Digital Repository Doctoral Projects Spring 2023 Implementation of a Pediatric Celiac Disease Care Process Model Chelsea Pike Weber State University Follow this and additional works at: https://dc.weber.edu/collection/ATDSON Pike, C. (2023). Implementation of a Pediatric Celiac Disease Care Process Model Weber State University Doctoral Projects. https://cdm.weber.edu/digital/collection/ATDSON This Project is brought to you for free and open access by the Weber State University Archives Digital Repository. For more information, please contact archives@weber.edu. IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL 1 Implementation of a Pediatric Celiac Disease Care Process Model by Chelsea Pike A project submitted in partial fulfillment of the requirements for the degree of DOCTOR OF NURSING PRACTICE Annie Taylor Dee School of Nursing Dumke College of Health Professions WEBER STATE UNIVERSITY Ogden, Utah April 28, 2023 Chelsea Pike _______________________________________________ Chelsea Pike, DNP Executive Leadership, RN, CNE Kristy A. Baron _______________________________________________ Kristy Baron, PhD, RN ________________________________________________ Melissa NeVille Norton DNP, APRN, CPNP-PC, CNE Graduate Programs Director Note: The program director must submit this form and paper. April 28, 2023 _________________ Date April 28, 2023 __________________ Date April 28, 2023 __________________ Date IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL 2 Abstract Purpose: Lack of consistent care for children with celiac disease (CD) can lead to poor health outcomes due to diagnostic delays, misdiagnoses, and incomplete monitoring. Therefore, physicians in a pediatric clinic requested guidance for recognizing, diagnosing, and treating CD in children. This project aimed to create and introduce an evidence-based pediatric care process model to facilitate standardization of care among pediatric patients with CD. Methods: Interventions aimed to recommend, implement, and evaluate a pediatric CD care process model for use in a pediatric clinic. Collaboration with a CD content expert and a pediatrician from the intended clinic was essential to ensure the model’s accuracy and applicability. The model was introduced to the physicians with a PowerPoint presentation, including pre- and post-surveys, to measure the change in pediatricians’ comfort in managing pediatric CD. Results: Quantitative survey results (n = 4) showed increased physician comfort in managing pediatric CD. Similar qualitative results were obtained from physician feedback during the presentation. Implications for Practice: Implementing this care process model can decrease specialist referrals and reduce diagnostic delays for many children with CD. The model outlines consistent and evidence-based disease management to improve outcomes for children. The model was given to a second pediatric clinic within Intermountain Health and may be presented to the organization’s pediatric service line team to determine if application within other pediatric clinics is appropriate. Pediatricians’ consistent use of the care process model can improve children’s health. Keywords: pediatric celiac disease, care process model, diagnosing, treating, monitoring IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL 3 Table of Contents Implementation of a Pediatric Celiac Disease Care Process Model ................................................ 5 Background and Problem Statement ........................................................................................... 5 Diversity of Population and Project Site ..................................................................................... 7 Significance for Practice Reflective of Role-Specific Leadership .............................................. 8 Literature Review ............................................................................................................................ 9 Search Methods ........................................................................................................................... 9 Synthesis of the Literature ......................................................................................................... 10 Celiac Disease in Children .................................................................................................... 10 Symptoms .............................................................................................................................. 11 Long-Term Consequences ..................................................................................................... 12 Associated Conditions ........................................................................................................... 12 Diagnosis ............................................................................................................................... 13 Treatment ............................................................................................................................... 15 Continued Monitoring ........................................................................................................... 16 Provider Education ................................................................................................................ 16 Discussion.................................................................................................................................. 17 Implications for Practice............................................................................................................ 17 Framework and Project Application .............................................................................................. 18 Framework Description ............................................................................................................. 18 Framework Application to Project ............................................................................................ 18 Project Plan .................................................................................................................................... 19 Project Design ........................................................................................................................... 19 Needs Assessment/Gap Analysis of Project Site and Population ............................................. 20 Cost Analysis and Sustainability of Project .............................................................................. 21 Project Outcomes....................................................................................................................... 21 Consent Procedures and Ethical Considerations ....................................................................... 22 Instruments to Measure Intervention Effectiveness .................................................................. 22 Project Implementation ................................................................................................................. 23 Project Interventions .................................................................................................................. 23 PowerPoint Presentation ........................................................................................................ 23 Care Process Model ............................................................................................................... 24 Long-Term Program Outcome Interventions ........................................................................ 25 Project Timeline ........................................................................................................................ 25 Project Evaluation ......................................................................................................................... 25 Data Maintenance/Security ....................................................................................................... 26 Data Collection and Analysis .................................................................................................... 26 Findings ..................................................................................................................................... 27 Strengths ................................................................................................................................ 28 Weaknesses............................................................................................................................ 29 Discussion...................................................................................................................................... 29 Translation of Evidence into Practice........................................................................................ 30 Implications for Practice and Future Scholarship ..................................................................... 30 Sustainability ......................................................................................................................... 31 Dissemination ........................................................................................................................ 31 IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL 4 Conclusion ................................................................................................................................. 31 References ..................................................................................................................................... 33 Appendix A ................................................................................................................................... 39 Appendix B .................................................................................................................................... 40 Appendix C .................................................................................................................................... 43 Appendix D ................................................................................................................................... 46 Appendix E .................................................................................................................................... 57 Appendix F .................................................................................................................................... 61 IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL 5 Implementation of a Pediatric Celiac Disease Care Process Model The prevalence of celiac disease (CD) is estimated to be approximately 1% of the population worldwide (Russo et al., 2020). CD has become much more common over the last 50 years; its prevalence doubles approximately every 15-20 years (Elkin et al., 2018). This dramatic rise in incidence contributes to an increasing burden on society and healthcare systems (King et al., 2020; Ludvigsson et al., 2015). Individuals currently diagnosed with CD only account for an estimated 15% of people with the disease, leaving 85% undiagnosed and untreated (Elkin et al., 2018; Mearin, 2007). Some reports suggest that the number of undiagnosed cases of CD is closer to 90% (Laurikka et al., 2018; Oliveira et al., 2018). Continuity of care is essential for patients with CD. Primary care providers can best provide this care by recognizing symptoms, facilitating diagnostic testing, monitoring adherence, and managing complications. However, CD is often undiagnosed or misdiagnosed due to the variability of symptoms, especially in children who cannot verbalize when they feel unwell. As a result, children with CD are at an increased risk of long-term complications due to malabsorption that results from undiagnosed or unmonitored CD (Mearin, 2007). Continuity of care by a pediatrician trained in CD diagnosis and disease management can decrease the risks of complications and the costs associated with non-adherence to the prescribed treatment. The primary purpose of this project is to recommend, implement, and evaluate an evidence-based pediatric care process model for diagnosing, monitoring, and treating CD in the pediatric population of Bryner Pediatrics in Salt Lake City. Background and Problem Statement The prevalence of CD among children is approximately 1%, which is consistent with the prevalence found in the general population (Russo et al., 2020). While symptoms present early IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL 6 for some children, others may ingest gluten for many years before disease presentation (Mearin, 2007). Although the rates of children who are non-compliant with a gluten-free diet (GFD) are similar to those in adults, many diet-compliant children continue to have unknown gluten exposures while on the GFD. Russo et al. (2020) studied this aspect of GFD compliance; they reported that more than 40% of children with CD on a GFD who underwent a follow-up biopsy after 1.3 years did not have mucosal healing, indicating ongoing unintentional gluten ingestion. These accidental gluten exposures increase the risks of disease complications for children with CD. In a personal interview, Dr. Carrie Johnson, a pediatrician at Bryner Pediatrics, reported that children who test positive for CD at their clinic are usually referred to the Pediatric Gastroenterology Division, University of Utah Department of Pediatrics at Primary Children’s Hospital for endoscopic confirmation and follow-up care (personal communication, September 21, 2021). By preparing pediatricians through education about diagnostic tests, referrals, followup care, and overall disease management, the care for pediatric patients with CD can be shifted back to the primary care pediatricians leading to earlier diagnoses, better diet adherence, fewer short- and long-term complications, higher patient and family satisfaction, and lower costs (Hill et al., 2005; Laurikka et al., 2018; Ludvigsson et al., 2015; Zipser et al., 2005). Several pediatricians at Bryner Pediatrics identified the need for this project based on their lack of awareness regarding current recommendations for diagnosing and caring for children with CD. On a case-by-case basis, the pediatricians asked the pediatric gastroenterologists at the Pediatric Gastroenterology Division at Primary Children’s Hospital what CD diagnostic tests to order and what to do with the results obtained from the lab tests. The pediatricians have requested a care process model outlining evidence-based practice for IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL 7 diagnostic and follow-up care for children with CD to streamline and standardize the process. They want the care process model to include the diagnosis algorithm currently used by the Pediatric Gastroenterology Division at Primary Children’s Hospital, a list of labs that should be monitored immediately after diagnosis and thereafter, and what resources to give newly diagnosed patients and their parents to help them transition to a GFD. Diversity of Population and Project Site The proposed setting for the project is a pediatric primary care clinic in downtown Salt Lake City, Utah, called Bryner Pediatrics. Bryner Pediatrics is established within the organizational network of Intermountain Health (IH), based in Salt Lake City, Utah. The clinic is located within the larger Salt Lake Clinic, a large facility offering access to primary practices, many specialties, a laboratory, a pharmacy, radiology and imaging, and urgent care. The clinic is staffed by five pediatricians, an office manager, a practice administrator, three registered nurses, approximately seven medical assistants, four patient service representatives, two case managers, and one social worker. The providers at the clinic care for a diverse population of children in Salt Lake City and the surrounding area. They accept many insurance plans, including Medicaid, allowing patients from lower-income households to receive services. One pediatrician within the clinic is a developmental specialty pediatrician who treats patients with unique special needs throughout Utah and several neighboring states. The clinic is open daily during the week and Saturdays to accommodate weekend sick visits, giving their patients complete access to their primary care providers. In addition, two case managers and one social worker within the clinic facilitate care that addresses more than their patients’ physical well-being; they care for the whole person and provide for various other needs for their patients and their families. Their comprehensive care IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL 8 services include coordinating home health services and equipment, addressing insurance concerns, and monitoring patients’ mental and emotional well-being. The proposed project can benefit the diverse pediatric patients served by Bryner Pediatrics. Because some patients are underinsured or have high insurance deductibles or copays, this project offers the added benefit of primary care management of the disease. As a result, patients with celiac can receive monitoring with follow-up appointments by their primary care pediatrician, leading to better disease management and improved patient outcomes. Significance for Practice Reflective of Role-Specific Leadership As a DNP-prepared nurse leader, I can improve the health and outcomes of children with CD by evaluating and translating current research into practice, which is outlined as Domain 1: Knowledge for Nursing Practice in The American Association of Colleges of Nursing’s (AACN) Core Competencies for Professional Nursing Education (AACN, 2021). I will base the new care process model on recent evidence supporting the primary care management of CD, which exemplifies the leadership role of translating evidence from nursing science into practice. As a nurse leader, I will apply Domain 6: Interprofessional Partnerships of the AACN’s Role-Specific Core Competencies by integrating an evidence-based care process model to improve team effectiveness and outcomes (AACN, 2021). The care process model will facilitate interprofessional collaboration between the specialists at the Pediatric Gastroenterology Division at Primary Children’s Hospital and the pediatricians at Bryner Pediatrics, creating sustainability for the new care process model and efficiency for the transitions between clinics. Having worked at the identified clinic and as a nurse leader with passion and concern for the pediatric presentation of CD, I plan to advance CD care within the organization and community by creating a new pediatric care process model. IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL 9 Literature Review This literature review explores evidence-based practice standards for diagnosing, monitoring, and treating CD in the pediatric population, supporting the proposed care process model for use by pediatricians. Themes emerged from the literature review to guide the development of the care process model, including (a) approximately 85% of those with CD are undiagnosed and therefore at an increased risk of complications (Elkin et al., 2018; Mearin, 2007); (b) pediatric patients with certain levels of positive celiac lab results do not require a biopsy for disease confirmation; (c) children with certain conditions are at an increased risk of developing CD, and careful monitoring should be done for early detection of disease presentation in children with those conditions; and (d) children with CD should be monitored for return of normal lab values after diagnosis and initiation of a GFD. The Iowa Model Revised: EvidenceBased Practice to Promote Excellence in Healthcare is the framework used to guide this project. Search Methods Search terms for this project include celiac disease, gluten-free diet, clinical practice guidelines, non-adherence, incidence, symptoms, intestinal symptoms, extraintestinal symptoms, complications, disease presentation, biopsy, referral, gastroenterology, lab tests, testing, IgA test, pediatric, pediatrician, diagnosis, recommendations, nutritional deficiencies, monitoring, practice guideline, nutritionist, and algorithm. Exclusion criteria included articles more than 20 years old. Moreover, the sources used for the literature search were Google Scholar, AHRQ for Clinical Practice Guidelines, and the Weber State University Stewart Library. IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL 10 Synthesis of the Literature The literature was synthesized to provide evidence for the proposed pediatric CD care process model. The topics discussed include CD in children, symptoms, consequences, diagnosis, treatment, associated conditions, monitoring, and provider education. Celiac Disease in Children CD is a chronic, systemic, autoimmune, enteropathic disorder with symptoms triggered by ingesting dietary gluten (Rosen et al., 2011). Since its identification in 1888, the prevalence has continued to rise, increasing greater than fourfold in the United States during the last 50 years (Rubio-Tapia et al., 2009). Furthermore, because it is a factor in the development of chronic health conditions, it has become a public health concern due to its increasing burden on society and the overall healthcare system (King et al., 2020; Mearin, 2007; Rubio-Tapia et al., 2009). As previously stated, an estimated 85% of patients with CD are undiagnosed, causing many with the disease to be unaware of their risk of complications due to lack of treatment (Elkin et al., 2018; Mearin, 2007). Several authors describe CD symptoms as an iceberg, with the visible top of the iceberg representing patients with typical disease presentations and the hidden but massive part of the iceberg under the water line representing patients with atypical and possibly undiagnosed disease (Bingham & Bates, 2020; Mearin, 2007; Rubio-Tapia et al., 2009; Scanion & Murray, 2011). Patients with atypical disease presentations are susceptible to many complications due to misdiagnosis or delayed diagnosis (Elkin et al., 2018). Whether the disease presentation is typical or atypical, symptoms range from nonexistent to mild to severe, often presenting as gastrointestinal, extraintestinal, or dermatological. IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL 11 Symptoms This disorder, which affects genetically-predisposed individuals, is a condition in which the body has an inappropriate response to ingested gluten, leading to malabsorption of nutrients and damage to the intestinal villi of the small intestine (Elkin et al., 2018). As a result, children with the disease may present to their pediatrician with gastrointestinal symptoms, extraintestinal symptoms, both, or none at all. The most common clinical manifestations of CD, which are more commonly seen in children, include diarrhea or constipation, abdominal pain, tiredness, weight loss, anemia, steatorrhea, bloating, anorexia, failure to thrive, and flatulence (Barker et al., 2005; RemesTroche et al., 2018; Rosen et al., 2011; Rubio-Tapia et al., 2013). Less common symptoms are extraintestinal and include abnormal liver function, anemia, bone disease, skin disorders, arthritis, ataxia, depression, dental enamel hypoplasia, short stature, infertility, alopecia, amenorrhea, anxiety, arthralgias, headache, stomatitis, chronic fatigue, irritability, myalgias, dermatitis herpetiformis, and peripheral neuropathy (Mearin, 2007; Remes-Troche et al., 2018; Rubio-Tapia, 2013; Scanion & Murray, 2011). Some symptoms of CD are specific to children. These symptoms include poor growth, delayed puberty, short stature, anemia (seen in 23% of children with untreated CD), nutritional deficiencies (present in 20%-38% of children with CD), neurological symptoms (present in approximately 20% of children at diagnosis), and dental enamel defects (Di Nardo et al., 2019; Laurikka et al., 2018; Oliveira et al., 2018). Interestingly, some reports show that poor growth, dental enamel defects, and neurological symptoms may be the initial presenting symptoms for some children, which complicates diagnosis when providers are watching for classic symptoms IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL 12 of CD when determining testing criteria and disease risk (Laurikka et al., 2018; Oliveira et al., 2018). Long-Term Consequences The long-term consequences of undiagnosed or untreated CD vary but have been shown to include every body system and may be reversible or permanent. Poor absorption causes many of the consequences and complications of CD, including deficiencies in macronutrients, micronutrients, vitamins, and minerals (Di Nardo et al., 2019). The severity of the resulting deficiencies is related to the length of time since the start of the disease, the location and extent of damage to the small intestine, and the degree of malabsorption (Di Nardo et al., 2019). These deficiencies can result in delayed growth and development, including deficits in cognitive, motor, and psychomotor abilities and adverse effects on social skills and school performance (Afzal et al., 2020). Untreated, CD’s additional long-term consequences include joint disease, bone disease, tooth enamel defects, liver damage, neuropathy, dementia, and fractures (Laurikka et al., 2018). Furthermore, individuals with CD also have an increased risk of malignancy, nutritional deficiencies, osteoporosis, depression, type 1 diabetes, anemia, arthritis, and other associated autoimmune diseases, including autoimmune hepatitis, Sjogren’s Syndrome, Addison’s disease, and peripheral neuropathies (Bingham & Bates, 2020; Elkin et al., 2018; Mearin, 2007; Rosen et al., 2011). Associated Conditions In addition to testing patients based on clinical manifestations, pediatricians should also identify patients with an increased risk of developing the disease, including patients with other associated disorders or a family history of CD. For example, childhood conditions that have a IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL 13 higher prevalence of CD include autoimmune diseases such as type 1 diabetes (5%-15% prevalence), autoimmune thyroid disease, autoimmune IgA deficiency (1.7%-7.7% prevalence), and chromosomal abnormalities such as Down Syndrome (5%-12% prevalence), Williams Syndrome (9.5% prevalence), and Turner Syndrome (4.1%-8.1% prevalence) (Hill et al., 2005; Husby et al., 2019; Oliveira et al., 2018). Furthermore, children with first-degree relatives with CD have an increased risk of 5%-10% of having the disease (Mearin, 2007; Russo et al., 2020). Therefore, children with these conditions should be monitored closely for early identification of disease symptoms. Diagnosis Diagnosing CD in children can be challenging and slow due to the variability and lack of specificity of symptoms. A thorough assessment is essential to evaluating possible disease symptoms and complications. The assessment should begin with an in-depth history of symptoms, other diseases or conditions, duration of symptoms, causes of relief or exacerbation, and family history of CD or other autoimmune diseases. The physical assessment should include assessing every body system because the disease and its related complications can affect organs, bones, teeth, neurological status, blood values, nutritional status, skin, and growth and development. An essential component of obtaining a diagnosis is evaluating CD-specific diagnostic lab tests. CD-specific labs include TTG IgA, total IgA, and EMA IgA (Husby et al., 2019). Additionally, a biopsy of the duodenal section of the small intestine may be performed, during which samples are taken, and the mucosa is inspected for villous atrophy (Hill et al., 2005). Earlier diagnoses in children have become possible by recent evidence showing that not all children with positive serology require a biopsy for a definitive diagnosis (Bingham & Bates, IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL 14 2020; Bishop et al., 2018; Ermarth et al., 2017). In 2017, Ermarth et al. (2017) published a retrospective study (n = 3,555) of pediatric patients demonstrating that a single test, the TTG IgA, could determine if biopsies are indicated for pediatric patients and that it could predict more than 96% of positive CD cases in patients with certain antibody levels. They addressed the remaining 4% of cases stating that repeat serum testing could reduce it to 2%. For less severe histopathology results, however, the positive predictive value for CD is lower, supporting the importance of performing a biopsy to confirm the diagnosis (Ermarth et al., 2017). Ermarth et al. (2017) based their study on the European Society of Pediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) guidelines for diagnosis, questioning the need for biopsies to confirm a diagnosis. Conversely, the current North American guidelines include the standard to confirm a diagnosis with a biopsy for all patients with positive serology. Bishop et al. (2018) performed a prospective study (n = 104) based on the same ESPGHAN guideline and reported similar results. They further determined that using this guideline would result in a 50%-60% reduction in pediatric patients requiring biopsies for disease confirmation. However, they also evaluated EMA and HLA according to the ESPGHAN recommendations in contrast to Ermarth et al., who did not. Ermarth et al. (2017) addressed their decision not to evaluate EMA and HLA when they stated that these recommendations in the ESPGHAN guidelines are less applicable and more costly. Barker et al. (2005) attempted to prove the same hypothesis by performing a retrospective chart review (n = 103) of patients that had done both TTG titers and biopsy to determine if high TTG titers were consistent with positive biopsy results. Webb et al. (2015) presented their results on a cross-sectional CD screening study (n = 267) to evaluate the same correlation between high TTG-IgA and positive biopsy results. Klapp et al. (2013) also performed a retrospective review IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL 15 (n = 150) to determine the validity of using one lab test to achieve positive diagnoses in children with high results. Similar to Ermarth et al. and Bishop et al.; Barker et al., Webb et al., and Klapp et al. determined that the newly proposed approach to diagnosing appears accurate in its ability to diagnose CD without a biopsy. The collection of these studies supports the change in practice to determine the need for a biopsy based on a patient’s serology when diagnosing a child with CD. Treatment After diagnosis, a strict GFD is the only treatment for CD and is effective if done correctly (Hill et al., 2005; Laurikka et al., 2018). However, the most common cause of poor response to treatment is non-adherence to the GFD (Laurikka et al., 2018). Studies report different adherence rates ranging from 45%-90%, with a broad variability due to varying definitions and methods to determine adherence (Wieser et al., 2021). Another element complicating adherence percentages is that inadvertent ingestion, sometimes called accidental contamination, happens more frequently than intentional ingestion. Both forms of non-adherence damage the intestines and increase the risk of complications. Strict adherence to a GFD is challenging for several reasons, including high food costs, low palatability, availability limitations, cross-contamination, improper labeling, lack of support, and social consequences (Rodrigues et al., 2018; Wieser et al., 2021). Some individuals also report non-adherence due to the challenges of finding gluten-free options while eating out or traveling. Other reports show that poor adherence to a GFD may be partly related to a lack of knowledge about how to avoid gluten entirely (Namvar et al., 2021). Lack of follow-up care is another cause of non-adherence. Follow-up care should include monitoring adherence with both serology and patient reports, verifying the normalization of lab IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL 16 values and assessment abnormalities, assessing for complications, and consulting with a dietician (Rubio-Tapia et al., 2013). Follow-up serology can identify individuals who believe they comply with the GFD but still have inadvertent exposures. Appointments with a dietician or nutritionist can assist in identifying unknown sources of gluten contamination. Continued Monitoring Recommendations for children diagnosed with CD include monitoring for symptom improvement, return of abnormal lab values, growth and development, adherence to the GFD, and nutritional abnormalities while on the GFD. However, many reports differ in their description of how the monitoring should be conducted. The Celiac Disease Guideline Committee recommends evaluating CD-specific labs six months after patients begin the GFD to monitor diet adherence and recovery and then yearly if the patient is asymptomatic. Moreover, providers should continue to measure these labs in individuals with symptoms that persist after changing to a GFD because this can indicate intentional or unintentional non-adherence. Hill et al. (2005) recommend that children have periodic visits with their pediatrician to receive followup and monitoring of growth, diet adherence, disease complications, symptoms, and nutritional deficiencies. Provider Education Diagnosing and post-diagnosis monitoring are vital tasks for pediatricians. Preventing a delay in diagnosis is best accomplished by educating providers about the disease’s typical and atypical presentations. Unfortunately, many providers are unsure what, when, and how to monitor the disease to produce the best patient outcomes for children with CD. Elkin et al.’s (2018) quasi-experimental study of nurse practitioners (n = 25) showed improved confidence and knowledge in recognizing and treating CD after participating in an online learning module. IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL 17 Providers should also be educated about the disease, diagnosis processes, necessary dietary changes, potential complications from non-adherence to the diet, and future monitoring recommendations. When they understand these essential concepts, they are more prepared to educate patients, leading to improved adherence to the diet and decreased risk for complications (Ludvigsson et al., 2015). Besides individual patient education, group education has also proven beneficial to patient knowledge of the disease and diet adherence. The value of support groups should be discussed in the education providers share with their patients. Namvar et al. (2021) used a parallel design randomized control trial (n = 130) to show that group-based education regarding CD and a GFD is superior to individual-based education. Discussion The literature on CD shows the importance of early diagnosis and treatment to prevent complications associated with untreated disease. Several studies strengthen the validity of achieving a diagnosis without a biopsy in patients with high levels of TTG IgA, which aids in early diagnoses and eliminates unnecessary risks and costs. The literature also outlines the importance of assessing children for intestinal and extraintestinal symptoms, with added attention to children with conditions associated with an increased disease prevalence. Finally, the literature supports continuing to monitor for diet adherence, normalization of lab values, and possible complications. Implications for Practice The increasing prevalence of CD should align with pediatricians’ preparation to identify children with symptoms and risk factors, provide diagnostic care based on recent evidence, and monitor for complications. By meeting these outcomes, children with CD will be able to receive IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL 18 less invasive and faster diagnostic care, receive more comprehensive care from their primary care pediatricians, and have fewer risks of disease complications. Framework and Project Application This quality improvement project utilizes the Revised Iowa Model. A team of nurses developed this model from the University of Iowa Hospitals and Clinics and College of Nursing to outline the process for implementing quality improvement projects based on evidence-based practice (Iowa Model Collaborative, 2017). The process guides nurses and other clinicians in making decisions about day-to-day practice changes that will impact patient outcomes (Cullen et al., 2018). This model facilitates the implementation of an evidence-based pediatric CD care process model for use by the pediatricians at Bryner Pediatrics. The recent revision of this model adds to the strength of its application (Iowa Model Collaborative, 2017). Framework Description The Revised Iowa Model outlines several action steps and questions to guide the implementation of evidence-based practice process changes. The steps include (a) identifying triggering issues/opportunities; (b) stating the question or purpose; (c) deciding if the topic is a priority (d) forming a team; (e) assembling, appraising, and synthesizing the body of evidence; (f) deciding if there is sufficient evidence; (g) designing and piloting the practice change; (h) deciding if the change is appropriate for adoption in practice; (i) integrating and sustaining the practice change; and (j) disseminating the results (Iowa Model Collaborative, 2017). Framework Application to Project The Revised Iowa Model is used in planning, implementing, and evaluating quality improvement projects. This project aims to develop an evidence-based pediatric care process model to guide diagnosing and treating children with CD at Bryner Pediatrics. As an identified IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL 19 priority of the clinic, the project will move forward with a formulated team, including Dr. Carrie Johnson as the project consultant and representative of the pediatricians at the clinic; Dr. Anna Ermarth, a pediatric gastroenterologist at the Pediatric Gastroenterology Division, University of Utah Department of Pediatrics at Primary Children’s Hospital as the content expert; Kristy Baron, as the faculty advisor; and me as the DNP student. As the team leader, I summarize the evidence supporting the project and guide the care process model development. The project is piloted in the clinic and evaluated by Drs. Johnson and Ermarth for adoption into practice. Following adoption, steps are be identified for sustainability, and the results are disseminated. By addressing each step outlined in the Iowa Model Revised, the project is focused, based on evidence, sustainable, and shareable. Project Plan This project included designing and implementing a pediatric CD care process model in a Salt Lake City, Utah, pediatric clinic. The care process model outlined definitions, symptoms, clinical significance, diagnostic processes, evidence-based practice changes, clinical disease management, and resources for patients, families, and providers. In addition, the project plan included the project design, needs assessment, cost analysis, sustainability plan, project outcomes, ethical considerations, and evaluation instruments. Project Design The implementation of the CD care process model was a quality improvement project designed to improve the quality of care for children with CD. Provider education about symptom surveillance, diagnostic processes, and disease management potentially decreased diagnostic wait times and long-term disease complications while improving patient satisfaction and health outcomes. IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL 20 Needs Assessment/Gap Analysis of Project Site and Population This quality improvement project impacted Bryner Pediatrics, an IH pediatric clinic in downtown Salt Lake City, Utah, whose pediatricians have served a large pediatric patient population in the area. The pediatricians within the clinic have played a vital role in diagnosing and monitoring children with CD. Unfortunately, they felt unprepared for this role due to their lack of knowledge about diagnosis and treatment recommendations. Their lack of knowledge about recommended CD monitoring and follow-up placed children at an increased risk for nonadherence to the diet, disease complications, and nutritional deficiencies (Hill et al., 2005). Therefore, providing these pediatricians with a better awareness of the disease and treatment potentially improved the care, quality of life, and overall health of their patients with CD. The target population for the proposed project was the five pediatricians within the clinic, two of whom voiced their desire to improve their understanding of CD management. These pediatricians were the key stakeholders of the project, with additional stakeholders including the specialists at the Pediatric Gastroenterology Division at Primary Children’s Hospital, who have shared the burden of care for the pediatric patient population in the geographical area. Improving the care provided by the pediatric clinic possibly lessened the appointment wait times and patient burden on the gastroenterology clinic because some patients could be diagnosed by their primary care pediatrician and did not require appointments or biopsies by the specialists. Furthermore, through the standardization of CD care, their large, diverse patient population possibly received improved diagnostic care and disease follow-up and reduced risk of CD complications. Additionally, by facilitating CD diagnosis and treatment by pediatricians, many underserved and underinsured patients could receive comprehensive disease care. IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL 21 Cost Analysis and Sustainability of Project The minimal budgetary requirements for this project have been discussed with the project team and approved for physician education and printing. The pediatric clinic covered the project costs, including physician training time and printed training materials (see Appendix A). Approximately 25 printed bifold care process models were produced, providing 20 for Bryner Pediatrics and five for the Pediatric Gastroenterology Division, University of Utah Department of Pediatrics at Primary Children’s Hospital. In addition to printed copies, the digital version was given to Drs. Johnson and Ermarth, allowing them to print copies for patients and families. The sustainability of the process improvement project included maintaining printed materials by making updates based on evidence-based practice changes or by replacing the used and worn-out care process models as needed. Sustainability also included presenting the care process model to an IH pediatric service line representative to determine whether it can be applied to other pediatric clinics within the IH network. Project Outcomes The project outcomes were evaluated through goal measurement, including the following: • The care process model was evaluated for accuracy and completeness and approved for use by Dr. Ermarth, a pediatric gastroenterologist at the Pediatric Gastroenterology Division, University of Utah Department of Pediatrics at Primary Children’s Hospital, on September 30, 2022. • All five pediatricians attended the care process model introduction and training meeting at the physicians’ meeting on November 21, 2022. IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL • 22 The physicians reported improved comfort in diagnosing and treating CD in pediatric patients, as demonstrated by increased scores from pre- to post-instruction Likert scale Qualtrics surveys applied during the physicians’ meeting in November 2022. • After implementation at Bryner Pediatrics, the care process model was presented to Memorial Clinic, another IH pediatric clinic in Salt Lake City. If the implementation were successful at both pediatric clinics, the model would be presented to the IH pediatric service line specialists to determine its applicability in other pediatric clinics. Consent Procedures and Ethical Considerations Weber State University’s Institutional Review Board (IRB) determined that the project met the requirements for a quality improvement project and did not include human subject testing or research. IH’s IRB made the same determination about the nature of the project. As such, full IRB approval was not required for either Weber State University or IH; quality improvement project approval was obtained from both organizations. In addition, completion of the included Qualtrics survey was optional, anonymous, and confidential. Instruments to Measure Intervention Effectiveness Two instruments were used to evaluate the quality improvement project, one to evaluate the care process model and one to evaluate the intervention effectiveness. First, the AGREE II Instrument to evaluate clinical practice guidelines was utilized by Drs. Johnson and Ermarth to evaluate the care process model (AGREE Next Steps Consortium, 2017). Second, pre- and postinstructional Qualtrics surveys (see Appendices B and C) were utilized to evaluate the increase in the physicians’ self-reported comfort in surveilling, diagnosing, and treating CD in children. Finally, the compared results of the pre-and post-instructional Qualtrics surveys were used to determine the usefulness and applicability of the instructional content and care process model IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL 23 and whether the care process model should be presented to other IH pediatric clinics and the IH pediatric service line specialists. Project Implementation The care process model was implemented after the model was approved by the collaborating content expert and the physicians at the intended clinic, followed by educational training provided to the intended users. The new model was adopted at the pediatric clinic where the gap was initially identified, directing their pediatricians’ diagnostic and ongoing care for pediatric patients with CD. Project Interventions Project interventions were planned around recommending, implementing, and evaluating a pediatric CD care process model in a pediatric clinic. Collaboration with the content expert and pediatrician from the intended clinic was essential for effective implementation to ensure the accuracy and applicability of the care process model. Dr. Ermarth from the Pediatric Gastroenterology Division, University of Utah Department of Pediatrics at Primary Children’s Hospital, served as the CD content expert due to her research and experience treating children with the disease. Equally important, collaborating with Dr. Johnson as Bryner Clinic’s representative ensured the model was applicable within her clinic. PowerPoint Presentation Educating the providers about the appropriate use of the care process model was a critical aspect of the project implementation. Therefore, the project was presented to the pediatricians at their monthly physician meeting on November 21, 2022, with attendance by office administrators and all five clinic pediatricians. Included in the 45-minute training were a brief pre-instructional survey, a PowerPoint presentation, and a similar post-instructional survey. The IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL 24 physicians accessed and completed the pre- and post-surveys (see Appendices B and C) on their cell phones with a QR code in the PowerPoint. The surveys evaluated the physicians’ selfreported comfort in diagnosing and treating CD among pediatric patients. The PowerPoint presentation (see Appendix D) described the importance of proper and timely diagnosis and summarized the components of the care process model. A question-andanswer session followed the PowerPoint presentation, which allowed the pediatricians to discuss the information, ask questions, and request clarifying revisions to the care process model. Care Process Model The care process model, designed for pediatricians to facilitate disease surveillance, diagnosis, and management, was presented during the training meeting. The model outlined background information, including typical and atypical symptoms, prevalence, conditions associated with increased prevalence, and long-term disease complications. The model also included the algorithm used by the collaborating expert to guide the pediatricians through the diagnosing process. Furthermore, it outlined follow-up labs and ongoing disease monitoring recommendations. Finally, the care process model provided disease-related links and websites for providers and patients. The four-page bifold care process model was printed and given to all attendees (see Appendix E). Revisions were made to the model to address the concerns and questions communicated during and after the provider training. The revisions were completed in collaboration with Drs. Johnson and Ermarth. At her request, extra copies were provided for future use within the clinic and given to Dr. Ermarth. Digital copies were also provided to accommodate future printing needs. Additionally, updates to the digital copy can promote changes based on up-to-date disease treatment and management recommendations. IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL 25 Long-Term Program Outcome Interventions Following the short-term implementation outcome within the pediatric clinic, the longterm outcome of utilizing the care process model in other pediatric settings was pursued. First, this included providing the model to Dr. Ermarth at the Pediatric Gastroenterology Division, University of Utah Department of Pediatrics at Primary Children’s Hospital. Second, Dr. Johnson introduced the model to Memorial Clinic, a nearby IH pediatric clinic, with the goal of presenting it to IH’s pediatric service line representatives to evaluate its applicability in other pediatric clinics. Finally, collaborating with Dr. Ermarth on content accuracy and generalizability was essential to reach these long-term goals. Project Timeline The project timeline (see Appendix F) included the completion status for the project proposal and literature review, project plan, implementation, and evaluation. First, the project proposal was created in collaboration with the project consultant based on current evidencebased practices identified in the literature review. Second, the project plan included many steps, including presenting the proposal; obtaining WSU and IH IRB approval; and creating the care process model, PowerPoint presentation, and Qualtrics surveys. Third, project implementation involved presenting the care process model to the physicians, completing the pre-and postinstructional surveys, and implementing the care process model within the clinic. Finally, project evaluation involved analyzing the survey results, presenting the final project to faculty, and submitting the final report. Project Evaluation The project outcomes were met through educational training on the new pediatric CD care process model. The five participants completed a pre-intervention survey, which showed IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL 26 they were not entirely comfortable diagnosing and treating children with CD. However, postintervention survey results showed increased self-reported confidence and comfort in each category. These survey results supported the project goals and showed the project’s impact on guiding patient care for physicians in a pediatric clinic. Data Maintenance/Security The results of the pre- and post-surveys were anonymous and did not require personal information. The results were collected in Qualtrics and stored on a computer; both the program and the computer were password-protected. The results were reported in a manner that did not distinguish the participants’ responses. Data Collection and Analysis Two surveys were administered to the five participants, one immediately before the presentation and one immediately after (see Appendices B and C). The survey questions were created to measure physicians’ comfort in several categories of diagnosing and treating pediatric CD, the same categories that were outlined in the care process model. The surveys were similar, with one additional question added to the post-survey. They were administered with a Likert scale through Qualtrics, a program used to create and administer the surveys and then collect and evaluate the results. The results were reported in percentages for each Likert-scale category. In addition to survey results, qualitative feedback was also collected from the physicians after the presentation. They requested clarification on the care process model for the follow-up lab tests and yearly monitoring recommendations. They also asked that information about IgA deficiency be added to the model to direct physicians’ care when it is identified during the CD diagnostic process. Revisions to the model were made based on this feedback. IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL 27 Findings The survey data demonstrate that the project outcomes were met (see Table 1). Before (and after) the intervention, the physicians’ comfort in diagnosing and treating pediatric CD showed the following among the participants: 40% (100%) were comfortable recognizing gastrointestinal symptoms, 20% (50%) for non-gastrointestinal symptoms, 60% (100%) for associated diseases and conditions, 40% (100%) for which labs to order, 0% (75%) for which patients to refer for a biopsy, 60% (50%) for monitoring recommendations, and 0% (50%) for what education to provide to patients and families. The other Likert-scale categories also showed improvements, indicating that the presentation and new care process model improved physicians’ comfort in diagnosing and treating the disease in children. The last question was only included in the post-survey because they were given the diagnostic algorithm during the presentation, so their comfort with it could not be measured in the pre-survey. Table 1 Pre(Post)-Instructional Measures of the CD Care Process Model Survey Survey Question Q2. How comfortable are you recognizing the gastrointestinal symptoms of celiac disease in a pediatric patient? Q3. How comfortable are you recognizing the non-gastrointestinal symptoms of celiac disease in a pediatric patient? Q4. How comfortable are you recognizing the associated diseases and conditions that place pediatric patients at an increased risk for developing celiac disease? Q5. How comfortable are you knowing what labs to order for a patient suspected of having celiac disease? Q6. How comfortable are you knowing which patients should be referred for a diagnostic biopsy? Slightly Uncomfortable 0 (0) Percentage of Participants Neutral Slightly Comfortable 0 (0) 60 (0) Very Comfortable 40 (100) 0 (0) 0 (0) 80 (50) 20 (50) 0 (0) 0 (0) 40 (0) 60 (100) 20 (0) 0 (0) 40 (0) 40 (100) 40 (0) 20 (0) 40 (25) 0 (75) IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL Survey Question Q7. How comfortable are you with knowing the treatment and monitoring recommendations for a patient with celiac disease? Q8. How comfortable are you providing celiac disease education to patients? Q9. How comfortable are you using the diagnostic algorithm? Percentage of Participants Slightly Neutral Slightly Uncomfortable Comfortable 20 (0) 20 (0) 0 (50) 28 Very Comfortable 60 (50) 0 (0) 20 (0) 80 (50) 0 (50) N/A (0) N/A (0) N/A (0) N/A (100) Note: n = 5 (n = 4) This table demonstrates participant responses in the CD care process model survey before (and after) the training intervention. The survey results indicate that the lowest levels of physician comfort were in the questions regarding ordering diagnostic testing (labs and biopsy), monitoring after diagnosis, and recommending patient education. The care process model addressed these topics by including a diagnostic algorithm, listing the yearly monitoring recommendations, and outlining educational information and websites for patients and families. Strengths Participation in the presentation and surveys was voluntary, with survey responses anonymous. Two physicians were primarily invested in the project’s success as they requested the care process model to guide them in their care of patients. All physicians asked questions during and after the presentation, which indicated their interest in the project. Some feedback led to changes in the care process model to provide clarification, while others led to further conversations about evidence and best practices in CD care. The care process model went through a few additional revisions to address all the questions and concerns of the participants, with final approval by the CD content expert. All invested parties approved the final version. IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL 29 The diagnostic algorithm was selected due to its consistency with current evidence-based practices and changes. In addition, using a current and established algorithm provided accuracy, applicability, and consistency with the referring gastroenterology office, which also uses the same algorithm. Weaknesses A few limitations were noted during the study. First, the study included a small sample size (n = 5), leaving some uncertainty about the generalizability of the project to other clinics and environments. Second, although there were five total participants, only four completed the postsurvey for unknown reasons. Fewer completed post-surveys could have resulted from two participants joining via zoom from home, which may have posed challenges in completing the second survey. Third, the survey questions subjectively measured physicians’ self-reported comfort, which may be incongruent with competence. Discussion This quality improvement project implements a pediatric CD care process model to assist pediatricians in recognizing, diagnosing, and managing CD in their pediatric patients. The interventions include presenting the care process model to the pediatricians in Bryner Pediatrics, a primary care pediatric clinic. The care process model is based on previous evidence revealing best practices for patients with CD. Baseline data reveals that some pediatricians were not entirely comfortable diagnosing and treating patients with CD. However, project findings demonstrate increased comfort after participation in the care process model training. This project reflects evidence translation, improving health outcomes and supporting future scholarship. IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL 30 Translation of Evidence into Practice The new care process model integrated evidence into practice in a pediatric clinic by outlining diagnostic process changes, pediatric disease monitoring, and yearly assessment recommendations. For example, recent evidence has shown that many children can be diagnosed with CD without a confirmation biopsy, reducing diagnostic wait times and the number of specialist referrals (Ermarth et al., 2017). In addition, reduced numbers of biopsies can lead to improved patient outcomes and satisfaction. Furthermore, identifying common and less common pediatric disease symptoms and conditions associated with increased CD prevalence can enable pediatricians to recognize the disease quickly. After diagnosis, the yearly monitoring recommendations can help physicians expand their patient care and improve pediatric health by reducing the chances of disease complications (Hill et al., 2005; Laurikka et al., 2018; Ludvigsson et al., 2015; Mearin, 2007; Zipser et al., 2005). Implications for Practice and Future Scholarship The survey results demonstrate that the project outcomes were met; the target pediatricians report increased comfort in diagnosing and treating pediatric CD. Based on this project, opportunities for future practice scholarship of potential projects are possible in two ways. First, quantifying the number of patients diagnosed using this care process model can help establish its validity in reducing the time from symptom presentation to diagnosis and the number of biopsies performed. Second, the project consultant, Dr. Johnson, is introducing the care process model to the physicians at Memorial Clinic, a nearby IH pediatric clinic. If successfully implemented at Memorial Clinic, it could be presented to the IH pediatric service line representatives for implementation in other pediatric clinics. Broader implementation of the care process model can positively impact CD outcomes and patient satisfaction. IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL 31 Sustainability Project sustainability is encouraging as there is no cost to using the digital format of the care process model. The pediatricians at Bryner Pediatrics plan to use the digital version in the care of their patients, with printed models available for patients and families. Similarly, Dr. Ermarth plans to do the same at her clinic at the Pediatric Gastroenterology Division at Primary Children’s Hospital. In addition, because the care process model included feedback from all interested parties, it represents a more valid, user-friendly, and applicable tool. Revisions were made based on feedback from the project consultant, the other target pediatricians, and the CD content expert. The requested revisions include content and process clarifications. Therefore, revisions agreed upon by all parties facilitate the creation of an accepted care process model. Dissemination Dissemination of this quality improvement project includes presenting the results as a poster presentation at the Western Institute of Nursing conference in April 2023. Additionally, this project is available through the WSU Stewart Library Repository as an online resource. Employing additional dissemination methods may lead to future related practice implications. Conclusion Delayed diagnoses or incomplete CD care lead to short- and long-term disease complications for children with the condition. Utilizing a pediatric CD care process model can guide pediatricians in recognizing, diagnosing, and treating CD in children. The project intervention includes five physicians at Bryner Pediatrics who attended training on the new care process model, which outlined symptoms, associated conditions, a diagnostic algorithm, recommendations for yearly monitoring, and important patient education. After the training, the IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL results indicate that pediatricians reported increased comfort in recognizing, diagnosing, and treating their pediatric patients with the condition. Therefore, an evidence-based care process model can standardize CD care among pediatricians. 32 IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL 33 References Afzal, E., Khan, S., Ali, Ibad, Aftab, K., Arshad, R., & Abbas, A. (2020). Developmental status in children with severe acute malnutrition. Journal of Islamic International Medical College, 15(4), 245-249. http://jiimc.riphah.edu.pk/wp-content/uploads/2020/12/9-ErumAfzal.pdf AGREE Next Steps Consortium. (2017). The AGREE II Instrument [Electronic version]. https://www.agreetrust.org/ American Association of Colleges of Nursing. (2021). The essentials: Core competencies for professional nursing education. https://aacnnursing.org/AACN-Essentials Barker, C. C., Mitton, C., Jevon, G., & Mock, T. (2005). Can tissue transglutaminase antibody titers replace small-bowel biopsy to diagnose celiac disease in select pediatric populations? Pediatrics, 115(5), 1341-1346. https://doi.org/10.1542/peds.2004-1392 Bingham, S. M., & Bates, M. D. (2020). Pediatric celiac disease: A review for nongastroenterologists. Current Problems in Pediatric and Adolescent Health Care, 50(5), 16. https://doi.org/10.1016/j.cppeds.2020.100786 Bishop, J., Reed, P., Austin, P., Hurst, M., Ameratunga, R., Craigie, A., McFarlane, J., Chin, S. E., Mouat, S. M., & Evans, H. M. (2018). Prospective evaluation of the ESPGHAN Guidelines for diagnosis of celiac disease in New Zealand children. Journal of Pediatric Gastroenterology and Nutrition, 67(6), 749-754. https://doi.org/10.1097/MPG.0000000000002065 Cullen, L., Hanrahan, K., Farrington, M., DeBerg, J., Tucker, S., & Kleiber, C. (2018). Evidence-based practice in action: Comprehensive strategies, tools, and tips from the IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL 34 University of Iowa Hospitals and Clinics. Sigma Theta Tau International. ISBN: 9781940446936 Di Nardo, G., Villa, M. P., Conti, L., Ranucci, G., Pacchiarotti, C., Principessa, L., Raucci, U., & Parisi, P. (2019). Nutritional deficiencies in children with celiac disease resulting from a gluten-free diet: A systematic review. Nutrients, 11(1588), 1-12. https://www.doi.org/10.3390/nu11071588 Elkin, A., Grant, C., Coleman, T., & Sereika, S. M. (2018). Use of an educational model to improve confidence and knowledge of celiac disease among nurse practitioners in Pennsylvania. Gastroenterology Nursing, 41(5), 412-423. http://doi.org/10.1097/SGA.0000000000000336 Ermarth, A., Bryce, M., Woodward, S., Stoddard, G., Book, L., & Jensen, M. K. (2017). Identification of pediatric patients with celiac disease based on serology and a classification and regression tree analysis. Clinical Gastroenterology and Hepatology, 15(3), 396-402. https://doi.org/10.1016/j.cgh.2016.10.035 Hill, I. D., Dirks, M. H., Liptak, G. S., Colletti, R. B., Fasano, A., Guandalini, S., Hoffenberg, E. J., Horvath, K., Murray, J. A., Pivor, M., & Seidman, E. G. (2005). Guideline for the diagnosis and treatment of celiac disease in children: Recommendations of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition. Journal of Pediatric Gastroenterology and Nutrition, 40(1), 1-19. https://journals.lww.com/jpgn/Fulltext/2005/01000/Guideline_for_the_Diagnosis_and_Tr eatment_of.1.aspx Husby, S., Murray, J. A., & Katzka, D. A. (2019). AGA clinical practice update on diagnosis and monitoring of celiac disease: Changing utility of serology and histologic measures: IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL 35 Expert review. Gastroenterology, 156(4), 885-889. https://doi.org/10.1053/j.gastro.2018.12.010 Iowa Model Collaborative. (2017). Iowa Model of Evidence-Based Practice: Revisions and validation. Worldviews on Evidence-Based Nursing, 14(3), 175-182. https://doi:10.1111/wvn.12223 King, J. A., Jeong, J., Underwood, F. E., Quan, J., Panaccione, N., Windsor, J. W., Coward, S., deBruyn, J., Ronksley, P. E., Shaheen, A.-A., Quan, H., Godley, J., van Zanten, S. V., Lebwohl, B., Ng, S. C., Ludvigsson, J. F., & Kaplan, G. G. (2020). Incidence of celiac disease is increasing over time: A systematic review and meta-analysis. American Journal of Gastroenterology, 115(4), 507-525. https://doi.org/10.14309/ajg.0000000000000523 Klapp, G., Masip, E., Bolonio, M., Donat, E., Polo, B., Ramos, D., & Ribes-Koninckx, C. (2013). Celiac disease: The new proposed ESPGHAN diagnostic criteria do work well in a selected population. Journal of Pediatric Gastroenterology and Nutrition, 56(3), 251256. https://doi.org/10.1097/MPG.0b013e318279887b Laurikka, P., Nurminen, S., Kivela, L., & Kurppa, K. (2018). Extraintestinal manifestations of celiac disease: Early detection for better long-term outcomes. Nutrients, 10(8), 1-14. https://doi.org/10.3390/nu10081015 Ludvigsson, J. F., Card, T., Ciclitira, P. J., Swift, G. L., Nasr, I., Sanders, D. S., & Ciacci, C. (2015). Support for patients with celiac disease: A literature review. United European Gastroenterology Journal, 3(2), 146-159. https://doi.org/10.1177/2050640614562599 IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL 36 Mearin, M. L. (2007). Celiac disease among children and adolescents. Current Problems in Pediatric and Adolescent Health Care, 37(3), 86-105. https://doi.org/10.1016/j.cppeds.2007.01.001 Namvar, Z. A., Mahdavi, R., Shirmohammadi, M., & Nikniaz, Z. (2021). The effect of groupbased education on knowledge and adherence to a gluten-free diet in patients with celiac disease: Randomized controlled clinical trial. International Journal of Behavioral Medicine, 28, 583-590. https://doi.org/10.1007/s12529-020-09949-7 Oliveira, G. N., Mohan, R., & Fagbemi, A. (2018). Review of celiac disease presentation in a pediatric tertiary centre. Arguivos de Gastroenterologia, 55(1), 86-93. https://doi.org/10.1590/S0004-2803.201800000-17 Remes-Troche, J. M., Uscanga-Dominguez, L. F., Aceves-Tavares, R. G., Calderon de la Barca, A. M., Carmona-Sanchez, R. I., Cerda-Contreras, E., Coss-Adame, E., Icaza-Chavez, M. E., Lopez-Colombo, A., Milke-Garcia, M. P., Morales-Arambula, M., Pelaez-Luna, M., Ramos Martinez, P., Sanchez-Sosa, S., Trevino-Mejia, M. C., Vazquez-Frias, R., Worona-Dibner, L. B., Zamora-Nava, L. E., & Rubio-Tapia, A. (2018). Clinical guidelines on the diagnosis and treatment of celiac disease in Mexico. Revista De Gastroenterologia De Mexico, 83(4), 434-450. https://doi.org/10.1016/j.rgmxen.2018.09.007 Rodrigues, M., Yonamine, G. H., & Satiro, C. A. F. (2018). Rate and determinants of nonadherence to a gluten-free diet and nutritional status assessment in children and adolescents with celiac disease in a tertiary Brazilian referral center: A cross-sectional and retrospective study. BMC Gastroenterology, 18(15), 1-8. https://doi.org/10.1186/212876-018-0740-z IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL 37 Rosen, A., Ivarsson, A., Nordyke, K., Karlsson, E., Carlsson, A., Danielsson, L., Hogberg, L., & Emmelin, M. (2011). Balancing health benefits and social sacrifices: A qualitative study of how screening-detected celiac disease impacts adolescents’ quality of life. BMC Pediatrics, 11(32), 1-10. https://doi.org/10.1186/1471-2431-11-32 Rubio-Tapia, A., Hill, I. D., Kelly, C. P., Calderwood, A. H., & Murray, J. A. (2013). ACG clinical guidelines: Diagnosis and management of celiac disease. American Journal of Gastroenterology, 108, 657-676. https://www.doi.org/10.1038/ajg.2013.79 Rubio-Tapia, A., Kyle, R. A., Kaplan, E. L., Johnson, D. R., Page, W., Erdtmann, F., Brantner, T. L., Kim, W. R., Phelps, R. K., Lahr, B. D., Zinsmeister, A. R., Melton, L. J., Murray, J. A. (2009). Increased prevalence and mortality in undiagnosed celiac disease. Gastroenterology, 137(1), 88-93. https://doi.org/10.1053/j.gastro.2009.03.059 Russo, C., Wolf, R. L., Leichter, H. J., Lee, A. R., Reilly, N. R., Zybert, P., Green, P. H. R., & Lebwohl, B. (2020). Impact of a child’s celiac disease diagnosis and management on the family. Digestive Diseases and Sciences, 65, 2959-2969. https://doi.org/10.1007/s10620020-06316-0 Scanion, S. A., & Murray, J. A. (2011). Update on celiac disease – etiology, differential diagnosis, drug targets, and management advances. Clinical and Experimental Gastroenterology, 2011(4), 297-311. https://doi.org/10.2147/CEG.S8315 Webb, C., Norstrom, F., Myleus, A., Ivarsson, A., Halvarsson, B., Hogberg, L., Lagerqvist, C., Rosen, A., Sandstrom, O., Stenhammar, L., & Carlsson, A. (2015). Celiac disease can be predicted by high levels of anti-tissue transglutaminase antibodies in population-based screening. Journal of Pediatric Gastroenterology and Nutrition, 60(6), 787-791. https://doi.org/10.1097/MPG.0000000000000688 IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL 38 Wieser, H., Ruiz-Carnicer, A., Segura, V., Comino, I., & Sousa, C. (2021). Challenges of monitoring the gluten-free diet adherence in the management and follow-up of patients with celiac disease. Nutrients, 13(7), 1-22. https://doi.org/10.3390/nu13072274 Zipser, R. D., Farid, M., Baisch, D., Patel, B., & Patel, D. (2005). Physician awareness of celiac disease: A need for further education. Journal of General Internal Medicine, 20(7), 644646. https://doi.org/10.1111/j.1525-1497.2005.0107.x IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL Appendix A Project Budget Items Cost per Unit Number of Unis Total Cost Physician Training (hours) $100 5 $500.00 Bifold Care Process Model Copies $3.30 25 $82.50 Total $582.50 39 IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL 40 Appendix B Care for the Pediatric Patient with Celiac Disease Pre-Instructional Survey Q1 The following is a pre-instructional online survey that takes approximately 5 minutes to complete. The survey questions will be about diagnosing and managing celiac disease in children. By participating in this survey, you are giving your consent. The results of this survey are reported using aggregated data, keeping responses anonymous and confidential. Data will be used for educational or quality improvement purposes to improve outcomes. If you have any questions, please contact Chelsea Pike (chelseapike@weber.edu). o I agree to participate o I do not agree to participate Q2 How comfortable are you recognizing the gastrointestinal symptoms of celiac disease in a pediatric patient? o Very comfortable o Slightly comfortable o Neutral o Slightly uncomfortable o Very uncomfortable Q3 How comfortable are you recognizing the non-gastrointestinal symptoms of celiac disease in a pediatric patient? o Very comfortable o Slightly comfortable o Neutral o Slightly uncomfortable o Very uncomfortable IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL 41 Q4 How comfortable are you recognizing the associated diseases and conditions that place pediatric patients at an increased risk for developing celiac disease? o Very comfortable o Slightly comfortable o Neutral o Slightly uncomfortable o Very uncomfortable Q5 How comfortable are you knowing what labs to order for a patient suspected of having celiac disease? o Very comfortable o Slightly comfortable o Neutral o Slightly uncomfortable o Very uncomfortable Q6 How comfortable are you knowing which patients should be referred for a diagnostic biopsy? o Very comfortable o Slightly comfortable o Neutral o Slightly uncomfortable o Very uncomfortable Q7 How comfortable are you with knowing the treatment and monitoring recommendations for a patient with celiac disease? o Very comfortable o Slightly comfortable o Neutral o Slightly uncomfortable o Very uncomfortable IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL Q8 How comfortable are you providing celiac disease education to patients? o Very comfortable o Slightly comfortable o Neutral o Slightly uncomfortable o Very uncomfortable Powered by Qualtrics 42 IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL 43 Appendix C Care for the Pediatric Patient with Celiac Disease Post-Instructional Survey Q1 The following is a post-instructional online survey that takes approximately 5 minutes to complete. The survey questions will be about diagnosing and managing celiac disease in children. By participating in this survey, you are giving your consent. The results of this survey are reported using aggregated data, keeping responses anonymous and confidential. Data will be used for educational or quality improvement purposes to improve outcomes. If you have any questions, please contact Chelsea Pike (chelseapike@weber.edu). o I agree to participate o I do not agree to participate Q2 How comfortable are you recognizing the gastrointestinal symptoms of celiac disease in a pediatric patient? o Very comfortable o Slightly comfortable o Neutral o Slightly uncomfortable o Very uncomfortable Q3 How comfortable are you recognizing the non-gastrointestinal symptoms of celiac disease in a pediatric patient? o Very comfortable o Slightly comfortable o Neutral o Slightly uncomfortable o Very uncomfortable IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL 44 Q4 How comfortable are you recognizing the associated diseases and conditions that place pediatric patients at an increased risk for developing celiac disease? o Very comfortable o Slightly comfortable o Neutral o Slightly uncomfortable o Very uncomfortable Q5 How comfortable are you knowing what labs to order for a patient suspected of having celiac disease? o Very comfortable o Slightly comfortable o Neutral o Slightly uncomfortable o Very uncomfortable Q6 How comfortable are you knowing which patients should be referred for a diagnostic biopsy? o Very comfortable o Slightly comfortable o Neutral o Slightly uncomfortable o Very uncomfortable Q7 How comfortable are you with knowing the treatment and monitoring recommendations for a patient with celiac disease? o Very comfortable o Slightly comfortable o Neutral o Slightly uncomfortable o Very uncomfortable IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL Q8 How comfortable are you providing celiac disease education to patients? o Very comfortable o Slightly comfortable o Neutral o Slightly uncomfortable o Very uncomfortable Q9 How comfortable are you using the diagnostic algorithm? o Very comfortable o Slightly comfortable o Neutral o Slightly uncomfortable o Very uncomfortable Powered by Qualtrics 45 IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL Appendix D PowerPoint Presentation Pediatric Celiac Disease Care Process Model By Chelsea Pike, MSN, RN, CNE WSU DNP Student Pre-Instructional Survey Physicians Only Please scan this QR code to take a short survey about diagnosing and managing celiac disease in children. 46 IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL Background Information v Definition v Significance v Prevalence How to Utilize the Care Process Model 1. 2. 3. 4. Recognize Diagnose Treat Monitor Image 1 47 IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL Recognize Gastrointestinal Symptoms of CD in Children The following symptoms are commonly seen in children with celiac disease: • Diarrhea • Constipation • Abdominal pain • Flatulence • Weight loss • Bloating • Poor appetite • Poor weight gain Image 2 48 IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL Extraintestinal Symptoms of CD in Children The following symptoms are less commonly seen in children with celiac disease: • Headache • Alopecia • Delayed puberty • Anemia • Myalgias • Abnormal liver function • Bone disease • Short stature • Irritability/Anxiety • Skin disorders • Amenorrhea • Dermatitis Herpetiformis • Arthritis • Arthralgias • Peripheral neuropathy • Ataxia • Stomatitis • Dental enamel hypoplasia • Depression • Tiredness • Nutritional deficiencies Image 3 Associated Diseases and Disorders Autoimmune Disorders • Type 1 Diabetes* • Autoimmune Thyroid Disease • Autoimmune IgA Deficiency Syndromes • Down Syndrome* • Williams Syndrome • Turner Syndrome Children with first-degree relatives with CD Image 4 49 IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL Diagnose Diagnostic Algorithm Celiac Disease Testing for Symptomatic Individuals Click here for topics associated with this algorithm INDICATIONS FOR TESTINGa Chronic and unexplained gastroenterologic symptoms, poor growth, weight loss, iron deficiency anemia, and delayed puberty, among other symptoms Test Key tTG IgA and Total IgA Celiac Antibodies, Tissue Transglutaminase (tTG), IgA and IgA, Total Immunoglobin tTG IgG Tissue Transglutaminase Antibody, IgG DGP IgA Deamidated Gliadin Peptide (DGP) Antibody, IgA DGP IgG Deamidated Gliadin Peptide (DGP) Antibody, IgG EMA IgA Endomysial Antibody, IgA by IFA EMA IgG Endomysial Antibody, IgG HLA Celiac Disease HLA-DQ Genotyping Normal IgA levels Negative tTG IgA result (≤3 U/mL) ORDER tTG IgA and Total IgAb Undetectable IgA levels (<7.0 mg/dL) Negative tTG IgA result (≤3 U/mL) Normal IgA levels Weak-moderate positive tTG IgA (4-40 U/mL)c Normal IgA levels High positive tTG IgA (≥41 U/mL) ORDER EMA IgA OR DGP IgA ORDER EMA IgA (preferred) OR DGP IgA Suggests selective IgA deficiency Celiac disease unlikely Abbreviations ESPGHAN European Society of Paediatric Gastroenterology, Hepatology and Nutrition HLA Human leukocyte antigen IFA Indirect fluorescent antibody IgA Immunoglobin A IgG Immunoglobin G ORDER tTG IgG Negative Positive Negative Positive Celiac disease unlikely ORDER EMA IgG (preferred) OR DGP IgG Negative Negative Positive EMA CONSIDER Biopsy AND HLA genotyping Celiac disease confirmed CONSIDER HLA genotyingd Negative CONSIDER HLA genotyingd Negative EMA/DGP OR Positive DGP Positive Positive Celiac disease ruled out CONSIDER Biopsy Positive Marsh 0-1 Marsh ≥2 • Consider early-phase disease; continue gluten-containing diet and retest after 1-3 months • Consider false-positive serology Celiac disease confirmed Celiac disease ruled out a All testing should be performed when patient is on a gluten-containing diet. b Celiac Dual Antigen Screen with Reflex includes tTG IgA, DGP IgA, tTG IgG, and DGP IgG and may be ordered with Immunoglobulin A as an alternate initial testing option. c ESPGHAN recommends that weak-to-moderate positive tTG IgA results be followed directly by biopsy. d Consider testing if there is risk of false seronegativity. References Husby S, Koletzko S, et al. European Society Paediatric Gastroenterology, Hepatology and Nutrition guidelines for diagnosing coeliac disease 2020. J Pediatr Gastroenterol Nutr. 2020;70(1):141-156. Leonard MM, Lebwohl B, Rubio-Tapia A, et al. AGA clinical practice update on the evaluation and management of seronegative enteropathies: expert review. Gastroenterology. 2021;160(1):437-444. © 2021 ARUP Laboratories. All Rights Reserved. Revised www.arupconsult.com Content reviewed: December 2021 (ARUP Laboratories, 2022) Last updated: February 2022 50 IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL INITIAL DIAGNOSTIC LABS The initial labs to order include the following: • Immunoglobulin A • TTG IgA Image 5 REMEMBER: Patients must be on a gluten-containing diet for at least 3 months to be tested for CD FOLLOW-UP DIAGNOSTIC LABS Ø If initial labs show normal IgA levels AND high positive TTG IgA, order the following 4 weeks after the initial laboratory test • TTG IgA • EMA IgA • TSH with reflex to free T4 Ø Follow the ARUP algorithm for repeat diagnostic laboratory studies and/or biopsy indications Image 6 51 IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL Treat Treatment 1. Initiate a gluten-free diet (GFD) AFTER diagnosis 2. Refer patients for a consult with a dietician 3. Some patients will benefit from a low-lactose diet initially Please Note Failure to see symptom improvement and/or a decline in TTG IgA levels after 6 months on a GFD suggests continued gluten ingestion or complicated disease 52 IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL RECOVERY LABS/ASSESSMENTS Assess the following after 6 months on a gluten-free diet • TTG IgA • Nutrition • CBC • Growth and development Image 7 Monitor 53 IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL After Diagnosis: Yearly Monitoring/Assessments • Adherence to GFD (history and serology) • TSH with reflex to free T4 • CBC DON’T FORGET • Growth and development Reassess TTG IgA in children with persistent or recurrent • Disease complications disease symptoms Resources Patients and Families • National Library of Medicine • National Celiac Association • Celiac Disease Foundation • Celiac.com • GIKids Providers • Medical Home Portal • IgA Deficiency Information Image 8 54 IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL Post-Instructional Survey Physicians Only Please scan this QR code to retake the same short survey about diagnosing and managing celiac disease in children. References • ARUP Laboratories. (2022). Celiac Disease Testing for Symptomatic Individuals. ARUP Consult. https://arupconsult.com/algorithm/celiac-disease-testing-symptomaticindividuals-algorithm • Barker, C. C., Mitton, C., Jevon, G., & Mock, T. (2005). Can tissue transglutaminase antibody titers replace small-bowel biopsy to diagnose celiac disease in select pediatric populations? Pediatrics, 115(5), 1341-1346. • Bingham, S. M., & Bates, M. D. (2020). Pediatric celiac disease: A review for non-gastroenterologists. Current Problems in Pediatric and Adolescent Health Care, 50(5), 1-6. • Bishop, J., Reed, P., Austin, P., Hurst, M., Ameratunga, R., Craigie, A., McFarlane, J., Chin, S. E., Mouat, S. M., & Evans, H. M. (2018). Prospective evaluation of the ESPGHAN Guidelines for diagnosis of celiac disease in New Zealand children. Journal of Pediatric Gastroenterology and Nutrition, 67(6), 749-754. • Di Nardo, G., Villa, M. P., Conti, L., Ranucci, G., Pacchiarotti, C., Principessa, L., Raucci, U., & Parisi, P. (2019). Nutritional deficiencies in children with celiac disease resulting from a gluten-free diet: A systematic review. Nutrients, 11(1588), 1-12. • Elkin, A., Grant, C., Coleman, T., & Sereika, S. M. (2018). Use of an educational model to improve confidence and knowledge of celiac disease among nurse practitioners in Pennsylvania. Gastroenterology Nursing, 41(5), 412-423. • Ermarth, A., Bryce, M., Woodward, S., Stoddard, G., Book, L., & Jensen, M. K. (2017). Identification of pediatric patients with celiac disease based on serology and a classification and regression tree analysis. Clinical Gastroenterology and Hepatology, 15(3), 396-402. • Hill, I. D., Dirks, M. H., Liptak, G. S., Colletti, R. B., Fasano, A., Guandalini, S., Hoffenberg, E. J., Horvath, K., Murray, J. A., Pivor, M., & Seidman, E. G. (2005). Guideline for the diagnosis and treatment of celiac disease in children: Recommendations of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition. Journal of Pediatric Gastroenterology and Nutrition, 40(1), 1-19. • Husby, S., Murray, J. A., & Katzka, D. A. (2019). AGA clinical practice update on diagnosis and monitoring of celiac disease: Changing utility of serology and histologic measures: Expert review. Gastroenterology, 156(4), 885-889. 55 IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL References (continued) • Klapp, G., Masip, E., Bolonio, M., Donat, E., Polo, B., Ramos, D., & Ribes-Koninckx, C. (2013). Celiac disease: The new proposed ESPGHAN diagnostic criteria do work well in a selected population. Journal of Pediatric Gastroenterology and Nutrition, 56(3), 251-256. • Laurikka, P., Nurminen, S., Kivela, L., & Kurppa, K. (2018). Extraintestinal manifestations of celiac disease: Early detection for better long-term outcomes. Nutrients, 10(8), 1-14. • Mearin, M. L. (2007). Celiac disease among children and adolescents. Current Problems in Pediatric and Adolescent Health Care, 37(3), 86-105. • Oliveira, G. N., Mohan, R., & Fagbemi, A. (2018). Review of celiac disease presentation in a pediatric tertiary centre. Arguivos de Gastroenterologia, 55(1), 86-93. • Remes-Troche, J. M., Uscanga-Dominguez, L. F., Aceves-Tavares, R. G., Calderon de la Barca, A. M., Carmona-Sanchez, R. I., Cerda-Contreras, E., Coss-Adame, E., Icaza-Chavez, M. E., Lopez-Colombo, A., Milke-Garcia, M. P., Morales-Arambula, M., Pelaez-Luna, M., Ramos Martinez, P., Sanchez-Sosa, S., Trevino-Mejia, M. C., Vazquez-Frias, R., Worona-Dibner, L. B., Zamora-Nava, L. E., & Rubio-Tapia, A. (2018). Clinical guidelines on the diagnosis and treatment of celiac disease in Mexico. Revista De Gastroenterologia De Mexico, 83(4), 434-450. • Rosen, A., Ivarsson, A., Nordyke, K., Karlsson, E., Carlsson, A., Danielsson, L., Hogberg, L., & Emmelin, M. (2011). Balancing health benefits and social sacrifices: A qualitative study of how screening-detected celiac disease impacts adolescents’ quality of life. BMC Pediatrics, 11(32), 1-10. • Rubio-Tapia, A., Hill, I. D., Kelly, C. P., Calderwood, A. H., & Murray, J. A. (2013). ACG clinical guidelines: Diagnosis and management of celiac disease. American Journal of Gastroenterology, 108, 657-676. • Russo, C., Wolf, R. L., Leichter, H. J., Lee, A. R., Reilly, N. R., Zybert, P., Green, P. H. R., & Lebwohl, B. (2020). Impact of a child’s celiac disease diagnosis and management on the family. Digestive Diseases and Sciences, 65, 2959-2969. • Scanion, S. A., & Murray, J. A. (2011). Update on celiac disease – etiology, differential diagnosis, drug targets, and management advances. Clinical and Experimental Gastroenterology, 2011(4), 297-311. • Webb, C., Norstrom, F., Myleus, A., Ivarsson, A., Halvarsson, B., Hogberg, L., Lagerqvist, C., Rosen, A., Sandstrom, O., Stenhammar, L., & Carlsson, A. (2015). Celiac disease can be predicted by high levels of anti-tissue transglutaminase antibodies in population-based screening. Journal of Pediatric Gastroenterology and Nutrition, 60(6), 787-791. References (continued) • Images Image 1: Pixabay. (2022). [Clip art]. https://pixabay.com/images/id-1207270/ Image 2: Pixabay. (2022). [Clip art]. https://pixabay.com/images/id-3176411/ Image 3: Pixabay. (2022). [Clip art]. https://pixabay.com/images/id-3273216/ Image 4: Pixabay. (2022). [Clip art]. https://pixabay.com/images/id-2639869/ Image 5: Pixabay. (2022). [Clip art]. https://pixabay.com/images/id-4303024/ Image 6: Pixabay. (2022). [Clip art]. https://pixabay.com/images/id-1847346/ Image 7: Pixabay. (2022). [Clip art]. https://pixabay.com/images/id-2458540/ Image 8: Pixabay. (2022). [Clip art]. https://pixabay.com/images/id-1073638/ 56 IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL Appendix E Pediatric Celiac Disease Care Process Model 1 Care Process Model January 2023 PRIMARY CARE MANAGEMENT OF Celiac Disease in Pediatric Patients Ages 2-18 years This care process model was developed for the pediatricians at Bryner Pediatrics by Chelsea Pike, MSN, RN, DNP Student, in collaboration with the Pediatric Gastroenterology Division, University of Utah Department of Pediatrics. This model includes current recommendations for an evidencebased approach to diagnosing, monitoring, and managing celiac disease in pediatric patients ages 2-18 years old. WHAT IS CELIAC DISEASE (CD)? CD is a chronic, systemic, autoimmune, enteropathic disorder triggered by the ingestion of dietary gluten found in wheat, barley, rye, and oats that are contaminated in the milling process.14 WHY IS PEDIATRIC CD MANAGEMENT IMPORTANT? Undiagnosed or untreated CD is associated with many short- and long-term health risks involving every system of the body, many resulting from the malabsorption associated with damage to the small intestines. In addition to physiologic consequences, there are also many psychological consequences of untreated CD. WHAT ARE THE SYMPTOMS OF CELIAC DISEASE? More common, gastrointestinal symptoms1,13-15 • • • • Diarrhea Constipation Abdominal pain Flatulence • • • • Weight loss Bloating Poor appetite Poor weight gain Less common, extraintestinal symptoms 4,10-13,15,17 • • • • • • • • • • • Headache Anemia Bone disease Skin disorders Arthritis Ataxia Depression Myalgias Short stature Alopecia Delayed puberty • • • • • • • • • • Amenorrhea Arthralgias Abnormal liver function Stomatitis Tiredness Irritability/anxiety Dental enamel hypoplasia Dermatitis herpetiformis Peripheral neuropathy Nutritional deficiencies WHAT IS THE PREVALENCE OF CD? 1% in the general population16 85-90% of people with CD are undiagnosed due to absent or non-classic symptoms.5,10-12 WHAT DISEASES OR CONDITIONS HAVE A HIGHER CD ASSOCIATION THAN THE NATURAL POPULATION?7,8,11,12,16 Autoimmune Disorders • Type 1 diabetes • Autoimmune thyroiditis • Autoimmune IgA deficiency Syndromes • Down syndrome • Turner syndrome • William syndrome First-degree relatives with CD CELIAC DISEASE VS. GLUTEN INTOLERANCE • CD is a chronic autoimmune intestinal disease indicating a permanent sensitivity to gluten. Even in the absence of symptoms, noncompliance to a gluten-free diet (GFD) is associated with many health risks. • Gluten intolerance does not affect the intestinal lining or absorption of nutrients but may cause irritable bowel symptoms. 57 Test Key Positive Celiac disease confirmed • Consider early-phase disease; continue gluten-containing diet and retest after 1-3 months • Consider false-positive serology CONSIDER Biopsy Positive Marsh ≥2 Negative ORDER EMA IgG (preferred) OR DGP IgG Positive Positive CONSIDER Biopsy AND HLA genotyping Negative EMA/DGP OR Positive DGP © 2021 ARUP Laboratories. All Rights Reserved. Revised www.arupconsult.com Celiac disease confirmed Positive EMA Content reviewed: December 2021 Last updated: February 2022 All testing should be performed when patient is on a gluten-containing diet. Celiac Dual Antigen Screen with Reflex includes tTG IgA, DGP IgA, tTG IgG, and DGP IgG and may be ordered with Immunoglobulin A as an alternate initial testing option. c ESPGHAN recommends that weak-to-moderate positive tTG IgA results be followed directly by biopsy. d Consider testing if there is risk of false seronegativity. b a Celiac disease ruled out Negative CONSIDER HLA genotyingd Negative ORDER EMA IgA (preferred) OR DGP IgA ORDER EMA IgA OR DGP IgA Positive Normal IgA levels High positive tTG IgA (≥41 U/mL) Abbreviations ESPGHAN European Society of Paediatric Gastroenterology, Hepatology and Nutrition HLA Human leukocyte antigen IFA Indirect fluorescent antibody IgA Immunoglobin A IgG Immunoglobin G Normal IgA levels Weak-moderate positive tTG IgA (4-40 U/mL)c Marsh 0-1 Celiac disease unlikely Negative ORDER tTG IgG Suggests selective IgA deficiency Undetectable IgA levels (<7.0 mg/dL) Negative tTG IgA result (≤3 U/mL) ORDER tTG IgA and Total IgAb INDICATIONS FOR TESTINGa Chronic and unexplained gastroenterologic symptoms, poor growth, weight loss, iron deficiency anemia, and delayed puberty, among other symptoms References Husby S, Koletzko S, et al. European Society Paediatric Gastroenterology, Hepatology and Nutrition guidelines for diagnosing coeliac disease 2020. J Pediatr Gastroenterol Nutr. 2020;70(1):141-156. Leonard MM, Lebwohl B, Rubio-Tapia A, et al. AGA clinical practice update on the evaluation and management of seronegative enteropathies: expert review. Gastroenterology. 2021;160(1):437-444. Celiac disease ruled out Negative CONSIDER HLA genotyingd Celiac disease unlikely Normal IgA levels Negative tTG IgA result (≤3 U/mL) tTG IgA and Total IgA Celiac Antibodies, Tissue Transglutaminase (tTG), IgA and IgA, Total Immunoglobin tTG IgG Tissue Transglutaminase Antibody, IgG DGP IgA Deamidated Gliadin Peptide (DGP) Antibody, IgA DGP IgG Deamidated Gliadin Peptide (DGP) Antibody, IgG EMA IgA Endomysial Antibody, IgA by IFA EMA IgG Endomysial Antibody, IgG HLA Celiac Disease HLA-DQ Genotyping Click here for topics associated with this algorithm Celiac Disease Testing for Symptomatic Individuals IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL 58 IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL 3 Care Process Model DISCUSSION Several points from the algorithm and current practice guidelines are outlined below. Laboratory Studies Diagnostic Laboratory Tests Order the following initial laboratory tests:7 REMEMBER: Patients must be on a gluten-containing diet for at least 3 months to be tested for CD.8 • Total IgA (IgA deficiency is common in CD and may cause false negative IgA-based serology.) • TTG IgA If low IgA levels, please see PROVIDER RESOURCES on page 4. If normal IgA levels AND high positive TTG IgA, order the following labs 4 weeks after initial laboratory test • TTG IgA • EMA IgA • TSH with reflex to free T4 Follow the ARUP algorithm for repeat diagnostic laboratory studies and/or biopsy indications Recovery Laboratory Studies/Assessments Assess the following after 6 months on a gluten-free diet7 • TTG IgA8 • Nutrition, including iron (ferritin, CRP, serum iron, TIBC), folic acid, 25-hydroxy vitamin D, vitamin B12 • CBC (to assess Hgb and MCV) • Growth and development Treatment The only treatment for celiac disease is strict avoidance of gluten. Successful management of CD requires both knowledge and diligence. Refer patients for a consult with a dietician upon diagnosis and again if deficiencies don’t resolve and/or continued gluten contamination is suspected. Failure of symptom improvement and failure of decline in TTG IgA levels after 6 months on a GFD suggests continued gluten ingestion or more complex disease.7 Some patients benefit from a low-lactose diet initially. Special attention should be given to the following disease treatment principles: Avoiding gluten in the diet • Wheat, rye, and barley • Oats should be avoided for 3-4 months and then those that are not contaminated in the milling process can be reintroduced Avoiding gluten-containing non-food sources • Medications, make-up, lotions, lip balms, stamps, envelopes, playdough, toothpaste Avoiding cross contamination • Toasters, waffle irons, restaurants, oil fryers, food double-dipping (butter, sugar, mayonnaise, peanut butter, etc.) January 2023 IS A BIOPSY ALWAYS NECESSARY FOR A DIAGNOSIS OF CD? • New guidelines indicate biopsies may be unnecessary in 50-60% of patients.2,3,6,9,18 WHAT ASSESSMENTS SHOULD BE COMPLETED YEARLY? • Adherence to GFD with history and serology (TTG IgA)7,8 • TSH with reflex to free T4 • CBC • Growth and development • Disease complications • TTG IgA in individuals with persistent or recurrent disease symptoms7 WHEN SHOULD A CHILD BE REFERRED TO A PEDIATRIC GASTROENTEROLOGIST • Indications for a biopsy • CD symptoms despite negative serology • IgA deficiency with CD symptoms • Continued symptoms despite compliance on a gluten-free diet HOW AND WHEN SHOULD FAMILY MEMBERS BE TESTED FOR CD? • CD testing (TTG IgA) should be completed on all asymptomatic, first-degree siblings, with repeat testing only if they show symptoms. 59 IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL 4 Care Process Model January 2023 CAREGIVER RESOURCES REFERENCES National Library of Medicine medlineplus.gov/celiacdisease.html • Symptoms • Genetics • Clinical trials • Living with celiac disease • Related Issues • Pediatric celiac disease • Nutritional considerations 1. Barker, C. C., Mitton, C., Jevon, G., & Mock, T. (2005). Can tissue transglutaminase antibody titers replace small-bowel biopsy to diagnose celiac disease in select pediatric populations? Pediatrics, 115(5), 1341-1346. 2. Bingham, S. M., & Bates, M. D. (2020). Pediatric celiac disease: A review for nongastroenterologists. Current Problems in Pediatric and Adolescent Health Care, 50(5), 1-6. 3. Bishop, J., Reed, P., Austin, P., Hurst, M., Ameratunga, R., Craigie, A., McFarlane, J., Chin, S. E., Mouat, S. M., & Evans, H. M. (2018). Prospective evaluation of the ESPGHAN Guidelines for diagnosis of celiac disease in New Zealand children. Journal of Pediatric Gastroenterology and Nutrition, 67(6), 749-754. 4. Di Nardo, G., Villa, M. P., Conti, L., Ranucci, G., Pacchiarotti, C., Principessa, L., Raucci, U., & Parisi, P. (2019). Nutritional deficiencies in children with celiac disease resulting from a gluten-free diet: A systematic review. Nutrients, 11(1588), 1-12. 5. Elkin, A., Grant, C., Coleman, T., & Sereika, S. M. (2018). Use of an educational model to improve confidence and knowledge of celiac disease among nurse practitioners in Pennsylvania. Gastroenterology Nursing, 41(5), 412-423. 6. Ermarth, A., Bryce, M., Woodward, S., Stoddard, G., Book, L., & Jensen, M. K. (2017). Identification of pediatric patients with celiac disease based on serology and a classification and regression tree analysis. Clinical Gastroenterology and Hepatology, 15(3), 396-402. 7. Hill, I. D., Dirks, M. H., Liptak, G. S., Colletti, R. B., Fasano, A., Guandalini, S., Hoffenberg, E. J., Horvath, K., Murray, J. A., Pivor, M., & Seidman, E. G. (2005). Guideline for the diagnosis and treatment of celiac disease in children: Recommendations of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition. Journal of Pediatric Gastroenterology and Nutrition, 40(1), 1-19. 8. Husby, S., Murray, J. A., & Katzka, D. A. (2019). AGA clinical practice update on diagnosis and monitoring of celiac disease: Changing utility of serology and histologic measures: Expert review. Gastroenterology, 156(4), 885-889. 9. Klapp, G., Masip, E., Bolonio, M., Donat, E., Polo, B., Ramos, D., & Ribes-Koninckx, C. (2013). Celiac disease: The new proposed ESPGHAN diagnostic criteria do work well in a selected population. Journal of Pediatric Gastroenterology and Nutrition, 56(3), 251-256. National Celiac Association nationalceliac.org • Disease facts • Resources • Support group information • Events • Newsletters • Recipes • Dining out Celiac Disease Foundation celiac.org • Disease facts • Celiac disease news • Gluten-free living • Ways to get involved Celiac.com • Disease Facts • Articles • Recipes • Blogs • Clubs GIKids gikids.org/celiac-disease/ • Pediatric celiac disease • Quick Facts • Find a provider • Diet helps • Support organizations PROVIDER RESOURCES Medical Home Portal – Celiac Disease medicalhomeportal.org IgA Deficiency Information https://primaryimmune.org/aboutprimary-immunodeficiencies/specificdisease-types/selective-iga-deficiency/ 10. Laurikka, P., Nurminen, S., Kivela, L., & Kurppa, K. (2018). Extraintestinal manifestations of celiac disease: Early detection for better long-term outcomes. Nutrients, 10(8), 1-14. 11. Mearin, M. L. (2007). Celiac disease among children and adolescents. Current Problems in Pediatric and Adolescent Health Care, 37(3), 86-105. 12. Oliveira, G. N., Mohan, R., & Fagbemi, A. (2018). Review of celiac disease presentation in a pediatric tertiary centre. Arguivos de Gastroenterologia, 55(1), 86-93. 13. Remes-Troche, J. M., Uscanga-Dominguez, L. F., Aceves-Tavares, R. G., Calderon de la Barca, A. M., Carmona-Sanchez, R. I., Cerda-Contreras, E., Coss-Adame, E., Icaza-Chavez, M. E., Lopez-Colombo, A., Milke-Garcia, M. P., Morales-Arambula, M., Pelaez-Luna, M., Ramos Martinez, P., Sanchez-Sosa, S., Trevino-Mejia, M. C., Vazquez-Frias, R., Worona-Dibner, L. B., Zamora-Nava, L. E., & Rubio-Tapia, A. (2018). Clinical guidelines on the diagnosis and treatment of celiac disease in Mexico. Revista De Gastroenterologia De Mexico, 83(4), 434450. 14. Rosen, A., Ivarsson, A., Nordyke, K., Karlsson, E., Carlsson, A., Danielsson, L., Hogberg, L., & Emmelin, M. (2011). Balancing health benefits and social sacrifices: A qualitative study of how screening-detected celiac disease impacts adolescents’ quality of life. BMC Pediatrics, 11(32), 1-10. 15. Rubio-Tapia, A., Hill, I. D., Kelly, C. P., Calderwood, A. H., & Murray, J. A. (2013). ACG clinical guidelines: Diagnosis and management of celiac disease. American Journal of Gastroenterology, 108, 657-676. 16. Russo, C., Wolf, R. L., Leichter, H. J., Lee, A. R., Reilly, N. R., Zybert, P., Green, P. H. R., & Lebwohl, B. (2020). Impact of a child’s celiac disease diagnosis and management on the family. Digestive Diseases and Sciences, 65, 2959-2969. 17. Scanion, S. A., & Murray, J. A. (2011). Update on celiac disease – etiology, differential diagnosis, drug targets, and management advances. Clinical and Experimental Gastroenterology, 2011(4), 297-311. 18. Webb, C., Norstrom, F., Myleus, A., Ivarsson, A., Halvarsson, B., Hogberg, L., Lagerqvist, C., Rosen, A., Sandstrom, O., Stenhammar, L., & Carlsson, A. (2015). Celiac disease can be predicted by high levels of anti-tissue transglutaminase antibodies in population-based screening. Journal of Pediatric Gastroenterology and Nutrition, 60(6), 787-791. 60 IMPLEMENTATION OF A PEDIATRIC CELIAC DISEASE CARE PROCESS MODEL 61 Appendix F Project Timeline Task Start End Status Gap Discussed with Pediatricians 8/1/21 8/31/21 Complete Analysis of Current CD Care in Bryner Pediatrics 9/1/21 9/16/21 Complete Literature Review 10/10/21 12/6/21 Complete Complete Project Proposal 1/15/22 2/24/22 Complete Present Project Proposal 6/21/22 6/21/22 Complete Create Pre- and Post-Instructional Surveys 6/10/22 6/25/22 Complete Obtain WSU IRB Approval 6/15/22 7/30/22 Complete Obtain IHC IRB Approval 6/15/22 7/5/22 Complete Create Physician Training PPT 6/10/22 8/24/22 Complete Create a Care Process Model 6/10/22 9/30/22 Complete Evaluate the Care Process Model 8/10/22 9/15/22 Complete Care Process Model Edits 9/1/22 9/30/22 Complete 11/21/22 11/21/22 Complete 1/1/23 1/31/23 Complete 11/21/22 11/21/22 Complete 11/21/22 2/1/23 Complete Present Final Project to Faculty 3/1/23 3/31/23 Complete Submit Final Project Report 4/1/23 4/15/23 Complete Project Proposal and Literature Review Project Plan Project Implementation Physician Training Implement Care Process Model Administer Pre- and Post-Instructional Surveys Project Evaluation Evaluate Post-Instructional Survey Results