Blood
Medicines for Sickle cell disease
An inherited blood disorder in which red blood cells become rigid and sickle-shaped and break down, causing anaemia and blocking small blood vessels — leading to episodes of severe pain (sickle cell crises) and a raised risk of infection, stroke and organ damage. It needs lifelong specialist care, including daily protective antibiotics, full vaccinations, medicines to reduce crises, pain relief and sometimes transfusions.
Education and reference only. This explains which medicines are used and why, in plain language — it deliberately contains no doses and is not a substitute for advice from your doctor or pharmacist. Always discuss your own treatment with a qualified clinician, and check the BNF and the product labelling for prescribing detail.
Quick answer
What is Sickle cell disease?
Sickle cell disease is an inherited condition affecting the red blood cells, which carry oxygen around the body. Normally these cells are round and flexible, letting them flow easily through even the smallest blood vessels.
- How it is treated: Care for sickle cell disease is lifelong and specialist-led, and aims both to prevent complications and to treat crises promptly when they happen.
- Self-care: Day-to-day measures help reduce how often crises happen and keep you as well as possible: drinking plenty of fluids to stay well hydrated, keeping warm and avoiding sudden chills (cold is a common trigger), resting and not over-exerting, and steering clear of things that can bring on a crisis such as dehydration, infection and extremes of temperature.
- When to seek help: People with sickle cell disease, and those caring for them, should know the warning signs that need urgent hospital care and act on them quickly — call 999 or go straight to A&E for a high fever (which can mean a serious infection, since the spleen works poorly), chest pain or breathlessness (which can signal a dangerous complication called acute chest syndrome), a severe pain crisis that home measures and your usual pain relief cannot control, or any signs of a stroke such as sudden weakness or numbness of the face, arm or leg, difficulty speaking, or a sudden severe headache.
What it is
Sickle cell disease is an inherited condition affecting the red blood cells, which carry oxygen around the body. Normally these cells are round and flexible, letting them flow easily through even the smallest blood vessels. In sickle cell disease, an abnormal form of haemoglobin (the oxygen-carrying protein) causes red cells to become rigid and shaped like a crescent or sickle. These misshapen cells break down faster than normal, which leads to anaemia, and they can clump together and block small blood vessels. When a blood vessel is blocked, the tissue it supplies is starved of oxygen, causing episodes of severe pain known as sickle cell crises, which can come on suddenly and may need hospital treatment. Because the spleen — an organ that helps fight certain infections — works poorly in sickle cell disease, there is a much higher risk of serious infection. Over time, repeated blockages and the strain of the condition can damage organs, and there is a raised risk of stroke. Sickle cell disease is present from birth, lifelong, and is looked after by specialist teams who help people stay as well as possible and prevent and treat complications.
How it is treated
Care for sickle cell disease is lifelong and specialist-led, and aims both to prevent complications and to treat crises promptly when they happen. A great deal of effort goes into prevention. Because the spleen works poorly, the risk of serious infection is high, so daily protective antibiotics (penicillin) are taken to guard against it, and a full set of vaccinations is given and kept up to date — these are central, not optional, parts of staying well. Crises are reduced by keeping well hydrated, staying warm, and avoiding known triggers such as cold, dehydration, infection and over-exertion. Hydroxycarbamide is a medicine used to reduce how often crises and other complications occur by changing the make-up of the blood. When a painful crisis does happen, strong pain relief, fluids and warmth are used, often in hospital for a severe episode. Blood transfusions are used in certain situations, including to treat or prevent serious complications such as stroke, and there are now newer therapies for sickle cell disease. Throughout, signs of serious infection, severe pain, breathing problems or stroke are treated as emergencies needing urgent hospital care.
For this condition, these medicines
Medicine classes used for Sickle cell disease
Each links to a full, dose-free guide — what it is, how it works, who can and cannot use it, side effects, interactions and FAQs.
Symptom checker
Symptoms that can point to Sickle cell disease
Sickle cell disease can be one cause of these symptoms. Each guide explains the other possible causes and the red-flag warning signs that mean you should get urgent help:
Beyond medication
Lifestyle and self-care
Day-to-day measures help reduce how often crises happen and keep you as well as possible: drinking plenty of fluids to stay well hydrated, keeping warm and avoiding sudden chills (cold is a common trigger), resting and not over-exerting, and steering clear of things that can bring on a crisis such as dehydration, infection and extremes of temperature. Taking your daily protective antibiotic and keeping right up to date with all your vaccinations are essential, because the spleen does not protect you well against infection. Carrying information about your condition, attending all your specialist appointments, and getting medical help quickly when you feel unwell or feverish are all important parts of living well with sickle cell disease.
When to get help
When to see a doctor
People with sickle cell disease, and those caring for them, should know the warning signs that need urgent hospital care and act on them quickly — call 999 or go straight to A&E for a high fever (which can mean a serious infection, since the spleen works poorly), chest pain or breathlessness (which can signal a dangerous complication called acute chest syndrome), a severe pain crisis that home measures and your usual pain relief cannot control, or any signs of a stroke such as sudden weakness or numbness of the face, arm or leg, difficulty speaking, or a sudden severe headache. Sudden swelling or pain in the tummy, a very pale or unusually tired and breathless child, or a painful, persistent erection in boys and men also need urgent assessment. For ongoing care, keep to your specialist appointments and contact your team if you are getting more frequent crises, and never delay seeking help if you feel feverish or unwell.
Not sure how urgent it is? It is always OK to call NHS 111 for advice, day or night.
Answers
Sickle cell disease: frequently asked questions
What medicines are used for sickle cell disease?
Several medicines are used together, mainly to prevent complications and to manage crises. A daily protective antibiotic — penicillin — is a central, lifelong part of care, taken to guard against the serious infections that people with sickle cell disease are prone to because the spleen works poorly; keeping all vaccinations up to date is just as important. Hydroxycarbamide is used to reduce how often painful crises and other complications happen. Strong pain relief is used to treat crises, often in hospital for severe episodes. Blood transfusions are used in certain situations, including to treat or prevent serious complications such as stroke, and newer therapies for sickle cell disease now exist too. Your specialist team decides the right combination for you.
What is a sickle cell crisis?
A sickle cell crisis is an episode of severe pain that happens when sickle-shaped red blood cells clump together and block small blood vessels, starving the tissue beyond the blockage of oxygen. The pain can come on suddenly and be felt in the bones, joints, back, chest or tummy, and it can range from manageable at home to severe enough to need hospital treatment with strong pain relief and fluids. Crises can be triggered by things such as cold, dehydration, infection, over-exertion or stress, which is why staying warm, well hydrated and avoiding triggers matters. If a crisis is severe and your usual measures and pain relief are not controlling it, you should seek urgent hospital care.
Why are daily antibiotics and vaccinations so important?
In sickle cell disease the spleen — an organ that helps the body fight certain bacteria — works poorly, which leaves people at much higher risk of serious, sometimes life-threatening infections. To protect against this, a daily protective dose of penicillin is taken long term, and a full, up-to-date set of vaccinations is given. These are central, not optional, parts of staying well, and they are why a fever in someone with sickle cell disease is always taken seriously and treated as a possible emergency. Taking the daily antibiotic, keeping vaccinations current, and seeking help quickly if you feel feverish are some of the most important things you can do to stay safe.
Is sickle cell disease passed on to children?
Yes — sickle cell disease is inherited, meaning it is passed down through genes from parents to children. A child develops the disease only if they inherit an affected gene from both parents. Someone who inherits the gene from just one parent has what is called sickle cell trait: they are usually well and do not have the disease, but they can pass the gene on. This is why couples are offered information and testing about their carrier status, particularly when planning a family, so they understand the chances of having a child with the condition. A specialist or genetic counsellor can explain what your own situation means and discuss the options available.
Keep reading
Related articles
Sources
Where this is drawn from
- Sickle Cell Society: About sickle cell.
- NICE CKS: Sickle cell disease.
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