Sickle Cell Crisis – Priapism

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Haematology Department Sickle Cell Crisis – Priapism Information for parents and carers (268kB vnd.openxmlformats-officedocument.wordprocessingml.document)

Introduction

This leaflet aims to provide you with general information about the signs and symptoms your child may have when they are having a sickle cell crisis. If you are ever worried about your child please contact your Sickle Cell Team or take your child to the Accident and Emergency Department.

What is priapism?

Priapism is a prolonged painful erection of the penis. It is normal for boys of all ages to get erections for short periods of time but these are not normally painful and will subside. If the erection is painful or does not subside then you should seek medical advice immediately.

What causes priapism?

Priapism is caused by sickled red blood cells blocking the vessels in the penis. This disrupts the blood flow to the penis causing the tissues not to get enough oxygen. There may be no obvious trigger to priapism, but being dehydrated, drinking alcohol and having an infection can all trigger priapism.

Is priapism dangerous for my son?

Recurrent priapism or prolonged episodes cause damage to the tissues in the penis. The damage causes the blood flow to be disrupted and leads to erectile dysfunction where they cannot get or sustain an erection.

What are the symptoms of priapism?

There are two types of priapism – stuttering and fulminant.

• Stuttering priapism is short attacks that resolve without any treatment but keep happening. The repeat episodes can happen within hours or days and sometimes are not painful. Stuttering priapism can go on to become a more severe episode.
• Fulminant priapism is a severe episode where the erection lasts for more than a few hours and does not resolve by itself. This type of priapism needs medical attention.

If your son gets an episode or priapism then you must let your Sickle Cell Team know or bring them straight to the hospital if it does not resolve within one hour.

Can I manage priapism at home?

You should manage priapism as you would any crisis by giving pain medicines and increasing the amount of fluid to your son. You can also try asking your son to empty their bladder. Heat packs and/or a warm shower or bath may help. Do not use a cold pack on the penis as this will make it worse by causing more sickling of the red blood cells.

When should my son come to the hospital?

If the priapism last for more than one hour then you should bring them to the Accident and Emergency Department immediately. Many boys feel very embarrassed when they get priapism and do not want to come to the hospital. It is important that they come to the hospital because the longer they stay at home the more damage will occur.

What is the treatment for priapism?

The treatment for priapism is pain relief, intravenous (IV) fluids and surgery. Surgery is needed to wash blood out of the penis and to inject a drug to stop the priapism reoccurring. The surgery will be done under a general anaesthetic by the Urology Team. Sometimes it works, but sometimes the priapism returns and surgery has to be done again or a different operation is needed.

Can my son get priapism again?

Priapism can happen more than once. There are medicines that can help to prevent priapism occurring again and it may be suggested that your son takes these.

How can I prevent my son getting priapism?

There are some things that can be done to try to prevent priapism. Even if these things are tried it is no guarantee that priapism will be prevented. The things to try are ensuring your child passes urine before they go to bed and when they get up first thing in the morning; to avoid drinking alcohol; drink plenty of fluids and avoid stressful situations. Encourage your son to tell you when they have priapism so that you can seek medical attention if necessary.

Further information

If you have any questions or want any further information please contact your Sickle Cell Team on 0151 252 5070.

Useful websites

www.sicklecellsociety.co.uk

Alder Hey Children’s NHS Foundation Trust is neither liable for the contents of any external internet site listed, nor does it endorse any commercial product or service mentioned or advised on any of the sites

This leaflet only gives general information. You must always discuss the individual treatment of your child with the appropriate member of staff. Do not rely on this leaflet alone for information about your child’s treatment.

This information can be made available in other languages and formats if requested.

PIAG: 0068