SC disease, an autoimmune recessive condition, is an inherited abnormality of red blood cells. Affected children inherit two copies of an abnormal hemoglobin gene, one from each parent. For couples where both individuals carry one copy of the abnormal gene, described as having SC trait or being a carrier for SC disease, there is a 1 in 4 chance of future children being affected by the disease (?) From a biomedical perspective, a high potential for benefit from sharing research-generated SC disease findings stems from a positive health impact of comprehensive forms of health care. Without care, symptoms can be very severe and life threatening, mainly resulting from obstruction to small blood vessels, chronic anemia, acute breakdown of blood cells and increased risk of serious infection. Although environmental and genetic factors influence severity, without care many children in malaria endemic settings are likely to die in their first few years of life. In contrast, quality of life is significantly improved where comprehensive care programs are in place, typically in high-income settings, leading to a median adult survival of 48 years SC trait is generally seen as a benign condition whose main implication is an increased future reproductive risk for the disease. The disease originated in at least 4 places in Africa and in the Indian/Saudi Arabian subcontinent. It exists in all countries of Africa and in areas where Africans have migrated .It is most common in West and Central Africa where as many as 25% of the people have sickle cell trait and 1-2% of all babies are born with a form of the disease. In the United States with an estimated population of over 270 million, about 1,000 babies are born with sickle cell disease each year. Approximately 70,000 - 100,000 individuals in the United States have sickle cell disease and 3 million have sickle cell trait. In contrast, Nigeria, with an estimated 1997 population of 90 million, 45,000-90,000 babies with sickle cell disease are born each year. The transatlantic slave trade was largely responsible for introducing the sickle cell gene into the Americas and the Caribbean. However, sickle cell disease had already spread from Africa to Southern Europe by the time of the slave trade, so it is present in Portuguese, Spaniards, French Corsicans, Sardinians, and Sicilians, mainland Italians, Greeks, Turks and Cypriots. Sickle cell disease appears in most of the Near and Middle East countries including Lebanon, Israel, Saudi Arabia, Kuwait and Yemen. Sickle Cell has also been reported in India and Sri Lanka. Sickle cell disease is an international health problem and truly a global challenge. The most common genotype is homozygous SS disease. S-Hemoglobin C (SC) disease, Sβ+ thalassemia and Sβ0 thalassemia are also relatively common and is a result of migration of people originating from these regions. People who carry just one βS mutation have the sickle cell trait (HbAS), and are generally asymptomatic. However there is emerging data which suggest that having sickle cell trait may be associated with increased risk for adverse health outcomes such as venous thrombosis and obstetric complications. The frequency of the sickle cell trait (AS) has been estimated is as high as 25% in Riyadh, Saudi Arabia; 31% in Parthenon, Greece; 38% in Southeast India and 40% in Central Africa which reports that each year about 300,000 infants are born with a hemoglobinopathy, 200,000 of whom are born in Africa (World Health Organization 2006). Whereas SCD occurs in 1 in 500 African-American births, in Jamaica it occurs in 1 in 150 births. Most countries do not have exact figures for SCD prevalence, but estimated suggest that there are 12,000 persons with SCD in United Kingdom (Streetly A et al. 1997) and about 72,000 in the United States (Bonds 2005).
In assessing the seriousness of this disease, no one should underestimate its emotional and social impact. The patient endures not...
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