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Sickle cell anaemia Q&As

Published by Bupa's health information team, October 2008.

Answers to questions about sickle cell anaemia

This section contains answers to common questions about this topic. Questions have been suggested by health professionals, website feedback and requests via email.

 

What pain relief should I have?

Strong medicines based on opium, such as morphine, are the most effective for treating severe sickle cell pain. It's very important that you have a good relationship with the team of people caring for you in order to get the best treatment, including getting effective pain relief when you need it.

Explanation

Some people with sickle cell anaemia have pain every day. Others only have pain if they get a painful episode (also known as a 'crisis'), which may be triggered by infection, cold, dehydration or strenuous exercise, but can also come on without any clear reason.

You may find that measures such as warming the painful area, resting and relaxation techniques help to relieve pain. In addition, for mild pain, you may find paracetamol useful. For stronger pain you may need paracetamol plus a medicine such as codeine or dihydrocodeine. Ibuprofen (eg Nurofen) and other non-steroidal anti-inflammatory drugs (NSAIDs) such as naproxen and diclofenac may be used too. If you have very severe pain, you may need quick treatment with an injection or drip containing morphine, or similar strong painkillers. Always ask your doctor for advice and read the patient information leaflet that comes with your medicine.

The Sickle Cell Society has published guidelines on the best practice in managing sickle cell anaemia. These guidelines state that people having a painful episode should get pain relief within 30 minutes of arriving at hospital, and that pain should be controlled within two hours.

There are certain risks with using these strong painkillers. Side-effects can include:

  • slowing down of your breathing rate
  • drowsiness
  • feeling sick or vomiting

There is very little evidence to show that people who have sickle cell anaemia can become addicted to the strong painkillers they may be prescribed.

It's also important that if you have sickle cell anaemia, you have the opportunity to agree with your GP and local hospital a personalised treatment plan. This will describe how your crises will be managed so that you can get treatment as soon as possible when you need it.

Further information

Sources

  • Shapiro B. Pain in sickle cell disease. Pain Clinical Updates 1997; V:2. www.iasp-pain.org
  • Standards for the clinical care of adults with sickle cell disease in the UK. Sickle Cell Society. www.sicklecellsociety.org, accessed 21 July 2008
  • Geller AK, O'Connor MK. The sickle cell crisis: a dilemma in pain relief. Mayo Clinic Proceedings 2008; 83:3
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I'm a sickle cell carrier. What is the chance that my children will inherit the disease?

This depends on the status of your partner. If your partner is completely unaffected by the sickle cell gene and has the HbA form of haemoglobin, your children won't be affected by the disease, although they may also be carriers. However, if your partner is also a carrier, there is a one in four chance that your children will inherit the HbS gene from both you and your partner and so have sickle cell disease.

Explanation

Children inherit an equal number of genes from each parent. One particular pair of genes - one gene from each parent - determines whether a child will have sickle cell anaemia, be a carrier or be totally unaffected. A baby will only have sickle cell anaemia if both genes are the sickle form (HbS).

There are other abnormal haemoglobins that can be inherited in the same way as HbS, and the combination of some of these with a single HbS gene can lead to a condition similar to sickle cell anaemia.

If you and your partner are both carriers (also called sickle cell trait), you will both have one usual form of the gene and one sickle cell gene. When you and your partner's genes mix at conception there is a one in four chance that your sickle gene will be partnered with his or her sickle gene. Therefore, for each pregnancy there is a one in four chance that your child will have the disease. It's important to recognise that this chance is the same for each pregnancy. So even if you have already had three children and they have been unaffected, there is a still only a one in four chance of the fourth child being affected. Equally, if all three of your children have the disease, any further children still have a one in four chance of also being affected.

It's possible to diagnose sickle cell disease early in pregnancy with chorionic villus sampling.

If you or your family are affected by sickle cell disease, your GP will be able to refer you to a genetic counsellor for personal advice, based on your family history and test results.

Further information

Sources

  • Genetic disease profile: sickle cell anaemia. Human Genome Project Information. www.ornl.gov, accessed 13 September 2008
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How many people have sickle cell anaemia? Why do people with an African or Caribbean background get it most?

About 13,000 people in the UK have sickle cell anaemia. Most of these people have a black African, black British or Caribbean background. However, people from India, the Middle East and parts of the Mediterranean can also be affected. Globally, around one in 20 people carry the sickle cell gene. According to the UK Sickle Cell Society, between one in 10 and one in 40 black African or Caribbean people are sickle cell carriers. As partnerships become more common between people of different ethnic backgrounds, it's likely the disease will increasingly affect less obvious groups.

Explanation

In the areas of Africa worst affected by malaria, lots of people carry the sickle cell gene. This is because if you are a carrier of the gene for sickle cell anaemia, you're more likely to survive malaria. It isn't fully understood why being a sickle cell carrier gives this protection, it's possible that having some red blood cells affected by sickle cell allows the body to develop a stronger immunity to the parasite that causes malaria.

People with sickle cell anaemia in the areas of Africa affected by malaria have a poor life expectancy due to the limited healthcare in these countries. Therefore, they are much less likely to have children than unaffected people and so won't pass on the sickle cell genes. However, carriers have a better chance than unaffected people of surviving malaria during childhood and so going on to have their own children. When carriers have children the malaria protection can be passed on with the sickle cell gene.

Further information

Sources

  • Sickle-cell disease and other haemoglobin disorders. World Health Organization. www.who.int, accessed 21 July 2008
  • Malaria and sickle cell trait. Wellcome Trust. http://malaria.wellcome.ac.uk, accessed 21 July 2008
  • A sickle crisis? National Confidential Enquiry into Patient Outcome and Death, 2008. www.ncepod.org.uk
  • Standards for the clinical care of adults with sickle cell disease in the UK. Sickle Cell Society. www.sicklecellsociety.org, accessed 21 July 2008
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I have sickle cell anaemia. Do I need to take malaria tablets when I visit a malarial area?

Yes. Although being a sickle cell carrier does give some protection against malaria, the risk of the illness is still high and the Health Protection Agency recommends that you take adequate precautions, including antimalarial medicines. This is because malaria can cause serious problems, particularly if you have sickle cell anaemia. Therefore, it's especially important to take the tablets if you have sickle cell anaemia.

Explanation

It's very important to protect yourself against mosquito bites. You can do this by covering up with appropriate clothing, mosquito repellents and bed nets treated with insecticide (so you don't get bitten while you sleep). In addition, you will need to take a course of preventive tablets - you generally start these before you travel and continue taking them for some time after you get back. Get advice from your GP surgery before you travel to find out which type of antimalarial medicine is recommended for the area you're visiting.

If you have sickle cell anaemia, it's especially important to be protected. This is because malaria is one of the main causes of a painful crisis in people with the disease who live in malarial areas.

If your spleen has been damaged by sickle cell anaemia, you may be advised not to travel to a malarial area at all because you will be more likely to get a severe form of malaria. If you must travel, you will have to take careful precautions to make sure you don't get bitten and must take the full course of the preventive medicines exactly as recommended.

Sources

  • Guidelines for malaria prevention in travellers from the United Kingdom. Health Protection Agency, 2007. www.hpa.org.uk
  • Malaria prophylaxis - management. Clinical Knowledge Summaries. http://cks.library.nhs.uk, accessed 22 July 2008
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How will I know if my child is affected by the trait or the disease?

Your baby can be tested with a blood test. This is now performed as part of routine screening soon after a baby is born.

Explanation

In areas where the population is commonly affected by sickle cell, there is routine screening of all newborn babies. This is called 'universal screening' because it's offered to all babies, whatever his/her ethnic background. In other areas, only people from communities where the condition is common are tested, although universal screening is being introduced across the whole of the UK.

The test is done on a spot of blood that is already routinely taken from a needle prick to a baby's heel in the first few days after birth. This is analysed in a laboratory. Your baby can be tested for up to four other conditions, including an underactive thyroid gland and cystic fibrosis, at the same time.

Sources

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I'm a sickle cell carrier. Can I climb mountains or go scuba diving?

There is mixed evidence as to whether activities such as mountaineering and scuba diving, where there is less oxygen available to the body, can cause problems if you are a sickle cell carrier. Check with your doctor before starting any new activity.

Explanation

If you have sickle cell anaemia, you shouldn't do intense exercise of any kind or go scuba diving as these can set off a crisis. However, if you are a carrier for sickle cell anaemia, you may be able to take part in such activities.

If you are a sickle cell carrier, there is a risk that some of your red blood cells may become 'sickled' when there is a severe shortage of oxygen. Usually this is balanced by the healthy red blood cells and normal haemoglobin and carriers generally don't have any health problems, even when exercising. There have been a number of elite athletes who are sickle cell carriers who have competed successfully at altitude.

However, extreme situations can cause problems. High up a mountain (higher than any of the relatively low UK mountains) there is much less oxygen in the atmosphere and there is a risk of blood cells becoming sickled. This may cause pain and possibly serious health problems including a stroke. Also, intense exercise under extremely hot conditions increases the risk of life-threatening health problems for people who are sickle cell carriers, particularly if they aren't properly trained for that level of exercise.

If you are sickle cell carrier, take regular, moderate exercise and check with your doctor before taking up any new, vigorous sport.

Scuba diving is unsuitable for many people with a variety of medical conditions. However, being a sickle cell carrier shouldn't automatically prevent you from doing it. Speak to your doctor before taking up scuba diving and always dive according to the safety rules of the sport.

Sources

  • Shaskey D, Green G. Sports haematology. Sports Med 2000; 29(1):27-38
  • The medical examination and assessment of divers. Health and Safety Commission, 2008, MA1. www.hse.gov.uk
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Related topics

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This information was published by Bupa's health information team and is based on reputable sources of medical evidence. It has been peer reviewed by Bupa doctors. The content is intended for general information only and does not replace the need for personal advice from a qualified health professional.

Publication date: October 2008

 

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