Celiac Disease-An Overview

The following is an academic paper I wrote for a pathophysiology course while working towards a bachelor degree in nursing so the writing style is a bit stiff.  All I have added are subtitles for easier navigation, and photographs. Although new science has shed further light on this topic, I think you will still find this information helpful and enlightening for overview information on celiac disease.

Celiac Disease
Vanessa L. Marsden
Liberty University
August 2014

Abstract

The focus of this paper is on celiac disease, the consideration of it being an autoimmune disorder, the significant increase of its diagnosis in recent years, and its connection with gluten, a protein found in wheat and other grains such as rye and barley. Therefore, gluten-sensitivity and gluten-intolerance will also be addressed. This paper will discuss that the implications of this disease for the victim goes far beyond gastrointestinal symptoms, affecting future eating patterns and lifestyle; and it is common that other organ systems can be adversely affected as well, such as the endocrine and neural systems. It also deals with how diagnoses are obtained, clinical manifestations, and what implications celiac disease has to the practice of nursing. It will end with what is known about the history of this disease.

Keywords: Celiac disease, gluten, gluten-sensitivity, gluten-intolerance, gastrointestinal disorder, leaky gut, grains, autoimmune

Introduction

Celiac disease is an autoimmune disease that is thought to affect 1% to 6% of the population (Grossman & Porth, 2014) that causes intestinal damage due to inflammation, villous atrophy, and crypt hyperplasia (Memon et al, 2013). Also referred to as celiac sprue or gluten-sensitive enteropathy (Grossman & Porth), celiac disease appears to be on the rise (Coutts, 2013). It is caused primarily by ingesting foods containing gluten, which is a protein that is found in grains such as wheat, rye, and barley (Coutts; Grossman & Porth). Until recently very few people had ever heard the word “gluten”. Now restaurants have gluten-free menus, stores are stocked with gluten-free labeled products, and there are many blogs on the internet about gluten-free living. Celiac disease can be a devastating disease affecting, the gastrointestinal tract, particularly the small bowel, but also many other organs and body systems (Horowitz, 2011). Because of the apparent increase in its diagnosis (Horowitz) and its destructive effects on the health of many people, celiac disease has become more important to consider and disseminate knowledge gained about it. This paper will cover what causes celiac disease, what gluten is, what are the signs and symptoms, what are complications and related conditions, how it is treated, possible preventive measures, as well as lifestyle ramifications, history of the disease, nursing implications, and spiritual aspect.

 

What is Celiac Disease

As stated before, celiac disease (CD) is an autoimmune disease that causes the upper part of the small intestine, the duodenum and sometimes even the jejujum, to become inflamed in response to the ingestion of gluten (James, 2014). This inflammation is due to a “T-cell-mediated immune response” (Grossman & Porth, 2014), and causes intestinal permeability (James) and destruction to the intestinal villus. which leads to malabsorption since there is less surface area to absorb nutrients (Grossman & Porth). “This T-cell-mediated reaction produces antigliadin antibodies immunoglobulin A (IgA) and immunoglobulin G (IgG) to tissue transglutaminase (tTG)” (Horowitz, 2011, p.93). There is also an innate response that causes a regulatory protein called zonulin to be released. Zonulin causes an increase in the permeability of the intestinal lining (Coutts, 2014), also known as “leaky gut” according to Dr. Alessio Fasano, a pediatric gastroenterologist (2014). Furthermore, due to the destruction of the intestinal villi, there is less surface area for nutrient absorption (Horowitz). People with the genetic markers of HLA-DQ2 and HLA-DQ8 are at risk for developing CD; it is estimated that most people diagnosed with CD have one of these two genes (Fasano, 2014; Grossman & Porth, 2014; Scanlon & Murray, 2011). HLA stands for Human Leukocyte Antigen which is a protein on the surface of white blood cells which help the body differentiate between self and non-self (Grossman & Porth, 2014). It is also thought that CD can be triggered by environmental factors, microbial infections, and other things such as medications that increase intestinal permeability (James, 2014). Liliane Papin, a Doctor of Oriental Medicine, proposes that CD could be caused by a nitrogen imbalance due to increased exposure to chemicals and consumption of processed foods (2009). She explains that wheat is high in nitrogen as well as chemical fertilizers and industrial emissions.

Types of Celiac Disease

There are three types of celiac disease. First of all, there is classic celiac disease, which more frequently presents in infants after being exposed to gluten, but it can also develop in older children and adults. Next there is atypical celiac disease. This is usually less severe and sometimes “mistaken for irritable bowel syndrome” (Coutts, 2013, p.28). Another type is latent celiac disease that is found in people with the HLA-DQ2 or DQ8 gene. This group does not have villous atrophy although they usually feel better not eating gluten (Coutts). Lastly, there is the non-responsive celiac disease, otherwise known as refractory sprue or refractory celiac disease according to Fasano, which occur in one to five percent. Even on a strict gluten-free diet, these patients not only fail to improve, but also continue to get worse (2014).

What is Gluten

Gluten, it should be explained, is a protein, prevalent in many of the foods commonly eaten in the Western world. It is in anything containing wheat, barley, and rye. Oats are also thought to be problematic, either because of cross-contamination with the other gluten grains during farming, or storage and processing in graineries, or lack of toleration of it in a small percent of celiac patients. Most foods such as pasta, bread, breakfast cereals, cakes, pies, cookies, bagels, donuts, and pizza contain gluten. Gluten can also be found in some medications, vitamins, and supplements; in Play-Doh (Fasano, 2014); and in surprising foods like imitation crab, red licorice, bleu cheese, Twix candy bars, gravy, seasonings, beer, and Pringles potato chips. It can even be found in orthodontic retainers and cosmetics (Memon et al, 2013).

 

Signs & Symptoms of Celiac Disease

There are many signs and symptoms of CD. These include anemia, diarrhea or constipation, abdominal pain, depression, weight loss or gain, and other gastrointestinal complaints such as bloating and flatulence (Grossman & Porth, 2014; James, 2014). According to Dr. Thomas O’Bryan, in a personal interview with Horowitz (2011), other signs and symptoms of CD can be infertility, miscarriage, chronic fatigue, joint pain, and dermatitis herpetiformis, a rash, which the name indicates. Coutts adds even more signs and symptoms to this list: vitamin deficiency due to malabsorption, failure to thrive in children, and osteopenia (2013). Even more can added to this already extensive list neurological symptoms such as ataxia, migraines, schizophrenia, and peripheral neuropathy (Fasano, 2014). In a study done in Iran between the years 2008 and 2013 diarrhea was the most common symptom (Ganji et al, 2014).

There can be a variety of complications and other conditions associated with celiac disease: Type I diabetes, thyroiditis, Sjorgens syndrome, osteoporosis, epilepsy, and biliary cirrhosis, intestinal lymphoma and pneumococcal infection; the latter being due to atrophy of the spleen, thus from the immune system not working as well. There can also be unexplained infertility and low birth weights (James, 2014; Smyth & Smyth, 2014). However, the line can be rather blurred between the signs and symptoms, and complications and related conditions.

Diagnosing Celiac Disease

There are several ways to diagnose celiac disease, but they are usually used in conjunction with a few different tests. Fasano maintains that most celiac patients will test positive to at least four of the five criteria listed next (2014). These include serological tests, esophagastroduodenoscopy (EGD) obtained tissue samples from the small intestines looking for histological changes, gene testing for the HLA DQ2 or HLA DQ8 gene, clinical presentation, and lastly a positive response to a gluten-free diet as most people feel better after eliminating gluten from their diet. Serologically, testing for anti-endomysium antibodies (EMA), anti-gliadian antibodies (AGA), and anti-tissue transglutaminase (tTG) antibodies can help confirm the diagnosis (Fasano, 2014; Horowitz, 2011; James, 2014; Scanlon & Murray, 2014). However, to most gastroenterologists, the “gold standard” is the intestinal biopsy taking from several different areas in the duodenum that indicate villus atrophy, crypt hyperplasia, or other mucosal changes (Coutts, 2014; Scanlon & Murray, 2104).

The Prevalence of CD

The prevalence of celiac disease according to Fasano (2014) is approximately one in 133 people in the United States, Australia, South America, and Europe. However, another source indicates 1%-6% of the general population (Grossman & Porth, 2014). One study showed a higher ratio of women to men being diagnosed, 2.16:1 (Ganji et al, 2014). Scanlon and Murray estimate that in the last 60 years the diagnosis of CD has quadrupled (2011). It is thought that one reason for the rise in CD is due to how wheat has been genetically altered, and therefore not as well tolerated by the body (Papin, 2009; Scanlon & Murray, 2014).

Preventing Celiac Disease

Not much is known regarding prevention of celiac disease at this time, however, it is thought that delaying the introduction of gluten to a child, modifying how it is introduced to the child’s diet, eating gluten less frequently, eating food more seasonally, and avoiding genetically modified foods may help. In addition, it is thought that by improving the microbiome like by ensuring adequate amounts of good intestinal bacteria, lactobacilli for example, of infants the incidence of CD could be reduced (Fasano, 2014). It is also surmised that breastfeeding exclusively for the first six months may either prevent or delay the onset of celiac disease (Fasano).

Although the prevention of celiac disease is not well understood, there appears to be certain risk factors for developing CD. For instance, people who have the genetic disease Turner syndrome, which affects only the female population, where the person is missing part or all of the X chromosome are more at risk (Grossman & Porth, 2014). Other populations at a greater risk include people with other autoimmune diseases, such as Hashimoto’s thyroiditis and Type I diabetes (TIDM); and those who have inherited the HLA DQ2 and HLA DQ8 genes (Grossman & Porth, 2014). Since there is an association between celiac disease and Type I diabetes, it is recommended that all people with TIDM be screened for celiac disease (Smyth & Smyth, 2014). There is also an increased risk in those whose first-degree relatives have CD according to Memon et al (2013). In one study done by Ganji et al it was found that in Turkey the incidence was 10% that had first-degree relatives while Iran it was 17% (2014).

Treatment for Celiac Disease

The treatment for celiac disease is mostly dietary. It is essential to eliminate all sources of gluten from the diet and to also avoid non-dietary forms of exposure. This needs to be life long (Horowitz, 2011). According to Scanlon & Murray, “Symptoms should improve within 2 weeks” (2011), or in a few weeks (James, 2014). Fasano explains that three elements are needed for an autoimmune disease to develop: a genetic predisposition, a trigger, and intestinal permeability (2014). Since gluten is the trigger in celiac disease once the offending trigger is removed then “the intestine repairs itself and the autoimmune disorder is reversed”. Fasano further claims that CD is “the first autoimmune disease to be treated by removing its cause” (2014, p. 59, 60). Some cases of CD, 2%-5%, known as refractory celiac disease, do not respond to the elimination of gluten. It is thought that this may be due to nutritional deficiencies (Horowitz, 2011). Therefore, Horowitz explains, with these patients, as well as all patients with CD, nutritional supplementation is important. Anemia due to iron deficiency is very common, so it is advised iron, vitamin B12, vitamin D, and folic acid levels in particular should be checked and supplementation be done accordingly. Also, pancreatic enzymes may be helpful in those deficient. It is thought that synbiotics, which are the combined use of prebiotics and probiotics, may help repair intestinal permeability and alter the immune response as well as inhibit toxic and inflammatory effects. Horowitz further suggests that curcumin has been shown in research to reduce inflammation and maybe helpful; she also states that research is being done with the ingesting of helminthes. It has been shown through research with animals that the parasites quiet down the overactive immune response (Horowitz, 2011). Further research on CD continues, which hopefully will turn up a cure. Interestingly, one study done in Europe published in 2010 was able to eliminate inflammatory markers in petri dishes containing intestinal tissue from CD patients by adding vitamin C to the samples (Sardi, 2011). Sardi speculates that CD may be a manifestation of scurvy. However, this appears to be quite a leap.

Other Related Diseases

Related to celiac disease is gluten-sensitivity and wheat allergy. These two conditions differ from CD in that they do not have the HLA DQ2 and DQ8 genetic component necessarily, whereas those with CD do in 97% of the cases (Fasano, 2014). Another difference is with wheat allergies (WA) the onset of symptoms is from minutes to hours, with gluten sensitivity (GS) the onset is anywhere from hours to days, and with CD onset for the first onset of symptoms is weeks to years according to Fasano (2014). Also, Fasano notes that CD is the only one of the three gluten-related diseases to have autoantibodies and enteropathy present. However, all three can have both intestinal symptoms as well as other symptoms outside of the gastrointestinal tract. Gluten-sensitivity, which an innate immune response to gluten, is neither an allergy or an autoimmune disease. Seventy percent of gluten-sensitive patients report abdominal pain, 40% have rashes, 35% have migraines, 34% foggy thinking, as well as chronic fatigue, diarrhea, depression, anemia, paresthesia, and joint pain (Fasano, 2014). Also, only 40% of patients with gluten-sensitivity have the HLA-DQ2 or HLA-DQ8 gene (DiGiorgio & Volta, 2012). As for the wheat allergy, which is very different, is caused by an IgE response, manifests with such symptoms as runny nose; congestion; difficulty breathing or swallowing; itchy skin, throat, and eyes; urticaria, asthma, and nausea or vomiting (Fasano, 2014).

Ramifications of Being Gluten-Free

There are many ramifications of having to omit all exposure to gluten. As stated before, all gluten must be eliminated. Therefore, it can be devastating to receive a diagnosis of celiac disease, accompanied by a “strong sense of loss” due to the inability to ever consume ones favorite foods again (Fasano, 2014). Since so many foods involve gluten one may wonder at first what they will ever be able to eat. They may feel like the “freak that can’t eat wheat” (author’s invention). Food is so daily; it is the center of all celebrations; it is the highlight of many parts of life, it is an issue in going to restaurants, with traveling, with grocery shopping, and eating in friends and families homes. Suddenly one cannot just eat whatever is served to them but now has to inquire as to exact ingredients, read labels, and say no over and over (Fasano). Not only is food an issue but also the patient has to deal with health problems pertaining to celiac disease, which can vary in number and severity.

One must also consider non-dietary sources of possible gluten exposure, necessitating the reading of labels on cosmetics and personal products like shampoo, even having to check the composition of dental appliances (Memon, 2013). Memon et al report that a nine year old girl who was strictly compliant with the gluten-free diet was still symptomatic. It was found that her orthodontic retainer contained gluten in the polymerized plastic, and when this source was removed the child fully recovered (2013). Furthermore, the celiac patient now has to be concerned about gluten contamination when eating at a restaurant, or a friend or relatives house, or at a wedding because even small amounts can be a problem. Fasano states that all gluten “is considered a threat “ to some people with CD, while others can safely tolerate 10 milligrams per day, which is about one-eighth of a teaspoon of flour (2014, p. 85). Therefore, a newly diagnosed celiac patient needs to obtain a new toaster, carefully clean the barbecue grill racks, and throw out things like mayonnaise and peanut butter that may be contaminated with crumbs, instituting a no double-dipping rule. They must also go through all of their food products and staples looking carefully at the ingredients for any hidden sources of gluten. It therefore almost goes without saying that one needs to become an astute label-reader for both what one already has on hand and for future purchases. One also needs to find “safe” restaurants where they can feel confident that the food is not being cross-contaminated. Now one has to be concerned with things like fries being cooked in a dedicated fryer and not in the same oil with items containing gluten like chicken nuggets, and not having bread served on their entrée plate, or croutons on their salad (Fasano). One other impact of eating gluten-free is that it is estimated that it can be two to four times more expensive (James, 2014).

The patient should receive a dietary consult from someone with proper training (Coutts, 2013). There are still many foods to choose from even though, especially at first, it may seem like all choices have been removed. There are gluten-free grains such as rice and quinoa; and any kind of meat, fish, dairy, eggs, all vegetables and fruits, nuts, and beans can be eaten. There are many new baked goods and snacks now that are gluten-free as well. Because of the nutrient deficiencies associated with celiac disease one must ensure that the celiac patient is receiving adequate amounts of such things as vitamin B-12, vitamin D, calcium, and iron. A dietician can direct the patient towards the right foods that will meet the nutrient requirements (Coutts).

 

Celiac Disease and Spirituality

Celiac disease affects all realms of life: body, mind, and soul. This paper has thus far discussed many effects it has on the body. It even touched a little on the mind since CD can cause depression and other mental illnesses such as schizophrenia (Fasano, 2014). Celiac disease has implications in even the way one participates spiritually. For instance, the celebration of communion is done with bread, and the bread contains gluten. Furthermore, this disease can also affect the soul in many ways, needling away at contentment, causing self-absorption, the temptation to feel sorry for oneself, and the feeling of hopelessness. The only antidote to this is saturation with scripture; one must feed oneself with truth.

Several verses that could be helpful with this are listed here, but the Bible filled with more. Contentment is very important to God. Paul’s example in Philippians 4:11-12 can be encouraging; he says, “…for I have learned to be content in whatever circumstances I am…in any and every circumstance I have learned the secret of being filled and going hungry, both having abundance and suffering need”. One must also keep food in the proper prospective. Matthew 6:25 says, “…do not be anxious for your life, as to what you shall eat, or what you shall drink…Is not life more than food and clothing?”. Moreover, Paul instructs in I Thessalonians 5:16 to, “Rejoice always”, and in vs. 18 to, “in everything give thanks”. Life inevitably involves suffering, so it is important to maintain a good prospective. Romans 8:18, “For I consider that the sufferings of this present time are not worthy to be compared with the glory that is to be revealed to us”. One could also be reminded that celiac disease is a result of the Fall and should thus direct emotions and energy to fighting evil and furthering God’s kingdom. Lastly, someone afflicted with celiac disease can look forward to heaven where it says in Revelation 21:4 that there will be no more tears or death or crying or pain (NASB, 1978).

History of Celiac Disease on a Postage Stamp

The history of celiac disease is a bit sketchy. The first mention of it seems be about two thousand years ago by Aretaeus, a Greek doctor from Cappadocia. He called it “koiliakos” which means “suffering in the bowels” (Fasano, 2014, p. 6). Papin claims gluten-sensitivity was officially identified by Dr. Samuel Gee in England (2009). In the 1920’s, Dr. Sidney Haas, a New York City pediatrician, started treating his celiac patients with a bananas only diet with success. However, during World War II Dr. Willem-Karel Dicke, a pediatrician in the Netherlands, discovered that during a wheat flour shortage the incidents of CD was much lower, confirming the suspicion he already had about wheat (Fasano, 2014; Horowitz, 2011). In the 1980’s Dr. Luigi Cavalli-Sforza, an Italian geneticist performed many studies, which have greatly helped in the understanding of the disease epidemiologically and otherwise (Fasano).

Nursing Implications of Celiac Disease

Finally, nursing implications with celiac disease are numerous. A patient can greatly benefit from an astute clinician. Because of gastrointestinal motility disorders, one focus should be on risks related to diarrhea, and the risk for constipation on the other side of the spectrum. One nursing intervention for the former would be to maintain skin integrity and to ensure adequate hydration as well as an electrolyte balance. Interventions pertaining to constipation could be to assess bowel patterns and bowel sounds, and to encourage adequate fiber and fluid intake, adequate exercise, and to assess the need for interventions like stool softeners and enemas. Skin integrity should also be assessed in the cases presenting with dermatitis herpetiformis. Another nursing intervention is to assess for psychological issues such as depression that could be preexistent due to CD and its neurological effects or occurring post-diagnosis due to the gluten-free lifestyle change requirements, and social isolation that could be of a new onset caused by feeling different than others. Nutritional status should be assessed and addressed not only because malabsorption is often associated with CD, but also the patient needs to be taught what foods are permissible. The patient should also be assessed for acute and chronic pain as well, and the nurse should take measures to ensure optimal comfort and to help deal with the pain by encouraging music and laughter to release endorphins, use of analgesics sparingly, relaxation and breathing techniques. The patient may also be at risk for failure to thrive related to malabsorption, diarrhea, and endocrine imbalances due to celiac disease (Doenges, Moorhouse, & Geissler-Murr, 2004).

Conclusion

In conclusion, celiac disease is fairly common disease caused by a very commonly eaten food: gluten. In fact, it would not even exist without gluten, a protein found in wheat, rye, and barley. Although much is known about CD, there is still much yet to be discovered about this devastating disease in regards to prevention, diagnosis, and treatments. Once the diagnosis is obtained it requires the patient to make many lifestyle changes pertaining to eating gluten-free in order to reverse the intestinal damage, heal related conditions, and become as healthy as possible with an autoimmune disorder. There are many spiritual implications of coping with a chronic disease to consider as well. Celiac disease provides many challenges to the patient, the physician, and the nurses, as well as many others in the health care field.

I would love to hear any comments you might have about your experience with celiac disease.  Blessings to you!

 

Read this article I wrote about cross-reactive foods to gluten for additional important information. It also better reflects my writing style.

Also check out this one for a bit of humor on challenges with living gluten-free while traveling: The Freak Who Cannot Eat Wheat.

 

Contact me here if you would like to schedule a free, no obligation discovery call to see if you could benefit working with me as a health coach.

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References

Coutts, A. (2013). An overview of coeliac disease: patterns, causes, symptoms and management. Gastrointestinal Nursing, 11(3), 26-30.

De Giorgio, R., & Volta, U. (2012). New understanding of gluten sensitivity. Nature Reviews Gastroenterology & Hepatology, 9(5), 295

Doenges, M., Moorhouse, M., & Geissler-Muir, A. (2004). Nurse’s pocket guide: diagnoses, interventions, and ratiionales. (9^th ed.). Philadelphia, PA: F.A. Davis Co.

Fasano, A. & Flaherty, S. (2014). Gluten Freedom. New York, NY: Turner Publishing Company

Ganji, A., Esmaielzadeh, A., Aghayee, M. A., Goshayeshi, L., & Ghaffarzadegan, K. (2014). The clinical presentation of celiac disease: Experiences from Northeastern Iran. Middle East Journal       of Digestive Diseases 6(2), 93-96.

Grossman, S., & Porth, C. M. (2013) Porth’s pathophysiology: Concepts of altered health states (9th ed.). Philadelphia, PA: Lippincott-Raven Publishers.

Horowitz, S. (2011). Celiac disease: new directions in diagnosis, treatment, and prevention. Alternative & Comlementary Therapies, 17(2), 92-98.

James, S. (2014). Coeliac disease: a gluten-free commitment. Practice Nursing, 25(3), 116-123.

Memon, Z., Baker, S., Khan, A., Hashmi, H., & Gelfond, D. (2013). An orthodontic retainer preventing remission in celiac disease. Clinical Pediatrics, 52(11), 1034-1037.

Papin, L., & Cuzange, D. (2009). Gluten interolance and celiac disease. Macrobiotics Today, 49(6), 8-11.

Sardi, B. (2011). Celiac/gluten intolerance: are we chasing the wrong villain?. Functional Ingredients, (114), 34.

Scanlon, S. A. & Murray J. A. (2011). Update on celiac-etiology, differential diagnosis, drug targets and management advice. Clinical and Experimental Gastroenterology, (4), 297-311.

Smyth, D., & Smyth, T. (2014). Diabetes and celiac disease: implementing a gluten-free diet. Practice Nursing, 25(6), 292-297.