Sickle cell disease

Sickle cell disease | NHS inform Home Illnesses and conditions Symptoms and self-help Tests and treatments Healthy living Care, support and rights Scotlands Service Directory 0 Home Illnesses and conditions Blood and lymph Sickle cell disease Sickle cell disease About sickle cell disease \u200bSymptoms and diagnosis \u200bTreating sickle cell disease \u200bLiving with sickle cell disease \u200bCauses of sickle cell disease \u200bSickle cell disease carriers and screening 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: African Asian Caribbean Eastern Mediterranean Middle Eastern There are treatments to manage sickle cell disease, but no cure yet  its 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 youre a sickle cell carrier \u200bSymptoms 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 isnt 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 cant contact your GP or care team, go to your closest Accident and Emergency (A&E) department. Dial 999 for an ambulance if you arent able to travel yourself. Its 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 spine pelvis tummy 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. Infection Sickle cell disease leaves people  especially children  extra vulnerable to infection because their bodies arent 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. Anaemia Almost everyone with sickle cell disease has anaemia. Haemoglobin is found in red blood cells  its the substance that transports oxygen around the body. People with anaemia have low levels of haemoglobin in their blood. Most of the time anaemia doesnt cause symptoms, but if youre infected with parvovirus, the virus that causes slapped cheek syndrome, the anaemia can get worse. When that happens, it causes: headaches a fast heartbeat fainting dizziness 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 Theres 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 youre 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 doesnt 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 theres 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 babys parents werent 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 \u200bTreating 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 childs 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 its 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. Dont 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 youre at high altitude for any reason. However, plane travel shouldnt 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. Dont 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 youll need regular blood tests to make sure youre 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, youll 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 youre 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 youre 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. Treating anaemia Most of the time, anaemia doesnt have any symptoms and you wont 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. Its important to be aware that this type of anaemia isnt the same as the anaemia caused by iron deficiency, so dont 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. Learn more about living with sickle cell disease here \u200bLiving 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 youre due to have surgery, or youre brought in for surgery in an emergency, its 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. Its important that youre 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 its a good idea to talk to your care team before planning a pregnancy, for a number of reasons: theres a bigger chance of complications such as miscarriage, pre-eclampsia, anaemia and sickle cell pain while youre 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 Its important to use a reliable form of contraception if you arent 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 its still a serious condition. Sickle cell disease can have a big impact on someones 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, its hoped that the outlook will get better. \u200bCauses of sickle cell disease Sickle cell disease is caused by a gene problem that is inherited from your parents. It isnt something you can catch, and its 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 dont have sickle cell disease, but if their partner is a carrier too they may have a child with the condition. When both of a childs parents are sickle cell carriers: theres a 25% (one in four) chance that each of their children wont inherit any faulty genes  they wont have sickle cell disease, and they wont be able to pass it on to their children theres 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 theres 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 theyre born You can find more information about how sickle cell disease is inherited from the Sickle Cell Society . Learn more about sickle cell carriers and screening \u200bSickle 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 wont develop sickle cell disease. However, theres a risk that youll 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 : Africa Asia The Caribbean The Eastern Mediterranean The Middle East Most sickle cell carriers in the UK have an African or Caribbean family background  its 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 youre 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 doesnt suggest your child will have sickle cell disease. Its 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 theres 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 Youre 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 knowyou 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 youre never short of oxygen during regular, intensive physical activity  make sure you drink plenty of fluids during training and avoid extreme exhaustion Theres 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 youre 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: Your baby carries a gene for Sickle Cell Your baby carries a gene for unusual haemoglobin Source: NHS 24 - Opens in new browser window Last updated: 12 April 2023 How can we improve this page? Help us improve NHS inform Thank You Your feedback has been received Dont include personal information e.g. name, location or any personal health conditions. Email Address e.g. you@example.com Message Maximum of 500 characters Send feedback Add this page to\n            Info For Me NHS inform About NHS inform Editorial policy Contact us Webchat Give feedback about NHS inform Info for Me tool Terms and conditions Privacy and cookies policy Freedom of information (FOI) Accessibility Other languages and formats  2023 NHS 24  v1.1.1.17852