WHAT IS CROHN'S DISEASE?

Crohn's disease is an inflammatory bowel disease (IBD). It causes inflammation of the lining of your digestive tract, which can lead to abdominal pain, severe diarrhea, fatigue, weight loss and malnutrition. Inflammation caused by Crohn's disease can involve different areas of the digestive tract in different people.

The inflammation caused by Crohn's disease often spreads deep into the layers of affected bowel tissue.

Crohn's disease can be both painful and debilitating, and sometimes may lead to life-threatening complications.

While there's no known cure for Crohn's disease, therapies can greatly reduce its signs and symptoms and even bring about long-term remission. With treatment, many people with Crohn's disease are able to function well.

WHAT ARE ITS SIGNS AND SYMPTOMS?

In some people with Crohn's disease, only the last segment of the small intestine (ileum) is affected. In others, the disease is confined to the colon (part of the large intestine). The most common areas affected by Crohn's disease are the last part of the small intestine and the colon.

Signs and symptoms of Crohn's disease can range from mild to severe. They usually develop gradually, but sometimes will come on suddenly, without warning. You may also have periods of time when you have no signs or symptoms (remission).

When the disease is active, signs and symptoms may include:

▶Diarrhea. Diarrhea is a common problem for people with Crohn's disease. Intensified intestinal cramping also can contribute to loose stools.
▶Fever and fatigue. Many people with Crohn's disease experience a low-grade fever, likely due to inflammation or infection. You may also feel tired or have low energy.
▶Abdominal pain and cramping. Inflammation and ulceration can affect the normal movement of contents through your digestive tract and may lead to pain and cramping. You may experience anything from slight discomfort to severe pain, including nausea and vomiting.
▶Blood in your stool. You might notice bright red blood in the toilet bowl or darker blood mixed with your stool. You can also have bleeding you don't see (occult blood).

▶Mouth sores. You may have ulcers in your mouth similar to canker sores.
▶Reduced appetite and weight loss. ▶Abdominal pain and cramping and the inflammatory reaction in the wall of your bowel can affect both your appetite and your ability to digest and absorb food.
▶Perianal disease. You might have pain or drainage near or around the anus due to inflammation from a tunnel into the skin (fistula).

WHAT ARE THE OTHER SIGNS AND SYMPTOMS?

People with severe Crohn's disease may also experience:
▶Inflammation of skin, eyes and joints
▶Inflammation of the liver or bile ducts
▶Delayed growth or sexual development, in children

WHAT CAUSES IT?

The exact cause of Crohn's disease remains unknown. Previously, diet and stress were suspected, but now doctors know that these factors may aggravate but don't cause Crohn's disease. A number of factors, such as heredity and a malfunctioning immune system, likely play a role in its development.

▶Immune system. It's possible that a virus or bacterium may trigger Crohn's disease. When your immune system tries to fight off the invading microorganism, an abnormal immune response causes the immune system to attack the cells in the digestive tract, too.

▶Heredity. Crohn's is more common in people who have family members with the disease, so genes may play a role in making people more susceptible. However, most people with Crohn's disease don't have a family history of the disease.

WHAT ARE THE RISK FACTORS?

Risk factors for Crohn's disease may include:
▶Age. Crohn's disease can occur at any age, but you're likely to develop the condition when you're young. Most people who develop Crohn's disease are diagnosed before they're 30 years old.
▶Ethnicity. Although Crohn's disease can affect any ethnic group, whites and people of Eastern European (Ashkenazi) Jewish descent have the highest risk.
▶Family history. You're at higher risk if you have a close relative, such as a parent, sibling or child, with the disease. As many as 1 in 5 people with Crohn's disease has a family member with the disease.
▶Cigarette smoking. Cigarette smoking is the most important controllable risk factor for developing Crohn's disease.

Smoking also leads to more severe disease and a greater risk of having surgery. If you smoke, it's important to stop.
▶Nonsteroidal anti-inflammatory medications. These include ibuprofen (Advil, Motrin IB, others), naproxen sodium (Aleve, Anaprox), diclofenac sodium (Voltaren, Solaraze) and others. While they do not cause Crohn's disease, they can lead to inflammation of the bowel that makes Crohn's disease worse.
▶Where you live. If you live in an urban area or in an industrialized country, you're more likely to develop Crohn's disease. This suggests that environmental factors, including a diet high in fat or refined foods, play a role in Crohn's disease. People living in northern climates also seem to be at greater risk.


WHO CAN BE AFFECTED?

Crohn's disease is a relatively uncommon condition. There are currently at least 115,000 people living with the condition in the UK.

Crohn's disease can affect people of all ages, including children. However, most cases first develop between the ages of 16 and 30.

A large number of cases also develop between the ages of 60 and 80.

It affects slightly more women than men, but in children more boys are affected than girls.

The condition is more common in white people than in black or Asian people. It's most prevalent among Jewish people of European descent.


PREGNANCY AND CD

Crohn's disease is most often diagnosed between the ages of 15 and 25. This period also happens to be the peak of a woman's fertility.

If you're of childbearing age and have Crohn's, you may wonder if pregnancy is a viable option. The good news is that women with Crohn's are just as likely to get pregnant as those without Crohn's.

That said, scarring from pelvic surgery can inhibit fertility. This is especially true in cases of extreme procedures such as a total colectomy, a surgical removal of the large intestine.

Having Crohn's disease increases your chance for complications such as miscarriage and preterm delivery.

Assemble your team of specialists: a gastroenterologist, an obstetrician, and a general practitioner. Your medical team will track your progress as a high-risk obstetrics patient.

Your drug regimen to treat Crohn's warrants special attention if you want to conceive. Your obstetrician may recommend stopping Crohn's medications for the health of the fetus.

However, changing your drug regimen may affect your disease symptoms. Your gastroenterologist may advise you to continue a successful drug regimen.
You have to consider your health as well as your baby's. Work with both your gastroenterologist and your obstetrician before conception to construct the best plan to manage the disease during your pregnancy.


WHEN TO SEE A DOCTOR?

See your doctor if you have persistent changes in your bowel habits or if you have any of the signs and symptoms of Crohn's disease, such as:
▶Abdominal pain
▶Blood in your stool
▶Ongoing bouts of diarrhea that don't respond to over-the-counter (OTC) medications
▶Unexplained fever lasting more than a day or two
▶Unexplained weight loss


PREPARING FOR YOUR APPOINTMENT

Symptoms of Crohn's disease may first prompt you to visit your family doctor or general practitioner. Your doctor may recommend you see a specialist who treats digestive diseases (gastroenterologist).

Because appointments can be brief, and there's often a lot of information to discuss, it's a good idea to be well-prepared. Here's some information to help you get ready, and what to expect from your doctor.

▶▶WHAT CAN YOU DO?

Be aware of any pre-appointment restrictions. At the time you make the appointment, be sure to ask if there's anything you need to do in advance, such as restrict your diet.

▶Write down any symptoms you're experiencing, including any that may seem unrelated to the reason for which you scheduled the appointment.
▶Write down key personal information, including any major stresses or recent life changes.
▶Make a list of all medications, vitamins or supplements that you're taking.
▶Ask a family member or friend to come with you to your appointment. Sometimes it can be difficult to take in all the information provided during an appointment. Someone who accompanies you may remember something that you missed or forgot.
▶Write down questions to ask your doctor.

Your time with your doctor is limited, so preparing a list of questions can help you make the most of your visit. List your questions from most important to least important in case time runs out.

For Crohn's disease, some basic questions to ask your doctor include:

▶What's causing these symptoms?
▶Are there other possible causes for my symptoms?
▶What kinds of tests do I need? Do these tests require any special preparation?
▶Is this condition temporary or long lasting?
▶What treatments are available, and which do you recommend?
▶Are there any medications that I should avoid?
▶What types of side effects can I expect from treatment?
▶Are there any alternatives to the primary approach that you're suggesting?
▶I have other health conditions. How can I best manage them together?
▶Do I need to follow any dietary restrictions?
▶Is there a generic alternative to the medicine you're prescribing me?
▶Are there any brochures or other printed material that I can take with me? ▶What websites do you recommend?
▶If I have Crohn's disease, what is the risk that my child will develop it?
▶What kind of follow-up testing do I need in the future?

In addition to the questions that you've prepared to ask your doctor, don't hesitate to ask additional questions during your appointment.


▶▶WHAT TO EXPECT FROM YOUR DOCTOR?

Your doctor is likely to ask you a number of questions. Being ready to answer them may reserve time to go over points you want to spend more time on. Your doctor may ask:

▶When did you first begin experiencing symptoms?
▶Have your symptoms been continuous or off and on?
▶How severe are your symptoms?
▶Do your symptoms affect your ability to work or do other activities?
▶Does anything seem to improve your symptoms?
▶Is there anything that you've noticed that makes your symptoms worse?
▶Do you smoke?
▶Do you take nonsteroidal anti-inflammatory drugs (NSAIDs) — for example, ibuprofen (Advil, Motrin IB, others), naproxen sodium (Aleve, Anaprox), or diclofenac sodium (Voltaren, Solaraze)?


WHAT ARE THE TESTS AND DIAGNOSIS?

Your doctor will likely diagnose Crohn's disease only after ruling out other possible causes for your signs and symptoms. There is no one test to diagnose Crohn's disease.

Your doctor will likely use a combination of endoscopy with biopsies and radiological testing to help confirm a diagnosis of Crohn's disease. You may have one or more of the following tests and procedures:

▶▶BLOOD TESTS

▶Tests for anemia or infection. Your doctor may suggest blood tests to check for anemia — a condition in which there aren't enough red blood cells to carry adequate oxygen to your tissues — or to check for signs of infection. Expert guidelines do not currently recommend antibody or genetic testing for Crohn's disease.

▶Fecal occult blood test. You may need to provide a stool sample so that your doctor can test for hidden blood in your stool.

▶▶PROCEDURES

▶Colonoscopy. This test allows your doctor to view your entire colon using a thin, flexible, lighted tube with an attached camera.

During the procedure, your doctor can also take small samples of tissue (biopsy) for laboratory analysis, which may help confirm a diagnosis. Clusters of inflammatory cells called granulomas, if present, help confirm the diagnosis of Crohn's.
▶Flexible sigmoidoscopy. In this procedure, your doctor uses a slender, flexible, lighted tube to examine the sigmoid, the last section of your colon.
▶Computerized tomography (CT). You may have a CT scan — a special X-ray technique that provides more detail than a standard X-ray does. This test looks at the entire bowel as well as at tissues outside the bowel. CT enterography is a special CT scan that provides better images of the small bowel. This test has replaced barium X-rays in many medical centers.
▶Magnetic resonance imaging (MRI). An MRI scanner uses a magnetic field and radio waves to create detailed images of organs and tissues. MRI is particularly useful for evaluating a fistula around the anal area (pelvic MRI) or the small intestine (MR enterography).
▶Capsule endoscopy. For this test, you swallow a capsule that has a camera in it. The camera takes pictures, which are transmitted to a computer you wear on your belt. The images are then downloaded, displayed on a monitor and checked for signs of Crohn's disease.

The camera exits your body painlessly in your stool. You may still need endoscopy with biopsy to confirm the diagnosis of Crohn's disease.
▶Double-balloon endoscopy. For this test, a longer scope is used to look further into the small bowel where standard endoscopes don't reach. This technique is useful when capsule endoscopy shows abnormalities, but the diagnosis is still in question.
▶Small bowel imaging. This test looks at the part of the small bowel that can't be seen by colonoscopy. After you drink a liquid containing barium, doctors take X-ray, CT or MRI images of your small intestine.


WHAT ARE THE DRUGS AND TREATMENT?

Treatment for Crohn's disease usually involves drug therapy or, in certain cases, surgery. There is currently no cure for the disease, and there is no one treatment that works for everyone. Doctors use one of two approaches to treatment — either "step-up," which starts with milder drugs first, or "top-down," which gives people stronger drugs earlier in the treatment process.

The goal of medical treatment is to reduce the inflammation that triggers your signs and symptoms. It is also to improve long-term prognosis by limiting complications. In the best cases, this may lead not only to symptom relief but also to long-term remission.

▶▶ANTI-INFLAMMATORY DRUGS

Anti-inflammatory drugs are often the first step in the treatment of inflammatory bowel disease. They include:
▶Oral 5-aminosalicylates. These drugs may be helpful if Crohn's disease affects your colon, but they aren't helpful treating disease in the small intestine. They include sulfasalazine (Azulfidine), which contains sulfa, and mesalamine (Asacol, Delzicol, Pentasa, Lialda, Apriso). These drugs, especially sulfasalazine, have a number of side effects, including nausea, diarrhea, vomiting, heartburn and headache. These drugs have been widely used in the past but now are generally considered of limited benefit.

▶Corticosteroids. Corticosteroids such as prednisone can help reduce inflammation anywhere in your body, but they have numerous side effects, including a puffy face, excessive facial hair, night sweats, insomnia and hyperactivity. More-serious side effects include high blood pressure, diabetes, osteoporosis, bone fractures, cataracts, glaucoma and increased chance of infection.

Also, corticosteroids don't work for everyone with Crohn's disease. Doctors generally use them only if you don't respond to other treatments. A newer type of corticosteroid, budesonide (Entocort EC), works faster than do traditional steroids and appears to produce fewer side effects. However, it is only effective for Crohn's disease that's in certain parts of the bowel.

Corticosteroids aren't for long-term use. But they can be used for short-term (three to four months) symptom improvement and to induce remission. Corticosteroids may also be used with an immune system suppressor — the corticosteroids can induce remission, while the immune system suppressors can help maintain it.

▶▶IMMUNE SYSTEM SUPPRESSORS

These drugs also reduce inflammation, but they target your immune system, which produces the substances that cause inflammation. For some people, a combination of these drugs works better than one drug alone.

Immunosuppressant drugs include:
▶Azathioprine (Imuran) and mercaptopurine (Purinethol). These are the most widely used immunosuppressants for treatment of inflammatory bowel disease. Taking them requires that you follow up closely with your doctor and have your blood checked regularly to look for side effects, such as a lowered resistance to infection.

Short term, they also can be associated with inflammation of the liver or pancreas and bone marrow suppression. Long term, although rarely, they are associated with certain infections and cancers including lymphoma and skin cancer. They may also cause nausea and vomiting. Your doctor will use a blood test to determine whether you can take these medications.

▶Infliximab (Remicade), adalimumab (Humira) and certolizumab pegol (Cimzia). These drugs, called TNF inhibitors or "biologics," work by neutralizing an immune system protein known as tumor necrosis factor (TNF). They are used for adults and children with moderate to severe Crohn's disease to reduce signs and symptoms. They also may induce remission. Researchers continue to study these drugs to compare their benefits.
TNF inhibitors may be used soon after diagnosis, particularly if your doctor suspects that you're likely to have more severe Crohn's disease or if you have a fistula. Sometimes they are used after other drugs have failed. They also may be combined with an immunosuppressant in some people, but this practice is somewhat controversial.

People with certain conditions can't take TNF inhibitors. Tuberculosis and other serious infections have been associated with the use of immune-suppressing drugs. Talk to your doctor about your potential risks and have a skin test for tuberculosis, a chest X-ray and a test for hepatitis B before starting these medications. They are also associated with certain cancers, including lymphoma and skin cancers.

▶Methotrexate (Rheumatrex). This drug, which is used to treat cancer, psoriasis and rheumatoid arthritis, is sometimes used for people with Crohn's disease who don't respond well to other medications.

Short-term side effects include nausea, fatigue and diarrhea, and rarely, it can cause potentially life-threatening pneumonia. Long-term use can lead to bone marrow suppression, scarring of the liver and sometimes to cancer. You will need to be followed closely for side effects.

▶Cyclosporine (Gengraf, Neoral, Sandimmune) and tacrolimus (Astagraf XL, Hecoria). These potent drugs, often used to help heal Crohn's-related fistulas, are normally reserved for people who haven't responded well to other medications. Cyclosporine has the potential for serious side effects, such as kidney and liver damage, seizures, and fatal infections. These medications aren't for long-term use.

▶Natalizumab (Tysabri) and vedolizumab (Entyvio). These drugs work by stopping certain immune cell molecules — integrins — from binding to other cells in your intestinal lining. Natalizumab is approved for people with moderate to severe Crohn's disease with evidence of inflammation who aren't responding well to any other medications.

Because the drug is associated with a rare but serious risk of progressive multifocal leukoencephalopathy — a brain disease that usually leads to death or severe disability — you must be enrolled in a special restricted distribution program to use it.

Vedolizumab recently was approved for Crohn's disease. It works like natalizumab but appears not to carry a risk of brain disease.

▶Ustekinumab (Stelara). This drug is used to treat psoriasis. Studies have shown it's useful in treating Crohn's disease as well and may be used when other medical treatments fail.


▶▶ANTIBIOTICS

Antibiotics can reduce the amount of drainage and sometimes heal fistulas and abscesses in people with Crohn's disease. Some researchers also think antibiotics help reduce harmful intestinal bacteria that may play a role in activating the intestinal immune system, leading to inflammation.

Antibiotics may be used in addition to other medications or when infection is a concern, such as with perianal Crohn's disease. However, there's no strong evidence that antibiotics are effective for Crohn's disease. Frequently prescribed antibiotics include:

▶Metronidazole (Flagyl). At one time, metronidazole was the most commonly used antibiotic for Crohn's disease. However, it can cause serious side effects, including numbness and tingling in your hands and feet and, occasionally, muscle pain or weakness. If these effects occur, stop the medication and call your doctor.
▶Ciprofloxacin (Cipro). This drug, which improves symptoms in some people with Crohn's disease, is now generally preferred to metronidazole. A rare side effect is tendon rupture, which is an increased risk if you're also taking corticosteroids.


▶▶NUTRITION THERAPY

Your doctor may recommend a special diet given via a feeding tube (enteral nutrition) or nutrients injected into a vein (parenteral nutrition) to treat your Crohn's disease. This can improve your overall nutrition and allow the bowel to rest. Bowel rest can reduce inflammation in the short term.

Your doctor may use nutrition therapy short term and combine it with medications, such as immune system suppressors. Enteral and parenteral nutrition are typically used to get people healthier prior to surgery or when other medications fail to control symptoms.

Your doctor may also recommend a low residue or low-fiber diet to reduce the risk of intestinal blockage if you have a narrowed bowel (stricture). A low residue diet is designed to reduce the size and number of your stools.


▶▶SURGERY

If diet and lifestyle changes, drug therapy or other treatments don't relieve your signs and symptoms, your doctor may recommend surgery. Up to one-half of individuals with Crohn's disease will require at least one surgery. However, surgery does not cure Crohn's disease.

During surgery, your surgeon removes a damaged portion of your digestive tract and then reconnects the healthy sections. Surgery may also be used to close fistulas and drain abscesses. A common procedure for Crohn's disease is strictureplasty, which widens a segment of the intestine that has become too narrow.

The benefits of surgery for Crohn's disease are usually temporary. The disease often recurs, frequently near the reconnected tissue. The best approach is to follow surgery with medication to minimize the risk of recurrence.


LIFESTYLE AND HOME REMEDIES

Sometimes you may feel helpless when facing Crohn's disease. But changes in your diet and lifestyle may help control your symptoms and lengthen the time between flare-ups.

▶▶FOODS TO AVOID

▶Limit dairy products. Many people with inflammatory bowel disease find that problems such as diarrhea, abdominal pain and gas, improve by limiting or eliminating dairy products. You may be lactose intolerant — that is, your body can't digest the milk sugar (lactose) in dairy foods. Using an enzyme product such as Lactaid may help as well.
▶Try low-fat foods. If you have Crohn's disease of the small intestine, you may not be able to digest or absorb fat normally. Instead, fat passes through your intestine, making your diarrhea worse. Try avoiding butter, margarine, cream sauces and fried foods.
▶Limit fiber, if it's a problem food. If you have inflammatory bowel disease, high-fiber foods, such as fresh fruits and vegetables and whole grains, may make your symptoms worse. If raw fruits and vegetables bother you, try steaming, baking or stewing them.
In general, you may have more problems with foods in the cabbage family, such as broccoli and cauliflower, and nuts, seeds, corn and popcorn. You may be told to limit fiber or go on a low residue diet if you have a narrowing of your bowel (stricture).
▶Avoid other problem foods. Spicy foods, alcohol, and caffeine may make your signs and symptoms worse.


▶▶OTHER DIETARY MEASURES

▶Eat small meals. You may find you feel better eating five or six small meals a day rather than two or three larger ones.
▶Drink plenty of liquids. Try to drink plenty of fluids daily. Water is best.

▶Alcohol and beverages that contain caffeine stimulate your intestines and can make diarrhea worse, while carbonated drinks frequently produce gas.
▶Consider multivitamins. Because Crohn's disease can interfere with your ability to absorb nutrients and because your diet may be limited, multivitamin and mineral supplements are often helpful. Check with your doctor before taking any vitamins or supplements.
▶Talk to a dietitian. If you begin to lose weight or your diet has become very limited, talk to a registered dietitian.


▶▶SMOKING

Smoking increases your risk of developing Crohn's disease, and once you have it, smoking can make it worse. People with Crohn's disease who smoke are more likely to have relapses and need medications and repeat surgeries.

Quitting smoking can improve the overall health of your digestive tract, as well as provide many other health benefits.

HOW CLOSE ARE WE TO THE CURE?

At this time, there is no cure for Crohn's disease, though the disease can be successfully managed. Researchers do have suspects as to the culprit of Crohn's, however.

Genetic and environmental factors are both believed to play a role in causing the disease. At least two of the 46 human chromosomes—chromosome 5 and chromosome 10—have been associated with Crohn's.

Even the possibility of an indescribable bacteria or virus as the cause or a contributor of Crohn's is being explored. The cause could involve one factor, or many factors in combination.

Until the cure is found, there are steps that someone can take to minimize the disease's impact on his or her life.

Identifying these subsets within the broader Crohn's population is one of the foundation's research goals, he said.
"Certainly within the next ten years, and I'm hoping in the next five years, we'll be able to identify subsets," Dr. Sartor reported.