What is Sickle Cell Disease?

Sickle cell disease (SCD) is an inherited health condition that affects red blood cells, resulting in severe anaemia.(1)

The condition is called ‘sickle’ cell because the blood cells become shaped like a farmer’s sickle.(2)

In the United Kingdom, the common types of SCD include sickle cell anaemia (HbSS), Sickle Haemoglobin C disease (HbSC), and different variations of sickle beta thalassaemia, for instance, sickle beta plus thalassaemia (HbS/β+ThaIassaemia) and sickle beta zero thalassaemia (HbS/β0 Thalassaemia).(2)

People with SCD produce unusually shaped red blood cells called ‘sickled red blood cells’. These cells do not live as long as healthy blood cells, which can result in a number of complications, including iron deficiency anaemia, a condition in which there aren’t enough red blood cells to carry oxygen to the body’s tissues, and other complications.(1,2)

Sickled red blood cells are also not as flexible as normal red blood cells, which means they are not always able to pass through blood vessels.(2)

If the sickled cells get trapped in a blood vessel, this reduces the blood supply to that part of the body and causes pain and sometimes damage. This is often referred to as a ‘painful crisis’ which can begin suddenly and may last several hours or days. It usually does not cause permanent damage.(2)

SCD is a serious and lifelong health condition; however treatment can help manage most symptoms so people diagnosed with SCD can lead active lives(1)

What is the cause of SCD?

SCD is an inherited condition which means that the child inherited a faulty haemoglobin gene from both parents.(2)

What can cause a crisis?(1)

Sudden change in body temperature, particularly skin cooling

Infection

Dehydration

Stress

Excessive physical exertion

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