SICKLE CELLS: THE ODD-SHAPED KILLERS THAT GET STUCK IN VEINS, BLOCKING BLOOD FLOW TO VARIOUS TISSUES IN THE

BODY
Sickle cell disease is an inherited blood disorder characterized by defective haemoglobin. It causes unbearable pain, organ damage and early death. Normal haemoglobin cells are smooth and round which eases their movement through tiny blood vessels. Sickle cell haemoglobin molecules are stiff and sickle-shaped which hampers their mobility through blood veins. Unlike normal haemoglobin cells whose lifespan is up to 120 days, sickle cells die after just about 10 to 20 days, resulting in a chronic shortage of red blood cells and anaemia. Due to their defective nature sickle cells easily get stuck in blood veins, leading to clustering and reduced blood flow to various tissues in the body. Clustering in the arms, legs, chest or abdomen is usually registered as strong bouts of pain referred to as sickle cell crisis or pain crisis. If the vessel blockage happens in the brain it can cause a stroke. Studies indicate that strokes affect about one in every ten children who have sickle cell disease. Parents with children two years or older suffering from this ailment are advised to take them for regular tests to determine the level of stroke risks. Signs of strokes in children include weak limbs, slurred speech, failure to walk plus any other unusual behaviour. Another common problem children suffering sickle cell disorder is spleen infection. Located in the upper left area of the abdomen the spleen is designed to filter out abnormal red blood cells and helps the bodys immune system in fighting infections. In the case of sickle cell patients, sometimes red blood cells are trapped in the spleen, called splenic sequestration, which can lead to the organs enlargement and damage. By the age of eight most children with acute sickle cell do not have a functioning spleen either from surgical removal or from repeated episodes of splenic sequestration. This opens the doors for infections which is the major cause of death in children with this condition under the age of five years. Other sickle cell symptoms in children include swollen limbs, yellowing of skin and eyes, swollen abdomen, painful and persistent erections [priapism], gallstones, fatigue, breathing, hearing and seeing problems, paleness of skin and nail beds and chronic chest infections. Bacteraemia, presence of bacteria in the blood, is also a common cause of infections among patients.

Although there is no cure for sickle cell early detection increases the effectiveness of treatment. Testing through a laboratory technique called Haemoglobin Electrophoresis differentiates between normal haemoglobin [A] and sickle haemoglobin [S]. The highest number of sickle cell cases in both adults and children has been registered among people originating from Africa, Central and South America, Caribbean, Mediterranean, Asia regions. Early diagnosis and prevention of complications is critical in sickle cell disease management. Among the most common treatment methods include pain medications, drinking plenty of fluids, blood transfusion, penicillin, folic acid bone marrow transplants, administering hydroxyurea and in some cases bone marrow transplant. Although early detection is one of the most effective methods of fighting this disease 90 percent of children in sub-Saharan Africa with sickle cell anaemia, the common type of this blood disorder, die before the diagnosis can be made. Sickle cell is a blood condition seen mostly recorded among people from Africa, India, Middle East and the Mediterranean regions.