Treatment - Sickle cell disease

Sickle cell disease usually requires lifelong treatment.

Children and adults with sickle cell disease are supported by a team of different healthcare professionals working together at a specialist sickle cell centre.

Your healthcare team will help you learn more about sickle cell disease, 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.

You may be advised to:

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

Get more advice about living with sickle cell disease

Medicine for sickle cell pain

If you continue to have episodes of pain, a medicine called hydroxycarbamide (hydroxyurea) may be recommended. You usually take it 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 you'll usually have regular blood tests to monitor your health.

Self-help for treating a sickle cell crisis

If you have a sickle cell crisis, you can usually manage it at home.

The following things can help:

• take over-the-counter painkillers, such as paracetamol or ibuprofen (do not give aspirin to children under 16 unless a doctor has prescribed it) – if the pain is more severe, your GP may prescribe stronger painkillers
• have plenty to drink
• use a warm towel or a wrapped heated pad to gently massage the affected body part – many pharmacies sell heat pads that you can use for this purpose
• distractions to take your 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 do not work or the pain is particularly severe. If this is not possible, go to your local A&E.

You may need treatment with very strong painkillers, such as morphine, in hospital for a few days.

Preventing infections if you have sickle cell disease

People with sickle cell disease are more vulnerable to infections.

Most people need to take a daily dose of antibiotics, usually penicillin, often for the rest of their life.

Long-term use of antibiotics will not pose any serious risks to your health.

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

Treatments for sickle cell-related anaemia

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

But dietary supplements like 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 is not the same as the more common iron deficiency anaemia.

Do not 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, hydroxycarbamide or voxelotor may be necessary.

Stem cell or bone marrow transplants

Stem cell or bone marrow transplants are the only cure for sickle cell disease, but they'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. They 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, 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 have not 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 of other problems that may need to be treated.

For example:

• a short course of hormonal medicine may be prescribed to 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 and painful erections (priapism) may require medication to stimulate blood flow or using a needle to drain blood from the penis
• leg ulcers can be treated by cleaning the ulcer and dressing it with a bandage
• people at increased risk of having a stroke, or those who have 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 – hydroxycarbamide may be needed to prevent further episodes

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

A helpful leaflet about sickle cell disease

The Sickle Cell Society has a parent's guide to managing sickle cell disease.