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Sickle cell disease is the name for a group of inherited conditions that affect the red blood cells. The most serious type is called sickle cell anaemia.
Sickle cell disease mainly affects people of African, Caribbean, Middle Eastern, Eastern Mediterranean and Asian origin. In the UK, it's particularly common in people with an African or Caribbean family background.
People with sickle cell disease produce unusually shaped red blood cells that can cause problems because they don't live as long as healthy blood cells and they can become stuck in blood vessels.
Sickle cell disease is a serious and lifelong condition, although long-term treatment can help manage many of the problems associated with it.
This page covers:
People born with sickle cell disease sometimes experience problems from early childhood, although most children have few symptoms and lead normal lives most of the time.
The main symptoms of sickle cell disease are:
Some people also experience other problems such as delayed growth, strokes and lung problems.
Read more about the symptoms of sickle cell disease.
Sickle cell disease is caused by a faulty gene that affects how red blood cells develop.
If both parents have this faulty gene, there's a 25% chance of each child they have being born with sickle cell disease.
The child's parents often won't have the condition themselves because they're only carriers of the sickle cell trait (see below).
Read more about the causes of sickle cell disease.
Sickle cell disease is often detected during pregnancy or soon after birth.
Screening for sickle cell disease in pregnancy is offered to all pregnant women in England to check if there's a risk of a child being born with the condition, and all babies are offered screening as part of the newborn blood spot test (heel prick test).
Blood tests can also be carried out at any age to check for the condition or to see if you're a carrier of the faulty gene that causes it.
Read more about screening and testing for sickle cell disease.
People with sickle cell disease will need specialist care throughout their lives.
A number of treatments are available to help manage problems caused by the condition. For example:
Stem cell or bone marrow transplants can potentially cure sickle cell disease, but they're not done very often because of the significant risks involved.
Sickle cell disease is very variable, but most children with it will lead happy and normal lives.
However, it can still be a serious condition that can have a significant impact on a person's life.
It can lead to problems such as strokes, serious infections and lung problems, which can occasionally be fatal.
Overall, the life expectancy for someone with sickle cell disease tends to be shorter than normal, but this can vary depending on the exact type of sickle cell disease they have, how it's treated, and what problems they experience.
Currently, people with sickle cell anaemia typically live until 40-60 years of age, although milder types of sickle cell disease may have no impact on life expectancy. It's hoped the outlook will continue to improve as newer treatments are developed.
A carrier of sickle cell is someone who carries one of the faulty genes that causes sickle cell disease, but doesn't have the condition themselves. It's also known as having the sickle cell trait.
People who carry sickle cell won't develop sickle cell disease, but are at risk of having a child with the condition if their partner is also a carrier.
You can request a blood test to check if you have carry sickle cell from your GP surgery or nearest sickle cell and thalassaemia centre.
Read more about being a sickle cell carrier.
Sickle cell disease can cause a wide range of symptoms.
These can start from a few months of age, although many children have few or no symptoms if treatment is started early on.
Some of the main symptoms are outlined below, although not everyone with the condition will experience all of these.
Episodes of pain – known as sickle cell crises – are one of the most common and distressing symptoms of sickle cell disease. They occur when blood vessels to part of the body become blocked.
The pain can be severe and lasts for up to seven days on average.
A sickle cell crisis often affects a particular part of the body, such as the:
How often someone with sickle cell disease gets episodes of pain varies a lot. Some people may have one every few weeks, while others may have less than one a year. The average is one bad episode a year.
It's not always clear what triggers bad pain, but sometimes they can be caused by the weather (such as wind, rain or cold), dehydration, stress or strenuous exercise.
People with sickle cell disease are more vulnerable to infections, particularly when they're young.
This is because the condition can mean the body isn't very good at dealing with bacteria and viruses.
Vaccinations and daily doses of antibiotics can help reduce the risk of many infections.
Nearly all people with sickle cell disease have anaemia, where the haemoglobin in the blood is low. Haemoglobin is the substance found in red blood cells that's used to transport oxygen around the body.
This doesn't usually cause many symptoms, but sometimes it can get worse if you become infected with the virus that causes slapped cheek syndrome (parvovirus).
This can lead to a sudden drop in the number of red blood cells and may cause additional symptoms such as headaches, a rapid heartbeat, dizziness and fainting. It's usually treated with a blood transfusion.
In young children, swelling of the spleen can also cause sudden anaemia that requires a blood transfusion.
Sickle cell disease can also sometimes cause a wide range of other problems, including:
See treatments for sickle cell disease for information about how many of these problems are treated.
Sickle cell disease is caused by faulty genes that a child inherits from their parents.
It's not caused by anything the parents did before or during the pregnancy and you can't catch it from someone who has it.
Genes come in pairs. You inherit one set from your mother and one set from your father.
To be born with sickle cell disease, a child has to inherit a copy of the faulty sickle cell gene from both of their parents.
This usually happens when both parents are "carriers" of the faulty gene – also known as having the sickle cell trait.
Sickle cell carriers don't have sickle cell disease themselves, but there's a chance they could have a child with the condition if their partner is also a carrier.
If both parents are sickle cell carriers, there's a:
The Sickle Cell Society has more information about the inheritance of sickle cell disease, including what the risks are if a parent has sickle cell disease themselves.
Sickle cell disease mainly affects people with an African, Caribbean, Middle Eastern, Eastern Mediterranean and Asian family background.
In the UK, it's most commonly seen in people of African and Caribbean backgrounds. It's estimated that one in every 10 people in this group carries sickle cell.
A simple blood test will show whether you're a carrier. This is done routinely during pregnancy and after birth, but you can ask to have the test at any time.
Your genes are the set of instructions found inside every cell in your body. They determine characteristics such as the colour of your eyes and hair.
People with sickle cell disease have a problem with the genes involved in the development of haemoglobin – a substance found in red blood cells that carry oxygen around the body.
Normal red blood cells are flexible and disc-shaped, but in sickle cell disease they can become rigid and shaped like a crescent or sickle because the haemoglobin inside them clumps together.
These unusual cells can cause symptoms of sickle cell disease because they don't live as long as normal red blood cells and can become stuck in blood vessels.
Sickle cell disease is usually detected during pregnancy or soon after birth.
Blood tests can also be carried out at any time to check for the condition or to see if you're a sickle cell carrier and are at risk of having a child with the condition.
Screening to check if a baby is at risk of being born with sickle cell disease is offered to all pregnant women in England.
In parts of England where conditions such as sickle cell disease are more common, pregnant women are offered a blood test to check if they carry sickle cell.
In areas where these conditions are less common, a questionnaire about your family origins is used to determine whether you should have a blood test for sickle cell.
You can also ask to have the test even if your family origins don't suggest your baby would be at high risk of sickle cell disease.
Screening should ideally be carried out before you're 10 weeks pregnant, so you and your partner have time to consider the option of further tests to find out if your baby will be born with sickle cell disease.
Read more about screening for sickle cell disease during pregnancy.
In England, screening for sickle cell disease is offered as part of the newborn blood spot test (heel prick test).
This can help to:
If newborn screening suggests your baby may have sickle cell disease, a second blood test will be carried out to confirm the diagnosis.
Read more about the newborn blood spot test.
A blood test can be done at any time to find out if you carry sickle cell and are at risk of having a child with sickle cell disease. This is also known as having the sickle cell trait.
Getting tested can be particularly useful if you have a family history of the condition or your partner is known to carry sickle cell.
If you think you could be a carrier, you can ask for a test from your GP surgery or nearest sickle cell and thalassaemia centre.
Both men and women can have the test.
Read more information about sickle cell carriers.
Sickle cell disease usually requires lifelong treatment.
Some of the main treatments that may be used are outlined below:
Children and adults with sickle cell disease will be supported by a team of different healthcare professionals working together in a specialist sickle cell centre.
Your care team will help you learn more about the condition and work with you to come up with an individual care plan that takes into account all your needs and health concerns.
The main thing you can do to reduce your chances of experiencing a painful episode (sickle cell crisis) is to try avoiding possible triggers.
This may mean you need to:
Read about living with sickle cell disease for more advice.
If you continue to experience episodes of pain, a medication called hydroxycarbamide (hydroxyurea) may be recommended. This is usually taken as a capsule once a day.
Hydroxycarbamide can lower the amount of other blood cells, such as white blood cells and platelets (clotting cells), so regular blood tests will usually be recommended to monitor your health.
If you experience a sickle cell crisis, you can usually manage it at home. The following can help:
Contact your GP if these measures don't work or the pain is particularly severe. If this isn't possible, take your child to your local accident and emergency (A&E) department.
Treatment with very strong painkillers (such as morphine) in hospital for a few days may be needed.
As people with sickle cell disease are more vulnerable to infections, treatment will usually be needed to reduce this risk.
Most people need to take a daily dose of antibiotics (usually penicillin), often for the rest of their life. Long-term use of antibiotics won't pose any serious risks to your health.
Anaemia often causes few symptoms and may not require specific treatment.
However, dietary supplements such as folic acid (which helps stimulate the production of red blood cells) may sometimes be required to help improve anaemia if your child has a restricted diet, such as a vegetarian or vegan diet.
Anaemia caused by sickle cell disease isn't the same as the more common iron deficiency anaemia. Don't take iron supplements to treat it without seeking medical advice as they could be dangerous.
If anaemia is particularly severe or persistent, treatment with blood transfusions or hydroxycarbamide may be necessary.
Stem cell or bone marrow transplants are the only cure for sickle cell disease, but they're not done very often because of the significant risks involved.
Stem cells are special cells produced by bone marrow (a spongy tissue found in the centre of some bones) that can turn into different types of blood cells.
For a stem cell transplant, stem cells from a healthy donor are given through a drip into a vein. These cells then start to produce healthy red blood cells to replace the sickle cells.
A stem cell transplant is an intensive treatment that carries a number of risks. The main risk is graft versus host disease, which is a life-threatening problem where the transplanted cells start to attack the other cells in your body.
Stem cell transplants are generally only considered in children with sickle cell disease who have severe symptoms that haven't responded to other treatments, when the long-term benefits of a transplant are thought to outweigh the possible risks.
Sickle cell disease can also cause a number of other problems that may need to be treated. For example:
People who need a lot of blood transfusions may also need to take medication to reduce the amount of iron in their blood to safe levels. This is called chelation therapy.
If you'd like more information about treatments for sickle cell disease, the NHS Sickle Cell and Thalassaemia Screening Programme produces a detailed parent's guide to managing sickle cell disease (PDF, 3.57Mb) that you might find helpful.
There are a number of things you can do, and precautions you need to take, to ensure you stay as healthy as possible if you have sickle cell disease.
See below for information about:
You can reduce your risk of experiencing painful episodes (sickle cell crises) by avoiding things that can trigger them. You should try to:
Your care team can give you more advice about avoiding triggers.
Read more about treatments for sickle cell disease.
You'll usually be given antibiotics and advised to have vaccinations to help prevent most serious infections, but there are also things you can do to reduce your risk.
For example, you should make sure you follow good food hygiene measures to prevent food poisoning.
You should also make sure you speak to your GP or care team if you're planning on travelling aboard, as this may mean you need extra medication or vaccinations. You may also need to take extra food and water precautions.
Women with sickle cell disease can have a healthy pregnancy, but it's a good idea to speak to your care team for advice first because:
If you're not planning a pregnancy, you should use a reliable form of contraception.
It's important to let your care team know if you need to have an operation under general anaesthetic at any point. You should also tell your surgeon that you have sickle cell disease.
This is because general anaesthetic can cause problems for people with sickle cell disease, including an increased risk of experiencing a sickle cell crisis.
You may need close monitoring during surgery to ensure you're getting enough fluids and oxygen and are kept warm.
Sometimes you may be need a blood transfusion beforehand to reduce the risk of complications.
It's important to make sure you know when to get medical advice and where to go because sickle cell disease can cause a number of serious problems that can appear suddenly.
Problems to look out for include:
Contact your GP or care team immediately if you develop any of the above symptoms. If this isn't possible, go to your nearest accident and emergency (A&E) department. If you aren't well enough to travel to hospital yourself, dial 999 for an ambulance.
Make sure that the medical staff looking after you are aware that you have sickle cell disease.
If you're a carrier of sickle cell it means you carry one of the faulty genes that causes sickle cell disease, but you don't have the condition yourself.
It's also known as having the sickle cell trait.
People who carry sickle cell won't develop sickle cell disease, but may be at risk of having a child with the condition and may occasionally need to take precautions to stop them becoming unwell.
You can find out if you're a carrier of sickle cell by having a simple blood test.
This page covers:
The NHS Sickle Cell and Thalassaemia Screening Programme also has a detailed leaflet about being a sickle cell carrier (PDF, 773kb) that you might find useful.
Anyone can be a carrier of sickle cell, but it's much more common in people from certain ethnic backgrounds.
People with family members originally from the following parts of the world are most at risk:
In the UK, most people who carry sickle cell have an African or Caribbean family background. It's estimated that around 1 in 10 people in this group may be a carrier.
Screening for sickle cell disease is offered to all pregnant women in England, although most women will be at low risk and won't need to have a blood test to check if they're a carrier.
Read more about screening for sickle cell disease in pregnancy.
Anyone can ask to have a free test to find out if they're a carrier at any point. This can be useful if:
You can request the test from your GP surgery or nearest genetic counsellor who will discuss the result and implications with you if you're found to carry sickle cell.
If you carry sickle cell, you're at risk of having children with sickle cell disease, although this can only happen if your partner is also a carrier or has sickle cell disease themselves.
If you're planning to have a child and you know you're a carrier, it's a good idea for your partner to be tested.
If you and your partner both carry sickle cell, there's a:
If both of you are carriers and you're planning to have a baby, talk to your GP about getting a referral to a genetic counsellor who can explain the risks to your children and what your options are.
PGD is similar to IVF, but the resulting embryos are tested to check that they don't have sickle cell disease before they're implanted in the womb. The Human Fertilisation and Embryology Authority (HFEA) has more information about PGD.
You're not at risk of developing sickle cell disease if you carry sickle cell.
The only time you may be at risk of health problems is in rare cases where you might not get enough oxygen, such as:
There's also a very small risk of developing kidney problems associated with carrying sickle cell.
Apart from these uncommon situations, you can lead a completely normal and healthy life if you're a sickle cell carrier.
People who are carriers of sickle cell are also at risk of having a child with a blood disorder if their partner is a carrier of a different type of blood disorder.
You can find more detailed information about some of the other types of carrier in the following leaflets: