Common conditions: sickle cell disease


Everything you need to know about the group of health conditions known as sickle cell disease. By Meredith Jones Russell

Sickle cell disease is the name for a group of inherited health conditions that affect red blood cells. The most serious type is called sickle cell anaemia.

People with sickle cell disease produce unusually shaped red blood cells that can cause problems because they do not live as long as healthy blood cells and can block blood vessels.

Sickle cell disease is a serious and lifelong health condition, although treatment can help manage many of the symptoms.

It is often detected during pregnancy or soon after birth, via screening offered to all pregnant women in England, as well as to babies as part of the newborn blood spot test, or heel prick test.

WHAT ARE THE SYMPTOMS?

People born with sickle cell disease tend to have problems from early childhood, although some children have few symptoms and lead normal lives most of the time.

The main symptoms are:

  • painful episodes called sickle cell crises, which can be very severe and last up to a week. Pain often affects a particular part of the body, such as hands or feet (especially in young children), ribs and breastbone, spine, pelvis, tummy, legs and arms
  • an increased risk of serious infections
  • anaemia (where red blood cells cannot carry enough oxygen around the body), which can cause tiredness and shortness of breath.

Some people also experience other issues, such as delayed growth, strokes and lung problems.

WHAT ARE THE CAUSES?

Sickle cell disease is caused by a gene that affects how red blood cells develop. If both parents have the gene, there is a one in four chance of each child they have being born with sickle cell disease.

The child’s parents often will not have sickle cell disease themselves and will only be carriers of the sickle cell trait.

Sickle cell disease is particularly common in people with an African or Caribbean family background.

HOW CAN IT BE TREATED?

People with sickle cell disease need treatment throughout their lives, often from a specialist sickle cell centre. Treatments include:

  • drinking plenty of fluids and staying warm to prevent painful episodes
  • use of painkillers, such as paracetamol or ibuprofen
  • daily antibiotics and having regular vaccinations to reduce the chances of getting an infection
  • use of a medicine called hydroxycarbamide (hydroxyurea) to reduce symptoms
  • regular blood transfusions if symptoms continue or get worse, or there are signs of damage caused by sickle cell disease
  • an emergency blood transfusion if severe anaemia develops.

The only cure for sickle cell disease is a stem cell or bone marrow transplant, but they are not done very often because of the risks involved.

Adapted from the NHS website, https://www.nhs.uk/conditions/sickle-cell-disease

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