Priapism in patients with sickle cell disorder

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 disorder.

While the artery to the penis opens wider and blood rushes into it, the 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.

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 day-to-day life.

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 disorder 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 disorder 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 disorder. 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 which can last from a few minutes to several hours. This is known as stuttering priapism. 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 pseudoephedrine. These are usually given by mouth or may be given by injection into the penis. They may also use a different medicine which stops erections by lowering testosterone. 
• Drainage of some blood from the penis. 
• Numbing the penis with local anaesthetic and 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

Tel: 020 8961 7795

Email: info@sicklecellsociety.org

Website: sicklecellsociety.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 07950 869 745
• ward T11 south 020 3456 7890 ext. 71103 or 71143

Apheresis:

020 3447 1803

Address:

Haematology Department, 3rd Floor West, 250 Euston Rd, London, NW1 2PG

The Red Cell Network: uclh.nhs.uk/theredcellnetwork

Consultants: 

• Dr Emma Drasar
• Dr Perla Eleftheriou
• Dr Andrea Leigh
• Dr Ryan Mullally
• Professor John Porter
• Dr Sara Trompeter

Matron: 

Bernadette Hylton

Specialist nurses:

• Christopher Dean
• Enitan Roberts
• Alexandra Saville