What is Stuttering Priapism?
Stuttering priapism is an uncommon but potentially severe condition that causes frequent, short-lived erections which may last under 3 to 4 hours at a time. Even though these erections tend to go away on their own, they can escalate to a more serious form of priapism known as ischemic priapism. This progression requires immediate treatment to prevent problems such as erectile dysfunction. Stuttering priapism is often found among those with sickle cell disease, and treatment approaches for this condition can vary.
Priapism refers to a prolonged and continuous erection of the penis, typically extending over 3 to 4 hours without any sexual stimulation. This condition can be divided into three categories:
* Low-flow, or ischemic priapism, is the most frequently observed type.
* High-flow, or oxygenated priapism, often arises from a an injury to the penis.
* Recurrent, or stuttering priapism, is the least common type.
Although stuttering priapism lasts less than 3 to 4 hours per episode and often resolves itself, it can progress into a full-blown case of ischemic priapism in about a third of incidents. This requires immediate treatment as the latter type can lead to several health issues, including erectile dysfunction. Therefore, it’s suggested for patients to get treated within 4 hours to lessen the risk of long-term complications or permanent damage to the erecting structures or corpora of the penis.
The penis is made up of three parts—the corpora cavernosa, corpus spongiosum, and the urethra. The penile corpora are like beds of blood vessels and spaces, along with smooth muscles, nerves, and capillaries, that facilitate blood flow. These corpora receive blood from the common penile artery and cavernosal arteries, both being branches of the internal pudendal artery. The blood exits the penis through several venous systems that empty into two veins. The nerves that trigger the penis for erection are the cavernosal nerves, stemming from a group of nerve cells in the pelvic region.
During sexual arousal, these nerves trigger the release of chemical messengers that relax the smooth muscles within the corpora, leading to dilation of the small arteries and arterioles. This results in a significant increase in blood flow, filling up the corpora cavernosa to maximum capacity just like how a tire fills with air. Once the corpora cavernosa reach their full capacity, pressure builds up, blocking the veins that would normally drain the blood and trapping blood inside.
This stoppage of blood outflow results in the pressure within the corpora cavernosa rising, akin to filling a car tire with 200 lbs of pressure per square inch. Just like how the steel belts in a tire react to added pressure—becoming stiffer and more rigid without stretching or enlarging—the pressure inside the corpora cavernosa causes rigidity and a full erection.
What Causes Stuttering Priapism?
Just like a condition called ischemic priapism, stuttering priapism is caused by blood issues, the use of certain drugs, nerve disorders, and cancer. Sickle cell disease, a condition where a person lacks healthy red blood cells for carrying oxygen, is the leading cause of stuttering priapism.
Even though stuttering priapism is not common among the general population, it is relatively common among men with sickle cell disease. It affects about 30% to 45% of all adult men who have sickle cell disease. Most men with sickle cell disease who have priapism, a condition characterized by a persistent, often painful, erection, tend to have stuttering priapism. This condition can also occur among people who have the sickle cell trait, which means they carry one sickle cell gene and one normal gene. Other blood-related matters such as leukemia, a cancer of blood-forming tissues, thalassemia, an inherited blood disorder, and abnormal platelets could also lead to priapism.
There are certain medications, particularly psychoactive drugs and improperly used erectile dysfunction drugs, that are closely connected to priapism. However, testosterone supplements, urination, or a full bladder do not seem to play a significant part in causing priapism.
Risk Factors and Frequency for Stuttering Priapism
Priapism is a condition with varying rates across different populations, making it hard to identify a definitive prevalence for each type. For example, one study found that 42% of men with Sickle Cell Disease (SCD) experienced a type of priapism known as stuttering priapism. Meanwhile, a different study reported that 65% of children and adolescents with SCD might experience it. It’s also important to note that 82% of patients with SCD who’ve had any form of priapism had the stuttering variety, and almost half of these individuals have had a severe episode of it.
The frequency of these episodes ranges widely – some people experience it as often as four times in a week, and others have it only three times in one year. On average, it occurs thrice in a month. In terms of age, it’s most common among children between the ages of 5 to 10, and among adults aged 20 to 50. For boys with SCD, there’s a 12% chance of experiencing priapism in their first decade of life. This likelihood increases to 50% at age 15, and by age 20, almost 90% have had at least one episode. The average age to experience the first episode is 15 years old. Finally, it’s worth mentioning that virtually all patients with SCD had their first episode before the age of 30.
- 42% of men with Sickle Cell Disease (SCD) experience stuttering priapism.
- Amongst children and adolescents with SCD, the incidence rate increases to 65%.
- 82% of all patients with SCD who’ve had priapism experienced the stuttering variety.
- Priapism episodes vary in frequency – they can occur anywhere from 4 times a week to 3 times a year, with an average of 3 times a month.
- It’s most common amongst the ages of 5 to 10 in children, and 20 to 50 in adults.
- Boys with SCD have a 12% chance of experiencing priapism in their first decade of life, 50% by the age of 15, and around 90% by age 20.
- The average age to experience the first episode is 15 years old.
- Almost all patients with SCD have their first episode before turning 30.
- Two-thirds of children who show signs of priapism also have SCD.
- In children with SCD, the rate of pediatric priapism is between 25% and 30%.
Signs and Symptoms of Stuttering Priapism
In the emergency room, it’s vital to figure out what type of priapism (a prolonged erection that can cause pain and isn’t due to sexual excitement) a person is experiencing so treatment can start promptly. There are different forms such as ischemic priapism, which comes with increasing pain and can be linked to certain health conditions like sickle cell disease, other blood disorders, or neurological illnesses. High-flow priapism, another form, often stems from injury to the genital or pelvic area, or specific procedures, and leads to a long-lasting erection but no pain.
Another type is stuttering priapism, and doctors need relevant information about occurrences, including frequency, length, triggers, and any associated health conditions or medications. This form usually happens as an erection that doesn’t go away in the morning or after sexual arousal. Often, patients have found ways to manage it at home, like using warm or cold packs, taking cold baths, or exercising. Doctors also need to know if it ever turned into an acute ischemic attack and how it affects the person’s life, since it can have long-term effects on sexual functions.
The physical exam on someone with acute priapism also aims to identify the type of priapism. With ischemic attacks, the corpora cavernosa (the two chambers in the penis that fill with blood to produce an erection) are very rigid and could be tender. But in high-flow priapism, they’re full but comparatively soft and painless. Doctors look for signs of injury, which is a leading cause for high-flow priapism, by examining the area around the genitals.
In people with recurring priapism, healthcare professionals should look for any abnormalities in the testicles, stomach area, the area around the genitals, rectum, and prostate. While it’s rare, lumps found in these areas can sometimes be a cause of priapism. Any fever alongside priapism is not related and could be a sign of another health condition.
Testing for Stuttering Priapism
When you visit the emergency department due to priapism or prolonged and often painful erection, doctors will run a series of tests to determine the root cause. These may include a complete blood count (CBC) and tests that assess how well your blood clots to check for anemia and infections, as well as any abnormalities related to your blood cells. If the doctors suspect you’ve been using recreational drugs, they might test your urine and blood to confirm this.
If you have a history of mental health issues, your doctors will want to see if there are psychoactive drugs (medications that change brain function and lead to alterations in perception, mood, consciousness, cognition, or behavior) in your blood. Also, a sample of blood from the erection can be tested right away to eliminate the possibility of ischemic priapism, which is caused by trapped blood in the erection. The blood sample will show an acidic pH level, high PCO2, and low PO2 if it’s ischemic priapism.
Furthermore, if you constantly experience priapism, a sickle cell screen might be needed since Sickle Cell Disease (SCD) can cause this condition. The doctors will also want to rule out other blood-related issues like leukemia, thalassemia (an inherited blood disorder that affects the body’s ability to produce hemoglobin and red blood cells), and abnormalities related to platelets (blood cells that help the blood clot to prevent excessive bleeding).
Along with the blood tests, imaging can improve the accuracy of the diagnosis. For instance, color duplex Doppler ultrasonography, a type of ultrasound that measures blood flow, might be performed to assess the blood flow in the penis. If you’re experiencing ischemic priapism, this test won’t show any significant blood flow in the cavernous arteries which are responsible for erections. Certain values of peak and average blood velocities will suggest the presence of ischemic priapism.
Under specific conditions, a CT scan or MRI can help rule out cancer as a potential cause of priapism or assess the state of health of the smooth muscle in the penis, which plays a crucial role in erections. However, a penile arteriography, imaging test that allows doctors to see the arteries in your penis, is typically only done if high-flow priapism (a rare form of priapism usually not painful and caused by ruptured arteries or veins that prevent blood in the penis from circulating normally) is suspected, since this condition doesn’t typically cause recurrent priapism.
Treatment Options for Stuttering Priapism
The main aim in treating the type of long-lasting, recurring erections known as stuttering priapism is to prevent future instances from occurring. Emergency treatment is crucial when a person experiences an episode of this condition where the erection lasts more than four hours. Doctors need to draw blood from the penis and inject medication that affects blood vessels within 24 hours. Despite this treatment, people who experience this type of episode are at high risk of complications, and that’s why the main aim of treatment is to prevent it from happening.
Recent breakthroughs in understanding how stuttering priapism works have allowed medical researchers to test new medications. The goal of these treatments is to prevent the condition from occurring. There are a few different types of treatments currently in use. These include medications that change the levels of certain hormones, drugs that influence muscle tone, and other medications that may have a positive impact on the condition.
Hormonal treatments work by reducing the levels of testosterone in the body. A study has shown that drastically reducing testosterone levels can help reduce the number of priapism episodes. These treatments last anywhere from two weeks to two months, and their effects are long-lasting. There are a few options for hormone treatment, and they all come with risks and potential side effects. It’s important to remember that these treatments can affect sperm production and sexual growth, so they’re not recommended for men who are still growing or who are trying to have children.
Other treatment options for stuttering priapism include injections of diluted pseudoephedrine directly into the tissues of the penis. This generally happens during a severe episode of priapism and isn’t used to prevent new episodes. Other medications may help to increase muscle tone or affect the body’s production of a hormone that is linked to the production of red blood cells. These medications have been successful in treating the condition in some people.
People that deal with stuttering priapism due to Sickle Cell Disease (SCD) should be aware that those with this condition typically have more severe disease, higher likelihood of stroke, more instances of acute chest syndrome, and lower average hemoglobin levels. There are treatments specifically tailored for these patients which can be helpful in preventing episodes of priapism. Studies have shown that a low daily dose of sildenafil can help regulate blood flow and prevent episodes of priapism in both men with and without SCD.
Finally, in severe or stubborn cases where other methods have failed, there are surgical options such as the placement of a penile prosthesis. This involves a device that is implanted into the penis which can help to restore sexual function, however it does come with its own set of risks and complications. It is recommended to try all other treatment options before resorting to this surgical intervention. As of now, there are no clear guidelines for managing and preventing stuttering priapism. More research is needed on this topic.
What else can Stuttering Priapism be?
It’s crucial to tell the difference between stuttering priapism and acute ischemic priapism right away. This immediate identification and treatment is key to prevent further problems.
What to expect with Stuttering Priapism
Stuttering priapism, a condition involving abnormal, prolonged penile erections, only leads to erectile dysfunction (ED) in about one-quarter of patients. However, it’s important to note that around half of all people with stuttering priapism may develop a more severe condition called acute ischemic priapism. This condition can cause ED if not treated promptly and correctly. So, it’s crucial to seek medical help if you’re experiencing symptoms of priapism to avoid potential complications.
Possible Complications When Diagnosed with Stuttering Priapism
The complications linked to stuttering priapism are not due directly to the condition itself. Instead, they develop from acute, severe episodes of low blood flow to the penis if they occur. If untreated within a day, this condition can result in hardening of the tissues in the penis (corpus cavernosum). Over time, this can lead to severe erectile dysfunction (ED). More than half of patients who experience even a single incident of prolonged, painful erections (priapism) will develop ED. This risk increases with each additional episode.
Doctors treating this condition are responsible for ensuring patient outcomes, however, upset patients due to poor results or repeated priapism episodes can increase the risk for the physicians. Therefore, detailed record-keeping is advised. It’s important to note that stuttering priapism can have a significant psychological impact as well. Feelings of sadness in 70% of patients, embarrassment in 62%, fear in 44%, and fatigue in 39% have been reported. It’s essential that these emotional issues aren’t overlooked and are appropriately managed.
Complications and Considerations:
- Fibrosis of the penis leading to ED, if not treated within 24 hours
- Risk of ED increases with each occurrence of prolonged priapism
- Relatively high responsibility for the doctors treating this condition
- Emotional effects including sadness, embarrassment, fear, and fatigue
Preventing Stuttering Priapism
A survey found that only 7% of individuals with Sickle Cell Disease (SCD), who have not experienced priapism, knew that this condition could be a complication of SCD. “Priapism” is a long-lasting, often painful erection that isn’t related to sexual arousal or desire. The same survey found that only half of the people with SCD who had experienced priapism had sought medical advice for it. These findings suggest that priapism may be more widespread among those with SCD than previously known.
Educating people known to have a disease that places them at an increased risk of priapism, such as SCD, certain blood diseases, and different types of cancer, is crucial. This education should include explaining techniques they can use at home to relieve episodes of priapism, and when it’s important to seek medical advice to prevent or reduce complications. Discussing the potential impact of these conditions on a person’s future sexual health could also help lessen the emotional and social impact on their life and family.
