What is sickle cell anaemia?

Sickle cell anaemia is a genetic disorder where the body produces red blood cells that are unstable. Usually, red blood cells are round. In most people, they can bend to go through small vessels and pop back into their normal shape. In people with sickle cell disease, the red blood cells can form into a crescent, or sickle shape, but they can’t go back to normal.

These sickle cells are mopped up and destroyed by the spleen, a large organ under the ribs on the left. Usually, red cells last between 90 to 120 days before being destroyed by the spleen. But sickled cells last only 10 to 20 days.

In sickle cell anaemia, this destruction of red blood cells leads to the anaemia, which can cause breathlessness, tiredness and dizziness.

Both men and women can have sickle cell anaemia. For a child to inherit the condition, both parents must pass on the faulty gene. The parents may not have sickle cell anaemia themselves, or even know that they carry the gene.

Sickle cell anaemia diagnosis

Sickle cell anaemia is often discovered through tests during pregnancy or soon after a child’s birth. Blood tests can also reveal if you carry the gene that causes sickle cell anaemia.

Sickle cell anaemia signs and symptoms

Sickle cell anaemia causes illness which usually has a fairly big impact on people’s lives.

People with sickle cell anaemia often feel tired and listless due to their chronic anaemia. They also have more chance of getting infections.

People with sickle cell anaemia can have:

• chronic pain
• pain crises, in which a person gets sudden attacks of pain, often in the abdomen, bones and joints
• fever
• mild jaundice
• chest pain
• shortness of breath
• a lot of infections
• a swollen and tender spleen
• delayed growth in children
• problems in the long term with the heart, kidneys, eyes
• priapism, or a prolonged painful erection, in men

In children, fingers and toes may become swollen and painful due to blockages in the blood flow to the hands and feet.

Sickle cell anaemia treatment

People with sickle cell anaemia need a range of treatments throughout their lives. These treatments include:

• drinking plenty of fluids and staying warm to prevent episodes of pain
• taking pain relief medicines (for example, paracetamol or ibuprofen) to manage pain
• being fully vaccinated to prevent infection and sometimes taking daily antibiotics
• having regular blood transfusions (usually every 3–4 weeks) to treat the anaemia

Sometimes children who have significant symptoms from sickle cell anaemia may be offered a bone marrow transplant. Some researchers are now experimenting with gene therapy to treat the disease.

Living with sickle cell anaemia

If you have sickle cell disease, it’s important to try to and stay as healthy as possible. Take steps to manage pain, avoid infections and practice good food hygiene.

If you want to get pregnant, consider genetic testing or genetic counselling with your partner.

If you need surgery, it’s important to tell your surgeon and anaesthetist about your sickle cell anaemia, as anaesthetics can cause problems, such as pain episodes.

Other types of haemoglobin disorders

Other inherited blood disorders that affect the red blood cells are beta thalassaemia, alpha thalassaemia and haemoglobin E.

Resources and support

For support regarding sickle cell anaemia, and other inherited disorders affecting red blood cells, visit Thalassaemia and Sickle Cell Australia website.

Visit our genetic disorders guide to learn more about genes, types of genetic disorders and where to go for help and more information.