Recent findings indicate that approximately 1 of 133 Americans have CD.¹ Advances in recognizing its multisystem effects and new diagnostic tests have led to the realization that CD is much more common than previously thought.² CD is now recognized as a common systemic condition that if not appropriately diagnosed and managed, can produce serious health problems. With knowledge about the risks factors for CD and CD’s clinical presentation, nurses can help prevent those problems.

CD and some of its consequences were known in the 19th century, but it was not until the middle of the 20th century that it was observed that patients with CD who eliminated wheat products from their diet experienced a dramatic improvement in their symptoms. CD, also known as gluten-sensitive enteropathy or celiac sprue, is an autoimmune disease that occurs in genetically predisposed people who have an immune response to gluten, the major protein constituent of grains such as wheat, barley, and rye.³ When people with CD eat foods or use products that contain gluten, their immune systems respond by causing intestinal inflammation. Villi in the small intestine, tiny hair-like projections that absorb nutrients, become the target of damage. Without healthy villi, people with CD become malnourished, regardless of their food intake.¹ The Marsh classification is used to describe the progression of damage to the intestinal mucosa, ranging from Marsh Type 1 (least involvement) to Marsh Type 4 — a rare occurrence indicating irreversible injury caused by chronic inflammation.⁴

There is a strong genetic predisposition to CD, with increased risk occurring in people with specific human leukocyte antigen (HLA) markers. Glutens interact with these HLA markers to activate an abnormal immune response that produces tissue damage.^1,2 CD is classified as an autoimmune disorder because the body’s immune system causes the damage. It is also considered a disease of malabsorption because nutrients are not absorbed.¹

Although Americans are not routinely screened for CD, people who have a higher than normal risk for CD can benefit from screening. For example, between 5% and 15% of first-degree relatives — parents, siblings, or children — of a person who has biopsy-proven CD also test positive for the disease.¹

People with other autoimmune disorders, such as Type 1 diabetes mellitus, thyroid disease, systemic lupus erythematosus, liver disease, collagen vascular diseases, rheumatoid arthritis, and Sjogren’s syndrome (an autoimmune disorder that causes dryness of the eyes and mouth), have an increased risk of developing CD.¹ Between 5% and 10% of people with Down syndrome are diagnosed with CD.¹

Traditionally, CD was viewed as a GI disorder that began in infancy. Now it is known that CD can appear at any time in life, producing intestinal damage that eventually can affect the skin, liver, joints, uterus, brain, and other organs. With the delay of introducing wheat into infant diets, a CD diagnosis is now increasingly made in older children and adults. Environmental factors, such as the amount and timing of gluten consumption, influence CD symptoms and disease progression.

If CD is undiagnosed or inappropriately treated, long-term complications such as iron-deficiency anemia, osteoporosis, vitamin and mineral deficiencies, and GI malignancies can occur.² Unfortunately, the longer a person with CD is undiagnosed or untreated, the greater the chance of developing complications.² Studies report an increased risk of adenocarcinoma of the small intestine and an increased risk of other GI malignancies, such as intestinal lymphoma and cancer of the pharynx and esophagus.⁵ Enteropathy-associated T-cell lymphoma is an aggressive form of non-Hodgkin’s lymphoma of the upper small intestine that is specifically associated with CD.⁵ It is thought that people with CD have an increased cancer risk because of the increased permeability of damaged intestinal walls to carcinogens, chronic inflammation and antigenic stimulation, and release of proinflammatory cytokines.⁵ GI cancer risk falls when people with CD follow a gluten-free diet. Malnutrition from CD is a particularly serious problem for children, who need adequate nutrition for normal growth and development.

What to be alert for

The clinical presentation of CD varies greatly, ranging from people with no symptoms to those who have severe malnutrition.6 Recognizing CD is challenging because some symptoms are similar to those of other diseases, such as irritable bowel syndrome, iron-deficiency anemia due to menstrual blood loss, Crohn’s disease, diverticulitis, and chronic fatigue syndrome.^1,2 The length of breastfeeding, the age at which a person starts eating foods containing gluten, and the amount of gluten in the diet are thought to be factors that influence when and how CD appears. Some studies have shown that the longer a person was breastfed, the later CD symptoms appear and the more atypical the symptoms are.^1,7

People with CD can exhibit classic intestinal and extraintestinal symptoms. Classic intestinal symptoms include abdominal cramping, pain, and distention; increased frequency of bowel movements; and malabsorption.⁶ In contrast, the now common atypical, or extraintestinal, signs of CD include anemia, fatigue, malaise, delayed puberty, infertility, recurrent fetal loss, osteoporosis, vitamin deficiencies, protein calorie malnutrition, neurological problems, and dental enamel weakness.^1,6 Iron-deficiency anemia is the most common laboratory abnormality found in people with CD.² Iron is absorbed in the proximal small intestine, where CD damage is most prominent. As a result, iron malabsorption is common.² Sensitive new serologic diagnostic tests are identifying many people with nonclassical or extraintestinal symptoms.⁷

Dermatitis herpetiformis (DH) is a skin manifestation of CD. Consumption of gluten triggers an immune response that results in the deposit of immunoglobulin A under the top layer of the skin, producing skin blisters and an intensely itchy rash on the extensor surfaces of the extremities. Not all people with CD develop DH, and about 20% of those with DH have intestinal symptoms of CD.¹ DH rash symptoms can be controlled with medications such as dapsone; however, medication does not treat intestinal damage. People being treated with dapsone to reduce skin rash symptoms must also maintain a gluten-free diet.¹

Clinical presentation tends to differ by age groups. Infants and children with CD often have classic GI symptoms, such as diarrhea, abdominal distention, or signs of malnutrition, such as failure to thrive, short stature, or developmental delay.² Irritability is one of the most common symptoms of CD in children.³ Adults with CD may have both GI and extraintestinal symptoms. Because the small intestine has considerable functional reserve, some people have few or no symptoms and often no evidence of malabsorption.⁴ Those without symptoms are still at risk of developing CD complications.¹

CD is diagnosed by positive serologic testing, small bowel biopsy showing atrophy of the villi, and symptom abatement on a gluten-free diet. All diagnostic tests must be done while the person is consuming glutens.^1,2 The first step in diagnosis is serologic testing. Immunoglobulin A, IgA antiendomysial antibody, and IgA antitissue transglutaminase antibody tests are used to help diagnose CD.^1,2 Small bowel biopsies are done in people who have positive CD antibody tests. CD symptoms can occur in all age groups. Symptoms are varied and can often mimic other intestinal disorders.

No more gluten products — ever

The basis of CD treatment is lifelong adherence to a gluten-free diet. Education about the disease process, consultation with a skilled dietitian, and access to advocacy groups help those with CD learn self-care skills and coping strategies. Eliminating gluten from the diet stops tissue damage, allows the intestinal mucosa to heal, and reverses malabsorption.^1,2 The small intestine is usually completely healed within six months in children and younger adults and within two years in older adults.^1,2 Depending on a person’s age at diagnosis, problems such as delayed growth may not be completely reversible.¹ A gluten-free diet requires eliminating wheat, rye, and barley. Oat products are also excluded from a CD diet in the United States because of concerns about the potential contamination of commercially prepared oats.⁸

Adhering to a strict gluten-free diet is a tremendous challenge for several reasons. First, wheat and wheat-based products are American dietary staples.⁹ Common food items such as hot and cold cereals, bread, bagels, pancakes and waffles, pizza, hotdog and hamburger buns, pasta, cookies, crackers, and snack bars all contain gluten. Less obvious items that often contain gluten include baked beans, communion wafers, meatloaf, soups, and salad dressings.⁹ Second, increasingly hectic lifestyles have resulted in more meals eaten away from home and the increased use of packaged convenience foods, which often contain wheat.⁹ Third, gluten is an unexpected ingredient in many foods. Potential harmful ingredients in food products include unidentified or modified food starch, hydrolyzed vegetable or plant protein, texturized vegetable protein, binders, fillers, extenders, and malt.¹⁰

Despite these dietary restrictions, people with CD can eat a well-balanced diet with a variety of foods. Plain meat, fish, rice, fruits, and vegetables form the basis of a gluten-free diet. People with CD can eat all fresh, frozen, or canned vegetables prepared with allowed ingredients but should avoid creamed or breaded vegetables and canned baked beans.1 Potato, rice, soy, and other wheat flour substitutes can be used. People with CD can also buy gluten-free bread, pasta, and other products from organic food or specialty stores.¹ Increasingly, gluten-free products are available from regular grocery stores, and some restaurants offer gluten-free menu choices. The Gluten Intolerance Group (GIG) publishes dining-out suggestions in several languages and offers tips on avoiding gluten when eating away from home. GIG recently posted a list of restaurants in the Los Angeles area that offer gluten-free menu choices on their website. The organization plans to add more information on restaurants across the country that partner with CD customers.

Some people with CD show no improvement on a gluten-free diet. One study found that 51% of people with CD who were following a gluten-free diet continued to have symptoms because of their inadvertent consumption of gluten. The most common causes were a result of eating out or eating corn or rice cereals with barley malt flavoring.¹¹ Gluten is also an inactive ingredient in some vitamin and mineral preparations and herbal supplements and in many common medications. Products most people use every day, such as stamp and envelope adhesives, contain gluten. Having a health provider add “as ordered if gluten-free” or “provide a gluten-free equivalent” to a prescription alerts the pharmacist to supply a product that is gluten-free.⁸

A gluten-free diet is often low in vitamins, minerals, and fiber. In addition, few gluten-free products are enriched or fortified, adding to the risk of nutritional deficiencies.⁸ Traditionally, rice, corn, and potatoes were substituted for gluten-containing grains. Today, a number of nutrient-dense grains, seeds, legumes, and nut flours offer increased variety and higher nutritional quality.⁸

CD isn’t easy

Following a lifelong gluten-free diet has a major impact on quality of life. One study showed that 86% of people with CD reported that the diet had a negative impact on their ability to eat out, 82% reported difficulties with traveling, 67% said the diet interfered with family life, and 41% reported a negative impact on work and career.¹² To help those with CD follow an effective treatment plan, healthcare professionals must understand the emotional and psychological impact of the disease and the diet, as well as the complex quality-of-life issues patients and their families face every day.¹²

Nurses can help those with CD by suggesting resources and advocacy groups.  Nurses should caution people with CD that the Internet is unregulated and may contain inaccurate or misleading information about CD. Patients should be advised that they should look to physicians and dietitians who specialize in CD for an accurate diagnosis and appropriate treatment.

Compliance barriers include the poor taste of some gluten-free foods and confusing food labeling. Food labels on prepared food often don’t indicate whether an item contains wheat or was processed with a gluten-containing product. The 2004 Food Allergen Labeling and Consumer Protection Act requires food manufacturers to state clearly on the label if a product contains any of the top eight food allergens: milk, eggs, fish, crustaceous shellfish, tree nuts, peanuts, soybeans, and wheat.¹³ The law also requires the FDA to issue rules defining the term gluten-free and permitting the term on food labeling.

Because CD is often atypical or even clinically silent, many cases go undiagnosed, exposing people with CD to the risk of long-term complications. Clinicians in a variety of specialties should have a high index of suspicion of the potential for celiac disease, especially for people in high-risk groups.¹⁴

The National Institute of Diabetes and Digestive and Kidney Diseases is the primary National Institutes of Health organization for CD research. The NIH and NIDDK, in association with the Centers for Disease Control and Prevention, are trying to raise awareness of CD among physicians, nurses, dietitians, and the public. Part of this initiative is to develop educational materials for medical training that will lead to earlier diagnosis and treatment of celiac disease. Nurses can help with this effort by suggesting that people who have increased CD risk factors talk with their healthcare providers about screening and diagnosis.