The Campaign to control Genetic Blood Diseases in Bahrain

In Bahrain and neighbouring countries inherited disorders of haemoglobin, i.e. sickle-cell disease, thalassaemias and glucose-6-phosphate dehydrogenase (G6PD) deficiency, are common. As part of the National Student Screening Project to determine the prevalence of genetic blood disorders and raise awareness among young Bahrainis, we screened 11th-grade students from 38 schools (5685 students), organized lectures and distributed information about these disorders. Haemoglobin electrophoresis, high performance liquid chromatography, blood grouping and G6PD deficiency testing were performed. Prevalences were: 1.2% sickle-cell disease; 13.8% sickle-cell trait; 0.09% -thalassaemia; 2.9% -thalassaemia trait; 23.2% G6PD deficiency; 1.9% G6PD deficiency carrier. Health education, carrier screening and premarital counseling remain the best ways to reduce disease incidence with potentially significant financial savings and social and health benefits.

Annals of Saudi Medicine, 2010

BACKGROUND AND OBJECTIVES:Previous studies that have assessed patient awareness of the management of sickle cell disease (SCD) indicated a lack of awareness of the disease and possibly a need for more public education. Therefore, we measured public awareness in Bahrain of SCD.METHODS:The study was conducted from December 2006 to February 2007. A questionnaire was distributed among 2000 persons selected from among the general public. The participants had face-to-face interviews with either a health professional or a trained interviewer.RESULTS:Most (93%) had heard of SCD and 89% knew that it can be diagnosed by a blood test, but 51% did not know the prevalence of SCD in Bahrain. Eighty-four percent recognized it as a hereditary disorder and 72% said that it can skip generations. Females showed better knowledge than males and married persons seems to know more about SCD than unmarried ones.CONCLUSION:There is a good level of knowledge about SCD among the public, though some of the respondents were confused about the difference between the carrier state of a disease and the disease itself. There is wide acceptance and appreciation of the SCD prevention campaigns being conducted in Bahrain, such as the premarital service and the student screening program.

‫هايحس‬ ‫وويفا‬ ‫العريط،‬ ‫سالم‬ ‫شيخة‬ ‫الىراثية‬ ‫الهيمىغلىبيه‬ ‫اظرابات‬ ‫البحريه‬ ‫في‬ ‫جىحشر‬ . ‫جبلغ‬ ‫مجمىعة‬ ‫علً‬ ‫دراسة‬ ‫أجريىا‬ ‫ولقذ‬ 56198 ‫ب‬ ً ‫مىطىا‬ ‫يقيمىن‬ ً ‫حريىيا‬ ‫أن‬ ‫ووجذوا‬ ‫المسحشفي،‬ ‫وطاق‬ ‫في‬ 2 % ‫وأن‬ ‫المىجلية،‬ ‫النريات‬ ‫بمرض‬ ‫مصابىن‬ ‫المىاليذ‬ ‫مه‬ 18 % ‫المىجليةة،‬ ‫لة‬ ّ ‫الخ‬ ‫لذيه‬ ‫مىهم‬ ‫مان‬ ‫بيىما‬ 24 % ‫األلفا‬ ‫الحالسيمية‬ ‫جيىات‬ ‫يحملىن‬ . ‫أن‬ ‫لىةا‬ ‫جبةيه‬ ‫البحةريىييه‬ ‫بةيه‬ ‫المىجليةة‬ ‫النريةات‬ ‫مرض‬ ‫طبيعة‬ ‫دراسة‬ ‫ولذي‬ ‫المرض‬ ‫مه‬ ‫الخفيف‬ ‫الشنل‬ ‫الىطاق‬ ‫واسعة‬ ‫سريرية‬ ‫اخحالفات‬ ‫هىاك‬ ‫ماوث‬ ‫وإن‬ ‫الغالب،‬ ‫هى‬ . ‫فةيهم‬ ‫الةذمىيات‬ ‫َةيم‬ ‫ق‬ ‫أن‬ ‫وجذوا‬ ‫مما‬ ‫المرض‬ ‫مه‬ ‫الخفيف‬ ‫الشنل‬ ‫اوحشار‬ ‫يغلب‬ ‫حيث‬ ‫السعىدية،‬ ‫العربية‬ ‫بالمملنة‬ ‫الشرقية‬ ‫المىطقة‬ ‫مرظً‬ ‫لذي‬ ‫الحي‬ ‫لحلل‬ ‫مماثلة‬ .

‫الثحشٌٗ٘٘ي‬ ‫لذٓ‬ ‫الوٌجلٖ‬ ‫الذم‬ ‫فقش‬ ‫طث٘عح‬ ‫هي‬ ‫للرحقق‬ ‫الذساسح‬ ‫ُزٍ‬ ‫أجشٗد‬ . ‫ّاسع٘ي‬ ‫قسو٘ي‬ ‫الٔ‬ ‫الذساسح‬ ‫ُزٍ‬ ‫قسود‬ ‫ّقذ‬ ‫ّصع‬ ‫ح٘ث‬ ‫هشرشك‬ ‫ذٌظ٘ن‬ ‫راخ‬ ‫جواعح‬ ‫علٔ‬ ‫ٗعروذ‬ ‫األّل‬ ‫قثل‬ ‫هي‬ ‫لوثلَ‬ ‫اسرث٘اى‬ 011 ‫الوٌجلٖ‬ ‫الذم‬ ‫تفقش‬ ‫هصات٘ي‬ ‫طالة‬ ‫اح‬ ‫هشاجعا‬ ‫ذواد‬ ‫اث‬ ‫ح٘ا‬ ‫افٔ‬ ‫الوسرشا‬ ‫هشفأ‬ ‫أ‬ ‫علا‬ ‫ٗعرواذ‬ ‫ااى‬ ‫فيا‬ ‫الثااًٖ‬ ‫ان‬ ‫القسا‬ ‫أهاا‬ ، 01 ‫اذم‬ ‫الا‬ ‫اش‬ ‫تفقا‬ ‫هصاات٘ي‬ ‫أ‬ ‫لوشفا‬ ‫هلا‬ ‫الوسرشفٔ‬ ‫الٔ‬ ‫ادخلْا‬ ‫قذ‬ ‫كاًْا‬ ‫الوٌجلٖ‬ . ‫للثاشد‬ ‫الرعاش‬ ُٖ ‫االّل‬ ‫القسن‬ ‫فٖ‬ ‫الوؤدٗح‬ ‫الشئ٘س٘ح‬ ‫العْاهل‬ ‫أى‬ ‫ّجذًا‬ ‫ّقذ‬ ‫ّاالسُاا‬ ‫ّاالًراًاخ‬ ‫ّالحؤ‬ . ‫ّفاْحا‬ ‫االعاشا‬ ‫اكثاش‬ ‫هاي‬ ‫ُاٖ‬ ‫ّااللان‬ ‫الحوأ‬ ‫ّكاًاد‬ . ‫ّٗعرقاذ‬ 01 % ‫ُاؤال‬ ‫هاي‬ ‫جاذا‬ ‫اساس٘ح‬ ‫الحاالخ‬ ‫ُزٍ‬ ‫فٖ‬ ‫الوشْسج‬ ‫اى‬ ‫الوشفٔ‬ . ‫فاٖ‬ ‫الشئ٘سا٘ح‬ ‫االعاشا‬ ‫كاًاد‬ ‫فقاذ‬ ‫الثااًٖ‬ ‫القسان‬ ‫أهاا‬ 29 % ‫هاي‬ ‫الوجاس‬ ‫فٖ‬ ‫ّالرِاب‬ ‫الصفشاّٗح‬ ‫اليثذ‬ ‫ّفخاهح‬ ‫تطٌ٘ح‬ ‫آالم‬ ُٖ ‫الحاالخ‬ ‫الثْل٘ح‬ ٕ .

2006

Haemoglobin electrophoresis screening of 2341 infants from the oases of eastern Saudi Arabia, performed in an attempt to detect cases early and then to follow up and give better management to patients with sickle cell disease, showed 20% with S-trait and 43 with sickle cell disease (37 HbSS and 6 S-r° thalassaemia). On follow-up from birth (or from 3 months) for a mean of 31 years there was more morbidity and mortality than in matched Saudi Arab controls, but these rates were lower than for affected black infants in the US or Jamaica. Serial haemoglobin levels were

Journal of Epidemiology and Global Health, 2017

Nursing & Primary Care, 2019

Background: Sickle cell disease takes an exceptional place among the most common inherited disorders in Saudi Arabia, remarkably in the Eastern Province of the Kingdom. Understanding the disease and its effects as a result of consanguineous marriages is critical. The level of perception among the Saudi population regarding genetic testing and screening needs to be measured. Therefore, the main objectives of this study were to assess the current health knowledge and beliefs of the Saudi population in Al-Ahsa regarding Sickle cell disease and Sickle cell trait, and how this population perceived genetic testing and screening program of sickle cell disease and sickle cell trait in order to establish baseline data. Methods: This is a cross-sectional descriptive study of 205 Saudi subjects who attended the "Health Matters Seminar 2" (January 7-11, 2016). The subjects were selected by a purposive sampling technique, using a selfadministered structured questionnaire. Results: The study cohort comprised 205 Saudi subjects (32% male and 68% female) ranging in age between 10-60 years old. The subjects were interviewed and more than half (62.9%) had limited knowledge on how sickle cell disease and sickle cell trait are inherited. Conclusion: There is a need to raise awareness on how sickle cell disease and sickle cell trait are inherited. In addition, there is a need to provide genetic counseling for the Saudi population in Al-Ahsa as part of the premarital screening (PMS) program.

Kashmir J Med Sci, 2015

Background: Inherited haemoglobin disorders (haemoglobinopathies) are predominantly single-gene autosomal recessive disorders that result in production of structurally abnormal haemoglobin variants (sickle-cell disease) or a reduction in the synthesis of structurally normal globin (β-thalassemia). The objective of this study was to see the prevalence of thalassemia and sickle cell disease in Northern Border Region of Saudi Arabia and to counsel the couples for pre-marital screening of blood to avoid the disease. Methods: This was retrospective study carried out in the Department of Physiology, College of Medicine, Northern Border University with Collaboration of Central Hospital Arar, during Muharram to Zulaghaeda 1434 H. We collected data of 23,522 male and female patients who visited the Central Hospital Arar, Marriage Center of Northern Border Region in Arar during 1425 H to 1431 H; where the couples were counselled for pre-marital screening of blood to avoid the disease. Results: Prevalence of haemoglobinopathies in 1425 H was 2.8% compared to the prevalence of haemoglobinopathies in 1430 H (0.4%). Prevalence of sickle cell carriers was 44.75% in 1427 compared to prevalence in 1427 H (5.4%). Prevalence of β-Thalassemia was (92.7%) in 1425 H compared to prevalence in 1427 H (55.3%). Conclusion: The cases of haemoglobinopathies are present in our area. Premarital screening and genetic counselling markedly reduced the number of atrisk marriages, which may considerably reduce the genetic disease over the coming decades. Keywords: β-thalassemia, Sickle Cell Disease, Saudi Arabia, haemoglobinopathies, autosomal recessive disorders

This paper looks at some of the studies on genetic disorders conducted in Bahrain. The disorders coverd include: genetic blood disorders, metabolic disorders, chromosomal disorders, including Down syndrome, and cystic fibrosis. The rate of consanguinity in Bahrain and the results of premarital counseling are also discussed.

Background: genotypic analysis in Bahraini SCD patient has not been investigated. Aim: To investigate gene or allele frequency have any effects on SCD patients compared to controls. Methods: Alkaline Phosphatase VDR and PTH genes were investigated using real Results: Genotypic analy mutant allele) among both SC patients and controls. AG genotypes was more frequent than the GG genotypes in patient and controls (p<0.012). The percentage of allele A frequency was 6 66.1% within patient and controls, respectively with no statistical difference. Yet, the analysis showed no association within sex between genotypes and allele's frequency. In the PTH gene, the GG genotype was more frequent than the AG genotype in of A allele was 34.3% in patients and 25.8% in controls, but the difference was not significant. No association was found between GG genotype in sex and allele frequency and sex in PTH gene. Conclusion genotype is associated with SCD. There was no statistical difference in allele's frequencies between patients and controls in both VDR and PTH genes. Moreover, no association and PTH gene polymorphisms and their corresponding serum levels suggesting that allele A may be associated with vitamin D serum level.