What is sickle cell disorder and why does it impact large numbers of the Black community?
September has been named as sickle cell awareness month. Leicester’s Lord Mayor George Cole, is currently working to raise awareness of the disorder.
The Lord Mayor is supporting The Sickle Cell Society – a national charity which supports people with the condition – throughout his term of office. And this month he will be carrying out a number of engagements to increase knowledge of the disorder and its symptoms, and to highlight the work being carried out to support people in Leicester.
So what exactly is sickle cell? And why in comparison to other ethnic groups, are the Afro-Caribbean community more likely to have the disorder?
What is sickle cell?
In a normal body, red blood cells which carry haemoglobin – a protein that carries oxygen – round the body, are round, flexible and can pass through all the blood vessels – even the tiniest ones. Haemoglobin is what gives blood its red colour.
Sickle cell is a genetically inherited disorder that means haemoglobin does not absorb as much oxygen. And when there is less oxygen in the red blood cells, they start to change shape.
They become sickle or banana shaped, and become quite sticky – sticking together and jamming up the blood vessels. This affects oxygen distribution around the body.
What does this do to the body?
The parts of the body that are not getting blood supply, become painful. Carriers tend to experience pain in their legs, arms, joints and bones and can even go into what is called a sickle cell crisis., which can last for a few days or even longer.
Individuals with sickle cell are also less resistant to infection because of damage to the spleen. The spleen is an organ which filters blood and destroys old red blood cells, and helps to fight disease and infection.
People with sickle cell often have spleens that don’t work, and are at risk of complications such as stroke, acute chest syndrome, blindness and bone damage. Over time people with sickle cell can experience damage to other organs too such as the liver, kidney, lungs and heart.
Death can also result from complications of the disorder. Treatment of sickle cell mostly focuses on preventing and treating complications.
Is there a cure?
The only possible cure for the disorder is bone marrow transplant but this is only possible for a small number of people who have a suitable donor. A medicine called Hydroxyurea, can significantly reduce the number of painful crises.