Ulcerative colitis is a form of inflammatory bowel disease (IBD).  It is characterized by varying degrees of active and chronic inflammation of the colon and rectum. The usual symptoms include diarrhea, rectal bleeding, and abdominal pain. Nobody knows exactly what causes ulcerative colitis however, activation of the immune system, exposure to bacteria in the gut and genetic factors seem to be involved.  Ulcerative colitis is treated with medicines such as corticosteroids, other anti-inflammatory agents, and immunomodulators.  It has been seen in children less than 2 years of age rarely, however it is much more commonly diagnosed in teenagers and adults.  Removal of the colon prevents the long-term risk of colon cancer and cures ulcerative colitis.  Once the colon is removed however, kids may have frequent stooling and inflammation of the newly formed rectum-like structure called a "pouch."  Some children will require an ostomy, which diverts the intestines to the outside of the body where stool can be collected in a bag.

What is ulcerative colitis?

Ulcerative colitis is a disease with an unknown cause that leads to inflammation (ulcers, redness, swelling, pain) in the colon and rectum. When the inflammation is severe, children can become very ill. 

What causes ulcerative colitis?

Nobody knows for sure, but it seems to be a combination of environmental factors and genes.  Over-reaction of the immune system to bacteria in the colon seems to play a role. 

How common is ulcerative colitis?

Ulcerative colitis affects about 2 out of 100,000 people.  About 30% of cases of inflammatory bowel disease are diagnosed in children.  It is much more common in children who have family members with inflammatory bowel disease.

What are the symptoms of ulcerative colitis?

For some children, the symptoms can be subtle and the diagnosis is not made for years.  Diarrhea and abdominal pain that persists for weeks or months are the most common complaints. Often, poor weight gain, anemia, decreased energy level or blood in the stool can be the first sign.

• Abdominal pain
• Diarrhea (frequently with blood and mucus)
• Poor weight gain
• Decreased energy level
• Delayed puberty

Red Flags (seek medical care immediately)

Really, any of the above symptoms should be evaluated by a physician as soon as possible.

How is ulcerative colitis diagnosed?

There is no single test for ulcerative colitis.  Often the diagnosis is not made until symptoms have persisted for months or years.  The combination of the patient's history, blood tests, stool studies, imaging studies and endoscopy can confirm the diagnosis.

• Blood tests - Anemia (or low red blood cell count) is common in ulcerative colitis.  This is due to loss of blood in the gut and poor production of red cells due to the overall illness.  Markers of inflammation (such as ESR and CRP) are often elevated.  Severe diarrhea may cause electolyte abnormalities and blood protein may be low due to poor nutritional state and loss of protein in the stool.
• Stool studies - Several infections can cause bloody diarrhea that may be confused with ulcerative colitis.  These include salmonella, shigella, campylobacter, E. coli, Clostridium difficile, giardia, entamoeba histolytica.  Tests should be done to rule out these infections.

Imaging studies -

• CT scans are fairly good for seeing bowel wall thickening due to inflammation and collections of fluid or pus in the abdomen.  This amount of radiation should be avoided in children if possible. 
• MRI is also a decent study for looking at the intestines and colon.  MRI takes about 45 minutes or longer, so little children may have difficulty staying still for this long.  Sedation is sometimes used. 
• X-ray studies with swallowed contrast are fairly good for evaluating the intestines for narrowing or large ulcers due to inflammation.  If small intestinal disease is found, the diagnosis of Chron's disease should be considered.
• Endoscopy, including upper gastrointestinal endoscopy and colonoscopy are the best test for confirming the diagnosis.  Inflammation can be seen with the endoscope camera.  Biopsy samples can be taken for a Pathologist to review under a microscope.  These procedures require sedation or anesthesia and are usually done by a Pediatric Gastroenterologist.
• Capsule endoscopy is a large pill-like camera that is swallowed.  It has a pulsating light and camera that sends pictures to a device worn on the belt.  Usually only kids over 8 years old can swallow this pill, but in younger children, the pill can be placed in the stomach during upper gastrointestinal endoscopy.  Once the camera has traveled through the gut and has taken thousands of pictures, it is pooped out and discarded.  The pictures are downloaded and reviewed by a Gastroenterologist.

How is ulcerative colitis treated?

5-ASA derivatives -

Medicines like mesalamine (Asacol, Pentasa, Lialda) and sulfasalazine (Azulfadine) are anti-inflammatory medicines similar to aspirin.  They are relatively benign medicines and are the main treatment for ulcerative colitis.

Immunomodulators and immunosuppressants-

• Mercaptopurine (6-MP) or azathioprine (Imuran) are chemotherapy agents that are used in relatively low doses to "cool down" the immune system.  These medicines are the standard therapy for ulcerative colitis.  Blood counts and liver function tests must be watched closely for unwanted side effects.  Cyclosporine is sometimes used.
• Glucocorticoids ("steroids") like prednisone and prednisolone are effective treatments for ulcerative colitis and have a rapid onset.  They are commonly used at the beginning of the disease until other medicines like the 5-ASA derivatives can take effect.  They are also commonly used for ulcerative colitis "flares."
• Antibodies to TNF-alpha - medicines like Remicade and Humira contain antibodies to one of the major inflammatory proteins in ulcerative colitis, TNF-alpha.  These antibodies "block" the effects of TNF-alpha.  Remicade is given as an intravenous infusion every few weeks and Humira is given as a shot.  These medicines seem to be more effective for Crohn's disease than ulcerative colitis.

Antibiotics

Antibiotics are sometimes useful, especially if there is a bacterial infection of the intestines complicating ulcerative colitis.  Medicines like metronidazole (Flagyl) and ciprofloxacin (in older children) are used.

Rectal suppositories and enemas

The 5-ASA deriviatives and glucocortocoids are available as enemas and suppositories.  They may be helpful for disease in the rectum and last part of the colon.

What about surgery for ulcerative colitis?

Removal of the colon cures ulcerative colitis and removes the long-term risk of colon cancer.  Kids who have the colon removed may require an ostomy - at least temporarily.  The end of the small intestines are brought to the outside of the body where stool can collect in a bag.  In most cases, the ostomy can be removed and a rectum-like structure called a "pouch" can be created with the end of the small intestines.  The pouch returns the bowel function to a more normal state.  Frequent, loose stooling usually persists, although most children will have less than 5 stools per day and can regain stool continence.

What are the complications of ulcerative colitis?

Flares of ulcerative colitis can be severe and require hospitalization.

• Severe diarrhea - Diarrhea can lead to dehydration and poor absorption of vitamins and nutrients.
• Anemia - Blood loss and poor blood production may require a blood transfusion.
• Infections - Use of immunomodulators increases the risk of developing infections anywhere in the body.
• Severe disease - Sometimes surgery is required to remove the colon.  The benefit of surgery is that it "cures" ulcerative colitis.  Complications after removal of the colon include frequent stooling and inflammation in the "pouch" or newly-created rectum-like structure.  Often an "ostomy" is created either temporarily or permantently.  The ostomy diverts stool to the outside of the abdomen into a bag that must be replaced frequently.
• Cancer - Children with ulcerative colitis have a 10% to 20% chance of developing cancer of the colon for each 10 years they have had the disease.  Children on immunomodulators are probably at increased risk for developing cancer also.  This risk is low and may be less than the risk of developing cancer from poorly-treated  or long-term ulcerative colitis.

What happens with ulcerative colitis long-term?

Well-treated ulcerative colitis should not limit a normal lifestyle and lifespan.  The risk of developing colon cancer is significant however.  Removal of the colon should be considered for poorly-controlled or long-term disease.