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The recurrent crises frequently cause absenteeism from school and work, eventually leading to school dropouts and job losses. This is made worse by the limited and often inaccessible formal social support structures that can help patients and families cope better with the psychosocial burden of SCD. Periodic crises, the direct and indirect costs of frequent hospitalizations, the economic burden of SCD, and the early deaths that sometimes occur can lead to poor family relationships. Crises are often precipitated by dehydration, infection, exposure to extremes of temperature (fever or cold exposure), and hypoxia.
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Severe pain is the most common manifestation of sickling crisis. Sickling crisis results from episodes of increased red blood cell sickling and capillary sludging causing poor organ perfusion. Hb SC patients tend to have a higher steady state hemoglobin level of about 11g/dl but more frequent tissue infarctions (e.g. In the “stable state”, most patients with Hb SS have a steady state hemoglobin level of 8g/dl. Clinically, the disease is characterized by chronic hemolytic anemia interspersed with episodes of acute vaso-occlusive sickling crises. The resulting microcirculatory obstruction gives rise to vaso-occlusive crises which may occur repeatedly in the presence of certain triggers. The polymerized hemoglobin reduces red blood cell deformability and causes “sickling” of erythrocytes within capillaries and end-arterioles. Sickle cell anemia is a severe recessive genetic disorder the homozygous state results in an abnormal hemoglobin that is prone to polymer formation under cellular deoxygenation. Like many other chronic diseases, SCD has a huge psychosocial burden. We believe such an approach is cost-effective, and consistent with sound ethical principles and good conscience. We emphasize the critical importance of self-management, especially self-awareness, in assuring a good quality of life for persons with SCD. In this review, we recommend control of SCD on three levels – preconception genetic testing and strategic reproductive choices, PND and education for carrier parents, and holistic management of persons with SCD. In the light of the demonstration of improved prognosis in recent times, PND and selective abortion appears to be applying capital punishment to the unborn child for “crimes” only the parents can be responsible for. PND and selective abortion therefore raises a myriad of ethical dilemmas which are considered in this review. Advances in bone marrow transplantation have shown the disease is curable in selected patients. In the current era, SCD has a good prognosis and fairly reasonable quality of life. As a means of controlling the disease, some authorities have recommended prenatal diagnosis (PND) and selective abortion. Recent studies indicate that 2% of Ghanaian newborns are affected by SCD one in three Ghanaians has the hemoglobin S and/or C gene. Sickle Cell Disease (SCD) is a significant public health burden in Ghana.