Sickle Cell disease is the name for the group of genetic blood disorders. Our red blood cells contain a protein called haemoglobin which carries oxygen from the lungs to the tissues of the body, as well as transporting CO2 and hydrogen ions back to the lungs.
Sickle cell is inherited from both parents who carry the trait, and comes in many forms:
- Haemoglobin SS
- Haemoglobin SC
- Haemoglobin SB (beta) thalassemia
- Haemoglobin SD
- Haemoglobin SB+ Beta Thalassaemia
- Haemoglobin Beta-Zero Thalassaemia
Sickle Cell is pre-dominantly found in African and Caribbean people. Other people affected are Asian, Arab and Mediterranean. The most common form is Sickle Cell Anaemia. People with this have Sickle haemoglobin (HbS). This means that the red blood cells are devoid of oxygen and are unable to move around like normal blood cells (Hba), which are donut shaped.
Because the cells are unable to move, they stick together and end up blocking blood vessels which causes tissue and organ damage, as well as severe bouts of pain. Such episodes of pain are known as a ‘Sickle Cell Crisis’ or a ‘Vaso-Occlusive Crisis.’
A crisis can be triggered by:
- A sudden change in temperature
A crisis can last anywhere from a few minutes to a number of days or months.
So what causes Sickle Cell Anaemia?
A mutation in the gene that instructs the body to produce haemoglobin.
What are the health problems Sickle Cell Anaemia can lead to?
- Iron over-load
- Swollen joints
- Breathing difficulties
- Permeant damage resulting in the removal of the spleen
- Reduced bone density
- Priapism (painful swollen and lumpy penis which can cause erectile dysfunction)
- Organ failure
- Weakened immune system
How is it inherited?
You will have sickle cell when you inherit the ‘defective’ gene from both parents. If you only inherit the gene from one parent then you have the sickle cell trait. It is likely that your blood will contain some sickle cells, but you will still be able to produce normal haemoglobin, and you won’t usually experience any symptoms.
However, inheriting the trait means you are a carrier. This means that you could pass the gene on to your children – but not the sickle cell diseases, unless your partner also has the trait. This is why it is important we test our blood to see if we have the trait or not.
Sickle Cell Anaemia is a very serious genetic condition, which is why it is essential that we understand, empathise and advocate for our people who are living in their thousands in the UK, with this disease. And let’s not forget our Melenated brothers and sisters in parts of Africa and also in America too. Sickle Cell is real and it needs to be acknowledged individually and globally.
We are now media correspondents and advocates for the Sickle Cell Cause support group based at St Ann’s Hospital, George Marsh centre, in Tottenham, London. We will also be taking part in the Community Mile for Sickle Cell charity walk on Sunday July 23, 2017.
To get involved and to find out more about sickle cell, please click here.
If you would like to sponsor us, please contact us on: firstname.lastname@example.org
The Jus’ Caribbean Festival 2017 is an official sponsor for the Sickle Cell Cause support group. The event will take place Saturday 19 – Sunday 20. Come and enjoy authentic tastes of the Caribbean, and support our own, food-wise and health-wise!
Eunice and Ogechi