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Sickle cell disease

3-minute read

Sickle cell disease is not a single disease, but a group of inherited disorders that affect red blood cells. Sickle cell anaemia is the most common and most serious form of sickle cell disease. People with sickle cell anaemia need ongoing treatment to avoid or manage episodes of pain and reduce their chances of infection.

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 by the spleen, a large organ under your ribs on the left, and destroyed. Usually, your red cells last between 90-120 days before being destroyed by the spleen. But sickled cells last only 10-20 days.

This destruction of red blood cells leads to 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 disease diagnosis

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

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
  • a swollen and tender spleen
  • problems in the long term with their 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
  • taking daily antibiotics and being fully vaccinated to prevent infection
  • having regular blood transfusions (usually every 3–4 weeks) to limit damage caused by the sickle-shaped cells.

Living with sickle cell anaemia

If you have sickle cell disease, it’s important to try 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 sickle cell disease

Milder forms of sickle cell include haemoglobin SC, haemoglobin CD, haemoglobin SE and haemoglobin S beta thalassaemia.

Where to go for help?

­

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

More information

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

Last reviewed: August 2016

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