Treatment

Sickle cell disease usually requires lifelong treatment.

Some of the main treatments that may be used are outlined below:

Preventing painful episodes

Treating pain

Preventing infections

Treatments for anaemia

Stem cell or bone marrow transplants

Treating other problems

Links to more information

Children and adults with sickle cell disease will be supported by ateam of different healthcare professionals working together in a specialist sickle cell centre.

Your care team will help you learn more about the condition and work with you to come up with an individual care plan that takes into account all your needs and health concerns.

Preventing painful episodes

The main thing you can do to reduce your chances of experiencing a painful episode (sickle cell crisis) is to try avoiding possible triggers.

This may mean you need to:

• drink plenty of fluids to avoid dehydration
• wear appropriate clothing to stop you getting cold
• avoid sudden temperature changes, such as swimming in cold water

Readabout living with sickle cell disease for more advice.

If you continue to experience episodes of pain, a medication called hydroxycarbamide (hydroxyurea) may be recommended. This is usually taken as a capsule once a day.

Hydroxycarbamide can lower the amount of other blood cells, such as white blood cells and platelets (clotting cells), so regular Blood tests will usually be recommended to monitor your health.

Treating pain

If you experience a sickle cell crisis, you can usually manage it at home. The following can help:

• takeover-the-counter painkillers, such as paracetamol or ibuprofen (aspirin should not be given to children under 16) if thepain is more severe, your GP may prescribe stronger painkillers
• ensure you haveplenty to drink
• use a warm towel or a heated pad to gently massage the affected body part many pharmacies sell pads that can be used for this purpose
• try suitable distractionsto takeyour mind off the pain for example, children might like to read a story, watch a film or play their favourite computer game

Contact your GP if these measures don't work or thepain is particularly severe. If this isn't possible, take your child to your local accident and emergency (A&E) department .

Treatment withvery strong painkillers (such as morphine) in hospital for a few days maybe needed.

Preventing infections

As people with sickle cell disease are more vulnerable to infections, treatment will usually be needed to reduce this risk.

Most people need to take a daily dose of antibiotics (usually penicillin), often for the rest of their life. Long-term use of antibiotics won't pose any serious risks to your health.

Children with sickle cell disease should also have all the routine vaccinations , and possibly also additional vaccinations such as the annual flu vaccine and the hepatitis B vaccine .

Treatments for anaemia

Anaemia often causes few symptoms and may not require specific treatment.

However, dietary supplements such as folic acid (which helps stimulate the production of red blood cells) may sometimes be required to help improve anaemia if your child has a restricted diet, such as a vegetarian or vegan diet.

Anaemia caused by sickle cell disease isn't the same as the more common iron deficiency anaemia . Don't take iron supplements to treat it without seeking medical advice as they could be dangerous.

If anaemia is particularly severe or persistent, treatment with blood transfusions orhydroxycarbamide may be necessary.

Stem cell or bone marrow transplants

Stem cell or bone marrow transplants are the only cure for sickle cell disease, butthey're not done very often because of the significant risks involved.

Stem cells are special cells produced by bone marrow (a spongy tissue found in the centre of some bones) that can turn into different types of blood cells.

For a stem cell transplant, stem cells from a healthy donor are given through a drip into a vein. These cells then start to produce healthy red blood cells to replace the sickle cells.

A stem cell transplant is an intensive treatment that carries a number of risks. The main risk is graft versus host disease, which is a life-threatening problem where the transplanted cells start to attack the other cells in your body.

Stem cell transplants are generally only considered in children with sickle cell disease who have severe symptoms that haven't responded to other treatments, when the long-term benefits of a transplant are thought to outweigh the possible risks.

Treating other problems

Sickle cell disease can also cause a number ofother problems that may need to be treated. For example:

• ashort course of hormonal medication may be prescribed to help trigger puberty in children with delayed puberty
• gallstones may be treated with gallbladder removal surgery
• bone and joint pain can be treated with painkillers, although more severe cases may require surgery
• persistent priapism (a persistent and painful erection) may require medication to stimulate blood flow orusing a needle to drain blood from the penis read more about treatments for priapism
• leg ulcers can be treated by cleaning the ulcer and dressing it with a bandage (read more about treating leg ulcers )
• people at increased risk of having a stroke , or those who've had a stroke, may need regular blood transfusions or treatment with hydroxycarbamide
• acute chest syndrome(a serious lung condition) usually requires emergency treatment with antibiotics, blood transfusions, oxygen and fluids given into a vein hydroxycarbamidemay be needed to prevent further episodes

People who need a lot of blood transfusions may also need to take medication to reduce the amount of iron in their blood to safe levels. This is called chelation therapy.

More information

If you'd like more information about treatments for sickle cell disease, the NHS Sickle Cell and Thalassaemia Screening Programme produces a detailed parent's guide to managing sickle cell disease (PDF, 3.57Mb) that you might find helpful.