Living with - Sickle cell disease

There are a number of things you can do, and precautions you can take, to stay as healthy as possible if you have sickle cell disease.

Managing sickle cell pain

You can reduce your risk of painful episodes (sickle cell crises) by avoiding things that can trigger them.

Try to:

• drink plenty of fluids, particularly during hot weather – dehydration increases the risk of a sickle cell crisis
• avoid extreme temperatures – you should dress appropriately for the weather and avoid sudden temperature changes, such as swimming in cold water
• be careful at high altitudes – the lack of oxygen at high altitudes may trigger a crisis (travelling by plane should not be a problem because planes are pressurised to maintain a steady oxygen level)
• avoid very strenuous exercise – people with sickle cell disease should be active, but intense activities that cause you to become seriously out of breath are best avoided
• avoid alcohol and smoking – alcohol can cause you to become dehydrated and smoking can trigger a serious lung condition called acute chest syndrome
• relax – stress can trigger a sickle cell crisis, so it may help to learn relaxation techniques, such as breathing exercises

Your healthcare team can give you more advice about avoiding triggers.

It's also a good idea to be prepared for treating painful episodes at home. Keep a ready supply of painkillers (paracetamol or ibuprofen) and consider buying some heated pads to soothe the pain.

Find out more about treatments for sickle cell disease

Avoiding infections

You'll usually be given antibiotics and advised to have vaccinations to help prevent most serious infections, but there are also things you can do to reduce your risk.

For example, you should make sure you follow good food hygiene measures to prevent food poisoning.

Make sure you speak to your GP or healthcare team if you're planning to travel aboard, as this may mean you need extra medication or vaccinations.

For example, if you're travelling to an area where malaria is found, it's important to take antimalarial medication.

You may also need to take extra food and water precautions abroad.

Pregnancy and contraception

Women with sickle cell disease can have a healthy pregnancy, but it's a good idea to speak to your healthcare team for advice first.

It may be useful to find out if your partner is a carrier of sickle cell and discuss the implications of this with a genetic counsellor.

Some sickle cell disease medicines, such as hydroxycarbamide, can harm an unborn baby. You may need to be stop taking them before trying to get pregnant.

There's an increased risk of problems, such as anaemia, sickle cell pain, miscarriage and pre-eclampsia, during pregnancy.

And you may need extra monitoring and treatment during pregnancy to help prevent problems.

If you're not planning a pregnancy, use a reliable form of contraception.

Surgery precautions if you have sickle cell disease

It's important to let your healthcare team know if you need to have an operation under general anaesthetic at any point. Tell your surgeon that you have sickle cell disease.

This is because general anaesthetic can cause problems for people with sickle cell disease, including an increased risk of experiencing a sickle cell crisis.

You may need close monitoring during surgery to make sure you're getting enough fluids and oxygen and are kept warm.

Sometimes you may need a blood transfusion beforehand to reduce the risk of complications.

When to get medical advice

Make sure you know when to get medical advice and where to go, as sickle cell disease can cause a number of serious problems that can appear suddenly.

Problems to look out for include:

• a high temperature over 38C (or any increased temperature in a child)
• severe pain that's not responding to treatment at home
• severe vomiting or diarrhoea
• a severe headache, dizziness or a stiff neck
• breathing difficulties
• very pale skin or lips
• sudden swelling in the tummy
• a painful erection (priapism) lasting more than 2 hours
• confusion, drowsiness or slurred speech
• fits (seizures)
• weakness on 1 or both sides of the body
• changes in vision or sudden vision loss

Contact your GP or healthcare team straight away if you develop any of these symptoms.

If this is not possible, go to your nearest A&E. If you're not well enough to travel to hospital yourself, dial 999 for an ambulance.

Make sure that the medical staff looking after you know you have sickle cell disease.