Moving from child to adult sickle cell disease services

Transcription

1 What will happen when I move from child to adult? When you move over from child to adult sickle cell services you will be under the care of the Sickle Cell and Thalassaemia (SCaT) team at City Hospital. You will be given an appointment to come to the centre to meet the doctors who will be responsible for your treatment. After this first appointment you will be able to telephone or come to the SCaT centre without an appointment, anytime during opening hours, if you have any problems. If you do need to come into the centre please try to come in as early in the day as possible so we have enough time to assess you and give you the correct treatment or advice. As well as being able to come to the centre when you have any problems, you will also be offered planned appointments with our doctors and/or specialist nurses. In your first few appointments the doctors will plan your care with you. Is there anything I need to do? Now that you are moving into adult services it is important that you understand your condition, how to take care of yourself and what to do if there is a problem. If you have a boyfriend or girlfriend it is also a good idea to start thinking about contraception if you are going to have a sexual relationship. This is because if you have sickle cell disease any children you have can also have the condition as it is passed on through genes. It is particularly important to think about contraception if you are female, because women with sickle cell disease can develop complications in pregnancy and need extra care. Most forms of contraception are suitable for people with sickle cell disease; your GP or SCaT centre nurse or doctor can give you advice about this. What is sickle cell disease? Sickle cell disease is a condition where the red blood cells, which are normally round, become shaped like a sickle (crescent shape). How did I get sickle cell disease? You inherited 1 haemoglobin (blood) gene from your dad and 1 haemoglobin gene from your mum. To have sickle cell disease you need to have inherited either 2 sickle cell haemoglobin genes (1 from your dad and 1 from your mum), or 1 sickle cell haemoglobin gene and 1 other abnormal haemoglobin gene. The diagram below shows how people inherit sickle cell disease. A means a normal blood gene, and S means a sickle cell blood gene. Page 1

2 AA SS Normal haemoglobin Sickle cell trait Sickle cell trait Sickle cell disease When people inherit 1 sickle cell haemoglobin gene from their father and 1 from their mother we call this haemoglobin S+S (each S stands for sickle cell), or HbSS for short. This is the most common type of sickle cell disease. When people inherit 1 sickle cell haemoglobin gene and 1 other abnormal haemoglobin gene we call this haemoglobin S+letter that stands for the abnormal gene. When people inherit 1 normal haemoglobin gene and 1 sickle cell haemoglobin gene, this means they have sickle cell trait. This means that they carry a sickle cell gene and can pass it on if they have any children, but they do not have sickle cell disease and do not have any of the symptoms of sickle cell disease. How is sickle cell disease diagnosed? Sickle cell disease is diagnosed by testing a sample of your blood. All newborn babies can have a blood test when they are a few days old to find out if they have sickle cell disease or a number of other conditions. Page 2

3 A sample of the water around a baby in the womb can also be tested during pregnancy to see if a baby has sickle cell disease. What problems can sickle cell cause for me? Pain The sickle-shaped blood cells you have can block your small blood vessels and cause pain; this is called a sickle cell crisis (or vaso-occlusive crisis). The pain usually affects the bones in your arms, legs, back or ribs. The pain can sometimes be very severe. It is important to make sure you have a supply of painkillers at home and become familiar with what dose you can take of them and when. You can find this information in the leaflet that comes with them. The leaflet will also tell you what side effects the medicines can cause. When you are having a sickle cell crisis you should: 1. Start by taking paracetamol. 2. If this doesn t work you can then try taking diclofenac or ibuprofen (these are antiinflammatory painkillers) up to 3 times a day, provided that you don t have a stomach ulcer or kidney problems. 3. If you are still in pain you can then try codeine which is stronger; you can take this up to 4 times a day. 4. If this is not effective you can take a combination of paracetamol, an anti-inflammatory painkiller and codeine, provided that you have not taken them in the last few hours and will not be taking more than the recommended dose for each. 5. If you have tried all of these and are still in pain you should contact the SCaT centre as you may need to be given a stronger painkiller, such as morphine, in hospital. Anaemia The red blood cells that are sickle-shaped survive for a shorter time than normal red blood cells; this causes anaemia. We test for anaemia with a blood test to measure the level of haemoglobin in your blood. Haemoglobin is a chemical in red blood cells which makes them red and carries oxygen. It is important that you know your haemoglobin level (ask your doctor or nurse what yours is). Infections You are also more likely to get infections which can make you very unwell. This is because your spleen (an organ in your tummy which filters your blood) doesn t work properly. To Page 3

4 reduce the chance of you getting an infection you might be given a low dose of antibiotics to take regularly. Other complications People with sickle cell disease are also at risk of: Acute chest syndrome (where the sickle cells block the blood vessels in your lungs) Damage to your bones (usually hips and shoulders) Anaemia that suddenly becomes worse Priapism (in males) this is an unwanted, painful erection that goes on for a long time and can damage the penis Stroke How is sickle cell disease treated? Treatment for sickle cell disease involves: Keeping healthy by looking after yourself Having immunisations (vaccinations) to reduce your risk of getting ill. Avoiding things that can make you have a sickle cell crisis (triggers). Painkillers for when you have a sickle cell crisis. Taking folic acid to help your body make red blood cells. Taking antibiotics regularly to reduce your risk of getting an infection. You might also be prescribed medication to reduce the amount of sickle cell crises you have. Your sickle cell team will talk to you about any treatment you need and explain the benefits and risks. When do I need to get medical help? If you have any of the following problems you should contact the Sickle Cell and Thalassaemia Centre for advice: Severe pain that is not improving with painkillers An episode of priapism that lasts for more than 1 hour Symptoms of an infection: A high temperature (38 C or above) Uncontrollable shivering Page 4

5 Severe sore throat Coughing up sputum (phlegm) A burning feeling when you pass urine (wee) Smelly or cloudy urine Symptoms of acute chest syndrome: Shortness of breath Chest pain Symptoms of anaemia that is getting worse: Tiredness that is not normal for you or is getting worse Looking pale Yellowing of the skin Yellowing in the whites of your eyes Symptoms of stroke (dial 999 if you have any of these symptoms): Your face has dropped on one side You have difficulty speaking or you are slurring your words You can t lift both of your arms and keep them there It is important that you get help early on because people with sickle cell disease can become very unwell very quickly. In an emergency, or if the sickle cell centre is closed, please go to A&E. How can I learn more about sickle cell disease? You can learn about sickle cell disease and living with the condition by asking your doctors and nurses questions and reading good, reliable information. The SCaT centre has information leaflets on the following, which you can ask for: Living with sickle cell disease this includes information on avoiding things that trigger a sickle cell crisis, what vaccinations you should have and advice about travel. Pregnancy advice for women with sickle cell disease this includes information on planning pregnancy, the complications that can occur during pregnancy and the care you will need. Priapism this includes information on what priapism is, what causes it, how it is treated Page 5

6 Sickle Cell and Thalassaemia and how to reduce your risk of having an episode of priapism. These leaflets are also on the patient information section of our website. You can also find good quality information about sickle cell disease on the NHS Choices website: Who can I ask if I have any questions about my condition? If you have any questions about sickle cell disease, your treatment or living with the conditions you can call the SCaT centre to chat to one of the team. You can also drop in to the centre to speak to one of the team. How can I contact the SCaT centre? Sickle Cell and Thalassaemia Centre at City Hospital Monday Thursday, 9am 5pm Friday, 9am 4pm Further information For more information about sickle cell disease and the support available to you, visit the sickle cell society website: Sickle Cell Society You can also read more about sickle cell disease on the NHS Choices website: NHS Choices For information about the support available visit: Organisation for Sickle Cell Anaemia Research and Thalassaemia Support (OSCAR) Birmingham: Sandwell: For more information about our hospitals and services please visit our websites and follow us on and like us on Facebook Page 6

7 Sickle Cell and Thalassaemia Sources used for the information in this leaflet Sickle Cell Society, Standards for the Clinical Care of Adults with Sickle Cell Disease in the UK, 2008 NHS Evidence Clinical Knowledge Summaries, Sickle cell disease, November 2010 If you would like to suggest any amendments or improvements to this leaflet please contact the communications department on or A Teaching Trust of The University of Birmingham Incorporating City, Sandwell and Rowley Regis Hospitals Sandwell and West Birmingham Hospitals NHS Trust ML3982 Issue Date: March 2013 Review Date: March 2015 Page 7