Sickle cell disease (sickle cell anaemia)
Sickle cell disease is the name for a group of inherited conditions that affect the red blood cells. The most serious type is called sickle cell anaemia. Sickle cell disease is a serious and lifelong condition, although long-term treatment can help manage many of the problems associated with it.
Symptoms of sickle cell disease
People with sickle cell disease produce unusually shaped red blood cells. These can cause problems because they don't live as long as healthy blood cells and they can become stuck in blood vessels.
People born with sickle cell disease sometimes experience problems from early childhood, although most children have few symptoms and lead normal lives most of the time.
The main symptoms of sickle cell disease are:
- painful episodes called sickle cell crises, which can be very severe and can last up to a week
- an increased risk of serious infections
- anaemia (where red blood cells can't carry enough oxygen around the body), which can cause tiredness and shortness of breath
Some people also experience other problems such as delayed growth, strokes and lung problems.
Causes of sickle cell disease
Sickle cell disease is caused by a faulty gene that affects how red blood cells develop.
If both parents have this faulty gene, there's a 25 per cent chance of each child they have being born with sickle cell disease.
The child's parents often won't have the condition themselves because they're only carriers of the sickle cell trait (see below).
People commonly affected by sickle cell disease
Sickle cell disease mainly affects people of African, Caribbean, Middle Eastern, Eastern Mediterranean and Asian origin. In the UK, it's particularly common in people with an African or Caribbean family background.
Screening and testing for sickle cell disease
Sickle cell disease is often detected during pregnancy or soon after birth. All babies are offered screening as part of the newborn blood spot test (heel prick test).
Blood tests can also be carried out at any age to check for the condition or to see if you're a carrier of the faulty gene that causes it.
Treatments for sickle cell disease
People with sickle cell disease will need specialist care throughout their lives.
A number of treatments are available to help manage problems caused by the condition. For example:
- painful episodes can sometimes be prevented by drinking plenty of fluids and staying warm
- pain can often be treated with ordinary painkillers such as paracetamol or ibuprofen, although sometimes treatment with stronger painkillers in hospital may be necessary
- the risk of infections can be reduced by taking daily antibiotics and ensuring you're fully vaccinated
- a blood transfusion may be needed if severe anaemia develops
- a medicine called hydroxycarbamide (hydroxyurea) can reduce the symptoms if they're interfering with your or your child's life
- having regular blood transfusions (usually every 3-4 weeks) may be helpful if problems continue or get worse, or there are signs of damage caused by the condition
Stem cell or bone marrow transplants can potentially cure sickle cell disease, but they're not done very often because of the significant risks involved.
Outlook for sickle cell disease
Sickle cell disease is very variable, but most children with it will lead normal lives. It can still be a serious condition that can have a significant impact on a person's life.
It can lead to problems which can occasionally be fatal, such as:
- strokes
- serious infections
- lung problems
Overall, the life expectancy for someone with sickle cell disease tends to be shorter than normal, but this can vary depending on:
- the exact type of sickle cell disease they have
- how it's treated
- what problems they experience
Currently, people with sickle cell anaemia typically live until 40-60 years of age, although milder types of sickle cell disease may have no impact on life expectancy.
Carriers of sickle cell (sickle cell trait)
A carrier of sickle cell is someone who carries one of the faulty genes that causes sickle cell disease, but doesn't have the condition themselves. It's also known as having the sickle cell trait.
People who carry sickle cell won't develop sickle cell disease, but are at risk of having a child with the condition if their partner is also a carrier.
You can request a blood test to check if you have carry sickle cell from your GP surgery.
More useful links
The information on this page has been adapted from original content from the NHS website.
For further information see terms and conditions.