SICKLE CELL DISEASE IN ORISSA,INDIA. ~ From A Clinician's Bioscope

Human red blood cells, like those of other mam...

Red blood cells(RBC) in blood are responsible for carrying 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 transforms the RBC in a oxygen deprived condition . It is hereditary 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 microscopic 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 conjunction 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

Micrograph of a piece of karan bagga's, studen...

Sickle cell RBC

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,anaemia 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.
Treatment 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 pregnancy.
Folate deficiency precipitates this condition as well as acidosis .So folate supplementation and avoiding oxygen deprived situation are two important steps .Prevention of infection by different vaccinations against infectious diseases and prophylaxis for malaria will enable the patient to lead a near normal life.