pediatric Crohn’s disease

December 31, 2014

Synonyms

Overview

Crohn's disease is a type of inflammatory bowel disease (IBS), the general name for conditions that cause inflammation in the gastrointestinal (GI) tract. Common signs and symptoms include abdominal pain and cramping, diarrhea, and weight loss. Other general symptoms include feeling tired, nausea and loss of appetite, fever, and anemia. Complications of Crohn's disease may include intestinal blockage, fistulas and anal fissures, ulcers, malnutrition, and inflammation in other areas of the body. Crohn's disease can occur in people of all age groups but is most often diagnosed in young adults. The exact cause is unknown, but is thought to involve both genetic and environmental factors. It appears to run in some families. Treatment is aimed at relieving symptoms and reducing inflammation, but some people require surgery

Children with Crohn’s disease can face growth problems as a result of poor of nutrients during the digestive process. They may tend to be underweight or small-boned. They may also feel tired and unable to stay as active as their peers and friends.

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).

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

Causes

The exact cause of Crohn's disease remains unknown. Previously, diet and stress were suspected, but now doctors know that although these factors may aggravate existing Crohn's disease, they don't cause it. Now, researchers believe that a number of factors, such as heredity and a malfunctioning immune system, play a role in the development of Crohn's disease.

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, leading experts to suspect that one or more genes may make people more susceptible to Crohn's disease. However, most people with Crohn's disease don't have a family history of the disease.

Prevention

Best way to prevent the disease is by raising the awareness for 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.
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.

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.

Prognosis

Prognosis for Crohn's disease: Some people have long periods of remission, sometimes years, when they are free of symptoms. Good nutrition, exercise, effective treatment, and communication with your child and his or her doctor, teachers, and other family members can help limit the effects of the disease as well as help with symptom control.

Crohn's disease may lead to one or more of the following complications:

Inflammation. Inflammation may be confined to the bowel wall, which can lead to scarring and narrowing (stenosis), or may spread through the bowel wall (fistula).
Bowel obstruction. Crohn's disease affects the thickness of the intestinal wall. Over time, parts of the bowel can thicken and narrow, which may block the flow of digestive contents. You may require surgery to remove the diseased portion of your bowel.
Ulcers. Chronic inflammation can lead to open sores (ulcers) anywhere in your digestive tract, including your mouth and anus, and in the genital area (perineum).
Fistulas. Sometimes ulcers can extend completely through the intestinal wall, creating a fistula — an abnormal connection between different body parts. Fistulas can develop between your intestine and skin, or between your intestine and another organ. Fistulas near or around the anal area (perianal) are the most common kind.

When fistulas develop in the abdomen, food may bypass areas of the bowel that are necessary for absorption. Fistulas may occur between loops of bowel, into the bladder or vagina, or out through the skin, causing continuous drainage of bowel contents to your skin.

In some cases, a fistula may become infected and form an abscess, which can be life-threatening if not treated.
Anal fissure. This is a small tear in the tissue that lines the anus or in the skin around the anus where infections can occur. It's often associated with painful bowel movements and may lead to a perianal fistula.
Malnutrition. Diarrhea, abdominal pain and cramping may make it difficult for you to eat or for your intestine to absorb enough nutrients to keep you nourished. It's also common to develop anemia due to low iron or vitamin B-12 caused by the disease.
Colon cancer. Having Crohn's disease that affects your colon increases your risk of colon cancer. General colon cancer screening guidelines for people without Crohn's disease call for a colonoscopy every 10 years beginning at age 50. Ask your doctor whether you need to have this test done sooner and more frequently.
Other health problems. Crohn's disease can cause problems in other parts of the body. Among these problems are anemia, osteoporosis, and gallbladder or liver disease.
Medication risks. Certain Crohn's disease drugs that act by blocking functions of the immune system are associated with a small risk of developing cancers such as lymphoma and skin cancers. They also increase risk of infection.

Corticosteroids can be associated with a risk of osteoporosis, bone fractures, cataracts, glaucoma, diabetes and high blood pressure, among others. Work with your doctor to determine risks and benefits of medications.

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.

Approved therapies

Adalimumab (Humira) - FDA-approved indication: Treatment of Juvenile Idiopathic Arthritis. Reducing signs and symptoms and inducing and maintaining clinical remission in patients 6 years of age and older with moderately to severely active Crohn's disease who have had an inadequate response to corticosteroids or immunomodulators such as azathioprine, 6-mercaptopurine, or methotrexate.
Infliximab (Remicade) - FDA-approved indication: For reducing signs and symptoms and inducing and maintaining clinical remission in pediatric patients with moderately to severely active Crohn's disease who have had an inadequate response to conventional therapy

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.

Antobiotics

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.

Other medications
In addition to controlling inflammation, some medications may help relieve your signs and symptoms, but always talk to your doctor before taking any over-the-counter medications. Depending on the severity of your Crohn's disease, your doctor may recommend one or more of the following:

Anti-diarrheals. A fiber supplement, such as psyllium powder (Metamucil) or methylcellulose (Citrucel), can help relieve mild to moderate diarrhea by adding bulk to your stool. For more severe diarrhea, loperamide (Imodium) may be effective. Anti-diarrheals should only be used after discussion with your doctor.
Pain relievers. For mild pain, your doctor may recommend acetaminophen (Tylenol, others) — but not other common pain relievers, such as ibuprofen (Advil, Motrin IB, others), naproxen sodium (Aleve, Anaprox). These drugs are likely to make your symptoms worse, and can make your disease worse as well.
Iron supplements. If you have chronic intestinal bleeding, you may develop iron deficiency anemia and need to take iron supplements.
Vitamin B-12 shots. Crohn's disease can cause Vitamin B-12 deficiency. Vitamin B-12 helps prevent anemia, promotes normal growth and development, and is essential for proper nerve function.
Calcium and vitamin D supplements. Crohn's disease and steroids used to treat it can increase your risk of osteoporosis, so you may need to take a calcium supplement with added vitamin D.

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.

Resources

Refer to Research Publications.