William Spivak, M.D.
This section was written to help parents of children with Crohn's Disease and Ulcerative Colitis understand the nature and treatment of their child's disease and to answer some common questions about these diseases. It is intended to act as a basis for further discussion between parent and physician.
Crohn’s Disease and Ulcerative Colitis are chronic (that is they are recurrent) inflammatory bowel diseases that usually have their onset in adolescence, but may begin at any age. The symptoms of Inflammatory Bowel Disease or IBD, as Crohn’s Disease and Ulcerative Colitis are sometimes called, are very variable and usually a child will have only a few of the symptoms at any given time. The symptoms can vary from very mild to severe enough to require hospitalization. Symptoms will usually completely or partially resolve with medication, and in about 25% of cases, will temporarily resolve without any treatment.
What are some of the symptoms of IBD in children?
Symptoms can be divided in to:
1) symptoms that are a direct result of inflammation of the intestine such as abdominal pain, diarrhea, rectal bleeding or vomiting;
2) symptoms that are related to the systemic or extraintestinal manifestations of the disease such as fever, arthritis (swollen, painful joints), inflammation of the liver, skin or eyes; and
3) symptoms that are clearly related to the disease but are not necessarily inflammatory in nature such as growth failure, depression and poor appetite.
What tests are done to make the diagnosis of IBD?
IBD is a clinical diagnosis, there is no specific laboratory test that will either confirm or reject the diagnosis. Since there are a number of other diseases that can mimic the symptoms of IBD in children (e.g. infectious or antibiotic associated colitis), the diagnosis cannot always be made with certainty during the early stages of symptoms. It is the recurring nature of the symptoms that will usually clinch the diagnosis, especially when associated with the extraintestinal manifestation of IBD.
Your doctor will have to do blood tests to test for anemia, liver disease and inflammation. X-Rays of the intestinal tract are frequently required. However, X-rays sometimes are misleading and only give an indirect view of the intestinal tract. Therefore, your doctor may need to do a colonoscopy under intravenous sedation. In addition to helping to determine the extent and severity of inflammation, a colonoscopy allows your doctor to obtain small amounts of tissue for microscopic examination (in order to distinguish between Crohn’s Disease and Ulcerative colitis) and to obtain fresh stool samples for culture and parasitic examination.
Why is it necessary to distinguish between Ulcerative Colitis and Crohn’s Disease and how are they different?
Ulcerative Colitis involves inflammation of the colon (large intestine) only, the rest of the intestinal tract is spared. Crohn’s disease usually involves the small and large intestine with inflammation. In addition, there is a significant increased risk of cancer of the colon in patients with ulcerative colitis for greater than 10 years; a much, much smaller risk of malignancy exists in Crohn’s patients. Whereas medications such as sulfasalazine are much more effective in Ulcerative Colitis than in Crohn’s disease, prednisone is much more effective in bringing about a rapid improvement in moderate to severe Crohn’s disease than it is in moderate to severe Ulcerative Colitis in children. While exacerbations of Ulcerative Colitis will almost always present with excessive diarrhea and rectal bleeding, exacerbations of Crohn’s disease may have more subtle symptoms such as weight loss and anorexia. Thus, by knowing the difference between Ulcerative Colitis and Crohn’s disease, you and your physician will have a better idea of the symptoms, treatment and prognosis of your child’s condition.
Newer forms of therapy
Remicade and Humira are both potent biologic inhibitors of TNF alpha that suppress the immune response without steroid side effects. However, both drugs can be associated with severe infections and should be avoided in people with active infections or a history of tuberculosis. A negative chest x-ray and a negative tuberculin test should be present before the use of these drugs. Remicade is approved for moderate to severe Crohn's disease in children and for Ulcerative Colitis and Crohns disease in adults. It is extremely effective in inducing remission in patients with moderately active to severe Crohn's disease. The drug is expensive and has to be administered every 2-6 weeks to sustain a remission. Severe reactions can occur during infusion inlcuding anaphylaxis, so patients must be carefully monitored during infusion. Response usually takes 3-7 days and lasts 4-6 weeks. Remicade is especiallyy effective in patients with fistulizing disease. A rare form of lymphoma has been reported in children and young adults; it is usually fatal. The chance of developing this form of fatal lymphoma in children is less than 1 in 10,000. Humira is approved for Crohn's disease only in adults, and is given by injection every several weeks. Once it is given a few times, it can be given at home by the patient or parent.
What is the role of Surgery in IBD in Children?
Surgery for Ulcerative Colitis is infrequently required in children, especially with the recent use of more effective drugs to control the disease. However, on rare occasion, a child may require colectomy (removal of the colon) because of intractable bleeding unresponsive to medical management or because of the inability to remove potent immunosuppressive drugs without a severe exacerbation of the disease. Often, the surgeon can make a continent ileostomy (not requiring a bag) or perform an ileo-anal pull-through procedure that will allow the child to pass stools through the anus. Both of these procedures have added risks and require a frank discussion with an experienced surgeon.
The need for surgery in Crohn’s disease is more common, and this is because of the association of Crohn’s with stricture (narrowing) of the intestine, abscess formation and fistulae (abnormal communications between inflammed bowel and other organs). Although 60% of patients with Crohn’s disease will ultimately require an operation, only about 10-15% of the time will this be necessary in childhood. Most abscesses or obstruction of the intestine in Crohn’s can be treated with antibiotics and steroids without the need for surgery.
If my child requires steroids, will he/she have severe side effects?
Will his/her growth be stunted?
Corticosteroids such as prednisone have potent anti-inflammatory effects and have the potential to cause significant side effect including growth failure, bone demineralization, hypertension, cataracts and obesity. However, most experienced pediatric gastroenterologists use prednisone sparingly and only as long as necessary to suppress symptoms. Usually, most children who require prednisone will require the medication for a period of several weeks to a few months. Once the child is in remission, the dose is tapered slowly to prevent an early recurrence of symptoms. When used in this manner, significant side effects are rare. A child may actually grow better while on low-dose prednisone if the prednisone is suppressing an otherwise active disease.
I have heard terrible stories about how people have suffered with IBD. Will my child suffer the same way?
IBD is not rare; in fact many famous people have had it and perhaps some of your friends or neighbors have IBD without your knowing about it. What we frequently here about from friends and relatives are the worst cases. In fact, when children with IBD receive good medical care, the disease generally tends to be mild. Most of children in my medical practice with IBD have not been admitted to the hospital more than one time (and many have never been admitted), and have not missed excessive amounts of school once they were under treatment. Stress may not cause the disease but may aggravate the disease. This is all the more reason for both you and your child to have an optimistic approach to this disease. Psychological counseling should not be avoided if undue stress is present.
What other medications are effective in IBD?
Sulafasalazine (Azulfidine) is an amino salycilate drug that is effective in the treatment of mildly active colitis and decreases the number of relapses in children with inactive colitis. Sulfasalazine, unfortunately, has frequent side effects, most of which are mild and reversible, including stomach upset, skin rash, headaches and bone marrow suppression. Since most of the reactions to Azulfadine are related to the Sulfa portion of the drug, forms of the drug that do not contain sulfa have been developed and are FDA approved. These are Rowasa (5-ASA enemas), Asacol (enteric coated 5-ASA) and Pentasa. These similar drugs have fewer side effects and may be effective in some patients. However, there are some patients who only respond to Azulfadine.
Cortifoam and Cortenemas are steroid enemas that are effective when the inflammation effects primarily the rectum and the sigmoid colon. They act locally on the colon to bring down the inflammation. These steroid enemas are used for mild to moderate acute exacerbations of the disease. Becausethey are used as an enema, it is inconvenient to use them for more than a period of several weeks. Cortenemas are generally more effective than Cortifoam, but Cortenema has a much higher systemic absorption of the steroid.
Antibiotics are sometimes effective in Crohn's disease, especially if there are either abdominal abscesses or perianal fistulae (tracts draining pus around the anus)Flagyl (Metronidazole), Cipro and Keflex and other oral antiotics may be effective.
6-MP, Imuran and cyclosporine have been used in adults and children to suppress inflammation in patients who are steroid dependent. Both are potent immunospressants with potentially serious side effects and therefore require close monitoring. Children on these drugs or on steroids should not receive live virus vaccines such as measles, mumps or rubella. If exposed to one of these viruses or to chickenpox, their physician should be contacted immediately.
My adolescent son refuses to take medicine in school. Is there a long acting medicine for his colitis?
Although not yet approved for children, Lialda is a 5-ASA medication that is specially coated so that it can be given just once a day.