General Information About Sickle Cell Anemia
Disease Details and Frequently Asked Questions
It is a hereditary disorder where the oxygen-carrying hemoglobin protein found inside red blood cells is manufactured incorrectly (HbS) due to a genetic coding error.
Normal red blood cells are round and flexible. However, the red blood cells in this disease lose their elasticity and stiffen when deprived of oxygen or subjected to stress, taking on the shape of a 'sickle' (or crescent) used in agriculture.
If both the mother and father are carriers of the sickle cell trait, there is a 25% chance with each pregnancy that the child will be born fully affected by sickle cell anemia.
The rigid, sickle-shaped cells get stuck while passing through narrow capillaries, obstructing the blood vessel. Because the organ supplied by the blocked vessel (such as bone, chest, or spleen) does not receive oxygen, the patient experiences unbearable pain crises lasting for days, often requiring hospitalization.
It can be detected during newborn heel prick blood screening. In adults or children, definitive diagnosis is made by detecting the defective HbS protein via a 'Hemoglobin Electrophoresis' blood test.
During pain crises, analgesics and intravenous fluids are administered. To improve the patient's quality of life, hydroxyurea pills are used to prevent the cells from changing shape. The only curative method for the disease is Bone Marrow (Stem Cell) Transplantation from a compatible donor.
Our health library contents are prepared for informational purposes only and with scientific data available at the time of recording. For all your questions, concerns, diagnosis, or treatment regarding your health, please consult your doctor or a healthcare institution.
