Sickle Cell Disease
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Understanding sickle cell disease
Sickle cell disease is a serious and lifelong condition that’s particularly common in people with an African or Caribbean family background. The most serious type of sickle cell disease is called sickle cell anaemia. Sickle cell disease varies from mild to serious, but most people who have it can lead a happy, normal life. Sickle cell disease is the name for a group of inherited health conditions that affect the red blood cells.
If you have any of the following symptoms, contact your GP or healthcare team immediately. If this isn’t possible, go to your nearest A&E or call 999 for an ambulance:
- Temperature over 38C (or any increased temperature in a child)
- Extreme pain that you’re not able to manage at home
- Vomiting or diarrhoea
- Severe headache, dizziness or a stiff neck
- Breathing difficulties
- Pale skin or lips
- Swollen tummy
- Painful erection that lasts more than two hours
- Confusion, drowsiness or slurred speech
- Having fits
- Weakness on one or both sides of the body
- Vision changes or sudden vision loss
Symptoms
Sickle cell disease can cause a wide range of symptoms. Symptoms can appear from a few months of age, although with early treatment many children have few or no symptoms. Currently the only cure for sickle cell disease is stem cell or bone marrow transplants, but these procedures come with significant risks, so they aren’t done very often.
The main symptoms of sickle cell disease include:
Painful episodes
Painful episodes happen when blood vessels become blocked. These pain episodes are known as sickle cell crises and can last for several days or weeks. A sickle cell crisis can affect any part of the body, but is most common in the arms, legs and back.
How often painful episodes occur can vary from person to person. Some people may have a sickle cell crisis every week, whereas others may have less than one painful episode a year.
It can be unclear what causes painful episodes in people with sickle cell disease, but a sickle cell crisis can sometimes be brought on by extreme weather (wind, rain and cold), dehydration, everyday stress or strenuous exercise.
Regularly getting infections
People with sickle cell disease, especially those who are young, are more susceptible to getting infections. These can range from mild infections, such as colds, to more serious and potentially life-threatening infections, such as meningitis.
People with sickle cell disease can reduce their risk of infections by keeping up with vaccinations. Your GP may also recommend a daily dose of antibiotics to help reduce the risk of infections. If you're interested in learning more about meningitis, we've got you covered.
Anaemia
Most people with sickle cell disease have anaemia, while this won’t necessarily cause many symptoms itself, it may get worse if someone with sickle cell disease becomes infected with parvovirus, which is the virus that causes slapped cheek syndrome (a bright red rash that appears on both cheeks).
This can cause the number of red blood cells to drop, which may bring additional symptoms such as headache, rapid heartbeat, dizziness and fainting,
If you have sickle cell disease and become infected with parvovirus, your healthcare professional may choose to treat this with a blood transfusion.
Sickle cell disease can sometimes be related to other problems, including:
- Complications in pregnancy
- Delayed growth during childhood and delayed puberty
- Gallstones, which may cause stomach pain and yellow skin and eyes (known as jaundice)
- Bone and joint pain
- A painful erection, which can sometimes last several hours
- Leg ulcers
- Strokes or problems relating to the flow of blood to the brain
- A serious lung condition called acute chest syndrome – this may cause a fever, cough, chest pain and breathing difficulties
- Swelling of the spleen, may cause shortness of breath, a rapid heartbeat, stomach pain, a swollen stomach and anaemia
- Eyesight problems, may include floaters, blurred or patchy vision, reduced night vision and, less commonly, sudden vision loss
- High blood pressure
- Kidney or urinary problems, this may include blood in the urine and bedwetting
Causes
Sickle cell disease is a genetic condition, which means it’s caused by inheriting a copy of the sickle cell gene from both parents, who would need to be carriers of the sickle cell gene. You can also inherit sickle cell disease if one parent has sickle cell disease and the other parent is a carrier of it. You can’t catch sickle cell disease from someone else or develop it later in life.
Who can be a carrier of sickle cell disease?
Anyone can be a carrier of sickle cell, but it's more common in people from certain ethnic backgrounds. In the UK, most people who carry the sickle cell trait have an African or Caribbean family background. If you have a family history of sickle cell disease, but you don't have it yourself, you may be a carrier. This is also known as having the sickle cell trait.
Sickle cell carriers won't develop sickle cell disease but may be at risk of having a child with sickle cell disease if their partner is also a carrier. You can find out if you're a carrier of sickle cell by having a simple blood test, which will be carried out routinely during pregnancy and after birth. You can also request a blood test at any age to check if you're a sickle cell carrier. If you or your partner have the sickle cell trait and are thinking of trying for a baby, it's a good idea to speak to your GP first.
Management & treatment
Living with sickle cell disease
Sickle cell disease is a lifelong condition and requires long-term management and treatment. If you have sickle cell disease, your healthcare professional will be able to help create an individual care plan that’s right for you.
You can manage a sickle cell crisis at home by:
- Taking over-the-counter painkillers, such as paracetamol or ibuprofen, if suitable for you
- Drinking plenty of fluids to stay hydrated, check out our tips on drinking more water
- Using a warm towel or heated pad like the Beurer HK44 Soft Cosy Heat Pad to gently soothe the affected area
- Distracting yourself to help take your mind off the pain, reading, watching a film or playing a computer game may all help
Contact your GP or healthcare professional if these measures don’t help or if the pain is too severe, as they may be able to provide stronger painkillers. If you can’t speak to your GP or healthcare professional, for example if it’s out of hours, go to your nearest A&E.
You may be able to help reduce your risk of sickle cell pain by avoiding possible triggers. It may be a good idea to:
- Stay hydrated by drinking plenty of fluids
- Avoid getting cold by wearing warm clothing
- Avoid sudden temperature changes, such as swimming in cold water
- Be careful at high altitudes (lack of oxygen may trigger a sickle cell crisis)
- Keep active, but avoid intense exercise that leaves you severely out of breath
- Avoid alcohol and stop smoking if you do smoke. If you’re thinking of quitting smoking, our Stop Smoking Treatment Service22can help
- Relax, as everyday stress can trigger a sickle cell crisis – check out these four relaxation exercises to help you unwind
You can have a healthy pregnancy even if you’ve got sickle cell disease, but it’s important to speak to your healthcare team for advice if you’re planning on getting pregnant.
Some sickle cell disease medications can be harmful to unborn babies, so your healthcare professional may advise you to stop taking them before trying for a baby. Speak to your healthcare team for advice.
If you have sickle cell disease, you may need extra monitoring and treatment during pregnancy to help prevent problems such as anaemia, sickle cell pain, miscarriage and pre-eclampsia, as there’s an increased risk of these.
If you have sickle cell disease or know you’re a carrier of sickle cell disease and are planning on trying for a baby you should discuss this together with your healthcare team or a professional called a genetic counsellor.
If you have sickle cell disease and aren’t planning on getting pregnant, you should use a reliable form of contraception, such as condoms.
All pregnant women are offered screening for sickle cell disease, to check if there’s a risk of their baby being born with the condition. In England, all newborn babies are offered screening as part of the routine newborn heel prick test. This test can help to:
- Determine whether your baby has sickle cell disease
- Show if your baby is a carrier of sickle cell disease
- Identify if your baby has certain other rare conditions, like cystic fibrosis
Children with sickle cell disease may be thinner and slightly shorter than children who don’t have the condition, but they usually still grow at a steady rate. Children with sickle cell disease may go through puberty later than average, but they continue to grow for longer to eventually reach their normal adult height.
If your child has sickle cell disease, you can help prevent complications by:
- Helping them stay hydrated, as dehydration can make cells more likely to sickle
- Avoiding extremes of temperature, especially the cold, as this can also trigger cells to sickle
- Avoiding high altitudes where there is less oxygen
- Encouraging them to avoid very vigorous exercise where they get extremely out of breath, as this can decrease the amount of oxygen that can get to the blood
- Regular hand washing
- Avoiding sick people where possible
- Ensure they get all recommended vaccinations
Your child's doctor or medical team can help you understand key warning signs to watch out for.
Seek medical help immediately if you notice any of the following:
- Increased temperature in a child
- Extreme pain that's not easing with treatment at home
- Vomiting or diarrhoea
- Severe headache, dizziness or stiff neck
- Breathing difficulties
- Pale skin or lips
- Swelling of the stomach
- Confusion or drowsiness (including slurred speech)
- Fits
- Weakness on one or both sides of the body
- Vision changes or sudden vision loss
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Frequently Asked Questions
Sickle cell disease is a genetic disease, which means it’s inherited and present from birth. Children begin showing symptoms at around a few months of age, although many children have few or no symptoms if treatment is started early on.
Symptoms depend on how severe the sickle cell disease is – some people may find it has little or no effect on their daily life, whereas some people may find their symptoms get worse or become more difficult to manage with age.
Mild sickle cell disease may have little or no impact on a person’s day-to-day life however, someone with more serious sickle cell disease may notice it has a significant effect on their life.
Sickle cell disease can lead to health problems such as strokes, serious infections and lung problems, which can be fatal. Overall, the life expectancy for someone with sickle cell disease may be shorter than normal, but this depends on the exact type of sickle cell disease they have, how it’s treated and what problems they encounter.
Sickle cell disease can affect anyone, but in the UK, sickle cell disease is particularly common in people with an African or Caribbean family background.
You can’t catch sickle cell disease from someone who has it, and you can’t develop the condition later in life. Sickle cell disease is a genetic condition that’s present at birth.
This usually happens when both parents are carriers of the sickle cell gene (known as the sickle cell trait). It can also happen when one parent has sickle cell disease and the other parent has the sickle cell trait.
Carriers of the sickle cell gene don’t show symptoms, as they don’t have sickle cell disease themselves, but there is a chance they could have a child with sickle cell disease if their partner is also a carrier or has sickle cell disease themselves.
Stem cell and bone marrow transplants are currently the only cure for sickle cell disease, but these aren’t done very often because of the significant risks involved. These procedures are usually only considered when the long-term benefits outweigh the risks involved, for example in children with severe symptoms who aren’t responding to other treatments.
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