I’m Dr. Sébastien Beley, a urological surgeon and andrologist based in Paris, and today I’d like to talk to you about a subject that affects many people, particularly in sub-Saharan Africa: sickle cell disease. This genetic disease, often associated with serious complications, can also cause painful, prolonged erections, a phenomenon known as priapism. My aim is to explain to you what sickle cell disease is, how it affects male sexual health, and above all, what means exist to prevent and treat these complications in order to preserve your quality of life.

What is sickle cell disease? A genetic disease with African roots

Sickle cell anemia is a genetic disease that mainly affects people living in sub-Saharan Africa. It results from a mutation in hemoglobin, the protein present in red blood cells that transports oxygen throughout the body. In affected individuals, the red blood cells take on an abnormal “sickle” or “crescent” shape, hence the name “sickle cell anemia” (from the Greek “drepanon” meaning sickle).

This particular form of blood cell is in fact an evolutionary adaptation against malaria, a widespread infectious disease in these regions. Sickle-shaped red blood cells are less likely to be infected by the malaria parasite, conferring a degree of protection on carriers of this genetic mutation. However, this adaptation comes at a price: it gives rise to a series of important medical complications.

The effects of sickle cell disease on the body

Sickle red blood cells are less flexible and can become blocked in small blood vessels, causing painful seizures and organ damage. Among the most frequent complications are:

• Episodes of intense pain, known as vaso-occlusive attacks.
• Increased risk of infection.
• Chronic anemia due to premature destruction of red blood cells.
• Organ damage, particularly to the lungs, liver and kidneys.

But one of the lesser-known, yet major, aspects is the impact of sickle cell disease on erectile function in men, via a complication called priapism.

Priapism in sickle cell disease: a danger often underestimated

Priapism is a prolonged, often painful erection that occurs without sexual stimulation. In patients with sickle cell disease, this is a frequent and dreaded medical emergency. This phenomenon is due to the obstruction of blood vessels in the corpus cavernosum of the penis by sickle cell red blood cells, preventing the blood from circulating normally and deflating.

Why is priapism so common in sickle-cell patients?

Priapism usually occurs at night or in the early hours of the morning, when oxygen levels in the blood are low. Under these conditions, the sickle-shaped red blood cells stiffen and clump together, thickening the blood and causing an obstruction in the corpora cavernosa. This obstruction prevents the blood from draining away, resulting in a prolonged, painful erection.

The pain experienced during priapism is linked to hypoxia (lack of oxygen) and tissue acidosis, i.e. an accumulation of lactic acid in the tissues of the penis. This pain is often described as an intense, persistent cramp.

The consequences of untreated priapism

If priapism is not treated promptly, it can lead to irreversible damage. Prolonged oxygen deprivation leads to fibrosis of erectile tissue, which is replaced by non-functional scar tissue. This fibrosis prevents the penis from filling properly with blood during erections, leading to severe erectile dysfunction or even total loss of erection.

Fibrosis can also lead to a reduction in penile size, retraction and loss of natural flexibility. These consequences are devastating for patients’ sexual and psychological quality of life.

How to prevent and treat priapism in patients with sickle cell disease?

The management of priapism in patients with sickle cell disease is based on a number of key preventive and curative measures.

1. The importance of good oxygenation and hydration

To reduce the risk of priapism, it’s vital to optimize blood oxygenation and ensure proper hydration. Adequate oxygenation limits the formation of rigid sickle cell red blood cells, while sufficient hydration thins the blood and prevents vascular blockages.

Patients must therefore be informed and monitored to maintain these conditions, especially during high-risk periods such as the night.

2. Early management of pain and episodes of priapism

Prompt treatment of pain and the first signs of priapism is essential to limit the damage. It’s crucial that patients know how to recognize the symptoms and seek immediate medical attention.

In some cases, patients can be taught to inject vasoactive drugs intra-cavernously to interrupt priapism before it becomes prolonged and destructive. This technique, if properly supervised, can help prevent progression to severe fibrosis.

3. Specialized medical follow-up

In the event of prolonged priapism (more than 6 hours), urgent consultation with a urologist is essential. The doctor can then perform specific maneuvers to evacuate stagnant blood and reduce fibrosis. These early interventions are often decisive in preserving erectile function.

4. Drug treatments to prevent fibrosis

After an episode of priapism, certain medications usually prescribed to treat erectile dysfunction can be used to help rehabilitate the cavernous tissues. These treatments can help limit the severity of fibrosis and maintain the flexibility of the corpora cavernosa.

5. Recourse to surgery for advanced fibrosis

Unfortunately, when fibrosis is too severe and erectile function is compromised, the only effective solution is penile prosthesis. This surgical procedure restores the ability to have a functional erection by implanting a mechanical device inside the penis.

This solution is generally reserved for the most advanced cases, but offers excellent quality of life for the patients concerned.

The doctor’s role and practical advice for patients

If you have sickle cell disease, it’s vital that your doctor or specialist clearly informs you of the risks associated with priapism, and what to do in the event of a painful episode. Good communication means you can act quickly and effectively.

Here are a few practical tips to keep in mind:

• Learn to recognize the first signs of priapism.
• Maintain optimal hydration and avoid situations that can reduce oxygenation.
• Talk to your doctor about learning to self-inject vasoactive drugs.
• In the event of a painful, prolonged erection, consult a specialist as soon as possible.
• Follow prescribed treatments scrupulously to prevent long-term complications.

A disease not to be neglected: raising awareness and comprehensive care

Sickle cell disease is a complex condition requiring multidisciplinary management. In addition to general complications, its effects on sexual health must be taken very seriously. Priapism, if poorly managed, can lead to permanent loss of erectile function, which has a profound impact on patients’ intimate lives and psychological well-being.

As a healthcare professional, my commitment is to help sickle cell patients understand their disease and prevent complications. Raising awareness is key, both among patients and medical teams, to ensure rapid and appropriate care.

Conclusion: Save your erections, protect your quality of life

Sickle cell anemia, although common in some parts of the world, remains a little-known disease in terms of its impact on male sexuality. Priapism is a serious complication, but thanks to better information, early treatment and appropriate therapies, it is possible to limit its devastating effects.

If you’re concerned, don’t hesitate to consult a specialist and discuss the subject openly. Prevention, rapid intervention and, if necessary, surgery can help you regain a fulfilled and serene sex life.

I invite you to stay informed, ask your questions and take care of your sexual health.

For more information and other tips on male sexual health, you can follow me on my YouTube channel and social networks.