Priapism and Sickle Cell Disease
These instructions are for Children's Hospital of Philadelphia (CHOP) patients with sickle cell disease to learn more about how to prevent and manage priapism.
Important information about priapism:
Priapism is a painful erection of the penis that lasts for an extended time. Priapism can begin at a very young age. If it is not treated, it can cause impotency (not able to have an erection), infertility (not able to have children), scarring or permanent damage to the penis.
In children with sickle cell disease, priapism is caused by the sickling of red blood cells in the penis. Normally, an erection occurs because of stimulus. The blood vessels in the penis relax and allow more blood to flow into the penis. At the same time, other vessels contract and result in reduced blood flow out of the penis, causing an erection. Usually, after the stimulus is removed, the penis returns to its normal non-rigid state.
With sickle cell disease, 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.
Patient instructions for priapism:
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Encourage your child to drink plenty of liquids. Staying hydrated can help prevent priapism and help episodes resolve.
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Apply a warm towel to the area or take a warm shower.
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Offer distraction techniques such as going for a walk.
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If this happens often, your healthcare team will recommend a visit with a urologist. There are medicines that prevent priapism or can be taken at the beginning of an episode.
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If the priapism lasts more than 2 hours, please call your healthcare team. You may be referred to the Emergency Department for treatment. Medicines that treat priapism work best if given in the first 4 hours after the beginning of an acute episode.
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Taking daily sickle cell disease medicines as prescribed will help your child be their healthiest.
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Discuss priapism with your child. Make sure they understand this is a serious medical issue and they need to tell a parent or trusted adult if it happens so they can get medical attention.
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Have your child go to the bathroom before going to bed each night.
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Encourage your child not to drink alcohol, smoke or use street drugs. All of these can cause sickling and severe health problems in children with sickle cell disease.
Contact your CHOP hematology team with questions or concerns.
215-590-3535
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CHOP Main patients:
8:30am-5:00pm, Monday-Friday
215-590-3437
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Voorhees patients
8:00am-4:30pm, Monday-Friday
856-435-7502
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King of Prussia patients:
8:00am-4:30pm, Monday-Friday
267-425-3320
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Weekends, holidays and every day after
5:00pm, all patients: 215-590-1000, ask the
hospital operator for the hematologist on call.
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For non-urgent issues, send your team a message in the MyCHOP portal.
Reviewed on June 22, 2023, by Alexandra Kaspin, RN, MSN