Sickle Cell Anemia Epidemiology of sickle cell disease in Saudi Arabia Sickle Cell Anemia 01 Sickle cell disease (SCD). 04 Symptoms of sickle cell anemia. 02 03 What is sickle cell disease? What are the risk factors for sickle cell disease? 05 06 Statistics. information from the scientific articles. Sickle cell disease (SCD) Sickle cell disease (SCD) was first described in 1910, in a dental student who presented with pulmonary symptoms. Herrick coined the term “sickle-shaped” to describe the peculiar appearance of the RBC of this patient. However, given the patient’s symptoms, he was not sure at the time whether the blood condition was a disease sui generis or a manifestation of another disease. In the next 15 years, several similar cases were described, supporting the idea that this was a new disease entity and providing enough evidence for a preliminary clinical and pathological description. Shortly thereafter, Hahn and Gillespie suggested that anoxia caused RBC sickling by demonstrating that shape changes could be induced by saturating a cell suspension with carbon dioxide What is sickle cell disease? Sickle cell disease is an inherited blood disorder. It is marked by flawed hemoglobin. That’s the protein in red blood cells that carries oxygen to the tissues of the body. So, sickle cell disease interferes with the delivery of oxygen to the tissues. Red blood cells with normal hemoglobin are smooth, disk-shaped, and flexible, like doughnuts without holes. They can move through the blood vessels easily. Cells with sickle cell hemoglobin are stiff and sticky. When they lose their oxygen, they form into the shape of a sickle or crescent, like the letter C. These cells stick together and can’t easily move through the blood vessels. This can block small blood vessels and the movement of healthy, normal oxygencarrying blood. The blockage can cause pain. Sickle cell is an inherited disease caused by a defect in a gene. A person will be born with sickle cell disease only if two genes are inherited—one from the mother and one from the father. What are the risk factors for sickle cell disease? Having a family history of sickle cell disease increases your risk for the disease. In the United States, it mainly affects African Americans. Symptoms of sickle cell anemia Stroke Splenic sequestration (pooling). Pain crisis, or sickle crisis. Jaundice, or yellowing of the skin, eyes, and mouth. Anemia Priapism. Statistics The population of Saudi Arabia is 23.98 million. Saudi Arabia has limited information about SCD prevalence, but studies have found that SCD is a fairly common genetic disorder in this country. A percentage of carriers with SCD ranged from 2% to 27%, and up to 1.4% had SCD in some areas. SCD is not screened for at birth, so these estimates of frequency are likely to be underestimated. The HardyWeinburg principle states that, except for specific perturbing influences, both allele and genotype frequencies in a population remain in equilibrium from generation to generation, after a nationwide survey of randomly collected blood samples. As consanguineou s marriage rates exceed 50%, autosomal recessive diseases are fairly common in this part of the world. It was estimated that the prevalence of sickle cell trait was 4.2% and SCD was 0.26% in the Saudi Premarital Screening Program, with the highest prevalence noted in the Eastern province (roughly 17% for sickle-cell trait and 1.2% for SCD). There is a disadvantage to premarital screening in that it is dependent on the incidence of disease at the time of marriage, individual survival patterns until the age of marri age, and disease severity. Therefore, this type of screening program underestimates the true prevalence of SCD, especially in the Western province, which has a more severe form. Over a nine-year period in the Eastern province, a regional screen for SCD revealed a prevalence of approximately 21% for sickle-cell trait and 2.6% for SCD (compared with 17% and 1.2% for premarital screening).Saudi Arabia possesses no information on the impact of SCD on under-five mortality and studies on patterns of mortality are scarce. The Eastern province's hospital-based studies, however, show that 73% of deaths occur under 30 years old, most commonly from acute chest syndrome followed by infection. information from the scientific articles. Information on impact of SCD on under-five mortality in Saudi Arabia is absent and studies on mortality patterns are limited. However, hospitalbased studies from the Eastern province show that 73% of deaths occur under the age of 30 years, with acute chest syndrome followed by infection as the major cause of deaths.26Neonatal diagnosis allows provision of simple protective measures, including information for the parents, prophylaxis with penicillin, hydroxyurea, and transfusion therapy, all giving a better quality of life.20–23 Neonatal diagnosis is useful only when there is appropriate counseling for the parents and adequate primary and follow-up care for those affected Conclusions Sickle cell illness is a long lasting condition. Although the difficulties of sickle cell infection will be unable to be forestalled altogether, carrying on with a sound way of life can decrease a portion of the crisis . Like eating good food e.x ( organic products, vegetables, protein... also, drink a heaps of liquids. There are factors that trigger an emergency model (cold weather , swimming in cool water..) Avoid infection by having a yearly influenza chance, cleaning up much of the time, keeping away from the individuals who are debilitated, and getting customary dental tests References ● Jastaniah, W. (2011, June 2). Epidemiology of Sickle Cell Disease in Saudi Arabia ... Annals of Saudi Medicine. Retrieved November 13, 2021, from https://www.annsaudimed.net/doi/10.4103/02564947.81540. Thank you. Student name: - Dina Hassan Tayyib. - Ghadeer Jaafar ALSheef. - Tahani Mesleh Alqahtani. - Zainab Ahmed Quraish. - Zainab Ali ALBaqer.