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Celiac Disease

7 min read

References:

  1. Wong’s Nursing Care of Infants and Children, 11th Edition, ISBN 978-0-323-54939-4, by Marilyn J. Hockenberry, David Wilson, and Cheryl C. Rodgers (Ch. 25, pp. 862–866)
  2. Maternal & Child Health Nursing: Care of the childbearing & childrearing family, 8th Edition, ISBN 978-1-4963-4813-5, by JoAnne Silbert-Flagg and Adele Pillitteri (Ch. 45, pp. 2788–2791)
  3. Saunders Comprehensive Review for the NCLEX-RN Examination, 9th Edition, ISBN 978-032-37-9530-2, by Linda Anne Silvestri, Angela E. Silvestri, and Jessica Grimm (Ch. 34, pp. 431)

Malabsorption syndromes are characterized by chronic diarrhea and malabsorption of nutrients. An important complication of malabsorption syndromes in children is failure to thrive. Most cases are classified according to the location of the supposed anatomic or biochemical defect. The term celiac disease is often used to describe a symptom complex with four characteristics: (1) steatorrhea (fatty, foul, frothy, bulky stools), (2) general malnutrition, (3) abdominal distention, and (4) secondary vitamin deficiencies.

Celiac Disease

Celiac disease—malabsorption syndrome, gluten-induced enteropathy, celiac sprue—is an autoimmune disorder triggered by the ingestion of gluten in genetically susceptible individuals. This disorder results in permanent intestinal intolerance to dietary gluten, a protein present in (mn. BROW) barley, rye, oats, and wheat that causes damage to the villi in the small intestine.

Celiac disease is characterized by villous atrophy in the small intestine in response to protein gluten. When individuals are unable to digest the gliadin component of gluten, an accumulation of a toxic substance (glutamine) occurs that is damaging to the mucosal cells. Damage to the mucosa of the small intestine leads to villous atrophy, hyperplasia of the crypts, and infiltration of the epithelial cells with lymphocytes. Villous atrophy leads to malabsorption due to the reduced absorptive surface area.

  1. Incidence: Children with unexplained iron deficiency anemia, recurrent aphthous stomatitis, dental enamel defects, type 1 diabetes, Down syndrome, selective immunoglobulin A deficiency, autoimmune thyroid disease, Turner syndrome, or Williams syndrome are more susceptible to being diagnosed with the disease. This disease is seen more frequently in Europe and the United States in approximately 1% of these populations, and it is rarely reported in Asians or African Americans
  2. Etiology: Genetic predisposition is an essential factor in the development of celiac disease. Membrane receptors involved in preferential antigen presentation to CD4+ T cells play a crucial role in the immune response characteristic of celiac disease. Children with genetic susceptibilities, namely HLA-DQ2 or HLA-DQ8, are more susceptible to being diagnosed with celiac disease.
  3. Clinical Manifestations: Symptoms of celiac disease appear when solid foods such as beans and pasta are introduced into the child’s diet between the ages of 1 and 5 years. There is usually an interval of several months between the introduction of gluten into the diet and the onset of symptoms. Intestinal symptoms are common in children diagnosed within the first 2 years of life, appearing 3 to 6 months after the introduction of gluten into the diet. Other symptoms include failure to thrive, chronic diarrhea, abdominal distention and pain, muscle wasting, aphthous ulcers, and fatigue.
    • Impaired fat absorption: steatorrhea (excessive large, pale, oily, frothy stools), exceedingly foul-smelling stools. Acute or insidious diarrhea may be present.
    • Impaired nutrient absorption: malnutrition, muscle wasting (especially prominent in legs and buttocks), anemia, anorexia, abdominal distention
    • Behavioral changes: irritability, uncooperativeness, apathy
    • Celiac crisis (in very young children), includes acute, severe episodes of profuse watery diarrhea and vomiting. It may be precipitated by infections (especially gastrointestinal), prolonged fluid and electrolyte depletion, fasting, and emotional disturbance.

Diagnostic Evaluation

Gluten should not be excluded from the diet until the diagnostic evaluation is complete so proper identification can occur.

  1. The first step is a serologic blood test for autoantibodies—tissue transglutaminase (tTG-IgA) and anti-endomysial antibodies (EMAs)—in children 18 months of age or older. EMAs are found in 95% of celiac patients. To be accurate, these tests should be performed while the child is still consuming a gluten-containing diet.
  2. Positive serologic markers should be followed by an upper GI endoscopy with biopsy. The diagnosis of celiac disease is based on a biopsy of the small intestine demonstrating the characteristic changes of mucosal inflammation, crypt hyperplasia, and villous atrophy.

Due to better testing and earlier diagnosis, the classical picture of a child with celiac disease—a thin child with a distended abdomen—is rarely seen.

Therapeutic Management

Treatment of celiac disease consists primarily of dietary management. Although a gluten-free diet is prescribed, it is actually low in gluten because it is impossible to remove every source of this protein. Because gluten is found primarily in wheat and rye, but also in smaller quantities in barley and oats, these four foods (mn. BROW) are eliminated. Corn, rice, and millet are substitute grain foods.

Foods Allowed

Beef, pork, poultry, and fish; eggs; milk and some dairy products (unless lactose intolerance is present); vegetables, fruits, rice, corn, gluten-free flour, puffed rice, cornflakes, cornmeal, and precooked gluten-free cereals

Clients with untreated celiac disease may have lactose intolerance, especially if their mucosal lesions are extensive. Lactose intolerance usually improves as the mucosa heals with gluten withdrawal. Specific nutritional deficiencies, such as iron, folic acid, and fat-soluble vitamin deficiencies, are treated with appropriate supplements.

Prognosis

Celiac disease is regarded as a chronic disease; its severity varies greatly among children. The most severe symptoms usually occur in early childhood and again in adult life. Most children who comply with dietary management are healthy and remain free of symptoms and complications; however, children should be evaluated annually for nutritional deficiencies, impaired growth, delayed puberty, and reduced bone mineral density.

Nursing Care Management

The main nursing consideration is helping the child adhere to the dietary regimen. Considerable time is involved in explaining the disease process to the child and parents, the specific role of gluten in aggravating the disorder, and those foods that must be restricted. It is difficult to maintain a diet indefinitely when the child has no symptoms and temporary transgressions result in no difficulties. However, the majority of individuals who relax their diet will experience a relapse of their disease. Control is easier during infancy, but becomes more difficult in school-aged children or adolescents.

  1. Sources of gluten: chiefly cereal and baked goods, but grains are frequently added to processed foods as thickeners or fillers. Gluten is also added to many foods as hydrolyzed vegetable protein, which is derived from cereal grains. Advise parents to read all label ingredients carefully to avoid hidden sources of gluten.
    • Bread, cake, cookies, crackers, donuts, pies, spaghetti, pizza, prepared soups, hot dogs, luncheon meats, and some prepared hamburgers.
    • Luncheon preparation away from home is particularly difficult because bread, luncheon meats, and instant soups are not allowed.
    • This becomes another financial burden because many inexpensive or convenient foods cannot be used.
  2. Other dietary alterations:
    • In severe mucosal damage, the digestion of disaccharides is impaired, especially in relation to lactose. An additional restriction of all milk products may be necessary, but intolerance may resolve after gluten withdrawal.
    • In general, dietary management includes a diet high in calories and proteins with simple carbohydrates such as fruits and vegetables, but low in fats. Because the bowel is inflamed as a result of the pathologic processes in absorption, the child must avoid high-fiber foods such as nuts, raisins, raw vegetables, and raw fruits with skin until inflammation has subsided.
  1. Imbalanced nutrition, less than body requirements, related to malabsorption of food
    • Outcome evaluation: Child’s weight is maintained on a percentile curve on a growth chart, skin turgor is good, stool pattern and consistency is normal; parents verbalize appropriate gluten-free food choices.
    • Parents should be counseled on a gluten-free diet. If possible, they should meet with a registered dietitian. They need to recognize that gluten is a part of wheat flour, gravy, soups, and sauces as well as packed and frozen foods.
    • Because children are anorectic when they are first introduced to the new diet, getting them to eat it may be a problem. Remember that toddlers often eat small servings more readily than larger ones. Help parents create incentives to eat, such as inviting dolls to “tea” or having a picnic outside.
    • Until children can recognize which foods they can or cannot eat, parents often find it difficult to let them stay at friends’ houses or go to summer camp, activities that are important to children learning independence.

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