Sickle Cell Disease/Disorder

Sickle Cell Is A Disorder of the Hemoglobin in the Red Blood Cells.

Hemoglobin is the substance in red blood cells that is responsible for the color of the cell and for carrying oxygen around the body.

People with sickle cell disorder are born with the condition, it is not contagious. It can only be inherited from both parents each having passed on the gene for sickle cell.

The main symptoms of sickle cell disorder are anaemia and episodes of severe pain. The pain occurs when the cells change shape after oxygen has been released. The red blood cells then stick together, causing blockages in the small blood vessels.

These painful episodes are referred to as sickle cell crisis. They are treated with strong painkillers such as morphine to control the pain.

People with sickle cell are at risk of complications stroke, acute chest syndrome, blindness, bone damage and priapism (a persistent, painful erection of the penis).

Over time people with sickle cell can experience damage to organs such as the liver, kidney, lungs, heart and spleen. Death can also result from complications of the disorder. Treatment of sickle cell mostly focuses on preventing and treating complications.

The only possible cure for the disorder is bone marrow transplant but this is only possible for a limited number of affected individuals who have a suitable donor. A medicine called Hydroxyurea, can significantly reduce the number of painful crises.

Most people with Sickle Cell Trait usually don't have any symptoms of Sickle Cell Disease.

If you have the trait, the majority of red cells in the blood are normal round shaped cells. Some sickle shaped cells may be present under certain conditions.

Most people who have sickle cell trait are healthy. However, anesthetics can cause problems. If you have sickle cell trait Always notify your dentist or doctor before treatment commences to be on the safe side.

There is a small chance that you may experience pain at high altitudes (generally above 10,000 feet), including long-haul flying in unpressurised planes and mountain climbing. It is important you say you have sickle cell trait before undertaking such activities as you may need to breathe oxygen. Extreme exercise may also precipitate problems and if you are a professional athlete you should have a training program that takes account of this.

The trait is not an illness, but if you are planning to have children, then certain factors have to be considered.

If your partner does not have sickle cell trait, then any children you have will not have sickle cell disorder, but they could have the trait (50% chance).

If you and your partner both have the trait, there is a 25% chance that any child conceived may have sickle cell disorder and 50% chance they will have the trait.