28/09/2024
How sickle cell disease affects the body
Your genes are the set of instructions found inside every cell in your body. They determine characteristics like the colour of your eyes and hair.
People with sickle cell disease do not make haemoglobin properly. Haemoglobin is a substance in red blood cells, which carries oxygen around the body.
Normal red blood cells are flexible and disc-shaped, but in sickle cell disease, they can become rigid and shaped like a crescent or sickle because the haemoglobin inside them clumps together.
These unusual cells can cause symptoms of sickle cell disease because they do not live as long as normal red blood cells and can become stuck in blood vessels.
Sickle cell disease can cause a wide range of symptoms.
These can start from a few months of age, although many children have few or no symptoms if treatment is started early on.
The main symptoms are:
painful episodes
getting infections often
anaemia
Painful episodes
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 last for several days or weeks.
A sickle cell crisis can affect any part of the body, but is most common in the limbs or back.
How often someone with sickle cell disease gets episodes of pain varies a lot.
Some people may have one every week, while others may have less than 1 a year.
It's not always clear what triggers pain, but sometimes painful episodes can be caused by the weather (such as wind, rain or cold), dehydration, stress or strenuous exercise.
Infections
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.
Anaemia
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 can lead to a sudden drop in the number of red blood cells and may cause additional symptoms, such as headaches, a rapid heartbeat, dizziness and fainting.
It's usually treated with a blood transfusion.
Other problems
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 er****on of the p***s (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
