World Sickle Cell Day: What You Should Know About The Painful But Preventable Disease

Sickle cell disorders are a group of illnesses which affect the red blood cells. It is a genetic condition, meaning it is passed on from parents to their unborn children. The United Nations recognizes June 19th of every year as World Sickle Cell Day.

What is sickle cell disease?

Sickle cell disorder or disease occurs when normally round and flexible blood cells become stiff and sickle-shaped, stopping the blood cells, and the oxygen they carry, from being able to move freely around the body and causing pain. This can cause episodes of severe pain. These painful episodes are referred to as sickle cell crisis. They are treated with strong painkillers such as morphine to control the pain.

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People with sickle cell are also at risk of complications such as 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.

Facts About The Disease

1. SCD is inherited from both parents; sickle cell trait is inherited from one parent.
2. SCD can affect anyone, although it predominantly affects people from African and Caribbean backgrounds.
3. 1 in 76 babies born in the UK carry sickle cell trait.
4. Approximately 15,000 people in the UK have sickle cell disorder.
5. Approximately 270 babies with SCD are born in the UK every year.
6. A simple blood test will tell whether you have sickle cell trait or the disorder
7. Children with SCD are at increased risk for stroke, the risk is highest between the ages of 2 and 16.
8. Episodes of pain may occur in sickle cell disorder and are generally referred to as a crisis

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What You Should Know About The Sickle Cell Trait

Sickle cell trait is inherited when only one of your parents has passed on the sickle gene, and will never develop into sickle cell disorder. You do not have symptoms from sickle cell trait, so it is a good idea to have a blood test to see if you have sickle cell trait. 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.

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.

Treatment

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.