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Sickle Cell & Thalassaemia

Sickle Cell

Sickle cell disorders are inherited blood conditions. The different types include sickle cell anaemia, haemoglobin sc, and sickle beta thalassaemia. They are all slightly different, the most common and severe disorder is sickle cell anaemia.

The conditions are inherited from both parents. It predominantly affects people of African and African-Caribbean descent but can also affect those from India, Pakistan, the Middle East and the eastern Mediterranean.

Some people do not have sickle cell disorder but carry the sickle cell gene; this is called sickle cell trait. These people will be healthy and will never develop the disease, but if two people with sickle cell trait have children there is a one in four chance that the child will have sickle cell disease.

Sickle cell anaemia affects the body’s ability to carry oxygen around the body in red blood cells because instead of being round some of the blood cells are sickle shaped. The sickled cells can get stuck as they try to move through small blood vessels. This causes pain and can lead to tissue damage and other more serious problems such as stroke or blindness. Sickled blood cells also die earlier than normal blood cells which creates a shortage of blood cells in the body. This causes breathlessness and tiredness.

People with sickle cell disease can have a normal home and school life but will sometimes get bouts (called crises) of pain, infections, anaemia or jaundice. They may also have periods when they feel constantly exhausted. 

It is important for people with sickle cell disease to keep warm and dry and avoid strenuous outdoor exercise during cold wet weather as the cold and the wet can trigger a crisis. As dehydration can trigger the formation of sickle cells it is important to drink plenty of water, at least 1.2 litres a day, more on hot days and not drink too much alcohol. Stress can also cause a crisis to start, learning relaxation techniques (link) or joining a support group can help.

Treatment during a crisis depends on how serious the pain is. It may be enough to take over the counter pain killers such as paracetomol. If the pain is severe a doctor will prescribe stronger painkillers and possibly a blood transfusion to increase the number of healthy red blood cells in the body.

Young people should always see a doctor if they are having a crisis as there is a greater risk of infection. They should also have an annual flu injection and regular vaccines for pneumonia.

The only way to cure sickle cell anaemia is to have a bone marrow transplant but there are risks associated with this and it is not suitable for everyone.

If you think there’s a chance you might have either sickle cell trait or sickle cell disease you can have a quick blood test to find out. It is important to know. If you have sickle cell trait you won’t suffer any of the problems of sickle cell disease but you will need extra oxygen whilst under anaesthetic (if you ever have an operation) and you should be careful when doing sports such as scuba diving or high mountain climbing as your oxygen supply might be reduced during these activities. This is because sickle cell trait affects a body’s ability to use oxygen efficiently. It is also good to know if you might pass on the condition to your children.

If you want to take a test or you have sickle cell and would like some help and support you can drop in and see us at the house or contact the Sickle Cell and Thalassaemia Centre:

Sickle Cell & Thalassaemia Centre
457 Queensbridge Road
Hackney
E8 3TS

020 7683 4570

You can read about one girl’s experience of living with sickle cell on the NHS website.

There’s also a website for young people with sickle cell where you can read about young people’s experience with sickle cell.

Thalassaemia

Thalassaemia is an inherited blood disorder that affects the body’s ability to create red blood cells.  It is usually found in people from countries where malaria was common such as Greece, Cyprus, Italy the middle east, Asia and Africa.

There are two types of thalassaemia, alpha and beta. Alpha thalassaemia will have no effect on a person if there is only one genetic mutation, but both types can lead to mild anaemia (a condition which makes you feel weak) or in more severe cases can result in needing life long blood transfusions.

Thalassaemia causes fatigue, weakness, shortness or breath and jaundice (yellowing of the skin). It can also lead to puberty being delayed or not starting at all.

Testing for thalassaemia can be done before a baby is born or after by doing a blood test followed by a DNA test to determine the type of thalassaemia.

At the moment there are only two cures for the disease, a bone marrow transplant or core blood transfusion which is a transfusion using blood from the umbilical cord of a baby in the womb.

The disease is usually treated by regular blood transfusion followed by chelation therapy because repeated blood transfusions cause a build up of iron in the body which can lead to liver damage or heart disease if it is not removed. Chelation therapy involves having a transfusion of chemicals slowly pumped into your body over a few hours. There is current research trying to find an easier form of chelation therapy which can be taken as a tablet.

If you have thalassaemia and you catch an infection it can be more serious so it is important to deal with infections as soon as you start having symptoms. Young People should have their height and weight checked every year during puberty to check they are developing normally.

Low bone density is common in people who have thalassaemia and it helps to have a diet high in calcium and vitamin D such as dark green vegetables and milk and cheese as this helps strengthen bones.  Your doctor may also recommend a vitamin D supplement. It is important to do regular weight bearing exercise at least 3 or 4 times a week, to help strengthen bones. Weight bearing exercises include running aerobics weight training and press ups.

If you want to take a test for thalassaemia or you would like some help and support you can drop in and see us at the house or contact the Sickle Cell and Thalassaemia Centre:

Sickle Cell & Thalassaemia Centre
457 Queensbridge Road
Hackney
E8 3TS

020 7683 4570

Related Questions

  • Age: 14
    Gender: Female

    Question:

    ive got a blood test in a couple of weeks and im scared coz I've been told they inject into ur vein, could you tell me what they really do because im dreading it :/

    Answer:

    A blood test is a very routine test, and the person doing it will be very proficient. A very thin needle is inserted into a vein, usually in the bend of your arm where veins are prominent, and a small amount of blood is taken out.  It can be uncomfortable but not really painful; the thought of it is worse than the reality. 

    It is possible to have some numbing cream put on beforehand. This takes about 20-40 minutes to work, so sometimes it is better to get it over with quickly than get even more anxious while waiting for the cream to work!

  • Age: 14
    Gender: Female

    Question:

    ive had a bladder problem for over half of my life, and its got worse. ive just managed to get some medication for it, would i be on them for long?

    Answer:

    As you have had this condition for most of your life you probably have a specialist or your doctor who sees you regularly. If this is the case its probably best to speak with them as they will have good picture of the history. Having said that we would be very happy to talk through your condition and treatment and help clarify any questions or concerns you may have. Naturally we would need more information which would best shared in a private consultation. If you are local please drop in to one of our clinics or if you are further away give us a call and we can talk on the phone.

  • Sickle Cell