Priapism and Sickle Cell Disease

What is priapism?

Priapism is a painful erection of the penis that lasts for an extended time. The erection is not related to sexual stimulus or arousal.

Priapism can begin at a young age in boys with sickle cell disease. It can cause impotency (not able to have an erection), infertility (not able to have children), scarring, or permanent damage to the penis.  

Priapism in sickle cell patients

In boys with sickle cell disease, priapism is caused by the sickling of red blood cells in the penis.

Sickled red blood cells stick together, block vessels, and slow the movement of oxygen to the cells. The vessels in the penis are small and easily blocked when red blood cells sickle and stick together.

Blood cannot flow out of the penis because the vessels are blocked by sickled blood cells. If the erection continues, this is called priapism.

Priapism can be very painful and is a serious condition. Long episodes of priapism can result in scarring and impotence (the loss of the ability to have an erection).

If your child has an erection for more than 2 hours, take him to the emergency room for treatment.

Types of priapism

Acute or prolonged priapism

Acute or prolonged priapism is an episode that lasts for more than 2 hours. This is a serious event, and you should take your child to the emergency room right away.

Medicines to treat priapism work best if given within the first 4 hours after the beginning of an acute episode. If it is not treated, it can result in permanent damage.

The penis will be erect, painful, and tender.

Chronic priapism

Chronic priapism is a state of semi-erection that does not go away. It can last for days or weeks. It usually is not painful. It may keep the person from having a complete erection while the chronic priapism is present.

Chronic priapism can change to acute priapism very quickly. Talk with your child’s care team if they are having chronic priapism. 

Tips for families

Your child cannot prevent priapism. But some things may help.

• Discuss priapism with your child. Make sure they understand how important it is to tell you if they are having this problem.
• Begin talking about priapism early with your child. Encourage them to ask questions. Stress that you are always available to talk.
• Be understanding and sensitive to your child’s needs, fears, and privacy.
• Reduce feelings of embarassment when talking about priapism by approaching it as a serious medical condition.
• Encourage your child to talk to their care team if they are not comfortable talking to you.
• Discuss therapies with your child’s health care team.
• Monitor your child for any signs of infection or fever.
• Make sure your child drinks plenty of liquids.
• Have your child go to the bathroom before going to bed each night. 

Talk with your child’s care team

If you have questions about priapism, your child’s care team can help. Reach out to them to learn more about the condition.

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