Sickle cell disease (SCD) is an inherited blood disorder that affects red blood cells. The normal red blood cell contain mostly hemoglobin A but in the individual with SCD, hemoglobin* S (an abnormal type of hemoglobin) is also found in variable fraction along with HbA . These red blood cells may become sickle-shaped (crescent shaped) under variable circumstances like dehydration, infection, stress or pregnancy etc.
often includes antibiotics, pain management, intravenous fluids, blood transfusion and many at times needs comprehensive multi-disciplinary care. Life style modification and psychosocial support plays an important role. In general, to avoid complications, penicillin prophylaxis, vaccination against pneumococcus and folic acid supplementation needs to be added.
The defective gene from the carrier parents ( carrying one defective gene) are transmitted to their offspring. If one defective gene is inherited from either of one parent, then it is a carrier (50% chances) and if two defective genes are transmitted from both the parents, then it will manifest with disease ( 25% chances). But there is also 25% chances to inherit normal genes from both parents, then it is neither a carrier nor a disease. At present, in unawareness, several combinations are seen occurring at the social level. Fig.2, Therefore, in outcome, the number of disease sufferers are increasing day by day.
