Episodes of pain known as sickle cell crises are one of the most common and distressing symptoms of sickle cell disease.
They happen when blood vessels to part of the body become blocked.
The pain can be severe and lasts for up to 7 days on average.
A sickle cell crisis often affects a particular part of the body, such as the:
- hands or feet (particularly in young children)
- ribs and breastbone
- legs and arms
How often someone with sickle cell disease gets episodes of pain varies a lot.
Some people may have one every few weeks, while others may have less than 1 a year. The average is 1 bad episode a year.
It's not always clear what triggers bad pain, but sometimes painful episodes can be caused by the weather (such as wind, rain or cold), dehydration, stress or strenuous exercise.
People with sickle cell disease are more vulnerable to infections, particularly when they're young.
Infections can range from mild, such as colds, to much more serious and potentially life threatening, such as meningitis.
Vaccinations and daily doses of antibiotics can help reduce the risk of many infections.
Nearly all people with sickle cell disease have anaemia, where the haemoglobin in the blood is low.
Haemoglobin is the substance found in red blood cells that's used to transport oxygen around the body.
This does not usually cause many symptoms, but sometimes it can get worse if you become infected with the virus that causes slapped cheek syndrome (parvovirus).
It's usually treated with a blood transfusion.
Sickle cell disease can also sometimes cause a wide range of other problems.
- 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