Creation of Experience-based Celiac Benchmarks: The First Step in Pretransition Self-management Assessment.

OBJECTIVES: Transition planning for children with chronic disease includes the development of independence in many self-management tasks. Conditions that depend on diet have distinct skill sets not well assessed by the traditional transition-readiness tools. There has been literature that describes age-appropriate skill acquisition for diabetes and food allergy patients. There are, however, no age-appropriate benchmarks established for celiac disease (CD). METHODS: CD experts (including physician, nurse, dietician, social worker, patient, and parent) created a list of celiac-related tasks, which formed the basis of the survey. Patients with CD, and their parents, were recruited from outpatient celiac clinic and support groups, and invited to report the age each task was mastered. RESULTS: Respondents included 204 patients and 155 parents. Mean age was 12 years (standard deviation 4.6) with average of 4 years since diagnosis. The earliest tasks were mastered by a median age of 8 years, such as recognizing GF as gluten-free, eating safely in a shared space and recognizing basic unsafe foods. Describing the effects of eating gluten or explaining CD to a friend or stranger occurred around age 10. Asking about gluten-free preparation in a restaurant, and identifying gluten-free medications or vitamins was mastered around age 12, whereas tasks involved with safe domestic travel or assessing risk in a job environment occurred between 14 and 16. The interquartile range was about 4 years for each question. No significant difference seen between patient and parent reports. CONCLUSIONS: This novel patient-centered celiac skill list may improve anticipatory guidance and accelerate self-management skills.

Value of IgA tTG in Predicting Mucosal Recovery in Children With Celiac Disease on a Gluten-Free Diet.

OBJECTIVES: Our objective was to determine the rate of mucosal recovery in pediatric patients with celiac disease on a gluten-free diet. We also sought to determine whether immunoglobulin A tissue transglutaminase (tTG) correlates with mucosal damage at the time of a repeat endoscopy with duodenal biopsy in these patients. METHODS: We performed a retrospective chart review of 103 pediatric patients, younger than 21 years, with a diagnosis of celiac disease defined as Marsh 3 histology, and who underwent a repeat endoscopy with duodenal biopsy at least 12 months after initiating a gluten-free diet. RESULTS: We found that 19% of pediatric patients treated with a gluten-free diet had persistent enteropathy. At the time of the repeat biopsy, tTG was elevated in 43% of cases with persistent enteropathy and 32% of cases in which there was mucosal recovery. Overall the positive predictive value of the autoantibody tTG was 25% and the negative predictive value was 83% in patients on a gluten-free diet for a median of 2.4 years. CONCLUSIONS: Nearly 1 in 5 children with celiac disease in our population had persistent enteropathy despite maintaining a gluten-free diet and immunoglobulin A tTG was not an accurate marker of mucosal recovery. Neither the presence of symptoms nor positive serology were predictive of a patient's histology at the time of repeat biopsy. These findings suggest a revisitation of monitring and management criteria of celiac disease in childhood.

Non-responsive celiac disease in children on a gluten free diet.

BACKGROUND: Non-responsive celiac disease (NRCD) is defined as the persistence of symptoms in individuals with celiac disease (CeD) despite being on a gluten-free diet (GFD). There is scant literature about NRCD in the pediatric population. AIM: To determine the incidence, clinical characteristics and underlying causes of NRCD in children. METHODS: Retrospective cohort study performed at Boston Children's Hospital (BCH). Children < 18 years diagnosed with CeD by positive serology and duodenal biopsies compatible with Marsh III histology between 2008 and 2012 were identified in the BCH's Celiac Disease Program database. Medical records were longitudinally reviewed from the time of diagnosis through September 2015. NRCD was defined as persistent symptoms at 6 mo after the initiation of a GFD and causes of NRCD as well as symptom evolution were detailed. The children without symptoms at 6 mo (responders) were compared with the NRCD group. Additionally, presenting signs and symptoms at the time of diagnosis of CeD among the responders and NRCD patients were collected and compared to identify any potential predictors for NRCD at 6 mo of GFD therapy. RESULTS: Six hundred and sixteen children were included. Ninety-one (15%) met criteria for NRCD. Most were female (77%). Abdominal pain [odds ratio (OR) 1.8 95% confidence interval (CI) 1.1-2.9], constipation (OR 3.1 95%CI 1.9-4.9) and absence of abdominal distension (OR for abdominal distension 0.4 95%CI 0.1-0.98) at diagnosis were associated with NRCD. NRCD was attributed to a wide variety of diagnoses with gluten exposure (30%) and constipation (20%) being the most common causes. Other causes for NRCD included lactose intolerance (9%), gastroesophageal reflux (8%), functional abdominal pain (7%), irritable bowel syndrome (3%), depression/anxiety (3%), eosinophilic esophagitis (2%), food allergy (1%), eating disorder (1%), gastric ulcer with Helicobacter pylori (1%), lymphocytic colitis (1%), aerophagia (1%) and undetermined (13%). 64% of children with NRCD improved on follow-up. CONCLUSION: NRCD after ≥ 6 mo GFD is frequent among children, especially females, and is associated with initial presenting symptoms of constipation and/or abdominal pain. Gluten exposure is the most frequent cause.

Sequence of acquisition of self-management skills to follow a gluten-free diet by adults with celiac disease.

BACKGROUND: Celiac disease (CD) treatment is lifelong adherence to a gluten-free diet (GFD), requiring mastery of numerous skills to maintain health. AIMS: To assess the rate of self-management skill acquisition following diagnosis, and the influence of demographic factors on this rate. METHODS: Patients attending a celiac center were invited to complete an anonymous survey which reported demographic information and time for mastery of self-management skills relevant to CD. RESULTS: Completed surveys were returned by 137 patients (79% female). Most participants reported mastering skills that involved identifying gluten-containing versus gluten-free foods within 6 months. Explaining CD and GFD to others required 1-2 years. Identifying gluten in medications and supplements required 3-5 years. Traveling internationally with GFD adherence required more than five years to learn. Demographic factors were not associated with the rate of acquisition. CONCLUSIONS: This is the first description of a timeline for relevant skill acquisition following diagnosis for CD. A sequence emerges, with most patients learning skills relevant to home, then social settings, then the workplace, and, finally, unfamiliar settings. Awareness of this progression of mastery, and particular recognition of difficult skills will allow physicians and dietitians to provide CD patients with targeted education and resources to facilitate adherence.

Race and Ethnicity-Conscious Clinical Research Best Practices.

This Viewpoint describes the concept of race-conscious medicine in oncology.