What
is IC / PBS?
Interstitial
cystitis (IC) is a condition that results in recurring discomfort
or pain in the bladder and the surrounding pelvic region.
The symptoms vary from case to case and even in the same
individual. People may experience mild discomfort, pressure,
tenderness, or intense pain in the bladder and pelvic area.
Symptoms may include an urgent need to urinate (urgency),
a frequent need to urinate (frequency), or a combination
of these symptoms. Pain may change in intensity as the bladder
fills with urine or as it empties. Women's symptoms often
get worse during menstruation. They may sometimes experience
pain with vaginal intercourse.
Because
IC varies so much in symptoms and severity, most researchers
believe that it is not one, but several diseases. In recent
years, scientists have started to use the term painful bladder
syndrome (PBS) to describe cases with painful urinary symptoms
that may not meet the strictest definition of IC. The term
IC / PBS includes all cases of urinary pain that can't be
attributed to other causes, such as infection or urinary
stones. The term interstitial cystitis, or IC, is used alone
when describing cases that meet all of the IC criteria established
by the National Institute of Diabetes and Digestive and
Kidney Diseases (NIDDK).
In
IC / PBS, the bladder wall may be irritated and become scarred
or stiff. Glomerulations (pinpoint bleeding caused by recurrent
irritation) often appear on the bladder wall. Hunner's ulcers
are present in 10 percent of patients with IC. Some people
with IC / PBS find that their bladders cannot hold much
urine, which increases the frequency of urination. Frequency,
however, is not always specifically related to bladder size;
many people with severe frequency have normal bladder capacity.
People with severe cases of IC / PBS may urinate as many
as 60 times a day, including frequent nighttime urination
(nocturia).
IC
/ PBS is far more common in women than in men. Of the estimated
1 million Americans with IC, up to 90 percent are women.
What
causes IC?
Some
of the symptoms of IC / PBS resemble those of bacterial
infection, but medical tests reveal no organisms in the
urine of patients with IC / PBS. Furthermore, patients with
IC / PBS do not respond to antibiotic therapy. Researchers
are working to understand the causes of IC / PBS and to
find effective treatments.
In
recent years, researchers have isolated a substance found
almost exclusively in the urine of people with interstitial
cystitis. They have named the substance antiproliferative
factor, or APF, because it appears to block the normal growth
of the cells that line the inside wall of the bladder. Researchers
anticipate that learning more about APF will lead to a greater
understanding of the causes of IC and to possible treatments.
Researchers
are beginning to explore the possibility that heredity may
play a part in some forms of IC. In a few cases, IC has
affected a mother and a daughter or two sisters, but it
does not commonly run in families.
How
is IC / PBS diagnosed?
Because
symptoms are similar to those of other disorders of the
urinary bladder and because there is no definitive test
to identify IC / PBS, doctors must rule out other treatable
conditions before considering a diagnosis of IC / PBS. The
most common of these diseases in both genders are urinary
tract infections and bladder cancer. IC / PBS is not associated
with any increased risk in developing cancer. In men, common
diseases include chronic prostatitis or chronic pelvic pain
syndrome.
The
diagnosis of IC / PBS in the general population is based
on
-
presence
of pain related to the bladder, usually accompanied
by frequency and urgency
-
absence
of other diseases that could cause the symptoms
Diagnostic
tests that help in ruling out other diseases include urinal)
M.>. urine culture, cystoscopy, biopsy of the bladder
wall, distention of the bladder under anesthesia, urine
cytology, and laboratory examination of prostate secretions.
Urinalysis
and Urine Culture
Examining
urine under a microscope and culturing the urine can detect
and identify the primary organisms that are known to infect
the urinary tract and that may cause symptoms similar to
IC / PBS. A urine sample is obtained either by catheterization
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,
the doctor may consider a diagnosis of IC / PBS.
Culture
of Prostate Secretions
Although
not commonly done, in men, the doctor might obtain prostatic
fluid and examine it for signs of a prostate infection,
which can then be treated with antibiotics.
Cystoscopy
Under Anesthesia With Bladder Distention
The
doctor may perform a cystoscopic examination in order to
rule out bladder cancer. During cystoscopy, 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 might also distend or stretch the bladder to its
capacity by filling it with a liquid or gas. Because bladder
distention is painful in patients with IC / PBS, they must
be given some form of anesthesia for the procedure.
The doctor may also test the patient's maximum bladder capacity—the
maximum amount of liquid or gas the bladder can hold. This
procedure must be done under anesthesia since the bladder
capacity is limited by either pain or a severe urge to urinate.
Biopsy
A
biopsy is a tissue sample that can be examined under a microscope.
Samples of the bladder and urethra may be removed during
a cystoscopy. A biopsy helps rule out bladder cancer.
Future
Diagnostic Tools
Researchers
are investigating and validating some promising biomarkers
such as anti-proliferative factor (APF), some cytokines,
and other growth factors. These might provide more reliable
diagnostic markers for IC and lead to more focused treatment
for the disease.
Cytoscope
What
are the treatments for IC / PBS?
Scientists
have not yet found a cure for IC / PBS, 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,
they may return after days, weeks, months, or years. Scientists
do not know why.
Because
the causes of IC / PBS are unknown, current treatments are
aimed at relieving symptoms. Many people are helped for
variable periods by one or a combination of the treatments.
As researchers learn more about IC / PBS, the list of potential
treatments will change, so patients should discuss their
options with a doctor.
Bladder
Distention
Many
patients have noted an improvement in symptoms after a bladder
distention has been done to diagnose IC / PBS. In many cases,
the procedure is used as both a diagnostic test and initial
therapy.
Researchers
are not sure why distention helps, but some believe that
it may increase capacity and interfere with pain signals
transmitted by nerves in the bladder. Symptoms may temporarily
worsen 24 to 48 hours after distention, but should return
to predistention levels or improve within 2 to 4 weeks.
Bladder
Instillation
During
a bladder instillation, also called a bladder wash or bath,
the bladder is filled with a solution that is held for varying
periods of time, averaging 10 to 15 minutes, before being
emptied.
The
only drug approved by the U.S. Food and Drug Administration
(FDA) for bladder instillation is dimethyl sulfoxide (DMSO.
RIMSO-50). DMSO treatment involves guiding a narrow tube
called a catheter 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 who respond to DMSO
notice improvement 3 or 4 weeks after the first 6- to 8-week
cycle of treatments. Highly motivated patients who are willing
to catheterize themselves may, after consultation with their
doctor, be able to have DMSO treatments at home. Self-administration
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, it may reach tissue more effectively to
reduce inflammation and block pain. It may also prevent
muscle contractions that cause pain, frequency, and urgency.
A
bothersome but relatively insignificant side effect of DMSO
treatments is a garlic-like taste and odor on the breath
and skin that may last up to 72 hours after treatment. Long-term
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.
Oral
Drugs
Pentosan
polysulfate sodium (Elmiron)
This first oral drug developed for IC was approved by the
FDA in 1996. In clinical trials, the drug improved symptoms
in 30 percent of patients treated. Doctors do not know exactly
how it works, but one theory is that it may repair defects
that might have developed in the lining of the bladder.
The
FDA-recommended oral dosage of Elmiron is 100 mg, three
times a day. Patients may not feel relief from IC pain for
the first 2 to 4 months. A decrease in urinary frequency
may take up to 6 months. Patients are urged to continue
with therapy for at least 6 months to give the drug an adequate
chance to relieve symptoms.
Elmiron's
side effects are limited primarily to minor gastrointestinal
discomfort. A small minority of patients experienced some
hair loss, but hair grew back when they stopped taking the
drug. Researchers have found no negative interactions between
Elmiron and other medications.
Elmiron
may affect liver function, which should therefore be monitored
by the doctor.
Because
Elmiron has not been tested in pregnant women, the manufacturer
recommends that it not be used during pregnancy, except
in the most severe cases.
Other
oral medications
Aspirin and ibuprofen may be a first line of defense against
mild discomfort. Doctors may recommend other drugs to relieve
pain.
Some
patients have experienced improvement in their urinary symptoms
by taking tricyclic antidepressants (amitriptyline) or antihistamines.
Amitriptyline may help to reduce pain, increase bladder
capacity, and decrease frequency and nocturia. Some patients
may not be able to take it because it makes them too tired
during the day. In patients with severe pain, narcotic analgesics
such as acetaminophen (Tylenol) with codeine or longer acting
narcotics may be necessary.
All
drugs—even those sold over the counter—have
side effects. Patients should always consult a doctor before
using any drug for an extended amount of time.
Diet
There
is no scientific evidence linking diet to IC / PBS, but
many doctors and patients find that alcohol, tomatoes, spices,
chocolate, caffeinated and citrus beverages, and high-acid
foods may contribute to bladder irritation and inflammation.
Some patients also note that their symptoms worsen after
eating or drinking products containing artificial sweeteners.
Patients may try eliminating various items from their diet
and reintroducing them one at a time to determine which,
if any, affect their symptoms. However, maintaining a varied,
well balanced diet is important.
Smoking
Many
patients feel that smoking makes their symptoms worse. How
the by-products of tobacco that are excreted in the urine
affect IC / PBS is unknown. Smoking, however, is the major
known cause of bladder cancer. Therefore, one of the best
things smokers can do for their bladder and their overall
health is to quit.
Exercise
Many
patients feel that gentle stretching exercises help relieve
IC / PBS symptoms.
Bladder
Training
People
who have found adequate relief from pain may be able to
reduce frequency by using bladder training techniques. Methods
vary, but basically patients decide to void (empty their
bladder) at designated times and use relaxation techniques
and distractions to keep to the schedule. Gradually, patients
try to lengthen the time between scheduled voids. A diary
in which to record voiding times is usually helpful in keeping
track of progress.
Surgery
Surgery
should be considered only if all available treatments have
failed and the pain is disabling. Many approaches and techniques
are used, each of which has its own advantages and complications
that should be discussed with a surgeon. Your doctor may
recommend consulting another surgeon for a second opinion
before taking this step. Most doctors are reluctant to operate
because the outcome is unpredictable: Some people still
have symptoms after surgery.
People
considering surgery should discuss the potential risks and
benefits, side effects, and long- and short-term complications
with a surgeon and with their family, as well as with people
who have already had the procedure. Surgery requires anesthesia,
hospitalization, and weeks or months of recovery. As the
complexity of the procedure increases, so do the chances
for complications and for failure.
To
locate a surgeon experienced in performing specific procedures,
check with your doctor.
Two
procedures—fulguration and
resection of ulcers—can
be done with instruments inserted through the urethra. Fulguration
involves burning Hunner's ulcers with 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
are done under anesthesia and use special instruments inserted
into the bladder through a cystoscope. Laser surgery in
the urinary tract should be reserved for patients with Hunner's
ulcers and should be done only by doctors who have had special
training and have the expertise needed to perform the procedure.
Another
surgical treatment is augmentation,
which makes the bladder larger. In most of these procedures,
scarred, ulcerated, and inflamed sections of the patient's
bladder are removed, leaving only the base of the bladder
and healthy tissue. A piece of the patient's colon (large
intestine) is then removed, reshaped, and attached to what
remains of the bladder. After the incisions heal, the patient
may void less frequently. The effect on pain varies greatly;
IC / PBS can sometimes recur on the segment of colon used
to enlarge the bladder.
Even
in carefully selected patients—those with small, contracted
bladders—pain, frequency, and urgency may remain or
return after surgery, and patients may have additional problems
with infections in the new bladder and difficulty absorbing
nutrients from the shortened colon. Some patients are incontinent,
while others cannot void at all and must insert a catheter
into the urethra to empty the bladder.
A
surgical variation of TENS, called sacral nerve
root stimulation, involves permanent implantation
of electrodes and a unit emitting continuous electrical
pulses. Studies of this experimental procedure are now under
way.
Bladder removal, called a cystectomy, is another, very infrequently
used, surgical option. Once the bladder has been removed,
different methods can be used to reroute the urine. In most
cases, ureters are attached to a piece of colon that opens
onto the skin of the abdomen. This procedure is called a
urostomy and the opening is called a stoma. Urine empties
through the stoma into a bag outside the body.
Some
urologists are using a second 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
puts a catheter into the stoma and empties the pouch. Patients
with either type of urostomy must be very careful to keep
the area in and around the stoma clean to prevent infection.
Serious potential complications may include kidney infection
and small bowel obstruction.
A
third method to reroute urine involves making a new bladder
from a piece of the patient's colon and attaching it to
the urethra. After healing, the patient may be able to empty
the newly formed bladder by voiding at scheduled times or
by inserting a catheter into the urethra. Only a few surgeons
have the special training and expertise needed to perform
this procedure.
Even
after total bladder removal, some patients still experience
variable IC / PBS symptoms in the form of phantom pain.
Therefore, the decision to undergo a cys-tectomy should
be made only after testing all alternative methods and after
seriously considering the potential outcome.
Are
there any special concerns?
Cancer
There
is no evidence that IC / PBS increases the risk of bladder
cancer.
Pregnancy
Researchers
have little information about pregnancy and IC / PBS but
believe that the disorder does not affect fertility or the
health of the fetus. Some women find that their IC / PBS
goes into remission during pregnancy, while others experience
a worsening of their symptoms.
Coping
The
emotional support of family, friends, and other people with
IC / PBS is very important in helping patients cope. Studies
have found that patients who learn about the disorder and
become involved in their own care do better than patients
who do not. See the Interstitial Cystitis Association of
America's website under "Support Groups" to find
a group near you.
Hope
Through Research
Although
answers may seem slow in coming, researchers are working
to solve the painful riddle of IC / PBS. Some scientists
receive funds from the Federal Government to help support
their research, while others receive support from their
employing institution, drug pharmaceutical or device companies,
or patient support associations.
NIDDK's
investment in scientifically meritorious IC / PBS research
across the country has grown considerably since 1987. The
Institute now supports research that is looking at various
aspects of IC / PBS, such as how the components of urine
may injure the bladder and what role organisms identified
by nonstandard methods may have in causing IC / PBS. In
addition to funding research, NIDDK sponsors scientific
workshops where investigators share the results of their
studies and discuss future areas for investigation.
Clinical Research Network
The
Interstitial Cystitis Clinical Research Network (ICCRN)
is a product of two NIDDK programs: the Interstitial Cystitis
Database (ICDB) Study and the Interstitial Cystitis Clinical
Trials Group (ICCTG). Established in 1991, the ICDB was
a five-year prospective cohort study of more than 600 men
and women with symptoms of urinary urgency, frequency, and
pelvic pain. The study described the longitudinal changes
of urinary symptoms, the impact of IC on quality of life,
treatment patterns, and the relationship between bladder
biopsy findings and patient symptoms.
The
ICCTG was established in 1996 as a follow up to the ICDB
study. The clinical trials group developed two randomized,
controlled clinical trials of promising therapies, one using
oral therapies—pentosan polysulfate sodium (Elmiron)
and hydroxyzine hydrochloride (Atarax)— and the other
administering intravesical treatment using Bacillus Calmette-Guerin
(BCG). BCG is a vaccine for tuberculosis that stimulates
the immune system and may have an effect on the bladder.
The ICCTG also developed and conducted ancillary studies
of various biomarkers such as heparin-binding-growth-factor-like-growth-factor
(HB-EGF) and anti-proliferative factor (APF).
In
2003, the ICCTG became the Interstitial Cystitis Clinical
Research Network (ICCRN), which is conducting additional
clinical trials, either sequentially or concurrently, over
a second five-year period. Ancillary studies will be developed
and conducted in conjunction with the trials. One of these
trials is studying the effectiveness of amitriptyline (Elavil)
in treating painful bladder syndrome, which includes IC.
Amitriptyline has FDA approval for the treatment of depression,
but researchers believe the drug may work to block nerve
signals that trigger pain in the bladder and may also decrease
muscle spasms in the bladder, helping to cut both pain and
frequent urination. Participants in the trial will be randomly
assigned to take up to 75 milligrams of amitriptyline or
a placebo each day for 14 to 26 weeks.
Suggested
Reading
The
materials listed below may be found in medical libraries,
in many college and university libraries, through interlibrary
loan in most public libraries, and at bookstores. Items
are listed for information only; inclusion does not imply
endorsement by NIH.
Articles
and Book Chapters
Keay
SK, Warren JW. Is interstitial cystitis an infectious disease?
International Journal of Antimicrobial Agents, 2002, 19(6):480-3.
The
Interstitial Cystitis Clinical Trials Group. A randomized
controlled trial of intravesical bacillus Calmette-Guerin
for treatment of refractory interstitial cystitis. Journal
of Urology, 2005,173(4):1186-91.
The
Interstitial Cystitis Clinical Trials Group. A pilot clinical
trial of oral pentosan polysulfate and oral hydroxyzine
in patients with interstitial cystitis. Journal of Urology,
2003,170(3):810-15.
Books
and Booklets
Moldwin
RM. Interstitial cystitis survival guide: your guide to
the latest treatment options and coping strategies. Oakland,
CA: New Harbinger Publications, Inc.; 2000. (Available by
calling 1-800-HELP-ICA.)
Sandier
GG, Sandier A. Patient to patient: managing interstitial
cystitis and overlapping conditions. New Orleans, LA: Bon
Ange LLC; 2000.
Sant
G, ed. Interstitial cystitis. Philadelphia: Lippincott-Raven;
1997.
The
U.S. Government does not endorse or favor any specific commercial
product or company. Trade, proprietary, or company names
appearing in this document are used only because they are
considered necessary in the context of the information provided.
If a product is not mentioned, the omission does not mean
or imply that the product is unsatisfactory.
For
More Information
American
Foundation for Urologic Disease
1000 Corporate Boulevard, Suite 410
Linthicum, MD 21090
Phone: 1-800-828-7866 or 410-689-3990
Email: admin@ afud.org
Internet: www.afud.org
American
Pain Society
4700 West Lake Avenue Glenview, IL 60025
Phone: 847-375-4715
Email: info@ampainsoc.org
Internet: www.ampainsoc.org
American
Urogynecologic Society
2025 M Street NW, Suite 800 Washington, DC 20036
Phone: 202-367-1167
Fax: 202-367-2167
Email: augs@dc.sba.com
Internet: www.augs.org
International
Association for the Study of Pain
909 Northeast 43rd Street, Suite 306 Seattle, WA 98105-6020
Phone: 206-547-6409
Email: iaspdesk@juno.com
Internet: www.iasp-pain.org
Interstitial Cystitis Association of America
110 North Washington Street, Suite 340
Rockville, MD 20850
Phone: 1-800-HELP-ICA (435-7422) or
301-610-5300
Fax: 301-610-5308
Email: icamail@ichelp.org
Internet: www.ichelp.org
National
Chronic Pain Outreach Association
7979 Old Georgetown Road, Suite 100 Bethesda, MD 20814-2429
Phone: 301-652-4948 Fax: 301-907-0745
National
Kidney Foundation
30 East 33rd Street
New York, NY 10016
Phone: 1-800-622-9010 or 212-889-2210
Email: info@kidney.org
Internet: www.kidney.org
National
Organization of Social Security Claimants' Representatives
6 Prospect Street Midland Park, NJ 07432-1691
Phone: 1-800-431-2804
Email: webmaster@nosscr.org
Internet: www.nosscr.org
Social
Security Administration
Write or call your local office: look in the
telephone book under U.S. Government,
Department of Health and Human
Services or call 1-800-772-1213,
visit www.ssa.gov
on the Internet,
or write to
Social Security Administration
Office of Public Inquiries
Windsor Park Building
6401 Security Boulevard
Baltimore, MD 21235-6401
United
Ostomy Association
19772 MacArthur Boulevard, Suite 200
Irvine, CA 92612
Phone: 1-800-826-0826 or 949-660-8624
Fax: 949-660-9262
Email: info@uoa.org
Internet: www.uoa.org
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