What is a Sickle Cell Anemia disease?

World Sickle Cell Awareness Day is celebrated on June 19th of each year to raise the public awareness about the sickle cell disease and its treatment methods.

Sickle cell anemia, which affects millions of people around the world, is a serious  life threatening disease which has negative effects on the quality of life. Dr. Suat Günsel University of Kyrenia Hospital Internal Medicine Specialist Züleyha Özer Yazgan provide important information about disease:

First of all, what is a Sickle Cell Anemia Disease?
In sickle cell disease, a genetic mutation occurs in the structure of hemoglobin in the red blood cells(the oxygen-carrying protein found in red blood cells), and glutamic acid is replaced by valine . This seemingly simple change causes the formation of abnormal hemoglobin called hemoglobin S. Due to low-oxygen environment the red blood cells distort into a sickle shape.  These irregularly shaped cells can get stuck in small blood vessels.  It may lead to blood clotting.

What are the symptoms?
Some of the symptoms are episodes of pain, anemia, severe lung infection,  ischemic priapism splenic sequestration which is extremely serious. Therefore it must be carefully monitored. In time it may  lead to delayed growth, bone and joint diseases, central nervous system related disorders, cardiovascular diseases, respiratory system diseases, digestive system diseases, eye problems and leg ulcers.

How is this disease diagnosed?
The first diagnosis of the disease is made primarily by the health history provided by the patient. This method is followed by a peripheral blood smear and hemoglobin electrophoresis, and DNA anaylysis can be used to diagnose the disease.

What are the treatment methods?
It is very important to prevent infection and to take preventive measures for patients diagnosed with sickle cell anemia. It is essential to use preventive antibiotics in children, in addition to the Pneumococcal vaccine, Haemophilus influenzae vaccine, influenza vaccine and Meningococcal vaccine, as well as taking plenty of fluids, and the required amount of folic acid and zinc supplements, paying attention to dental health and following up periodic examinations by a pediatric physician. The only cure available to patients with sickle cell disease is stem cell transplantation. which provided to patients who meet a certain criteria.”