaps exist about sickle cell disease. Emergent findings can underpin government, policymakers’ and stakeholders’ contextual strategies to prevent sickle cell through public health enlightenment and other relevant means.

Motivators and barriers to blood donation among potential donors of African and Caucasian ethnicity.

Minority blood donation, especially from individuals of African ethnicity, is a focus for many countries with diverse populations. As the need for antigen-negative RBC transfusions for patients with Sickle Cell Disease (SCD) continues to grow, inclusion of more African blood donors is essential to ensure this demand is met. This study aims to explore barriers and motivators to blood donation and awareness of SCD among potential donors of diverse ethnic backgrounds in Ireland. Following ethical approval, patients attending the National Sickle Cell Disease and Thalassemia service at St James's Hospital were invited to share an online anonymous survey within their local communities to achieve snowball-sampling. 387 respondents completed the survey, including 311 non-donors (median age 25 years, 67% female). Ethnic backgrounds included: African or African-Irish (59%), White or Caucasian (25%), Asian (8%), Hispanic or Latino (3%), Middle Eastern (3%), Multiracial or Biracial (2%). The most commonly identified barrier overall was lack of information on blood donation. African respondents were significantly more likely to report lack of information and malaria-related barriers than Caucasians. Motivators also varied across ethnic groups, with African respondents more likely to donate to help someone within their own community or for religious motivators. Awareness of SCD was higher among African respondents. While some barriers to blood donation are shared across all ethnic groups including lack of information, notable differences exist between Caucasian and African respondents. Specific actions to recruit and retain African blood donors should focus on these key areas.

Sickle cell disease: caregiver’s awareness and phenotype distribution among children presenting to children emergency of a tertiary hospital in Lagos, Nigeria

Background: Sickle cell disease accounts for significant morbidity and mortality in sub-Saharan Africa and the burden is expected to increase further by 2050. Nigeria is known to bear the highest burden of sickle cell disease in the world with about 2.69–5% of the population affected by the disease.Aim: This study determined awareness of sickle cell disease among caregivers and phenotype distribution of children presenting to children emergency in Lagos University Teaching Hospital (LUTH), Nigeria.Methods: The study was crosssectional and descriptive in design and data was collected using a pretested, structured intervieweradministered questionnaire among 250 caregivers and children. HemoTypeSC™ rapid test kit was used to determine the hemoglobin phenotype in whole blood of the respondents who were consecutively recruited following the caregiver’s consent. The Statistical Package for Social Sciences version 21 software was used for analysis.Univariate and bivariate analyses were carried out with a level of significance set at p ≤ 0.05.Results: The mean age of the children was 50.27±50.91 months. There were more females 141 (56.4%) than males. Almost all 242 (96.8%) caregivers did not know the children’s Hb phenotype. Most 173 (69.2%) of the children had HbAA;55(22.0%) were HbAS; 6(2.4%) were HbAC; 15 and 1 (6.0% and 0.4%) were HbSS and HbSCphenotypes respectively. Education was statistically significant with awareness of SCD (p=0.002) and awareness of SCD was statistically significant with knowledge of prevention (p&lt;0.001) among the caregivers.Conclusion: Awareness of SCD among the caregivers of children was high, although the majority of them did not know the children’s Hb phenotype. Most of the children had HbAA with a high proportion of HbSS and HbSC phenotypes. A routine neonatal/early infant screening program for SCD is highly recommended in Nigeria for early diagnosis and prevention of SCD complications.

Assessing and managing people with sickle cell disease presenting with vaso-occlusive crisis in the emergency department.

Sickle cell disease is a group of inherited blood disorders characterised by atypical haemoglobin molecule structures (haemoglobin S) which can manifest as painful vaso-occlusive crises, chronic anaemia and progressive organ damage. This article aims to raise greater awareness of sickle cell disease, its acute manifestations and the potential for rapid clinical deterioration that can occur in patients with this condition. The article outlines the main aspects of the assessment and management of patients presenting to the emergency department (ED) with vaso-occlusive crisis, describes the barriers to effective care that they commonly experience and explains how their care could be improved. The authors emphasise the importance of timely management of these patients by ED staff, including emergency nurses.

Advocating for and raising awareness of sickle cell disease in Sierra Leone

My name is Kadie Deen; I am 51 years old. I am a mother of a teenage boy named Elijah, an international advocate, an author, an entrepreneur, and founder of Sickle Smart Foundation. I have sickle cell disease and I was born in Sierra Leone.

Effect of health education on knowledge and awareness of sickle cell disease among adolescents

Introduction: Majority of Nigerians are not aware of their personal genotype, and the knowledge about sickle cell disease is quite low. We hypothesize that health education will improve the knowledge and awareness of sickle cell genotype among adolescents. This study aimed to determine the effect of health education on knowledge and awareness of sickle cell genotype among adolescents. Materials and Methods: Adolescent students of Federal Government College were recruited, and necessary data on sociodemography, knowledge, and awareness of sickle cell were obtained through a pretested sickle cell knowledge assessment questionnaire. Following health education, the same questionnaire was re-administered to the participants for assessment of any change in knowledge. Results: Majority (58.1%) of the respondents heard about sickle cell during lessons in class, while mass media was the next common source (16.5%) of knowledge and awareness. Following health education, there was an improvement in all the specific knowledge questions, especially the prevalence of sickle cell trait in Nigeria (28.1% vs. 75.2%) and the probability of carrier couple having a child with sickle cell anemia (SCA) (10.3% vs. 48.2%). There was a statistically significant increase in mean knowledge score (t = −14.203, P

Screening for the Sickle Cell Trait in Schools is more Efficient than during the Pre-Nuptial Period for the Prevention of Sickle Cell Anemia in a Sub-Saharan Africa Country. [Sickle Cell Trait Scree-ning and Sickle Cell Anemia Prevention in Sub-Saharan Africa

Sickle cell disease is a hereditary disease of hemoglobin which has been declared a public health priority by international health decision-making institutions. Among strategies to combat this disease, WHO recommends a reduction of births with sickle cell anemia through awareness and screening for the sickle cell gene in countries where the sickle cell gene is prevalent. Approaches to make this strategy efficient in Sub-Saharan Africa have not been studied. This study aim was to evaluate the efficacy of screening for the sickle cell gene for the primary prevention of sickle cell anemia among two target populations in a country of Sub-Saharan Africa. This was a prospective study comprising a free and voluntary screening for the sickle cell gene at various intervals, then to evaluate the impact of the screening on engagement or marriage decision-making on two target populations following raising awareness of sickle cell disease. Data was collected through a questionnaire administered at the headquarters of the NGO SS Assistance in Lubumbashi or by phone. 136 pupils with SCT and 111 married couples despite being screened for SCT during engagement were the subject of the study. In the target group of 136 SCT carriers (AS) in schools, 49 (48%) were married with an AA partner, 53 were engaged among whom 42 with an AA partner, 3 with an AS partner and 8 with a partner with an undetermined hemoglobin phenotype, 34 did not have any commitment. 91 (67%) pupils with SCT avoided a risk for SCA birth. Among the 111 couples married despite their screening as SCT carriers during pre-marital tests, 7 (6, 3%) have not registered SCA birth but 104 (93.7%) had 141 SCA births (extremes: 1 and 3 per couple). Sickle cell gene screening in a population without engagement or marriage plans is more productive than that conducted in the prenuptial period. However, results highlight the need to conduct more studies on sickle cell gene screening in Sub-Saharan Africa with the best strategies to reduce the psychological, social and economic burden of such studies especially when adults are targeted.

Sickle cell disease awareness among school children in Saudi Arabia

Background: Sickle cell disease (SCD) affects millions of people throughout the world. Previous studies suggested the need for more public education. This study aimed to assess and compare the knowledge about SCD among intermediate and high school students. Methodology: An analytic cross-sectional study was conducted among 1,500 high school and intermediate students in Al-Ahsa region, from December 2018 to February 2019. The data were collected through a questionnaire and interviews. Results: Most of the students (89%) had heard of SCD and 80% knew that it can be diagnosed by a blood test, but 60% did not know how common was SCD in Saudi Arabia. Despite the high prevalence, 65.4% answered the hereditary nature of the disease, but only a small proportion thought that consanguineous marriages were a risk factor for SCD spread. Females showed better knowledge than males and married people knew more about SCD than unmarried ones. Conclusion: There was a good level of knowledge about SCD. SCD awareness in secondary school educational program should be incorporated, and public awareness message in media is recommended.

The Assessment and Sustainable Management of Sickle Cell Disease in the Indigenous Tharu Population of Nepal

Sickle cell disease is an inherited hemoglobinopathy associated with significant morbidity and mortality. Reports suggest a high sickle cell disease burden among the indigenous Tharu population of Nepal, who for centuries have inhabited regions where malaria is endemic. Unfortunately, health care resources are limited and often inaccessible for Tharu individuals suffering from sickle cell disease. We conducted a large-scale screening effort to estimate the prevalence of Hb S (HBB: c.20A>T) among the Tharu population and delivered community-based education sessions to increase sickle cell disease awareness. A total of 2899 Tharu individuals aged 6 months to 40 years in the rural district of Dang in Western Nepal were screened using a sickling test, of whom, 271 [9.3%; 95% confidence interval (95% CI): 8.3–10.4%] screened positive for Hb S. Those who screened positive were offered diagnostic gel electrophoresis testing. Of the 133 individuals who underwent diagnostic testing, 75.9% (n = 101) were confirmed to be Hb AS heterozygotes, 4.5% (n = 6) were confirmed to be Hb SS homozygotes and 19.5% (n = 26) were false positives. These findings support a large burden of sickle cell disease among the Tharu population and highlight the importance of appropriate resource allocation and management. With a positive predictive value of 80.0% (95% CI: 73.0-87.0%), the sickling test in conjunction with raising local sickle cell disease awareness may be a simple and sustainable way to promote access to health resources.

Raising awareness of sickle cell disease for healthcare assistant practice: Nigerian perspectives

Raising awareness of sickle cell disease for healthcare assistants (HCAs) is important if they are to understand not only key aspects of the condition, but also if they are to communicate effectively with people and their families and carers who may be in the midst of acute illness or its potentially devastating aftermath. Sickle cell disorder (SCD) is a life-threatening genetic disorder due to abnormal haemoglobin. The condition is most prevalent in Africa. More than 10%-40% of African people carry sickle cell trait (SCT), leading to an estimated sickle cell prevalence of at least 2%. Nigeria has the largest population of people with SCD in the world. More than 25% of Nigerians are healthy carriers of SCT. Couples where both have SCT face a high likelihood of passing on the condition to their children. This explains why more than 150 000 babies with SCD are born in Nigeria every year. Having a working knowledge of the nature of the condition and its implications is useful for HCAs who may be working in areas of the UK with high percentages of Nigerian families in local communities, such as the south east of England. This article provides a holistic insight for HCAs into the condition and key considerations for everyday clinical practice when working with individuals or their families.