Priapism is the medical term for prolonged erection. It is not always related to sexual intercourse or stimulation, and is often painful. If left untreated priapism can cause to the penis, which at worst may result in loss of ability to have a normal erection (impotence). Priapism is common in boys and men who have sickle cell disease. This information will help you learn how to recognise it, and explain what you can do about it.

Priapism is the medical term for a prolonged erection. It is not always related to sexual intercourse or stimulation and is often painful. If left untreated priapism can cause lasting damage to the penis which at worst may result in loss of the ability to have a normal erection (impotence).

Priapism is common in boys and men who have sickle cell disease. This information leaflet will help you recognise it and explains what you can do about it.
During a normal erection blood flows into the penis and it becomes rigid. After ejaculation, hormones are released which allow the blood to drain. If there is a blockage of the blood vessels caused by sickled red blood cells this does not happen and the penis remains erect. Normally an erection lasts for no more than 30 minutes and is not painful. In priapism an erection can last for hours or even days. The shaft of the penis is always rigid, but the head may sometimes be soft and tender to touch.

Two main types of priapism can occur in sickle cell disease

• Recurrent acute or ‘stuttering’ priapism: erections that last for more than 30 minutes and up to 4 hours and happen on several occasions. These may get better on their own or with the measures described below without requiring medical treatment.

• Acute prolonged or ‘fulminant’ priapism: a painful erection which lasts for more than 4 hours. This requires urgent medical assistance. Delayed treatment carries a risk of permanent damage including impotence.


Who does priapism affect?

Priapism can develop from an early age but is most common in teenagers and adults (for whom this leaflet is intended). It occurs in all forms of sickle cell disease though is more common in the most severe forms of sickle cell anaemia such as HbSS. Studies suggest that up to 50 per cent of males suffer an episode at some point during their life.

It can be embarrassing to talk about priapism but it is very important to report the problem to the team caring for you so appropriate advice can be given and long term consequences avoided.

If you have an erection that is painful or lasts for longer than 30 minutes immediately do the following:

• Drink plenty of fluids

• Take pain killers such as paracetamol if needed

• Walk around or take gentle exercise (such as running on the spot)

• Have a warm shower or bath

• Pass urine frequently to empty the bladder

• Keep the area warm - DO NOT apply anything cold as this may make matters worse

• Try to ejaculate

If despite the above measures the penis remains erect after TWO HOURS go to the Accident and Emergency department IMMEDIATELY.

How is priapism treated in hospital?

If you have to attend hospital with priapism you will be cared for jointly by the Haematology and Urology teams and will need to stay in hospital until the episode has completely resolved.

The treatment you receive may include:

• Pain killers – if simple pain killers don’t work you may need stronger pain killers like morphine/diamorphine or as per your individual protocol

• Intravenous fluids

• Pseudoephedrine - a tablet which constricts the blood vessels. This moves blood away from the penis and may help the erection subside.

• Draining some blood from the penis (aspiration) – this is done with a small needle and syringe by a Urology doctor after giving a local anaesthetic to numb the area. This usually helps to relieve the pain. Blood may be washed out with sterile salt solution at the same time. Sometimes more than one treatment may be needed.

• An injection into the penis – this is often done at the same time as aspiration to help the erection subside. This works like pseudoephedrine but has a more potent effect

• Blood transfusion – a red blood cell exchange may be of benefit

• An operation - this may be considered if other measures fail and is carried out by a Urology specialist. This may involve transfer to a different hospital. There are several different types of operation. These involve implanting a small device called a shunt into the penis to re-route the flow of blood out of the penis. Some men who have surgery for priapism find it difficult to have an erection afterwards. It is difficult to estimate the exact risk of this because priapism itself can cause the same problem.

These treatments and procedures will be discussed fully with you.

If you have had more than one episode of priapism treatment may be advised to try to prevent future episodes. The team caring for you will explain the options. They include taking medication on a daily basis such as pseudoephedrine and hydroxycarbamide. If this does not work a regular exchange transfusion programme to reduce the number of sickle cells in the blood may help.

For further information please visit Imperial NHS