Painful erection with sickle cell disease

This information resource explains about painful erections, called ‘priapism’ with sickle cell disease.

If you need more advice after reading this please speak to your doctor or nurse at Sheffield Children’s Hospital.

What is priapism?

Priapism is an erection that occurs without any sexual desire or stimulation, and lasts for a long time. It is often painful and distressing. Priapism often starts during early puberty and men can often continue to have symptoms to 30 or 40 years old.

There are 2 common types of priapism:

• Stuttering priapism
• Acute (fulminant) priapism

Stuttering priapism

Stuttering priapism is the most common type of priapism. It is called stuttering if the erection lasts less than 1 hour but comes back. They go away on their own but can happen again after a short period of time. They typically happen at night.

Acute (fulminant) priapism

Acute priapism is an erection which lasts for more than 1 hour and can persist for hours or even days without treatment. Acute priapism is usually very painful and is considered a major complication of sickle cell disease.

Some people may often experience repeated episodes of stuttering priapism before an attack of acute priapism.

Why does priapism happen?

Priapism happens because the blood in the penis becomes trapped and unable to return to the circulation. The trapped blood keeps the penis in the erect state.

The trapped blood in the penis becomes starved of oxygen and this causes pain and tissue damage the longer it persists.

What are the triggers for priapism?

Priapism is often triggered by usual sickle cell complications, such as:

• stress
• infection
• dehydration
• alcohol
• hot and cold environments
• physical activity

Often priapism happens early in the morning which is the normal time for erections. It is associated with having a full bladder. Always make sure you wee before bed to try and to try prevent early morning priapism.

Some people develop priapism during sexual intercourse.

What do I do if I get priapism?

Both men and teenage boys often feel embarrassed by these episodes and do not talk about them, not even to their doctor. However, it is important to discuss these episodes as they can be serious and could result in permanent damage if left untreated (see below).

If you are suffering from repeated episodes of stuttering priapism it is important to seek help immediately.

Management at home

There are a few things you can do to manage priapism at home including:

• take painkillers such as paracetamol or ibuprofen at the dose prescribed to you in clinic
• drink extra fluids
• a warm bath
• try to have a wee
• gentle exercise such as jogging, squats, and running up and down stairs

What will happen if I come to hospital?

If you are admitted to hospital you may be given intravenous fluid (fluid into your vein), oxygen, and painkillers.

If this does not work, there are other medicines we can offer you such as Etilefrine.

Sometimes, if your erection persists, we will contact the Urology team who may consider draining the blood from your penis. This would be offered under a general anaesthetic.

If you are admitted to hospital you will need to stay until the priapism is better. Your doctor may give you medication to prevent further attacks.

What can happen if I ignore an episode of priapism?

People who suffer from recurrent priapism can suffer from scarring and damage to the penis. This can disrupt the normal flow of blood in the penis and can lead to loss of normal sexual function (erectile dysfunction).