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 disorder.

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.

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 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 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.

Types of priapism

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 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 (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 drinking alcohol or taking illicit drugs.

Can I do anything to prevent it?

  • 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.

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 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 North Middlesex Hospital directly to avoid delay in receiving treatment. You will be seen by the urology team who may recommend:

  • 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 urology team can provide more information.

What happens if I get priapism repeatedly?

If you suffer from recurrent priapism you may be referred to the UCLH andrology team (a specialist urology team) as an outpatient. UCLH is the regional centre for this specialist service. In addition you may be offered hydroxycarbamide (if not already taking) and/or red cell exchange.