What’s Priapism?

In a normal erection, blood flow to the penis increases. The extra blood makes the penis larger and rigid and compresses the veins that drain it. When you’re no longer aroused, blood flow decreases, allowing the veins to drain the extra blood.

The penis then goes back to how it was before. Normal erections last 30 minutes or less.

With priapism, blood can get trapped in the penis. The erection lasts much longer than expected, occurs without sexual stimulation, and is often painful.

Types of Priapism in SCD

The type of priapism that usually happens with sickle cell disease is called ischemic priapism. In ischemic priapism, blood cannot flow out of the penis normally.

As time goes on, the trapped blood runs out of oxygen. This can damage the penis and cause erectile dysfunction (difficulty getting or maintaining an erection).

Stuttering Priapism

Priapism can be a single long-lasting erection or multiple shorter episodes that go away and return. This is known as stuttering priapism.

Stuttering episodes can last from a few minutes to three hours. They are often painful but usually go away on their own.

It’s important not to ignore stuttering priapism. Episodes may last longer and become more painful when they come back.

If you or your child has a painful erection that lasts more than an hour, contact the sickle cell care team right away. Repeated shorter episodes should also be reported. Tracking when episodes happen, how long they last, and other symptoms can help the team assess the problem.

When Is Priapism an Emergency?

An erection that lasts four hours or longer is a urologic emergency. However, people with sickle cell are often advised to seek emergency care sooner. If the erection has not gone away after two hours, go to the emergency department.

If you or your child has an erection that is not going away, it can be hard to know when to seek help, especially if you feel embarrassed, unsure, or worried about how you’ll be treated. Talking with your sickle cell care team before this happens can help you make a plan for when to call, where to go, and what to say if you need urgent care. If you are unsure what to do, call your healthcare provider.

Don’t Count on Home Remedies

Warm baths or showers are sometimes suggested early on. However, cold water or cold compresses should be avoided in people with sickle cell because cold can make the problem worse. Ejaculation isn’t likely to make priapism go away.

Home remedies should never delay emergency care. By four hours, priapism is a urologic emergency, but people with sickle cell should seek emergency care earlier, as described above.

If the erection has lasted less than two hours and your provider has told you to try home measures first, they may suggest:

• Drinking water
• Going for a walk or doing some light exercise
• Taking pain medication your healthcare provider has recommended
• Trying to urinate

The biggest concern with attempting to treat priapism at home is lost time. Trying one home remedy after another can delay treatment while the tissues of the penis are being deprived of oxygen.

If your doctor has given you a specific home plan for early episodes, follow that plan. If the episode doesn’t resolve within the recommended time frame, seek medical care.

What You Can Do To Prevent Priapism

If you or your child has recurrent priapism, ask whether a prevention plan is appropriate.

Treatment for sickle cell disease itself may help reduce recurrent priapism. Options may include hydroxyurea, regular exchange transfusions, or medicines such as pseudoephedrine. Crizanlizumab has also been studied as a possible way to reduce recurrent episodes.

The best way to prevent recurrent ischemic priapism is not yet clear, so treatment should be individualized. Some hormonal treatments may reduce episodes, but they can impair fertility and sexual function.

Don’t start a medication, supplement, or other treatment for priapism without talking with a healthcare professional first. Some treatments can have serious side effects or may not be appropriate for someone with SCD.

Advice for Parents

Parents and caregivers can help children by staying calm. A child may feel uncomfortable talking about an erection, and caregivers may feel unsure about how to respond.

Let them know this is a health issue, not something shameful. Reassure them that they didn’t do anything wrong.

For younger children, caregivers may need to watch for signs such as unusual genital pain, swelling, difficulty sitting comfortably, waking during the night, or complaining of penis pain. They should share information about SCD and possible complications with their daycares and schools.

Your child’s healthcare provider can help you understand what to look for and where to get help. They can also give you tips on how to talk to your child and help them manage their condition as they get older.

Keep Track of Symptoms

Keeping track of when episodes happen, how long they last, and what other symptoms occur can help your healthcare team understand the pattern.

Even episodes that resolve on their own are worth mentioning. Frequent short episodes can affect quality of life and sleep and may increase the risk of future problems.

Your healthcare team can discuss ways to reduce the frequency and severity of episodes. Priapism can be difficult to talk about, but you don’t have to manage it alone.

Ask your care team about prevention, make sure you understand your emergency plan, and seek urgent care when needed. Knowing what to do can help protect your health and make it easier to act quickly if priapism happens again.