Sickle Cell Disease is an inherited blood disorder that affects the red blood cells. Normal red blood cells are round and shaped like a donut. They carry oxygen and nutrients throughout the body. Sickle cells, however, are abnormally shaped and cannot carry oxygen well, and they have a shorter life span than normal red blood cells. As a result, anemia is constantly present. Sickle Cells are rigid and their abnormal, “sickle” shape causes them to clog in the blood vessels preventing the delivery of oxygen to tissues and organs. The anemia and obstruction of blood flow are the cause of the many health problems associated with Sickle Cell Disease. 

The most common and impactful feature of the disease is the occurrence of pain attacks caused by the sickle cells clogging the blood vessels. These attacks may occur at any time and in any part of the body and are of varying severity and duration. Sickle Cell Disease also causes fatigue, higher risk of infections, stroke, necrosis, loss of appetite, and other serious manifestations throughout the body and organ systems. 

Bone marrow transplant is the only known cure for sickle cell. However, not all sickle cell patients may qualify for this treatment. The drug Hydroxyurea is now being widely used as a treatment for sickle cell and has had a beneficial effect for many sickle cell patients. In 2017, Endari was approved as the second pharmaceutical product for the disease.  2019 saw two additional drug therapies for the disease as well, Adakveo and Oxbryta.  Conventional treatment consists of managing the health problems as they occur.  New gene therapies are now under investigation and providing additional hope for the Community.

Persons who carry the gene for Sickle Cell Disease have what is known as Sickle Cell Trait. Persons with Sickle Cell Trait are healthy and rarely have physical manifestations of the disease except under very extreme conditions. Persons wanting to have children should know their sickle cell status. If two people who have sickle cell trait conceive a child, they have a one in four chance of having a child with Sickle Cell Disease.