Sickle cell disease is caused by a genetic defect in the globin gene. Globin is required for the correct production of hemoglobin - the molecule that carries oxygen in our red blood cells.

SCTI is designing a cure for sickle cell disease first by taking a few blood stem cells from a patient with sickle cell disease. In these stem cells, the defective globin gene will be replaced by a healthy gene. In a second step these now healthy blood stem cells will be grown to large numbers and then given back to the patient. This way, the patient's own cells can now produce healthy globin and the patient no longer would suffer from the symptoms of sickle cell disease.

Stem cells have the ability to divide for indefinite Sickle Cell Disease is a group of inherited red blood cell disorders. Normal red blood cells are round like doughnuts, and they move through small blood tubes in the body to deliver oxygen. Sickle red blood cells become hard, sticky and shaped like sickles used to cut wheat. When these hard and pointed red cells go through the small blood tube, they clog the flow and break apart. This can cause pain, damage and a low blood count, or anemia.meaning that its potential is total. In the first hours after fertilization, this cell divides into identical totipotent cells.

Most States now perform the sickle cell test babies are born. The simple blood test will detect sickle cell disease or sickle cell trait . Other types of traits that may be discovered include:

Hemoglobin C trait

Hemoglobin E trait

Hemoglobin Barts -
which indicates an alpha thalassemia trait

Beta thalassemia trait

What is sickle cell trait?

Sickle cell trait is a person who carries one sickle hemoglobin producing gene inherited from their parents and one normal hemoglobin gene. Normal hemoglobin is called type A. Sickle hemoglobin called S. Sickle cell trait is the presence of hemoglobin AS on the hemoglobin electrophoresis. This will NOT cause sickle cell disease. Other hemoglobin traits common in the United States are AC and AE traits.

Are there different types of sickle cell disease?

There are three common types of sickle cell disease in the United States.

1. Hemoglobin SS or sickle cell anemia
2. Hemoglobin SC disease
3. Hemoglobin sickle beta-thalassemia

Each of these can cause sickle pain episodes and complications, but some are more common than others. All of these may also have an increase in fetal hemoglobin which can protect the red cell from sickling and help prevent complications. The medication hydroxyurea also increases fetal hemoglobin.

What makes the red cell sickle?

There is a substance in the red cell called hemoglobin that carries oxygen inside the cell. One little change in this substance causes the hemoglobin to form long rods in the red cell when it gives away oxygen. These rigid rods change the red cell into a sickle shape instead of the round shape.

How can I be tested?