Introduction
For many patients receiving a diagnosis of inflammatory bowel disease
(IBD) can be quite a shock. Often there is an initial feeling of relief
that the blood in their stool is not a sign of cancer; but this feeling
can be fleeting when patients realize they have a disease that will require
life-long management. Patients want to know what is going to happen to
them and what they can expect in the future if their disease progresses.
The development of a disease over a period of time is called the clinical
course. This newsletter will focus on the clinical course of IBD to help
patients and their families understand how their disease may affect them
over time.
Many people with IBD have questions about their bodies, their quality
of life, and their future. They may be concerned about their energy level,
the effects of medications, the nature of their disease, and whether they
will need surgery. Patients have spoken with me about feeling angry and
betrayed by their bodies. Sometimes they report feeling isolated and lonely
and worry about their ability to be intimate, perform sexually, or have
children. If you have had these feelings you are not alone. These are
normal concerns and many resources are available to help including doctors,
support groups, and foundations.
It’s important that you share these concerns with your physician.
Open communication is a major component of maintaining wellness and may
help avoid or reduce the severity of IBD-associated complications. Participating
in an IBD support group may be very helpful to you and your family members
as you work through these feelings. You can visit www.CCFA.org
(or other Web sites) to learn about different IBD groups. Many medical
centers where IBD is treated also provide support groups. Research has
shown that patients who understand their disease do a better job sticking
with the things they need to do to help maintain their wellness.
Volume 1 of this newsletter series, Staying on Your Medication
by Stephen B. Hanauer, MD, provides excellent information on things you
can do to help yourself and stay healthy.
The Clinical Course (Development) of IBD
Crohn’s disease (CD) and ulcerative colitis (UC) are collectively
known as IBD. The clinical course is different for patients suffering
from UC than those with CD. It also varies based on age of onset, sex,
disease location, frequency and severity of relapses, the number and severity
of complications, coexisting disease, and how well patients adhere to
their treatment plans and take care of themselves in general. Thus, it
is impossible to predict a precise clinical course for any given individual.
We can, however, discuss risk factors and the likelihood of complications
and treatment options for these complications. Most patients have a mild
or moderate clinical course. Patients can do a lot to take control of
their disease and reduce the possibility of it becoming severe.
Staying on Your Medication provides excellent information on
the importance of adhering to treatment plans and offers suggestions on
how to remain compliant with your medication.
UC is confined to the colon, also called the large intestine. Some physicians
do not believe that UC is a single disease, but rather a blend of several
conditions that all have inflammation spread through the colon and ulcers
in the colon wall as their common characteristics. About 46% of people
with UC have disease only in their rectum and sigmoid colon (the lower
parts of the colon), called proctosigmoiditis. Another 17% have colitis
confined to the left side of their colon, which does not extend beyond
the splenic flexure (the junction between the left colon and the transverse
colon), called left-sided or distal disease. And, almost 37% have colitis
of the entire colon, called pancolitis. Those who have pancolitis tend
to have the most complications.
Without treatment, approximately 70% of patients with UC experience a
relapse within one year. With treatment, this relapse rate can be reduced
to 21%. However, most UC patients will experience a relapse within 3 years
despite treatment. Those diagnosed at a young age experience more relapses
than those who are older when first diagnosed. In one study, patients
with left-sided disease and pancolitis generally relapsed within 2-3 years,
and patients with distal colitis experienced a relapse within 9-10 years.
Worsening of colitis or relapse is not caused by short-term stress; however,
the risk of worsening or relapse over a period of months or years can
be tripled by long-term perceived stress. The good news is that patients
who take their medicine regularly even when symptoms are not present experience
fewer relapses than those who do not. Thus taking medication even when
you do not have symptoms and trying to keep life stresses under control
are important long-term strategies for remaining in remission.
UC can be cured with surgery to remove the colon. Because this surgery
has other consequences, it is generally reserved for people whose disease
cannot be adequately treated with medicines or for those people with signs
of pre-cancer (dysplasia) in the colon. While surgery is rarely an initial
treatment strategy, approximately 20-25% of UC patients will have surgery
to remove their colon within 10 years of diagnosis. Patients with pancolitis
(nearly 37%) are more likely to need surgery than those with disease confined
to the left side. Only 10% of UC patients with disease limited to the
rectum will have surgery within 5 years of diagnosis.
For CD, the clinical course is different. Unlike UC, CD can affect the
entire intestinal tract and cannot be cured with surgery. Approximately
80% of CD patients have a high level of disease activity at the time of
diagnosis. Within two years after diagnosis, 58% of patients will have
a relapse. By 10 years after diagnosis, that percentage increases to 88%.
The good news is that treatment works to control symptoms and restore
people to their normal function. Only 10% of patients have continuously
active disease (always experiencing symptoms of CD) in the first 2 years
of treatment. This number declines to only 1% by 10 years. Over the first
5 years of treatment, approximately one-quarter of patients remain in
remission, over one-half have recurrent episodes with symptom-free periods,
and one-quarter have persistent active disease. Thus, while periodic relapses
are likely, only a small proportion of patients experience symptoms on
an ongoing basis. About 75% of patients with CD may require surgery to
handle complications of the disease at some point in their lives. Surgery
for IBD will be discussed in a future newsletter.
- A skin tag is a soft, painless extra
fold of skin near the anus. Frequently an external hemorrhoid lies beneath
the tag.
- Hemorrhoids are abnormally swollen veins in the rectum
and anus that when irritated cause surrounding membranes to swell, burn,
itch, and bleed.
- An anal fissure is a small tear or cut in the skin
lining the anus which can cause pain and/or bleeding.
- A stricture is a tight band of scar tissue that constricts
the anal opening, interfering with the ability to pass stool comfortably.
- A fistula is an abnormal connection, usually between
two organs, or leading from an internal organ to the body surface (between
the anus and skin surface — perianal fistula
of the rectum and of the vagina — rectovaginal fistula)
usually caused by a trapped pocket of pus called an abscess.
- Anorectal Cancer can be one of several
different types of cancer that afflict the anus and the rectum.
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Complications of IBD
People with IBD are at increased risk for certain conditions compared
with the general population. Most of these conditions are treatable and
are not life-threatening. These include perianal disease, colorectal cancer,
eye and skin diseases, several forms of arthritis, some blood conditions,
liver and bile duct diseases, and kidney disease. Like UC and CD, these
are conditions associated with malfunctions of the immune system.
Perianal Disease: Approximately a third of patients
with CD experience problems around the anus called perianal disease. Perianal
disease includes skin tags, hemorrhoids, anal fissures and ulcers, anorectal
strictures, perianal and rectovaginal fistulas, and anorectal cancer.
These diseases are not solely complications of IBD and occur in the general
population as well. CD patients whose disease is confined to the colon
are more likely to develop perianal disease than those whose disease is
located mainly in the small intestine. Treatment depends on the type and
extent of disease and can vary from no treatment to topical creams to
antibiotics to surgery, depending on the type of perianal disease present.
Patients with CD should discuss any anal or perianal discomfort with their
doctor.
Cancer: Patients with UC and patients whose CD affects
their colon have an increased risk of colon cancer. The overall risk is
about 18 times higher than for people who do not have IBD. Thus having
regular colonoscopy screening for cancer is imperative. Recent studies
have demonstrated that taking a 5-ASA-based medication as prescribed significantly
reduces the risk of colorectal cancer in patients with IBD. A future newsletter
will deal with IBD and cancer in greater detail.
Fertility and Pregnancy: Fertility in women with IBD
is related to how well the symptoms of their disease are controlled—the
better the symptoms are controlled with medications, the more likely women
will get pregnant and carry the baby to term without complications. Indeed
the most common reason for reduced fertility in women with CD is voluntary
avoidance of pregnancy. When women are in remission at the time of conception,
they are more likely to stay in remission during the entire pregnancy
and deliver a baby of normal weight. Many women are discouraged from continuing
medicines for their IBD prior to becoming pregnant or during their pregnancy,
although some medications used to treat IBD are not thought to cause birth
defects. It is important that women consult with a physician knowledgeable
in the therapies used for IBD when planning to become pregnant to discuss
the risks and benefits of continuing medication. Recent studies in women
with UC who have had surgery to remove the colon and create a pouch from
small intestine (ileal pouch anal anastomosis or J-pouch) suggest that
they may have a more difficult time getting pregnant. Again the risk of
surgery must be weighed against the difficulty in getting pregnant caused
by the underlying UC.
For men, most therapies to treat IBD are not associated with any problems
of fertility. The exception is sulfasalazine (a 5-ASA agent used to treat
ulcerative colitis), which reduces sperm counts. When sulfasalazine is
discontinued, sperm counts return to normal. It’s important to remember
that most men and women with UC raise normal, healthy children.
Menstrual and Sexual Issues: About 23-31% of women with
IBD experience menstrual difficulties. They experience greater bowel changes,
such as diarrhea, during menstrual periods than women without IBD, and
are more likely to experience nausea and vomiting, back pain, urinary
frequency, headache, and mood changes. These increased symptoms are usually
temporary and subside with the end of menstruation. A small percentage
of women experience pain and bowel incontinence during intercourse. Women
who have known hypercoagulability (too much blood clotting) or liver disease
associated with their IBD should discuss appropriate contraception with
their physicians because birth control pills are contraindicated for patients
with these conditions.
Irritable Bowel Syndrome (IBS): Episodes of IBD can
leave the intestinal tract disturbed even though the IBD itself may be
in remission. Approximately 33% of UC patients and 57% of CD patients
whose IBD is in remission experience symptoms of IBS, such as alternating
constipation and diarrhea, a sensation of incomplete bowel movement(s),
bloating, cramping, and abdominal pain. Patients who experience these
IBS symptoms should discuss them with their doctor as treatment options
are available.
Intestinal Obstruction: Patients with CD, especially
those with inflammation in the small intestine, may have pain with eating
due to swelling of the tissue and a narrowed passageway. Over the years,
inflammation can create a build up of fibrous scar tissue that progressively
narrows the intestinal canal through which food passes. Food and air back-up
can cause cramping, pain, nausea, and/or vomiting. This narrowing of the
canal, called a stricture, is a common characteristic of CD. Strictures
are usually treated with medications and diet, but some eventually require
surgery. Strictures occur less commonly in UC, and because UC patients
with strictures are at a higher risk for colorectal cancer, they may require
removal of part of the colon.
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People with IBD sometimes have to contend with extraintestinal complications
of IBD. These are conditions outside the intestinal tract that occur more
commonly in people with UC or CD than in the general population. It’s
important to remember that most people with IBD do not develop these diseases.
Eyes:
People with IBD are at higher risk for inflammation of different parts
of the eye. These conditions affect about 2% of women and 1% of men. Iritis
is inflammation of the iris (colored part of the eye). Patients have headaches,
eye pain, sensitivity to light, increased tearing, and less crisp vision.
Uveitis is inflammation of the uvea, which is a larger area that includes
the iris and surrounding structures (choriod and ciliary body). The area
in front of the lens fills with pus; the pupil opens abnormally; and the
cornea becomes inflamed. These conditions are treated with steroid eye
drops. Left untreated, scar tissue may develop and can cause vision loss.
Episcleritis is inflammation of the white of the eye. It causes burning,
itching, and tearing. It is also treated with steroid drops and is less
serious than iritis and uveitis.
Bones and Joints: About 25% of IBD patients experience
aches and pains of the bones and joints even without signs of arthritis
(joint swelling). Approximately 20% of IBD patients develop arthritis
in the knees, ankles, elbows, wrists, and shoulders, called peripheral
arthritis. Joints can be swollen, warm, stiff, and painful. Fortunately,
this condition is usually transitory and self-resolves in about 2 months,
although it can last longer. In general, the joint pain and arthritis
symptoms occur along with IBD activity. Nonsteroidal anti-inflammatory
drugs (NSAIDs), for example, Advil®, Motrin IB®,
Nuprin®, Aleve®, and various types of aspirin,
are commonly taken for the aches and pains of arthritis. However, people
with IBD should avoid these, as well as prescription NSAIDs, because they
may cause a flare of the bowel disease. As with any medication, you should
talk to your doctor about stopping or starting these drugs and discuss
alternative medications for pain that might be suitable for you. Be sure
to discuss any unusual side effects with your physician as well.
Lower bone density is common in patients with IBD, especially CD. The
causes are many but most importantly include corticosteroid use. Women
are more likely than men to have reduced bone mineral density regardless
of whether they take corticosteroids, and thus are at higher risk for
osteoporosis. A common strategy for treatment is to avoid corticosteroids
and participate in regular exercise, but you should talk to your doctor
about the best strategy for you. You may be advised to take calcium and
vitamin D supplements especially if you do not have regular sun exposure
or do not eat food products high in calcium. An assessment of bone density
may be a good choice for patients on corticosteroids.
Liver,
Bile Duct, and Pancreas: A serious liver condition is primary
sclerosing cholangitis (PSC). Approximately 4% of people with IBD develop
PSC, which occurs more commonly in UC than CD and more frequently in men
than in women. PSC can occur in CD patients whose disease is confined
to the colon. In PSC the bile ducts inside and outside the liver become
inflamed leading to a build up of scar tissue. The scar tissue blocks
the pathway for the elimination of bile and waste. The build up of scar
tissue in the liver can cause jaundice (yellow eyes and skin), weight
loss, nausea, and itching.
About 11% of CD patients and 7.5% of UC patients develop gallstones.
People over age 40 and women tend to have them more commonly than men.
The risk for gallstones in UC patients is not much higher than that for
the general population (5.5%). The presence of gallstones can be a very
painful condition if the bile duct becomes blocked. It is usually treated
by surgical removal of the gall bladder. People can live and function
normally without their gall bladders, but they may need to be more careful
with their diet.
Inflammation of the pancreas, called pancreatitis, can develop as a complication
of gallstones, as a side effect of medications used to suppress the immune
system, called immunosuppressants, or for no known reason. It is rare,
occurring in only about 1.5% of IBD patients, but is potentially a very
serious condition. Treatment depends on what is causing the inflammation.
Kidneys:
Kidney stones, also called nephrolithiasis, occur in 1-5% of IBD patients.
It is often asymptomatic, but can become extremely painful if the stone
passes into the ureter. It’s important that an acute episode of
kidney stones not be confused with a flare up of IBD. Kidney stones are
treated either surgically or with a procedure called extracorporeal shockwave
lithotripsy or lithotripsy for short. Lithotripsy is a noninvasive procedure
that sends sound waves through water to break up the stones.
Skin: Some IBD-associated skin problems are more common
in women, but others occur equally in women and men. Some are uncomfortable
and annoying like canker sores in the mouth or skin tags around the anus
but others may be more serious. It’s important to remember that
less than 10% of patients develop one of these complications.
Erythema nodosum are red- to reddish-purple-colored bumps that appear
most commonly on the lower legs but can also occur on the arms. They are
quite sore and can be hot and hard like a raised bruise. The size can
vary from as small as a pimple to as big as a cookie cutter. Up to 4%
of UC patients and up to 15% of CD patients get them. They usually occur
just before or during a flare up of IBD and disappear when the IBD is
treated.
Pyoderma gangrenosum, which is a pus-filled skin ulcer, occurs more frequently
with UC than CD and affects 1-5% of IBD patients. It should be reported
to a physician at the first sign. It often starts on the top of feet or
on the legs, but can occur anywhere on the body and can occur singly or
in groups. Early diagnosis and intervention is important. You should be
sure to let the doctor treating your IBD know about these symptoms. Physicians,
who do not recognize the ulcers or are not familiar with your IBD, may
not recognize the ulcers as a symptom of IBD. It is important to treat
the IBD even if you are not having typical symptoms of IBD.
Blood: Anemia (having less than the normal number of
red blood cells or less hemoglobin than normal in the blood) is a common
complication of both UC and CD. It is caused by intestinal bleeding or
in CD patients, who have severe ileal disease, by poor absorption of vitamin
B12. Anemia can be treated by eating foods high in iron, and
by taking iron and B12 supplements when prescribed by a physician.
Hypercoagulation, which means that blood clots form more readily than
in the general population, affects UC and CD patients equally (1.6% of
IBD patients). Blood clots typically, but not always, occur when the patient
is ill and bedridden. They usually occur in the legs and appear as tender
areas under the skin that are hot and hard to the touch. They are treated
with medicines designed to dissolve the blood clots, called anticoagulants.
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Summary
IBD is a chronic illness that has a wide variety of presentations and
severity. Volume 2 in this newsletter series, What Causes Inflammatory
Bowel Disease? by Daniel Present, MD, discusses current thinking
about the causes of IBD. Some people have just one attack in their lifetime,
while others may have it nearly all the time. Most people spend the majority
of their lives in remission with the disease breaking through only from
time to time.
The clinical course can vary widely. A significant majority of people
with IBD have a mild-to-moderate form of the disease that requires small
changes in lifestyle and the incorporation of daily medication into their
life routine. For a minority of patients, the disease course can be quite
severe, and they may ultimately need surgery to remove part or all of
their colon.
While most people with IBD do not develop significant complications,
some people do get IBD-related diseases that provide further challenges
in their lives. These conditions are usually easy to treat; however some
can be severe. If you have IBD, you cannot prevent the occurrence of these
conditions entirely. Thus active cooperation with your physicians in planning
treatment, adhering carefully to your individualized treatment plan, and
having regular follow-up visits with your physician to ensure that treatment
is achieving its objectives are essential ingredients of living well with
IBD. You are in charge! Your interest in taking care of yourself, learning
about your disease, and maintaining a positive outlook will help you stay
well.
The next newsletter in this series by Asher Kornbluth, MD, will discuss
the treatment options for inflammatory bowel disease. To sign up for future
newsletters click here.
This information has been reviewed and approved by CCFA's National Scientific Advisory Committee.
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