Irritable Bowel Syndrome
Ulcerative colitis and Crohn’s disease account for most inflammatory bowel disease seen in primary care practice. Abdominal pain, diarrhoea, and bleeding are among the presenting manifestations. The first priority is to distinguish inflammatory bowel disease from other causes of diarrhoea. The chronicity, potentially disabling symptoms, risk of malignancy (in the case of ulcerative colitis), and occasional refractoriness to medical therapy make management a major challenge. The primary care physician needs to know how to treat exacerbations, maintain remissions, and psychologically sustain these patients through difficult times. Competent care is based on a thorough understanding of the roles for medical and surgical therapy and skill in providing psychological support. Although patients with severe or refractory disease may need to be referred to the gastroenterologist, most others can be well managed by the primary care physician.
Ulcerative colitis is an idiopathic diffuse inflammatory disease of the bowel mucosa. Although pathogenesis remains poorly understood, there is growing evidence of a primary immune mechanism (e.g., high prevalence of antibodies to intestinal epithelial antigens in asymptomatic relatives of patients and in patients themselves). The disease typically begins in adolescence or young adulthood but may occur at almost any age. Whites are affected more often than African-Americans. Prevalence is highest among Jews of Eastern European descent, and there is a 10-fold increase in risk for having the disease among first-degree relatives of patients.
Clinical Presentation. The cardinal symptoms are bloody diarrhea and abdominal pain; in severe cases, fever, anorexia, and weight loss are present as well. The variability of presentations is remarkable, ranging from malaise and no symptoms referable to the colon, to fever, prostration, abdominal distention, and passage of large volumes of liquid stool. The disease need not be confined to the bowel; extracolonic manifestations include arthritis, uveitis, jaundice, and skin lesions. The course is characteristically chronic, recurrent, and unpredictable. An insidious presentation does not predict a benign course, and a fulminant onset may be followed by long relatively asymptomatic periods.
Ulcerative colitis almost always involves the distal colon and rectum, making diagnosis possible by sigmoidoscopy. The mucosa becomes edematous, obscuring the fine network of sub-mucosal vessels. The moist glistening mucosal surface is lost, and a granular appearance develops. The bowel wall is friable, bleeding spontaneously or when touched with a swab. In advanced cases, pseudopolyps and discrete ulcers may be seen. Smears of mucus from the bowel wall show polymorphonuclear leukocytes. Barium enema documents the extent of disease. Radiologic findings range from mucosal denudation to frank ulceration, with loss of haustral markings and a tubular appearance. There are no skip areas. Liver involvement occurs in the form of pericholangitis and fatty infiltration; these are common histologic findings in ulcerative colitis but are seldom symptomatic. Much less frequently, chronic active hepatitis, cirrhosis, or sclerosing cholangitis is seen. A migratory monoarticular arthritis affecting the large joints develops in 10% of patients. This arthritis often coincides with an exacerbation of colitis and resolves with control of the underlying disease. Ankylosing spondylitis also occurs but runs a course independent of the colitis. Uveitis or episcleritis may be seen at any time during the course of the disease. Erythema nodosum, pyoderma gangrenosum, or oral aphthous ulcerations are found in about 5% of patients, usually during active colitis.
Course. The prognosis for patients with ulcerative colitis seen in the primary care setting is far better than that for patients studied in referral centers who are likely to have more severe disease. Long-term community-based studies find that nearly 90% go into complete remission after the first attack and less than 10% develop chronic persistent disease. Among those with chronic disease, nearly three fourths have disease limited to the distal bowel (rectum or rectosigmoid). Overall mortality in community-based populations of ulcerative colitis patients is no different from that of the general population, although it is increased in patients with severe first attacks or extensive disease.
There is an increased risk of cancer that correlates with the extent and duration of disease and age at diagnosis. Risk begins to increase substantially after 8 years of illness. Absolute risk of colon cancer in the setting of pancolitis has been found to be 30% after 35 years from time of diagnosis. Those with pancolitis diagnosed before the age of 15 manifest an absolute risk of 40%. These figures are slightly lower than traditional risk estimates of 1% to 2% per year derived from referral center cohorts.
Typically, the patient with ulcerative proctitis is a young adult who presents with rectal bleeding and tenesmus. The bleeding is usually not severe; it is sometimes mistakenly attributed to hemorrhoids. Diarrhea or constipation may accompany the bleeding, but often there are only small frequent bowel movements associated with a small amount of mucus. On sigmoidoscopy, an edematous friable rectal mucosa is observed; the bowel above the rectosigmoid is uninvolved. On barium enema or colonoscopy, the remainder of the large bowel is normal. The clinical presentation of ulcerative proctitis is not pathognomonic; the condition must be distinguished from infectious forms of proctocolitis, including AIDS-related etiologies.
Ulcerative proctitis is a variant of ulcerative colitis, distinguished by the limited extent of inflammation, its good prognosis, and paucity of serious complications. However, relapses are common. Fewer than 15% progress to generalized ulcerative colitis. The distant complications of ulcerative colitis are rare, and carcinoma of the rectum develops no more often than in unaffected individuals.
Pathophysiology. Crohn’s disease is a chronic relapsing inflammatory disorder of the alimentary tract that appears to have an autoimmune pathophysiology. There are high levels of selected T-cell populations and high prevalence of antibodies to intestinal epithelial antigens in patients and in their asymptomatic relatives. Of therapeutic interest is the role of inflammatory mediators. T helper cells in the bowel mucosa of patients with Crohn’s disease appear to produce increased amounts of tumor necrosis factor-a. Infusion of monoclonal antibodies directed against these cytokines can produce remission of otherwise refractory disease (see below). Other potentially important cytokines include interleukin-12 and interferon-g. There may also be inadequate production of counter-regulatory substances. Pathologically, the distribution of bowel inflammation is discontinuous, with diseased segments of bowel separated by normal areas. Because the granulomatous inflammatory process may extend through all layers of the bowel wall, it has a tendency to cause strictures, fistulas, and abscesses.
Clinical Presentation. Peak incidence is in the second and third decades. The condition often affects the distal ileum and right colon, but frequently it involves only the small bowel or colon. It may occur in any portion of the alimentary tract, from the buccal mucosa to the anus. Extraintestinal involvement occurs in 15% to 20% of cases, with arthritis, ankylosing spondylitis, uveitis, erythema nodosum, aphthous oral ulcers, and pyoderma gangrenosum being the predominant manifestations of disease outside the bowel. In addition, cholelithiasis and nephrolithiasis have a higher incidence in these patients than in the general population.
Symptoms vary, depending on the location and extent of disease. Diarrhea and abdominal pain (particularly in the right lower quadrant) are cardinal symptoms, occurring in almost 80% of patients. Weight loss, vomiting, fever, perianal discomfort, and bleeding are also common complaints. Constipation may be an early manifestation of obstruction. Symptoms can develop subtly or can present in fulminant fashion with the patient systemically toxic.
Physical examination may reveal a discrete abdominal mass, especially in the right lower quadrant, but usually a normal abdomen or doughy loops of bowel are found. Abdominal or perianal fistulous tracts are noted on examination in up to 10% of patients. Extraintestinal findings include inflamed joints, spinal deformities, erythema nodosum, pyoderma, uveitis, and aphthous ulcers.
Sigmoidoscopy is abnormal in fewer than 20% of cases; fistulous tracts and discrete inflammatory ulcers are sometimes encountered in the rectosigmoid. Barium enema and upper gastrointestinal series often show segmental involvement of large and small bowel, often with strictures, fistulas, and ulcers. The primary abnormality in Crohn’s disease is submucosal, causing radiologic studies to sometimes appear normal. In such cases, colonoscopy aids diagnosis by demonstrating segmental disease and ulceration that may be missed on barium enema.
Prognosis. Although it is difficult to extrapolate from referral center data to patients seen in primary care settings, a pattern emerges of disease activity that waxes and wanes over many years. Disease-free intervals may last as long as several years or even decades, but recurrences are the rule. Several years of relief from symptoms may be afforded by surgical resection, but there is no evidence that any medical and surgical therapy alters the ultimate course of the illness. In referral center series, as many as 70% of patients ultimately require surgical resection.
Proper management requires confirming the diagnosis and determining the extent of disease. One proviso should be kept in mind: Because these illnesses often occur in women of childbearing age, attempts should be made to minimize their x-ray exposure and carefully select only the most necessary radiologic studies.
Diagnosis. The diagnosis is usually based on the clinical presentation, sigmoidoscopic demonstration of inflammation, and the exclusion of bacterial and parasitic infections by culture and examination for ova and parasites. Because the disease almost invariably affects the distal colon and rectum, sigmoidoscopy is an essential component of the workup. The procedure is best performed without cleansing preparations, so as not to distort the appearance of the bowel mucosa. In acute phases of the illness, the mucosa appears friable and inflamed; there is loss of the normal vascular pattern. As the disease progresses, a purulent exudate and discrete small ulcers may form. With severe colitis, there may be pus and spontaneous bleeding and large ulcers. Chronic phases of the disease are characterized by a granular mucosa and inflammatory pseudopolyps (tags of damaged mucosa and granulation tissue).
When the sigmoidoscopic picture is nonspecific, one should culture and examine the stool for Clostridium difficile, Entamoeba histolytica, Campylobacter, Shigella, Salmonella, and Neisseria gonorrhea. Rectal biopsy is indicated when one needs to confirm the diagnosis and exclude conditions such as Crohn’s disease of the rectosigmoid, amoebic colitis, pseudomembranous colitis, cytomegalovirus infection, and herpetic pancolitis. Barium enema or colonoscopy can be used to provide supportive evidence when the diagnosis is in doubt and helps document the extent of disease. However, they should not be performed during a flare-up because there is a small risk of perforation when the procedure is performed on an acutely inflamed bowel.
Estimating Disease Activity and Severity. The appearance of the bowel mucosa on colonoscopy remains the mainstay of assessment of disease activity. Scoring systems based on clinical parameters are sometimes used in research settings but have little utility in clinical practice. A host of radionuclide imaging methods has been tried, but they lack specificity. Disease severity is defined more clinically. Mild disease is defined as less than four bowel movements a day and no signs of toxicity (i.e., no fever, tachycardia, anemia, or elevation of sedimentation rate). Moderate disease is characterized by four or more bowel movements a day plus minimal toxicity. Severe disease is manifested by six or more bowel movements a day and/or signs of toxicity.
Diagnosis. Crohn’s disease of the colon may mimic ulcerative colitis clinically. Differentiating features include skip areas in the colon, significant small bowel involvement, fistulas, and granulomas on biopsy. The diagnosis is suggested by a history of recurrent postprandial lower abdominal pain and altered bowel habits in a young person; it is reinforced by finding on physical examination a mass or tenderness in the right lower quadrant. Radiologic contrast studies are needed for a more definitive assessment. The small bowel phase of an upper gastrointestinal series shows segmental narrowing, areas with loss of the normal mucosal pattern interspersed with areas of normal mucosa, fistula formation, and the “string sign” (a narrow band of barium flowing through an inflamed or scarred area) in the terminal ileum.
Colonic disease may be documented by air contrast barium enema, with asymmetric segmental changes distinguishing Crohn’s disease of the large bowel from ulcerative colitis. Disease of the terminal ileum can often be detected on barium enema; however, radiologic involvement of the terminal ileum is not unique to Crohn’s disease. Some ulcerative colitis patients also demonstrate inflammatory changes in the terminal ileum (“backwash ileitis”), but they lack the skip pattern characteristic of Crohn’s disease.
Sigmoidoscopy demonstrates rectosigmoid inflammation in the 20% to 50% of patients with disease in this area; however, the findings are often nonspecific (mild erythema). Colonoscopy is needed in difficult cases and helps in judging the extent and severity of disease. Biopsy can be diagnostic but is usually unnecessary unless the diagnosis remains unsubstantiated; it should be avoided when acute inflammation is present.
Estimating Disease Activity and Severity. Disease activity in the colon is best assessed by colonoscopy and in the small bowel by barium contrast study. Disease severity is categorized clinically. Mild-to-moderate disease is defined as having symptoms but functioning adequately on an ambulatory basis, maintaining oral intake of food and fluids, and showing no signs of toxicity or complications. In moderate-to-severe disease, symptoms are more severe, sometimes interfering with daily activity and not responding fully to treatment. In severe disease, there may be toxicity, complications, and failure to respond to full doses of oral corticosteroids.
The inflammatory bowel diseases are chronic illnesses that require long-term comprehensive management. Such management entails attention to the patient’s medical, psychological, and nutritional needs and support for the family. For the most part, treatment is empirical and directed at providing symptomatic relief; however advances in the understanding of inflammatory bowel disease pathophysiology are leading to a host of new treatments that have the potential to improve treatment outcomes.
Because the disease typically follows a relapsing course with acute exacerbations and intervals of remission, the approach to treatment depends on the patient’s current clinical status. During remission, treatment is prophylactic; during flare-ups, the goal is control of the inflammatory process. Surgery is a consideration for those with refractory disease, especially when it is widespread.
Dietary and Nutritional Measures. No specific diet improves or exacerbates ulcerative colitis. However, reduction in dietary fiber may be of some benefit during periods of active disease. In patients with inactive disease, 1 or 2 teaspoons of psyllium hydrophilic colloid (Metamucil) in water daily often helps to bind the stool. There is an increased incidence of lactase deficiency in these patients; an empirical trial of a milk-free diet is reasonable when diarrhea persists despite other evidence of clinical remission. Those who are anemic from blood loss need oral or parenteral iron supplementation. Oral iron may be poorly tolerated, necessitating parenteral administration. Anemia may also be due to folic acid deficiency. Folic acid supplementation is indicated when intake of leafy vegetables and fresh fruits is poor or when sulfasalazine is being taken (see below). Anemia may also be due to chronic disease and may not respond to dietary and nutritional measures.
Sulfasalazine. The drug is recommended as initial treatment for mild to moderate disease and for prevention of relapses. After oral administration, about 70% reaches the colon, where it is metabolized by intestinal bacteria, resulting in the local release of sulfapyridine and the salicylate analogue 5-aminosalicylate (5-ASA), which is believed to be the active moiety. Sulfasalazine’s precise mechanism of action remains speculative. Hypotheses include effects on prostaglandin synthesis (particularly arachidonic acid metabolism) and inhibition of migration of polymorphonuclear leukocytes.
Efficacy. Randomized controlled studies have shown the drug to be effective as initial treatment for patients with mild to moderate symptoms when given in doses of 4 g/d for 2 to 4 weeks. About 80% of patients respond. Because sulfasalazine is less effective than corticosteroid therapy, it is reserved for relatively mild cases; however, combined use with steroids has been suggested for early treatment of severe disease.
Controlled studies have also documented the drug’s efficacy for prophylaxis and maintaining remissions. In one major study, more than 65% of patients given maintenance doses of 2 g/d remained symptom-free for at least 1 year compared with 25% of patients given placebo. The prophylactic effect of maintenance therapy persists when the drug is continued beyond 1 year. The optimum dose is 2 g/d (4 g/d provides even better protection, but the frequency of side effects is markedly increased).
Adverse Effects. Sulfasalazine is usually well tolerated (being safe enough to use during pregnancy and when breastfeeding), but up to 20% experience adverse effects, mostly due to the sulfapyridine moiety. These range from common forms of dose-related gastrointestinal upset (nausea, vomiting, anorexia, heartburn) and mild hypersensitivity reactions (rash, fever) to uncommon but potentially serious idiosyncratic reactions such as agranulocytosis, hepatocellular injury, and lupuslike phenomena. Patients unable to tolerate full doses of sulfasalazine because of gastrointestinal upset often do better when reintroduced to the drug more gradually. Taking it with meals also helps. Other potential hematopoietic effects include anemia (from folic acid deficiency, hemolysis, or marrow suppression), granulocytopenia, and thrombocytopenia. Low sperm counts and qualitative sperm abnormalities have been noted in men taking the drug, usually after about 2 months of therapy; these conditions reverse when the medication is stopped. Desensitization (starting with fractions of a tablet and slowly advancing over 2 to 4 weeks to therapeutic doses) can overcome minor allergic reactions (rash, fever), but most often sulfa-intolerant patients are now switched to a sulfa-free 5-ASA preparation (see below).
Drug interactions associated with sulfasalazine include inhibition of folic acid absorption (usually not clinically significant) and a 25% reduction in digoxin bioavailability. Sulfasalazine’s metabolism is slowed when cholestyramine or broad-spectrum antibiotics are used concurrently, an effect of uncertain clinical significance. Ferrous sulfate appears to have a similar effect on sulfasalazine, although iron absorption is not appreciably hindered; these drugs should not be taken at the same time. Because of the risk of neutropenia, any concurrent use with azathioprine or 6-mercaptopurine (6-MP) requires extreme caution and close monitoring.
Sulfapyridine-free 5-Aminosalicylate Agents. These agents were developed to provide a sulfapyridine-free means of delivering 5-ASA to affect bowel and in doing so eliminate most the adverse effects seen with sulfasalazine. The first developed was olsalazine, which is just two 5-ASA molecules bound by an azo bond that is cleaved by bacteria in the colon, releasing the active 5-ASA moiety. It is useful for achieving control of mild-to-moderate disease and for maintaining remissions. Diarrhea is the most common side effect, is dose related, and is diminished by increasing dose gradually and by giving the drug with meals.
Mesalamine is 5-ASA specially coated to produce delayed release. The oral tablet formulation (Asacol) dissolves at a pH of 7, the lumenal pH of the terminal ileum and colon. The methylcellulose capsule formulation (Pentasa) releases 5-ASA into the small bowel and the colon. In controlled trials, oral 5-ASA agents perform at least as well as sulfasalazine in the treatment of active mild-to-moderate ulcerative colitis and in maintaining remissions. Benefit appears to be dose related. Side effects are nil, but 5-ASA–related interstitial nephritis (seen in animal studies and case reports) is a concern with long-term high-dose use. Idiosyncratic reactions include pleuropericariditis, pancreatitis, and nephrotic syndrome. Those with very active disease should probably take mesalamine with meals if they note that the coated tablet is being passed undissolved, because taking the pill with food can slow transit and allow more time for dissolution.
Although these oral 5-ASA agents appear better tolerated than sulfasalazine, they are considerably more expensive. Nonetheless, patients who do not tolerate sulfasalazine deserve a trial of a sulfapyridine-free agent.
Topical 5-ASA enemas were also developed as an alternative to sulfasalazine and represent a reasonable option in patients with distal colitis. At maximum dose, they are more effective than hydrocortisone enemas and useful for persons with mild-to-moderate disease limited to the distal large bowel. Relapse is common with cessation of enema therapy; however, unlike hydrocortisone enemas, a maintenance program (alternate-day dosing) of topical 5-ASA therapy helps to maintain remission. The safety profile is excellent; adverse effects are nil other than idiosyncratic reactions, and the only common side effects are local itching and mild rectal irritation. Cost is considerably higher than that of a hydrocortisone enema. However, unlike steroid enemas, there is no concern about systemic steroid absorption.
Glucocorticosteroids. Steroids suppress the inflammatory process of ulcerative colitis and have important roles both as systemic agents in severe disease and as topical agents in disease confined to the rectosigmoid. When uveitis and colitis flare simultaneously, oral steroids are often effective for both. (In the absence of active colitis, the uveitis may be treated with topical steroids and mydriatics.) The best means of treating other systemic manifestations (erythema nodosum, pyoderma gangrenosum, oral aphthous ulcerations) is to control the underlying disease with systemic steroids. Patients with severe disease requiring daily steroids should receive calcium and vitamin D supplementation, because absorption may be impaired.
Systemic preparations are used in moderate-to-severe and severe disease, especially in markedly symptomatic patients with extensive bowel involvement. Those without systemic toxicity can be treated on an outpatient basis starting with the equivalent of 60 mg/d of prednisone. Patients too ill for oral therapy (i.e., those with vomiting, high fever, or signs of bowel distention) should be admitted to the hospital. Once symptoms lessen, steroids are gradually tapered over 4 to 8 weeks to the lowest dose that maintains control. Whenever possible, every effort should be made to taper and terminate systemic steroid therapy, both because only some ulcerative colitis patients benefit from chronic steroid use and because the adverse effects of chronic steroid therapy can be disabling. Alternate-day steroid administration often suffices after a flare-up has been brought under control and poses much less risk of steroid side effects. Newer corticosteroids with fewer systemic effects are under development. The best studied is a slow-release oral preparation of budesonide, which provides release in the colon with minimal systemic side effects.
Topical preparations are useful for disease confined to the rectosigmoid area. Hydrocortisone enemas as widely prescribed and effective, but prolonged high-dose use can lead to systemic steroid side effects (some hydrocortisone does get absorbed). The topically active steroid beclomethasone has been found equally efficacious to topical hydrocortisone but with less systemic absorption and no effect on serum cortisol levels; however, cost is greater.
Immunosuppressive Agents. Patients who require chronic high-dose steroid therapy might benefit from a trial of steroid-sparing immunosuppressive therapy with 6-MP or azathioprine. Because onset of action can be slow (up to 6 months), concurrent therapy with a second agent is necessary until the drug’s effect sets it. Side effects are less frequent than with systemic steroids, but these can be serious (see below). Intravenous administration of cyclosporine is sometimes used in very ill patients not responding to intravenous steroids. Results from controlled trials are variable and risk of toxicity is high.
Opiates are useful for providing symptomatic relief of diarrhea during acute phases of illness and chronic active colitis. They must be used with caution in acutely ill patients because of the risk of precipitating toxic dilatation. Diphenoxylate, codeine, tincture of opium, paregoric, and loperamide all limit the number of bowel movements. They are given before meals and at bedtime. Loperamide is among the most effective and least addicting but is considerably more expensive. Codeine is excellent for short-term use and superior to diphenoxylate in efficacy. Tincture of belladonna and other anticholinergics help to control cramps.
Psychological Support. Although psychological disturbances are more prevalent in patients with inflammatory bowel disease than in control subjects, there is little evidence that psychiatric disease is etiologically linked to the development of ulcerative colitis. However, recent data suggest a correlation between stress, immunologic dysfunction, and onset of symptoms. Formal psychotherapy directed at uncovering intrapsychic conflict has not proven useful, but a close, supportive, and empathetic patient–doctor relationship is invaluable to psychologically sustaining the patient through this illness (see below). Fears and worries about debility, surgery, colostomy, and body image contribute markedly to the psychosocial impairment and disability associated with this illness.
Surgery. Total colectomy offers the potential for complete cure of bowel disease and remission of most peripheral manifestations. As such, it represents an important therapeutic consideration, albeit a difficult one. Indications include high-grade dysplasia, suspected cancer, and unresponsiveness of bowel or systemic symptoms to maximal medical management. Patients with severe persistent disease requiring continuous high-dose corticosteroids that cannot be tapered after 6 to 12 months also warrant serious consideration for surgery, as do those with frequent severe relapses or complications from prolonged exposure to systemic steroids.
The traditional procedure is proctocolectomy with Brooke ileostomy. It has the advantage of being the fastest and safest procedure. The disadvantages of an ileostomy include incontinence, need to frequently empty an external ileostomy appliance, skin excoriations, and potential need for stomal revision (in about 10% to 20%). Total proctocolectomy with continent ileostomy (Koch pouch) creates a continent ileostomy that does not require wearing an appliance; it is best suited for those who desire control of stomal output. Park’s procedure (total colectomy, rectal mucosectomy, ileal reservoir, and ileoanal anastamosis) provides the opportunity to retain continence and avoid a stoma. Although there is likely to be some incontinence initially, this usually passes. About four to eight bowel movements per day are common, helped by chronic use of low-dose antidiarrheal therapy (e.g., loperamide). Potential complications include pelvic infection, strictures, small bowel obstruction, and “pouchitis.” Although complications occur in up to 30%, most patients are pleased with the procedure and its outcomes.
Regardless of the surgical procedure chosen, the morbidity of active disease and the threat of cancer must be weighed against the risks of major surgery. The mortality of elective colectomy is 1% to 3%, with most patients having no postoperative complications. Stoma revision is necessary in 10% to 20% of cases.
All patients with clinical or radiologic evidence of pancolitis of 7 or more years should be considered for colon cancer screening. At this point, cancer risk begins to rise substantially. Because the bowel cancer is often multicentric, the best method of screening is colonoscopy with multiple biopsies. Cancer screening should begin at 7 years with 3-year intervals until 20 years, when it should become more frequent. Worrisome findings include stricture and dysplasia. Mild dysplasia is an indication for repeat study in 3 months. Severe dysplasia and stricture formation require consideration of colectomy; the risk of cancer is very high in the presence of such findings.
Most patients can be treated on an outpatient basis by judicious use of medications and careful follow-up. A strong working alliance between the patient and primary physician is essential, because the disease is chronic, relapsing, and incurable.
Diet. Adequate nutrition is critical to the promotion of healing. Sufficient protein and calories must be provided but in a manner that limits the stress put on an inflamed and often strictured bowel. Patients with cramps and diarrhea should have the fiber content of their diet reduced; those with steatorrhea will benefit from a decrease in fat intake to less than 80 g/d. An empiric trial of restricting milk products may terminate diarrhea due to lactase deficiency, which often accompanies the illness. More severely ill patients require partial bowel rest, which removes the stimulus that food has on bowel motility and secretion. Elemental diet preparations (e.g., Magnacal, Ensure, Sustacal, Isocal) have been found to induce remission, improve symptoms, and decrease disease activity in patients with acute disease. They are convenient and usually well tolerated sources of the extra nutrition needed during exacerbations. Total parenteral nutrition should be used in patients whose oral intake is not adequate or in whom surgery is indicated.
Vitamin and mineral deficiencies are common and must be corrected for proper healing and avoidance of such complications as anemia and bone disease. Folic acid supplementation is particularly important in patients taking sulfasalazine, which impairs its absorption. Patients who have had ileal surgery may need extra vitamin B12. Vitamin D levels are likely to be low when intake is poor or steatorrhea is a problem. An oral supplement of 4,000 IU or more usually suffices. Most vitamin and mineral deficiencies can be overcome by taking a multiple vitamin containing about five times the normal daily vitamin requirements and such minerals as iron, calcium, magnesium, and zinc.
Antidiarrheal Agents. The use of these agents in Crohn’s disease is similar to that in ulcerative colitis (see above). The risks include addiction and exacerbation of obstructive symptoms.
Sulfasalazine and Other 5-Aminosalicylate Preparations. The National Cooperative Crohn’s Disease Study demonstrated modest efficacy of sulfasalazine in patients with disease of the colon and no benefit in those with disease limited to the small bowel. Doses of 2 to 4 g/d are used to treat acute exacerbations of abdominal pain and diarrhea in patients with colonic involvement. Improvement typically occurs within 4 to 8 weeks. Patients who respond to sulfasalazine but experience an adverse effect attributable to the sulfa moiety are candidates for a sulfa-free 5-ASA preparation (e.g., mesalamine or olsalazine). Olsalazine delivers 5-ASA exclusively to the colon, making it useful only for those with disease confined to the large bowel. Mesalamine tablets (Asacol) are formulated to deliver 5-ASA to the cecum and large bowel; mesalamine capsules (Pentasa) release 5-ASA to the entire small bowel and colon. Because they are costly, the 5-ASA preparations should not be used before sulfasalazine for treatment of active disease. Sulfasalazine does not sustain remissions, but meta-analytic data and subsequent controlled trials suggest that mesalamine may reduce risk of recurrence by up to 40%, with benefit most notable for disease of the small bowel and for sustaining remission and after surgery. Nonetheless, the safety and efficacy of routine long-term prophylactic use of mesalamine in Crohn’s disease is not established. Combination of sulfasalazine with corticosteroids has not been shown to have a steroid-sparing effect or allow more rapid tapering of steroids once a remission has been induced.
The education and support of the patient and the family are essential. Fears abound when patients are told they have ulcerative colitis or Crohn’s disease. These diagnoses conjure up images of colostomy, recurrent hospitalizations, invalidism, and social isolation. It is important to emphasize that the vast majority of patients lead fully functional lives and many obtain satisfactory control of their disease through medical therapy.
Because these are chronic diseases that affect young adults, the questions of conception, pregnancy, and childbearing will arise. Although there is some familial pattern to the occurrences of the inflammatory bowel diseases, transmission is not purely genetic, and there is ample evidence that when the disease is in remission, fertility is essentially normal, and healthy full-term infants can be delivered. However, conception might be a problem when the male patient is taking sulfasalazine (see above).
The issue of cancer risk in patients with long-standing and extensive ulcerative colitis can be addressed directly and clearly, reassuring those with minimal disease that their risk is no greater than that of the general population. Even those with extensive disease appreciate knowing the magnitude of the risk.
The primary physician can do much to prepare the patient who requires colectomy and subsequent ileostomy. Thorough patient education combined with a caring approach that includes a willingness to listen to concerns and fears is invaluable and greatly appreciated. Many patients have fears and anxieties that they will not discuss unless they are broached by the physician. Also helpful in preparation for ileostomy is to have an ileostomy patient of the same age and sex discuss the procedure and its consequences with the surgical candidate. Seeing that one can go on to lead a fully active life is comforting. Where available, a local association of ostomates is a valuable resource. Finally, the more widespread use of ileoanal anastomosis in carefully selected patients may increase the acceptability of surgery.
Many patients can be taught to adjust their medication within a prearranged set of guidelines and limits. Dosages of sulfasalazine to be used for mild exacerbations can be specified and extra supplies of medication can be provided, allowing the patient to play an active role in his or her care and ensuring prompt treatment of a flare-up. Antidiarrheal agents can also be provided for as-needed use but only to reliable patients who are not likely to abuse them. Patients should be instructed to call if fever develops, diarrhea worsens, bleeding occurs, or abdominal pain becomes marked.
The need for a steady exchange of information with the patient, including careful explanation of procedures and therapies, and the importance of close follow-up and availability cannot be overemphasized. Attentiveness and responsiveness alleviate much of the fear and worry that accompany inflammatory bowel disease and support the development of an effective therapeutic alliance. Additional information and support is available to the patient from the local Chapter of the National Ileitis and Colitis Foundation.
General Measures