New animation raises awareness of painful erections in teens with sickle cell disease

A new animation is raising awareness of painful penile erections in teenage boys with sickle cell disease and the need for prompt action. This potentially harmful complication of the condition can lead to a medical emergency. 

Sickle cell disease predisposes people to pain, anaemia, 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 due to extreme pain. It can damage erectile tissue if not treated promptly.

Launched on World Sickle Cell Day (Monday 19 June), the animation was developed in collaboration with the Sickle Cell Society and experts at Guy’s and St Thomas’ with the support of Boston Scientific.

It tells the story of Leon, a 13-year-old with sickle cell disease who recovers from a sudden and painful penile erection after his mother acts promptly, taking him to the Emergency Department (A&E).

Sickle cell disease 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. 

Dr Rachel Kesse-Adu, consultant haematologist at Guy’s and St Thomas’, said: "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%.

"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 improve overall quality of life for those experiencing priapism. However, this requires knowledge of symptoms from both young sufferers and their caregivers, and a readiness to disclose the situation – which can be particularly embarrassing for children.

Mr Majed Shabbir, consultant urological surgeon at Guy’s and St Thomas’, said: "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.

"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."  

As part of the collaboration, educational materials have been created and distributed via the Sickle Cell Society to help young children and their families understand priapism and how to manage it. 

John James, CEO of the Sickle Cell Society, said: "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 them equip with the language to talk about this issue.

"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."

Miguel Aragon, vice president EMEA, Urology at Boston Scientific, said: "We believe in acting with compassion to support our communities.

"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."

To see the animation and to find out more about priapism in people with sickle cell disease visit the Sickle Cell Society website.