Sickle cell disease: four things we should all know on World Sickle Cell Day

Sickle cell affects thousands of people in the UK but it’s a condition that is often overlooked. This World Sickle Cell Day, Stylist breaks down the important things you need to know about the disease.

Did you know one in 76 babies born in the UK carry sickle cell trait? And approximately 15,000 people in the UK have sickle cell disorder, according to The Sickle Cell Society, a UK charity set up to raise awareness of the disease. Sickle cell disease is a serious and lifelong health condition that centres around the red blood cells. The NHS states that people with the disease produce unusually shaped red blood cells that can cause problems because the affected cells do not live as long as healthy blood cells, and can block blood vessels leading to further health complications down the line.

So, who’s at risk? Although the sickle gene can be found in all ethnic groups, sickle cell disease is particularly common in people with an African or Caribbean family background; the inheritance of a genetic disease like sickle cell depends on a person’s ancestry. Dr Zoe Williams, an NHS GP and TV presenter, tells Stylist: “The vast majority of people with sickle cell disease come from Afro-Caribbean backgrounds. This is because in sub-Saharan Africa, where malaria is a huge killer, having sickle cell trait (where you have one sickle cell gene and one normal haemoglobin gene) provides a protective advantage against malaria.” Therefore people with sickle cell trait – just one sickle cell gene – where malaria was prevalent have a survival advantage, thus the gene was, and is, more common. But, despite thousands of people in the UK living with the disease, it’s still widely misunderstood.   

So today, on World Sickle Cell Day, Stylist takes a look at four things we should all know about the disease. 

What is sickle cell disease?

The Sickle Cell Society writes on its website: “Sickle cell disorders causes your normally round and flexible blood cells to 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.”

The main symptoms of sickle cell disease are:

• painful episodes called sickle cell crises, which can be very severe and last up to a week

• an increased risk of serious infections

• anaemia (where red blood cells cannot carry enough oxygen around the body), which can cause tiredness and shortness of breath

What causes sickle cell disease?

It’s all about genetics. Genes come in pairs, you inherit one set from your mother and one set from your father. To be born with sickle cell disease, a child has to inherit a copy of the sickle cell gene from both their parents.

But that doesn’t mean the parents will have sickle cell disease themselves, they could just be carriers of the gene that affects how red blood cells develop.

If both parents have the gene, there’s a one in four chance of each child they have being born with sickle cell disease. There’s a one in two chance each child they have will just inherit a copy of the sickle cell gene from one parent and be a carrier. 

What is the prognosis like, if diagnosed with sickle cell disease?

Dr Zoe tells Stylist: “The truth is it effects different people in different ways. The only cure for sickle cell disease is a stem cell or bone marrow transplant, but they’re not done very often because of the risks involved and a perfect donor match has to be found.

“If people lead a healthy, active lifestyle they can reduce the complications. And that includes practicing good self-care: always drinking fluid, staying well hydrated. Avoiding very cold temperatures, because that’s a common trigger. And people living with sickle cell disease might take medication, including Hydroxycarbamide to reduce symptoms and antibiotics every day to reduce risk of infections.

“Some people have mild disease, and that may not impact much on their life. But some people can suffer with the disease more severely. Later in life it can lead to complications with blood vessels being blocked, leading to higher risk of strokes, serious infections and lung problems.”

Where can I get more information on sickle cell disease?

We’ve collected several digital resources together to help people who want to read more about sickle cell disease below. 

  • The Sickle Cell Society

    The Sickle Cell Society is a UK charity set up in 1979. On the website it states it was formed by a group of patients, parents and health professionals who were all concerned about the lack of understanding and the inadequacy of treatment for people living with sickle cell disorders.

  • OSCAR Sandwell

    OSCAR Sandwell is a registered charity that was established in 1988 to promote the well being of sufferers of Sickle Cell Anaemia and Thalassaemia. OSCAR was developed to provide a direct service to the public and act as a link between Sickle Cell sufferers and the communities that were at risk from the conventional service providers.

  • British Society for Haematology

    BSH is the largest UK organisation for haematology professionals. This is a helpful website for more in-depth, new research into sickle cell disease. 

  • UK Thalassaemia Society

    Thalassaemia is one of a group of genetic blood disorders referred to as haemoglopinopathies. These disorders, mainly comprising different types of thalassaemia and sickle cell anaemia, are among the most common hereditary diseases worldwide. The UK Thalassaemia Society - a charity - holds a lot of information on the website about sickle cell disease. 

  • The NHS


    The NHS website provides an overview of sickle cell disease, a symptom guide and much more information on sickle cell disease. Click the link below for more information. 

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