Interstitial Cystitis
Progress Against Disabling Bladder Condition

The woman knew she was going to be fired from her job. Since her 20s, this insurance firm middle manager has suffered from interstitial cystitis (IC), an inflammatory disease of the bladder wall.

The chief symptoms Marsha (not her real name) has are a smaller than normal bladder capacity, urgent and frequent urination, feelings of pressure and pain around the bladder and in the pelvic area, and painful sexual intercourse. Now 46 years old, she has coped with these symptoms fairly well until recently, when they have worsened. On some days, Marsha is in such agony that she can barely walk.

Though sympathetic at first, Marsha's supervisor became impatient with her frequent bouts of pain and trips to the bathroom, as well as time lost from sick days and doctor's appointments, even though she's made up every minute. When she started to miss policy meetings, Marsha began to get indications that she would be fired. Her boss would comment that she couldn't know what was going on because, "You aren't always here."

Finally, when Marsha needed to urinate every 20 minutes at work and 10 times during the night, leaving her exhausted and depressed in the morning, she became so fearful of being asked to leave that she decided to retire on disability instead. She hopes to return to her job when she feels better, but so far it hasn't been possible.

Marsha counts herself among the more fortunate of IC sufferers because she receives long-term disability benefits and has both Medicare and private medical insurance. Also, unlike many other women, whose marriages and relationships are put under severe stress by IC, Marsha is lucky to have a supportive husband.

Cause Elusive

No one knows what causes IC. It wasn't recognized as a disorder until about 20 years ago. In 1978, the Food and Drug Administration approved Rimso-50, a purified form of the industrial solvent dimethyl sulfoxide (DMSO), for symptomatic relief of IC. Before that, many patients were neither diagnosed nor treated.

Because physicians could find no organic cause, the prevailing medical opinion was that IC was a "hysterical female condition," even though at least 10 percent of cases are in men. Even Campbell's Urology, the definitive text of urologic diseases, stated as late as 1986 that IC was "daunting in its evasion of being understood. [It] may represent the end stage of a bladder that has been made irritable by emotional disturbance." The book further states that interstitial cystitis may be a pathway for the discharge of unconscious hatreds.

People with IC have had to put up with this type of disbelief for a long time. Kristene E. Whitmore, M.D., chairwoman of the Department of Urology, Philadelphia Graduate Hospital, Philadelphia, Pa., says, "The average number of doctors seen before diagnosis is five, and it takes three to five years to get that diagnosis."

When the columnist Ann Landers wrote about IC in 1987, she received 10,000 letters from patients or their families, relieved that the condition was finally being recognized. A 1987 study conducted by the Urban Institute in Washington, D.C., found that IC makes people so miserable that they contemplate suicide four times more often than the general population and that they rate their quality of life lower than those who undergo kidney dialysis. Nearly 30 percent of IC patients can't work full-time, according to the study.

Although no bacteria or fungi or viruses are found in patients' urine, many researchers believe it's possible that IC is caused by an infectious agent that hasn't yet been identified.

Researchers have also suggested it may be an autoimmune disorder of the bladder's connective tissue, in which the body's defense mechanisms against invading bacteria turn suddenly against healthy tissue. In some patients, special white blood cells called mast cells, which are associated with inflammation, are found within the bladder's mucous lining. Or, some scientists theorize that the disorder may be an allergic reaction, because many patients have a history of allergies.

Some women go into remission during pregnancy, while others get worse, suggesting that in some patients hormones may be involved. Complicating the picture, many women with IC also suffer from a variety of other conditions, such as irritable bowel syndrome, migraine headaches, fibromyalgia (chronic aching of the muscles, joints, and connective tissues), low back pain, and similar disorders.

One theory in favor at present holds that the inner lining of the bladder (the glycosaminoglycan or GAG layer) that protects the bladder wall from toxic effects of urine may be "leaky," allowing substances in the urine to penetrate the bladder wall and trigger IC symptoms. A California study found that 70 percent of IC patients they examined had a "leaky" bladder lining.

More likely, any or all of these factors may exist, leading many researchers to conclude that IC is a syndrome, or a collection of signs and symptoms, rather than a specific disease. Others, such as Whitmore, believe it's more than one disease and is different in every person.

Making a Diagnosis

Although there is no test that identifies IC, urologists rely on several criteria to make a diagnosis:

• Frequent and urgent urination, and pelvic or bladder pain, especially as the bladder is filling.
• Pinpoint hemorrhages that can often be seen on the bladder wall during cystoscopy (an examination of the bladder's interior with a long, lighted tube, performed under anesthesia). This is called nonulcerative IC, seen in about 95 percent of patients.
• Cracks, scars, and star-shaped sores called Hunner's ulcers that are found in the bladder wall in ulcerative IC. Bladder capacity is decreased because the usually elastic bladder walls become stiff and don't expand normally.

Although about 10 times more women than men get IC, it's possible that men have been underdiagnosed. "We haven't been real sensitive in screening our prostatitis patients, so maybe more men have IC than we think," says Whitmore.

Symptoms usually begin between 20 and 50 years of age, but the average age of onset is 40. Some cases have been diagnosed in children. About 450,000 people in the United States are believed to have IC, but true numbers are hard to come by, because many cases are either undiagnosed or misdiagnosed. Although occasionally more than one member of a family has IC, the disorder is not believed to have a genetic component.

Treating the Condition

There is no cure for IC. All doctors can do is try to relieve the symptoms, a challenging task, because they vary from person to person. People may have flare-ups and remissions, and different patients respond to different treatments. A particular type of therapy may work for a while and then lose its effectiveness. Sometimes, stress or a change of diet triggers symptoms. Occasionally, IC goes into remission spontaneously.

Paradoxically, the cystoscopy used to diagnose IC also seems to make some people feel better. To enable the doctor to look inside the bladder with the cystoscope, the bladder is filled with water. This bladder distention helps about 30 percent of patients, at least for the short term, probably because the bladder is stretched and capacity is increased. It's also possible that the procedure may interfere with the transmission of pain signals by nerves in the bladder. The fact that IC can only be diagnosed by cystoscopy under anesthesia explains why many cases are overlooked even by urologists.

In a similar procedure, Rimso-50 is instilled directly into the bladder by a catheter. The solution is retained in the bladder for about 15 minutes before being expelled by spontaneous voiding. This treatment is given every two weeks until maximum symptomatic relief is obtained, then repeated as needed.

For some patients, Rimso-50 treatments become less effective over time. About 50 percent of patients experience significant pain relief for an average of about 10 months. The drug works by penetrating the bladder wall to reduce inflammation and acts as a muscle relaxant by preventing muscle contractions that cause pain, frequency and urgency.

Disadvantages of Rimso-50 include a garlic-like odor on the skin and breath that may last up to 72 hours. Some patients may develop a chemical cystitis after use of the drug that goes away within one or two days. Patients taking Rimso-50 also require a blood test every six months to make sure the blood count and liver and kidney function are normal. Periodic ophthalmologic examinations are also recommended.

"You have to customize therapy for the person," says Whitmore, who advocates a number of untraditional therapies, many of which have not been reviewed by FDA for this purpose. They include acid-restricted diets, alkalization of urine, bladder holding and retraining (delaying voiding for increasingly longer intervals), biofeedback and electric stimulation, acupuncture, muscle relaxants, antidepressants, anti-inflammatories, antihistamines and analgesics, and an experimental bladder "wash" consisting of an anesthetic, an antibiotic, an anticoagulant, and hydrocortisone.

From 40 to 60 percent of IC patients may benefit from low doses of the tricyclic antidepressant amitriptyline (Elavil and others), according to Vicki Ratner, M.D., and colleagues in the Journal of Women's Health, Vol. 1, No. 1, 1992. Physicians prescribe it not only to treat the depression that is common in IC patients, but to take advantage of its bladder-relaxing, allergy-fighting, pain-blocking, and sedating properties.

When pain is severe, some people may benefit from transcutaneous electrical nerve stimulation (TENS). Mild electrical impulses delivered to the body through wires placed on the lower back or abdomen or through devices implanted in the body may alter nerve transmissions to the bladder and help trigger release of pain-blocking hormones.

A bland diet helps some IC people. Doctors recommend avoiding high-acid foods, such as citrus fruits, that may irritate the bladder, or spicy foods that may cause the release of histamine. Restricting alcoholic beverages, carbonated sodas, coffee and other caffeinated products, and beverages and foods with artificial sweeteners appears to reduce symptoms in some people.

Surgery is an option when all else fails. Some urologists may remove the diseased portion of the bladder and attach a piece of the patient's bowel to the remaining healthy tissue to make a larger bladder. In other cases, the bladder is completely removed and urine is rerouted to a bag outside the body or a pouch inside the abdomen. However, about half of patients don't get pain relief from this procedure.

"I don't take the bladder out unless I've used all the tricks up my sleeve," says Whitmore. "When patients have bladders the size of a walnut or smaller, or when they have intractable pain, then they're candidates for cystectomy [bladder removal]. The operation has allowed some people to get out of the house and have a life."

Whitmore tells her patients that, as with all disorders of chronic pain, there is going to be a certain amount of anger, anxiety and depression. "I say to them, 'I have an 85 percent chance or greater to make you better, but I can't teach you how to cope with your illness, so you've got to get some help.' I encourage them to go for self-hypnosis, self-relaxation, and other coping techniques, or to seek therapy with psychologists or psychiatrists. I tell them, 'if you can't cope, you're not going to get better.'"

Researchers funded by the federal government, drug companies, and the Interstitial Cystitis Association have stepped up their efforts to find out more about the disorder. Philip Hanno, M.D., chairman, Department of Urology, Temple University School of Medicine, Philadelphia, Pa., expects that in the next decade, treatment for IC will be more beneficial than the therapy available now. He believes that ultimately there will be a cure for most cases of this painful and disabling condition.

Evelyn Zamula is a freelance writer in Potomac, Md.

IC Symptoms

• urgent need for frequent urination both day and night

• reduced bladder capacity

• feelings of pressure, pain and tenderness around the bladder, pelvis and genital area, which may increase as the bladder fills and decrease as it empties.

• painful sexual intercourse

• in men, discomfort or pain in the prostatic area.

For More Info

Interstitial Cystitis Association
51 Monroe St., Suite 1402
Rockville, MD 20850
Telephone: 301-610-5300
Fax: 301-610-5308
Toll-free: 1-800-Help ICA
E-mail: ICAmail@ichelp.com
www.ichelp.com

American Foundation for Urologic Disease, Inc.
300 West Pratt St.
Suite 401
Baltimore, MD 21201
(1-800) 242-2383

FDA Consumer magazine (November 1995)
(Address for Interstitial Cystitis Association updated by clb 1999-APR-28)