Inheriting sickle cell anaemia disorder

A sickle-shaped red blood cell next to healthy red blood cells

There are certain inherited blood conditions that mainly affect people of African, Caribbean, Mediterranean, Middle Eastern or Asian origin.

These conditions are called haemoglobin disorders, and the most well known are sickle cell disorder (SCD) and beta thalassaemia major. 

Sickle cell disorder affects around 12,500 people in England, making it the most common inherited disease in the country. It evolved thousands of years ago in areas of the world where malaria was common.

Sickle cell disorder is a group of conditions. The most serious (and common) is sickle cell anaemia. Most people affected by sickle cell anaemia are either of African or Caribbean origin.

Find out more information about thalassaemia.

What is sickle cell anaemia? 

Sickle cell anaemia affects red blood cells. Healthy red blood cells are disc-shaped, flexible and move easily through blood vessels. They contain a protein called haemoglobin, which helps carry oxygen around the body. 

In people with sickle cell anaemia, the haemoglobin doesn’t work properly, and turns red blood cells into sickle (crescent) shapes. These can get stuck in blood vessels and therefore block the flow of blood.

How do you get sickle cell anaemia?

People are born with sickle cell anaemia, inheriting it genetically from their parents. If both parents have the gene for sickle haemoglobin, there is a one-in-four chance the child will have sickle cell anaemia.

If one parent has the gene for sickle haemoglobin, there is a 50% chance the child will carry the gene for sickle haemoglobin. This is known as being a carrier, or having sickle cell trait. People who are carriers do not have symptoms.

Testing for sickle cell anaemia

A simple blood test will show whether a person is a carrier of the sickle cell gene.

In England, all pregnant women are offered screening (either a blood test or a questionnaire) to find out if they are a carrier.

If a pregnant woman is a carrier, the baby's father should be offered a screening blood test. There is also screening to find out whether an unborn baby has sickle cell disorder. All newborn babies are offered a test for sickle cell disorder as part of the routine newborn bloodspot test.

Men and women should find out whether they are a carrier before they start a family, so that they have time to get information and find out what choices they can make. You can find out more from the NHS sickle cell and thalassaemia screening programme, including How to get tested for sickle cell. You can also talk to your GP.

What are the symptoms?

Symptoms of sickle cell anaemia vary from person to person. Common symptoms include: 

  • episodes of pain (known as a crisis) caused by blood cells getting stuck in the blood vessels. The pain can be in the bones, joints, stomach and chest
  • swollen hands and feet (often the first sign of sickle cell anaemia in babies)
  • chronic anaemia (low iron levels in the blood, which make you feel tired and out of breath); however, people with sickle cell anaemia should not take extra iron because their bodies can't cope with it
  • jaundice (yellowing of the skin and eyes because the liver can't get rid of dead sickle cells)
  • damage over time to the heart, lungs and liver

People with sickle cell anaemia can be more vulnerable to infections, such as pneumonia. Therefore, they may be offered a daily dose of penicillin to protect against this.

Where do I go for help and information?

Treatment

Sickle cell crises in children need to be treated under medical supervision (a GP or hospital) because of the risk of infection.

Adults can treat a mild sickle cell crisis at home by keeping warm and taking painkillers, such as paracetamol. If over-the-counter painkillers don’t help, your GP can prescribe something stronger for you.

If you can't manage the pain at home, or you have a fever, you may need hospital treatment. This might include: 

  • strong painkillers
  • intravenous fluids
  • antibiotics
  • oxygen
  • blood transfusion

Preventing a crisis

People with sickle cell anaemia can help prevent a crisis by following healthy lifestyle advice that applies to everyone:

In addition, people with sickle cell anaemia should avoid:

  • getting too hot or cold, because extremes in temperature can trigger a crisis
  • getting dehydrated (not drinking enough water)
  • doing activities in an environment where there's less oxygen, for example scuba-diving, mountain-climbing or having an anaesthetic
  • stress

People with sickle cell anaemia and sickle cell carriers should be careful when having an operation under anaesthetic. They should make sure the anaesthetist (or dentist, if it's a dental procedure) knows about their condition or carrier status.

The Sickle Cell Society offers help and advice on living with sickle cell disorder, and can put you in touch with a local centre.




Sickle cell disease: Rosalind's story

Rosalind has sickle cell disease, an inherited blood disorder. She explains how understanding the illness helps her to manage everyday life.

Media last reviewed: 21/10/2013

Next review due: 21/10/2015

Page last reviewed: 11/06/2012

Next review due: 11/06/2014

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