Celiac Disease in Children

           In Children.  The symptoms of celiac disease in children typically become apparent three to five months after first consuming gluten- containing foods although for some few cases, the interval may be as short as one month.  Several of the experts on infant feeding advise that solid foods should not be introduced to a baby’s diet until nearly five months old and that gluten-containing cereal should be avoided for the first six months of life. 

             The celiac, but otherwise normal baby, thrives until gluten is introduced into the diet and then begins to refuse feedings and fails to gain weight.  The child may gradually become irritable or listless and develop a large abdomen.  The stools will typically become abnormal, perhaps large, pale and offensive, or representative of a loose-like diarrhea.  Stools generally float because of the high content of air and fat.  The child may also vomit from time-to-time or in some cases exhibit forceful projectile vomiting with the consumption of selected gluten-containing foods.  Many children lose weight or have a failure to gain weight and the buttocks become flattened.  Some few children may become quite ill with acute diarrhea and dehydration.  Symptoms vary and are different from one celiac child to the next with no two being alike in how the condition “acts out” for them and in their bodies. 

             Older children with more subtle symptoms of  poor appetite, poor growth, and anemia are much more difficult to diagnose as there are many other reasons for failure to grow in childhood. Clinical symptoms often diminish or disappear during puberty [adolescence], although biochemical or morphologic abnormalities of the celiac condition may persist.  More active symptoms will again reoccur in early adult life following the period when the immune system appears to “give more of its attention” to sexual develop- ment.  While the teen may feel that he or she has “grown out of the disease,” the actuality is that the condition continues and should [must] be treated with the same strict gluten-free diet.

             Symptoms to Expect.  Personality changes may occur in children with celiac disease; selected children may become unable to concentrate, be irritable, cranky, and have difficulties with mental alertness and memory function; however, the same process may also occur in teens and adults.  Before removal of gluten from the diet, celiac patients may experience selected neuro-psychiatric symptoms including mood changes, irritability, and depression.  The celiac parent may need to reduce expectancies in learning, following explicit directions, and in carrying out selected aspects of basic discipline for their celiac child.

             The damage to the mucosa lining of the small intestine is the same for both children and adults.  There appears to be a rapid loss of cell surface with the result, even with increasing cell recovery, it is unable to keep up.  The loss of absorptive surface cells of the mucosa of the small intestine results in failure to digest and absorb food from the small intestine into the blood.  The concentration of gluten is highest in the upper part of the small intestine, just beyond the duodenum, where the absorption and the damage to the bowel occur. 

             In the person with celiac disease, products derived from wheat, which contain proteins commonly called gluten or gliadin, cannot be digested because of an immunological  reaction to the toxic prolamins in these proteins.  Products produced from barley, rye, and oats cause the same immune response because their prolamin content has similar amino acid sequences.  Wheat, however, is the only grain that contains all of the toxic prolamins.  It is from wheat gluten that we have the base nomenclature for the gluten-free diet.  

             The continued consumption of the toxic prolamins in a person who is affected with celiac disease causes a reaction that destroys the villi in the small intestine [the jejunum], resulting in malabsorption of vitamins, minerals, proteins, amino acids, sugars, and fats.  In children, this malabsorption may also cause bone problems because of lack of calcium, varying levels of abdominal distention, vomiting, muscle wasting, and failure to properly grow and develop. 

             Why Celiac Disease in my Child.  Celiac disease presents a wide spectrum of symptoms.  The three central factors that appear to relate to onset include the following:  inheriting the “right” genes, the appropriate gliadin from a grain protein to cause a  toxicity, and the action of the immune system.  The genes that determine celiac disease can come from one or both parents in the blood line.  In from 2 to 15 percent of families in which one parent has celiac disease, multiple members will have the potential to develop the condition.  Most researchers indicate that same family siblings appear to have a 30 to 40 percent risk of developing celiac disease.  While the HLA genes are necessary to cause and develop celiac disease, they alone are not sufficient to cause the condition to be activated.    

         The Gluten-Free Diet.  Beginning with the diagnosis of celiac disease, it is important to find the best way of helping that child to accept the fact that there are certain foods that he or she cannot eat.  Parental and sibling attitudes and the attitudes of friends in the community will be important as well.  If family members and friends can accept celiac disease and the diet as a way of life, it will be much easier for the child—now and later in life.  The dictates of a strict gluten-free diet can be more easily communicated to children if the emphasis is on “what you can have” rather than “what you can’t have.”  Lists on the refrigerator, having their snack drawer, and a program of gluten-free food items that includes everyone in the family at meal time will be helpful.  Having their lists at restaurants, snack shops, and at school will be supportive and helpful.  

             With a celiac child, the family then becomes a celiac family.  For most families, it is typically best if the entire family is on the gluten-free diet.  That is not to say there should not be special occasions and specialty foods from time-to-time.  If all main dishes can be the same, much of the feeling of being different in the home will be removed.  Exceptions are most often breads, cakes, and pastries, but even these staples should be provided for all family members if there can be acceptance and a good attitude. 

             It is important that the child understand as many aspects of the gluten-free diet as necessary for his/her stage in life.  The symptoms and difficulties that may be encountered in going off the diet can be dealt with in a simple, straightforward manner.  These matters, of necessity and in the interests of self-care, must be handled without threat and without instilling fear or guilt.  As the child learns about himself and his/her body, he or she needs also to learn about the digestive system, this particular disease and how to handle it appropriately.

Treatment of the Celiac Child.  Special needs, special skills, special interests should be treated as such; and, without making the child to feel different or “not quite good enough.”  Attitude and parenting skills on the part of the parents and extended family members will go a long way toward not having the child enter into any aspect of what may be referred to as “the sick child syndrome.”  Since one or both parents may find themselves overextended or overprotective with their own child  who has the celiac condition, it is often helpful to review and discuss management practices with the child’s pediatrician and the school nurse or counselor.  Teach and represent these skills to grandparents, friends, and extended family.  Big ears, big feet, having an allergy or diabetes, getting a C in English, and having celiac disease are all a part of reality and life.  Keep them all in their place.  Do not help to create nor set yourself up for a “poor sick child illness” that could be worse [and is typically always worse] than the celiac condition. 

It is also highly desirable for parents not to become obsessed with symptoms [should they recur], nor to become obsessive about checking stools and questioning behavior at school and away from home.  Do not blame every minor illness and infection on the celiac condition.  All children become ill; treat minor illnesses as matter-of-factly as possible.  If your child should “hold onto illnesses” such as infections, ear aches, and colds for a longer time and take a longer time to respond to medication and to get back to normal routines—make it a fun time for being together, for learning and reading together, for sharing with one another.  Look for opportunity and you will find it; look for despair and you can find it, too. 

Encourage your child to accept invitations to parties and dine out as often as reasonable for your household.  Simply take along a few gluten-free foods that are appropriate for the occasion and regard that as standard operating procedure.  As a parent, make yourself aware of school and holiday functions and be prepared; at times such as Halloween, take treats to the several homes which your child will visit; for holiday and birthday parties, take and provide the appropriate foods for your child.  Make some extras so that other interested children might also sample and participate. When guests come to your home or you are hosting parties or after-school snacks for your child, provide gluten-free food for everyone; it will help your child to feel more secure in his or her own home. 

Eating Out.  Do not avoid eating out; obtaining a gluten-free meal in most restaurants and fast food establishments need not be difficult.  Fruits, grilled meats, vegetables, salads, eggs, and potatoes can be staple choices in many locations.  Teaching a child how to make wise choices will help him or her to be self-sufficient now as a child and later on as a teen when greater independence is allowed.  Look for restaurants that offer the most variety and that have staff members who will take the time to assist with making gluten-free choices.  Concentrate on food choices available that you can have rather than “sawing through” and negating all of the items you can’t have.  Help your child to develop “menu cards” for his or her billfold for the restaurants and hotels you visit most often. The name of the restaurant or fast food shop along with four or five appropriate food choices can then be readily available and provide a level of independence that does not then need parental input for each and every visit. Enlist and encourage cooperation and support from chefs and food staff members by expressing your thanks and appreciation for their thoughtful assistance.  Consider reminding your child to follow up your good experiences with either a telephone call or a note.

 At School.  For the school system, it will be helpful to have a “prescription” or letter from the child’s pediatric gastroenterologist regarding the diagnosis along with a brief description of the illness and the basic needs for the strict gluten-free diet.  A visit with the school nurse to highlight present behavior and present health status, and to learn how best to handle the needs related to the prescription diet in that particular school district will help to develop good connections within the school.  It may be possible to develop a team of the physician involved, the school nurse, and the school dietitian or head of the dietary department to consider the needs of the child and how he or she might best fit into the school represented.  

  Woven into the plan of action at school must be “helping the child to help himself” to move toward independence, self-management, and self-care.  Above all the plan must move out of and away from all potential for any child being labeled as a “sick child”—especially within the family and in the family’s introduction of the child to the school and community.  Do not look to the development of a protection system, but to the development of a facilitation program that relates to the needs represented in this student [a student who happens to have celiac disease].   The wise parent will not emphasize differences, problems, what my child can’t have, etc. and will then avoid getting onto the trail for the development of the sick child syndrome.   

If your particular school cafeteria program can not be accommodating to gluten-free food selections, try sack lunches.  Hot and cold foods in thermos jars may need to be provided to supplement either a school cafeteria menu or a sack lunch.  Packing gluten-free sack lunches for dad or grandpa and enlisting evaluations of food selections from both children and adults can be become a meaningful involvement and brings the prescription diet into the family system.  Share with three or four of the commercial food vendors your need to prepare sack lunches.  Their dietitians and customer service representatives will come forward with excellent food offerings and sack lunch menu suggestions. 

In Teens.    Most celiac teens will do an excellent job of keeping strictly to the gluten-free diet; a few do not.  Those teens who can stay on the prescription diet appear to be able to rise to any challenge and adapt without bother for what could be perceived as any kind or level of  health problem.

Some teens do have a tough time “being different” from the crowd.  They may have a high need to impress friends in their peer group and also may feel they cannot be different in any way in the eyes of an employer or an authority figure in the school or community.  They may need extra encouragement and support and time to discuss their feelings and particular circumstances.  Above all, they must know and gain awareness and understanding that they have a life-long condition for which gluten is harmful.

As teens learn to know and understand their bodies and changes which occur, they may need to have assurance from both parents and their monitoring physician regarding the high attention the immune system will be giving to sexual changes and associated body developments.  For, it is during these months that it may appear that celiac disease has “gone into remission” or an even greater myth—“that they have grown out of the disease.”  Both of these concepts have no scientific basis.  The disease holds forth—perhaps with fewer or recognized overt symptoms.  Thus, the teen and young adult must be helped to understand that “once a celiac, always a celiac” is not just a trite or cute saying dreamed up by someone in the medical community.  It’s a well-researched fact.  And, most of all—teens and young adults need to maintain a strict adherence to the details of the gluten-free diet—now and for life.

High School and College.  Dozens of boarding schools, college dormitory systems as well as Big 10/Big 12 as well as schools in the  Pacific Rim, and Ivy League have adequate flexibility to handle the gluten-free diet along with any associated specialty needs such as lactose intolerance and specific food sensitivities.   These schools and colleges want to help, they like be asked to help and are anxious to do their part if there can be communication regarding the need.  So, if the teen will be going away to college and chooses to live within a public system, there is good support and a high interest in dealing with the details of the gluten-free diet.  Some colleges will request a prescription from the physician; most schools prefer having special needs regarding diet indicated to them at the time of application for housing.   Students who may choose to live off campus but who wish to have some support from the university food services will typically find this service readily available to them.  With only a bit of pre-planning, foresight, and communication—meeting special dietary needs such as the prescription gluten-free diet will not be a problem for a college or boarding school student.  

 For some celiac patients, the intolerance to gluten appears to heighten periodically and to be close to remission at other times.  At this point, there are many case histories and much recorded data, but there appears to be no concrete answers as to why these patterns occur.  Most of all, such patterns must not be perceived by the celiac as an active or inactive phase of the disease.  The disease continues; its symptoms may be covert, but nevertheless, continue to be the primary illness for the individual.   And with the illness in place, the strict gluten-free diet must also continue to be in place.  

            The Gluten-Free Diet for Life.  The critical reason for banning gluten for life is that every small particle of gluten may do some damage even though the patient may not be aware of it as a problem at the time.  The realization and learning must be:  one molecule of gliadin can be as bad as ten thousand.  Case history data appears to define that later on in life, the patient who goes on and off the diet or who cheats here and there, is the patient who will run the potential risk of a severe relapse, the addition of other immune-related conditions, or new or related health problems will be introduced.   The message is very clear—the basic and cardinal premise for self-management of celiac disease is to avoid all gluten in any and all of its varying formats.  Saying “no” can be the ultimate of self-care. 

 

A Case Study Review

   The following case study is found in Pediatric Case Studies, published by Medical Examination Publishing Co., Inc. Garden City, NY.  Authored by William M. Liebman, M.D. University of California School of Medicine, San Francisco.   pp  178 – 185. 

    Questions:

1.     Celiac Disease is one of the most common causes of malabsorption in infants and children.  Which one of the following is the other most common cause”

a.      Zollinger-Ellison syndrome

b.      Giardiasis

c.      Shwachman-Diamond syndrome

d.      Cystic Fibrosis

e.      Crohn’s Disease

2.     The possible pathogenetic mechanisms in celiac disease include which of the following?

a.      Hypergastroenemia

b.      Hypoperfusion

c.      Peptidase deficiency

d.      Infectious [viral]

e.      Immunologic disorder

          True of False:

3.     Abnormal fecal fat excretion [steatorrhea] is invariably present [95 to100 percent].

4.     The histological features of celiac disease include which of the following?

a.      Shortening of villi

b.      Increased cellularity of lamina propria, predominantly polyps.

c.      Increased cellularity of lamina propria, predominantly plasma cells.

d.      Lacteal dilation in lamina propria

e.      Granuloma formation in submucosa

5.     Treatment may include which of the following?

a.      Gluten-free diet

b.      Milk-free diet

c.      Antacids

d.      Anticholinergics

e.      Corticosteroids

6.     Common complications of this condition include which of the following?

a.      Crisis

b.      Peptic ulcer

c.      Pancreatitis

d.      Inflammatory bowel disease [Crohn’s Disease]

e.      Malignancy

Brief Answers and Comments:

 

1.     [d]  Celiac disease and Cystic Fibrosis constitute the most common causes of malabsorption in infants and children.  Celiac disease is disorder of the small intestine, producing clinical evidence of malabsorption, characterized by structural [histological] abnormality of the mucosa that is then reversed by gluten withdrawal.  The incidence of this condition is significantly higher in Europe than in the United States, i.e., as frequent as 1:300 to 1:500 in western Ireland.  More than one member of a family can be affected, e.g., small intestinal mucosal abnormalities in first-degree relatives.  In addition, a link has been found genetically, histocompatibility antigens, most notably HL-A8.  The likely explanation for this finding rests with immune response genes, which have been linked to the histocompatibility loci in man, as well as in animals.  Yet, 20 to 25 percent of patients with celiac disease do not have the HL-A8 antigen.  Other HL-A8 antigens have been associated with celiac disease, too, e.g., HL-A1antigen.  Therefore, other factors such as environmental and genetic play a role in the development of celiac disease.

2.     [c, e]  In the 1950s, Dicke initially, others later, observed the association of gluten in the diet with the resultant enteropathy, celiac disease.  The consumption of gluten [wheat protein] or one of the ethanol extract fractions, gliadin produces this condition, while their strict dietary elimination results in remission.  Acidic peptide products of gliadin, such as fraction IX or alpha gliadin, have been particularly implicated.

Two major proposals have been frequently mentioned as the cause[s] of the intestinal lesion upon exposure to gluten or toxic peptides.  In the 1980s, the intestinal peptidase deficiency was emphasized.  Its theory is based on the lack of an intestinal peptidase, necessary for the detoxification of gluten.  Although peptidase deficiency is present, mucosal injury is also present.  This deficiency is more likely secondary to the intestinal lesion because healed mucosa has normal peptidase concentration, suggesting a non-primary nature.

The theory that celiac disease is an immunological disorder is the present consideration, especially because of studies with in vitro organ cultures.  Biopsy specimens from the small intestine mucosa of normals, patients with gluten enteropathy, and from patients with other intestinal disorders have been studied by this technique.  Immunoglobulin synthesis, IgA and IgM antibody with antigluten specificity, was found to be increased only in patients with gluten enteropathy.  In addition, IgA complexes have been found in the small intestinal mucosa of patients with gluten enteropathy after gluten challenge.  No effect with gluten challenge [protein] was noted in specimens of patients in remission.  Therefore, these results suggest that gluten protein must first produce an internal change in tissue integrity of susceptible individuals, e.g., activation of the endogenous effector[s], before toxicity occurs.  This hypothesis has been strengthened further by the production of mucosal changes in the presence of gluten protein only when cultured with the mucosa of a patient with active disease. 

Other aspects of the immune status in gluten enteropathy have been extensively studied.  Atrophy of the spleen ahs been confirmed in several reports.  Up to a third of patients may be affected.   The cause remains undefined.  An increase of blood-born infections is a possibility.   Humeral [B-cell] investigations have been numerous.  Serum IgG levels has been generally high, while serum IgM levels have usually been low.  Elevated, as well as low or normal, serum IgE levels have been reported.   Two percent of patients have associated isolated IgA deficiency.  Conversely, elevated levels of IgG, IgA, and IgM are usually found in intestinal secretions.  Immunocytes [IgA, IgG, IgM] in the intestinal mucosa are increased in number; and increased synthesis of IgA and IgM has been noted in organ culture experiments. Cell-mediated [T-cell] studies have been numerous, too.  Reduced numbers and function of circulating T-cells have been reported, using resetting technique and lymphocyte PHA [pyhtohemagglutinin] responsiveness, respectively.  However, clinical evidence of T-cell deficiency has not been observed, e.g., viral/fungal infections. 

The other choices [the other answers] have not been demonstrated to be present in gluten enteropathy.

3.     [f] Steatorrhea [excessive fat excretion] is absent in 10 to 30 percent of children with confirmed celiac disease and is apparently related more to extent than severity of the intestinal mucosal lesion.  Usually the 3-day stool fat collection between nonabsorbably markers in untreated celiacs reveals fat excretion over 10 to 15 percent [normal, 5% or less].

Other laboratory studies, including d-xylose absorption, have been performed in the evaluation of possible celiac disease.  Both urinary excretion [5 hours] and blood levels should be measured after an oral dose of d-xylose.  Some authors have reported the complete separation of untreated celiacs from controls by use of only the fasting and one-hour blood levels.  Based on this abnormal value, small intestinal biopsy has or has not been performed.  Serum total protein and albumin levels have been decreased in only 50 to 70% of untreated celiacs.  In addition, nitrogen losses in stools are abnormally high in only 50%.  The cause[s] of the protein abnormalities appears to be abnormal exudation through damaged mucosa, increased cell exfoliation, and diminished amino acid uptake by the damage mucosa.

Altered pancreatic functional status, specifically reduced volume, bicarbonate, and enzyme output, is probably related to impaired release of secretin and CCK-PZ [cholecystokinin-pancreozymin] from damaged small intestinal mucosa.  Altered release and/or synthesis of endogenous CCK-PZ also results in decreased bile delivery and impaired fat absorption.  Low serum iron and foliate levels, and less so vitamin B-12, have been found, as have low prothrombin levels.

Radiological studies may be helpful.  Bone age may be retarded in long-standing, untreated celiacs.  Osteoporosis and osteomalacia are not infrequent.  Rickets, per se, is not frequent.  The “classical signs” of celiac disease on small bowel series include dilation [jejunum especially], segmentation [large barium clumps with dilated loops], and hypersecretion [flocculation].  In addition, transit time may be slow and diffuse thickening of the mucosal fluids may be visualized.

4.     [a, c]  The histological features of celiac disease have been well described.  The small intestinal tissue is obtained by peroral small biopsy, suing one of several types of biopsy capsules, e.g., Crosby-Kugler.  Initially, there is an accelerated rate of enterocyte loss [shedding].  This results in a compensatory increase to the proliferative zone of the intestinal crypts.  The mucosal architecture will then be altered when the compensatory effort is inadequate.  Subtotal or total atrophy of villi occurs, specifically shortening and clubbing.  The crypts become elongated, increased in diameter and tortuous.  This produces an alteration in the normal villous-to-crypt ration [over 2:1].  In addition, there is increased cellularity of the lamina propria, predominately plasma cells, less so lymphocytes.  Increased collagen may be noted in the subepithelial part of the lamina propria.  These changes are particularly prominent in the proximal jejunum.

Disaccharidase assay of the involved mucosa has revealed decreased levels, particularly lactase.  In addition, decrease alkaline phosphatase, peptide hydrolase, and other enzymes, have also been found.

The other answers, granuloma formation, particularly suggestive of granulomatous bowel disease, and lacteal dilation, suggestive of lymphatic obstruction or disorder, e.g., intestinal lymphangiectasia, are incorrect.

5.     [a, b, e]  The obvious form of treatment is a gluten-free diet.  The initial response is usually dramatic and reasonably rapid, usually within days.  The stools become less frequent and more formed, and the appetite and disposition improve.  Catch-up growth [height, weight] is noted within months.  Normal mucosal architecture is usually achieved within 3 to 6 months.  Conversely, celiac disease is a permanent condition, and re-introduction of gluten to the diet will be followed by recurrence of mucosal abnormalities.  Yet, many authors maintain children on a gluten-free diet for at least 2 years and then reintroduce gluten.  Tolerance to gluten is truly a matter of trial and error, requiring careful, continued observation in order to assure adequate appetite, activity, and growth development.  These clinical assessments should be substantiated periodically by repeat laboratory tests and repeat peroral intestinal biopsy.  If symptomatology reappears or there is a lack of clinical response after 6 months while on a gluten-free diet, repeat evaluation, including intestinal biopsy, is in order.  A diagnosis other than celiac disease is likely.

Because the disaccharidase of the small intestinal mucosa is frequently reduced in celiac disease, particularly lactase, the corresponding substrates [sugars] should be restricted in the diet. 

Corticosteroids have produced variable responsiveness, including improved appetite, activity, growth, and decreased stools.  Their use has been primarily in more severely affected children and in infrequent crises.  Antacids and anicholinergics do not have a place in the usual management.

6.     [a, e]  Celiac crisis is fortunately uncommon since it is a life-threatening complication.  It may be precipitated by prolonged fasting or intercurrent infection.  Dehydration and metabolic acidosis occur due to the protracted diarrhea and vomiting. Treatment includes nasogastric suction, intravenous fluid therapy, colloid administration if shock or undue hypoproteinemia is present, and usually parenteral corticosteroid administration.

     With celiac disease in adults, malignancy may develop.  Lymphoma of the small intestine is most common but carcinoma of the gastrointestinal tract, such as the esophagus, may develop.  Strict adherence to the gluten-free diet does decrease this incidence.  The other answers have not been consistently associated with celiac disease. 

 

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Updated on 02.08.02   webmaster@e-celiacs.org