Shelley Martin, a Manhattan accountant, was in her mid-60s when she learned after a routine colonoscopy that she had Crohn’s disease, a chronic inflammatory disorder characterized by abdominal pain and diarrhea. She said when friends learned of her diagnosis, several said “How can that be? Crohn’s starts in childhood.”
Actually, this often debilitating disease, which typically affects the area where the small intestine joins the colon, can occur at any age. “If you’re born with the right genetics, it can first appear in young kids to people in their 80s or 90s,” said Dr. Joseph D. Feuerstein, gastroenterologist at Beth Israel Deaconess Medical Center in Boston. “It’s rising in incidence and prevalence throughout the world,” he said, and gastroenterologists are still trying to figure out why it shows up when it does in different people.
Crohn’s disease was first described in 1932 by Dr. Burrill B. Crohn and colleagues and is one of two chronic inflammatory bowel diseases (ulcerative colitis is the other) that have no specific cause. Together, they afflict about three million people in the United States. Crohn’s in adults starts on average at age 30, with peak incidence between ages 20 and 30 and a second peak around age 50. The disease tends to run in families, but the genetic risk is not large. One in 10 to one in four patients have a close family member who is affected, and only half of identical twin pairs get it.
In decades past, Crohn’s was thought to primarily afflict people of Ashkenazi Jewish descent, but “we’re now seeing it everywhere — in Asia, Latin America, all over the world,” said Dr. Feuerstein.
Experts speculate that its rise is somehow linked to industrialization and a Western-style diet rich in meats and processed foods. Some suggest a link to living in an overly hygienic environment that may prompt the immune system to attack the body’s healthy tissues instead of infectious organisms.
And even though the bowel is the disease’s most prominent target, “it can also involve the eyes, joints, liver, skin,” said Dr. Gary R. Lichtenstein, gastroenterologist at the University of Pennsylvania School of Medicine. “It’s not one distinct disorder — over 200 genes have been identified as associated with Crohn’s. It results from a complex interaction between the environment and genetics” and can be initiated by an individual’s response to exposures ranging from infectious agents to medications.
Two well-established instigators are the frequent use of nonsteroidal anti-inflammatory drugs (NSAIDs), like ibuprofen and naproxen, and cigarette smoking. Both can trigger onset of the disease or cause flare-ups in those who already have it, Dr. Lichtenstein said. In fact, he said, smoking not only creates a greater risk of developing Crohn’s, it can also result in a more virulent course of the disease.
Unlike Ms. Martin, who had no inkling anything was wrong until her routine colon exam, most people with Crohn’s have unexplained symptoms for many months or even years before the correct cause is determined. Following the diagnosis, she said she developed “mild but annoying diarrhea,” but she considers herself relatively lucky given the potential complex of symptoms associated with Crohn’s.
In addition to abdominal pain and diarrhea that can be bloody, possible signs and symptoms include unexplained weight loss, anemia, fever, fatigue, nausea and vomiting, loss of appetite, eye and joint pain and tender, red bumps on the skin. In children, the disease can result in a failure to grow.
Prompt diagnosis and appropriate therapy to suppress inflammation in the digestive tract are extremely important because a delay can result in scar tissue and strictures that are not reversed by medication, Dr. Feuerstein said. Another possible serious complication is development of a fistula — an abnormal connection between different organs, like the colon and bladder, requiring surgical repair that, in turn, can cause further intestinal damage.
Understandably, considerable stress, anxiety and depression can accompany the disease and may even cause a worsening of symptoms. Last summer, when Ms. Martin’s disease suddenly raged out of control after she was treated with a drug to keep breast cancer at bay, severe diarrhea kept her tied to the bathroom in her Manhattan apartment. Dr. Lichtenstein said the class of drugs Ms. Martin took, called checkpoint inhibitors, is especially challenging to Crohn’s patients who may have to choose between trying to prevent a recurrence of cancer and suppressing their intestinal disease because the cancer drugs can sometimes cause an inflamed colon.
If severe inflammation and debilitating symptoms are present when Crohn’s is diagnosed, patients are usually treated with steroids to bring the disease under control before they are placed on medication specific for the condition. “Steroids,” Dr. Feuerstein said, “are a Band-Aid to arrest the inflammatory process, but then we have to do something to suppress the disease and allow the body to heal.”
Sometimes before starting medication, patients are temporarily placed on a restricted liquid diet to rest the bowel and give it a chance to heal, said Dr. Lichtenstein, the lead author of the latest management guidelines for Crohn’s disease developed by the American College of Gastroenterology.
There are now multiple drug options for treating Crohn’s, although keeping symptoms under control often involves trial and error. For example, following Ms. Martin’s diagnosis five years ago, the specialist she consulted told her there were four possible oral drugs to try in succession. Each worked for several months, but after the fourth drug no longer relieved her symptoms, she was given an infusion of a remedy called Entyvio, which she said “worked immediately like a miracle.”
Entyvio, the trade name for vedolizumab, is what’s known as a biologic, a drug made from living cells that is typically given by infusion or injection, one of several such drugs now available for Crohn’s. It acts specifically on the gut to counter inflammation, and with her colon still inflamed, Ms. Martinneeds to be treated with the drug every four weeks. If this one stops working, she can try one of the others.
Ms. Martin knows, however, that Crohn’s is not curable and most patients have to stay on medication indefinitely. That can create yet another stumbling block. The biologics are very costly, averaging over $100,000 a year, and although they are usually covered by insurance, there is a steep co-payment. To afford the therapy, many patients depend on co-pay assistance programs administered by the drug companies, Dr. Feuerstein said.
However, as Ms. Martin recently learned, Medicare will cover the expense if she gets the infusion in a hospital or if her doctor can arrange for a nurse to come to her home to administer the drug.
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