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About sickle cell disease
Sickle cell disease is the name for a group of blood disorders. The most severe is sickle cell anaemia. These disorders are inherited, meaning they are passed on through your genes.
You can have a blood test at any time to find out if you carry the gene for sickle cell disease, or if you have sickle cell disease.
Sickle cell disease affects how your body produces red blood cells. Normal red blood cells are round – red blood cells affected by sickle cell disease harden and become sickle-shaped, like a crescent moon. This causes them to die too quickly and block blood vessels, leading to symptoms that are often painful.
People from particular ethnic backgrounds are more likely to have sickle cell disease or carry the gene. You are more likely to have sickle cell disease if you are from one of these ethnic backgrounds:
- Eastern Mediterranean
- Middle Eastern
There are treatments to manage sickle cell disease, but no cure yet – it's a lifelong condition.
Find out about symptoms and diagnosis
Find out about treating sickle cell disease
Find out about living with sickle cell disease
Find out about screening to see if you're a sickle cell carrier
Symptoms and diagnosis
The main symptoms of sickle cell disease are sickle cell crises (very painful episodes affecting different parts of the body), infections, and anaemia.
Getting immediate medical advice
There are a number of serious problems that can appear suddenly as a result of sickle cell disease. If you experience any of the following symptoms, you should get medical advice immediately:
- high temperature (a fever) going to 38C (100.4F) or higher
- difficulty breathing
- drowsiness, confusion, or slurred speech
- a severe headache, stiff neck, or dizziness
- skin or lips that are very pale
- fits (seizures)
- serious pain that isn't responding to treatments at home
- sudden swelling in the tummy
- priapism – a painful erection lasting two hours or more
- weakness on one or both sides of your body
- sudden vision loss, or changes in your vision
If you develop any of the symptoms listed above, phone your GP or care team immediately. If you can't contact your GP or care team, go to your closest Accident and Emergency (A&E) department. Dial 999 for an ambulance if you aren't able to travel yourself.
It's important to make sure that the medical team looking after you know that you have sickle cell disease.
Sickle cell crises
Episodes of pain known as sickle cell crises happen when the blood vessels that go to one part of your body become blocked. The pain can be severe.
Sickle cell crises are one of the most common symptoms of sickle cell disease and, on average, occur once a year and last up to seven days. Some people have sickle cell crises every few weeks – others experience them less than once a year.
A sickle cell crisis typically affects one part of the body at a time. The most common parts affected are:
- hands or feet (this is especially common in young children)
- ribs and breastbone
- legs and arms
Sickle cell crises have different causes. They can be triggered by cold, rainy or windy weather, stress, dehydration, or exercise that leaves you severely out of breath.
Sickle cell disease leaves people – especially children – extra vulnerable to infection because their bodies aren't as effective at dealing with bacteria and viruses. This leaves them more vulnerable to viruses like the common cold, as well as severe and potentially life-threatening conditions like meningitis.
As well as keeping up to date with their vaccinations, many people with sickle cell disease take daily doses of antibiotics in order to prevent infections.
Almost everyone with sickle cell disease has anaemia. Haemoglobin is found in red blood cells – it's the substance that transports oxygen around the body. People with anaemia have low levels of haemoglobin in their blood.
Most of the time anaemia doesn't cause symptoms, but if you're infected with parvovirus, the virus that causes slapped cheek syndrome, the anaemia can get worse. When that happens, it causes:
- a fast heartbeat
If these symptoms occur, the anaemia is usually treated with a blood transfusion.
Sudden anaemia can happen in young children if they experience swelling of the spleen. This is also treated with a blood transfusion.
Other sickle cell disease symptoms
There's a wide range of other issues that can be caused by sickle cell disease. These include:
- a serious lung condition called acute chest syndrome that can cause chest pain, a cough, breathing difficulties and fever
- pain in bones and joints
- delayed puberty
- delayed growth in childhood
- kidney or urinary problems, including bedwetting and blood in the urine
- gallstones – these are stones in the gallbladder that can cause jaundice (yellow eyes and skin) and abdominal (tummy) pain
- ulcers on the lower legs (open sores that can be very painful)
- priapism – a painful, persistent erection that can last for several hours
- high blood pressure (pulmonary hypertension)
- transient ischaemic attacks (TIAs) or strokes – these happen when blood flow to the brain is interrupted or blocked
- enlarged spleen – this can cause a fast heartbeat, worsening of anaemia, a swollen tummy, shortness of breath and abdominal pain
- vision problems like floaters, worsened night vision, blurred vision, patchy vision and, occasionally, sudden vision loss
These symptoms can be treated – see Treating sickle cell disease for more information.
Diagnosing sickle cell disease
You can have a blood test any time to find out if you have sickle cell disease, or find out if you're a carrier who could have a child with the condition.
Sickle cell disease is usually diagnosed during pregnancy – free screening to find out if a baby is at risk of having the condition is offered to every pregnant woman in Scotland.
Even if your family background doesn't make it likely your child will have sickle cell disease, you can still request a test.
If possible, screening should be done before the tenth week of pregnancy, so that there's time to think about further tests that can find out if your baby will be born with sickle cell disease.
Newborn babies are screened for sickle cell disease as part of the newborn blood spot test – the heel prick test. There are a few reasons for this:
- if a baby's parents weren't screened during pregnancy, this test identifies if the child has sickle cell disease
- if screening during pregnancy showed that the baby had a high risk of having sickle cell disease, but no more testing was done, the heel prick test will determine if the child has the condition or not
- this test shows if the baby is a carrier of sickle cell, and could one day have children of their own who have the condition (find out more about sickle cell carriers here)
- the heel prick test identifies other inherited conditions, like cystic fibrosis
Another blood test will be used to confirm the diagnosis if the heel prick test suggests that the baby could have sickle cell disease.
Learn about being tested to see if you carry the sickle cell gene
Treating sickle cell disease
People of all ages with sickle cell disease are supported by a team of medical professionals in a specialist sickle cell centre. A specialised care plan will be developed with you that helps you to fully understand the condition and find the best way of managing it.
Treating and preventing sickle cell crises
Sickle cell crises can usually be managed at home. The following steps are recommended for adults or children experiencing a sickle cell crisis:
- over the counter painkillers like paracetamol and ibuprofen can help – although aspirin should not be given to anyone under 16 – and if needed, your GP may prescribe stronger painkillers
- heating pads or warm towels can be placed on the area and massaged to ease the pain – pharmacies usually sell heating pads you can use for this
- drink plenty – staying hydrated will help
- relaxing distractions like reading, videos, and computer games are a good way to distract your mind, or your child's mind, from the pain
If none of the above measures help, or the pain is very severe, phone your GP. You or your child should go to the local accident and emergency (A&E) if it's not possible to contact your GP. It may be necessary for you or your child to spend a few days in hospital being treated with stronger pain relief.
The best way to prevent a sickle cell crisis is by being aware of potential triggers and avoiding them.
- Prevent dehydration by drinking plenty of fluids –especially water.
- Avoid alcohol – alcohol causes dehydration.
- Stay warm with enough layers of clothing to avoid getting cold, and avoid extreme temperatures.
- Don't expose yourself to sudden changes in temperature – for example, avoid swimming in cold water.
- The lack of oxygen at high altitudes can trigger a sickle cell crisis, so be careful if you're at high altitude for any reason. However, plane travel shouldn't be an issue because planes maintain a consistent oxygen level due to being pressurised.
- Make sure to stay active, but avoid activities that leave you severely out of breath.
- Don't smoke – smoking can trigger acute chest syndrome.
- Stay relaxed as much as you can – sickle cell crises can be triggered by stress, so consider learning relaxation techniques like breathing exercises.
Your care team may recommend a medication called hydroxycarbamide (hydroxyurea) if you keep experiencing sickle cell crises. This is a capsule that is taken once a day. It works by reducing how many other types of blood cell, like white blood cells and platelets (clotting cells) there are in the body.
This means that you'll need regular blood tests to make sure you're healthy.
Treating and preventing infections
Children who have sickle cell disease should be kept up to date on all of their routine vaccinations, and may also need additional vaccinations like the hepatitis B vaccine and the annual flu vaccine.
If you have sickle cell disease, you'll most likely need to take a dose of antibiotics every day, usually penicillin. This will not pose serious health risks.
Reduce risk in day-to-day life by being careful about activities that can make you vulnerable to infection. Always follow good food hygiene measures, for example.
If you're planning to go abroad, talk to your GP as far in advance as you can. You might need extra vaccinations or medication, such as anti-malarials if you're going to a place where malaria is a risk. It may also be a good idea to take extra precautions when it comes to food and water.
Most of the time, anaemia doesn't have any symptoms and you won't require any treatment for it. However, children with sickle cell anaemia who are also on a restricted diet (such as a vegan or vegetarian diet) may need folic acid supplements. Folic acid helps your body to create more red blood cells, so it can help to improve anaemia.
It's important to be aware that this type of anaemia isn't the same as the anaemia caused by iron deficiency, so don't take iron supplements without talking to your care team first. Taking iron supplements with the anaemia caused by sickle cell disease can be dangerous.
Blood transfusions or hydroxycarbamide may be necessary for serious or persistent anaemia.
Treating other sickle cell disease-related issues
There are a range of treatments for other problems that can be caused by sickle cell disease, depending on the type of symptom and how severe it is:
- people with acute chest syndrome need emergency treatment – this involves oxygen, blood transfusions, antibiotics, and fluids given through a vein, as well as possibly hydroxycarbamide to prevent it happening again
- painkillers can treat joint and bone pain
- a short course of hormones can be given to children in order to trigger delayed puberty
- gallbladder removal surgery is used to treat gallstones
- for persistent priapism, medication can stimulate blood flow, or a needle can be used to drain blood from the penis
- regular blood transfusions or hydroxycarbamide are given to people who have had a stroke or who are at greater risk of having one
In addition to the treatments above, chelation therapy involves taking medication to make the level of iron in your blood lower, bringing it to safe levels. This treatment is given to people who need a lot of blood transfusions.
Living with sickle cell disease
Sickle cell disease is a lifelong illness, which can be managed well with treatment and self-help. In addition to preventing symptoms and crises as much as possible, there are a few things you can do that will make it easier to live with the condition and stay in the best possible health.
Getting surgery when you have sickle cell disease
If you're due to have surgery, or you're brought in for surgery in an emergency, it's very important you take some precautions.
You should tell your care team about any surgeries you have planned that involve a general anaesthetic.
Tell your surgeon on the day that you have sickle cell disease.
General anaesthetic can cause a number of problems, including increasing the risk of a sickle cell crisis. It's important that you're monitored closely during the surgery, and are given plenty of fluids as well as being kept warm.
It might be necessary to have a blood transfusion before the surgery to reduce the chances of complications.
Pregnancy and birth with sickle cell disease
You can have a healthy pregnancy if you have sickle cell disease, but it's a good idea to talk to your care team before planning a pregnancy, for a number of reasons:
- there's a bigger chance of complications such as miscarriage, pre-eclampsia, anaemia and sickle cell pain while you're pregnant
- you may have to stop taking some of the medications for sickle cell disease, like hydroxycarbamide, before getting pregnant, as these can harm a foetus
- it may be a good idea to find out if your partner is a sickle cell carrier and talk with a counsellor about what this could mean
- extra monitoring during pregnancy, as well as extra treatment, might be necessary
It's important to use a reliable form of contraception if you aren't planning a pregnancy.
Learn about pregnancy and newborn screening for sickle cell disease
The outlook for people with sickle cell disease
Most children with sickle cell disease lead normal, happy lives. However, sickle cell disease can be very different from person to person, and it's still a serious condition.
Sickle cell disease can have a big impact on someone's life. It can lead to serious infections, strokes and transcient ischaemic attacks (TIAs), and lung problems, which can be fatal.
The overall live expectancy for people with sickle cell disease is shorter than normal, but this changes depending on the type of sickle cell disease, what problems they experience, and how the condition is treated.
Milder types of sickle cell disease may not impact life expectancy. People with sickle cell anaemia have a typical life expectancy of between 40 and 60 years of age. As newer treatments are developed, it's hoped that the outlook will get better.
Causes of sickle cell disease
Sickle cell disease is caused by a gene problem that is inherited from your parents. It isn't something you can catch, and it's not caused by anything the parents did during or before pregnancy.
Inheriting sickle cell disease
You inherit one set of genes from your mother, and one from your father. Genes always come in pairs. Children born with sickle cell disease inherit a copy of the sickle cell gene from both of their parents, so each one of the pair of genes is faulty.
This happens when both parents are "carriers" – they carry the faulty gene for sickle cell. This is also known as having the sickle cell trait.
Carriers don't have sickle cell disease, but if their partner is a carrier too they may have a child with the condition.
When both of a child's parents are sickle cell carriers:
- there's a 25% (one in four) chance that each of their children won't inherit any faulty genes – they won't have sickle cell disease, and they won't be able to pass it on to their children
- there's a 50% (one in two) chance that each child will inherit a copy of the faulty gene from just one parent, and be a sickle cell carrier
- there's a 25% (one in four) chance that each child will inherit copies of the sickle cell gene from both of their parents, and have sickle cell disease when they're born
You can find more information about how sickle cell disease is inherited from the Sickle Cell Society.
Sickle cell disease carriers and screening
Sickle cell disease carriers are people who carry the faulty gene that causes sickle cell disease, but do not have the disease. This is also called having the sickle cell trait.
If you have the sickle cell trait, you won't develop sickle cell disease. However, there's a risk that you'll have a child with the condition, or who is also a sickle cell disease carrier.
Sickle cell disease carriers
The sickle cell gene can be carried by anyone, but people from certain ethnic backgrounds are much more likely to be carriers.
You are more likely to be a carrier if you have family from one of these areas :
- The Caribbean
- The Eastern Mediterranean
- The Middle East
Most sickle cell carriers in the UK have an African or Caribbean family background – it's estimated around one in ten people with this background may be a sickle cell carrier.
Finding out if you are a sickle cell carrier
You can request a blood test from your GP at any time to find out if you're a sickle cell carrier. This is a particularly good idea if your family background makes it more likely that you have the gene.
Both men and women are able to get the test.
Every pregnant woman in Scotland is offered screening to check if their baby is at risk of being born with sickle cell disease. You can ask for a test even if your family background doesn't suggest your child will have sickle cell disease. It's a good idea to have your partner screened for the gene too.
If possible, screening should be done before the tenth week of pregnancy, so that there's time to think about further tests that can find out if your baby will be born with sickle cell disease.
Read more about diagnosis and screening for the sickle cell disease gene here
Other health risks for sickle cell carriers
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:
- having surgery under general anaesthetic – make sure your medical team know you carry sickle cell before your operation so they can ensure you get enough oxygen
- during extreme sports such as deep sea diving and climbing at high altitudes – if you do sports like these, ensure you're never short of oxygen
- during regular, intensive physical activity – make sure you drink plenty of fluids during training and avoid extreme exhaustion
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.
Other blood disorders
Sickle cell carriers 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 pages:
18 January 2023
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