Priapism in patients with sickle cell disease

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.

There are two types of priapism:

• Priapism associated with low blood flow: this is linked to sickle cell disease and occurs when the vein running from the penis is blocked. This may lead to tissue damage.
• Priapism associated with high blood flow: this is very rare in sickle cell disease. It 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).

• Injury to the penis or genital area
• Damage to the nervous system
• Thalassaemia
• Leukaemia and multiple myeloma
• After drinking alcohol or taking illicit drugs.

• Avoid alcohol and 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.

Do the following things immediately:

• Drink lots of fluids
• Take simple painkillers such as paracetamol
• Try to pass urine

Some people also find that jogging or other types of exercise help.

These should help to stop the episode within an hour. It is important that you report it to the haematology team caring for you in 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, go to UCLH directly to avoid delay in receiving treatment. 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.

Your consultant or CNS can refer you if you experience problems with priapism or impotence. Some of the andrology team’s clinics run alongside the Wednesday evening haematology clinic.

Sickle Cell Society

Telephone: 020 8861 7795
Website: sicklecellsociety.org

UK Thalassaemia Society

Telephone: 020 8882 0011
Email: office@ukts.org
Website: ukts.org

University College London Hospitals NHS Foundation Trust cannot accept responsibility for information provided by external organisations.

Haematology admin team: uclh.redcelladminteam@nhs.net
Haematology clinical nurse specialists (CNSs): uclh.redcell.cnsteam@nhs.net
Haematology advice line (office hours, adults and children): 020 3447 7359
Adult haematology advice line (out of hours): 07852 220 900

Paediatric helpline (out of hours):
Nurse in charge: 07961 081 645
Ward T11 south: 020 3456 7890 ext. 71103 or 71143

Apheresis: 020 3447 1803

Address: The Joint Red Cell Unit, Department of Haematology
3rd Floor West, 250 Euston Rd London NW1 2PG
Website: www.uclh.nhs.uk/joint-red-cell-unit