Genetics Northern California

Bladder concerns

Neurogenic bladder
Every part of the body needs nerves to function properly. Nerves carry signals from the various parts of the body to the brain and back again. This allows the body and brain to communicate with each other. If these signals are interrupted, they may send the wrong message, or no message at all.

A "neurogenic" bladder results when the nerves connected to the bladder do not function properly.  The bladder cannot tell the brain that it is full, and the brain cannot tell the bladder to empty completely or at an appropriate time. A neurogenic bladder affects people with spina bifida in several important ways, including the problems described below.

Urinary reflux and kidney disease
Normally, urine flows from the kidneys, down tubes called ureters, into the bladder, and out of the body through another tube called the urethra. With neurogenic bladder, the smooth muscle of the bladder wall does not have normal muscle tone, making it less able to empty completely. The bladder can also be spastic or overly excitable.

If the bladder muscles stay contracted, and if the strong muscle at the base of the bladder (called a sphincter) is also tight, the pressure within the bladder rises. In this case the only place for the urine to go is back up into the kidneys. This backflow is called reflux, and reflux can cause kidney infections and permanent kidney damage.

The kidneys are critical for healthy body functioning. Therefore, people with spina bifida must learn and maintain a good bladder management program and be monitored routinely for the development of kidney problems. This is why regular tests for the kidneys are recommended, including the annual renal/bladder ultrasound.

Urinary incontinence
Urinary incontinence is an uncontrolled leaking of urine. This is a common problem for people with neurogenic bladder. The incontinence can be complicated by a spastic bladder, a small bladder, or a weak outlet sphincter. It can be aggravated by a urinary tract infection, bowel impaction (as result of severe constipation), or tethered cord syndrome.

Generally a person with a neurogenic bladder cannot tell when their bladder is full, or control the muscles that allow the bladder to empty. Without treatment, a person with urinary incontinence has to wear diapers, but there are alternatives. The main treatment for people with neurogenic bladders is establishing a regular bladder program using a combination of bladder catheterization and medications. In some cases these measures will not be enough, and surgery will be recommended.

Clean intermittent catheterization (CIC)
A bladder management program is a routine in which a person empties their bladder at regular intervals throughout the day. To maintain a healthy urinary system and prevent leaking, the bladder should be completely emptied every 3-4 waking hours (for a total of 5-6 times per day). However, if a person cannot urinate in the ordinary way, another technique must be used to empty the bladder. This technique is called clean intermittent catheterization or CIC.

A catheter is a small tube that is inserted through the urethra (opening at the end of the penis in males or in the vulvar area in females) into the bladder in order to drain the urine. When this is done at different times throughout the day, it is referred to as "intermittent" catheterization. Catheterization is "clean" when the person uses careful handwashing, cleans the catheter after every use, and prevents their hands or the catheter from touching dirty surfaces before the procedure.

There is rarely any need to use sterilized catheters or to take special care to sterilize the urethral opening. Typically catheters can be used over and over again, as long as care is taken to clean and air-dry them between uses. Research has shown that as long as the person uses clean procedure, there is little or no added risk of infection. CIC is usually begun in infancy and performed by the child's caregivers. However, most people with spina bifida can learn to independently catheterize themselves between the ages of six and eight if they have adequate fine motor control of their upper extremities.

Cleaning urinary catheters
We suggest that when cleaning your catheters following CIC that you wash the catheters using warm soapy water. Rinse well. Allow them to air dry and place in a clean plastic bag for reuse the next day. This is the simplest most straightforward method for cleaning your catheters. It is both effective and reliable. 

We suggest that you start each day by placing all the catheters that you will need for the day in a plastic bag marked "clean". Carry it with you to school or work. Each time you catheterize, use a different catheter. After each catheterization, place the used catheter in a separate bag labeled "used". At the end of the day, gather all the used catheters and clean them.  The catheters are now ready for repeat usage.

Please note that CIC is not a sterile procedure and as soon as the catheter is removed from the bag (or package, even if new) it is no longer sterile. Again, CIC is a CLEAN procedure. The catheters can be cleaned many times before they need to be thrown away. These cleaning procedures for catheter reuse should be done daily until the catheters become stiff or discolored. When they do, discard them.

Medications for neurogenic bladder
As helpful as clean intermittent catheterization (CIC) is in emptying the bladder and preventing infections, it is often not enough to prevent urine leakage between catheterizations. This is because of other conditions that are often present, such as:

  1. small bladder size

  2. high-pressure bladder

  3. weak urethral sphincter muscle

These conditions all require some extra help, in addition to CIC, to achieve urinary continence. The medications most frequently prescribed to promote urinary continence are described below:

Bladder antispasmodics:
These medications allow the bladder to relax and hold more urine. They are helpful for people with small or high-pressure bladders.

  • Oxybutynin (Ditropan)

  • Tolterodine tartrate (Detrol)

  • Hyoscyamine (Levbid)

Bladder neck tone increasers:
These medications cause the outlet sphincter to contract more tightly, and prevent urine from leaking. They are helpful for people with weak urethral openings.

  • Imipramine (Tofranil)

  • Pseudoephedrine with or without Chlorpheniramine (Deconamine or Sudafed)

  • Amitriptyline (Elavil)

Urinary tract infections/colonization
The presence of microorganisms (bacteria or germs) in the bladder is common among people with spina bifida, especially those who catheterize. This is not surprising, considering that a foreign object (i.e., the catheter) is inserted into the bladder five to six times a day throughout the lifetime.

We call the presence of bacteria in the urine in the absence of symptoms (such as fever, nausea/vomiting, foul smelling urine, flank/abdominal pain, urgency, and blood in the urine) "colonization", not infection. We do not treat colonized urine because the mere presence of these microorganisms usually does not cause symptoms.  Over-treatment with antibiotics can lead to antibiotic resistance (the usual antibiotics no longer work to treat the infection), and can also allow stronger (harder to treat) bacteria to grow in the bladder. We do not want this to happen.

There are exceptions to this general rule. For instance, people with active urinary reflux or problems with kidney function may be given daily, low-dose antibiotics by their doctor to prevent infections, even though they are not experiencing any symptoms. This is because reflux can allow an ordinary bladder colonization to spread to the kidneys. This could lead to "pyelonephritis", or a kidney infection, which is much more serious.

Signs and symptoms of UTI:
Urinary tract infections (UTIs) are caused by unwanted microorganisms entering and growing in the bladder. There are many possible signs and symptoms of a UTI. A person with a UTI may experience one or more of the following:

  • fever

  • nausea / vomiting

  • foul-smelling urine

  • flank / abdominal pain

  • urgency

  • blood in urine

Note:  Cloudy urine is not a reliable sign of a UTI, because it could be due to sediment rather than an infection. Cloudy urine is often an indication that the person needs to drink more water in order to flush the sediment out. In some cases, your urologist may recommend direct irrigation of the bladder (see Bladder Irrigation).

Diagnosis
When a UTI is suspected, a urine sample will usually be requested. Your clinician will order some lab tests:  a urinalysis, and a urine culture and sensitivity test. The "culture" part of the test determines what kind of microorganism is causing the infection. It may take up to 72 hours for the culture to be completed. The "sensitivity" part of the test determines which antibiotics will be most effective.

Remember that urine cultures will be positive in 80% of people on CIC, and so it is difficult to be sure whether a true infection exists. This is why we check the clinical symptoms (as listed above) in addition to the presence of bacteria when making the decision whether or not to treat with antibiotics.

Treatment:
Treatment is based on the results of the laboratory tests. The medication prescribed by the doctor, (an antibiotic) must be taken exactly as prescribed. If it is ordered for 10 days, but you feel better after five days, do not stop taking the antibiotic. If all of the microorganisms have not been killed, those few remaining can develop resistance to the medication, making that medication less effective the next time it is needed .

Prevention:
There are steps you can take to help prevent UTI:

  1. Use clean procedure. Be especially careful with handwashing before catheterization. This greatly reduces the chance of any harmful organisms entering the bladder during catheterization.

  2. Completely empty the bladder with each catheterization. Urine left in the bladder for long periods of time is more likely to grow bacteria.

  3. Maintain good hygiene by following regular programs of bowel and bladder care. Dirty or wet diapers can be a breeding ground for infection-causing organisms.

  4. Drink plenty of liquids. Water is best. Adults should try to drink six to eight 8-ounce glasses per day, and young children four to six glasses per day.

  5. Avoid drinks with carbonation, caffeine and sugar. Carbonated drinks have been associated with bladder irritation.  Caffeinated drinks will dehydrate the body and aggravate a spastic bladder. Sugar adds unnecessary calories, is a food source for bacteria, and can even cause constipation. Sugar-free mixes (e.g. Crystal Light, Kool-Aid) can be used to flavor water if desired.

Bladder irrigation
Patients who have had bladder surgery, known as "augmentation", which uses a part of the intestine to increase the bladder size, are encouraged to irrigate their bladders. This is typically a lifelong necessity. After the operation, mucus continues to be produced by the intestinal tissue. Mucus left in the bladder can lead to stone formation and a higher incidence of urinary tract infections. In addition, mucus left in the bladder will interfere with proper urine drainage.

Clients prone to urinary tract infections may also be instructed to irrigate their bladders once or twice a day for a short period of time, and will be told to drink more fluids when they notice early warning signs such as cloudy looking urine. The irrigation process is straightforward: After catheterizing, the catheter is left inside the urethra and 180 to 240mL of tap water or saline (sterile salt solution) is instilled into the bladder by attaching a large syringe to the other end of the catheter. The tap water or saline is then gently pumped in and out of the bladder with the syringe, which cleans the bladder of any remaining mucus or debris.  The mixture is then discarded.  As with catheters, the syringe may be reused many times if rinsed well after each use.

Although this irrigation procedure can often prevent urinary tract infections from occurring, it is not curative once you already have a UTI. Active infection with symptoms means that you should visit your primary care provider for treatment.

SURGICAL INTERVENTIONS

Bladder Surgeries 
Bladder augmentation (also known as augmentation cystoplasty):
This type of surgery is often recommended when the bladder is small.  During bladder augmentation surgery, the surgeon removes part of the small or large intestine and attaches it to the bladder. This makes the bladder larger so it can hold more urine. However, it is important to continue frequent catheterization, because the urine can still flow back into the kidneys (reflux) if the bladder becomes too full.

Long-term results of bladder augmentation surgery using intestinal tissue are good, and serious complications are uncommon. However, bladder irrigations are a lifelong necessity following bladder augmentations using bowel (intestinal) tissue because of increased mucus production from the added tissue.

Mitrofanoff (appendicovesicostomy):
The Mitrofanoff procedure provides an alternative for those people having difficulty self-catheterizing without assistance. Sometimes a person simply does not have the flexibility required for self-catheterization, such as after a spine fusion. Others need or want to be able to catheterize from their wheelchair without removing layers of clothing. This can be a particular problem for those regularly catheterizing in public bathrooms.

The Mitrofanoff procedure allows a person to catheterize simply by lifting his or her shirt and catheterizing through the umbilicus (belly button) rather than the urethra. This is done by surgically creating a small opening (stoma) through the umbilicus. The appendix or a section of the intestine is then used to form a "tunnel" between the umbilicus and the bladder. This allows the catheter to be placed in through the stoma, down the tunnel, and into the bladder in order to empty it. Leaking from the stoma is prevented by a one-way valve that lets the catheter pass in, but prevents urine from flowing out.

Urethral sphincter surgeries
The following surgeries are sometimes recommended when the bladder can hold a good amount of urine and is flexible, but the sphincter muscle (the muscle that holds in and then releases the urine) is too weak. Both of these surgeries are aimed at preventing incontinence by tightening the area around the bladder neck.

Bladder neck sling: This procedure is called a "sling" because a strip of strong tissue is used to wrap around the bladder neck and pull it up against the pubic bone (the bone in the pelvis that is close to the bladder). This combination of tightening and repositioning the bladder neck prevents urine from leaking out between catheterizations.

Artificial urinary sphincter (AUS): An artificial urinary sphincter can be implanted to do the job of the weak sphincter muscle. It fits like a cuff around the bladder neck and is filled with fluid to make it tighten around the bladder neck and hold urine in. When the person wants to void, he or she squeezes a pump (located in the scrotum for males and the labia for females) to empty the cuff and allow the urine to flow out. After voiding, the pump is released and the cuff automatically refills with fluid, tightening once again around the bladder neck.

It is important to continue frequent catheterization because after these surgeries the urine no longer has an easy way out. If the bladder becomes too full, the urine will reflux up into the kidneys, which can result in permanent kidney damage.