Introduction

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 diseasemainly affects people of African, Caribbean,Middle Eastern, Eastern Mediterranean and Asian origin.In the UK, it's particularly commoninpeople with an African or Caribbean family background.

People with sickle cell disease produce unusually shaped red blood cells that can cause problems because they don't live as long as healthy blood cells andthey can become stuck in blood vessels.

Sickle cell disease is a serious and lifelong condition, although long-term treatment can help manage many of the problems associated with it.

This page covers:

Symptoms

Causes

Screening and testing

Treatments

Outlook

Carriers

Symptoms of sickle cell disease

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 toa 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.

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.

It's hoped the outlook will continue to improve as newer treatments are developed.

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 cellwon'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 cellfrom your GP surgery or nearest sickle cell and thalassaemia centre .

It is pumped around the body by the heart.

Blood vessels

Blood vessels are the tubes in which blood travels to and from parts of the body. The three main types of blood vessels are veins, arteries and capillaries.

Bone marrow

Bone marrow is the soft, spongy tissue in the centre of bones that produces blood cells.

Disease

A disease is an illness or condition that interferes with normal body functions.

Genetic

Genetic is a term that refers to genes, the characteristics inherited from a family member.

Lungs

Lungs are a pair of organs in the chest that control breathing. They remove carbon dioxide from the blood and replace it with oxygen.

Oxygen

Oxygen is an odourless, colourless gas that makes up about 20% of the air we breathe.

Pain

Pain is an unpleasant physical or emotional feeling that your body produces as a warning sign that it has been damaged.

Tissue

Body tissue is made up of groups of cells that perform a specific job, such as protecting the body against infection, producing movement or storing fat.