Ehlers Danlos Urinary Retention

People who suffer from Ehlers-Danlos syndrome (EDS) may experience bladder issues such as stress incontinence or infection in the bladder. Some facts to be aware of when dealing with such symptoms and some tips on how to handle them are described below.

What is Ehlers-Danlos Syndrome?

Quite simply, EDS is a group of genetic disorders that affect the connective tissues in our joints, skin, blood vessels, and organs, among other things. Strength and flexibility are provided by connective tissue, which comprises a complex mixture of proteins (such as collagen). Different patients experience EDS in different ways, ranging from mildly loose joints to potentially life-threatening complications.

There are thirteen different types of EDS. EDS affects around 1 in every 5,000 persons globally, with some variants being extremely uncommon. While each has its unique set of features and consequences, they all result in very flexible joints, a condition known as hypermobility. Chronic joint pain, joint dislocation, early-onset arthritis, and slow wound healing are possible consequences of hypermobility.

EDS is a hereditary disorder that affects the nervous system. Researchers have discovered 19 distinct genes that are linked to the condition. The genes that encode the amount of collagen produced by the body are the most affected ones. The presence of a mutated collagen gene results in structural weakness, which then causes problems.

Genetic mutations confer a 50 percent probability of transmitting the gene to future generations in those who carry the mutations (whether they are silent carriers, have moderate symptoms, or have severe symptoms).

EDS does not have a cure at this time; however, effective therapies are available to control its symptoms.

What are the most prevalent subtypes of EDS, as well as their symptoms?

The most frequent variant of EDS is referred to as “classic EDS,” which is short for “classic EDS.” Extremely flexible joints, elastic skin, and delicate skin are some of the most common symptoms. Even within the subtypes, however, not every patient exhibits all of the associated symptoms. Hypermobility may be present in certain people without the presence of skin signs in others. In addition to the above symptoms, you may experience: easily bruised skin, muscle pain; muscle fatigue; redundant skin folds around the eyes; benign growths on pressure points (such as the elbows and knees); and heart valve problems. During a diagnosis, many doctors will use echocardiography in conjunction with genetic tests and a skin sample.

Another subtype of EDS that has the potential to cause cardiac issues is vascular EDS. It causes the aorta, as well as the arteries throughout the body, to become weakened. This significant blood artery can burst and cause death if it is not repaired in time. Vascular EDS can also cause the walls of the uterus and the large intestine to become weakened. The patient’s features include a thin nose, a thin upper lip, small earlobes, and prominent eyes, among other things. Their skin is generally light and transparent, and it bruises readily as a result of this.

Hypermobile EDS is the last and most frequent kind of EDS (hEDS). Some of the signs and symptoms of this condition include loose joints, easy bruising; muscular discomfort; muscle tiredness; chronic pain; chronic degenerative joint disease, early-onset osteoarthritis, and heart valve issues. Genetic testing is not accessible for hEDS since there has not yet been a gene associated with it discovered. To rule out other possible reasons, clinicians look for three things: joint hypermobility, persistent muscle and joint discomfort (which may be accompanied by a family history of EDS), and the elimination of any other possible causes.

What is the impact of EDS on the bladder?

It seems to reason that EDS might have an impact on the urinary system. Connective tissue in the bladder aids in the movement required to remove waste products from the body. That tissue can become aberrant, resulting in urinary incontinence, overactivity of the bladder, and retention of bladder contents.

Connective tissue dysfunction can also induce uterine, vaginal, or bladder prolapse, resulting in bladder discomfort and other complications. Compared to those who do not have EDS, patients with EDS are more than twice as likely to develop urinary incontinence.

Pelvic floor dysfunction is common in EDS patients, and patients who receive pelvic floor physical therapy will benefit from it.

Those suffering from hEDS are more likely than others to experience bowel symptoms. When connective tissue in the intestine becomes impacted, it can cause constipation and motility problems. In rare situations, it can even result in the paralysis of the digestive tract, which is quite dangerous.

What causes EDS to produce bladder problems?

EDS is a connective tissue disorder affecting connective tissue throughout the body, including the bladder. This tissue is critical in enabling the movement required for the expulsion of waste from the body. Any irregularity in the connective tissue has the potential to impair this function.

Bladder difficulties in EDS patients might be caused by various factors, including abnormalities in the urinary tract and pelvis architecture in certain people, malfunction of the involuntary portion of the nervous system that regulates the bladder, inflammation, or bowel disorders.

Urinary Retention and EDS

The inability to empty the bladder of pee is referred to as urinary retention. Retention can be total or partial, and it can be acute or chronic. Chronic retention of urine is described as a nonpainful bladder that remains perceptible after voiding. Chronic urinary retention (urinary retention) is used in research settings to indicate a persistent failure to empty the bladder despite the capacity to pee. This results in higher postvoid residual (PVR) urine volumes, which are measured in research settings. When it comes to treating and diagnosing urinary retention, there does not appear to be much consistency in terms of the amount of time or PVR volume required. PVR volumes more significant than 300 mL are frequently utilized in research investigations to diagnose urinary retention; but, smaller volumes like 100 mL, 400 mL, and 500 mL have also been employed.

It is not known what the frequency and prevalence of urinary retention are. Individuals with diseases often linked with urinary retention have been studied in small groups, but modest information has been obtained on the overall burden of urinary retention. However, it is well acknowledged that this disease affects older adults at a higher rate than any other population segment.

Urinary retention usually develops gradually for months to years, and it is not typically painful. Asymptomatic urinary retention is possible, although it is more common when coupled with symptoms of the lower urinary tract, such as frequent urination, urgency, or incontinence. Residual causes might be classified as obstructive, infectious or inflammatory, neurologic, or other. The obstructive causes of urethral strictures include male benign prostatic hyperplasia (BPH), female organ prolapse, and urethral strictures in male and female patients. For example, Guillain-Barre syndrome and the herpes simplex virus are both examples of infectious or inflammatory causes. Spinal cord damage, stroke, multiple sclerosis, and diabetes mellitus are only a few examples of neurologic causes. In addition to these factors, Fowler’s syndrome (in women), trauma, surgical complications, and psychogenic disorders are also possibilities.

Patients who have urinary retention may be at increased risk for urinary tract infections as well as acute urinary retention, which is defined as the sudden onset of the complete or near-complete inability to urinate despite the desire or effort to do so. Patients who have urinary retention may also be at increased risk for kidney stones. Acute urine retention is generally associated with lower abdomen discomfort, and it has the potential to cause infection, renal failure, and death if not treated promptly.

Assessments and Tests

Your physician may do the following procedures to diagnose your condition correctly:

  • In addition, a visual examination is performed to check for probable pelvic organ prolapse and other anatomical problems.
  • Conducting urine tests to rule out any other potential issues.
  • Requesting that you keep a bladder diary to record everyday occurrences relating to your bladder, such as the number of times you pee, the volume of urine produced, and the time it takes you to empty it.
  • Gently pressing on your bladder to determine the pressure in your bladder.
  • examining and testing the function and mobility of your urethra and pelvic floor muscles;
  • conducting a biopsy of the inner lining of your bladder.

Possible Treatments

The etiology of the condition determines the treatment for urinary retention. As a result, clinicians may first run testing to determine the cause of the problem. When making testing selections, the existence and severity of symptoms are taken into account. The following are examples of tests that are frequently performed:

  • Urinalysis
  • Urine culture Measures of renal function are available.
  • A test for prostate-specific antigen (PSA).
  • Urodynamic testing is performed.
  • Ultrasound of the kidneys, bladder, or transrectal prostate
  • Computed tomography of the brain or pelvis (CT)
  • Magnetic resonance imaging (MRI) of the brain or lumbosacral spine
  • Cystoscopy
  • Retrograde cystourethrography

Even though testing is widely used, there is no uniform set of tests or agreement on whether testing enhances treatment results or causes problems.

The therapy of urinary retention might include various options such as catheterization, surgical procedures, minimally invasive procedures, and pharmaceutical therapies. The underlying cause determines the treatment choices accessible to patients. If the retention is high-pressure or low-pressure (detrusor pressure after micturition), it may be crucial to establish whether it is high-pressure or low-pressure in males since this may influence treatment options. Although there is some agreement on the relative advantages and risks of the various treatment methods available for urinary retention, there is no agreement on how effective they are.

There are various approaches to managing an overactive bladder, including changes in one’s way of life. Among the alternatives are:

  • It is important to maintain a healthy weight since less weight in the abdomen area puts less strain on the bladder.
  • Drinking coffee, tea, carbonated beverages, alcoholic beverages and spicy meals, tomato-based goods, chocolate, and some acidic fruits such as oranges, grapefruit, lemons, and limes are all bladder irritants to avoid.
  • Avoiding things that contain a lot of fluid, such as soup.
  • Controlling the intake of water and other fluids.
  • It is necessary to retrain your bladder, which entails changing your behaviors, such as noting your urine pattern, increasing your pee intervals, and following a timetable.
  • Strengthening your pelvic floor muscles by performing specific workouts to make them stronger.
  • Increased physical exercise to the greatest extent is feasible.
  • Keeping on top of any persistent coughing spells, which might exacerbate your bladder condition.
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