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Sickle cell anaemia

Produced by Krysta Munford, Bupa Health Information Team, December 2011.

This factsheet is for people who have sickle cell anaemia, or who would like information about it.

Sickle cell disease is an inherited condition in which your red blood cells behave abnormally, leading to anaemia and a number of other problems. Anaemia is a condition in which the blood can’t carry enough oxygen to fully meet the needs of your body.

About sickle cell anaemia

Sickle cell anaemia develops when your body makes an abnormal form of the protein in your blood that carries oxygen, called haemoglobin. The abnormal haemoglobin (known as Haemoglobin S or HbS) causes your red blood cells to change structure and become shaped like sickles (crescent-shaped).

Unlike normal red blood cells, sickle cells are rigid, sticky and have a shorter life-span lasting 16 to 20 days. They clump together and block blood vessels, causing pain and damage to tissues. The painful episode is known as a sickle cell crisis. The frequency of the crises, how much pain you have and how long each crisis lasts varies between individuals. In between crises, you may feel well.

About 12,500 people in the UK have sickle cell anaemia. It’s most common in people of black African or Caribbean descent, but can also affect people of Middle Eastern, Asian and eastern Mediterranean background. Globally, around one in 20 people carry one sickle cell gene and this is known as sickle cell trait.

Symptoms of sickle cell anaemia

Common symptoms of all types of anaemia include:

  • feeling tired
  • looking pale
  • increased breathlessness
  • feeling your heart racing or thumping (called palpitations)

There are other symptoms that are more specific to sickle cell anaemia, including:

  • pain from a crisis, which can occur in any part of your body but most commonly in your chest, abdomen, back, arms or legs
  • yellowing of your skin and the whites of your eyes (called jaundice)
  • swelling in your hands and feet (known as hand-and-foot syndrome) – usually in children aged between six and 18 months

In babies, the symptoms don’t usually develop until after four months, because they are initially protected by another type of haemoglobin called HbF.

These symptoms may be caused by problems other than sickle cell anaemia. If you have any of these symptoms, see your GP for advice.

Complications of sickle cell anaemia

If you have anaemia, your heart has to work harder to get oxygen to your vital organs. If left untreated it can lead to problems with your heart and lungs. Specific complications of sickle cell anaemia include:

  • kidney damage
  • delayed growth and puberty
  • painful, long lasting erections in men (priapism)
  • ulcers on your lower legs
  • serious infections, such as pneumonia (inflammation of the lungs) and salmonellosis (infection with Salmonella bacteria)
  • stroke or heart attack
  • loss of vision
  • acute chest syndrome, which can be life-threatening, affects your lungs and the symptoms include pain in your chest, a temperature and difficulty breathing

Causes of sickle cell anaemia

Sickle cell anaemia is an inherited condition, which means you get the abnormal haemoglobin gene from your parents. You need to inherit one abnormal haemoglobin gene from each parent to have sickle cell anaemia.

If you inherit an abnormal haemoglobin gene from one of your parents but a normal haemoglobin gene from the other, you will have sickle cell trait. This means you won't develop sickle cell anaemia, but you could pass the abnormal haemoglobin gene on to your children.

Illustration to show how sickle cell conditions are inherited

Diagnosis of sickle cell anaemia

Your GP will ask about your symptoms and examine you. He or she may also ask you about your medical history and your family's medical history.

If your GP suspects that you have sickle cell anaemia, you will be asked to have a blood test. Your blood will be sent to a laboratory to be tested for the following.

  • A full blood count. This is to check the level of haemoglobin in your blood, how many of each of the different types of blood cells you have, the size of your red cells, and the amount of haemoglobin in each red cell.
  • A blood film. Your blood will be looked at under a microscope to check the size and shape of your red blood cells and to see if there are sickle cells present. The different white cells that are present will also be assessed.
  • A sickle screening test. This is to check for the presence and amount of HbS in your blood. Also, an additional test may be carried to identify the different types of haemoglobin in your red cells.

In the UK, babies are tested for sickle cell disease at birth in a national screening programme. Many pregnant mothers are also screened for haemoglobin disorders. If they are found to carry the sickle cell gene, it’s important to test the baby’s father to check whether the baby will have sickle cell anaemia.

Antenatal diagnosis (during your pregnancy) is also available – it’s possible to tell if your unborn baby has sickle cell disease from as early as 11 weeks of pregnancy. Ask your GP or midwife for more information.

Treatment of sickle cell anaemia

The aim of treatment is to help control the symptoms and limit the frequency of sickle cell crises. You will usually have a team of doctors to look after different aspects of your care. You will need regular check-ups with your medical team at a specialist unit to monitor your condition.

Medicines

Hydroxycarbamide is a medicine that can help to reduce the frequency of sickle cell crises and the need for blood transfusions. It works by stimulating the production of fetal haemoglobin (a type of haemoglobin that is found in unborn and newborn babies), which helps to reduce the number of sickle cells in your blood.

You may also need to take over-the-counter painkillers, such as paracetamol or ibuprofen, to help manage your pain during sickle cell crises. Always read the patient information that comes with your medicine and if you have any questions, ask your pharmacist for advice. If your pain is severe, your doctor can prescribe stronger medicines, such as morphine and you may need to be admitted to hospital.

Having sickle cell anaemia may increase your risk of getting an infection. It's therefore important to have vaccines, such as the pneumococcal vaccine (to protect you against the bacteria that cause pneumonia), meningitis vaccine and the seasonal flu vaccine. Your doctor may also suggest you take penicillin as a preventive measure. If you get an infection, your doctor can prescribe antibiotics.

Hospital treatment

If you have a sickle cell crisis, it’s important to make sure you have enough fluids. Alternatively, if you are admitted to hospital, you may be given fluids through a drip into a vein. You may also be given oxygen through a mask. This is because if you're not getting enough oxygen, more of your red cells may become sickle-shaped.

If your anaemia becomes severe, you may need to have a blood transfusion. This means you will be given blood or red blood cells from a donor. The blood will be given straight into your bloodstream through a small tube (cannula) put into a vein, usually in your arm.

Surgery

A possible cure for some people with sickle cell anaemia is a bone marrow or stem cell transplant, but this is usually only for children. Your bone marrow contains stem cells, which are the most basic kind of blood cell. These stem cells mature into red blood cells, as well as white blood cells and platelets.

The treatment involves replacing your bone marrow with stem cells donated from somebody who doesn't have sickle cell anaemia. If the transplant is successful, the new bone marrow begins producing healthy red blood cells that don't become sickle-shaped. However, a bone marrow transplant isn't suitable for everyone.

Special considerations

Sometimes, people who have sickle cell trait can develop complications under extreme conditions, for example mountain climbing at high altitude and scuba diving at increased pressure. Check with your GP before starting any new, physically demanding activities.

If you’re planning a family

It’s important to remember that if you and your partner both have the abnormal haemoglobin gene then your child can inherit sickle cell anaemia. So, if you or your partner has a family history of sickle cell disease, or if you both have sickle cell trait, it's recommended that you see your GP or a genetic counsellor before you try for a child.

If you’re pregnant

If you're pregnant and have sickle cell anaemia, your doctors will monitor your condition. You will be advised to drink enough fluids, take folic acid supplements (5 milligrams per day) and make sure you seek medical attention if you have an infection or crisis. Some women may need blood transfusions during their pregnancy.

 

For answers to frequently asked questions on this topic, see FAQs.

For sources and links to further information, see Resources.


  • This information was published by Bupa's Health Information Team and is based on reputable sources of medical evidence. It has been reviewed by appropriate medical or clinical professionals. Photos are only for illustrative purposes and do not reflect every presentation of a condition. The content is intended only for general information and does not replace the need for personal advice from a qualified health professional. For more details on how we produce our content and its sources, visit the about our health information page.

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  • Publication date: December 2011

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