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Crohn’s Disease: Chronic, Incurable, and Manageable Shannon C Bio 139 04/14/2010 Casey 1 Although not given a name until 1932, symptoms of Crohn’s disease were documented as early as the 19th century (Steinhart 2006). Crohn’s is grouped with other diseases called inflammatory bowel diseases (IBD) that effect more than one million people in the United States alone (Dettinger et. al. 2008). The common factor with IBDs is an inflammatory response in the gastrointestinal tract, and the two major diseases noted in this category are Crohn’s disease (CD) and ulcerative colitis. Although the two diseases present some of the same signs and symptoms, Crohn’s seems to be the most difficult to treat and diagnose because it manifests itself in many different ways. However, new diagnostic tools and treatments are allowing CD sufferers quicker diagnoses and better treatments (Steinhart 2006). Dr. Burrill Crohn and two of his colleagues at Mount Sinai Hospital in New York first characterized CD as inflammation of the terminal part of the small intestine, or the ileum, and they called the disease “regional ileitis” (Steinhart 2006). Although the doctors recognized regional ileitis in 1932, it took several years before the condition was given the name Crohn’s disease, and it wasn’t until the 1950s that scientists realized that CD could involve any part of the entire gastrointestinal (GI) tract (Steinhart 2006). CD could now be differentiated from ulcerative colitis, because doctors knew that ulcerative colitis only impacts the large intestine. Doctors could now focus specifically on treating the symptoms of CD, and researchers could begin to identify its cause(s) (Steinhart 2006). CD is sometimes hard to diagnose because sufferers often have periods of disease activity that are followed by periods of disease remission (Steinhart 2006). It is considered a chronic disease with symptoms that can vary from mild to severe, affect any part of the GI tract, and may even affect areas outside of the GI tract. When the disease is active, sufferers report a variety of symptoms including continuing bouts of diarrhea, abdominal cramps, fever, and rectal bleeding. Casey 2 CD has also been shown to affect the liver, skin, eyes, and joints of its sufferers, as well as cause fatigue and slowed growth and sexual maturation in children (www.ccfa.org 2010). CD is rarely diagnosed in children under 5 years old, but the disease is most prevalent in young people, especially those between the ages of 20 and 40 (Steinhart 2006). When the disease is active, doctors often look for skip lesions in the gastrointestinal tract. The inflamed areas of the small and large intestines are often referred to as lesions, and in CD, lesions can occur in different parts of the intestines at the same time. A person with CD may have lesions in the jejunum, or middle part of the small intestine, have a healthy ileum, and have lesions occurring again in the large intestine. Thus, the disease may skip around the gastrointestinal tract leaving lesions, hence the term skip lesions (Steinhart 2006). Another important sign in diagnosing CD is lesion penetration beyond the mucosal lining of the intestines. Ulcerative colitis presents with penetration of the mucosal lining, but CD lesions can penetrate through the submucosal lining and the muscular layer of the intestines, resulting in holes in the intestinal wall called abscesses. Abscesses can lead to infections in the wall of the abdomen (Steinhart 2006). Abscesses and infections in the abdomen are not the only complications of CD. Alternating episodes of disease remission and disease activity can leave scar tissue in the abdomen, caused by inflammation and the healing process. This scar tissue can build up overtime causing strictures, or narrowing segments of the intestinal lumen. Strictures alone are not a danger to the health of the CD patient, but they may lead to bowel obstructions. Bowel obstructions prevent substances in the intestines from passing through the GI tract, and their symptoms can include cramping, vomiting, fever, and a distended abdomen. Treatment of bowel obstructions can range from a few days on a liquid diet to surgical intervention, depending on their severity (Steinhart 2006). Casey 3 Along with strictures, CD patients also must be concerned with fistulas. A fistula occurs when an abscess is present, and the adhesive nature of the inflamed intestine connects to another part of the intestine or another organ (Steinhart 2006). The other organs most commonly affected by fistulas are the bladder, vagina, or skin, and fistulas often occur in the area around the anus (www.ccfa.org 2010). Some perianal fistulas, also called perineal fistulas, can leak intestinal fluid, mucus, blood, or stool. The site around the fistula can become infected or inflamed because it is hard to keep clean due to drainage of bodily fluids (Steinhart 2006). Although only approximately 30% of CD patients develop fistulas, it is the part of the disease that can show on the outside of the body (www.ccfa.org 2010). Therefore, it is often the most distressing complication of CD. The extra-intestinal effects of CD can be seen in the eyes, joints, skin, and liver. CD shows its affect on the eyes through inflammation that can occur in several parts of the organ. Joint inflammation normally shows up in the knees, ankles, wrists, and knuckles, and sacroiliitis, a form of arthritis that can be seen in some CD patients, normally causes only minor discomfort but occasionally causes fusion of vertebrae. Liver damage in CD patients is less common than the other complications. Skin lesions can be treated but may leave visible areas of scarring. Fatigue and weight loss can occur in CD because of problems with absorbing nutrients, especially vitamin B12, which is absorbed mainly in the ileum (Steinhart 2006). The symptoms of CD are difficult to ignore, and therefore, most patients seek medical intervention. Along with noting the symptoms the patient is already having, health-care practitioners often ask for the patient’s medical and family history and test blood and urine for abnormalities. An X-ray of the abdomen can be used to evaluate the bowel for obstructions or for any fissures, or tears, in the intestines. Fistulas and abscesses may also be visible on the X- Casey 4 ray. Of the different types of endoscopy, a colonoscopy is the most useful for diagnosing CD (Norton, et. al. 2008). A colonoscopy allows physicians to examine the entire length of the large intestine and the ileum of the small intestine, thus allowing easier viewing of skip lesions. The colonoscopy also allows for a tissue sample to be taken from the intestines for biopsy. Because a history of CD increases the risk of developing colon cancer, a biopsy of the area can be used for detecting warning signs of the cancer (www.ccfa.org 2010). Other useful diagnostic tests can include an ultrasound of the intestines, small bowel radiological examination, an MRI, a CT scan, and screening for specific antibodies (Norton, et. al 2008). Even with all the tests available for diagnosis, scientists still do not know for certain what causes a person to develop CD, but a family history of the disease is often a good indicator. If a close relative has CD, an individual has as much as a 20% chance of developing the disease (Steinhart 2006). Yet, the majority of CD sufferers have no family history of the disease. Therefore, scientists are beginning to investigate multiple genes and their mutations that may make an individual more susceptible to developing CD (Steinhart 2006). The question still remains as to what causes the intestinal inflammation associated with CD. One hypothesis is that one or more unidentified bacteria mount an attack on different parts of the GI tract causing inflammation. However, a more widely accepted theory is that the immune system, for some unknown reason, recognizes parts of the GI tract as foreign. As the immune system begins to attack the intestinal lining, it becomes inflamed. Because of this theory, CD is often referred to as an autoimmune disease (Dettinger et. al. 2008). Since the source of the inflammation is unknown, treating the it can be difficult. Many different treatments exist for CD including anti-inflammatory drugs, immunosuppressant drugs, surgeries, antibiotic drugs, steroid medications, and dietary methods Casey 5 (Steinhart 2006). Most doctors try first to treat CD patients with anti-inflammatory drugs. One such drug is mesalamine (Dettinger et. al. 2008). Mesalamine is also called 5-aminosalicylic acid or 5-ASA and shows anti-inflammatory properties almost exclusively in the GI tract. Another drug, sulfasalazine, is a combination of 5-ASA and a sulfa antibiotic, and therefore, it can treat both inflammation and infection. Both mesalamine and sulfasalazine have limited efficacy when the mechanisms of CD are highly active (Steinhart 2006). Fistulas and abscesses often lead to infections in CD patients, and antibiotic drugs, particularly metronidazole and ciprofloxacin, are prescribed to eliminate the infection. Steroid medications are useful in alleviating inflammation, but because of the side effects of these drugs, it is recommended that they be used no longer than 4 months (Dettinger et. al. 2008). Two newer medications do not fit into the normal category of drugs used in the treatment of CD, but both have shown promise in managing the disease. Infliximab, also known as remicade, is a laboratory engineered antibody used to inhibit the protein known as tumor necrosis factor (TNF) (Steinhart 2006). TNF is protein in the body that is vital in the inflammation process, and thus, TNF-blockers improve the symptoms of CD by intestinal inflammation reduction. Treatment with infliximab can be difficult because the medication must be given intravenously, and the long-term side-effects are not known (Steinhart 2006). Humira, also called adalimumab, is another TNF-blocker that has shown promise in treating CD (Steinhart 2006). In one scientific study, treatment with Humira not only improved the symptoms of the disease, but it also lead to remission. In fact, 64% of patients in the study remained in remission for 3 years (“Humira sustains” 2009). However, because of potential side effects of these drugs, they are often used as last-resort drugs for the treatment of CD. Casey 6 Dietary changes can help to manage the symptoms of CD when the disease is active. Eating smaller meals can decrease indigestion, and reducing or eliminating fried foods, “butter, margarine, cream sauces, and pork products” can help to lessen bouts of diarrhea (www.ccfa.org 2010). Reducing the amount of milk products, especially for those that are lactose intolerant, can alleviate some gas and abdominal cramping. Furthermore, several high-fiber foods, like seeds, nuts, and corn, can interfere with strictures and cause diarrhea and cramping. Therefore, these foods should be avoided when CD is active (www.ccfa.org 2010). Several surgeries are available for CD sufferers, and the type that is performed is dependent on the severity of CD and the type of complication that is being treated. A strictureplasty will widen the area of the intestines that is being restricted, but if a stricture is large enough, resection of the intestines may be necessary. Resection involves cutting away the stricture and rejoining the intestine, known as anastomosis (www.ccfa.org 2010). For more severe cases of CD, a colectomy or proctocolectomy may be required. A colectomy requires removal of the entire colon, and the terminal portion of the small intestine is joined to the rectum so that the patient can still have regular bowel movements. A proctocolectomy requires removal of the entire colon and the rectum. In this case, a stoma, or hole in the intestinal wall, is required for removal of digestive wastes. Surgery is always a last resort for treating CD, but it should not be avoided when all other efforts to control the disease have failed (www.ccfa.org 2010). Crohn’s disease is a chronic, incurable condition that affects approximately one million people in the United States alone (www.ccfa.org 2010). Its symptoms can range from mild to severe but most often require medical intervention. Symptoms of the disease often dictate the treatment required, and treatment can range from drug therapy to surgery. Many CD patients Casey 7 must alter their lifestyles to manage the disease. However, with the support of family, friends, and the medical community, people with CD can lead long and normal lives (Steinhart 2006). Casey 8 References (2009). Humira sustains remission for three years in Crohn's. British Journal of Healthcare Management, 15(7), 354-355. Retrieved from CINAHL with Full Text database. Crohn’s & Colitis Foundation of America. Retrieved April 12, 2010 from www.ccfa.org. Dettinger, M., Matzke, M., McKay, K., Sizer, R., Thoreson, M., & Wrobleski, D. (2008). Crohn's disease and ulcerative colitis. Journal Of Continuing Education In Nursing, 39(4), 151-152. Retrieved from MEDLINE database. Norton, C., Williams, J., Taylor, C., Nunwa, A. and Whayman, K. (Eds.). 2008. Oxford Handbook of Gastrointestinal Nursing. Oxford, NY: Oxford University Press, Inc. Steinhart, Hillary. (2006). Crohn’s & Colitis: Understanding and Managing IBD. Toronto, Ontario, Canada: Robert Rose Inc.