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 – it’s 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 you’re a sickle cell carrier

Symptoms

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 isn’t 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 can’t contact your GP or care team, go to your closest Accident and Emergency (A&E) department. Dial 999 for an ambulance if you aren’t able to travel yourself.

It’s 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 aren’t 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 – it’s the substance that transports oxygen around the body. People with anaemia have low levels of haemoglobin in their blood.

Most of the time anaemia doesn’t cause symptoms, but if you’re 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

There’s 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 you’re 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 doesn’t 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 there’s 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 baby’s parents weren’t 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