Managing Priapism in Young Sickle Cell Patients: Raising Awareness and Empowering Communities

19 June 2023 

Leon is a 13-year-old teenager from UK, he loves playing football and suffers from sickle cell disorder. In this animation, Leon tells us about his “worst day ever”, the first time he woke up with a painful penile erection which persisted and became increasingly painful, until he managed to overcome his fear and told his mum, who promptly brought him to the hospital emergency department.

Sickle cell disorder is an inherited blood condition and a genetic disease that affects people worldwide, particularly those from African, Middle East, Indian, Southern and Southeastern European origins.  Sickle cell carrier (trait) protects from malaria, but sickle cell disorders predispose people to pain, anemia, infections, and priapism.

Priapism is a medical emergency that occurs when blood flow to the penis is not properly regulated, causing an unwanted erection that lasts for hours. Red blood cells become abnormally shaped, which makes it difficult to flow smoothly through blood vessels. When priapism does not resolve spontaneously, it requires emergency medical attention, as beyond severe pain, it can damage erectile tissue if not treated promptly.

“There are about 15,000 people with sickle cell disease in the UK, and this continues to grow with around 250 births each year. Priapism is a common issue for young men with sickle cell and can affect up to 50%.” said Dr. Rachel Kesse-Adu, consultant haematologist at Guy’s and St Thomas’ NHS Foundation Trust. “It’s important to raise awareness of the serious effects if priapism is left untreated. Dedicated resources are needed to help break any taboo and encourage families to talk about it.”

Prompt care can help to prevent long-term complications and to improve overall quality of life for those experiencing priapism. However, this requires knowledge of symptoms from both young sufferers and their caregivers, beyond a readiness to disclose the situation, often a cause of embarrassment for young children.

“Attending the Emergency Department with an unwanted erection is the last thing any young man wants to do. But it is crucial if a prolonged erection lasts more than two hours, as delaying treatment can result in long-term damage to erections. The Emergency Department team is experienced in handling such cases, and the effectiveness of treatment increases with early intervention” explains Mr Majed Shabbir, consultant urological surgeon at Guy’s and St Thomas’ NHS Foundation Trust. “It’s also important to inform someone if there is a recurring pattern of short-lasting but disruptive night time erections, known as “stuttering” episodes as they can impact normal erectile function. Priapism can be caused by various medical issues, sickle cell disease being one of the most common causes in younger men. Even brief periods of priapism can lead to harm. Preventive measures can help maintain healthy erections.”  

Recently Boston Scientific worked together with the Sickle Cell Society in the UK and with Guy’s and St Thomas’ NHS Foundation Trust in South London, which is at the forefront of the specialist care for patients with sickle cell disorder, to raise awareness about the management of priapism. The objective was to generate understanding in younger sufferers around modern management of sickle cell and priapism, to provide the community of educators and health care professionals with additional resources to assist children and their families to manage this condition.

As part of the collaboration, educational materials have been created and distributed via the Sickle Cell Society to help young children and their families understanding priapism and how to manage it. “These materials include an animated story that explains the condition in an accessible and child-friendly way.  This is a valuable resource for young people, parents and healthcare professionals to help understanding priapism, learn what to do about it, what to expect if there is a need to go to hospital, and to equip with the language to talk about this issue.” commented John James, CEO of the Sickle Cell Society. “The educational materials emphasize the importance of seeking medical attention as soon as priapism is identified.”

Leon’s priapism was successfully treated at hospital, since he and his mum were able to react promptly, and his mum knew what to do.

“At Boston Scientific we believe in acting with compassion to support our communities”, said Miguel Aragon, vice president EMEA, Urology. “Expanding community education to raise awareness, educating children and their families, to ultimately reduce the burden of debilitating conditions on the health care system is part of our efforts to advancing science for life.”

Caring about human life is the basis for everything we do at Boston Scientific. Improving chronic disease management is one example of our commitment to improving standard of care for young children.

When we increase health awareness and educate providers about their role in health care, we contribute to better futures for people, their families and communities.


This article was released on June 19th 2023 on the occasion of Sickle Cell awareness day

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