Red blood cells(RBC) in blood are responsible for carriny oxygen to the cells and bring out carbondioxide from there.The protein iron complex in it called haemoglobin is the carrier of oxygen and carbondioxide.Normal hemoglobin is termed as HbA.But many abnormalities do occur.One is HbS ( Sickle haemoglobin) derived the name from sickle as it tramsforms the RBC in a oxygen deprived condition . It is hereditiry and mostly circulates in a closed area and consanguineous marriage spreads it in a local population.Western Orissa is the storehouse of this disease.
RBC is a mocroscopic cell of discoid shape and is biconcave.This means if a RBC will be likened to a coin the sides of coin should cave in to resemble a RBC.This shape facilitates it to deform while passing through a narrow road inside the smallest calibered blood vessel called capillary.This quality lacks in a RBC containing HbS.It may sometimes occurs in conjuction with other abnormal type of Hb such as HbF which is the cause of Thalassaemia.So what happens when these RBCs negotiate through a capillary and delivered
oxygen to cause a oxygen deprived environment where these get deformed to a sickle shape and become rigid to conglomerate with each other blocking the blood flow causing more oxygen deprived environment to form more sickles and the cascading effect.The blocked site hampers blood flow causing death of cells beyond the segment.So commonly this effect is seen in spleen,bone marrow,lungs,kidneys and liver etc. This causes various symptoms such as pain arising from the organ,blood in urine,anemia and infection of bone and lungs etc.
This can be screened by sickling test in a oxygen deprived environment and conformed by electrophoresis of haemoglobin.
Tretment is not very satisfactory.It can be prevented by marriage counselling and diagnosis of condition in utero where the parents may like to discontinue the pregnacy.
Folate deficiency precipitates this condition as well as acidosis .So folate suplimentation and avoiding oxygey deprived situation are two important steps .Prevention of infection by different vaccinations against infectious diseases and prophylaxis for malaria will eaable the petient to lead a near normal life.
Marriage counselling should be based on blood matching tests and not merely basing on horoscopes.