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A Guide to Sickle Cell Disease

shoing the differce between normal and sickle red blood cells
Normal and sickled red blood cells

What is Sickle Cell Disease?

Sickle cell disease is the name for a group of inherited health conditions that affect the red blood cells, the most serious type is called sickle cell anaemia. It is particularly common in people with an African or Caribbean family background. Typically, red blood cells are round, flexible, and able to smoothly travel through blood vessels. In SCD, a genetic mutation causes these cells to become rigid and take on a crescent or 'sickle' shape.

The Consequences of Sickle-Shaped Cells

This altered shape of red blood cells has significant consequences:

  1. Painful Crises: Sickle cells can block blood flow, leading to episodes of intense pain known as pain crises. These crises can occur suddenly and last for days.

  2. Reduced Oxygen Delivery: Sickle cells struggle to carry oxygen effectively resulting in fatigue, weakness and anemia.

  3. Organ Damage: Over time, SCD can cause damage to various organs, including the spleen, liver, kidneys and lungs.

  4. Complications: Individuals with SCD are at a higher risk of infections, stroke, and other health complications.

Genetic Inheritance

SCD is a genetic condition. To develop SCD, a person must inherit two abnormal haemoglobin genes, one from each parent. Individuals who inherit only one abnormal gene are carriers and typically do not show any symptoms of the disease.

A diagram showing how haemoglobin disorders are inherited
Genetic inheritance of a haemoglobin disorder

Screening and Testing for Sickle Cell Disease

Screening for sickle cell disease in pregnancy is offered to all pregnant women in England to check if there's a risk of a child being born with the condition, and all babies are offered screening as part of the newborn blood spot test (heel prick test).

Blood tests can also be carried out at any age to check for sickle cell disease or to see if you're a carrier of the gene that causes it.

Treatment for Sickle Cell Disease

People with sickle cell disease need treatment throughout their lives. This is usually delivered by different health professionals in a specialist sickle cell centre. It is also important for people with sickle cell disease to look after their own health using self-care measures, such as avoiding triggers and managing pain.

Treatments may include:

  • Painkillers, such as paracetamol or ibuprofen (sometimes treatment with stronger painkillers in hospital may be necessary)

  • Daily antibiotics to reduce your chances of getting an infection

  • Blood transfusions

  • Medications to prevent complications

The only cure for sickle cell disease is a stem cell or bone marrow transplant, these are not done often because of the risks involved.


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