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Overview
Sickle cell disease is the name for a group of inherited health conditions that affect the red blood cells. The most serious type is called sickle cell anaemia.
Sickle cell disease is 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 do not live as long as healthy blood cells and can block blood vessels.
Sickle cell disease is a serious and lifelong health condition, although treatment can help manage many of the symptoms.
Symptoms
People born with sickle cell disease tend to have problems from early childhood, although some children have few symptoms and lead normal lives most of the time.
The main symptoms of sickle cell disease are:
- painful episodes called sickle cell crises, which can be very severe and last for days or weeks
- an increased risk of serious infections
- anaemia (where red blood cells cannot carry enough oxygen around the body), which can cause tiredness and shortness of breath
Sickle cell disease can also sometimes cause a wide range of other problems.
These include:
- delayed growth during childhood and delayed puberty
- gallstones, which can cause tummy (abdominal) pain and yellow skin and eyes (jaundice)
- bone and joint pain
- a persistent and painful erection of the penis (priapism), which can sometimes last several hours
- painful open sores on the lower legs (leg ulcers)
- strokes or transient ischaemic attacks, where the flow of blood to the brain is blocked or interrupted
- a serious lung condition called acute chest syndrome, which can cause a fever, cough, chest pain and breathing difficulties
- swelling of the spleen, which can cause shortness of breath, a rapid heartbeat, tummy pain, a swollen tummy and anaemia
- eyesight problems, such as floaters, blurred or patchy vision, reduced night vision and occasionally sudden vision loss
- high blood pressure in the blood vessels that carry blood from the heart to the lungs (pulmonary hypertension)
- kidney or urinary problems, including blood in the urine and bedwetting
Causes
Sickle cell disease is caused by a gene that affects how red blood cells develop.
If both parents have the gene, there's a 1 in 4 chance of each child they have being born with sickle cell disease.
The child's parents often will not have sickle cell disease themselves and they're only carriers of the sickle cell trait.
Diagnosis
Sickle cell disease is usually detected during pregnancy or soon after birth.
Testing for sickle cell carriers
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.
Treatment Options
People with sickle cell disease usually need lifelong treatment. This is usually delivered by different health professionals in a specialist sickle cell centre.
A number of treatments for sickle cell disease are available. These include:
- painkillers, such as paracetamol or ibuprofen during a sickle cell crisis (sometimes treatment with stronger painkillers in hospital may be necessary)
- daily antibiotics and having regular vaccinations to reduce your chances of getting an infection
- a medicine called hydroxycarbamide (hydroxyurea) to reduce episodes of pain
- dietary supplements or blood transfusions for by sickle cell-related anaemia
- stem cell or bone marrow transplant - the only cure for sickle cell disease is a stem cell or bone marrow transplant, but they're not done very often because of the risks involved.
It's also important for people with sickle cell disease to look after their own health using self care measures, such as by avoiding triggers and managing pain. You may be advised to:
- drink plenty of fluids and staying warm to prevent painful episodes
- wear warm clothing to stop you getting cold
- avoid sudden temperature changes, such as swimming in cold water
When To See A Doctor
Make sure you know when to get medical advice and where to go, as sickle cell disease can cause a number of serious problems that can appear suddenly.
Problems to look out for include:
- a high temperature over 38C (or any increased temperature in a child)
- severe pain that's not responding to treatment at home
- severe vomiting or diarrhoea
- a severe headache, dizziness or a stiff neck
- breathing difficulties
- very pale skin or lips
- sudden swelling in the tummy
- a painful erection (priapism) lasting more than 2 hours
- confusion, drowsiness or slurred speech
- fits (seizures)
- weakness on 1 or both sides of the body
- changes in vision or sudden vision loss
Contact your GP or healthcare team straight away if you develop any of these symptoms.
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