Pulse
Not Just for Adults
With the incidence of inflammatory bowel disease increasing in children, more doctors will need to watch for this tough-to-recognize condition.
Caroline was just finishing her freshman year in high school last spring when her health took a troubling turn. What started as seemingly benign stomach cramping turned into bloody stools and then bloody diarrhea accompanied by exhaustion. After a month of persistent symptoms, the Blaine teenager finally told her parents, who scheduled an appointment with her pediatrician. When routine laboratory tests ruled out bacterial infection, Caroline’s doctor referred her to a pediatric gastroenterologist, who diagnosed her with ulcerative colitis, one of the two most common forms of inflammatory bowel disease (IBD), the other being Crohn’s disease. “The diagnosis really took me by surprise, and I work in health care,” recalls her mother, Renata. “I always thought it was an older people’s disease.”
Statistics show that Caroline’s case is far from unique. According to Sundeep Arora, M.D., a pediatric gastroenterologist with Minnesota Gastroenterology, the incidence of IBD peaks at two stages of life: before age 20 and between ages 40 and 60.
Signs of IBD
In children, inflammatory bowel disease can present in a variety of ways. For that reason, experts say pediatricians and family physicians need to have a high degree of suspicion for diseases such as Crohn’s and ulcerative colitis when they see any of the following symptoms:
- Abdominal pain
- Diarrhea
- Bloody stools
- Fatigue
- Skin tags in the perianal area
- Skin rashes
- Unexplained poor growth, even when a child is eating well
- Inflamed joints
- Joint pain
- Amenorrhea
- Recurrent swelling of the lip
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Studies have shown a documented increase in Crohn’s disease, ulcerative colitis, or both among children in Finland, the Czech Republic, Denmark, France, Norway, Central Saudi Arabia, and Canada. However, the data are voluminous and, in some cases, limited to specific populations. One study of children in Wisconsin published in the October 2003
Journal of Pediatrics did not demonstrate an increase from one year to the next but did find a disturbingly high incidence rate over a two-year period.
In Minnesota, Arora and others who treat children with GI problems have noticed they’re seeing more kids with IBD in recent years. “No one knows exactly what accounts for the rise, but there really is no denying that the incidence is increasing,” says Arora, who has been in practice for seven years and has seen patients as young as 6 months present with the disease. “When I first started working in pediatric gastroenterology, I was diagnosing one or two cases of IBD per month. Now there are times when I see that many in a week.”
The recent increase in the number of children seeking care prompted the University of Minnesota to open the Center for Pediatric Inflammatory Bowel Disease in January. The multidisciplinary center, which includes pediatric gastroenterologists, pediatric surgeons, psychologists, nutritionists, and even adult gastroenterologists, is one of a handful of its kind in the country. Since it opened, the center has served more than 100 patients from Minnesota, Iowa, North and South Dakota, and Wisconsin, including Caroline.
Hard to Detect
Caroline presented with common symptoms of IBD: bloody stools, fatigue, abdominal pain. But in many children, the signs are not so clear. “The disease is kind of tricky in nature because it can have all sorts of presentations in kids—low-grade fevers, recurrent oral sores, painful skin rashes, joint swelling or pain, anemia—and there is often no rhyme or reason to it,” Arora says. “We have diagnosed patients who have come in with an isolated recurrent swelling of the upper or lower lip, and that was their only presentation.”
Because of the inflammatory nature of IBD, the chemical mediators that cause inflammation of the intestines also can cause inflammation outside of the gut. “We call them extra-intestinal manifestations,” explains Boris Sudel, M.D., a pediatric gastroenterologist with the University of Minnesota Medical Center, Fairview and director of the Center for Pediatric Inflammatory Bowel Disease. “They’re presentations that you would not directly connect with IBD unless you think IBD.” Sudel says he’s had primary care physicians call asking whether patients with arthritis for whom treatment is not working may instead have IBD. And Arora says he’s diagnosed IBD in children with skin tags in the perianal area that were originally misconstrued as hemorrhoids as well as children who were just not growing, despite the fact that they ate well and had previous negative lab workups for IBD.
Sudel says such red herrings are reasons why pediatricians and other primary care providers need to be on high alert for the presence of IBD in young people and why it can take longer to diagnose IBD in children than in adults. “On average, it can take about a year from the time of initial presentation to diagnose IBD in kids,” he says.
Once doctors consider IBD as a possibility, diagnosis becomes somewhat straightforward. Standard laboratory tests—erythrocyte sedimentation rate, C-reactive protein levels in blood, platelet counts, hemoglobin levels—can identify nonspecific signs of inflammation. Stool samples can rule out infections that may be acquired during travel.
If tests point to IBD (for example, anemia, low albumin levels, or high platelet counts), doctors usually refer patients to a pediatric gastroenterologist for endoscopy or colonoscopy along with a biopsy to conclusively identify inflammation in the intestines.
Treatment Considerations
Management of IBD in pediatric patients can be daunting and complex. As with adults, medical management of IBD in kids usually involves a combination of therapies—steroids (usually prednisone); 5 aminosalicylic acid compounds; immunomodulators such as azathioprine, 6-mercaptopurine, and methotrexate; and biologic agents such as infliximab and adalimumab. In severe cases of ulcerative colitis, doctors may remove the colon. But the similarities between treating adults and children end there in part because kids are still growing.
Genetic Testing for IBD
Most experts agree that the pathogenesis of inflammatory bowel disease (IBD) involves the interaction of three separate factors: the patient’s environment, immune system, and genes. Often, the latter is what garners the most attention—and disproportionately so. About 5 percent to 10 percent of patients have a family history of IBD, according to Boris Sudel, M.D., director of the University of Minnesota Center for Pediatric Inflammatory Bowel Disease.
In 2001, researchers identified the first gene associated with IBD—referred to as the NOD 2, CARD 15 gene on chromosome 16. The mutation was found to be associated with Crohn’s disease. Since then, researchers have identified numerous other genetic associations, and “one or more associations with IBD have been found on almost every single chromosome in the human genome,” according to Sudel.
Today, tests that detect the presence of some of these genes are commercially available. But Sudel cautions against using them. “IBD is a polygenic disease, which means that no one gene is fully responsible for IBD, and we really don’t know yet what it means to carry these gene mutations in general,” he says. “We know that genetic linkages may one day explain differences in the disease presentation, predict its severity and complications, and guide therapy. But at this point, it’s a research tool, and I don’t believe it should be used in daily practice.” —J.M.
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Because IBD can cause malnourishment, doctors want to detect it in children in its earliest stages and then treat it aggressively to stave off any negative effects on growth and development. Long-term, uncontrolled IBD is associated with serious eye inflammations, lymphoma, and a 0.5 percent to 1 percent annual risk of developing colon cancer during the first 10 years after diagnosis. Yet long-term use of steroids, the first line of treatment, can affect growth in kids, and chronic use of other IBD medications can lead to an increased risk for infection. “With children, we walk a fine line,” Arora says. “We have to be cautious with therapy and not use the drugs in a manner that will affect growth, yet we have to be on the aggressive side with treatment so the child does not suffer a loss of height because of this disease.”
Doctors at the University of Minnesota are using a fairly novel approach—long-term nutritional therapy—to treat Crohn’s disease in order to avoid some of the side effects associated with other treatments. Patients receive continuous feeding through a gastric tube during the night, several times a week. During the day, they remove the tube and eat what they want. The verdict is still out on the exact mechanism by which nutritional therapy manages Crohn’s symptoms. “We speculate that continuous nutritional flow overnight through the nasogastric tube may, in addition to providing calories, help change intestinal bacterial flora, thus promoting healing possibly by driving harmful bacteria out and allowing ‘good’ bacteria to proliferate,” says Sudel. Such treatment is widely used in Europe but is offered at only a handful of centers in the United States, he explains.
Although the tube initially can be uncomfortable for patients to insert, Sudel says that it’s a small price to pay compared with the side effects associated with steroids, which can include a puffy face, acne, hypertension, and growth retardation. As with medication regimens for Crohn’s disease and ulcerative colitis, nutritional therapy, if successful, must be used long-term, usually for the duration of a person’s life, if they are to keep the disease in remission.
Unlike for adults with IBD, there are no guidelines for treating children with the condition. Sudel and others from the university are working on formulating them through their affiliation with the Pediatric IBD Network, a coalition of about a dozen pediatric IBD centers.
Back on Track
After being hospitalized twice to get nourishment and stabilize her condition, Caroline, who will turn 16 this month, is now managing her inflammatory bowel disease. Her appetite is back, and after much trial and error, her doctors have found a combination of medications that is producing good results. Her new regimen includes immunomodulators and ASA-5. “Things got worse before they got better, but I hear from other parents and doctors that that can be quite common,” says her mother. “Once the disease is under control, you can lead a pretty normal life again, and I think we’re finally getting close to that point.”—Jeanne Mettner