Interstitial Cystitis: A Bladder Disorder The urinary bladder is a storage organ that resembles a balloon in some of its properties. Like a balloon, the bladder's elastic walls relax and expand to store urine and contract and flatten when urine is emptied through the urethra. The typical adult bladder can store about 350ml of urine. Adults pass about a litre and a half of urine each day. The amount of urine varies, depending on the fluids and foods a person consumes. The volume formed at night is about half that formed in the daytime. Normal urine is sterile. It contains fluids, salts and waste products, but it is free of bacteria, viruses and fungi. The tissues of the bladder are isolated from urine and toxic substances by a coating that discourages bacteria from attaching and growing on the bladder wall. People with interstitial cystitis (IC) have an inflamed bladder wall. This inflammation can lead to scarring and stiffening of the bladder, decreased bladder capacity, glomerulations (pinpoint bleeding) and, in rare cases, ulcers in the bladder lining. IC, also known as painful bladder syndrome and frequency-urgency-dysuria syndrome, is a complex, chronic disorder that has baffled doctors for as long as it has been recognized. Estimates of the number of people who have IC run as high as 500,000, but no one knows for sure how many people have it. About 90 percent of IC patients are women. While people of any age can be affected, about two-thirds of patients are in their twenties, thirties, or forties. IC is rare in children. In a few cases, IC has afflicted both mother and daughter, but there is no evidence that the disorder is hereditary, or genetically passed from parent to child.
Two Types of IC Because IC varies so much in its symptoms and severity, most researchers believe that it is not one but several diseases. Two types of IC are usually described; they are mainly distinguished by whether ulcers have formed on the bladder wall. Most researchers believe that IC does not generally progress from the non-ulcerative to the ulcerative form.
Non-ulcerative IC This disorder is the most common type of IC. It usually affects young to middle-age women who have a normal, near normal, or increased bladder capacity when measured under general anaesthesia. Glomerulations can be seen in the bladder wall at cystoscopy.
Ulcerative IC This type of IC tends to be found in middle-aged and older women. Bladder capacity is low (less than 1 1/2 cups) when measured under general anaesthesia. The decrease is thought to result partly from fibrosis, the formation of threadlike tissue that makes the bladder stiff and small. Cracks, scars, and Hunner's ulcers (star-shaped sores) in the bladder wall may bleed when the bladder is filled to capacity during a cystoscopy.
Causes of IC No one knows what causes IC, but doctors studying the disorder believe it is a real, physical problem - not the result of an emotional problem. One area of research on the cause of IC has focused on the lining of the bladder called the glycocalyx, made up primarily of substances called mucins and glycosaminoglycans (GAGs). This layer normally protects the bladder wall from toxic effects of urine and its contents. Researchers at the University of California, San Diego, found that this protective layer of the bladder was "leaky" in about 70 percent of IC patients they examined and may allow substances in urine to pass into the bladder wall and trigger IC symptoms. The researchers also found that patients with Hunner's ulcers had "leakier" bladders than patients without the ulcers. Some people are diagnosed with IC after taking antibiotics for a presumed urinary tract infection. Therefore, it has been suggested that antibiotics may damage the bladder wall and make it "leaky." This idea has been studied carefully, but antibiotics have never been found to harm the bladder wall. It is possible that the infection started an autoimmune response against the bladder, the patient's original symptoms were from IC all along, or an infecting organism is in the bladder cells but is not detectable through routine tests.
Symptoms The symptoms of IC vary greatly from one person to another but have some similarities to those of a urinary tract infection:
In most women, symptoms usually worsen around the menstrual cycle. As with many other illnesses, stress may also intensify symptoms but does not cause them.
Diagnosis Because the symptoms of IC are similar to those of other disorders of the urinary system, and because there is no definitive test to identify IC, doctors must rule out other conditions before considering a diagnosis of IC. Among these disorders are urinary tract or vaginal infections, bladder cancer, bladder inflammation or infection caused by radiation to the abdomen, eosinophilic and tuberculous cystitis, kidney stones, endometriosis, neurological disorders, sexually transmitted diseases, low-count bacteriuria, and, in men, chronic bacterial and abacterial prostatitis.
The diagnosis of IC in the general population is based on
Medical tests that help identify other conditions include a urinalysis, urine culture, cystoscopy, and biopsy of the bladder wall and, in men, laboratory examination of prostate secretions.
Urinalysis and Urine Culture These tests can detect and identify the most common organisms in the urine that may be causing symptoms. There are, however, organisms such as the bacteria chlamydia that can't be detected with these tests, so a negative culture does not rule out all types of infection. A urine sample is obtained either by catheterisation or by the "clean catch" method. For a "clean catch," the patient washes the genital area before collecting urine "midstream" in a sterile container. White and red blood cells and bacteria in the urine may indicate an infection of the urinary tract, which can be treated with an antibiotic. If urine is sterile for weeks or months while symptoms persist, a doctor may consider a diagnosis of IC.
Culture of Prostate Secretions In men, the doctor will obtain prostatic fluid from the patient. This fluid will be examined for signs of an infection, which can be treated with antibiotics.
Cystoscopy under Anaesthesia With Bladder Distension For this, the doctor uses a cystoscope - an instrument made of a hollow tube about the diameter of a drinking straw with several lenses and a light - to see inside the bladder and urethra. The doctor will also distend or stretch the bladder to its capacity by filling it with water or saline. Because bladder distension is painful in IC patients, before the doctor inserts the cystoscope through the urethra into the bladder, the patient must be given either regional or general anaesthesia. These tests can detect inflammation; a thick, stiff bladder wall; Hunner's ulcers; and glomerulations (pinpoint bleeding) that may be seen only after the bladder is stretched. The doctor may also test the patient's maximum bladder capacity, the amount of liquid or gas the bladder can hold under anaesthesia. Without anaesthesia, capacity is limited by either pain or a severe urge to urinate. Many people with IC have normal or large maximum bladder capacities under anaesthesia. However, a small bladder capacity under anaesthesia helps to support the diagnosis of IC.
Biopsy A biopsy is a microscopic examination of tissue. Samples of the bladder and urethra may be removed during a cystoscopy and examined with a microscope later. A biopsy helps rule out bladder cancer and confirm bladder wall inflammation.
Treatment Scientists have not yet found a cure for IC, nor can they predict who will respond best to which treatment. Symptoms may disappear without explanation or coincide with an event such as a change in diet or treatment. Even when symptoms disappear, however, they may return after days, weeks, months, or years. Scientists do not know why. Because doctors do not know what causes IC, treatments are aimed at relieving symptoms. Most people are helped, for variable periods of time, by one or a combination of treatments described here. However, as researchers learn more about IC, the list of potential treatments may change. Patients should discuss treatment options with a doctor.
Bladder Distension Because some patients have noted an improvement in symptoms after a bladder distension done to diagnose IC, the procedure is often thought of as one of the first treatment attempts. Researchers are not sure why distension helps, but some believe that the procedure may increase bladder capacity and interfere with pain signals transmitted by nerves in the bladder. Symptoms may temporarily worsen 24 to 48 hours after distension, but should then return to pre-distension levels or improve after 2 to 4 weeks.
Bladder Instillation This procedure may also be called a bladder wash or bath. During a bladder instillation, the bladder is filled with a solution that is held for varying periods of time, from a few seconds to 15 minutes, before being drained through a narrow tube called a catheter. The only drug approved by the U.S. Food and Drug Administration (FDA) for bladder instillation is Dimethyl sulfoxide (DMSO, RIMSO-50). With DMSO treatments a catheter is guided up the urethra into the bladder. A measured amount of DMSO is passed through the catheter into the bladder, where it is retained for about 15 minutes before being expelled. Treatments are given every week or two for 6 to 8 weeks, and repeated as needed. Most people with IC who respond to DMSO notice improvement of symptoms 3 or 4 weeks after the first 6-to-8 week cycle of treatments. Highly motivated patients who are willing to catheterise themselves may, after consultation with their doctor, be able to have DMSO treatments at home. Self-administration of DMSO is less expensive and more convenient than going to the doctor's office. Doctors think DMSO works in several ways. Because it passes into the bladder wall, DMSO may more effectively reach tissue to reduce inflammation and block pain. It may also prevent muscle contractions that may cause pain, frequency, and urgency. A bothersome but relatively insignificant side effect of DMSO treatments is a garlic-like taste and odour from the breath and skin. This may last up to 72 hours after a treatment. Long-term DMSO treatment has caused cataracts in animal studies, but this side effect has not appeared in humans. Blood tests, including a complete blood count and kidney and liver function tests, should be done about every 6 months. A variety of other drugs have been used experimentally for bladder washes, including silver nitrate, sodium oxychlorosene (Clorpactin WCS-90), heparin, and pentosan polysulfate (Elmiron). Silver nitrate and oxychlorosene sodium are thought to work by first attacking the bladder lining. This triggers the body's immune system to step in and start the healing process. Some patients have been successfully treated with these drugs, but the frequent, painful treatments usually must be carried out under general anaesthesia. Neither drug can be used in people who have urinary reflux, a condition in which urine flows backward up the ureters into the kidneys. Heparin and pentosan polysulfate are thought to work by replacing or repairing the "leaky" bladder lining.
Oral Drugs All drugs--even those sold over-the-counter--have side effects. Patients should always consult a doctor before using any drug for an extended time. Aspirin and Ibuprofen are easy to obtain and may be a first line of defence against mild discomfort. However, they may make symptoms worse in some patients. Over-the-counter forms of phenazopyridine hydrochloride (Azo-Standard, Prodium, and Uristat) may provide some relief from urinary pain, urgency, frequency, and burning. Higher doses of the drug are available by prescription as Prodium and Pyridium. Oxybutynin chloride (Ditropan) and a blend of Atropine, Hyoscamine, Methenamine, Methylene blue, Phenyl salicylate and Benzoic acid (Urised) may help reduce bladder spasms that can cause frequency, urgency, and night-time trips to the bathroom. Urised may also inhibit the growth of organisms in the urine. Amitriptyline (Elavil) and Doxepin (Sinequan) act as antidepressants when given in large doses. In smaller doses, they can help IC symptoms by blocking pain, calming bladder spasms, and decreasing inflammation. Too much histamine in the bladder may cause some cases of IC. Antihistamine drugs such as Hydroxyzine (Vistaril and Atarax) and Cimetidine (Tagamet) relieve symptoms in some IC patients. If taken at bedtime, hydroxyzine may also help patients sleep. Nifedepine (Procardia) is a treatment for heart disease and high blood pressure, but it has reduced bladder pain and urgency in some IC patients. Pentosan polysulfate sodium (Elmiron) reduces bladder discomfort and pain in some people with IC. Doctors don't know exactly how the drug works, but they believe it may repair leaks in the bladder lining. Elmiron is the first oral drug developed for IC and approved by FDA.
TENS (Transcutaneous Electrical Nerve Stimulation) With TENS, mild electric pulses enter the body through wires placed on the lower back or the suprapubic region (between the navel and the pubic hair) or through special devices inserted into the vagina in women or into the rectum in men. Although scientists don't know exactly how it works, it has been suggested that the electric pulses may increase blood flow to the bladder, strengthen pelvic muscles that help control the bladder, and trigger the release of hormones that block pain. TENS is relatively inexpensive and allows the patient to take an active part in treatment. Within some guidelines, the patient decides when, how long, and at what intensity TENS will be used. TENS has been most helpful in relieving pain and decreasing frequency in IC patients who have Hunner's ulcers. Smokers do not respond as well as non-smokers. If TENS is going to help, change usually occurs in 3 to 4 months.
Diet There is no scientific evidence linking diet to IC, but some doctors and patients believe that alcohol, tomatoes, spices, chocolate, caffeinated and citrus beverages, and high-acid foods may contribute to bladder irritation and inflammation. Some patients also notice a worsening of symptoms after eating or drinking products containing artificial sweeteners. Patients may try eliminating such products from their diet and reintroduce them one at a time to determine which, if any, affect symptoms. It is important, however, to maintain a well-balanced and varied diet.
Smoking Many IC patients feel that smoking worsens their symptoms. Because smoking is the major known cause of bladder cancer, one of the best things a smoker can do for the bladder is to quit smoking. Bladder cancer developing smokers may go undetected in such patients.
Exercise Many IC patients feel that regular exercise helps relieve symptoms and, in some cases, hastens remission.
Bladder Training People who have found some relief from pain may be able to reduce frequency, using bladder-training techniques. Methods vary, but basically the patient decides to void at designated times and use relaxation techniques and distractions to help keep to the schedule. Gradually, the patient tries to lengthen the time between the scheduled voids. A diary of voids is usually helpful in keeping track of progress.
Surgery This option is considered only if an IC patient has failed all available treatments and the pain is severe. Most doctors are reluctant to operate because the outcome is unpredictable in individual patients as some people have surgery and still have symptoms. Anyone considering surgery should discuss the potential risks and benefits, side effects, and long- and short-term complications with a surgeon and family, as well as with people who already have had the procedure. Surgery requires anaesthesia, hospitalisation, and weeks or months of recovery. As the complexity of the procedure increases, so do the chances of complications and failure.
Transurethral fulguration and resection of ulcers: Fulguration involves burning Hunner's ulcers using electricity or a laser. When the area heals, the dead tissue and the ulcer fall off, leaving new, healthy tissue behind. Resection involves cutting around and removing the ulcers. Both treatments, done under anaesthesia, use special instruments inserted into the bladder through a cystoscope. Laser surgery in the urinary tract should only be done by doctors who have the special training and expertise needed to perform the procedure.
Denervation: It is a complicated procedure done by surgeons who have special training and expertise. Rarely used in the treatment of IC, it involves cutting some of the nerves to the bladder, interfering with pain signals. Many approaches and techniques are used, each of which has its advantages and complications that should be discussed with the surgeon. Augmentation makes the bladder larger, most often by adding a section of the patient's small intestine, a tube-like structure that absorbs and transports nutrients from food for use by the body. With this treatment, scarred, ulcerated and inflamed sections of the patient's bladder are removed, leaving only healthy tissue and the base of the bladder. A piece of the patient's small intestine is removed, reshaped, and attached to what remains of the bladder. After the incisions heal, the patient may be able to void normally. Even in carefully selected patients - those with small, contracted bladders - the pain, frequency, and urgency may remain or return after surgery and the patient may have additional problems with infections in the new bladder and difficulty absorbing nutrients from the shortened intestine. Some patients are incontinent while others cannot void at all and must insert a catheter into the urethra to empty urine from the bladder.
Bladder Removal (Cystectomy): Different methods can be used to reroute urine once the bladder has been removed. In most cases, the ureters are attached to a piece of bowel that opens onto the skin of the abdomen, called a stoma. Urine empties through the stoma into a bag outside the body. This procedure is called a urostomy. Some Urologists are using a technique that also requires a stoma but allows urine to be stored in a pouch inside the abdomen. At intervals throughout the day, the patient passes a catheter into the stoma and empties the pouch. Patients with either type of urostomy must use very clean, or sterile, steps to prevent infections in and around the stoma. With a third method, a new bladder is made from a piece of the patient's bowel (large intestine) and attached to the urethra in place of the removed bladder. After a time of healing, the patient may be able to empty the bladder by voiding at scheduled times or may insert a catheter into the urethra. Few surgeons have the special training and expertise needed to perform this procedure. Even after total bladder removal, some patients still experience variable symptoms of IC. Therefore, the decision to undergo a cystectomy should only be undertaken after serious deliberation on the potential outcome.
Electrical Nerve Stimulation: This surgical treatment is a variation of TENS, described previously, but involves permanent implantation of electrodes and a unit that emits continuous electrical pulses. This relatively new procedure has variable short-term results, unknown long-term effects and, therefore, is not widely used.
Special Concerns Cancer There is no evidence that IC increases the risk of bladder cancer. However, the long-term effects of
IC require further observation and research.
Pregnancy Researchers have little information about pregnancy and IC, but believe that the disorder does not affect fertility or the health of the foetus. Some women have a remission from IC during pregnancy, while others have more pain and pressure during the third trimester, possibly due to the weight of the foetus on the bladder.
Working Symptom flare-ups that result in frequent absences from work may make it difficult to get or keep a job. The Social Security Administration provides information on Social Security Disability benefits in countries where this is available.
Coping The emotional support of family, friends, and other people with IC is very important in helping patients cope with the disorder. Studies have found that IC patients who learn about the disorder and become involved in their own care do better than patients who do not. The Interstitial Cystitis Association can provide the address and phone number of the nearest support group wherever such associations and groups are active.
Other coping tips
Suggested Reading The materials listed below may be found in medical libraries, many college and university libraries, through inter-library loan in most public libraries, and at bookstores. Items are listed for information only; inclusion does not imply endorsement.
Articles and Book Chapters
Books and Booklets
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