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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 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.  

What causes priapism?

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.

In a child with sickle cell disease, the sickling of red blood cells can cause an erection. Blood is unable to 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.

Sickle cell disease is a genetic disorder that affects the red blood cells. A normal red blood cell is flexible, smooth, and round or doughnut-shaped (without the hole in the center). A sickle red blood cell is hard, sticky, and sickle-shaped (like a banana or quarter moon).

Sickle 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. Long episodes of priapism are very painful and 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 and tell your nurse case manager. 

Assessing priapism

There are different types of priapism: stuttering priapism, acute or prolonged priapism, and chronic priapism. 

Stuttering priapism

Stuttering priapism is an erection that lasts longer than 30 minutes but less than 2 hours. The erection may come and go over time but does not last more than 2 hours. Stuttering priapism may be a one-time event or it may happen more than once. The erection may go away by itself or after using some of these home treatments:

  • Have your child drink plenty of water
  • Medicine – Talk with your child’s doctor about the type of medicine that might help
  • Warm towel or warm water
  • Distraction techniques

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. You should tell the nurse case manager or doctor if your child is having chronic priapism. 

Can anything be done to prevent priapism?

Priapism cannot be prevented, but some things may help.

  • Discuss priapism with your child. Make sure he understands how important it is to tell you if he is having this problem.
  • Often, teenage boys do not like to talk about sexual issues with their parents. Begin talking about priapism early with your child. Encourage him to ask questions. Stress that your door is always open.
  • Be understanding and sensitive to your child’s needs, fears, and privacy.
  • Reduce the chance of embarrassing your child when talking about priapism by treating it as a serious medical condition.
  • Encourage your child to call his nurse case manager or a member of the health care team if he is not comfortable talking to you about priapism.
  • 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. 
  • Encourage your child to not drink alcohol, smoke, or use street drugs. All of these can cause sickling and severe health problems in children with sickle cell disease.

Questions?

If you have questions about priapism, please talk to a member of the hematology team. It is important for you to know that priapism is a serious condition and prolonged priapism (2 hours or more) requires urgent medical care. If you are outside the hospital, refer to your Important Phone Number Card or call the main operator at (901) 595-3300 or toll-free 1-866-2STJUDE (1-866-278-5833). Ask for your child’s nurse case manager. If calling after hours, please ask for the hematologist on call.


 

This document is not intended to take the place of the care and attention of your personal physician or other professional medical services. Our aim is to promote active participation in your care and treatment by providing information and education. Questions about individual health concerns or specific treatment options should be discussed with your physician.

St. Jude complies with health care-related federal civil rights laws and does not discriminate on the basis of race, color, national origin, age, disability, or sex.

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