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

Several serious problems 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.

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