A Guide for Children and Their Families
The following information is intended solely for educational purposes and not as medical advice. It is not a substitute for care by a trained medical provider. For advice about celiac disease, consult a physician.
Prepared by the Joint Ad Hoc Committee on Patient Information of The Patient Care Committees of the Association of Pediatric Gastroenterology and Nutrition Nurses (APGNN) and the North American Society for Pediatric Gastroenterology and Nutrition (NASPGN)
Stuart S. Kaufman, M.D., Robert A. Cannon, M.D., Laura Ernst Beerman, R.D., Alan M. Lake, M.D., Rosemary J. Young, B.S.N.
Illustrations by Kimberly J. Whitlow
Acknowledgements to Dr. Leon Rottmann and Elaine Hartsook for continued encouragement and support of this project.
Edited for electronic transmission by Richard B. Colletti, M.D. (11/95)
History of Celiac Disease
Glossary of Terms
Celiac Disease: What it is, What Causes it, How it Affects People
Diagnosis of Celiac Disease
Treatment of Celiac Disease -- For Parents
Treatment of Celiac Disease -- For Children
Long-Term Problems Associated with Celiac Disease
What's Gluten-Free and What's Not
HISTORY OF CELIAC DISEASE
In 250 A.D., Galen, a Roman physician, described childhood and adult celiac disease. In 1888, Samuel Gee described celiac disease in Britain and the role of diet in its control. Dicke in the Netherlands during 1950 suggested that certain dietary cereal grains were harmful to children with celiac disease. He also noted that persons previously diagnosed with celiac disease improved during World War II when grain products were in short supply. When grains became more plentiful after the war, the incidence of celiac disease returned to its pre-war levels. In 1954, Paulley first described the intestinal lesion, villus atrophy, in patients with celiac disease. Cyrus L. Rubin and co-workers in the United States demonstrated in 1958 that celiac disease in children and adults were identical disorders. In the 1980's, Michael Marsh and co- workers in Manchester, England, emphasized the role of the immune system in causing intestinal injury in celiac disease.
Antibodies: Substances made by the immune system that recognize and combine with foreign materials (antigens) that have gotten into the body. Antibodies are one part of the body's natural defense against invasion by germs and other potentially harmful substances.
Antigen: A substance, usually a protein rather than carbohydrate or fat, that is foreign to the body and triggers a reaction that destroys or eliminates that substance. Antigens may be living things such as bacteria or molds or products of living things such as poisons, animal hairs, pollens, or foods.
Biopsy:A A small piece of tissue, such as from the inside lining of the intestine, that has been removed to look for diseases and other problems.
Cancer: A mass of abnormal cells that can develop in any place in the body and which eventually interferes with normal body function.
Carbohydrate: A term for sugars and starches that are used by the body mainly as a source of energy. Food sources include grains (bread and cereals), pasta, potatoes, vegetables, fruits, and fruit juices.
Celiac disease (Sprue): A disease in which certain proteins found in grains such as wheat, barley, rye, and oats, act as antigens. One of these proteins is called gliadin. The resulting reaction by the body against gliadin, which includes production of anti-gliadin antibodies, eventually damages the inside lining of the intestine (mucosa). Damage to the intestinal mucosa interferes with digestion and absorption of food into the system, allowing undigested food to pass through the body into the stools (malabsorption).
Dermatitis herpetiformis: A red, bumpy, itchy skin rash that sometimes occurs in adults but rarely in children with celiac disease. It usually appears on both sides of the body, with knees, elbows, and buttocks most commonly affected.
Endoscope: A flexible tube that can be passed through the mouth down into the stomach, small, and large intestine. It is used to obtain biopsies to look for celiac disease and other disorders.
Fat: A type of food found primarily in oils, shortening, butter/margarine, nuts, meats, and dairy products. Fats are needed by the body for growth and can also be used for energy.
Gliadin: A protein found in wheat gluten. In people with celiac disease, gliadin and similar proteins in rye, oats, and barley work as antigens. The body reacts against these antigens, damaging the inside lining of the intestine (mucosa).
Gluten: A generic term for the water-insoluble proteins found in all cereal grains. Only glutens found in wheat, rye, oats, and barley are known to damage the intestine of those with celiac disease.
Immune system: The body's defense mechanism against foreign substances that includes antibodies, cells making antibodies, lymph glands, and white blood cells.
Inflammation: Damage to the body that can result from an attack by foreign substances (antigens). Inflammation continues until the foreign substance is destroyed or removed by the immune system.
Malabsorption: Failure of the intestine to break down food and get it into the body. Malabsorption causes food to be lost into stools, usually resulting in diarrhea.
Mucosa: The inside lining of the intestinal tract that absorbs food into the body.
Protein: A type of food found primarily in beef, pork, fish, poultry, nuts, legumes, and dairy products. Proteins are used for healing and to build muscle and bone.
Villus (plural = Villi): The "finger-like" pattern of intestinal mucosa that is required for absorption of food.
Villus atrophy: Damage (inflammation) to the intestinal mucosa in which the finger-like projections (villi) are "shaved-off". Villus atrophy occurs in people with celiac disease when they consume foods containing gluten (gliadin).
CELIAC DISEASE: WHAT IT IS, WHAT CAUSES IT, AND HOW IT AFFECTS PEOPLE
Celiac disease (sometimes known as sprue or gluten-sensitive enteropathy) is an illness in which the inside lining of the small intestine (mucosa) is damaged after eating wheat, rye, oats, or barley. Just how these grains, which are harmless to most people, damage the intestinal mucosa in people with celiac disease remains uncertain. At present, most indications are that gluten proteins trigger an immune reaction that results in damage to the intestinal mucosa. Thus, the intestine may be injured as a kind of "innocent bystander". This immune reaction may be similar to the "rejection" of transplanted organs (for example, kidney or heart) by the body. Because the immune system is controlled partly by heredity, it is not surprising that celiac disease runs in families. It is generally thought that close relatives of a person with celiac disease have about a 5-10% chance of developing celiac disease themselves.
The development of celiac disease requires a genetically predisposed person who is eating wheat, rye, oats, or barley. Even if these two factors are present, celiac disease may not develop until a "trigger factor" starts the abnormal immune system response. Sometimes, a viral illness appears to be that "trigger." After exposure to gluten, intestinal damage may develop within a few months or may be postponed for several years. Removal of gluten from the diet of a person with celiac disease permits the intestinal mucosa to heal.
As indicated, the normal intestinal mucosa is not smooth. Rather, it is organized into a series of ridges or leaf-like folds, termed villi, to increase the area over which digestion and absorption take place. Many digestive enzymes are present on the surfaces of villi. Exposure of the intestinal mucosa to gluten-containing proteins in a person with celiac disease destroys the villi along with the digestive enzymes. The resulting damaged mucosa is thus termed "flat." As a result of the loss of digestive enzymes and reduction in surface area for absorption, foods of all types tend to pass through the entire intestinal tract undigested.
Usually, passage of undigested food through the intestinal tract causes diarrhea. Because the diarrhea of a person with celiac disease usually contains a large amount of undigested fat, bowel movements tend to be bulky, gray in color, and exceptionally foul-smelling. The stools may even float in the toilet bowl and have obvious droplets of fat on them. Loss of large quantities of undigested food into the stools usually results in weight loss. Both body fat and muscle are lost. This results in a bloated abdomen, pencil-thin arms and legs, and flat buttocks. Although children with celiac disease do not usually have obvious abdominal pain, they tend to become listless and irritable. Furthermore, they tend to have poor appetites, which is quite surprising considering they appear as if they have been starved.
Children with celiac disease grow below their potential and sometimes quit growing completely. Stunting of growth may be the only symptom of celiac disease. This group of celiac children may not have diarrhea.
Children with celiac disease tend to have certain blood test abnormalities. In particular, a low red cell count (anemia) is common as a result of iron and vitamin deficiency. Not only do children with untreated celiac disease tend to be quite short, x- rays of their bones show a lack of calcium and an overall slowing of bone growth. Enamelization of teeth may be impaired. A gluten- sensitive skin rash (dermatitis herpetiformis) can be observed in adults with celiac disease. This rash appears as painful, itchy, raised red blotches on the elbows, knees and buttocks. However, this skin rash is uncommon in children.
Total withdrawal of gluten from the diet results in a disappearance of the symptoms of celiac disease. Initially, irritability goes away and appetite improves, usually within a matter of days following withdrawal of dietary gluten. Over a matter of weeks, weight gain resumes and diarrhea decreases. Several months later, growth in height returns as abdominal swelling disappears. Blood tests eventually come back to normal, too. These improvements in overall health and appearance of an affected person occur long before there has been healing of the intestinal damage.
Diagnosis of celiac disease requires a small intestinal biopsy, which demonstrates the typical pattern of intestinal mucosal injury (villus atrophy), and a clear-cut disappearance of symptoms and restoration of growth and weight gain occurring over a matter of weeks to months on a gluten-free diet.
Intestinal biopsy can be done in either of two ways depending upon factors that include the age of the person being evaluated and the tradition of the evaluating institution. Sometimes, a small flexible tube containing a tiny knife at the tip can be passed through the mouth down into the stomach and then directed into the small intestine through the use of an x-ray.
Alternatively, an endoscope, through which the doctor can see the inside of the digestive tract, can be passed through the mouth and then beyond the stomach without need for x-ray to obtain biopsies in the small intestine.
The biopsy specimens are processed and viewed under the microscope to identify or exclude the typical mucosal damage of celiac disease.
Obtaining intestinal biopsies by either method requires some form of sedation, and it can be unpleasant and always entails some risk. For this reason, various blood tests have been developed over the years in an attempt to help identify persons who are most likely to have celiac disease and, thus, who should undergo biopsy. These include blood levels of antibodies against the offending dietary protein (anti-gliadin antibodies), and against the damaged intestinal tissue (anti-reticulin antibodies and anti-endomysial antibodies). These antibodies tend to be present in persons with celiac disease who are consuming gluten. Conversely, amounts of these antibodies fall once a gluten-free diet has begun.
It is important to emphasize that blood antibody tests are not an absolute guide to the presence or absence of celiac disease. This is because the absence of any of these antibodies does not absolutely guarantee that a person does not have celiac disease. On the other hand, sometimes people who have these antibodies do not have celiac disease and do not need a gluten-free diet. In reality, the diagnosis of celiac disease includes:
- A suspicion of celiac disease based on symptoms, physical appearance, delayed growth, and abnormal blood tests.
- A small intestinal biopsy which shows damage to the villi and infiltration of immune system markers typical of celiac disease.
- DEFINITE improvement with a gluten-free diet.
There is no substitute for small intestinal biopsy in deciding who should or should not be on a gluten-free diet. The gluten-free diet should be continued for life after celiac disease is diagnosed. There is NEVER any chance that the disease, once confirmed, will disappear and allow safe reintroduction of gluten- containing foods sometime in the future.
On occasion, there may be some uncertainty about the correctness of the initial diagnosis. It is true that disorders other than celiac disease can cause a similar type of villus atrophy, particularly in children under the age of 2 years. When this situation arises, a second intestinal biopsy may be recommended following a period of prolonged gluten restriction in order to demonstrate healing. Thereafter, gluten may be temporarily reintroduced and a third biopsy performed weeks to months later in order to see if re-exposure of the intestine to gluten results in a return of the villus atrophy. If atrophy returns, then celiac disease is confirmed and the gluten-free diet should be resumed, this time for life.
TREATMENT OF CELIAC DISEASE: FOR PARENTS
When a child with undiagnosed celiac disease has become very sick with severe diarrhea and malnutrition, corticosteroids (such as prednisone) and intravenous ("I.V.") fluids may be temporarily given while the gluten-free diet is taking hold. Because celiac disease is probably diagnosed sooner now than in the past, corticosteroid therapy is rarely used. Thus, in the majority of instances, the only treatment necessary for celiac disease is absolute avoidance of gluten-containing foods.
The widespread use of gluten-containing grains in Western cultures makes adapting to a gluten-free diet challenging. For parents used to preparing gluten-containing meals, searching for and cooking with gluten-free products may seem strange at first. Changing cooking habits will be made easier by using gluten-free recipes and food products. Soon after the gluten-free diet is begun, the obvious benefits to the child with celiac disease results in its enthusiastic acceptance. As the child with celiac disease matures, that period of ill-health may be forgotten. The understandable desire for conformity, particularly during adolescence, may make the child reject the diet. Unfortunately, in older children, symptoms may not appear right away although intestinal damage is returning. This delay in return of symptoms may give a false sense of security. The child thinks that he or she has recovered from celiac disease when, in fact, they have not. Thus, it is always important for your child not to be made to feel abnormal and a nuisance to the rest of the family, which can often be quite unintentional. Family support is invaluable in facilitating acceptance of the diet.
Of course, new ideas and practices concerning celiac disease are being considered all of the time. If you hear something different from what you have been previously told or thought about celiac disease, you should discuss this concern with a health-care professional knowledgeable about celiac disease.
WHAT'S GLUTEN-FREE AND WHAT'S NOT
Grains, Breads, Cereals, Pastas
Rice, corn, soy, arrowroot, potato, and tapioca-containing products. Breads may contain flour prepared from white or brown rice, potato, tapioca, arrowroot, pea, corn, or bean. Cereals include those from corn meal, millet, buckwheat, hominy, puffed rice, crisp rice, and cream of rice. Malt or malt flavoring derived specifically from corn. Pasta from rice, corn, and/or beans.
Fresh, frozen, dried, or canned products whose labels indicate they are free of thickening agents. (Thickening agents often contain wheat flour).
Fresh, frozen, dried, or canned fruits.
Fresh, frozen, and canned meats.
All aged hard cheeses. Pasteurized processed cheeses including cottage cheese and cream cheese, and also ice cream that is free of gluten stabilizers. Most children with celiac disease tolerate milk sugar (lactose)-containing milk and yogurt soon after starting a gluten-free diet.
Many but not all salad dressings are gluten-free. Apple, wine and rice vinegars are generally acceptable.
Drinks and Juices
Freshly brewed coffee, tea, chocolate made from powdered cocoa, carbonated drinks, and juices made from fresh fruit.
Condiments and Additives
Soy sauce that does not contain wheat or barley. There is no evidence that FDA-approved food colorings or monosodium glutamate (MSG) are harmful to persons with celiac disease. Hydrolyzed or textured soy and corn vegetable protein. Corn malt. Starch (raw or modified from arrowroot, corn or maize, potato, and tapioca). Vegetable gum from carob, locust bean, cellulose gum, guar gum, gum arabic, gum acacia, gum tragacanth, xanthan gum.
FOODS NOT ALLOWED:
Grains, Breads, Cereals, Pastas
Anything made with or containing wheat, barley, rye, oats, spelt and kamut. Breads or cereals containing wheat starch contain small amounts of gluten; as do cereals and crackers containing wheat and oat bran, graham, wheat germ, and bulgar. Cereals or breads containing malt flavoring of unspecified origin. Regular spaghetti, macaroni, and noodles. Most packaged rice mixes such as Rice-a-roni.
Products containing thickening agents which may utilize food starches and stabilizers with gluten. (Thickening agents often contain wheat flour).
As with vegetables, avoid prepared fruits containing thickening agents found in fruit pie fillings.
Prepared meats including luncheon meats, sausages, and canned meats containing grain and starch fillers with gluten. Self-basting turkey and other fowl often contain fillers with gluten. Fresh ground meats containing "Oattrim"or "LeanMaker".
Cheese foods including spreads, soft cheeses, and dips often contain gluten. Some ice creams may contain gluten ice cream stabilizers.
Salad dressings containing grain vinegars including distilled, white vinegar or if the type of vinegar is not stated. Some contain emulsifiers and stabilizers with gluten.
Drinks and Juices
Some brands of flavored coffee, herbal tea, and instant cocoa mixes, including malted milk. Grain-derived drinks including Postum and Ovaltine.
Condiments and Additives
Many soy sauces contain gluten. Products with grain vinegar including catsup and mustard. Soups or broths containing bouillon. Unspecified texturized or hydrolyzed vegetable protein, vegetable gum from oats and any other product containing an unspecified flour or cereal additive. Barley malt. Wheat starch. Caramel candy may contain gluten. Read labels on margarine to check for flour additives. Flavorings made with alcohol. Some spray coatings for "non-stick" uses have unspecified ingredients added.
When using any commercial food product, the list of ingredients must be read carefully. Although ingredients are listed in order of decreasing content, any product containing even the smallest amount of gluten must be avoided. Any ingredient of unspecified grain origin should be assumed to contain gluten. Food manufacturers are generally willing to provide additional information about their products beyond what is listed on the label. Most labels have addresses of the manufacturers and many even have a toll-free telephone number. Similarly, some restaurants have ingredient lists for their products, either posted within the restaurant or available upon request.
Medications must also be checked. Fillers and excipients can be wheat-based. Example: Cough medicines often contain alcohol which could be derived from grain.
CELIAC DISEASE: FOR THE CHILD
You have been sick and not growing well because you have celiac disease. This means that the inside of your intestine is sore and does not digest food the right way.
Your sickness is caused by certain kinds of foods when they touch the inside of your intestine. Foods which harm your intestine are grains, like wheat and oats. These grains are in breads and cereals. Other foods may have them, too. You'll need help to learn which foods are safe to eat. The foods that you will eat that do not hurt your system are special and should make you feel better very soon.
There are lots of good foods for you to enjoy. They are made with special things, like rice flour. With special cookbooks, you can help your mom or dad make pizza, bread, cookies, and cakes. There are many crackers and cereals you can eat, too. Even spaghetti and macaroni and cheese can be made with rice or corn noodles.
Celiac disease will last forever. It may be hard for you to eat these special foods all the time. You may see a food or something to drink that you know you should not eat. Eating a wrong food will make your intestines hurt on the inside, even though you may not feel it right away. After a while you WILL start to feel sick.
When you are eating over at a friend's house, make sure someone talks to your friend's mom first. At school, you may bring your lunch from home to stay on the special diet. If someone talks to the school dietitian, then you may be able to get your lunch at school. You should talk to your friends about your special diet and even let them eat some of your special foods. Soon, you will be able to read labels on foods and know how to make good choices. Your good health depends on you!
LONG-TERM PROBLEMS ASSOCIATED WITH CELIAC DISEASE
There are two basic types of potential long-term problems associated with celiac disease. These include: 1) those problems that can happen even if one maintains a gluten-free diet religiously, and 2) those problems that occur as a direct result of continuing to consume gluten. Certain kinds of non-intestinal disorders are more common in people with celiac disease. These disorders include diabetes mellitus and possibly other hormonal problems, such as hypothyroidism. The increased chance of developing these non- intestinal problems probably relates to the same hereditary factors that predispose a person to develop celiac disease. The appearance of these disorders is not prevented by a gluten-free diet. Certain other disorders, some potentially health-threatening, seem to result from continued consumption of gluten with ongoing intestinal damage. In particular, there is an increased chance for intestinal cancer to develop in persons with celiac disease. Because intestinal cancer usually develops in older persons, it is not entirely certain that long-term consumption of a gluten-free diet will totally eliminate the chance for these kinds of tumors to develop. However, current indications and common sense suggest that this would be the case.
More information concerning celiac disease can be obtained from several organizations. These include:
- Gluten Intolerance Group of North America
P.O. Box 23053
Seattle, WA 98102-0353
Publishes quarterly newsletter. Provides fact sheet on celiac disease. Cookbooks, other diet instructions, and videotapes are available. Affiliate groups are located throughout the country. Sells xanthan gum for stabilization of home-made brown and white rice flour yeast bread.
- Celiac Sprue Association/ United States of America, Inc.
P.O. Box 31700
Omaha, NE 68131-0700
Publishes newsletter. cookbooks and other information of interest are available.
- Midwest Gluten Intolerance
8201 - 158th Lane N.W.
Anoka, MN 55303
Publishes quarterly newsletter. Provides information on recipes, gluten-free foods and support group meetings.
- American Dietetic Association
(800) 877-1600 ext. 5000
A recipe book available by mail order. "Gluten Intolerance" is a resource including recipes.
- Canadian Celiac Association
6519 B Mississauga Rd.
CANADA L5N 1A6
- American Celiac Society/Dietary Support Coalition
58 Musano Ct.
West Orange, NJ 07052
- Celiac Disease Foundation
P.O. Box 1265
Studio City, CA 91614-0265
- Me and The Right Food Choices (Ages 5-7 years) A coloring activity book about food choices for celiac disease. To inquire, contact Gluten Intolerance Group office (206) 325-6980
- The Gluten-Free Gourmet, by Bette Hagman
- More from the Gluten-Free Gourmet, by Bette Hagman
- The No-Gluten Solution's Childrens Cookbook by Pat Cassady Redjou Contact: "No-Gluten" Children's Cookbook, P.O.Box 731, Brush Prairie, WA 98606
- AlpineAire Foods
P.O. Box 926
Nevada City, CA 95959
Freeze-dried foods for backpacking. Vacuumed-packed. No preservatives, no added sugar, no artificial flavors or colors. Note: the vegetable pasta in Pasta Roma Vegetable Pasta Stew contains wheat flour. Mail orders accepted.
2120 Jericho Turnpike
Garden City Park, NY 11040
Corn pasta products, including ribbon noodles, macaroni and spaghetti.
- Dietary Specialties
P.O. Box 227
Rochester, NY 14601
A wide assortment of mixes, crackers, cookies and pasta. Many exclusive imported items. Mail orders accepted.
- Ener-G Foods, Inc.
P.O. Box 84487
Seattle, WA 98124-5787
Excellent assortment of flours and flour mixes. Will ship in bulk (20# boxes). Variety of baked products, dry soup mixes, flavorings, and pasta. Mail orders accepted.
- Lundberg Family Farms
Richvale, CA 95974
Interesting variety of combination rices. Brown rice cereals and rice cakes. Note: Sweet Dreams Brown Rice Syrup is made using barley malt enzyme. Products made with this syrup should be avoided. Mail orders accepted.
- Med-Diet Inc.
3050 Ranchview Lane
Plymouth, MN 55447
Carries various brands of breads, crackers, cookies, cake and muffin mixes, and pasta. Note: their order blank is not designed for those who must eliminate gluten. Request their list of "wheat/gluten-free foods which contain no wheat starch" so you'll know which things to order.
- Really Great Food Co.
P.O. Box 319
Malverne, NY 11565
Baked products and mixes.
- Red Mill Farms, Inc.
290 S. 5th Street
Brooklyn, NY 11211
Three suitable products which are also lactose-free: Dutch Chocolate Cake, Banana-Nut Cake, and Coconut Macaroons. All vacuumed packed. Mail orders accepted.
- Tad Enterprizes
Tinley Park, IL 60477
Carry a variety of flours for gluten-free baking. Mail orders accepted.