What is sickle cell anemia??
Normal red blood cells have a biconcave shape that guarantees their pliability. The human body has blood vessels of different diameters; and the pliability with a biconcave shape of an RBC, makes it easy for them to pass through the narrowest vessels (like capillaries with a diameter less than an RBC itself) easily.
Sickle cell disease is a disease of RBCs that gives them the shape of a sickle. This debilitating genetic disease renders the sufferer with RBCs of crescent shape that lose their flexibility and become sticky and stiff.
Usually, even the affected people carry normal RBCs in their blood. As the normal oxygen concentration of the body falls due to certain reasons (e.g moving towards high altitude), the blood cells lose their normal shape and resemble a crescent.
So why do RBCs acquire a crescent shape? It’s because they have a defective hemoglobin which in low levels of oxygen forms solid chains in RBC that resemble a crescent with poor oxygen carrying and circulating ability.
The disease comes when both the parents give their defective genes to the offspring. One defective gene will only give what’s called a “sickle cell trait”
Signs and symptoms:
– Anemia (with associated symptoms)
– Delayed growth
– Vision problems
– Enlarged spleen
– Frequent infections
– Severe sudden abdominal pain
– Hand and feet swelling
– Shortness of breadth
The sickle cells block blood vessels and cause decreased blood flow which means damage, to the organ.
Mainly, oxygen therapy.
Hydroxyurea- a drug that stimulates production of fetal hemoglobin prevents sickling.
Pain killers and antibiotics.
A bone marrow transplant may also work.