Have you ever heard of sickle cell disease (SCD)? A single amino acid mutation occurs in hemoglobin which causes an unusual shape in the red blood cells. The disorder leads to “sickled-shaped” red cells. It is an inherited blood disorder. Not many people are familiar with SCD.
Hemoglobin carries oxygen in blood. In sickle cell, hemoglobin levels are abnormally high. It also lets the sickle cells die early. So patients experience a constant shortage of red blood cells, causing sickle cell anemia (SCA). Another problem with sickle cells is that the cells can easily get stuck in the vessel which blocks blood flow due to the crescent moon-shaped cells. Many factors, temperature change, exercising passing maximum capacity, or dehydration may lead to a sickle cell crisis.
The only FDA-approved approach for treating SCA is a bone marrow transplant. It helps restore the damaged stem cells to healthy cells. It may lead to dangerous infections and other side effects. Only young people with severe cases of SCA are suggested by healthcare providers with this treatment. Several medications are used to treat SCA such as Hydroxyurea, L-glutamine oral powder (Endari), Crizanlizumab (Adakveo), and Voxelotor (Oxbryta). So SCA is often treated by taking medications and getting periodic blood transfusions. It is recommended to stay hydrated, keep a nutritious diet, and sleep for eight hours. Most of all, take medications consistently even if you feel OK.
The World Sickle Cell Awareness Day is June 19. It is observed annually to increase public knowledge of SCD.