University College Hospital

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1 University College Hospital Priapism in patients with sickle cell disease North Central London Haemoglobinopathy Network jointly with Whittington Health, Royal Free London and Luton and Dunstable NHS Foundation Trust Joint Red Cell Unit

2 2 If you need a large print, audio or translated copy of the document, please contact us on We will try our best to meet your needs. Contents What is priapism and who is affected? 3 How does an erection happen? 3 How do I know that there is a problem? 3 Types of priapism 3 Other causes of priapism 4 Can I do anything to prevent it? 4 What can I do if I get a priapism? 4 How can I get an outpatient appointment with the andrology team? 5 Contact details 6 Space for notes and questions 7 Where can I get more information? 8

3 3 What is priapism and who is affected? Priapism is a painful erection that lasts longer than 30 minutes. It can occur in males of all ages, including newborn babies, and is common in sickle cell disease. Most boys over the age of about 10 and healthy adults will have at least three to four erections a day. Many will happen at night and it is normal for boys and men to wake up with an erection. Normal erections are not painful and fade away as the person gets on with their normal life. How does an erection happen? The artery to the penis opens wider and blood rushes into it while veins carrying blood away from the penis shut down. Blood then becomes trapped inside the penis, making it hard and erect. An erection subsides when the veins open up and allow blood to flow out of the penis. How do I know that there is a problem? If the erection is painful, or if it does not fade away after about 30 minutes, this is called priapism. Priapism is very common in boys and men with sickle cell disease and needs to be treated promptly. If it is ignored, it may lead to future problems. At worst, the man may not be able to have normal erections. Types of priapism There are two types of priapism: Priapism associated with low blood flow This is usually associated with sickle cell disease and occurs when the vein running from the penis is blocked. This may lead to tissue damage.

4 4 Priapism associated with high blood flow This is very rare in sickle cell disease and occurs after injury to the penis, when the cavernosal artery (vessel leading to the penis) ruptures. The erection can occur as repeated short episodes (known as stuttering priapism), which can last from a few minutes to several hours. Or it can last for four hours or more (known as fulminant priapism). Other causes of priapism: Injury to the penis or genital area Damage to the nervous system Thalassaemia Leukaemia, and multiple myeloma After taking alcohol or illicit drugs. Can I do anything to prevent it? Avoid alcohol or illicit drugs. Keep well hydrated. Sometimes priapism may happen despite these precautions. If you have had it before, your haematologist may ask you to take a medication, such as etilefrine, on a regular basis to prevent it from recurring. What can I do if I get a priapism? Do the following things immediately: Drink lots of fluids Take simple painkillers such as paracetamol Try to pass urine. Some patients also find that jogging or other exercise help.

5 5 These should help to take the problem away within an hour. It is important that you report the episode to the haematology team caring for you within the next few days. If the problem does not go away, you must go to the Emergency Department (A&E). There is a risk of permanent damage if priapism lasts longer than four hours. If you can get to UCLH with ease, it is best that you come here directly rather than get transferred over from your local hospital to avoid delay in effective treatment. This is because UCLH is the regional centre for the specialist urology team who will need to see you. This team is known as the andrology team. The andrology team may recommend or perform the following: Medicines, such as etilefrine or pseudoephidrine, which are usually given by mouth or may be given by injection into the penis. A different medicine which stops erections by lowering testosterone may also be used. Drainage of some blood from the penis. Numbing the penis with local anaesthetic and then using a small needle to check how much acid and oxygen there is in the blood. This procedure is very important to save future penile function. An operation - your haematology or andrology team can provide more information. How can I get an outpatient apppointment with the andrology team? Your consultant or CNS can refer you for an outpatient appointment if you experience problems with priapism or impotence. Some of the andrology team s clinics run alongside the Wednesday evening haematology clinic.

6 6 Contact details Haematology advice line (office hours, children and adults): Adult haematology advice line (out of hours): Paediatric helpline (out of hours): nurse in charge ward T11S ext or Apheresis: Address: The Joint Red Cell Unit Department of Haematology 3rd Fl West, 250 Euston Rd London NW1 2PG Website: Haematoloy consultants: Professor John Porter Dr Sara Trompeter Dr Perla Eleftheriou Dr Bernard Davis Dr Farrukh Shah Specialist nurses: Bernadette Hylton (adults) Nancy Huntley (apheresis) Catherine Mkandawire (children)

7 Space for notes and questions 7

8 Where can I get more information? The Sickle Cell Society Tel: Website: NHS Sickle Cell and Thalassaemia Screening Programme Website: The UK Thalassaemia Society Tel: Fax: office@ukts.org Website: UCL Hospitals cannot accept responsibility for information provided by external organisations. First published: September 2013 Last review date: July 2015 Next review date: July 2017 Leaflet code: UCLH/SandC/CD/JRCU/PRIAPISM/2 University College London Hospitals NHS Foundation Trust