Sickle Cell Anaemia

Normal red blood cells are shaped like a doughnut, but if you have Sickle Cell Anaemia yours will be shaped differently. Find out the facts about the treatment and causes of sickle cell anaemia here.

What is Sickle Cell Anaemia?

Sickle Cell Anaemia is an inherited disorder affecting the haemoglobin in your red cells. It commonly affects people of Afro-Caribbean, Middle Eastern and Mediterranean origin. Haemoglobin (Hb) carries oxygen around the body, and in Sickle Cell you produce a different kind of haemoglobin, called HbS. This causes your red cells to become sickle-shaped (like a crescent moon) when they give up oxygen.

Sickle cells are very fragile compared to normal red cells. They don’t live as long because the body destroys them and has a poorer ability to carry oxygen around as a result. The cells can also become stuck in smaller blood vessels as they travel through your body. This can be very painful and cause damage to your organs.

Causes of Sickle Cell Anaemia

Sickle Cell is inherited in the genes we receive from our parents. If you inherit two faulty genes, you will develop Sickle Cell Anaemia, but with one faulty gene, less HbS is produced, and you are said to have Sickle Cell Trait

Sickle Cell Trait

Unlike Sickle Cell Anaemia, Sickle Cell Trait rarely causes any problems, but people with Sickle Cell Trait should be careful when taking part in activities where there is less oxygen than normal, for example, scuba diving and climbing.

Diagnosis of Sickle Cell Anaemia

Sickle Cell can be diagnosed by a blood test that analyses the haemoglobin molecules, a process called haemoglobin electrophoresis. Sometimes you can also see the sickle-shape of red cells by looking down a microscope.

With modern technology, babies can be diagnosed with sickle cell before they are even born, from 11 weeks into the pregnancy. Samples can be taken from the womb using techniques called chorionic villous sampling and amniocentesis.

Symptoms of Sickle Cell Anaemia

If Sickle Cell isn’t diagnosed during pregnancy it will usually be detected by one of the following:

• After birth, young children may present with jaundice (yellow-coloured skin and eyes). This is caused by broken-down sickle cells producing yellow coloured pigment.
• Children may also look pale and lack energy, due to a lack of oxygen in the blood.
• In some children, the hands and feet can become painful and swollen as the sickle cells become trapped.
• Sufferers are also at risk of having a “Sickle Cell crisis”. This can be triggered by several things, including a cold environment, dehydration, infection, or overexertion. Someone having a crisis may complain of pain in the chest or tummy, a headache or of having a stiff neck.
• The spleen is a collection point for the body’s white cells (immune cells). It is also involved in destroying and clearing the sickled cells, so comes into contact with lots of them. In Sickle Cell, the spleen is usually damaged before childhood is over. With the lack of a working spleen, sickle cell patients become vulnerable to infections, such as pneumonia and meningitis
• In some cases, bone marrow transplants are used to nurture stem cells, early cells that mature into red and white blood cells and platelets.

Treatment for Sickle Cell Anaemia

Unfortunately, no treatment will get rid of Sickle Cell, but there are ways to help control it:

• Patients can be given immunisations to prevent against certain infections, such as pneumonia and meningitis. They are also given penicillin antibiotic as a cover against possible disease.
• Folic acid supplements can help to replace the folic acid used up by the body when destroying sickle cells.
• During a crisis, oxygen and fluids may be given. Painkillers can also help stop the chest and tummy pain often seen in a crisis.
• If you suffer recurrent crises, blood transfusions can be given to try to reduce the number of abnormal red cells in the body.

Find out about other conditions like sickle cell anaemia in The Mix’s body problems resources.