Real Talk: Eosinophilic Diseases

Eosinophils are a type of white blood cell that play a role in immune responses and help fight off infections. When these cells build up and cause inflammation in the digestive system, tissues, organs, and/or bloodstream, without a known cause, it may be the result of an eosinophil-associated disease. Join the American Partnership for Eosinophilic Disorders (APFED) for a series of conversations with researchers, clinicians, patients, and other community members as we discuss practical strategies for disease management and treatments, research, and other topics of interest.

The Spoon Theory and Eosinophilic Disorders 04/30/2024

The Spoon Theory and Eosinophilic Disorders Co-host Ryan Piansky, a graduate student and patient advocate living with eosinophilic esophagitis (EoE) and eosinophilic asthma, and co-host Holly Knotowicz, a speech-language pathologist living with EoE, who serves on APFED’s Health Sciences Advisory Council, have a conversation about the Spoon Theory. In this episode, Ryan and Holly discuss the origin of the Spoon Theory, their experiences, and what the Spoon Theory means in their lives. Listen to this episode to learn how the Spoon Theory could work for you. Disclaimer: The information provided in this podcast is designed to support, not replace the relationship that exists between listeners and their healthcare providers. Opinions, information, and recommendations shared in this podcast are not a substitute for medical advice. Decisions related to medical care should be made with your healthcare provider. Opinions and views of guests and co-hosts are their own. Key Takeaways: [:50] Ryan Piansky and co-host Holly Knotowicz introduce the topic of today’s episode, the Spoon Theory. Both Ryan and Holly will discuss their experiences. [1:39] About ten years ago, fatigue became a challenge for Holly. As she researched options for managing her fatigue, Holly came across the Spoon Theory, a tool she uses and teaches now in conferences and talks. [2:05] The Spoon Theory is a story written and copyrighted by Christine Miserandino in 2003 to help explain how chronic illness affects the amount of physical and or mental energy a person has available for daily activities and tasks and how it can be limited. [2:28] About a year ago, in a Community Conversations episode of APFED’s podcast, guest Ashley Spencer discussed EGPA. Ashley and Holly brought up the Spoon Theory. Ryan calls the Spoon Theory a digestible way to convey the effect of living with fatigue from chronic illness. [2:46] Holly shared how Christine Miserandino developed the Spoon Theory while at brunch with a friend. Her friend asked Christine how she was coping living with lupus. Christine grabbed all the spoons from the table and explained that each task throughout the day costs a spoon. [3:21] Christine asked her friend to walk through every activity of her morning. As her friend started talking about the different things she does, Christine would remove a spoon from her pile of 12 spoons. When dinnertime came, there was only one spoon. That limited her choices for dinner; this was long before dinner delivery services. [4:19] Through this exercise, Christine’s friend learned how chronic illnesses use up a lot of energy just from existing. For listeners who want to read more, please check out Christines’ website, ButYouDontLookSick.com, linked in the show notes. [4:33] Ryan sees the Spoon Theory as an easy way to convey what living with a chronic illness is like. He asks, why is it 12 spoons? Does everyone have the same number of spoons? In interviews, Christine has said 12 was the number of spoons on the table but it is a good representation of the limited supply people with chronic illnesses have. [5:07] According to the theory, healthy people have an “unlimited” supply of spoons, while people with chronic illnesses have to ration their spoons to get through the day. Everybody’s number is slightly different but the theory uses 12. [5:22] Ryan shared a story about seeing his sister during the holidays. She doesn’t have a chronic illness. She seems to have unlimited spoons for activities she plans, while Ryan may run out of spoons around 10:00 a.m. [5:46] Ryan asks if it is always the same number of spoons per day. Holly says your baseline number is about 12 spoons. It can vary if you borrowed spoons from the day before or if you have spoons left over from the day before. Some say on a good day, you might wake up with 20 spoons but a bad day would start with 12 spoons. [6:34] Holly explains about borrowing spoons. If you run out of spoons on one day, before you finish your activities, you might borrow spoons from the next day by canceling a planned activity for the next day. Holly also explains it to people as a lending library. [7:49] When Ryan was young, he attended the APFED patient education conference every year. He recommends it. His parents warned him not to overextend himself but to take it easy and rest during the day. At every conference, he just kept going for 12-plus hours. When he got home, he would crash for a day. He had used up all his spoons! [8:36] Ryan asks what happens when you run out of spoons. Holly shares that when you run out of spoons, your body might have a flare-up, or be more susceptible to getting sick because you’ve become rundown. [9:10] It can also lead to comparison with others and feeling sad or anxious because you don’t have the energy that healthy people around you have. For the average person with chronic illness, cooking from scratch from a recipe could be three or four spoons. [9:51] For someone with a specialized diet, that could double. You’re not just reading the recipe, you have to think about substitutions and go buy them. You have to know if the recipe will taste good with substitutions. It’s mentally exhausting to follow recipes for specialized diets. [11:04] Holly is an extrovert but sometimes being with people can take too many of her spoons, so she carefully plans her socializing. Being with good friends might take three spoons. Presenting at a conference takes more spoons. Walking a dog could take two spoons. Taking medication or brushing her teeth could take one spoon. [12:10] For children with chronic illness, going to school might take four of their spoons. Playing soccer might take five spoons, so at times they don’t have the energy. This can result in absences from school because they want to have typical social lives but don’t have the energy and reserves to do so. It’s the same for adults but it’s a hard fact for children. [12:53] Ryan was diagnosed with EoE at age two. Ryan thinks back to high school. He woke up at 6:15 every day to get to high school before 8:00. Getting to school on time probably took most of his spoons. The rest of the day was exhausting. He never did any extracurriculars in high school. By 3:00, he was down for the count. [13:50] If Ryan’s friends wanted to spontaneously do something after school, he often had to refuse. He needed a few days to prepare physically and mentally for extra activities. Having something sprung on him at the last minute drained more of his spoons. He would like to have had the Spoon Theory to explain it to his friends. [14:52] Holly says there is a mental aspect to having a chronic illness. You have to think about things in advance, especially people living with eosinophilic diseases and/or those who have specialized diets. To consider going out to a meal, you might have to research a restaurant in advance or even talk to the chef. Thinking about and making these phone calls requires spoons. [16:06] Recently Ryan planned to go out with friends. After they picked a restaurant and Ryan chose what he would order, the friends decided to try a different restaurant. Ryan had to check the menus of six other restaurants before they settled on the original one. The extra effort depleted Ryan’s energy and he just sat quietly during dinner. [19:14] Ryan didn’t push himself to be social because it would have eaten into his reserve spoons for the following day. As it was, he slept in the next day. [20:07] Ryan has had days where he has had to use up the next day’s spoons, and then had the next day be equally busy. [20:28] The Spoon Theory can be applied to different chronic illnesses. Most of them are invisible illnesses. It makes sense that Christine’s website is named ButYouDontLookSick.com. [21:29] Holly loves that the Spoon Theory provides a visual representation of how our energy works and how we can manage it. Because many chronic illnesses are invisible, people don’t always understand why we have to cancel, abort, or decline plans. We often have to prioritize activities to protect our health. It’s a different standard. [22:10] We prioritize activities to protect our health and how we feel. Holly uses the Spoon Theory to explain why she declines plans in advance when she has too many things scheduled. She wouldn’t be her best self. Holly rarely schedules anything for after an eight-hour workday. [22:55] Holly thinks of herself as a dynamic person who brings a lot to the table. She doesn’t want to be in an activity where she can’t participate fully. It reflects on how much her diagnosis seeps into her life. She doesn’t like to share her EoE with everybody, even though many people in her life know it and she does this podcast! [23:25] Over the last six months, Holly’s goal has been to map out her week to keep her energy consistent. She plans when to work out in the morning, when to see patients, and when to fly for international conferences. She gives herself a rest day after the flight or she stumbles and mumbles during the presentation. This means she often declines dinner invitations. [24:45] Holly will accept invitations to destination weddings but then will not book anything extra for a week afterward or she knows she will get sick. It’s a pattern. [25:28] Ryan says sometimes people can tell when he’s not at his best, but for the most part, he looks relatively healthy. He’s up and about, at meetings and conferences but it’s such a limited amount of energy that he has available. It’s hard for people with unlimited spoons to gauge how many spoons Ryan has left. [25:57] Holly often presents at medical conferences about feeding therapy, eosinophilic diseases, food allergies, FPIES, and tube feeding, and she incorporates the Spoon Theory into some of her talks. She has spoons at the podium and starts dropping them as she goes, holding one or none by the end. That’s when questions come. [27:25] Holly likes people to know that when they’re working with kids with chronic illness, it’s important to pace out their therapy. For example, give a patient two things to work on until the next time, not ten, to be successful. [28:50] Ryan clusters his multiple specialist annual visits at the start of the fall semester and at the beginning of the spring semester. That means he misses some classes and lectures at the beginning of each semester. It is draining. Tests eat up half his spoons for the day. [30:35] Holly shares how applying the Spoon Theory impacts managing her health. The Spoon Theory helps her create and maintain boundaries. She adamantly tries to stick to a schedule that rarely depletes her spoons per day. It’s still a work in progress. Holly has a therapist who is helping her work on it. [31:08] Holly schedules social things on days when she has little to no other obligations. She has to maintain that schedule. She has good days and sometimes great weeks which leads her to add more to her plate, but then she runs out of spoons more quickly because she’s borrowing from the next day. Eventually, she has no spoons to borrow. [31:42] Holly went on vacation for her birthday and then last week she was doing great, taking some urgent referrals for babies. This week, she had to cancel things. She is learning that she needs to schedule time to recharge and rest even on good days. [32:26] Holly has learned there are ways to increase your number of spoons. The most important things are to be compliant with treatment and follow a specific diet (if recommended). A lot of chronic illnesses have a specific researched diet to help you stay healthy. When you have a cheat day, you’re harming yourself by taking spoons from your next day. [33:31] Working out helps with anxiety and depression. There are physical and mental health benefits. Holly started tracking over the last eight months how many spoons working out earned for her, compared to the spoon it took from her. She finds that it adds three to five spoons to her daily reserve. The endorphins boost her energy. [34:14] Ryan agrees. He goes to the gym at least twice a week. If he misses a day, he feels worse. He goes out for a walk on days he’s not going to the gym just to get moving and he feels better after that. Being stuck inside all day is mentally draining as well. Going for a walk takes extra effort but it does feel better. [35:32] Ryan and his mother have similar food allergy issues so they both carefully stick to their diet. If they vary their diets on vacation, even without eating triggers or allergens, they feel physically bad for a few days until they get back to their usual diets. [36:28] Once Holly learned about this theory and was making new contracts, she realized that there may be times when she might have to cancel and reschedule. When she sends an email about an engagement, she includes an article on the Spoon Theory and describes what she is struggling with, in case she has to reschedule. [37:42] The Spoon Theory is a good way to describe to friends or family why the person with the chronic illness isn’t hosting the holiday but may need to go to a room and rest at the host’s home. It’s a tool to inform loved ones and friends so they can be supportive. It’s a different way to share our struggles with our chronic illness. [38:27] When Ryan meets people and tells them about his health issues, he might say he has food allergies but then also explain how his conditions lead to a limited supply of energy, and then tell about the Spoon Theory. It’s a helpful tool we can all use, going forward. [39:08] Our listeners can learn more about the Spoon Theory by going to Christine Miserandino’s website, ButYouDontLookSick.com. [39:47] To learn more about eosinophilic disorders, visit APFED.org. If you’re looking for a specialist who treats eosinophilic disorders, use APFED’s specialist finder at APFED.org/Specialist. [40:05] To connect with others impacted by eosinophilic diseases, please join APFED’s online community on the Inspire Network at APFED.org/Connections. [40:14] Ryan thanks Holly for sharing information about the Spoon Theory. It means a lot to Holly that we have a platform to reach a lot of people. Holly hopes if you are struggling with a chronic illness that you are not alone and you can use this tool to bring your loved ones and friends closer. Maybe weed out the people who aren’t helping. [41:15] Holly thanks APFED’s education partners, AstraZeneca, Bristol Myers Squibb, GlaxoSmithKline, Sanofi, and Regeneron, linked below, for supporting this episode. [41:26] Ryan shares how he just met his partner’s friend and by discussing symptoms while picking a place to eat, it turns out she was diagnosed last year with EoE. It’s unusual for him to meet people with EoE out there randomly. Ryan is glad to be creating this resource for people. Holly agrees 1,000%. Mentioned in This Episode: APFED on , , , , Education Partners: This episode of APFED’s podcast is brought to you thanks to the support of , , , , and . Tweetables: “Approximately 10 years ago, fatigue became a real challenge for me and as I researched options on how to manage fatigue, I came across the spoon theory, which is what we’re going to specifically discuss today.” — Holly Knotowicz “[The Spoon Theory] is such an interesting story and it feels like such an easy way to convey what living with a chronic illness is like.” — Ryan Piansky “We all have people in our lives whom we love dearly, but they could maybe be taking too many of our spoons, so you have to be thoughtful about when you plan time with them.” — Holly Knotowicz /episode/index/show/eosinophil/id/31047268

Gastro Girl and GI Diseases: Conversation With Jacqueline Gaulin 03/29/2024

Gastro Girl and GI Diseases: Conversation With Jacqueline Gaulin Description: Co-host Ryan Piansky, a graduate student and patient advocate living with eosinophilic esophagitis (EoE) and eosinophilic asthma, and co-host Holly Knotowicz, a speech-language pathologist living with EoE, who serves on APFED’s Health Sciences Advisory Council, speak with Jacqueline Gaulin, the Founder of Gastro Girl, a source of information for people living with various gastrointestinal disorders. In this episode, Ryan and Holly interview Jacqueline Gaulin about her career, how she started Gastro Girl, its podcast, and the partnership between Gastro Girl, the American College of Gastroenterology, and GI on Demand. Listen in for more information about this empowering resource for GI patients and their families. Disclaimer: The information provided in this podcast is designed to support, not replace the relationship that exists between listeners and their healthcare providers. Opinions, information, and recommendations shared in this podcast are not a substitute for medical advice. Decisions related to medical care should be made with your healthcare provider. Opinions and views of guests and co-hosts are their own. Key Takeaways: [:51] Ryan Piansky and co-host Holly Knotowicz welcome Jacqueline Gaulin, Founder of Gastro Girl, a patient-centered company that focuses on empowering individuals living with digestive health conditions through evidence-based information, resources, and access to GI experts to help people make informed healthcare decisions. [1:42] Holly thanks Jqcqueline for having APFED’s Executive Director, Mary Jo Strobel, as a guest on the Gastro Girl Podcast last fall. There is a link in the show notes. [2:07] Jacqueline started Gastro Girl as a blog, in 2007/2008 while working for a startup, trying to do great things for healthcare. She was charged with the Digestive Health channel. They needed content on living with disease and empowering patients. She created a persona, Gastro Girl, and started a blog around her. [2:41] Gastro Girl became a popular part of the site. The company was bought but Jacqueline retained the URL, Gastro Girl, and the persona. She engaged on Twitter and then got a job at the American College of Gastroenterology (ACG) where she dug into GI and learned the pain points from both the provider and patient sides. [3:09] Jacqueline saw that patients didn’t have the resources, access, and champions they needed so she created a company out of Gastro Girl to meet their needs. In 2019, Gastro Girl partnered with ACG to create a provider-focused platform, GI On Demand, that provides ACG members and their patients access to multi-disciplinary GI expertise. [4:02] Ryan tells about his experiences with APFED as a person living with Eosinophilic Esophagitis and Eosinophilic Asthma. His family is passionate about the patient experience for people with eosinophilic disorders. Ryan sees that Jacqueline is also passionate about empowering patients. [4:39] Jacqueline loves that patients and their loved ones are involved in advocacy. There is no better voice than those who walk on that health path. Jacqueline, as a child, saw her grandmother needing laxatives to feel better. Jacqueline didn’t understand. Then Jacqueline got a dog with digestive problems that needed veterinary help. [5:51] Jacqueline was fascinated by the whole connection between the digestive system and our overall health. When she started Gastro Girl she was going through a lot of trauma and lost a lot of weight. She was diagnosed with irritable bowel syndrome (IBS). [6:48] Gastro Girl brings together resources and information that run the gamut of digestive-health-related topics. It has resources and partners, like APFED, that cover all the areas of GI and bring the pieces together to help patients find the information they need, when and how they need it. [7:21] Holly tells how she searched symptoms online, was able to find Gastro Girl, and did a deep dive to navigate many areas. Holly also tells how she saw herself in patients’ symptoms while she was helping in an EoE clinic before she was diagnosed with it. [8:38] Gastro Girl has baseline content on what eosinophilic gastrointestinal disorders are and how they are diagnosed. Then it points to expert partners like APFED to speak to eosinophilic disorder topics and support in-depth. Gastro Girl focuses on symptoms and identifying diseases earlier to get into care quicker. [9:25] The Gastro Girl podcast and YouTube channel have episodes on EoE. The comments that come in on the videos, like “EOE 101,” Jacqueline says are mind blowing. People say the videos help them identify what possibly could be going on and get them to seek care without alarming them. [10:34] Gastro Girl offers evidence-based information and resources so that people know they can come to the site and find sites like APFED and the ACG that are trusted and credible, that will not send them down a rabbit hole and confuse them. [12:07] A colleague of Jacqueline’s friend emailed Jacqueline with her young son’s symptoms. Jacqueline isn’t a doctor and can’t diagnose but the symptoms sounded like EoE. She recommended that the mother ask her son’s doctor if he could be tested. Two months later she heard he had eosinophilic asthma and EoE. [13:21] Jacqueline says the biggest message we could get out there is for patients to pay attention to credible resources. She cautions patients to be mindful and use discretion when going to the sites for their health information. Dr. Google is not great. Holly directs patients toward specific videos or episodes that offer the best information. [14:41] There are some times when Jacqueline does not approve a comment to a podcast or video if she is concerned the comment will do more damage than good. She urges listeners and viewers to exercise caution on what comments they listen to. [16:22] Jacqueline has interviewed many patients, caregivers, and providers in the GI space. She says patients want to be heard and feel that they are being listened to and that they’re getting access to the right information so they can feel better. That can mean different things to different patients in their quality of life. [17:47] Gastro Girl is about putting the patient at the center. Being patient-centric is seeing the patient’s view first. Gastro Girl supports patients having collaborative care and shared decision-making, giving information so they are more educated and informed as they ask questions and collaborate on decisions with their providers. [19:25] Thinking critically and asking the right questions makes you an active participant in your healthcare journey, and hopefully, gets you to a better result. [20:26] Jacqueline learns every day from her podcast guests. She has learned that healthcare providers, gastroenterologists, dieticians, and psychologists care about their patients. They want patients to get better. Their passion and caring are overwhelming. [21:20] Jacqueline has learned that patients are resilient and committed, not only to finding better ways to have a better quality of life but also to advocacy efforts, helping, and lifting their fellow patients. Jacqueline says together we are much stronger than to be isolated by ourselves, dealing with big healthcare issues. [22:55] Many doctors are involved in research, speaking, webinars, and educating fellow providers and patients. It’s mind blowing to Jacqueline! [23:16] Running Gastro Girl and GI on Demand during COVID-19 was hard and stressful for Jacqueline. She was inactive and gained weight. She worried about taking care of others, but not herself. She is back on track, exercising and losing weight. [26:08] Jacqueline says we have to be kind to ourselves; we have to have compassion and grace towards ourselves. We’re very kind and compassionate to others and give them grace. Make sure you extend grace to yourself. [26:27] Jacqueline cites Tara Cousineau, author of The Kindness Cure. Positive reinforcement is better than beating yourself up. When you feel good, you’re going to do the good things next time. [27:46] Jacqueline tells about GI on Demand. It’s provider-focused with multiple disciplines available for healthcare providers. Providers and patients can search for dieticians, GI psychologists, and genetic testing and counseling. This helps GI providers and their practices to help their patients better. [31:24] Jacqueline plans for Gastro Girl to continue to do great work for patients. She is working on an educational bus tour, “Gastro Girl to the Rescue Bus Tour!” with partners such as APFED, to bring information to individuals at home. Patients can be guided to resources, like getting a proper diagnosis or finding a provider. It’s a lot of logistics! [32:56] To check out Gastro Girl’s website, visit GastroGirl.com. To learn more about eosinophilic disorders check out the resources there and also visit APFED.org. If you’re looking for a specialist who treats eosinophilic disorders, you can use APFED’s Specialist Finder at APFED.org/Specialist. [33:21] If you’d like to connect with others impacted by eosinophilic diseases, please join APFED’s online community on the Inspire Network at APFED.org/Connections. [33:30] Ryan and Holly thank Jacqueline Gaulin again for joining them. Ryan would be happy to go on the Gastro Girl podcast any time to talk more! Holly thanks APFED’s education partners, linked below, for supporting this episode. Mentioned in This Episode: Special Guest Mary Jo Strobel APFED on , , , , Education Partners: This episode of APFED’s podcast is brought to you thanks to the support of , , , and . Tweetables: “I love when patients get involved in advocacy because there’s no better voice for the patient than those who walk in that path, whatever their health condition or journey is, or even if they have a loved one.” — Jacqueline Gaulin “What Gastro Girl really offers is evidence-based information and resources so that people know that they can come to our site and find sites like APFED and the ACG that are trusted and credible.” — Jacqueline Gaulin “The biggest message we could get out there is for patients to pay attention to the credible resources. I just caution patients to be mindful and use discretion when going to the sites for your health information.” — Jacqueline Gaulin “To think critically and to ask the right questions will get you, hopefully, a better result in your healthcare journey because you’re not just a passive participant, you’re actively playing an important role in your healthcare with your provider.” — Jacqueline Gaulin About Jacqueline Gaulin An early champion of a patient-centric, collaborative, and multidisciplinary approach to gastrointestinal health, I worked for several early-stage health-related start-ups, including Revolution Health. During my time with the American College of Gastroenterology (ACG), I led the ACG into the digital age by creating and executing strategic communications plans and patient education campaigns around a variety of digestive health topics and related research, including IBS, IBD, Liver disease and colorectal cancer awareness. After six years with the ACG, my passion for empowering patients inspired me to evolve my popular blog, Gastro Girl, into a digital health company in 2015, Gastro Girl, Inc., to provide patients with access to the GI expertise and evidence-based information and resources they need to follow their doctor’s treatment plan and make informed health care decisions with their care team for better health outcomes. /episode/index/show/eosinophil/id/30594403

The Family Risk of Eosinophilic Gastrointestinal Diseases 02/29/2024

The Family Risk of Eosinophilic Gastrointestinal Diseases Description: Co-host Ryan Piansky, a graduate student and patient advocate living with eosinophilic esophagitis (EoE) and eosinophilic asthma, and co-host Mary Jo Strobel, APFED’s Executive Director, speak with Dr. Kathryn Peterson, MD, MSCI, a Professor of Gastroenterology at the University of Utah Health. In this episode, Ryan and Mary Jo interview Dr. Peterson about the family risk of eosinophilic gastrointestinal diseases, discussing the studies she has done, future work she is planning, and other studies of related topics. She shares that she is a parent to a patient living with an eosinophilic disorder. She hints at future research that may lead to easier diagnosis of EGIDs. Listen in for more information on Dr. Peterson’s work. Disclaimer: The information provided in this podcast is designed to support, not replace the relationship that exists between listeners and their healthcare providers. Opinions, information, and recommendations shared in this podcast are not a substitute for medical advice. Decisions related to medical care should be made with your healthcare provider. Opinions and views of guests and co-hosts are their own. Key Takeaways: [:49] Ryan Piansky welcomes co-host Mary Jo Strobel. Mary Jo introduces Dr. Kathryn Peterson, a Professor of Gastroenterology at the University of Utah Health. Dr. Peterson specializes in diagnosing and treating diseases of the digestive system including eosinophilic esophagitis (EoE), Barrett’s esophagus, and inflammatory bowel disease. [2:00] Dr. Peterson works at the University of Utah in Salt Lake City. She co-directs an eosinophilic gastrointestinal disease clinic with Dr. Amiko Uchida. They also work closely with allergy, nutrition, and pharmacy in the clinic and are looking for additional ancillary services to come into the clinic. [2:27] Dr. Peterson takes care of all sorts of eosinophilic gastrointestinal diseases. She works closely with Dr. Gerald Gleich, as well. Dr. Peterson is a mother of a boy living with eosinophilic disease for 10 years, so she experiences both sides of eosinophilic diseases. She loves her job. [3:23] Familial risk refers to the risk of the disease in a patient when a family member is affected, compared to the general population. Looking at a proband (patient), is a first-degree family member (parent, sibling, or child) also affected with eosinophilic disease? Is a second-degree family member (grandparent) affected? Are cousins? [3:58] Dr. Peterson’s is trying to see if and how far out the risk for the disease goes within a family. Based on that, you can get an idea if some shared genes are involved, vs. shared environmental influence of the disease within family members. That’s the idea of doing family risk studies in complex diseases; eosinophilic diseases are very complex. [4:44] Dr. Peterson explains how she conducts a family risk study in Utah. The Utah Population Database is very helpful. The University of Utah has partnered with The Church of Jesus Christ of Latter-day Saints for large genealogical pedigrees that allow tracking disease through expanded pedigrees, with privacy and security limitations. [5:24] It’s necessary to clarify physician coding to make sure it’s realistic and coded appropriately so that results are believable. It’s very hard to recruit family members. Dr. Peterson feels extremely blessed to live in that area. The families are generous and giving. She also believes all eosinophilic families are generous. [6:34] The farther out you can identify the risk for disease, the more likely you will find a common gene that could be implicated in disease risk or onset. If the disease is tracked in extended relatives, it implies a shared gene more than a shared environmental risk. If the disease is isolated within nuclear families, it may indicate an environmental risk. [7:39] In doing familial research, Dr. Peterson is trying to develop a risk score. People are getting pretty good at diagnosing EoE, but Dr. Peterson would not say that the non-EoE EGIDs are well-established or well-diagnosed. They are missed commonly and often. To have a risk score from the extent of the disease in a family is helpful. [8:12] Dr. Peterson notes that studies of cancer risk in extended families have established cancer risk scores and related screening that is needed. [8:43] Dr. Peterson coauthored a paper in November 2020 about the familial risk of EoE, published in the Clinical Gastroenterology and Hepatology Journal. She and her colleagues looked at nuclear families. They were looking for how many members of the nuclear family of an EoE patient have esophageal eosinophilia. [9:28] They used a questionnaire on allergies, food allergies, and symptoms. They pulled in around 70 first-degree family members and scoped them for eosinophilia, pulled the records on the rest of the family members if they had been scoped, and assessed the risk for eosinophilia. [9:51] Including the records, and assuming that everyone who hadn’t had an endoscopy was negative, they found the risk for esophageal eosinophilia in first-degree family members was 14%, bordering on the familial risk for celiac disease. It’s probably higher since they assumed anyone who hadn’t been scoped didn’t have eosinophilia. [10:19] They called it esophageal eosinophilia, because the guidelines for diagnosing EoE suggest that the patient must complain of symptoms, and these family members did not have symptoms. It was interesting to find this high prevalence of eosinophilia in the nuclear family members of EoE patients. They had a higher risk of allergy, as well. [11:14] Dr. Peterson explains the differences between esophageal eosinophilia and eosinophilic esophagitis (EoE). Esophageal eosinophilia means eosinophils are in the esophagus, >15 per high-power field in a biopsy. That could qualify as EoE when you go through the criteria of symptoms. [12:19] We call it esophageal eosinophilia while we rule out everything else that could cause that cell to get recruited into the esophagus. It could be an allergic reaction to a medication, larger eosinophilic disorders, or parasitic infections. Esophageal eosinophilia means you had that initial biopsy that puts you at risk for EoE. [13:06] You have to go down the diagnostic steps: Do I have symptoms? Do I have anything else that explains it? If you have nothing else that explains the eosinophils, and you have esophageal dysfunction, then you can call it EoE. [13:33] Dr. Peterson, speaking personally, believes that educating doctors to ask patients about EoE symptoms would be useful in diagnosing EoE. People cope. You don’t want to focus on your symptoms because you want to be able to focus on your life. If symptoms aren’t brought to a doctor’s attention, a diagnosis can get missed. [16:47] Dr. Peterson discusses risks for EoE in families where allergies are present. Dr. Peterson is involved currently in another familial study to find more information about the risk of EoE where there are allergies in a family. In the preliminary data, it looks like there is a link with asthma. Asthma and EoE in a family seem to track together. [17:31] The risk of EoE seems to be higher with additional allergies within a family. Dr. Peterson says they are cleaning up the data to get a better answer. It appears that allergies in general go along with some of the genes that have been identified in EoE. Watch for Dr. Peterson’s papers going forward! [19:05] They looked at around 300 eosinophilic gastroenteritis (EGE) patients and about 170 eosinophilic colitis (EoC) patients. If you have a proband with EoE, is there a higher family risk of having EGE or EoC? It looks like EoE puts you at higher risk of these other conditions. But with such low numbers in the study, the jury is still out. [20:37] They looked at EGE codes because there is a subset of patients who have eosinophilic disease in their stomach and small bowel who don’t have EoE. They found that in patients who have eosinophilic disease in the stomach or the small bowel, EoE is still commonly seen throughout families. EoE seems to be a common theme. [21:54] Down the road, Dr. Peterson hopes to be able to identify enough families that they might be able to start looking at genes that might put people at risk for more extensive disease. [23:07] Dr. Peterson discusses the difficulty in diagnosing eosinophilic colitis, inflammatory bowel disorder, and other disorders. Having eosinophils does not categorize you as an EGID patient. There are other disorders where eosinophils are present. We need a better understanding of eosinophilic colitis. [25:21] Eosinophilic asthma and eosinophilic fasciitis are disorders that Dr. Peterson has not studied but are in the Institutional Review Board approved documentation for future study. [26:18] Dr. Peterson addresses whether your degree of risk for an EGID increases if you have an immediate family member with an EGID, vs. a second cousin with an EGID. She would say yes, based on the hazard ratios in the data and knowing that eosinophilic disorders are complex and twin studies show an environmental influence. [27:28] Dr. Peterson asks patients about their family history, especially when they have other symptoms besides EoE. It makes her more aware of what to test. [29:10] A paper Dr. Peterson is about to submit studied family members who weren't affected, who were siblings of probands. Their mucosa wasn’t entirely normal. They may be pre-diagnostic. These are patients who need to be followed. There may be things that set people up for the development of this disease, in the right environment. [31:04] Something fascinating from the familial study is the challenge of diagnosing EGIDs. Fifty percent of the people they brought in hadn’t had an endoscopy. We need to be proactive in identifying diseases in patients. In the study, there are a lot of general GI symptoms coded that Dr. Peterson wonders if they may be missed EGIDs. [32:34] The NIH gave Dr. Peterson’s team funding and they were able to do linkage analysis on several de-identified families that were at high risk for EGIDs. It looks like multiple genes have the potential to be involved. Personalizing medicine would be applicable if there were just one specific gene involved. [33:23] Down the road, we may find some genes that portend higher risk and other genes that portend risk where we can do preventative environmental care. We can develop risk scores to identify risks and point to interventions. [34:10] Mary Jo thanks Dr. Peterson for joining us today to share her expertise and help us learn and understand. [34:36] Future research needs to be done where we are able to recruit patients and do more work looking at genetic linkage and get to the point where we can diagnose and identify non-EoE EGIDs well enough to explore them more, including eosinophilic colitis. Defining those diseases is necessary and needed. [35:16] A lot of what Dr. Peterson is trying to do is to look further into combined diseases and hypereosinophilic states to determine if there is some gene within families that may help her to develop other therapies not focusing only on the GI tract but on a global approach to health for these patients. [35:48] There is current research being done to find less invasive ways of identifying disease, such as imaging, so people don’t have to undergo endoscopy. That research is being done on the commercial side. [36:44] Dr. Peterson has been looking at food-specific antibodies. Also, research by other doctors is being done to identify other markers of the foods that often trigger the disease. There has been some interesting preliminary data. This can help patients to eliminate fewer foods. [37:27] Dr. Peterson has been looking at less invasive ways to identify non-EoE EGIDs in ways that can avoid biopsy. [38:04] What’s being done to study Barrett’s esophagus? Dr. Peterson speaks of past and planned research, using the Utah population database. They looked at the risk for Barrett’s esophagus in patients with EoE and it was eight times higher than the normal population. Dr. Peterson correlates risks with reflux for Barrett’s and EoE. [39:26] There are still questions about which comes first, EoE, Barret’s esophagus, or reflux. She also talks about the relationship between achalasia, allergic diseases, and EoE. [41:05] To learn more about Dr. Peterson’s research, please see the links in the show notes. To learn more about eosinophilic gastrointestinal disorders, please visit . [41:29] To find a specialist, visit . To connect with others impacted by eosinophilic diseases, please join APFED’s online community on the Inspire Network at . [41:48] Ryan and Mary Jo thank Dr. Kathryn Peterson again for joining them. Mary Jo thanks APFED’s education partners, linked below, for supporting this episode. Mentioned in This Episode: (to release February 2024) (published November 2020) APFED on , , , , Education Partners: This episode of APFED’s podcast is brought to you thanks to the support of , , , and . Tweetables: “When we study familial risk, we’re looking at the risk of the disease in a patient when a family member is affected, compared to the general population.” — Dr. Kathryn Peterson “I think allergies, in general, kind of go along with some of the genes that have been identified in EoE.” — Dr. Kathryn Peterson “Fifty percent of the people we brought in [to this familial risk study] hadn’t had an endoscopy. We need to be proactive in identifying diseases in patients.” — Dr. Kathryn Peterson About Dr. Kathryn Peterson Kathryn Peterson, MD is a Professor of Gastroenterology at the University of Utah Health. She is certified by the American Board of Internal Medicine. Dr. Peterson specializes in diagnosing and treating diseases of the digestive system including eosinophilic esophagitis, Barrett’s esophagus, and inflammatory bowel disease. She completed her medical degree at the University of Texas Southwestern, followed by residency and a fellowship at the University of Utah and a master's program in Epidemiology at Harvard University. Bio: . /episode/index/show/eosinophil/id/30173488

Remodeling and Eosinophilic Esophagitis (EoE) 01/30/2024

Remodeling and Eosinophilic Esophagitis (EoE) Description: Co-host Ryan Piansky, a graduate student and patient advocate living with eosinophilic esophagitis (EoE) and eosinophilic asthma, and co-host Holly Knotowicz, a speech-language pathologist living with EoE, who serves on APFED’s Health Sciences Advisory Council, speak with Dr. Amanda Muir, an Assistant Professor of Pediatrics at the Children’s Hospital of Philadelphia. In this episode, Ryan and Holly interview Dr. Muir about tissue remodeling and eosinophilic esophagitis (EoE). Dr. Muir describes remodeling and stiffening, its effects, and how it relates to treatment and inflammation. Listen in for information on remodeling and a pediatric study Dr. Muir is planning. Disclaimer: The information provided in this podcast is designed to support, not replace the relationship that exists between listeners and their healthcare providers. Opinions, information, and recommendations shared in this podcast are not a substitute for medical advice. Decisions related to medical care should be made with your healthcare provider. Opinions and views of guests and co-hosts are their own. Key Takeaways: [:48] Co-host Ryan Piansky welcomes co-host Holly Knotowicz. Holly introduces Dr. Amanda Muir, an Assistant Professor of Pediatrics at the Children’s Hospital of Philadelphia (CHOP). She has a translational lab that investigates esophageal remodeling in the setting of EoE. Holly thanks Dr. Muir for joining us today. [1:51] Dr. Muir became interested in eosinophilic disorders as a GI Fellow. There were so many patients with eosinophilic esophagitis and eosinophilic gastrointestinal diseases but there weren’t many good therapies and little was known about the long-term results for children. [2:24] Dr. Muir’s first eosinophilic interest was eosinophilic esophagitis. She joined a lab that was looking at how the esophagus changes over time in the setting of inflammation. After being in the lab, training, and learning all the skills and techniques, she was able to launch her career and lab. [2:46] Dr. Muir started her own EoE clinic at CHOP (Children’s Hospital of Philadelphia) as part of their Center for Pediatric Eosinophilic Disorders. She sees patients at the clinic, then she can bring questions from the clinic to the lab and talk about them as a group. [3:28] Dr. Muir explains esophageal remodeling. There is remodeling that happens in the epithelial compartment of the esophagus. Then there’s remodeling that happens underneath the surface in the lamina propria. For the most part, when people talk about remodeling in eosinophilic esophagitis, they refer to the remodeling happening below the surface. [3:50] There is a burgeoning field dedicated to studying the surface of the esophagus, and Dr. Muir is also very interested in that. For today’s purposes, we are talking about the remodeling that happens under the surface. [4:03] Eosinophils that get to the esophagus secrete chemicals that excite the cells below the surface to secrete collagen. Collagen is the glue that holds the body together. They’re secreting glue to help the esophagus hold together, and the esophagus gets stiffer and stiffer, over time. That is remodeling. It’s the body trying to heal itself. [5:04] Are children and adults equally at risk for remodeling? Patients develop a stiffening of the esophagus more, later in life. It is thought that the more years you have this inflammation, the more stiff your esophagus gets. There are patients six to nine years old who already have signs of stiffening. [5:28] Dr. Calies Menard-Katcher from Colorado published a paper where she described all of the eosinophilic esophagitis patients at her institution who got dilated. Dilation is the process of a balloon stretching your esophagus open when it’s too narrow. She had patients as young as six in her cohort that she described as having EoE strictures. [5:49] Remodeling happens with younger patients but we’re not as good at finding it. [6:08] Any type of inflammation in the GI tract can lead to some stiffening. The typical gastrointestinal disease that we think of as remodeling is Crohn’s Disease. An inflammatory process happens in the small bowel or colon that leads to narrowing and stiffness in the intestines. [6:28] Also GERD (reflux) can lead to stricture, over time. It is just much more rare to see a GERD-induced stricture as opposed to EoE. [7:13] We are not sure, but to some extent, we think of remodeling as not being reversible. Once there is a certain degree of stiffness, the esophagus does not seem to open up without these dilations. If you can control the inflammation, you can halt the stiffening. Maybe there is some degree of reversibility. [7:44] In the Phase 2 dupilumab trials, investigators found that patients on dupilumab were seen to gain two millimeters in diameter of the esophagus, compared to the patients on placebo. We may be able to prevent some remodeling if we catch it soon enough. More research is needed. [8:33] Dr. Muir tells of the work she is doing in her lab. They take biopsies from patients and grow collagen-secreting fibroblasts in a dish. The research is to find out what calms the fibroblasts down from actively secreting collagen. [9:22] It’s tough to follow the symptoms of EoE when patients only have difficulty swallowing foods that are hard to swallow. If patients are not challenging their esophagus, they might not notice having daily trouble swallowing. It’s hard to ask a young kid who is eating a lot of soft foods if they feel like anything’s getting stuck. [10:06] Dr. Muir will ask teenage patients, “Do you ever want to eat chicken? Do you ever want to eat steak?” A lot of times they don’t want to eat it, perhaps because it felt uncomfortable at some point in their life and they don’t want to eat it, not based on taste but on repeated bad events. It’s hard to tease out the symptoms, sometimes. [10:27] Dr. Muir says, based on our Functional Luminal Imaging Probe (FLIP) studies, patients who had feelings of food that felt stuck in the last 30 days did seem to have a more narrow caliber esophagus. There is not a 100% correlation between symptoms and remodeling, but there seems to be some correlation. [11:31] Ryan tells how patients have tendencies to get around their EoE symptoms, with a personal example of keeping food in his mouth and chewing it for a long time before swallowing. A scope would show he had bad inflammation of the esophagus. He had been diagnosed when young and was under treatment and on a restricted diet. [12:26] Biopsies don’t always get a sample below the surface to check for fibrotic cells so it is hard to find remodeling with biopsies. There are some visual signs. Seeing rings or trachealization in the esophagus, or narrowing, can be signs that there is some remodeling under the surface. [13:38] For kids who have a lot of trouble swallowing, Dr. Muir performs an EndoFLIP test regularly. The test catches subtle narrowing that may not be visible to the endoscopist. Doing this test gives the doctor more information and a better sense of the patient's phenotype, such as inflammation, the esophagus being stretchy, or being stiff. [14:49] The EndoFLIP is a balloon with an imaging probe that includes a TV for the doctor to see how many millimeters the esophagus is in diameter as the balloon inflates along the whole body of the esophagus. It’s not an imaging test that goes to radiology. It’s a balloon that is blown up slowly with salt water and that gives this measurement. [15:18] The EndoFLIP is a helpful tool to help determine who may have some more stiffening or determine exactly what the diameter of the esophagus is before starting treatment. [15:33] One of the things that Dr. Menard-Katcher of Colorado, Dr. Ackerman of the University of Illinois, and Dr. Muir collaborated on was to look and see if they could find any markers in the esophagus that would relate to some of the things that are obtainable on biopsy or the esophageal string test. [15:57] What they found was that periostin — a protein made by the epithelium and by the fibroblasts, which is known to activate fibroblasts, and is very high in EoE — seemed to correlate with the EndoFLIP measurements. This makes Dr. Muir think that there might be some potential for biomarkers to detect remodeling. [16:16] The thing that everyone wants for this disease is to find a biomarker where we don’t have to do a scope. As far as finding a non-invasive biomarker, we’re not there, yet. There are some things going on at the tissue level that might clue us in on how distensible the esophagus is. [17:18] The thing Dr. Muir worries about the most with long-term inflammation is that the esophagus is going to get more narrow over time. That will make patients more susceptible to food impaction (although not all patients with food impaction have a stricture). [17:36] One worry is that the esophagus will get so narrow that an endoscope will not be able to pass a stricture. That will lead to more swallowing problems. That is what Dr. Muir hopes to be able to prevent as we get better at treating this. [18:09] Any of the treatments that stop the inflammation and help get you below that “magical” 15 eosinophil count that we all strive for, will help prevent remodeling. So, once you get everything calm, hopefully, the remodeling process will stop. However, with the stiffening, the fibroblasts get more excited and have a hard time turning off. [18:53] Simply turning off the inflammation will not turn off the fibroblasts. Many people within the GI space are looking at fibroblast-directed therapy, especially in Crohn’s disease, there’s a real need to prevent a lot of surgeries that are happening. Dr. Muir hopes to apply some of these to the esophagus, as well. [19:16] In the study by Dr. Menard-Katcher, Dr. Ackerman, and Dr. Muir, there were 80 patients. Some were on swallowed steroid treatment and others were on an elimination diet. There were not enough patients on each therapy to find a significant difference in remodeling between the therapies. Patients in remission had better distensibility. [19:44] Dr. Evan Dellon showed in a paper that patients who have sustained remission have fewer dilations, in the long term. While we don’t have a way to reverse the fibrosis that’s happened, we hope to prevent it from getting any worse. Dr. Muir’s research goal is to find something to calm fibroblasts down and prevent fibrosis or even reverse it. [20:31] Dr. Muir explains that cells under the surface level are fibroblasts. When eosinophils and T cells come in and secrete antagonizing chemicals, the fibroblasts turn on and start secreting collagen. The fibroblasts also turn on when the epithelium is angry and inflamed. There is also evidence that surface cells can secrete collagen. [22:46] Dr. Muir says it’s hard to know how far along in development some anti-fibrotic drugs are. We have many promising targets. Understanding how the remodeling happens is very important to be able eventually to treat this disease. Even though it seems like incremental progress, Dr. Muir believes research is moving the field forward. [24:16] Dr. Muir says her EoE patients at CHOP are generous with their blood and tissue. Getting consenting control patients for lab studies involves a lot of leaps of faith and trust that scientists will grow your cells ethically. Dr. Muir feels lucky she has a good research team that explains things in lay terms to control patients. [26:50] Dr. Muir’s team has videotaped pediatric EoE patients and control patients’ eating. The time EoE patients spent chewing and how long it took to swallow correlated to their esophageal distensibility measured by the EndoFLIP test. She believes that how we feed and the difficulty we have swallowing have to do with esophageal remodeling. [27:41] That’s Dr. Muir’s next area of study. It’s being spearheaded by Dr. Kanak Kennedy, a fellow in Dr. Muir's lab, trying to figure out the relationship between pediatric feeding and remodeling. [28:08] As part of their research, they are videotaping as many kids eating as they can. This involves many control patients who don’t have EoE. Another area of research is on the enzyme lysyl oxidase which organizes collagen into bundles and makes it stiff. She is looking into ways to decrease the organization of the collagen. [29:08] Ryan thanks Dr. Amanda Muir for coming on the podcast and giving a crash course on remodeling and EoE. [29:14] To learn more about eosinophilic esophagitis, visit . To learn more about Dr. Muir’s research, read her . [29:30] To find a specialist, visit . To connect with others impacted by eosinophilic diseases, please join APFED’s online community on the Inspire Network at . [29:47] Ryan and Holly thank Dr. Amanda Muir again for joining them. Holly thanks APFED’s education partners, linked below, for supporting this episode. Mentioned in This Episode: APFED on , , , , Education Partners: This episode of APFED’s podcast is brought to you thanks to the support of , , , and . Tweetables: “I was able to start my own EoE clinic at CHOP as part of their Center for Pediatric Eosinophilic Disorders. I see patients who have eosinophilic gastrointestinal diseases and then I can go back to the lab and bring those questions from my clinic to the lab.” — Dr. Amanda Muir “The thing that everyone wants for this disease is to find a biomarker where we don’t have to do a scope.” — Dr. Amanda Muir “Any of the treatments that stop the inflammation and help get you below that ‘magical’ 15 eosinophil count that we all strive for will help prevent remodeling. So, once you get everything calm, hopefully, the remodeling process will stop.” — Dr. Amanda Muir About Dr. Amanda Muir: Amanda B. Muir, MD, Attending Physician, Children’s Hospital of Philadelphia, Research Institute. Dr. Muir investigates the mechanisms underlying esophageal fibrosis to improve therapeutic and diagnostic approaches. /episode/index/show/eosinophil/id/29711018

Journey to an Eosinophilic Esophagitis (EoE) Diagnosis 12/22/2023

Journey to an Eosinophilic Esophagitis (EoE) Diagnosis Description: Co-host Ryan Piansky, a graduate student and patient advocate living with eosinophilic esophagitis (EoE) and eosinophilic asthma, and co-host Holly Knotowicz, a speech-language pathologist living with EoE, who serves on APFED’s Health Sciences Advisory Council, speak with Moises Velasquez-Manoff, a health and science writer living with EoE. He is a contributing writer for the New York Times Magazine. He writes about the human microbiome and its impact on health, as well as climate, COVID-19, and other health and science topics. In this episode, Ryan and Hollyinterview Moises Velasquez-Manoff about his and his search for help with his burning esophagus. They discuss his journey living with EoE, how he got diagnosed, and the treatments that help manage his symptoms. Moises speaks of various misdiagnoses he received that didn’t address his issues. After reflux was ruled out by a series of three tests, a biopsy during an endoscopy indicated EoE. Now on treatment, Moises is feeling much better. Listen in for a powerful story of a decades-long search for help. Disclaimer: The information provided in this podcast is designed to support, not replace the relationship that exists between listeners and their healthcare providers. Opinions, information, and recommendations shared in this podcast are not a substitute for medical advice. Decisions related to medical care should be made with your healthcare provider. Opinions and views of guests and co-hosts are their own. Key Takeaways: [:50] Co-host Ryan Piansky welcomes co-host Holly Knotowicz. Holly introduces Moises Velasquez-Manoff, a health and science writer living with eosinophilic esophagitis (EoE). He recently wrote an article that was published in NY Times Magazine entitled, “The Mystery of My Burning Esophagus,” in which he documented his journey and diagnosis of EoE. [1:51] Eosinophilic esophagitis (EoE) is a rare chronic allergic inflammatory disease of the esophagus. It is part of a complex group of diseases known as eosinophilic gastrointestinal disorders or EGIDs. [2:05] Approximately one out of 2,000 people of all ages and ethnic backgrounds [in the U.S] are diagnosed with EoE, and people with EoE commonly have other allergic diseases, such as rhinitis, asthma, or eczema. [2:20] Moises has had asthma for as long as he can remember. It was worse when he was a child and he sort of grew out of it. In adulthood, it was exercise-induced asthma. He has been allergic to sesame and peanuts for his whole life. They make him vomit. He has had eczema, hay fever, and alopecia areata. [3:43] Moises has had problems with his esophagus since his 20s. He is 49 now and only got diagnosed with EoE about two years ago after his burning pain became very bad. It took about a year to rule out reflux, first by using high-dose proton pump inhibitors (PPIs). While on the medication, he still felt the horrible burning pain. He also had unusually bad side effects from the PPIs. [5:25] After eight weeks of PPIs, an endoscopy showed his symptoms were almost gone. Moises believed he had reflux, but he still had the burning. His gastroenterologist suggested an alternative diagnosis, esophageal hypersensitivity, a pain syndrome from years of inflammation. [8:21] Moises went to a second doctor who was an expert on EoE. They did a series of tests to rule out reflux. He did the Bravo PH test or reflux, a peristalsis test, a tube that was left in for 48 hours, and a barium swallow test. Each test was uncomfortable. These tests ruled out reflux. [12:11] The doctor then believed it was esophageal hypersensitivity. The treatment was Cymbalta, an anti-depressant that also dampens pain signals. Moises was able to start eating again and started putting on weight that he had lost. A year after he stopped taking the PPIs, an endoscopy gave him the diagnosis of EoE. [15:48] Reading Moises’s article triggered many memories and emotions for Holly. She experienced symptoms since she was a baby and saw around 13 specialists before she received a diagnosis of EoE in her early 20s. By that time her eosinophil levels were out of control and her esophagus was so rigid she needed several dilations. [18:01] Moises had seen a gastroenterologist for reflux years ago and then two more doctors to get his EoE diagnosis. It was extreme pain that influenced him to seek the diagnosis. He also saw an ENT about sinus headaches, and he believes they were related to his EoE. He finally went to a doctor that specializes in EoE, just in case. [23:08] Today, Moises manages his EoE with an off-label use of the asthma medicine budesonide taken twice a day. He mixes the solution into honey and drinks it, the honey helps the medicine stick to his esophagus. Moises worries about potential side effects as it is a steroid but at small doses. [25:32] Ryan has taken the systemic steroids hydrocortisone and prednisone, as topical steroids were not effective for him. He was also on a restricted diet throughout his childhood. The diets didn’t clear up his EoE; the only thing that helped was high-dose steroids until he began taking a biologic after being diagnosed with eosinophilic asthma. [28:56] The treatment Moises is taking for EoE is localized. There shouldn’t be any systemic effect. He believes if he took a biologic, he may see improvements in his other allergic conditions, such as eczema and his sinus condition. [27:17] When his esophageal burning feeling was at its worst, Moises felt like he was suffocating. Doctors couldn’t explain it to him, but a research scientist told him that sometimes problems in one internal organ, like the esophagus, can confuse the brain stem, so it reads the problem as coming from another organ, like the lungs or the heart. [29:23] What helped with Moises’s gasping attacks was the neuromodulating medicine, the anti-depressant, which changed how the nervous system perceives what’s happening, lowered the ability for pain signals to be transmitted, and calmed his nerves. [30:53] Ryan talks about drugs being prescribed off-label when there is anecdotal evidence that they can improve symptoms of other disorders. Some EoE patients use the asthma medicine budesonide as a topical treatment of the esophagus. It is mixed into a slurry with Splenda and swallowed. Dupilumab was originally approved to treat eczema and has recently been approved to treat EoE. [31:40] Ryan is on benralizumab, a biologic, for eosinophilic asthma but as a side effect, it has also been helping his EoE. He doesn’t need systemic steroids anymore for his EoE. After seeing positive benefits from the biologic, he weaned off the steroids. His parents, who are doctors and involved in APFED, helped him through the process. [34:40] Moises tells how he came to write the article for New York Times Magazine. While he was suffering, he was not considering writing about it. When he finally got his diagnosis and was feeling better, he read an article another science writer published about their journey with a pain condition and was inspired to help other people by writing about his own journey. His first draft was more intense than the finished piece. [37:09] Holly describes the article as very powerful. She felt she was going through it with Moises. She could feel what he was describing as he searched for answers. She appreciates him writing it. Moises says people have written to him from around the world that related to his story. [40:43] Some even asked Moises about where they could find an EoE specialists, and Ryan mentions APFED’s Specialist Finder. To find a specialist who treats eosinophilic disorders, go to . Also, please check out in these show notes. [41:19] As a science writer, Moises has written a lot about the microbiome and its relationship to allergic disease and autoimmune disease before he noticed that this was happening to him. He had written a book, , 11 years ago about the root cause of these debilitating disorders and why allergies are increasing. [42:51] Moises believes that the human microbiome has been impoverished by our modern environment and diets and that has led to an increased risk of allergic conditions. Moises gives the example of European farmers, who live in a rich microbial environment and have fewer allergic and autoimmune conditions. [44:39] Moises says the research also shows that antibiotics early in life increase the risk of asthma, EoE, inflammatory bowel disease, and colon cancer. The more you take, the greater your risk. They have done research with animals, knocking out key microbes and seeing an increase in these diseases. [45:33] H. Pylori is associated with ulcers and stomach cancer, but everyone used to have it, and it is common in the developing world. Research indicates that h. pylori changes how your immune system works. Unless you kill it with antibiotics, you have it for the rest of your life. If you have h. pylori, your EoE risk goes down. [46:32] If you are breastfed, that also reduces your risk of EoE. Breastfeeding is thought to cultivate a healthy colony of microbes in the infant’s gut. Moises credits the microbial deprivation hypothesis for the increase of allergies and autoimmune disorders. There won’t be a treatment for microbial deprivation anytime soon. [47:18] Holly and Ryan thank Moises Velasquez-Manoff for coming on the podcast and allowing them to interview him today about his patient experience and background. Moises thinks it’s crazy how much good evidence there is that EoE has increased in prevalence. Unpublished results show that EoE incidence is approaching 1 in 1,000. [48:45] To learn more about eosinophilic esophagitis, visit . To find a specialist, visit . Ryan recommends reading . To connect with others impacted by eosinophilic diseases, join APFED’s online community on the Inspire Network at . [49:13] Ryan and Holly thank Moises Velasquez-Manoff again for joining them and invite listeners to read . They close by thanking APFED’s education partners, linked below, for supporting this episode. Mentioned in This Episode: NYT Magazine article by Moises Velasquez-Manoff: APFED on , , , , Education Partners: This episode of APFED’s podcast is brought to you thanks to the support of , , , and . Tweetables: “I have had problems with my esophagus probably since my 20s but I only got diagnosed [with EoE] about two years ago after things started getting really bad.” — Moises Velasquez-Manoff “I could not tolerate the PPIs, even though they worked very well to lower my eosinophil counts. But I could not handle the side effects. So we moved to swallowed budesonide slurry. … an off-label treatment.” — Moises Velasquez-Manoff “Science takes a long time, sometimes.” — Moises Velasquez-Manoff About Moises Velasquez-Manoff Moises Velasquez-Manoff is a contributing writer for The New York Times Magazine and author of An Epidemic of Absence: A New Way Of Understanding Allergies And Autoimmune Diseases. He's written a lot about the human microbiome and its impact on health, as well as climate, COVID-19, and other health and science topics. He lives in California. Website: NYT Magazine article: /episode/index/show/eosinophil/id/29193578

Community Perspective: Eosinophilic Gastritis (EoG) 10/26/2023

Community Perspective: Eosinophilic Gastritis (EoG) Description: Co-host Ryan Piansky, a graduate student and patient advocate living with eosinophilic esophagitis (EoE) and eosinophilic asthma, and co-host Mary Jo Strobel, APFED’s Executive Director, speak with Dr. Jenny Huang, an allergy and immunology fellow at Scripps Clinic, who has a unique perspective of also living with eosinophilic gastritis (EoG). In this episode, Ryan and Mary Jo interview Dr. Jenny Huang about various EGIDs, her allergy and immunology fellowship, her EoG diagnosis, and the elimination diet she follows to treat her EoG. Dr. Huang speaks about patients she sees, and how her experience of following an elimination diet gives her passion for helping people and guiding them to a treatment plan that works for their lifestyle. She advises patients to ask their doctors lots of questions and tell all their family and friends about their condition so they can be supportive. Disclaimer: The information provided in this podcast is designed to support, not replace the relationship that exists between listeners and their healthcare providers. Opinions, information, and recommendations shared in this podcast are not a substitute for medical advice. Decisions related to medical care should be made with your healthcare provider. Opinions and views of guests and co-hosts are their own. Key Takeaways: [:49] Co-host Ryan Piansky welcomes co-host Mary Jo Strobel. Mary Jo introduces Dr. Jenny Huang, an allergy and immunology fellow at Scripps Clinic living with eosinophilic gastritis (EoG). Dr. Huang is passionate about gastrointestinal disorders and spreading awareness about EGID. She recently published a commentary about her experiences. [2:20] Dr. Huang explains that eosinophilic gastrointestinal disorders (EGIDs) are diseases of the GI tract that result from eosinophils, a type of white blood cell causing inflammation. Depending on where the eosinophilic inflammation occurs, you can have different symptoms. [2:43] The role of the esophagus is to get food from the mouth to the stomach. When there is eosinophilic inflammation, you have eosinophilic esophagitis (EoE) that can cause difficulty swallowing or food getting stuck. If you have inflammation in the stomach, that is eosinophilic gastritis (EoG). This can cause abdominal pain or nausea. [3:31] Dr. Huang is an allergy and immunology fellow. She trained in internal medicine. Even in medical school, she knew she wanted to be an allergist. In her second and third years of residency, she began having symptoms that she thought were related to the stress of putting together her fellowship application while working in the hospital. [3:56] One day, Dr. Huang had a terrible episode of vomiting and she knew she needed to get medical advice rather than trying to treat herself. [4:17] Dr. Huang learned about EoG for the first time after she was being evaluated for it. During her residency, she had learned about EoE, but not other eosinophilic gastrointestinal disorders (EGIDs). Her allergist brought it up. Dr. Huang did a lot of reading and research after that. [4:48] Dr. Huang says there were times when she couldn’t finish breakfast because of so much nausea and abdominal pain. She started carrying around TumsⓇ, which was something she had never done. Those things made her realize that something was wrong. [5:11] Dr. Huang was treated by the assistant director of the allergy fellowship program where she is now training. She says he is one of the nicest, smartest people she knows. Dr. Huang had done her internal medicine residency in the same program, so she knew all of the allergists. Dr. Huang had no doubt the doctor would figure out her problem. [5:49] Dr. Huang remembers the day she had her appointment with him. The intake nurse seemed to recognize Dr. Huang from when she had done rotations in the office, but she couldn’t quite place her and didn’t ask. [6:19] Dr. Huang describes her difficulties in following the six-food elimination diet that she and her GI doctor decided were the best option for her to manage EoG. Deciding on a treatment plan is a personal decision that should fit the needs of the patient. Dr. Huang decided on the elimination diet with no idea how difficult it would be. [7:02] Dr. Huang went to the grocery store the same day and was overwhelmed trying to read all the labels. She had never really read nutritional labels before. She stopped going to a lot of restaurants because she had to ask so many questions before ordering. [7:27] Ryan shares childhood experiences of going from an elemental diet to a strict elimination diet. He relates to Dr Huang’s experiences at the grocery store and cooking. [8:15] When Dr. Huang first began the elimination diet, most of her allowed foods were fruits, vegetables, and meats. She was able to add back most foods within a year but the hardest food for her to avoid in the beginning was soy. Her favorite food is tofu. Soy was the first food she added back and she was so happy when she was okay. [8:44] When she started reading labels for her elimination diet, Dr. Huang learned that some soy sauce has wheat in it. So, she started buying soy sauce that doesn’t have wheat in it. [8:57] Dr. Huang did the elimination diet as an adult and found it incredibly difficult. She applauds Ryan for doing it as a child. Ryan is grateful to his parents for helping him navigate that along the way. [9:20] In Dr. Huang’s article, she recommended that providers try living on an elimination diet for a week. It’s challenging! She has not been able to convince anyone to try it. When she suggests it, the look on their face is telling! It’s so hard. If people don’t see a reason to do it, it’s just not done. [10:15] An anecdote from Dr. Huang’s article was about eating at the American College of Gastroenterology conference. She asked the waiter to help her find something free of milk, wheat, eggs, soy, seafood, and other ingredients she could not have. Ryan felt it was so relatable. Ryan was at a conference this summer where he could not eat. [11:13] Dr. Huang brings a lot of snacks with her when she travels. She sticks to brands that she knows are safe for her. She searches ahead of time for restaurants with allergen menus. At restaurants, she always shares what foods she is avoiding so they can help her with choices. [11:42] Mary Jo mentions that the APFED conferences provide appropriate menu choices for people on elimination diets. [12:17] Dr. Huang’s partner is a gastroenterologist specializing in esophageal diseases. He was in general GI fellowship training when Dr. Huang was diagnosed. He has been supportive of what Dr. Huang has been through with the diagnosis. He did the elimination diet with her, helped her cook, and supported her. [13:27] The elimination diet is the only way Dr. Huang has managed her EoG. She has had repeat endoscopies with biopsies and the diet has been working so far. She’s keeping her fingers crossed. [14:00] When Dr. Huang was first diagnosed, she wasn’t sure how bad her disease was or how much worse it would get and it frightened her. Fortunately, her symptoms have been controlled so it hasn’t impacted her career that much. But she feels like this diagnosis has given her a calling and a direction for her career. [14:33] Dr. Huang plans to study EGIDs. She has been doing EGID research studies during her fellowship. She wants to help people with it. She wants people to know that they’re not alone and even though we don’t have all the answers now, we will one day. [15:08] Dr. Huang gets excited when she sees that someone is coming in with EoE or EGIDs because she automatically feels a special connection to them. She spends a lot of time counseling on the diagnosis, making sure the treatment plan fits their lifestyle and is something the patient can realistically do. [15:29] Dr. Huang thinks back to when she would recommend dietary changes without understanding what that meant for the patient. Having gone through it, now she can better counsel patients. [16:38] Dr. Huang discusses quality of life issues of treatment with her patients. She explains how difficult an elimination diet is. Currently for EoE, the only FDA-approved treatment is dupilumab by injection. [18:02] Dr. Huang’s advice for those with a new diagnosis of EoG is to talk to your doctor and ask a lot of questions. Make follow-up in-person appointments. Make a list of all the questions you have and talk through it with your doctor. If you pursue an elimination diet, get with a dietician to help navigate that. It’s confusing in the beginning. [18:36] Patients, talk to your family and friends. They can help support you at medical appointments and procedures. They can call ahead to restaurants and make sure your dietary restrictions can be met there. They can accommodate your dietary restrictions at home. Don’t hide your diagnosis from the people around you. Hiding makes it more difficult. [19:05] Mary Jo thanks Dr. Jenny Huang for joining them to share her experience and ask for additional thoughts. [19:18] Dr. Huang wants people with EoG to know that they’re not alone. EoG is rare but it’s an increasingly recognized and diagnosed disease. It’s easy to feel alone when your doctor tells you that you have a rare disease. Someone else out there knows how you feel. [19:48] Ryan thanks Dr. Huang for sharing all of her experiences today. [19:51] To connect with others impacted by eosinophilic diseases, join APFED’s online community on the Inspire Network at . [20:04] To learn more about eosinophilic gastritis, visit . To find a specialist, visit . Ryan recommends reading Dr Huang’s article linked below. Ryan and Mary Jo thank Dr. Jenny Huang again for joining them and thank APFED’s education partners, linked below, for supporting this episode. Mentioned in This Episode: “” Commentary Published in Gastroenterology APFED on , , , , Education Partners: This episode of APFED’s podcast is brought to you thanks to the support of , , , and . Tweetables: “If you have [eosinophilic] inflammation in the stomach, that is called eosinophilic gastritis or EoG. That can cause symptoms like abdominal pain or nausea.” — Dr. Jenny Huang “Deciding on a treatment plan is a personal choice and it needs to fit someone’s lifestyle and their individual needs. What’s right for one person may not be right for someone else.” — Dr. Jenny Huang “Did you know that soy sauce has wheat in it? I had no idea until I started reading all the labels. So, we started buying soy sauce that doesn’t have wheat in it!” — Dr. Jenny Huang “When I was first diagnosed, I wasn’t sure how bad my disease was or how bad it was going to get. I read a lot about the possible outcomes and it really scared me. … I couldn’t imagine what it would be like if my symptoms progressed.” — Dr. Jenny Huang About Dr. Jenny Huang Dr. Jenny Huang is an allergy and immunology fellow at Scripps Clinic. She is passionate about eosinophilic gastrointestinal disorders and has made it her mission to spread awareness about what it is like to live with EGID. /episode/index/show/eosinophil/id/28424477

Community Perspective: Eosinophilic Esophagitis 09/27/2023

Community Perspective: Eosinophilic Esophagitis Description: Co-host Ryan Piansky, a graduate student and patient advocate living with eosinophilic esophagitis (EoE) and eosinophilic asthma, and Holly Knotowicz, a speech-language pathologist and feeding specialist living with EoE, who serves on APFED’s Health Sciences Advisory Council, talk with Declan, a teenager living with eosinophilic esophagitis. In this episode, Ryan and Holly interview Declan about his EoE diagnosis, his dietary restrictions, how he receives support from his parents and friends, and upcoming milestones such as transitioning clinical care to an adult provider and attending college. Disclaimer: The information provided in this podcast is designed to support, not replace the relationship that exists between listeners and their healthcare providers. Opinions, information, and recommendations shared in this podcast are not a substitute for medical advice. Decisions related to medical care should be made with your healthcare provider. Opinions and views of guests and co-hosts are their own. Key Takeaways: [:50] Co-host Ryan Piansky welcomes co-host Holly Knotowicz. Holly introduces the guest, Declan, a teenager living with eosinophilic esophagitis (EoE). Declan is 16 years old and has been living with EoE since 2019. [1:38] Declan likes to hang out with friends, play video games, and play the cello. Declan has been living with EoE for about four years. In the beginning, his EoE was hard to handle, in part because it was new to him. [2:03] Eliminating milk because of his EoE was hard for Declan. Once he started the milk-free diet, he got his eosinophils to zero in his second endoscopy. [2:24] Before Declan was diagnosed, his weight was low, he was having trouble swallowing, both of which are signs of EoE. He was at risk of having a feeding tube when he was diagnosed, but is now able to manage his EoE with diet therapy. He feels much better now. His mom helped him find substitutes for things that he can’t eat. He enjoys the substitutes. [3:03] In 2018, Declan lost a lot of weight, had trouble swallowing, and felt like food was stuck in his throat. Losing weight didn’t worry him at first. When he started getting the feeling of food getting stuck in his throat, he became concerned and sought help. [3:39] Ryan was diagnosed when he was two, so he doesn’t remember much of it but his parents told him about it later. [4:10] Holly had symptoms her whole life but they were not recognized as EoE for many years. She put ketchup on all her food and drank a lot of milk to be able to swallow. Holly was diagnosed in her 20s at which point she needed an esophageal dilation. [4:49] Declan was about 12 when he was diagnosed after a period of weight loss and then swallowing issues. [5:12] Declan can’t have milk so his friends thought he was lactose-intolerant. He had to explain EoE to them. They eventually understood. [5:58] Declan has to read labels for the ingredients. If something has casein or whey, he cannot eat it. Eating at restaurants is challenging if they don’t have an allergen menu. He often researches foods online. Declan’s Mom helps him a lot through this journey. [7:20] Declan’s last bad reaction happened shortly after he was diagnosed. He was on a cruise ship with his family. There was so much food with milk on the ship and he decided to have some. After the cruise, he felt terrible, had difficulty swallowing, and food once again got stuck in his throat. Ever since then, he has remained milk free, except if he consumes it by accident. [8:08] If Declan accidentally has milk, he takes a steroid that opens his esophagus a little more and takes away the temporary inflammation. [8:33] Even as an adult, it is still hard for Holly to resist the cheesecake or the pizza with real cheese. She also can’t have milk due to her EoE. [9:15] Declan and his mom recently baked milk free chocolate cookies using vegan butter and almond milk. Declan’s mom has substitutes that she uses in all recipes. One of Declan’s favorites is a homemade dairy-free pizza. [9:52] Since Holly was diagnosed, many new dairy alternatives and substitutes have come onto the market. There are many brands of cheese and milk alternatives. [11:16] Declan takes a pill every night that helps with his acid reflux. [11:42] Ryan describes his journey in the transition of care process from pediatric patient to adult patient. He started by learning the medicines and dosages he was on and having a say in what foods he was eating and what foods he was avoiding. [12:15] At the beginning of Declan’s care, his mom did everything. She made the appointments and spoke to the doctor for Declan. Now Declan is preparing for his transition of care. He is more aware of what he can and can’t eat. He speaks for himself, describing his symptoms and what’s going on. [13:18] School lunches can be difficult for Declan. His mother researched the ingredients of all the foods served at his school. When going to social events, he brings something for himself to eat or just doesn’t eat. [14:35] Declan explains to his new friends what EoE is and why he has to avoid milk. He explains that it doesn’t give him anaphylaxis but does irritate his esophagus. Holly tells her new friends the same kinds of things and that she wants to keep her esophagus healthy. [16:03] If you are a friend of a person living with EoE, you can help by supporting them and being there for them if they need help. Make sure that you and your other friends are aware of what your friend can and cannot eat, especially when visiting their homes, so they can eat with you and other friends. [17:01] Holly shares a story about being very impressed when a friend of hers called ahead to a restaurant to make sure their menu included dairy-free, gluten-free, and soy-free foods she can eat. [17:30] Ryan shares an experience from over the summer going out to eat with friends of his. They remembered his triggers and called ahead to make sure there were safe options for him at a restaurant. [18:20] Declan plans to go to college and his first choice of major is architecture, or maybe chemistry or another science. When in college, Holly was unable to eat the cafeteria food. [19:33] Ryan had a similar experience. After talking with Disability Services and Food Services, it was determined that he needed an apartment with a kitchen to cook for himself. He shared the apartment with three roommates. [20:44] When selecting a college, Declan plans to check in with the cafeteria and see if they have anything he can eat. On a recent college visit, he found a lot of places with dairy-free options that he could eat. [21:27] When Holly was in college, her cooking space included only a microwave and a hot plate. She took a position as a nanny, to have access to a kitchen. Today, the world is becoming more aware of dietary restrictions and making better accommodations. [22:40] If Declan’s family takes a trip, they always research the area where they will be staying to find places where he can eat. If they are traveling far, it becomes harder to research what is available at that location. [23:20] Ryan tells how he prepared for a semester abroad in France. [24:05] Declan’s biggest challenge of living with EoE is making sure he has a place to eat if he’s away from home or on vacation. He always has to research a restaurant to know if they have anything he can eat there. [24:39] Holly always carries a bag with safe snacks like protein bars, and medicines, in case she can’t find safe options at a restaurant or a store with foods she can eat. [25:37] Holly and Ryan thank Declan for sharing his experiences to help others. [25:46] Declan’s closing thought: “You shouldn’t let EoE define you. Know that there are a lot of people that are there to support you; your friends, your family, and even different communities like this one, that can help give you knowledge and support you. Also, you should definitely be open to trying new things.” [26:54] To learn more about eosinophilic esophagitis, visit , to access the Specialist Finder, visit . Ryan and Holly thank Declan again for a great conversation and thank APFED’s education partners, linked below, for supporting this episode. Mentioned in This Episode: APFED on , , , , Education Partners: This episode of APFED’s podcast is brought to you thanks to the support of , , , and . Tweetables: “I just like to do the normal stuff that kids my age do, whether that’s hanging out with friends or playing video games; I also play the cello. I’ve been living with EoE for about four years, now.” — Declan “Once I started the dairy-free diet, I eventually got my eosinophils to zero when I had my second scope, which was really good.” — Declan “Research is the key. … If we’re going to go to a different state, we always research to see in the area where we’re staying, what places I can and can’t eat.” — Declan “It’s a really interesting experience. My journey has a lot of highs and lows, but in the end, I got to experience so much.” — Declan About Declan: Declan is 16 years old and has been living with EoE since 2019. He is in 11th grade and has been playing the cello since 4th grade. He is a huge fan of classic movies, and his favorite classic movie of all time is “Jaws”. He has a passion for architecture and science and loves to play video games and hang out with his friends. /episode/index/show/eosinophil/id/28153256

Esophageal String Test: Monitoring Tool Use and Development 08/29/2023

Esophageal String Test: Monitoring Tool Use and Development Description: Co-host Ryan Piansky, a graduate student and patient advocate living with eosinophilic esophagitis (EoE) and eosinophilic asthma, and Holly Knotowicz, a speech-language pathologist and feeding specialist living with EoE who serves on APFED’s Health Sciences Advisory Council, talk with Drs. Robin Shandas and Steven Ackerman, are scientists who work together at EnteroTrack. In this episode, Ryan and Holly interview Drs. Shandas and Ackerman about the development of the esophageal string test (EST), or simply the string test. They discuss the initial concept and the work that led to the development of the string test to collect samples from the upper gastrointestinal mucosa. The test involves swallowing a capsule, similar in size to a TicTacⓇ, with a string attached. It began as an overnight test, and today is a one-hour test. During the conversation, the researchers explained the use of the string test as a monitoring device for EoE, not as a diagnostic device. They describe how the test is administered with no endoscope, no anesthesia, no recovery time, and only minor discomfort for some patients. They discuss the age range for using the device (as young as 4) and the advantages it offers over traditional endoscopy and biopsy for frequent monitoring of eosinophilic esophagitis. Finally, they advise patients on how to access the test: Ask your clinician about it! Disclaimer: The information provided in this podcast is designed to support, not replace the relationship that exists between listeners and their healthcare providers. Opinions, information, and recommendations shared in this podcast are not a substitute for medical advice. Decisions related to medical care should be made with your healthcare provider. Opinions and views of guests and co-hosts are their own. Key Takeaways: [:50] Co-host Ryan Piansky welcomes co-host Holly Knotowicz. Holly introduces the topic of a new unsedated diagnostic tool for EoE, the esophageal string test, or for the purposes of today’s podcast episode, the string test. [1:34] Holly introduces Drs. Robin Shandas and Steven Ackerman, are professional scientists who work together at EnteroTrack. Dr. Shandas is the Chief Executive Officer and Dr. Ackerman is the Chief Science Officer. [1:48] Drs. Shandas and Ackerman, with a team of entrepreneurs, engineers, medical doctors, and scientists, developed the EnteroTracker®, the esophageal string test, a new method for collecting and sampling upper gastrointestinal mucosa. [2:00] Holly thanks Drs. Shandas and Ackerman for joining us today. [2:19] Dr. Ackerman has worked on the biology and the roles of eosinophils in the pathogenesis of allergic diseases, including asthma, eosinophilic gastrointestinal disorders, and other hypereosinophilic syndromes for the past 40 years, starting when he was a post-doctoral fellow at the Mayo Clinic in Rochester, Minnesota. [2:44] About 18 years ago, Dr. Ackerman transitioned his lab to do more clinical translational research in eosinophilic GI diseases. His lab developed and validated the string test and the biomarkers and immunoassays that they use to evaluate the results. [3:10] Dr. Ackerman was also interested in studying how the eosinophil participates in tissue remodeling and fibrosis of the esophagus in EoE and other eosinophil-associated diseases. Notably, this started with a small research grant from APFED in 2007 to 2008, which was followed by a more substantial APFED grant in 2011. [3:51] Ryan was involved in APFED in 2007 and he remembers hearing about the early work that was going on for this new test. He thought it would be fantastic to have a test without anesthesia or a scope down his throat. It was exciting to hear about the development over time. [4:23] Dr. Ackerman and Dr. Glenn Furuta, a pediatric gastroenterologist, initially conducted eosinophilic disease research together at Harvard Medical School. Dr. Ackerman eventually went to Chicago but continued to interact with Dr. Furuta. They published papers together on eosinophils and their roles in GI diseases. [4:47] Dr. Ackerman was inspired to develop the string test when Dr. Furuta pointed out to him the tremendous need for a minimally-invasive or non-invasive test for EoE that would remove the need for children to have multiple repeat endoscopies and biopsies under sedation. [5:11] The endoscopies and biopsies were done to follow patient responses to treatment, especially food elimination diets. Dr. Ackerman’s goal was to provide a way to evaluate disease activity in children with EoE in a minimally invasive or non-invasive fashion. [5:42] Dr. Shandas is a biomedical engineer, entrepreneur, and educator. He has been a scientist for over 30 years. He is a Distinguished Professor of Bioengineering at The University of Colorado. His passion is to take ideas that he thinks can help people and go through the work. It’s really, really hard work to bring those ideas to the clinic! [6:07] Dr. Shandas has been working on the string test for some time. He started eight companies to support this type of activity. The idea of the string test, a simple, non-invasive way to collect samples from the esophagus to support frequent and granular monitoring of disease activity, was compelling when Drs. Furuta and Ackerman presented it to him. [6:38] After Drs. Furuta and Ackerman received funding from APFED they did a lot of basic science research to develop the foundation of the string test. They approached Dr. Shandas to see if he could take the idea and figure out how they could help patients with it. That was compelling to him. He learned a lot about GI and EoE in the process. [7:30] Dr. Ackerman and Dr. Furuta started working on the string test in 2006. It took more than 16 years from concept and pre-clinical lab studies to its marketing and adoption for routine clinical use. It was officially in the clinic at the beginning of 2023. [8:01] Holly Knotowicz was a clinical fellow at Children’s Hospital Colorado in 2010 where they were talking about this and she recognized this would be life-changing for patients in all populations not to have to participate in sedation to get updated information. [8:32] The esophageal mucosa has biological information that may reflect the status of the disease in the esophagus. The conventional way of evaluating what is happening in the esophagus is to put a scope down the throat, pinch out a biopsy from the esophagus, and analyze that tissue for inflammatory cells and inflammatory markers. [9:19] Dr. Shandas learned that the mucosa lining the esophagus has a lot of biological information. Because it is a barrier between the outside and your body it has a lot of biochemical reactions; it may be exposed to environmental allergens or food issues. These things interact with your body through the mucosal layer. It’s a complex layer. [10:08] Scientists are just starting to figure out the number of things that go on in the esophageal mucosal layer. The string test samples the esophageal mucosa from the throat down to the stomach, for the entire length of the esophagus. The mucosa is the repository of information on how your body interacts with EoE. [10:39] The molecules that the body releases as a function of the reaction that’s taking place are expressed in the mucosal layer. The string test collects samples of that mucosal layer. It’s a simple, non-painful, non-traditional method of sampling. [11:10] The researchers figured out there’s a lot of biological information in the mucosa. Dr. Furuta and Dr. Ackerman have both done clinical studies comparing the string test against traditional biopsies to show that there are great correlations. So they set out to develop a clinically available test. [11:30] For the past eight years, EnteroTrack has done work to answer questions such as: What is the process of swallowing the device and getting the sample? What is the process of analyzing the sample? And how do we put all of that into the framework of a clinical product that can be available to patients and clinicians? [11:49] The string test is a phenomenally simple, easy way to sample mucosal content and analyze it for biomarkers, with no need for any sedation or anything to help you swallow the device. It’s a very simple technique. [12:32] Dr. Ackerman was a skeptic when Dr. Furuta first approached him with the idea. He agreed on the need to sample at the site of inflammation. He was surprised at how good the test was in the 16-hour overnight format, compared to endoscopy and biopsy. It was hard to tell the figures apart between the two tests. [13:14] Dr. Ackerman learned they only needed to measure one eosinophil-specific biomarker, major basic protein-1, for a 16-hour test, but when they changed it to a one-hour test, they needed to measure two biomarkers to get enough of a sample. The second biomarker is eotaxin-3, which attracts the eosinophils into the esophagus. [14:18] Dr. Ackerman tells of clinical validation. After multiple applications to the FDA Rare Diseases Program, they were able to get substantial funding to do a Stage 2 clinical evaluation. It was a four-year grant but it took six years to recruit all the patients. They completed and published it in 2019 as the basis for the string test and EoE score. [15:03] Today, the string test is a one-hour test. The visit takes about 90 minutes, including the test, preparation, and removal of the string. The sample is sent to a reference lab. They analyze it and in about 10 days, send your doctor a report that describes the EoE score and the magnitude of the disease. [16:43] There is no recommended frequency for the string test. It can be done as often as your doctor wants to keep track of changes in your disease. Until the string test, there hasn’t been a real solution to monitor the disease, with samples every week, two weeks, or four weeks. The researchers encourage patients to ask their clinicians for the string test. [18:19] After setting a baseline, the frequency of tests can be determined by the patient’s progress and the clinician's findings. [18:45] The string test is not a replacement for endoscopy. Endoscopy plays an important role in definitively diagnosing the disease and getting a comprehensive sense of what’s going on with the disease. The string test is a complement to endoscopy. [19:23] An endoscopic pinch biopsy samples a tiny fraction of the esophageal surface area. EoE is a patchy disease. It can manifest in different places in your esophagus. You may need to do multiple biopsies to make sure you’re covering the esophagus nicely and identify a location where the disease manifests. [20:01] An advantage of the string test is that in one test, you get a sample from your throat to your stomach, the entire length of the esophagus. That allows you to get a very comprehensive evaluation of your esophagus. The ease, cost, time savings, out-patient setting, no endoscopy suite, no endoscopist, and no anesthesiologist, are advantages. [20:39] EnteroTrack is pursuing the possibility of doing point-of-care in-home use of the string test. This would be a significant advantage over the traditional way of monitoring the disease. [21:08] A string test cannot be used instead of an endoscopy to make the initial diagnosis of EoE. It’s intended as a monitoring tool. Its clinical indication is not as a diagnostic test but for monitoring the disease, in combination with a lab analysis of the assay. Endoscopy is used for the diagnosis. [21:45] In areas where endoscopy and biopsy are not locally available, doctors might use the string test to screen patients to consider referring them to a center where they can get an endoscopy and a biopsy as an initial diagnostic tool. [22:49] Holly is excited to have access to the string test for patients on elimination diets, to test more frequently as foods are reintroduced one at a time to the diet, without putting the patient under anesthesia for a traditional endoscopy. [23:58] During the monitoring of EoE, if the doctor sees the data is trending in a certain way, it may be useful to get an endoscopy and biopsy to get additional information. [24:28] Ryan has had dozens of traditional endoscopies and biopsies so he’s always excited to hear about new tests. He’s been putting off an endoscopy and he needs one soon. Is Ryan, an adult, a candidate for this procedure at his local hospital? Yes! He has been diagnosed with EoE, so he can be monitored by the string test. [25:12] The youngest patient to swallow the device was a four-year-old and they did great with it; the test was very successful. EnteroTrack recommends that parents talk to their child’s physician about the option to use this device. The key is if the patient, parent, and clinicians all feel that the patient can swallow the device. [25:35] The string test device is about the size of a TicTac®. It’s important for the swallowing procedure to be successful. EnteroTrack works with clinical groups to give them tips on how best to ensure that the procedure is successful. [25:56] Holly is a feeding specialist. One of the things she helps people with eosinophilic diseases to learn is how to swallow pills. She helps them practice with different kinds of candies and capsules. [26:40] The clinical validation study included patients aged seven and older. Now that the product is released, clinicians, parents, and patients can together make the decision if they can swallow it. EnteroTrack looks at de-identified data to evaluate how the string test is doing. They are seeing younger age groups successfully swallow it. [27:25] There are factors that contribute to successful swallowing. One is the parental motivation of their children. Also, practice swallowing something the size of the device. [27:39] EnteroTrack is providing training to physicians and nurses at the centers that are adopting the test. They are aware of how to make the swallowing part of this test as successful as possible with the least amount of stress. [28:02] The EnteroTracker® is considered a minimal-risk device, and minimal-risk procedure. If you are anxious about swallowing, or you have challenges as you swallow it, you may gag a little bit. [28:37] In the Phase 2 data, there was a small percentage who had challenges in terms of gagging, when swallowing the device. All of those issues were resolved. Even if the patient gags, the clinician has tips to help the patient get through that. Repeated gulps of water can help bring the capsule down and alleviate those issues. [29:37] In the clinical validation studies, there were hundreds of string tests performed and there were no serious adverse reactions or problems other than the minor problem of some gagging. During the 16-hour tests, there was one patient who chewed through the string while sleeping and swallowed it. The string just passed through the GI tract. [32:47] Dr. Shandas has personally swallowed about 50 string tests to understand the patient experience. After the swallow, when the string is in the esophagus, there is a feeling of tickling in the side of the throat in the beginning, but that resolves in a couple of minutes and it is not noticed at all. It helps if the patient has something to occupy their attention. [34:05] The nurses and clinicians are trained to pull the device out, not fast and not slow. It should take three to five seconds. There is some discomfort as the string is coming out. It may feel like burning in the throat but it happens quickly and it resolves immediately. They have not seen big challenges with this, much less than in swallowing. [34:50] The string has two parts. The part that goes from your mouth down your throat is like dental floss. It’s connected to the collection string that goes down the esophagus to the stomach. That collection string is a thicker nylon yarn designed to optimally collect mucosal samples. It would be uncomfortable in the throat, but not in the esophagus. [36:06] EnteroTrack has gotten a Medicare code for the assay and a Medicare reimbursement for the assay. The next step is getting private insurers to pay for the test. EnteroTrack is working on that. It will require educating insurance companies about the value of the test. Patients, clinicians, and groups like APFED can help. [37:51] The string test is clearly less expensive than doing an endoscopy and biopsy. That should be one of the motivating factors for insurance companies. It is significantly lower cost than the test that is used as the standard of care. [38:38] Some large centers have begun using the esophageal string test, including Denver, Phoenix, Tampa, Dallas, and Chicago. EnteroTrack has received interest from clinicians and patients around the country. [39:04] Patients and parents, let your clinicians know if you are interested in the esophageal string test (EST). Your doctor can contact EnteroTrack and ask how to get the test to their clinic. It’s a simple process and the clinical training is very straightforward. [39:35] APFED plans to add information about the esophageal string test to its Specialist Finder at apfed.org/specialist to help patients find clinicians offering this test. [39:53] Drs. Ackerman and Shandas anticipate that the number of centers offering this test will grow exponentially as people see that it’s working for early adopters. [40:10] Ryan and Holly thank Drs. Shandas and Ackerman for sharing their expertise to help others today in the podcast. Dr. Shandas thanks Ryan, Holly, and APFED for the opportunity to talk about the string test. [40:35] As entrepreneurs and researchers, they are constantly innovating, including looking for other applications for the string test. They have clinical trials going on to use the string test for esophageal issues, gastric issues, and small intestinal issues, analyzing the contents for different biomarkers that have correlations with a lot of upper GI diseases. [41:22] To learn more about eosinophilic esophagitis, visit , to access the Specialist Finder, visit , and to connect with patients and families living with eosinophilic disorders, visit . Ryan and Holly thank Drs. Shandas and Ackerman again for an interesting conversation and thank APFED’s education partners, linked below, for supporting this episode. Mentioned in This Episode: APFED on , , , , Education Partners: This episode of APFED’s podcast is brought to you thanks to the support of , , , and . Tweetables: “I was interested in studying how the eosinophil participates in tissue remodeling and fibrosis of the esophagus in EoE and other eosinophil-associated diseases. Notably, this started with a small research grant from APFED in 2007 to 2008.” — Dr. Steven Ackerman “The mucosa that lines your esophagus has a lot of biological information. … The mucosa is the barrier between the outside world and your body. It is this very interesting biological fluid and … it has a lot of biochemical reactions.” — Dr. Robin Shandas “Since we released the product earlier this year, we’ve had a four-year-old swallow the device; did great, very successful. And so, we recommend that patients, parents, talk to your physician.” — Dr. Robin Shandas Featured speakers: /episode/index/show/eosinophil/id/27873633

Live from APFED’s EOS Connection 2023 07/26/2023

Live from APFED’s EOS Connection 2023 Description: Ryan Piansky, a college student and patient advocate living with eosinophilic esophagitis (EoE) and eosinophilic asthma, and Holly Knotowicz, a speech pathologist living with EoE who specializes in feeding therapy and serves on APFED’s Health Sciences Advisory Council recorded this episode live at APFED’s annual patient education conference in Denver, CO. They shared their experiences with this year’s conference, discussed takeaways from the conference presentations, and offered ways to continue the conversation. Recordings of the conference presentations are available to watch on-demand at apfed.org/conference through December 2023. Disclaimer: The information provided in this podcast is designed to support, not replace the relationship that exists between listeners and their healthcare providers. Opinions, information, and recommendations shared in this podcast are not a substitute for medical advice. Decisions related to medical care should be made with your healthcare provider. Opinions and views of guests and co-hosts are their own. Key Takeaways: [:49] Co-host Ryan Piansky welcomes co-host Holly Knotowicz. They recorded this episode at the conclusion of APFED’s Annual Patient Education Conference, held June 30 and July 1, 2023 in Denver, CO, in collaboration with Children’s Hospital Colorado. [1:39] Also collaborating on this year’s conference was the Consortium of Eosinophilic Gastrointestinal Disease Researchers (CEGIR). There were a lot of great sessions at the conference. Holly attended almost all the conference sessions virtually. She felt as engaged as if she were present. She loved the talks on nutrition. [2:48] Holly loved the presentation on EGID diagnostics, both current and what’s coming down the pipeline. It had so much helpful information she can share with her patients that don’t receive care at a major hospital. [3:14] Ryan talks about an exhibit they couldn’t show virtually: a hands on transnasal endoscopy exhibit. People got to play with the controller and practice what it is like for a doctor to do a transnasal endoscopy on a model of the nasal cavity, esophagus, and stomach. Attendees of all ages were invited to try it. [3:38] Ryan found that he wasn’t very good at it. It takes practice! He recalls previous podcast episodes where this technology was discussed. [4:04] Holly received lots of text messages about feeding therapy strategies and transnasal endoscopy. She received at least 13 photos from people who tried the transnasal endoscope with their children. She was jealous not to have been at the conference. Families also reached out to Holly about the string test study. [4:58] Jane Robinson, PhD, presented ways patients can cope with and overcome some of the fears surrounding these new diagnostic methods and treatment options. A lot of kids are scared of getting shots to receive biologic treatments. She also met with an on-site teen group. Ryan loved going to APFED conferences as a teen. [5:49] Dan Atkins, MD also did a Q&A session with the teens. Holly missed being there in person to connect with other EoE patients. The teen group at the conferences were helpful when she was growing up. The teen pictures Holly received were a riot. It looked like a good time. [6:31] The main conference sessions were recorded and will be available on demand to view and engage with through the end of the year. [7:11] Oral Immunotherapy (OIT) was a hot topic in the Q&As, including who could qualify for it. Ryan is excited about biologics as an option for the treatment of EoE and eosinophilic diseases. Ryan was glad the CEGIR scholars were able to present virtually at the conference. [8:12] In 2024, APFED will host the EOS Connection conference in the Washington, DC. area. Ryan hopes to see everyone there in person or virtually. [8:29] Throughout the year, APFED also hosts a webinar series. Recordings of past webinars are posted at and shared on social media. They’re a great way to stay in touch with APFED and see what research is being done. Recent webinar topics include diagnostic tools and eosinophilic disorders, the microbiome and eosinophilic esophagitis, and creative cooking with eosinophilic disorders. Holly is all about the food! [8:59] Ryan and Holly host this podcast monthly. They interview experts in all different types of eosinophilic diseases from all over the world and individuals who share their experiences with eosinophil-associated diseases. [9:34] The last podcast episode that was released was about proton pump inhibitors (PPIs) and EoE, and was co-hosted by Holly Knotowicz and APFED’s Executive Director Mary Jo Strobel. Ryan is excited for the upcoming slate of podcasts including more patient perspectives. [9:59] Please check out to access all the conference sessions on demand, including presentations to help you get caught up on the latest research and wonderful patient perspectives from the conference panels both Friday and Saturday. [10:12] Ryan thanks all of the attendees for joining, especially those who traveled to attend in person. It was wonderful to see so many people back in person. Ryan is thrilled that many were also able to access the conference remotely. You can explore the virtual conference through December 2023 at apfed.org/conference, including a virtual exhibit hall and poster hall. [10:38] Ryan hopes to see you all at the EOS Connection 2024 conference in Washington, DC. Please stay tuned for APFED emails and updates through social media. Please tune in to the podcast and webinars. You are invited to stay connected! [10:53] Holly thanks everyone for tuning in to this episode of Real Talk — Eosinophilic Diseases, and she hopes to see you at APFED’s webinars and podcasts! [11:00] Special thanks to our EOS Connection 2023 collaborators and education partners , , , , , , , , , , , , , , , and . [11:34] To learn more about APFED, visit and follow APFED on social media. Mentioned in This Episode: APFED on , , , , Tweetables: “We’re working with Children’s Hospital Colorado … and … we have been thrilled to have them collaborating with us [on the conference] this year.” — Ryan Piansky “I feel like I was so connected because I have so many people there in person that were texting me and sending me pictures.” — Holly Knotowicz /episode/index/show/eosinophil/id/27567732

Proton Pump Inhibitors and EoE 06/23/2023

Proton Pump Inhibitors and EoE Description: Co-host Mary Jo Strobel, APFED’s Executive Director, is joined by co-host Holly Knotowicz, a speech-language pathologist and feeding specialist living with eosinophilic esophagitis (EoE) who serves on APFED’s Health Sciences Advisory Council. They talk with guest Dr. James Franciosi, Chief of the Division of Gastroenterology, Hepatology, and Nutrition at Nemours Children’s Health in Orlando, Florida. In this episode, Mary Jo Strobel and Holly Knotowicz interview Dr. James Franciosi about his research with proton pump inhibitors, personalized medicine, the different factors that influence the response EoE patients may have to very high dose PPI treatments and other treatments, including dietary eliminations, swallowed oral steroid medications, and for some patients, biologic medications. Dr. Franciosi explains the uses of these various treatments and compares the risks of medical treatments with the risk of untreated EoE. He also describes the potential for advances in treatment choices. Disclaimer: The information provided in this podcast is designed to support, not replace the relationship that exists between listeners and their healthcare providers. Opinions, information, and recommendations shared in this podcast are not a substitute for medical advice. Decisions related to medical care should be made with your healthcare provider. Opinions and views of guests and co-hosts are their own. Key Takeaways: [:49] Co-host Mary Jo Strobel welcomes co-host Holly Knotowicz. Holly introduces the topic of proton pump inhibitors and EoE. [1:28] Holly introduces Dr. James Franciosi, Chief of the Division of Gastroenterology, Hepatology, and Nutrition at Nemours Children’s Health in Orlando, Florida. [1:39] Since 2008, Dr. Franciosi has cared for children and teens with eosinophilic esophagitis (EoE) and eosinophilic gastrointestinal diseases (EGIDs). His team’s mission is to reduce the symptoms of EoE and EGIDs and they have published more than 60 peer-reviewed publications. [1:55] Holly thanks Dr. Franciosi for joining the podcast. [2:06] Dr. Franciosi “grew up” with Beth Mays (now Beth Allen), whose family suffered from eosinophilic GI disease. When Dr. Franciosi was at the Children’s Hospital of Philadelphia (CHOP), he became very interested in eosinophilic esophagitis, which was starting to emerge as something that was different from gastroesophageal reflux. [2:30] Dr. Franciosi had the opportunity to work with Dr. Liacouras, Dr. Spergel, and many others at CHOP. He transitioned to Cincinnati Children’s Hospital with the leadership of Dr. Marc Rothenberg, Dr. Phil Putnam, and a group trying to optimize the care for children with eosinophilic GI disease. [3:01] Dr. Franciosi then moved to a leadership role at Nemours Children’s Health in Orlando, Florida. He has been with Nemours Children’s Health for the past 11 years. There he has done additional research in eosinophilic disease and seen many changes for the good with these conditions. [3:37] Mary Jo notes that early in his career, Dr. Franciosi had worked with Beth Mays, now Beth Allen, who is one of the founders of APFED. Dr. Franciosi has been working with eosinophilic diseases since the time APFED was founded. [3:56] Dr. Franciosi has conducted research into how the role of proton pump inhibitors (PPIs) in the evaluation and treatment of EoE has evolved over time. His team is very interested in what therapies may be effective for eosinophilic esophagitis (EoE) for individual patients. [4:24] The research Dr. Franciosi has been doing for EoE and other conditions is trying to look at the right drug for the right patient, with the right dosing, etc. He calls that precision medicine or personalized medicine. [4:39] Proton pump inhibitors (PPIs) are medications that have been around for decades. There has been an evolution of thought about whether this is a medicine that we use to define the condition of EoE or now something we can use as a treatment option. [4:57] There have not been any randomized, placebo-controlled clinical trials for EoE using this medication and there has been a lot of variability in the literature about how well they work. That’s some of the research Dr. Franciosi has been looking into and also making sure that these are communicated as an option for the right patients with EoE. [5:27] PPIs are traditionally thought of as blocking one of the common pathways for acid in the stomach. A proton pump in parietal cells in the GI tract pumps acid into the stomach that helps you fight bad bacteria. Sometimes the acid can cause irritation in the GI tract, the stomach, and the esophagus. The PPIs work to block those pumps. [6:15] Over the past several decades, the thinking around EoE has evolved from it being thought to be a reflux condition that had a lot of eosinophils or allergic cells, that just didn’t get better, to being thought of as strictly different from reflux, to thinking that it may be a mix for some patients. [6:42] As the first guidelines for EoE were developed, the initial thought was to use the PPI medications to exclude gastroesophageal reflux. In the first consensus guidelines, patients had to fail the proton pump inhibitors at a high dose to be defined as EoE. This has changed over time. In 2018 there were new guidelines with new research. [7:15] The newer thinking is that the PPIs are not to make the diagnosis, but they’re one of several different therapies that we can use; dietary interventions and different medications, including PPIs, swallowed steroids, and some of the newer biologic medications. [8:14] H2 blockers are some of the older medications that block acid in the stomach, but they block it in a different way. They block the histamine type-2 receptor, one of the pathways by which acid is produced in the stomach. The proton pump inhibitors block the proton pump. They’re stronger medications and they work better for acid. [8:46] The H2RA medications have names that end in “tidine” like famotidine. The PPI medications have names that end with “azole” like pantoprazole or esomeprazole. [9:45] EoE is a condition that we’ve learned a lot about over the past several decades. The PPI medications may have different functions. They may block the acid in the stomach; they may also have a role in reducing some of the inflammation. This led to questioning if PPIs should be used to make a diagnosis or as a choice of therapy. [10:36] From the 1990s to the 2000 era, to the most recent guidelines in 2018, the thinking about the treatment of EoE and the use of PPIs has evolved. [10:49] One of the important things to know is that just because you’ve been on a PPI, doesn’t mean that you’re on a high dose of PPI therapy. This is important in children and different practices. [11:12] The general recommendation for PPI therapies is to use a high dose of PPI twice a day. For adults, that’s 40 mg of esomeprazole twice a day. For children, it’s 1 mg per kg of body weight twice a day. That’s a high dose. For some people, it may be that the medication was not used at a high dose. [11:37] The goal ultimately is to back off the high dose and to decrease the amount of medication that’s being administered. [11:47] Dr. Franciosi says the main surprise in his findings was that the studies in both adults and children are “all over the place.” Some people report a 30% or 20% response; other colleagues throughout the world have reported a response of about two-thirds of the patients. It was surprising to see how variable the response was. [12:18] Dr. Franciosi thinks there is variation in the choice of PPI medications, the dosing, and how they’re administered. Dr. Franciosi and his team are also looking at how people respond to these medications. That’s where personalized medicine comes in; your genes, and factors in your body can influence how well you are responding. [12:53] Precision medicine has been used in pediatric gastroenterology for inflammatory bowel disease with medications like 6-mercaptopurine, azathioprine, and some of the biologic medications. People can respond differently to medication. Genetic variation or other factors may create different patterns of response to the same medications. [13:25] Precision medicine and personalized medicine are interchangeable terms. [13:48] Before reviewing the literature for his most recent publication, Dr. Franciosi had done some genetic testing and identified that there are genetic variations that do influence how people respond or don’t respond to the PPI medications. [14:09] We need to learn more about genetic variations. Dr. Franciosi thinks for every new diagnosis of EoE, PPIs should be considered as a medication. It’s important for patients and families to know the different options. Providers may have their own biases but they should lay out the options for their patients and families to decide. [14:47] Dietary intervention has significant benefits for children, teens, and adults. There can be hardships with quality of life that have to be considered. Taking medication for the long term also has considerations. The newer biologic medications are a fantastic step forward but they are expensive. They are just starting to be used for EoE. [15:21] Dr. Franciosi likes to identify to his patients that there are options in treatments: dietary elimination, proton pump inhibitors, swallowed steroids, and biologic medication for people who have strictures (narrowing) in their esophagus or fibro stenosis (scar tissue). These patients are less likely to respond to PPI medication. [15:57] We need to do more research on this and more future prospective trials, patients with scarring are those where a different type of therapy beyond the PPI medications. Patients with inflammatory symptoms seem to respond to PPI medication. [17:04] Dr. Franciosi’s patients and families are making some choices together. Dietary elimination may not be the right choice for certain patients but they need to have the option. Dr. Franciosi presents dietary elimination as effective for many patients. It’s an investment upfront. It presents the benefit of not using long-term medications. [17:44] Sometimes when children have growth concerns, or the dietary interventions don’t work or are not possible, different medications may be considered. [18:13] The benefit of using PPIs in treatment is that you can reduce the inflammation that’s occurring, alleviate symptoms and make patients feel better. There has been some debate about whether this is related to the acid primarily in the stomach and some breaks in the esophagus lining that cause antigens to go in and cause inflammation. [18:51] Dr. Franciosi uses a balanced approach with patients and families. He tells them every treatment has risks associated with it. Also, not treating EoE has risks. He did studies at Cincinnati on what happened to patients 15 years later if they were untreated. It can involve more scar tissue, strictures, symptoms, and growth issues. [19:37] PPIs have gotten a lot of bad press over the past few years, primarily looking at the rates of infection. When you block stomach acid, that acid is no longer there to kill bad bacteria. So there are more associated respiratory infections and stomach infections like gastroenteritis, GI bugs, etc. [20:02] Other risks that have been talked about are dementia and kidney issues, often in older populations and patients who are generally sicker. Many people are on PPIs for general GI disorders unnecessarily. If you don’t need a drug, you shouldn’t be on it. [20:34] The risk/benefit analysis of the infections, and other people have talked about low bone mineral density, etc., have to be balanced with the risk of untreated disease and the potential risk of other medications, as well. [21:14] For the vast majority of patients, EoE is a chronic, long-term condition. Once you take the treatment away, whether that’s eliminating foods, or taking medication, the condition will come right back. That’s also true with proton pump inhibitors. [21:36] The recommended approach for PPIs is a high dose of medication twice a day, to start, a repeat endoscopy after eight to 12 weeks, and come down to maintenance, which would just be once a day. [21:55] Colleagues in Spain, who have published on this, have said about two-thirds of patients responded to the really high dose PPI medications and that among those people that respond, about two-thirds continue on maintenance and do well. Not everybody will necessarily respond or do OK with the lower dose of medication. [22:17] In general, if you take away the medications or the treatment that you’re using, the disease will come right back and those risks of progression or scar tissue, creating strictures, and developing symptoms would return as well. [22:39] If you are considering PPIs, ask your doctor their perspective on the different treatments that Dr. Franciosi has been discussing here. Considerations include PPIs, dietary elimination, swallowed steroid medications, and for some patients, biologic medications. Dr. Franciosi thinks biologics will become more used over the years. [23:43] Dr. Franciosi would recommend asking a treating provider about the choice of PPI medications, the dosing that they’re planning to use, whether or not it is high-dose medication, and even what the plan is for coming down off the medication with an endoscopy or a transnasal endoscopy in eight to 12 weeks, and maintenance. [24:03] In the U.S., high-dose PPI may not be covered by insurance. They may approve 40 mg of esomeprazole once a day but not twice a day, or make it challenging to get approved. The patients may do the second dose over the counter and Dr. Franciosi will walk them through how to do that. [24:58] Dr. Franciosi thinks the ideal would be that patients are diagnosed with eosinophilic esophagitis and then get a cheek swab to determine their profile and what kind of response they will have using esomeprazole or if he recommends oral viscous budesonide or fluticasone. It would also be great to identify who may respond or not respond to biologic medications. [25:37] The ideal of personalized or precision medicine is to be able to identify the right choice of treatment, including dietary approaches, for the right patient, at the right dosing. [25:50] An underappreciated area for EoE is recognizing that we don’t have good testing to identify food reactions, allergies, etc. It would be wonderful. Allergy testing doesn’t correlate because it’s mostly IgE based. So that’s a reason for dietary elimination. [26:38] Mary Jo and Holly thank Dr. Franciosi for participating in the podcast. Dr. Franciosi says the takeaway is that PPIs should be considered as an option for various patients. [27:20] To learn more about eosinophilic esophagitis, visit , , and . Holly and Mary Jo thank APFED’s education partners, linked below. Mentioned in This Episode: APFED on , , , , Education Partners: This episode of APFED’s podcast is brought to you thanks to the support of , , , and . Tweetables: “We’ve been very interested in what therapies may be effective for eosinophilic esophagitis, or EoE. The research that we’ve been doing … is trying to look at the right drug for the right patient, at the right dosing, etc.” — James Franciosi, MD “The main benefit [of using PPIs as a treatment for EoE] is that you can reduce the inflammation that’s occurring, [and] alleviate symptoms to make patients feel better.” — James Franciosi, MD “Once you take the treatment away, whether that’s eliminating foods, or taking medication, for the vast majority of people, the condition will come right back. That’s also true with proton pump inhibitors.” — James Franciosi, MD Featured speaker: Gastroenterology at /episode/index/show/eosinophil/id/27241587

A Global Look at Eosinophilic Disorders: Unmet Needs 05/18/2023

A Global Look at Eosinophilic Disorders: Unmet Needs Co-hosts Ryan Piansky, a graduate student and patient advocate living with eosinophilic esophagitis (EoE) and eosinophilic asthma, and Holly Knotowicz, a speech-language pathologist and feeding specialist living with EoE who serves on APFED’s Health Sciences Advisory Council talk with guest Florence Roufosse, MD, PhD, Professor of Medicine, Internist and Clinical Immunologist at CUB-Hôpital Erasme, Brussels, and President, International Eosinophil Society. In this episode, Ryan and Holly discuss with Dr. Roufosse some of the many unmet needs related to eosinophil-associated diseases. The discussion covers diagnosis, treatment, access to multi disciplinary care, research, and awareness. Dr. Roufosse identifies specific questions she sees an urgency to address and discusses clinical trials. Listen in for a better understanding of some of the unmet needs of patients with eosinophil-associated diseases. Disclaimer: The information provided in this podcast is designed to support, not replace the relationship that exists between listeners and their healthcare providers. Opinions, information, and recommendations shared in this podcast are not a substitute for medical advice. Decisions related to medical care should be made with your healthcare provider. Opinions and views of guests and co-hosts are their own. Key Takeaways: [0:50] Ryan welcomes co-host Holly Knotowicz. Holly introduces the topic: a global look at eosinophilic diseases and unmet needs. [1:28] Holly introduces Dr. Florence Roufosse. Dr. Roufosse is a professor of medicine, an internist, and a clinical immunologist at Erasme Hospital in Brussels and is President of the International Eosinophil Society. [1:43] In addition to her clinical work, Dr. Roufosse leads translational research projects to improve the understanding and treatment of hypereosinophilic syndromes (HES). She is also involved in international clinical trials evaluating treatment options for patients living with HES and eosinophilic granulomatosis with polyangiitis (EGPA). [2:18] Dr. Roufosse is an internist working in an academic hospital. An internist is a diagnostician, whom people see if they have many complaints that involve various organs to find a unifying diagnosis for their problems. [2:47] Dr. Roufosse’s interest in eosinophils started when she met Dr. Elie Cogan. He had just published a case report on a patient with hypereosinophilic syndrome who also had T-lymphocytes present. The T-cells were producing growth factors for eosinophils. [3:21] The hypereosinophilic syndrome in the patient was due to these abnormal T-lymphocytes. The case was in 1996. Dr. Cogan asked Dr. Roufosse if she would agree to do a doctoral fellowship on the topic and try to dive deeper into understanding the disease in this patient. [3:37] Dr. Roufosse has been working on HES ever since, doing translational research, working with blood samples from patients she sees in the clinic, and studying the cells functionally and on a molecular basis. Dr. Roufosse is foremost a clinician who sees patients in the clinic every day. [4:00] Dr. Roufosse regularly gets referrals for HES and EGPA. She has a large cohort of patients. Her main job consists of reaching a diagnosis and giving the best treatment to these patients. [4:44] The main mission of the International Eosinophil Society (IES) is to bring together clinicians, investigators, and researchers interested in eosinophils and eosinophilic disorders. The society held its first meeting in 2001. The pandemic prevented them from celebrating their 20th anniversary in person, but they will meet this summer. [5:20] IES brings together clinicians who see patients and people who investigate eosinophils in the lab. Close interactions between clinicians and investigators drive the motivation to understand the biology of these disorders, find the therapeutic targets, and work together, combining findings to make progress. [6:52] There are common unmet needs for eosinophilic diseases and specific unmet needs for individual diseases. Transitioning from pediatric to adult care is an unmet need in eosinophilic esophagitis because the disease begins in childhood. However, eosinophilic syndrome and EGPA occur most commonly in adults. [7:51] Diagnosis is a bottleneck. When people get the right diagnosis, they are already well on their journey of receiving treatment. Diagnosis combines the need for increased awareness among patients, the public, and physicians. A delayed diagnosis may have consequences for patients, such as a progressive disease that is not controlled. [8:31] High levels of eosinophils have the potential to damage organs and induce functional damage and functional alterations. They favor fibrosis. The longer eosinophils stay at high levels, the more harm can occur, so there is a consequence in delayed diagnosis. [8:55] Patients with high eosinophils in blood and tissues will often receive corticosteroid therapy. This is good for a few days but there are numerous side effects to corticosteroid therapy in the short-, middle-, and long-term. Also, the longer the journey is to diagnosis, the more people get discouraged and depressed. [10:02] Part of the unmet need in diagnosis is awareness. There’s a saying, “What you don’t know, you can’t diagnose.” Many physicians don’t know about these eosinophilic conditions. [12:13] APFED and Dr. Roufosse worked together to author “Improving Care in Eosinophil Associated Diseases: A Charter.” The charter outlines the needs and the rights of patients with eosinophil-associated diseases. [13:03] The rights of patients include the right to a timely diagnosis, which implies awareness about the diseases among the public, healthcare providers, and policy makers. Patients also have the right to access multi disciplinary care teams, as the disease may affect different tissues and organs. A patient may even have several disorders at once. [14:06] It’s important that if treatment options are available; patients need to have access to them. This is not the case in all countries. Patients need rapid access to the therapies once an indication has been decided by a specialist. [14:53] Quick diagnosis will decrease the accrual of damage due to the disease, inadequate treatment, and overexposure to corticosteroid treatment. From a psychological point of view, it’s very reassuring to rapidly feel that a label has been given to the disease and that there’s a plan. When there’s no label, there’s no plan. [15:47] The key to shortening the diagnostic timeline is awareness at all levels of healthcare education and specialization. General practitioners need to be educated on what it means and what to do when they see a high eosinophil count in blood or tissue. There needs to be access to the tools to diagnose the associated disorders. [17:00] Patients need easier access to referral centers where they see the experts and access the tools for diagnosis. Multidisciplinary team care is needed when diseases hit different organs. [17:44] When EGPA is controlled with immunosuppressive therapy, often asthma remains a serious problem and very difficult to manage. You need a pulmonologist to take care of that aspect. You need an ear, nose, and throat doctor for complications in those areas. Cardiovascular complications need a cardiologist. [19:21] Dr. Roufosse says it is still challenging for patients to access a multidisciplinary care team. Creating a multidisciplinary care team requires resources. It has to be supported by a hospital. The institution needs to be an academic institution with funding, as doctors meeting to discuss a difficult case does not generate income for the institution. [20:39] Ryan worked with a multi disciplinary care team as a young EoE patient. He traveled every year from his home in Georgia to the multi-disciplinary care team in Denver, Colorado. The fact that his family was able to travel out of state was wonderful. The team was effective and he misses it. Now he has separate appointments for each type of doctor. [22:32] New therapies, for those who have had access to them through clinical trials, are decreasing the need for corticosteroid therapy in many patients. Practically half of the patients with hypereosinophilic syndrome can be tapered off corticosteroids. Dr. Roufosse tells of one of her first patients, whose life was changed by these therapies. [24:04] Not every aspect of disease responds as well to these new therapies, revealing new unmet needs that are being tested with additional new therapies. Dr. Roufosse’s first patient has been helped greatly with biologics for almost 20 years now. [24:55] Dr. Roufosse speaks of more unmet treatment needs. Eosinophils are only a part of the picture. EoE is a complex disease about more than eosinophils. We need more understanding of what the key pathogenic events are across the range of eosinophil-related disorders to be able to identify new therapeutic targets. [26:00] With clinical trials, it can be difficult to find suitable endpoints for the trial and define the efficacy of a new treatment. When is the disease still active with treatment? When can we start decreasing the intensity of treatment? How long do we have to treat with these drugs? Many of these questions are still completely unanswered. [26:53] Over the past 25 years, the time to diagnosis has decreased. Dr. Roufosse rarely now has a patient referred to her that already has severe, irreversible damage. [27:21] How to score disease activity is an area where we still have a lot of work to do. [27:37] Medical codes are used to classify and group diseases for reporting statistical information. In the U.S., they are important for diagnostics and what treatments you get access to. In 2020, new codes (ICD-10) were approved for various subsets of eosinophilic diseases. [28:09] ICD-10 codes are used less in Belgium than in the U.S. but a disease does not “exist” if it doesn’t have a code. Without a code, there is no access to diagnostic tools or reimbursement for therapy for the disease. You need codes to justify hospitalization. C codes also allow Holly’s patients to receive feeding therapy. [30:43] To help patients and caregivers identify medical professionals offering care for eosinophil-associated diseases, APFED hosts a Specialist Finder on its website. It largely has clinicians in the U.S. You can access it at apfed.org/specialists. There is also a badge for specialists who are members of the International Eosinophil Society. [31:10] Dr. Roufosse suggests having handouts for patients listing patient associations and places to find information on their disease. Some people are more comfortable with something tangible and physical. Not everyone uses Google! Doctors need to use different means to share information on how to access specialists. [32:11] Policymakers are important in bringing together information to give access to proper care for these disorders. [33:56] Dr. Roufosse discusses unmet needs in research. We understand so little about these diseases, although great progress has been made in understanding that the eosinophil is a toxic cell that can cause damage and if you target those eosinophils, you are going to prevent some of that damage. We don’t yet have the full picture. [34:32] Some unmet needs are understanding what part of each of these disorders is influenced genetically or combines genetics with environmental triggers. What cells initiate the inflammatory process? What cells and mediators maintain the process? Which mediators should we target? How do we do it without compromising immunity? [35:12] It appears that targeting eosinophils in humans is innocuous, but what are they for? We are learning about what eosinophils are for in mice, but very little about what they’re for in humans. Are normal eosinophils irreplaceable or are there redundant functions with other cells? Some patients have high eosinophils without any illness. [35:46] Some patients have lower eosinophil counts than others and they have rapidly progressive cardiac damage. What characterizes a bad eosinophil? We don’t have the tools to recognize that, yet. We can’t predict which patients are going to require more aggressive therapy earlier in their disease course. That’s very important to research. [36:39] Dr. Roufosse recommends using the apfed.org website with its Specialist Finder and relevant patient resources. Awareness is the key to faster diagnosis, accessing treatment, and avoiding harmful treatment. [37:35] Ryan and Holly thank Dr. Roufosse for being on the podcast. Ryan shares the links including , , and links. Holly and Ryan thank APFED’s education partners, linked below. Publications discussed: Patient charter: Mentioned in This Episode: APFED on , , , , Education Partners: This episode of APFED’s podcast is brought to you thanks to the support of , , , , and . Tweetables: “The International Eosinophil Society is a dynamic ... society whose main mission is to bring together clinicians, investigators, and researchers ... interested in eosinophils and eosinophilic disorders. ” — Florence Roufosse, MD, PhD “Transitioning from pediatric to adult care is typically an unmet need in eosinophilic esophagitis, which very commonly begins in childhood.” — Florence Roufosse, MD, PhD “The unmet need, in … diagnosis, is awareness. … There’s a saying that what you don’t know, you can’t diagnose. … Many physicians don’t know about these eosinophilic conditions. … Turn toward someone … who is more likely than you to know.” — Florence Roufosse, MD, PhD Featured speaker: Professor of Medicine, Internist, and Clinical Immunologist at , President, Dr. Florence Roufosse is a Professor of Medicine, Internist, and Clinical Immunologist at CUB-Hôpital Erasme, Brussels, and is President of the International Eosinophil Society. She is in charge of a specialized consultation dedicated to diagnosing and treating eosinophil-related conditions, that is integrated in the European Reference Network: EuroBloodNet. She also manages patients with systemic auto-immune and auto-inflammatory conditions. Besides these clinical activities, Dr. Roufosse leads translational research projects to improve the understanding and treatment of lymphocytic variant hypereosinophilic syndrome (HES) and participates in international research efforts to better delineate disease course and treatment responses of HES. She is involved in the design and conduct of international clinical trials evaluating the efficacy of novel treatment options in patients with HES and eosinophilic granulomatosis with polyangiitis (EGPA), as well as sub-studies that aim to identify biomarkers and/or disease variants predicting treatment responses. /episode/index/show/eosinophil/id/26868096

Community Perspective: Eosinophilic Duodenitis and National Eosinophil Awareness Week 05/14/2023

Community Perspective: Eosinophilic Duodenitis and National Eosinophil Awareness Week Co-hosts Ryan Piansky, a graduate student and patient advocate living with eosinophilic esophagitis (EoE) and eosinophilic asthma, and Holly Knotowicz, a speech-language pathologist and feeding specialist living with EoE who serves on APFED’s Health Sciences Advisory Council talk with guest Jess Seidel, a patient advocate impacted by eosinophilic duodenitis. In this episode, Ryan and Holly discuss with Jess her diagnosis and experience with EoD, formerly known as eosinophilic gastroenteritis (EGE), some of the other health issues she experienced, and what her family’s support means to her. Ryan and Jess discuss visiting Washington, D.C., as young patients, on a trip to encourage U.S. legislators to support legislation related to medical formula coverage and the establishment of National Eosinophil Awareness Week so more people would have awareness of and access to the care they need. They all discuss the effects that National Eosinophil Awareness Week has had on access to diagnosis and care. Disclaimer: The information provided in this podcast is designed to support, not replace the relationship that exists between listeners and their healthcare providers. Opinions, information, and recommendations shared in this podcast are not a substitute for medical advice. Decisions related to medical care should be made with your healthcare provider. Opinions and views of guests and co-hosts are their own. Key Takeaways: [0:50] Ryan welcomes co-host Holly Knotowicz. Holly introduces the topic, eosinophilic duodenitis, or EoD (formerly referred to as eosinophilic gastroenteritis or EGE), and National Eosinophil Awareness Week. [1:27] Holly introduces Jess Seidel, a patient advocate impacted by EoD. [1:41] EoD is a condition that affects the digestive tract, caused by an increase of a type of white blood cell called eosinophils that causes inflammation. Jess was diagnosed with EoD when she was two years old. [2:30] Jess also had eosinophilic esophagitis and issues in the stomach, as well as the intestines. Her primary treatment was stopping food by mouth and using a feeding tube when she was seven years old. [3:11] Jess has early memories of hospital stays and doctor visits. An early memory that sticks out is trying to drink formula for lunch at school before getting a feeding tube. She notes that everyone was nice to her and curious about her drink. They asked her questions. [4:39] Jess’s younger brother was diagnosed with EoD when he was about seven years old. [4:48] Ryan’s older sister does not have an eosinophilic disorder. He speaks of how supportive she has always been of him and his needs. [5:28] Fortunately, Jess’s brother did not have the same intensity of issues as Jess. He didn’t need a feeding tube and his disease is under control at the moment. But there is something to be said for having somebody who just “gets it.” You don’t have to explain to them where you’re coming from. They just understand what you’re going through. [6:08] Ryan appreciates the annual APFED conference and hanging around people his age who have had similar experiences. [6:27] Holly is kind of jealous as she had an older brother who didn’t get it at all. He’s trying hard but he still struggles with it. He’s reading articles and learning more about why they have to pick certain restaurants. Holly enjoys working with people who have eosinophilic diseases. It’s therapeutic for her. [7:05] Jess is studying film and video production in college. Media has always been an escape for her. It allowed her to experience something different from what was going on in her life. But she always felt underrepresented in the media she consumed. She never saw well-rounded characters experiencing anything that she had. [8:14] When she got back into college and screenwriting and directing, she saw an opportunity not only to create more opportunities for escapism but also to champion more disability and chronic illness representation, both on the screen and behind the camera. [8:52] Holly and Ryan love that Jess is working advocacy into her studies and career path. Holly recently saw a medical drama that had a patient with a food impaction and she felt very seen. Patient advocacy is a huge part of APFED. [9:22] Ryan and Jess met at APFED conferences when Ryan was four or five but Ryan’s first memory of Jess was in 2007 in Washington, D.C. They had G-tubes and backpacks with pumps. They talked to legislators asking for help with formula coverage and the establishment of National Eosinophil Awareness Week. [10:43] Jess remembers the feeling of being in Washington, D.C. At age 11, she felt the gravity of the situation: how important it was that these legislators were listening and how important it was to get them to understand and pass medical formula coverage legislation so people could get the help they needed. It meant a lot to Jess. [11:40] Ryan was seven at the time. He was impressed by Jess as an older patient who clearly understood what they were doing in Washington, D.C. It was an inspiration to him, seeing someone who had these shared experiences with him, going out of the way to make sure more people could understand what patients are going through. [12:25] Jess points out that it’s such a rare disorder and at the time almost nobody knew anything about it. National Eosinophil Awareness Week allows us to put it in the forefront for a week and help amplify the voices of everybody that’s affected by it, from the patients to the families to the doctors working hard to try to treat it. [13:06] Jess has seen a huge uptick in awareness about eosinophilic disorders. When Jess got diagnosed at two, nobody knew what it was. She had to go to a specialist at a children’s hospital an hour from home to get the diagnosis. They went through doctors who told her parents they were overreacting or not being good parents by not making her eat. [13:39] The doctors didn’t know about eosinophilic disorders. There are more resources available now and it’s easier for people to access them. Jess started working with APFED at the beginning of its existence. Now, with a diagnosis, you can go to the internet and find great resources. [14:29] Ryan was diagnosed with EoE in 2002 when he was two. At first, the doctors said he was throwing up for attention. Today, the awareness of eosinophilic disorders like EoE has spread so much. We’re in a better place these days. [15:07] Holly’s journey was different, being diagnosed when older. Growing up, she and her mom were told they were crazy, or she was bulimic. Holly loves that there is now a dedicated week for eosinophil awareness. Patients can feel they have a community and be supported. [15:41] Jess’s condition varies from day to day or even hour to hour, affecting how she might be feeling. She can have great days and then days when she cannot function. She doesn't know what kind of day she’s going to have until she wakes. On a bad day, she’ll do what she needs to do to take care of herself and be kind to herself. [15:56] Since 2007, APFED has celebrated National Eosinophil Awareness Week during the third week of May. Resources for 2023 National Eosinophil Awareness Week, May 14th‒20th, may be found at . APFED currently has an awareness campaign running in New York, in Times Square, with a billboard raising awareness for eosinophilic esophagitis. [19:10] Jess thanks Ryan and Holly for having her on the Real Talk — Eosinophilic Diseases podcast. Jess says, if you’re dealing with an eosinophilic disorder, find a community to have people in your life who understand and support you. It is absolutely invaluable to have a support system and to know that you are not alone. Reach out. [20:02] Ryan and Holly thank Jess for taking the time to be on the podcast. Ryan shares resources including , , and . It’s all about raising awareness and finding a community of support. Holly thanks the education partners linked below. Mentioned in This Episode: APFED on , , , , Education Partners: This episode of APFED’s podcast is brought to you thanks to the support of , , , and . /episode/index/show/eosinophil/id/26832366

Community Perspective: EGPA 04/25/2023

Community Perspective: EGPA Description: Co-hosts Ryan Piansky, a graduate student and patient advocate living with eosinophilic esophagitis (EoE) and eosinophilic asthma, and Holly Knotowicz, a speech-language pathologist and feeding specialist living with EoE who serves on APFED’s Health Sciences Advisory Council talk with guest Ashley Spencer, patient advocate, a young adult from Bristol, PA, living with Eosinophilic Granulomatosis with Polyangiitis (EGPA). In this episode, Ryan and Holly discuss with Ashley her history with EGPA, how she was diagnosed, and some things you can do to advocate for yourself and others if you are living with EGPA. She explains the chronic nature of the disease and the treatments that help her in the day-to-day management of EGPA. You will appreciate Ashley’s positive attitude and determination to improve the outcomes of people living with EGPA. Disclaimer: The information provided in this podcast is designed to support, not replace the relationship that exists between listeners and their healthcare providers. Opinions, information, and recommendations shared in this podcast are not a substitute for medical advice. Decisions related to medical care should be made with your healthcare provider. Opinions and views of guests and co-hosts are their own. Key Takeaways: [0:50] Ryan welcomes co-host Holly Knotowicz. Holly introduces the topic: Eosinophilic Granulomatosis with Polyangiitis (EGPA) Today’s episode features the perspective of a patient living with EGPA. [1:52] Holly introduces Ashley Spencer, a young adult from Bristol, Pennsylvania, living with EGPA. [2:03] Ashley thanks Holly and Ryan for having her on the podcast. [2:19] Ashley says it is not a walk in the park living with EGPA. When Ashley was 16, she started displaying symptoms of EGPA. At the time, because of her age, doctors didn’t associate her symptoms with anything other than severe asthma. [2:45] Two years later Ashley developed sinus issues that required surgery. Every year she displayed more symptoms. [3:03] Ashley says EGPA has three stages and early diagnosis can halt its progress. [3:36] EGPA stands for Eosinophilic Granulomatosis with Polyangiitis. Eosinophils in the body are high, causing inflammation within the body including major organs. [4:01] Ashley had exercise-induced asthma. She played sports and danced, but all of a sudden, it went from exercise-induced asthma to severe asthma. She was admitted to the hospital for it and needed continuous albuterol treatments. From age 16 until now, Ashley has not been able to get off steroids, which can cause severe issues. [5:05] Ashley displayed sinusitis issues when she was 18. Within two years, she had four sinus surgeries in all eight of her sinus cavities for sinusitis and nasal polyps. [5:26] The polyps were starting to show eosinophilia but not enough for a full diagnosis. Because Ashley was moving from adolescence to adulthood, she had to be transitioned from doctors at Children’s Hospital Philadelphia to an adult doctor. Her family doctor told her she was getting worse. He sent her to National Jewish in Denver, Colorado. [6:12] Ashley checked into National Jewish Health for two weeks. Every day she saw doctors and had testing. Ashley was diagnosed with Churg-Strauss Syndrome, which is now known as Eosinophilic Granulomatosis with Polyangiitis (EGPA). [7:28] By the time Ashley got the diagnosis, she was in the last stage of EGPA, which is the vasculitic stage. [8:09] A common misconception about EGPA is that it doesn’t affect children and young adults. Another misconception is that EGPA patients may visually look healthy, similar to many autoimmune disease patients. EGPA affects the internal body. [9:18] Ashley’s lungs, sinuses, and her nervous system have been impacted. One morning she woke up and she was paralyzed from the waist down. This shut down her bladder function and she developed mononeuritis multiplex which caused severe peripheral neuropathy from her knees down. [9:48] Ashley was hospitalized for three weeks and then went to a rehab to relearn how to walk. Ashley worked with her urologist to get a medical device to signal when she needs to use the bathroom. [10:33] Ashley also has heart issues but if she stays on her daily maintenance medications, she does well. [11:09] Ashley talks about specialists she sees: a pulmonary doctor, an ear, nose, and throat specialist, a women’s healthcare team for bone health, a urologist, a neurologist, and an allergist and immunologist. [12:18] A good day for Ashley would be if she got out of bed, took a shower, and went to school. A bad day would be not being able to get out of bed and just staying in bed all day and sleeping. [13:17] Ashley and Holly discuss the “spoon theory.” You start the day with 10 spoons and each activity takes away one or more spoons. When your spoons are gone, you are done for the day. You don’t have more to give. [16:45] How you can help a friend with EGPA: Join a Facebook group for EGPA. Ashley shares a personal story of an online friend who became an in-life friend. She encourages listeners to explore Facebook groups related to eosinophilic diseases. [18:31] It’s hard for someonewith no experience with eosinophilic disorders to understand someone with EGPA. [20:33] There are situations you can explain that help your friends not living with eosinophilic diseases to better understand you. [21:41] Ashley has medication and a medical deviceto help her manage EGPA. [22:47] Ashley shares how her care has changed over the last ten years. When she started at the Cleveland Clinic, she went on a biologic to improve lung function that changed her life. She was able to work out again. Her stamina increased. It also helped a little bit with her neuropathy. [24:40] Ashley shares experiences with school and social activities with EGPA. She was in a college physical therapy program when her sinuses caused her to be admitted to the hospital. The doctors told her it was not feasible for her to continue to her senior year and she was heartbroken. She did not graduate or become a physical therapist. [26:23] Ashley’s career now is advocacy. She often speaks to new EGPA patients about the disease and treatment options. She also talks to allergy patients. Years ago, Ashley made national news by going into anaphylactic shock on a plane when flying to the Cleveland Clinic and a Cleveland Clinic doctor on the plane saved her life. [27:38] Ashley’s advice for traveling with EGPA is to wear medical identification jewelry. [30:03] Ashley refers to those living with EGPA as vasculitis warriors. She always invites them to connect with others on social media and to reach out if they need help. [30:58] Ryan shares the online resources to help with the day-to-day management of EGPA at apfed.org and apfed.org/connections. [31:28] Ryan and Holly thank Ashley for sharing her experience. Holly and Ryan thank APFED’s education partners, as well, linked below. Mentioned in This Episode: APFED on , , , , This episode of APFED’s podcast is brought to you thanks to the support of , , , , , and . Tweetables: “To be real with you, it’s not a walk in the park living with EGPA, let alone being diagnosed with EGPA.” — Ashley “When I was 16, I started displaying symptoms of EGPA. But at the time, because of my age, they didn’t associate it with anything other than just severe asthma. And then, two years later, I developed the sinus issue.” — Ashley “By the time I got the diagnosis, I was in the last stage [of EGPA], which is the vasculitic stage. So it was very sad.” — Ashley “It’s not really seen in youth patients and young adults.” — Ashley /episode/index/show/eosinophil/id/26637219

Community Perspective: EoE and Unsedated Transnasal Endoscopy 03/17/2023

Community Perspective: EoE and Unsedated Transnasal Endoscopy Description: Co-hosts Ryan Piansky, a graduate student and patient advocate living with eosinophilic esophagitis (EoE) and eosinophilic asthma, and Holly Knotowicz, a speech-language pathologist and feeding specialist living with EoE who serves on APFED’s Health Sciences Advisory Council talk with guest Eliana, a child living with EoE, about her experiences with endoscopies and unsedated transnasal endoscopies (TNE). In this episode, Ryan and Holly discuss with Eliana her history with EoE, her experience with an elemental diet, and the foods she is able to eat. They also discuss her experiences with endoscopies, including TNEs. You will enjoy her positive attitude and bravery. Disclaimer: The information provided in this podcast is designed to support, not replace the relationship that exists between listeners and their healthcare providers. Opinions, information, and recommendations shared in this podcast are not a substitute for medical advice. Decisions related to medical care should be made with your healthcare provider. Opinions and views of guests and co-hosts are their own. Key Takeaways: [0:57] Ryan welcomes co-host Holly Knotowicz. Holly introduces the topic: a follow-up to the recent episode with Dr. Joel Friedlander on unsedated or transnasal endoscopy. Today’s episode features a child living with eosinophilic esophagitis who has undergone several unsedated endoscopies, several of them with Dr. Joel Friedlander. [1:36] Holly introduces 10-year-old Eliana. During Holly’s time at Children’s Hospital Colorado, Holly had the opportunity to meet Eliana and her Mom. [1:49] Eliana thanks Holly and Ryan for having her on the podcast. [2:02] Eliana was diagnosed with EoE and food allergies at 18 months. She has a very restricted diet. Before her fourth birthday, she went on an elemental diet. This means she drinks a medical formula that provides nutrition. [2:41] Elemental means you can’t have foods, so you drink a specialized formula. Eliana can now tolerate approximately 12 foods in addition to the elemental formula. [3:30] Eliana’s safe foods include turkey, chicken, wheat, rice, corn, fish, flour, and natural flavors. Her favorite food is crispy-skin chicken. Her grandpa makes it extra-extra crispy for her! She has learned that one type of food can be five or six different foods if you get creative. [4:29] Eliana likes pan-seared salmon with oil and salt. She also enjoys when her family roasts or smokes a turkey. [4:44] Holly met Eliana when she was 18 months old. They didn’t know then what foods were her triggers. Eliana wasn’t interested in eating, which was a sign that something could be wrong. Now she eats foods with different textures. Her parents and brother help prepare food in different ways for her and her palate has expanded. [5:25] Since COVID-19 happened, Eliana and her family stayed home and cooked a lot. Sometimes Eliana enjoys cooking, but usually her brother or her dad cooks. [5:51] Because she was so young when she was diagnosed with EoE and food allergies, Eliana doesn’t remember the foods she would eat before she was diagnosed. [6:01] Eliana avoids the foods that she knows trigger symptoms. [6:23] Eliana is always excited when she is able to add new foods. [6:54] Ryan explains the endoscopy and transnasal endoscopy procedures and invites listeners to check out Episode 19 with Dr. Joel Friedlander. [7:58] Eliana has had 25 endoscopies, including traditional sedated procedures and unsedated TNEs. Twelve or more were TNEs. Eliana thinks the TNEs are way easier. She says it’s great to have a new way to do it. [8:29] Eliana was five when she had her first TNE following the introduction of corn, which was one of her first foods. She is always excited to get a new food added back to her diet. Her first TNEs were with Dr. Joel Friedlander. Dr. Friedlander doesn’t do them at Children’s Hospital Colorado anymore so Eliana has a new doctor now. [9:03] Eliana helped other doctors as they learned from Dr. Friedlander how to do the TNE procedure. She always got nervous when a new doctor would insert the tube. But she was brave and let them learn. [9:44] Eliana tells about her experiences with TNEs versus traditional endoscopies. The TNE is much faster and you don’t need anesthesia. [10:47] Eliana tells more about the TNE procedure. It starts like a normal check-up where you weigh yourself and they check your vitals and talk with you. They give you a big virtual reality setup and test it. You get to pick a show to watch. Then you head over to the procedure room and sit in a chair. Then they give you a nasal spray to numb your nasal passages. Eliana says it tastes like iron. [11:22] They send a tube up your nose and down your esophagus, which Eliana says feels big and you have to swallow it. It feels like choking. Then they take the biopsies and take the tube out. It takes about 10 minutes. She enjoys having hot tea or ice cream after the TNE. [12:00] With the virtual reality goggles, squishies, and stress toys, Eliana says it goes by faster than you think. [12:31] When the doctor takes the biopsies, Eliana says it’s quick and it feels like a scraping in your throat, like eating a cracker that gets a little stuck. Eliana asks for extra water when the doctor flushes her throat with water. It feels cold and fresh. [13:11] When she has a TNE, Eliana brings her own stress ball. They have stress balls but Eliana likes having hers. She also shares that it’s OK to ask for water or to turn up the volume. And she says taking deep breaths is helpful. [13:48] Eliana shares how watching a show on virtual reality goggles makes it fun. Eliana definitely prefers the TNEs to the sedated endoscopies. [14:29] Holly thanks Eliana for sharing her experiences to help others. [14:42] Eliana knows many people are nervous to get a TNE but says once you have your first one you’ll see it is much better than getting the anesthesia and waking up grumpy or tired. [15:05] Ryan thanks Eliana for coming on the show. [15:23] Ryan shares the APFED links shown below to find resources and specialists who treat eosinophilic esophagitis. [15:48] Holly thanks Eliana for sharing with us and being an inspiration to others. Holly hopes that when other patients who have EoE hear Eliana talk about her experience that they’ll be braver and more willing to try this out and that it can help them add more foods more quickly into their diet. APFED says “Thank you” to all the patients, families, and team members who have helped make unsedated endoscopy and new technologies possible. Mentioned in This Episode: APFED on , , , , Tweetables: “I was diagnosed at 18 months and I have EoE and lots of food allergies, so I have a very limited diet, and before my fourth birthday, I went elemental. … I still do drink a formula.” — Eliana “[Elemental] means you can’t have foods so you’re not eating any foods … only drinking the formula.” — Eliana “It takes a really long time to add in new foods because most of the time it doesn’t work out. … I have 12 foods or more now.” — Eliana “I like pan-seared salmon with oil and salt. Then we roast turkey and my brother smokes turkey sometimes.” — Eliana “[The first time I had a TNE] I was five and I was trying corn. Corn was one of my first foods.” — Eliana “I know plenty of people who are nervous to do [the TNE], but once you do your first one, it’s like, this is way better than the smelly anesthesia and waking up grumpy or tired!” — Eliana /episode/index/show/eosinophil/id/26256021

Pediatric Unsedated Endoscopy: Diagnostic Tool Use and Development 02/23/2023

Pediatric Unsedated Endoscopy: Diagnostic Tool Use and Development Description: Co-hosts Ryan Piansky, a graduate student and patient advocate living with eosinophilic esophagitis (EoE) and eosinophilic asthma, and Holly Knotowicz, a speech-language pathologist and feeding specialist living with EoE who serves on APFED’s Health Sciences Advisory Council talk with guest Joel Friedlander, DO, about the development and use of transnasal endoscopy (TNE), also known as unsedated endoscopy. Dr. Joel Friedlander is a co-founder of EvoEndo and a pioneer of pediatric transnasal endoscopy. Previously, he was the Gastroenterology lead of the Aerodigestive Program at Children’s Hospital Colorado and a bioethics consultant. In this episode, Ryan and Holly discuss with Dr. Friedlander the background and history of unsedated endoscopy. Dr. Friedlander talks about the team at Children’s Hospital Colorado who worked with him on the technology and the procedure. Dr. Friedlander first noticed a similar procedure being used by ENT physicians and speech and language pathologists and wanted to use it for pediatric eosinophilic esophagitis patients. He explains the research they did\. Now Dr. Friedlander and some of his colleagues from the multidisciplinary team at Children’s Hospital Colorado have co-founded a company to promote and spread the use of this technology around the country. Listen in for more information about the use of unsedated endoscopy for EoE. Dr. Joel Friedlander would like to extend special thanks to the individuals and groups who contributed to the advancement of the unsedated transnasal endoscopy for eosinophilic esophagitis. This includes Dr. Jeremy Prager, Dr. Robin Deterding, Dr. Emily DeBoer, the GI and AeroDigestive teams at Children’s Hospital Colorado, APFED, the Bunning Family, the Meister Family, CEGIR, CURED, the Cherry Family, the Friedlander Family, and the Smith Family. Disclaimer: The information provided in this podcast is designed to support, not replace the relationship that exists between listeners and their healthcare providers. Opinions, information, and recommendations shared in this podcast are not a substitute for medical advice. Decisions related to medical care should be made with your healthcare provider. Opinions and views of guests and co-hosts are their own. Key Takeaways: [1:07] Ryan welcomes co-host Holly Knotowicz. Holly introduces the topic: a new diagnostic tool for EoE: transnasal endoscopy, also known as TNE. Holly introduces the episode guest, Dr. Joel Friedlander. [1:50] During her time working at Children’s Hospital Colorado, Holly enjoyed collaborating with Dr. Friedlander to support children living with eosinophilic esophagitis. [2:22] At Children’s Hospital Colorado, Dr. Friedlander was part of a multidisciplinary program of ENT physicians, pulmonary physicians, a gastroenterology physician, feeding and speech therapists, occupational therapists, and nutritionists, to work with children who had upper airway disorders, or eosinophilic disorders of the upper GI tract. [2:44] Dr. Friedlander noticed that their ENT colleagues could look in individuals’ throats every day, without anesthesia, using tiny scopes, and speech and language pathologists would use little cameras to look in the back of people’s throats to watch swallowing. [3:00] The multidisciplinary team at Children’s Hospital Colorado wanted the same technology in gastroenterology. They put together some research, which led to a startup company. Dr. Friedlander is the Chief Medical Officer of the company, trying to bring unsedated endoscopic technology to patients around the world. [3:26] Unsedated endoscopy is especially important to patients with eosinophilic disorders that require numerous endoscopies with anesthesia. Doctors don’t want to have their patients go to sleep unless they have to. Although anesthesia is relatively safe, it’s not without potential risks, it drives up the cost of care, and it is inefficient. [3:57] Dr. Friedlander’s research was how to bring the unsedated endoscopic technologies their colleagues had to gastroenterology patients, specifically for eosinophilic disorders. Holly Knotowicz, as a person who has EoE and as a medical provider, loves to hear about creative ways to take care of these patients. [4:47] Holly knows that seeing doctors is scary, but she loved that Dr. Friedlander always had a joke for his pediatric patients. Holly asked Dr. Friedlander for a joke today and he provided a couple of his oldies but goodies. [5:45] One of the most important parts of doing any awake procedure with patients is having a relationship. It is a medical procedure. With this technology, there are different styles. You develop a style as a physician; Dr. Friedlander tells jokes. You also have to know your patient. Every patient is different. [7:35] Dr. Friedlander researched the use of unsedated endoscopic technology to remove sedation risks to pediatric patients, reduce their parents’ fears, make faster and safer diagnoses, optimize the patient’s experience, and lower the cost of care. [8:32] As Dr. Friedlander’s group was developing the technology for TNE, they learned that adult gastroenterologists had tried to introduce it over 20 years ago but it never took up much traction in the U.S. When they started doing it in pediatrics, initially they started using the smallest scopes available, but the little scopes were breaking. [10:01] They went to the hospital asking for another scope to make the technique even better. Originally, an ENT physician would do the procedure through the nose and a gastroenterologist would go into the esophagus, requiring two physicians for a short procedure. So the ENT physician taught the gastroenterologist the nasal procedure. [10:24] The other problem was how to keep a child calm for the four-minute procedure. They tried using video goggles and then moved to VR. After working through about 1,000 procedures in Colorado, they felt they had the right flow for the procedure and patient experience for the children, including Dr. Fiedlander’s jokes. [11:28] Some kids like jokes. Some kids like VR. The team had to choose which kids were the right fit for unsedated TNE. Unsedated TNE works for the vast majority of kids but it’s not for everyone. About five to 10% think it’s easy, about five to 10% say it’s not for them, and the rest can manage the few minutes it takes, vs. anesthesia. [13:30] A typical endoscope used for sedated upper GI tract endoscopy is nine or 10 mm in diameter. An adult transnasal scope (a baby scope for sleeping babies), or noodle scope, is about 5‒6 mm. A transnasal scope for pediatric TNE is 3‒4 mm, like a piece of spaghetti. The different scopes have different features. [14:54] The ENT physician of the group, Dr. Jeremy Prager, worked with Dr. Friedlander to teach him the TNE technique. Dr. Robin Deterding, Chief of Pulmonology at Children’s Hospital Colorado fostered the development of the technology. Dr. Emily DeBoer also collaborated on the initial project. [15:34] Dr. Friedlander, Dr. Prager, Dr. Deterding, and Dr. DeBoer are the co-founders of the company where Dr. Friedlander currently works. They all came together to figure out the best way to bring this unsedated technology to patients, through new scopes and constantly refining the techniques. [15:49] Dr. DeBoer developed 3D models for training doctors who had never used a 3 mm scope or navigated a child’s nasal passage. It takes time for a doctor to learn the technique and get comfortable with the smaller scopes. [17:26] The typical procedure starts with the patient having a conversation with the doctor who is doing it. The doctor will also talk during the procedure. If VR is used, the doctor orients the patient on how to use it and picks a program. There is the orientation to the scope and the procedure and how the patient may feel during and after it. [19:54] Then there is an orientation to the medication administration, including numbing spray in the nose. Dr. Friedlander asks patients ahead of time to practice nasal spray at home to see how it feels. [21:13] Some kids do well with the procedure and others do not. Dr. Friedlander has patients practice slow, deep breaths to help them relax if they start to feel uncomfortable. He tells patients not to hold their breath or pant like a dog. If they take slow, deep breaths and watch the show, it will be over before they know it. [22:21] After preparation, they go to the procedure room. They check who the patient is and their birthday. They use numbing spray on the nose (or throat), put on the VR goggles, have the parent sit behind if the child needs their handheld, turn on the show, turn down the lights, take the camera scope, lubricate it, and do the procedure. [23:10] The scope goes in the nose and down the back of the throat. Dr. Friedlander may ask the patient to swallow it like spaghetti. With the scope down, the doctor takes a few biopsies and removes the scope. The patient may feel it a little bit, based on how sensitive they are. [23:29] Afterward, Dr. Friedlander brings out an ice pop or a slushie to soothe the throat. He recommends the child takes little sips because their throat may still be a little numb. As it wakes up, they can drink more. He recommends eating slowly for the rest of the day. [24:21] As the scope may look in the stomach, patients are to take no food or drink for four hours before the procedure. If the scope will only look at the esophagus, patients are to take no food or drink for two hours before the procedure. Because the scope goes down the back of the throat, there is a slight risk of throwing up. The time for the procedure varies per doctor and location. [25:32] For EoE, usually distal and proximal biopsies are taken, between two and four biopsies each from the bottom and the top of the esophagus, depending on the physician. If going to the stomach or the small intestine, additional biopsies would be taken from those areas. [26:18] Your physician determines how frequently to do this procedure. Based on current data, it shouldn’t be done any sooner than six to eight weeks. With anesthesia involved, it wouldn’t be done that frequently because of the cost. With unsedated technologies, six weeks is OK. Ongoing research may lead to shorter intervals. [27:30] The usual biopsy frequency for patients with EoE is between three to six months depending on your physician, what diet you’re on, and your therapy. If your eosinophilic disorders are stable, and you’re doing well, it might be just once every two years. There is a lot of variation depending on the institution and the physician. [27:48] Before undergoing unsedated TNE, the child or adult needs to go through COVID-19 testing as the scope is going through the nose and there would be a risk of spreading COVID-19 if infected. Some kids find the COVID-19 test to be worse than the TNE. [28:51] TNE can also be done with sedation, so Dr. Friedlander refers to it as unsedated TNE. Dr. Friedlander discusses the benefits of unsedated TNE: 1.) no sedation, 2.) possible increased frequency of endoscopy, depending on your physician, 3.) increased efficiency with less downtime, and 4.) decreased cost, depending on your insurance. [30:37] When the fear and cost of anesthesia are out of the equation, the question is, how often do you need an endoscopy? Doctors have to be thoughtful about this. Just because you can scope doesn’t mean everybody needs a scope. But you can scope to see if your medication worked. It opens up opportunities for other diagnoses. [32:00] A patient with trouble swallowing could have EoE or they could have reflux esophagitis, which is treated very differently from an eosinophilic disorder. An unsedated TNE could show which esophagitis the patient has. [33:17] When a physician is getting started with unsedated TNE, older patients are the easiest because the anatomy is bigger and it’s easier to talk an older patient through it if the procedure is not going well. Some patients have been to ENT doctors and had good experiences with nasal tubes, they make good patients for unsedated TNE. [34:31] Older patients routinely calm down easily. A child (or adult) who screams for minutes after a blood draw is probably not the best patient for an unsedated TNE procedure. The ideal patient is someone the physician can talk to and calm down if they get upset, and patients that are used to nasal medications. [35:06] Until patients try the procedure, it’s not known how a patient will do. With kids under age five, it’s hard to use VR or talk to them. Some kids under five can do it. It’s about a 50-50 chance, compared to over 90% success with older kids. [36:23] For physicians first starting to perform unsedated TNE, 5-to-8-year-olds are more challenging than 9-year-olds and up. Even so, some teenagers are not the right candidates for unsedated TNE. Dr. Friedlander always recommends they try it. Sedated endoscopy requires a whole day off work or school, and unsedated TNE can be done in minutes. [37:38] Adult TNE has been around for years. Most adult centers have transnasal endoscopes but a lot of physicians haven’t been trained in TNE. Some use TNE in the high-risk adult population with significant heart or lung disease and for adult patients who don’t have a driver to take them home after sedation. [38:38] More adult physicians working with eosinophilic disorders are asking how they can offer unsedated TNE to their patients. Adult transnasal endoscopes are thicker than pediatric scopes. Dr. Friedman’s company has longer 3 mm transnasal endoscopes for more comfortable adult use. [39:29] Risks with unsedated TNE may include a sore or swollen nose, sore throat, sore chest, and a little bleeding from the biopsy sites. One to two percent may have some spitting up or throwing up. It’s a medical test. It may go well, it may not go well; probably it’s somewhere in the middle. It’s five to ten minutes and you’re back to school or work. [40:19] There are the same risks as other endoscopies. A scope can go where it’s not supposed to. That seems to occur less than with a regular scope because you may not be going as far and the patient isn’t asleep and can report if something hurts. [41:00] Unsedated endoscopy is a very important tool for a gastroenterologist to have. It’s not a replacement for all endoscopies. As of now, dilations are not routinely done unsedated. Transnasal endoscopes are not designed or sized for therapeutic use. They are for diagnosis and evaluation. [43:27] Holly recalls a mutual patient that couldn’t tolerate unsedated TNE but had a gastric tube and tolerated a trans-gastric endoscopy. Dr. Friedlander researched it and found that the TNE scope fits well through a gastric tube. It takes additional manipulation to use it in that manner. [45:50] If you are interested in trans-gastric endoscopy, discuss it with your physician. [46:40] Concerning insurance for unsedated TNE, the first step is to talk to your physician and care team. If they agree the procedure is appropriate, you can discuss the cost estimate with your insurance provider. [48:41] More and more centers around the country are doing unsedated TNE. Some have websites about it. Google transnasal endoscopy in your region or city and you’ll find websites. APFED is also working on adding information to its Specialist Finder to show these centers. Most importantly, ask your physician about it. [49:40] Holly thanks Dr. Friedlander for sharing his expertise. [49:50] Dr. Friedlander says he is passionate about getting this technology out to the right patients. It’s a good option for a large majority but it’s not for everybody. Know that this is an option. Ask your physicians about this option. Figure out if it’s the best option for you and your treatment plan. Physicians take their cues from their patients. [50:34] Dr. Friedlander thanks Ryan and Holly for allowing him to speak about unsedated TNE and everyone who allowed all this technology to get to where it is, from adult doctors working on it 20 years ago, pediatric doctors working on it now, the eosinophilic disorders community, and APFED. This is an important option for patients. [50:53] Dr. Friedlander asks you to talk to people about it. Let people know about it. It’s an important technology. It’s not for everybody but it’s for a lot of people. [51:08] Ryan shares the APFED links shown below to find resources and specialists who treat eosinophilic esophagitis and to make connections with others impacted by eosinophilic diseases by joining APFED’s online community. APFED says “Thank you” to all the patients, families, and team members who have helped make unsedated endoscopy and new technologies possible. Mentioned in This Episode: APFED on , , , , This episode is brought to you thanks to the support of our Education Partners , , , , and . Tweetables: “If our ENT colleagues could do [unsedated endoscopy] and if our pulmonology colleagues could do [unsedated endoscopy], we want to make sure that our gastroenterology colleagues have these same options for our patients.” — Dr. Joel Friedlander “As much as [anesthesia] scare[s] our patients, it also scares us as providers and doctors, because we don’t want to have our patients go to sleep unless we really need to because, although anesthesia is relatively safe, it’s not without its potential risks.” — Dr. Joel Friedlander “When a physician is first getting started [with unsedated TNE], usually an older patient is better because the anatomy is bigger. It’s easier to talk a patient through it if they’re not doing as well.” — Dr. Joel Friedlander “[Unsedated TNE] is an important option for our patients. … Talk to people about it. Let people know about it. It’s a really important technology. Even if not for everybody, it’s for a lot of people.” — Dr. Joel Friedlander /episode/index/show/eosinophil/id/26004123

Transition of Care from Pediatric to Adult Providers 01/18/2023

Transition of Care from Pediatric to Adult Providers Description: Host Ryan Piansky is joined by co-host Mary Jo Strobel, APFED’s Executive Director, to talk with guest, Girish Hiremath, MD, MPH, about the transition of care for people living with eosinophilic disorders from pediatric to adult providers. Dr. Hiremath is a pediatric gastroenterologist at Monroe Carell Jr. Children’s Hospital at Vanderbilt in Tennessee. His clinical practice focuses on pediatric patients with eosinophilic gastrointestinal disorders and his research concentrates on the epidemiology, diagnosis, and optimal management strategies to advance clinical outcomes in individuals affected by eosinophilic gastrointestinal diseases, in general, and eosinophilic esophagitis, in particular. In this episode, Ryan and Mary Jo discuss with Dr. Hiremath transition in health care from a pediatric to adult setting, his recently, published research on transition of care, and his interdisciplinary research program at Vanderbilt. Strategies to help facilitate transition and ages to do so, common barriers, and teaching children skills to manage their health are also discussed. Disclaimer: The information provided in this podcast is designed to support, not replace the relationship that exists between listeners and their healthcare providers. Opinions, information, and recommendations shared in this podcast are not a substitute for medical advice. Decisions related to medical care should be made with your healthcare provider. Opinions and views of guests and co-hosts are their own. Key Takeaways: [1:10] Ryan welcomes co-host Mary Jo Strobel. Mary Jo introduces the topic: transition of care from pediatric to adult providers, and the episode guest, Dr. Girish Hiremath. Mary Jo welcomes Dr. Hiremath to the podcast. [2:33] After medical school in India, Dr. Hiremath served in the Indian Navy. Later, he traveled to the U.S. to pursue a Master’s in Public Health at Johns Hopkins. He completed his pediatric residency at INOVA Fairfax Hospital for Children in Virginia and his pediatric GI fellowship at Texas Children’s Hospital, Baylor College of Medicine. [3:03] Dr. Hiremath joined Vanderbilt in 2015. He has taken care of numerous children with gastrointestinal problems. He has also had the unique opportunity to focus on eosinophilic gastrointestinal diseases (EGIDs) and build a multi-disciplinary EGID clinic to provide evidence-based care to pediatric EGID patients, and he developed an interdisciplinary research program. [4:03] Pediatric gastroenterologists will typically treat patients from newborn up to age 18 but there is some variability. Some facilities will treat patients of up to 22 years. The pediatric healthcare provider will look for the right time to transition the pediatric patient to an adult healthcare provider, based on the patient’s readiness and maturity. [4:44] The pediatric healthcare provider will assess if the patient is stable to transition to an adult healthcare provider. If the patient is not doing well from a disease standpoint, it may not be the time to transition this patient. The provider will hold on, stabilize the patient and get them on a good trajectory to transition to an adult healthcare provider. [5:41] Chronic diseases, such as EGIDs, are being identified early in life, in the pediatric age group. Many chronic conditions do not have a definitive cure. Pediatric patients with chronic disease carry their disease into adulthood. The transition of care is very important so they continue to receive due care and guidance to manage their condition. [6:17] The National Alliance to Advance Adolescent Health describes transition of care as the process of moving from a child- or family-centered model of healthcare to an adult- or patient-centered model of healthcare, with or without transferring to a new clinician. It involves planning, transfer, and integration into adult-centered healthcare. [6:41] A successful healthcare transition program will involve a flow of information through multiple stakeholders. The most important stakeholder is the patient on the verge of adulthood. Some other stakeholders are the parents, patient’s caregivers, healthcare providers, and patient advocacy groups, which play a big role in education. [7:20] A transition program can be considered a success if it can improve the ability of the youth and young adults to manage their healthcare and effectively use health services. It’s an organized process with an emphasis on getting the patients integrated into adult-centered healthcare from child/parent-centered healthcare. [8:26] In April 2022, Dr. Hiremath contributed to a publication on transition of care for patients with EGID. The co-authors were a team of experts in EGIDs, including pediatric gastroenterologists, adult gastroenterologists, allergists and immunologists, and experts from Europe and the U.S. on transition of care. It was a great team effort. [9:11] They reviewed data from other chronic conditions such as inflammatory bowel disease and asthma, where relatively more research has been done on transition of care from pediatric-based to adult-based healthcare. Like EGIDs, these conditions can be debilitating and require long-term therapy and office-based assessments and testing. [9:40] Understanding approaches used for other medical conditions to transition care can help the EGID field identify opportunities for smoother transfer of care. They found that from the patients’ perspective, there is a lack of awareness, and suboptimal adherence and compliance to medical therapies, one of the critical challenges that have been well-documented in the field. [10:02] Factors that contribute to these problems are the patients’ young ages and their lack of understanding about the disease’s activity status. For example, there is sometimes a perceived lack of benefit of medication when a patient is feeling well. That age group also exhibits risk-taking behaviors. Plus, education about insurance coverage is helpful. [10:44] All these factors contribute to understanding how to make transition of care more efficient. There are health-system-related issues, too. The providers have a lack of time to focus on the transition of care and education related to that. There is not enough institutional support. There is little reimbursement for services provided. [11:17] These are barriers that have been well-described for other chronic conditions that also apply to EGID patients. [11:40] Ideally, transition of care of EGID patients should focus on the continuity of care through a shared decision-making process. [11:55] It can be helpful to allow adolescents to be independent in care management decisions and to create a comfortable and supportive environment for transitioning to adult care. [12:19] The research team also felt that the transition programs for EGID should be a continuing process that facilitates ongoing quality improvement and learning for all stakeholders. [12:39] Dr. Hiremath and his team were surprised by how little attention has been paid to the transition of care for young EGID patients. Some patients feel good and are not convinced that EoE is a chronic, progressive disease so they do not think much about continued care in their transition to adulthood. There are also provider gaps. [13:17] The disease process of EoE is not fully understood. Other chronic diseases have been studied longer and the disease process is better known. This highlights a great opportunity for researchers to systematically study transition of care and make important contributions to the field. [14:34] There are differences between working with a pediatric doctor and an adult provider. Pediatric providers are part of the family-centered care environment where the patient, parents, and providers are engaged in creating a treatment plan. Adult providers are part of the patient-centered care model, interacting directly with patients. [15:16] EoE presents differently among different age groups. Pediatric providers take care of EoE patients in the inflammatory phase. The adult providers will often see EoE patients when they present with complications such as strictures or food impactions. [15:51] Ryan recalls how, as a pediatric patient, his parents were involved in his care and also expected him to be involved and to help make decisions about treatments. [16:47] In his clinical practice, Dr. Hiremath initiates the process of transition of care when a patient is 12 or 13 years old. He starts with a conversation and introduces the concept of getting the child involved in the decision-making process about their ongoing treatment, changes in treatment, and when to do an endoscopy. He encourages the child to participate. [17:27] Dr. Hiremath encourages patients to educate themselves on the disease. Vanderbilt has the EoE Education Clinic to educate parents and patients. The transition of care is a part of that discussion, about transitioning into adulthood and having an adult healthcare provider. [18:11] Parents need to research adult gastroenterologists in their area who will be able to assume care of their adolescent child when they are ready to transition. This involves logistics such as travel and insurance coverage. Giving parents time to think through the issues will make the process more seamless. [19:19] A child’s doctor may be able to recommend an adult provider. In his practice, Dr. Hiremath makes referrals to EoE gastroenterologists within the Vanderbilt system for a seamless transition.[21:15] The goal is a seamless transition from pediatric to adult care. Unfortunately, there’s no single model program that fits all clinics and patients. Structured transition models improve treatment plan adherence and chronic disease control. However, for EGID patients, a diverse group of providers is involved and they all play a critical role. [22:00] With such a variety of providers, it’s hard to have one standard transition model but there is a specialized-disease-based approach that focuses on engaging adolescent EGID patients and their caregivers, and the stakeholders can facilitate the progression of that care. [22:18] This approach is often very personnel-heavy. It has to be tailored to the needs of the specific institutions, and it requires considerable resources for implementation and delivery. It cannot be translated from Institution “A” to Institution “B.” Each provider at the institution has to assess what they can offer, can afford, and what is most efficient. [23:33] Dr. Hiremath tells how he introduces the topic of transition of care with the intent to give the parents and the patient enough time to think through the process of finding an adult provider without being rushed and educate themselves on what the options are. [26:29] Transitioning from a pediatric provider to an adult provider is an important period. A patient who feels good may think, “I’ve conquered EoE,” ignoring that it’s a chronic progressive disease. Or it may be difficult to locate an adult gastroenterologist who treats EoE patients. These factors contribute to a loss of follow-up. [27:55] Dr. Hiremath explains some of the concerns he hears from patients transitioning to adult care. They have questions about who to call for prescription refills and to set up their next endoscopy. As they turn 18, they are quite knowledgeable about managing their disease but they need to know a lot more about the logistics of the transition. [29:11] There is a limited understanding of the differences between symptoms, diagnostic testing, and treatment in children versus adult patients. In the adult world, there are limited allergists and immunologists who focus on EGIDs. There are fewer adult gastroenterologists who focus on EGIDs. [29:44] There’s a lack of training in transitional care and there are very few transitional programs. The electronic health record platforms of the two institutions may not talk to each other. Information can drop between the cracks. There is limited funding for infrastructure, educational materials, or administrative support for a transition program. [30:38] In the U.S., the patient’s insurance plays an important role in continued healthcare, specifically for EGID-related care. In some cases, it determines who the patient gets to see, how often they can receive procedures, and what medications and doses are covered. The kind of insurance can dictate the depth of care in the U.S. [31:39] EGIDs and EoE are chronic, progressive diseases. They are here to stay for a long time and they advance slowly but surely. If medications or treatments are stopped, the patient is at risk of developing complications. In EoE, people with poorly-controlled inflammation are at higher risk of complications. [32:18] People who are not adherent to their treatment plan are at a higher risk to develop complications, such as strictures or food impactions, requiring urgent or emergent endoscopy. Patients who have inflammation that is not well-controlled tend to withdraw from social circles, and avoid eating with friends. This affects emotional well-being. [33:53] Dr. Hiremath emphasizes again that EGIDs are chronic, progressive diseases. There will be periods where a patient will be feeling great and asymptomatic or will be compensating for their symptoms. The key is to control inflammation and minimize the future risk of complications. This requires long-term medical treatment. [34:20] Prepare early to transition to adult care. The child doesn’t have to know every medication, dose, and frequency, but start by explaining to the child about the medication they are taking. Over time, the patient will take ownership. Dr. Hiremath talks to both his patients and their parents. [36:10] Mary Jo thanks Dr. Hiremath for talking about transition of care and participating in the podcast and she asks Dr. Hiremath about additional resources for patients. [36:21] Online resources Dr. Hiremath recommends include GotTransition.org, The American Academy of Pediatrics, and sites for other chronic diseases that have information on the transition of care, such as Crohn’s & Colitis Foundation. [37:58] Ryan shares the APFED links shown below to find resources and specialists who treat eosinophilic-related diseases and to make connections with others impacted with eosinophilic diseases by joining APFED’s online community. [38:35] Ryan thanks Dr. Hiremath for sharing his research and experiences. Mentioned in This Episode: APFED on , , , , by Girish Hiremath, Adrian Chapa-Rodriguez, David A. Katzka, Jonathan M. Spergel, Benjamin Gold, Albert J. Bredenoord, Evan S. Dellon, Jeannie Huang, and Sandeep K. Gupta This episode is brought to you thanks to the support of our Education Partners , , , , and . Tweetables: “There’s a lot of variability here. Typically, pediatric gastroenterologists take care of newborns to children up to 18 years of age. Having said that, that’s not etched in stone. … It’s mostly patient-related factors.” — Girish Hiremath, MD, MPH “More and more chronic diseases, such as EGIDs, are being identified early in life, in the pediatric age group. Unfortunately, many of these chronic conditions do not have a definitive cure at this time.” — Girish Hiremath, MD, MPH “Unfortunately, there’s no single standard model that fits all transition programs. Structured transition models have been shown to improve medical treatment plan adherence and disease control.” — Girish Hiremath, MD, MPH “Efforts are needed to understand what kind of model would be optimal or most efficient for EGID patients.” — Girish Hiremath, MD, MPH “At the end of the day, it’s their health that they are responsible for.” — Girish Hiremath, MD, MPH “Maybe we can venture out into those [social media] platforms and capture a young audience so that we’re able to reach them in the language that they speak and connect with them.” — Girish Hiremath, MD, MPH /episode/index/show/eosinophil/id/25636986

Starting An Elimination Diet for Children With Eosinophil-Associated Diseases Such as Eosinophilic Esophagitis (EoE) 12/21/2022

Starting An Elimination Diet for Children With Eosinophil-Associated Diseases Such as Eosinophilic Esophagitis (EoE) Description: Host Holly Knotowicz, a speech-language pathologist who serves on APFED’s Health Sciences Advisory Council, talks with guest Laura Gearman, MS, RD, LD, CNSC about the use of dietary therapy to treat eosinophilic esophagitis (EoE). Laura is a Registered Dietitian who specializes in pediatric nutrition. She is the Clinical Nutrition Manager and a Pediatric Dietitian at the University of Minnesota Masonic Children’s Hospital. She has contributed to multiple publications regarding pediatric malnutrition and nutrition support. Plus, she is a mom of two, including a six-year-old daughter who has EoE. In this episode, Holly and Laura discuss working with children with EoE and their families. They explore elimination and elemental diets, and how to help children manage these special diets. They discuss misconceptions about these diets and when it is best to use them in the treatment of EoE. Disclaimer: The information provided in this podcast is designed to support, not replace the relationship that exists between listeners and their healthcare providers. Opinions, information, and recommendations shared in this podcast are not a substitute for medical advice. Decisions related to medical care should be made with your healthcare provider. Opinions and views of guests and co-hosts are their own. Key Takeaways: [1:09] Holly introduces today’s topic, Starting An Elimination Diet for Children With Eosinophilic Diseases Such as Eosinophilic Esophagitis (EoE), and today’s guest, Laura Gearman, a Registered Dietitian who specializes in pediatric nutrition. [1:58] Laura knew from a young age that she wanted to work with children and was also very interested in cooking and the science of food. With those interests, becoming a pediatric dietician made a lot of sense. Through her master’s degree at Tufts, Laura got exposure to pediatric clinical work and she knew that’s where she wanted to be. [2:32] For the past 12 years, Laura has been at the University of Minnesota Masonic Children’s Hospital, working with critically ill children, while raising her own two children. [3:09] An elimination diet consists of eliminating many or all of the most common allergens including dairy, egg, wheat, soy, peanuts, tree nuts, fish, and shellfish. Some EoE care teams encourage a six-food elimination diet. Some elimination diets may start with removing dairy (milk) and/or wheat, which are common EoE triggers. Elimination diets take a lot of planning. [3:38] First, the family needs education from a registered dietician familiar with EoE on what the elimination diet entails and what foods to avoid. Most importantly, education should be focused on what foods patients are still able to eat. Feeding children is instinctual and there is a lot of emotion involved in it. [4:56] The main goal of an elimination diet is to identify which foods may be triggers for EoE. A lot of centers are moving toward eliminating just a couple of foods at a time. It’s a long process to find which items may be triggering symptoms of EoE. The elimination diet will continue to evolve. [5:23] An elemental diet removes allergens and consists of an amino acid-based formula. It may include one or two simple foods, like lollipops. One or two low-allergen foods may be introduced to keep up oral skills. Formula comes in several flavors now. Children can experience fatigue of drinking. [6:16] Some kids may require a feeding tube to administer the formula. [7:18] An elemental diet could be used as a first-line treatment for someone with EoE as long as there are no medical contra-indications. It could be used if someone has failed diet therapy or medication therapy and are unable to get into remission with either of those. [7:43] A lot of kids are unable to stay on an elemental diet because of fatigue, social reasons, the difficulty of obtaining formula, or not being covered by insurance. Some tube-fed children with EoE remain on an elemental diet because it works for them and they are in remission. [8:33] Three months is typically the minimum length to be on an elimination or elemental diet to make sure there is enough time for any changes in the eosinophil count to occur. [8:54] The way to start an elemental diet with children depends on their age. With younger children, you might mix the formula half-and-half with their beverage of choice as a transition. Be very upfront with the child, whatever their age. Kids can sense changes quickly. Explain the change, and how it will help the child. [10:18] Laura discusses selective eaters versus adventurous eaters. Some people with EoE already avoid foods selectively that may be triggers to them. For adventurous eaters, you have a lot of options. It can be challenging to work with patients who are selective about textures. For example, oatmeal yogurt has a very different texture from regular yogurt. [11:36] Education is huge. It takes more than the initial appointment to teach a family how to follow an elimination diet. The family needs written information to refer to and resources to go to, including APFED. Patients and their families are encouraged to ask questions. [12:35] Misconceptions about dietary therapy include: 1. Diet therapy will cure the problem and everything will be fine. 2. Changing what I eat won’t work. 3. I can have pizza once a week. 4. I avoid all dairy, but I can still eat butter. [13:23] Education is important. In order for an elimination diet to work, the patient must commit to following the diet. [14:02] There is a learning curve to avoiding foods and finding hidden allergens. There can also be challenges in meeting nutritional needs, depending on how selective the child is with the foods they can eat. Parents may need to monitor what supplementation is needed for children with EoE who are eliminating whole food groups like dairy. [14:51] For families that are undergoing an elimination diet without access to dietician services, Laura recommends getting information from their care team, especially written material the clinic may provide. Go to APFED.org to connect with other families. [15:57] Elimination diets can be used in combination with medication. [17:11] Laura’s daughter was diagnosed with EoE at age five. She is now six. She was diagnosed with a food allergy at age two after contact with tree nuts. Prior to her EoE diagnosis, she experienced frequent vomiting, as well as gagging and choking on food. [18:50] When Laura’s daughter started drinking more water during meals to help her swallow, Laura realized that might have EoE. [19:47] Laura’s daughter is on a milk-only-elimination diet. She is not yet in remission. They are deciding on the next step, either more elimination or moving on to trying medication. [20:19] Laura’s family works together to manage the daughter’s diet. Each family member has a job, which helps. They all take part in shopping and looking for foods to incorporate into meals and pack as snacks and lunches for school and activities. Laura’s husband does the cooking. It takes planning. [22:05] Laura talks about the diet a lot with her daughter. They have a medical/allergy/asthma play kit that they use to play with dolls. They have play food for the dolls and talk about which items have dairy and which don’t. Working through play is important for kids to learn. [23:45] Laura explains how her work with her daughter has influenced her work as a pediatric dietician. She says you don’t have any idea what it’s like to live it until you do. It has given her more empathy for the families she works with because she understands their day a little bit better. [25:19] Holly thanks Laura for sharing her experiences on this podcast. Laura and Holly share resource links for listeners to learn more about EoE. Mentioned in This Episode: APFED on , , , , This episode is brought to you thanks to the support of our Education Partners , , , , and . Tweetables: “For the past 12 years, I’ve been at the University of Minnesota Masonic Children’s Hospital, working with mostly critically ill children, but also children in other settings, and I have learned a lot … as a pediatric dietician, [with] kids … to feed.” — Laura Gearman, MS, RD “A lot of centers are moving toward eliminating just a couple [of foods] at a time. We know it’s a long process to find which items may be triggers [for EoE].” — Laura Gearman, MS, RD “When you’re first learning about [EoE], it’s just so challenging.” — Holly Knotowicz “Working through play is so important for kids to learn.” — Laura Gearman, MS, RD “I think you don’t have any idea what it’s like to live [with EoE] until you do.” — Laura Gearman, MS, RD /episode/index/show/eosinophil/id/25390695

Being Diagnosed With Eosinophilic Esophagitis (EoE) as an Adult and Cooking With a Limited Diet 11/30/2022

Being Diagnosed With Eosinophilic Esophagitis (EoE) as an Adult and Cooking With a Limited Diet Co-hosts Ryan Piansky and Holly Knotowicz, a speech-language pathologist who serves on APFED’s Health Sciences Advisory Council, talk with guest Stephanie Ulrich, NTP. Stephanie Ulrich is a Nutritional Therapy Practitioner, allergy-friendly recipe developer, and patient advocate, living in Brooklyn, NY. Being diagnosed with eosinophilic esophagitis (EoE) and food allergies as an adult came as a complete surprise to Stephanie and left her feeling overwhelmed with learning how to navigate her new diagnosis. However, her love of food inspired her to experiment in the kitchen, developing nourishing allergy-friendly recipes that she shares on her website, The Open Cookbook. In addition, Stephanie is a health coach at ViuHealth, a virtual care solution designed to help people living with autoimmunity improve their everyday lives through a combination of data science and access to a dedicated clinician-led care team. In this episode, Ryan and Holly discuss with Stephanie how she navigates life with allergies, asthma, and EoE. They discuss the impact the conditions have on her life, and her journey to diagnosis and finding her medical care team. This includes a discussion about how she changed careers because of her EoE diagnosis and began a website to share allergy-friendly recipes. Listen in for advice from Stephanie Ulrich for adults newly diagnosed with EoE. Disclaimer: The information provided in this podcast is designed to support, not replace the relationship that exists between listeners and their healthcare providers. Opinions, information, and recommendations shared in this podcast are not a substitute for medical advice. Decisions related to medical care should be made with your healthcare provider. Opinions and views of guests and co-hosts are their own. Key Takeaways: [1:25] Holly introduces today’s topic, Being Diagnosed with Eosinophilic Esophagitis (EoE) as an Adult and Cooking with a Limited Diet, and today’s guest, Stephanie Ulrich, a Nutritional Therapy Practitioner, allergy-friendly recipe developer, and patient advocate who was diagnosed with EoE as an adult. [1:54] Stephanie specializes in supporting people with autoimmune diseases and a wide range of dietary restrictions. She knows what it is like to have a restricted diet. She grew up eating anything, but in her thirties, she was diagnosed with food allergies, EoE, and other autoimmune diseases. The diagnosis that impacts her the most is EoE. [3:40] Stephanie noticed she had bad heartburn and coughing at night when she was trying to sleep for years before she was diagnosed with EoE. A few months before her EoE diagnosis, she started burping all the time. It was embarrassing. Her heartburn was getting worse. She went to a GI doctor who first thought it might be related to IBS. [4:21] The doctor suggested an endoscopy for Stephanie but she put it off. Three months later, when she woke up and took a sip of water, she had the worst pain in her chest that she’d ever experienced. Everything got worse from there. Over the next month, Stephanie basically couldn’t eat. She couldn’t swallow. It was scary. [4:58] When Stephanie had severe chest pain, her doctor sent her to urgent care. After an EKG, the doctor suggested it was GI-related. Stephanie was directed to a gastroenterologist. She booked an endoscopy. That gave her the diagnosis of EoE. Ryan and Holly relate to Stephanie’s symptoms. [5:52] After Stephanie learned her doctor wasn’t experienced in treating EoE, she started on a journey to find a different doctor. This included attending an APFED patient education conference. She feels fortunate that she found a new doctor quickly. [7:38] Stephanie uses an elimination diet and medication to treat her EoE. She does not eat the top nine food allergens, plus legumes, corn, most nightshades, most spices, etc. She is in the process of gradually adding food back into her diet in collaboration with her doctor. She is trying to add eight new foods a year. At her lowest point, she was restricted to five foods. Now she can eat at least 50 foods. [8:21] Stephanie takes a swallowed steroid which has helped her expand her diet, so she is happy about that. She tells how good and bad days contrast for her. Food is emotional for her, and bad days are days when she misses the spontaneity of being able to go out and eat whatever she wants. The hardest days are travel days. [9:56] Stephanie is still figuring out how to manage eating while traveling. She’s experimenting with shorter trips and getting accommodations with a kitchen included. Her first stop on a trip is the grocery store. She brings a suitcase filled with food and snacks. [11:38] EoE has changed Stephanie’s career. She first met Ryan shortly after her diagnosis, when she was working as a landscape architect. The long working hours and significant travel were hard to manage. She spent all weekend prepping meals to eat at work and it was exhausting. [13:01] Stephanie stepped away from her job as a landscape architect. She did freelance work for a few months and then went back to school to become a Nutritional Therapy Practitioner. EoE played a huge role in terms of choosing her career. Now she has a flexible schedule, works from home, and makes food during the day as she needs it. [14:46] The Open Cookbook is where Stephanie shares allergy-friendly recipes and her experiences of living with EoE and other chronic conditions. She has made a lot of connections with people who have EoE and it has been great for her. She meets other people with EoE on her Instagram account. Developing the recipes helped her love food again. [16:33] Stephanie has two recipes that stand out for her. They have personal meaning, as they are similar to recipes from her childhood. The first is Beef Stroganoff, made with coconut cream and coco aminos. Stephanie uses a spiralizer to cut daikon into noodles. [18:42] Stephanie explains her process for creating recipes based on comfort meals people might be missing. She also discusses using a single ingredient in many different ways. She uses butternut squash as an example of a versatile food. [21:05] Stephanie discusses planning the nutritional content of a recipe. She aims for creating meals that balance being nutrient-dense, while also foods that people are happy to eat. She uses gluten-free, grain-free flours, like tigernut flour (from a tuber), and green banana flour. [24:19] Stephanie tries to keep her recipes flexible. She wants them to be accessible and inclusive. She encourages people to adapt recipes on the website to make them their own. Chia egg, flax egg, or gelatin egg are egg substitutes. Carob can be swapped for chocolate or cocoa. You can also filter the recipes by diet types for your needs. [25:46] Some ingredients are more readily available online. [26:17] Stephanie took a little hiatus from developing new recipes but has begun adding new foods to her diet and is starting to experiment with recipes using those ingredients. This year, she also started an online support group for adults who have EoE. [27:05] Stephanie has recently added new foods to her diet, including hemp hearts, flax seeds, and sacha inchi seeds to her diet. Flax seeds and sacha inchi seeds are high in Omega-3. Stephanie has also added chicken to her diet and is enjoying chicken wings. She previously had restricted her meats to turkey, bison, and beef. [28:12] Stephanie has some advice for adults diagnosed with EoE. You don’t have to figure everything out right away. Take your time. It’s OK to feel grief and loss around your diagnosis. Eventually, it will feel like it’s just part of your life and you’ll develop a new normal. Establish your boundaries. Connect with other adults who have EoE. [30:15] Stephanie shares advice for the family and friends of someone who has EoE. Please continue inviting them to events. If they are not comfortable, they will politely decline. If they come, they may just bring their own food. It’s about continuing to have social connections with family and friends, rather than just about food. [31:40] Would Stephanie have done anything differently? She would like to have been more patient with herself and her family and friends. EoE is a complex disease and it takes a while to understand what it is. Be patient with the process. You’ll make mistakes but you will get there. She suggests not limiting yourself in what you can do with EoE. [32:42] Holly thanks Stephanie for sharing her experiences on the podcast to help others. Stephanie thanks APFED for having her on the podcast. [33:01] Stephanie ends by reminding listeners that if you are an adult newly diagnosed with EoE, you are not alone. There are others who are happy to support you. If you need more medical support, keep looking until you find the right medical team for you. It will look different for every single individual. Keep going. You will get there. [33:30] Ryan thanks Stephanie for her advice. Ryan shares resource links for listeners to learn more and connect with Stephanie and with APFED. Mentioned in This Episode: APFED on , , , , This episode is brought to you thanks to the support of our Education Partners , , , , and . Tweetables: “I love working with people who have limited diets to think more creatively about how they can use those limited food choices. I want people to feel encouraged and excited about experimenting in the kitchen and feel excited about food again.” — Stephanie Ulrich, NTP “Growing up, … I could eat anything. … I did have asthma and environmental allergies. … It wasn’t until I was in my thirties that I was diagnosed with a wide range of chronic health conditions, including food allergies, EoE, and … autoimmune diseases.” — Stephanie Ulrich, NTP “I also take a swallowed steroid right now that does work for me and that’s really allowed me to expand my diet, so I feel pretty happy about that.” — Stephanie Ulrich, NTP “I started The Open Cookbook when I was able to start adding a few foods back into my diet. At the time I was in this major rut where I was eating the same soup every single day. [I said], ‘I love food and I can’t keep living like this.’” — Stephanie Ulrich, NTP “The baked goods have a mix of alternative gluten-free, grain-free flours. One of my favorites is a tigernut flour; … it’s a tuber and they just grind it up. It works well as a replacement for almond flour. … Another great one is green banana flour.” — Stephanie Ulrich, NTP “I honestly just love adding vegetables into everything. I’ll add pumpkin into brownies.” — Stephanie Ulrich, NTP “It’s also OK to feel that kind of loss and grief around your diagnosis because, honestly, EoE completely changes your life and it takes some time to get adjusted. So give yourself that time. Give yourself that grace.” — Stephanie Ulrich, NTP /episode/index/show/eosinophil/id/25165122

Access to Specialty Care for Eosinophilic Esophagitis (EoE) 10/26/2022

Access to Specialty Care for Eosinophilic Esophagitis (EoE) Description: Co-hosts Ryan Piansky and Mary Jo Strobel, APFED’s Executive Director, talk with guest Emily McGowan, MD, PhD, about access to specialty care for eosinophilic esophagitis (EoE). Emily McGowan MD, PhD, is an Associate Professor in the Division of Allergy & Clinical Immunology at the University of Virginia School of Medicine. Her primary research and clinical interest is food allergy, and her previous research focused on understanding the underlying causes of the increased prevalence of food allergy in the United States. When Dr. McGowan moved to the University of Virginia School of Medicine in 2016, her clinical and research interests shifted toward understanding the epidemiology and treatment options for eosinophilic gastrointestinal diseases (EGIDs). Dr. McGowan is certified by the American Board of Internal Medicine and the American Board of Allergy and Immunology and is an active member of the American Academy of Allergy, Asthma, and Immunology. In this episode, Ryan and Mary Jo discuss with Dr. McGowan her research and what brought her to study EoE. They discuss papers she wrote on the prevalence of diagnosis in urban and rural areas. They also explore how the question came up of whether EoE is less prevalent in rural and impoverished areas. A comparison of data on the location of gastroenterologists across the United States showed that rural and impoverished areas have the same prevalence of diagnosis among patients who see a gastroenterologist as patients from more urban areas. Listen in for information about Dr. McGowan’s research on access to care. Disclaimer: The information provided in this podcast is designed to support, not replace the relationship that exists between listeners and their healthcare providers. Opinions, information, and recommendations shared in this podcast are not a substitute for medical advice. Decisions related to medical care should be made with your healthcare provider. Opinions and views of guests and co-hosts are their own. Key Takeaways: [1:15] Mary Jo introduces today’s topic, Access to Care, and today’s guest, Dr. Emily McGowan, and shares Dr. McGowan’s research interests in food allergies and eosinophilic gastrointestinal diseases. [2:01] Dr. McGowan came to the field of EoE from the world of food allergies. Having a peanut allergy herself, Dr. McGowan has always wanted to improve the lives of patients with food allergies. [2:37] Dr. McGowan did her fellowship at Johns Hopkins researching pediatric food allergy and racial and ethnic disparities for food allergy. When Dr. McGowan joined the Johns Hopkins faculty in 2014, she began treating EoE patients. [3:12] Dr. McGowan’s found that the questions she asked about pediatric food allergy also applied to EoE; why are many more being diagnosed and why is there racial and ethnic disparity? [3:36] Dr. McGowan’s shift of focus was completed when she moved to the University of Virginia. She took over as the attending allergist in the pediatric EGID clinic. At the same time, they started an adult clinic. That allowed her to see EoE and EGID patients from early childhood through adulthood. [4:17] Dr. McGowan and co-authors from different institutions and specialties recently published a paper on diagnostic disparities in pediatric EoE in rural communities where there is a substantial distance to pediatric gastroenterology providers. It started with looking at over 18 million children enrolled in Medicaid in 2012. [4:56] The doctors were looking to estimate the prevalence of EoE and they were interested in how poverty and urban status affected the prevalence. The first thing they found was a striking inverse relationship with poverty. The more impoverished the neighborhood, the less likely the child was to be diagnosed with EoE. [5:20] The second thing they found was that EoE was less prevalent in rural areas in the United States. This finding caught the attention of two collaborators, Dr. Elizabeth Jensen and Dr. Amanda Muir, who reached out and asked if the decreased prevalence in rural areas was due to decreased access to pediatric gastroenterologists. The researchers combined data and collaborated on the newly published manuscript. [6:10] The doctors looked at different models and spliced the data in different ways and found an association between lower prevalence in rural areas and how far away the child lived from a physician who could diagnose the disease. [6:45] Dr. McGowan was interested that the children who lived in more impoverished areas were less likely to be diagnosed with EoE. For every 5% increase in income in that area, there was an increase in the prevalence of EoE. This brought up the question “are there other barriers to care that aren’t related to distance to provider, like co-pay?” [8:45] For research to advance, trust must be built within communities and researchers should meet patients where they are, which takes time and effort. Dr. McGowan notes that the NIH and other health institutions are making health disparities a research priority because everyone deserves good health and there’s a lot more work that needs to be done in this area. [9:33] Dr. McGowan has patients that travel a distance from all over Virginia and sometimes other states to see her for EGID care. Dr. McGowan tries to work with their local allergist or gastroenterologist to help ease the burden of their traveling for care. [10:51] Dr. McGowan empowers patients to advocate for themselves and their families. They should feel they can research the condition, the treatment options, and the providers. If you’re living in a community where the provider isn’t familiar with your condition, education is essential to ensure that you get adequate care. [11:43] Besides distance to the provider, the financial aspect often gets overlooked. In the Medicaid dataset, patients who lived with a higher level of poverty were less likely to be diagnosed with EoE. [12:18] There is an assumption that EoE primarily affects white males. But it may be that previous studies that have been used to define the risk factors for EoE have been biased by the people who were coming in for specialty care. It is important to hone in on that to see if this is truly a disease in which white males are more affected. [13:01] A lot of Dr. McGowan’s patients don’t have a local gastroenterology provider. Those patients often present later in the course of the disease. They tend to have fibrotic findings that may not be found in patients who are diagnosed early. The risk of not catching the disease early is that the inflammation progresses to fibrosis. [13:50] The first step to reducing disparities is to get a better sense of where they exist and why. This study shows that disparities may exist. Now we need to prove that by identifying factors we can change. When we move forward we can adjust specific things that have an impact. There are new tests that screen for EoE without an endoscopy. [14:37] We can increase awareness of EoE among providers and patients in rural areas so that when patients come in with compelling symptoms the provider thinks of EoE as one of the diagnostics. We can increase access to specialists in rural areas by having specialists commute or by using telemedicine. [15:52] There have been a lot of benefits resulting from the expansion of telemedicine during the pandemic. Some of Dr. McGowan’s patients who live far away use it instead of taking a day off to see her. It opens up opportunities to partner with rural areas to deliver specialty care at far distances. [16:23] There are some barriers to telemedicine. A lot of areas don’t have high-speed internet or smartphones. A physician needs to be licensed in the state where the care is being received. For EoE there is the issue that it is a tissue diagnosis so the patient will still need to be seen to get the biopsy or the noninvasive tests. [17:07] Dr. McGowan is currently involved in a few exciting projects, one of which is directly related to this topic. Based on this work and her clinical experience, she hypothesizes that EoE is probably underdiagnosed, even among patients who are seeing an allergist and have access to specialty care. [17:28] Dr. McGowan and her team did a pilot study of 100 patients of the allergy clinic at UVA, asking them if they had any symptoms of EoE. They found that 44% of those patients had difficulty swallowing, and 10% had intermittent food impaction over the past year for more than 30 minutes. Testing found more undiagnosed EoE than expected. [18:11] Dr. McGowan is working on getting more funding to extend the project for a larger screening study of EoE. She says people minimize their symptoms by eating slower and drinking more water when they eat without knowing that’s atypical. We don’t talk about what normal feeding behaviors are. The pilot study will be in print soon. [22:40] Ryan thanks Dr. McGowan for taking the time to be on the podcast. Dr. McGowan thanks Ryan and Mary Jo for having her talk about this important topic. Ryan shares resource links for listeners to learn more and connect. Mentioned in This Episode: APFED on , , , , This episode is brought to you thanks to the support of our Education Partners , , , , , and . Tweetables: “For every 5% increase there was in income in that [impoverished] area, there was an increase in the prevalence of EoE.” — Dr. Emily McGowan “This study was a good first step to show that these disparities may exist. Now we need to prove that and I think we need to dig deeper to identify the factors we can change.” — Dr. Emily McGowan “[Telemedicine] is a great first step and I’m really excited to see how it evolves within our specialty.” — Dr. Emily McGowan “Because this is such a slowly progressing disease, people eat slower or drink more water when they're eating and they don’t even realize that what they’re doing is necessarily atypical.” — Dr. Emily McGowan /episode/index/show/eosinophil/id/24800190

Longitudinal Research Studies of Eosinophilic Disorders 09/28/2022

Longitudinal Research Studies of Eosinophilic Disorders Description: Co-hosts Ryan Piansky and Holly Knotowicz, MS, CCC-SLP, talk with guest Kara Kliewer, RD, PhD, about longitudinal research studies. Dr. Kliewer is a clinical research manager at Cincinnati Children’s Hospital Medical Center in the Division of Allergy and Immunology. She has a doctorate in nutrition and has teamed up with other researchers to manage multi-site clinical trials in eosinophilic disorders, oversee coordinators managing industry-sponsored clinical trials, and collaborate and assist with research projects at the Cincinnati Center for Eosinophilic Disorders. She is also a member of the Consortium of Eosinophilic Gastrointestinal Disease Researchers, also known as CEGIR. In this episode, Holly and Ryan discuss with Dr. Kliewer her background and experience with research studies for eosinophilic disorders. Dr. Kliewer explains what a longitudinal study is, how long it can last, what kind of data it can gather, and the benefits of this type of research. She shares how easy it is to enroll in a longitudinal study and the time commitment that is often expected from participants. Dr. Kliewer also notes the types of interventional studies she works with and what kinds of commitments are expected of participating patients. She also shares how patients can enroll in longitudinal or cross-sectional studies. Finally, Dr. Kliewer discusses her current work and upcoming research. Listen in to learn the valuable findings longitudinal studies can produce. Disclaimer: The information provided in this podcast is designed to support, not replace the relationship that exists between listeners and their healthcare providers. Opinions, information, and recommendations shared in this podcast are not a substitute for medical advice. Decisions related to medical care should be made with your healthcare provider. Opinions and views of guests and co-hosts are their own. Key Takeaways: [1:25] Holly shares the topic of this episode, longitudinal research studies, and introduces today’s guest, Kara Kliewer, RD, PhD. [1:31] Dr. Kliewer is a clinical research manager at Cincinnati Children’s Hospital Medical Center in the Division of Allergy and Immunology. She has a Ph.D. in nutrition and is a registered dietitian. She is also a member of the Consortium of Eosinophilic Gastrointestinal Disease Researchers, also known as CEGIR. [2:02] Holly welcomes Dr. Kliewer to the podcast. [2:17] Dr. Kliewer’s background is in nutrition science. About eight years ago, Dr. Kliewer began managing and coordinating clinical trials for eosinophilic disorders. [2:27] At first, Dr. Kliewer’s studies were mostly about nutrition. They have expanded into other clinical trials, testing drug interventions, and doing cross-sectional studies, and longitudinal studies, all with patients with eosinophilic gastrointestinal diseases. [2:45] Dr. Kliewer assists with the design of investigator-sponsored studies, she manages the coordinators that are enrolling patients in studies, and she works with the statisticians at the end of the study to help with the analysis. She is engaged in all sides of the study from the beginning to the end. [3:13] A longitudinal study takes repeated measures of individual patients, over time. Almost always, those studies are observational. A well-known longitudinal prospective study is the Nurses’ Health Study. It started in Harvard in the 1970s and is ongoing. It studies the lifestyle habits of women and the diseases they develop over time. [4:00] Dr. Kliewer’s group started doing prospective longitudinal studies in eosinophilic disorders, looking at how chosen variables might change over time in relation to other variables. [4:18] Rarely, there can be some interventions associated with a longitudinal study, but it’s quite uncommon for there to be anything other than observation of the people in the study. [4:34] Longitudinal studies can last for decades. In general, they last one to two years or five to ten years. A short-term study can be for a couple of weeks. [4:51] Dr. Kliewer’s studies may last five to ten years. Because they are conducted at a children’s hospital, some patients “age out.” In some cases, the study team may follow up with those patients. If the study is coordinated with another organization, the patients can be followed when they transition to adult care. [5:43] The observational longitudinal studies, in general, are pretty open for participants who meet the age requirement and have the disease being studied, unlike interventional studies where the inclusion/exclusion criteria are rigorous, due to questions of drug safety. [6:30] Dr. Kliewer says that from these longitudinal studies we learn about relationships between various factors and the disease, its development, or progression. Usually, the studies are focused on particular variables and how they may change with time, how the disease progresses, and what factors might influence that. [6:59] You might observe how symptoms change over time and also how they change with eosinophilic counts. The studies generate hypotheses and give relationships to research further, focus on, and help to develop clinical interventions or treatments for the disease, and just generally, know more about the disease. [7:48] Dr. Kliewer tells how the data changing over time in a longitudinal study is helpful. Over time, you can observe more patterns. The longer you follow, the more you can learn about the disease’s progress. In a cross-sectional study, you are looking at a set of patients that is limited in terms of understanding all patients. [8:32] Patients in a longitudinal study enjoy participating. The time commitment is minimal because the study piggybacks off of the yearly or twice-yearly clinical visits, taking 15 to 30 minutes of their time. It’s a good way to be involved in research. [9:56] Administrative challenges include patients losing enthusiasm over the years and not being as interested to talk to the researchers when they are older. It is expensive to continue studies for years. Some patients age out of pediatric care and it’s a challenge to keep in contact with them by phone or catch up with them at another site. [10:49] For patients enrolling in the study, when they learn the time commitment is minimal, they are happy to participate in a longitudinal study. In general patients have a good grasp of what a longitudinal study entails. [11:40] Dr. Kliewer explains the commitments of an interventional study. Patients come in monthly in addition to their clinical care visits, and almost daily fill out a questionnaire. Developing a good relationship with the patients works well. The coordinators get to know the families. Everybody’s working toward a goal. [12:53] Dr. Kliewer tells how data from multiple studies can be pooled or compared. [13:36] Dr. Kliewer is involved in various clinical trials. Some are interventional, industry-sponsored, testing therapies. Some are observational. They participate in longitudinal studies as part of a consortium. Some cross-sectional, studies obtain biological specimens to try to understand the mechanisms that underlie the disorders. [14:53] Dr. Kliewer and her team learned about running a longitudinal study during COVID-19. When there were no clinic visits it was a challenge for the studies. The studies are up and running again. [15:40] CEGIR researchers have published some of the results of Dr. Kliewer’s longitudinal studies. Most publications have been on cross-sectional studies. [16:01] Dr. Kliewer’s publications include looking at the molecular changes of eosinophilic gastritis and a cross-sectional view of symptoms in children and parents’ symptoms compared to their children, which they found correlated. Now that they have reached the five-year mark, some of the longitudinal data is being analyzed. [17:02] Other longitudinal observational studies are listed on clinicaltrials.gov, including trials that are recruiting patients. [17:52] Patients hoping to get involved with an interventional study can ask their gastroenterologist or allergist for information on studies. Your provider knows what would be a good fit for you. For any study, carefully read the consent form, and be sure to ask the coordinator all the questions that you have, before enrolling in a study. [18:51] Consent forms vary depending on if the study is interventional and testing a pharmaceutical product, or observational. Consent forms include the benefits and risks, the time commitment, and the procedures patients will be asked to do. Know what your commitments are before enrolling. [19:37] Dr. Kliewer has a number of additional interventional studies in the pipeline, including research on drugs that could potentially affect eosinophilic esophagitis. Other upcoming research is focused on eosinophilic gastritis, enteritis, and colitis. [20:57] Dr. Kliewer is grateful that patients become engaged in this research. It’s one of the few ways we have to find out how the disease progresses and potentially develop better treatments. She hopes people have learned that the time commitment is not a lot but the benefit to research will be a lot. [22:15] Ryan invites listeners to look at apfed.org/research and clinicaltrials.gov to learn more about eosinophilic research. Ryan also encourages you to join the APFED online community at apfed.org/connections. Ryan thanks Dr. Kliewer for joining us today. [22:56] Dr. Kliewer thanks Ryan and Holly for having her on the podcast. Mentioned in This Episode: APFED on , , , , This episode is brought to you thanks to the support of our Education Partners , , , , , and . Tweetables: “A longitudinal study is one that involves taking repeated measures of … the same individuals over time. … Those studies are observational. … A well-known longitudinal prospective study is the Nurses’ Health Study and it started in Harvard in the ’70s.” — Dr. Kara Kliewer “Some [longitudinal studies] can last decades; others can be very short. In general, they’re at least a year long.” — Dr. Kara Kliewer “Some of the advice that we constantly give is always talk to your gastroenterologist or allergist, especially if you’re interested in an interventional study because your provider probably knows … what would be a good fit for [you].” — Dr. Kara Kliewer “Carefully read the [clinical trial] consent form, know what you’re getting yourself into, and be sure to ask the coordinator … all the questions that you have before beginning the study.” — Dr. Kara Kliewer /episode/index/show/eosinophil/id/24502497

Genetics and Eosinophilic Colitis (EOC) 09/09/2022

Genetics and Eosinophilic Colitis (EOC) Description: Co-hosts Ryan Piansky and Holly Knotowicz talk with guest Nicole Arva, MD, PhD, about genetics and eosinophilic colitis. Dr. Arva is a pediatric pathologist at Ann & Robert H. Lurie Children’s Hospital of Chicago. She is also an Associate Professor in the Department of Pathology at Northwestern University Feinberg School of Medicine. She has been a part of more than 50 publications, many of which explore eosinophilic diseases, and recently co-authored a manuscript entitled “Genetics of Eosinophilic Colitis Revealed.” In this episode, Holly and Ryan discuss with Dr. Arva her recent and upcoming research. They cover the purposes of molecular testing and a new molecular study that concludes that EoC is a unique disease, with specific genetic characteristics that set it apart from other EGIDs, Crohn’s disease, and IBD. Dr. Arva explains how that uniqueness was discovered, and what it means for therapeutic options for EoC. Listen in to learn about this innovative research. Disclaimer: The information provided in this podcast is designed to support, not replace the relationship that exists between listeners and their healthcare providers. Opinions, information, and recommendations shared in this podcast are not a substitute for medical advice. Decisions related to medical care should be made with your healthcare provider. Opinions and views of guests and co-hosts are their own. Key Takeaways: [1:44] Holly shares the topic of this episode, genetics and eosinophilic colitis (EoC), and introduces today’s guest, Dr. Nicole Arva. [1:51] Dr. Nicole Arva is a Pediatric Pathologist at Ann & Robert H. Lurie Children’s Hospital of Chicago and an Associate Professor in the Department of Pathology at Northwestern University Feinberg School of Medicine. [2:37] Dr. Arva wanted to understand the mechanisms of diseases to be able to treat them more efficiently. She tells of her professional journey after medical school, studying cancer and leukemia cell lines. She continued her medical training in pathology, specializing in pediatric pathology. [3:15] Dr. Arva’s work involves assessing laboratory slides from pediatric tissue samples to establish a diagnosis. [4:05] Dr. Arva explains molecular testing. An increasing number of diseases are found to have genetic abnormalities. Molecular testing can help lead to an accurate diagnosis. Dr. Arva describes getting a tumor diagnosis from molecular testing showing specific chromosomal translocations in correlation with microscopic examination. [5:23] By performing molecular testing, we can discover gene alterations that can help guide the development of new therapies. Molecular testing can uncover genes that can be targeted. [5:51] Genetic analysis can also provide insight into how a disease develops and shed light on the pathways that are involved. [6:35] Dr. Arva describes EoC. EoC affects the large bowel. Eosinophils cause inflammation in the colon. Patients usually have abdominal pain, diarrhea, which can be bloody, and fluid in the abdominal cavity. Patients can become malnourished. They may develop a bowel obstruction or perforation. [7:25] Studies have been performed on Eosinophilic Gastrointestinal Disorders (EGIDs) and Inflammatory Bowel Disease (IBD). But EoC is a poorly understood condition. When Dr. Arva and her colleagues started their study, they didn’t know whether EoC should be considered to be within the spectrum of EGIDs or as an IBD. [8:03] The research team was looking to determine where EoC belongs because that would affect the way patients would be treated. Although EoC is similar to other EGIDs because eosinophils drive the inflammation,, EoC has a lower incidence than EGIDs, more severe symptoms, and co-morbidities. [8:38] EoC is similar to IBDs in that it is an inflammation of the large bowel, but a different type of cell is predominantly involved in IBD (neutrophil) with some eosinophils present. [9:38] The diagnosis of EoC is challenging. Other, more common conditions can cause colonic eosinophilic inflammation. When pathologists encounter eosinophilia in the large bowel, they have to think of other medical conditions that can cause that. A diagnosis of EoC is established only after other causes of gastrointestinal eosinophilia have been ruled out. [10:14] IBD, intestinal parasites, autoimmune or connective tissue disorders, and vasculitis can all mimic EoC. Certain medicines can induce eosinophilia. Eosinophils are normally found in the large bowel. [10:51] Pathologists have to establish eosinophil count values for each segment of the large bowel to best evaluate colonic biopsy; everybody needs to follow the standards when diagnosing EoC. [11:42] Patients suspected to have EoC may undergo lab testing, imaging, and colonoscopy to reach a diagnosis. The findings may vary, depending on which section of the bowel wall is infiltrated by eosinophils. [13:02] A biopsy may reveal an increased number of eosinophils. All these test results have to be combined to reach a diagnosis of EoC. [13:27] The focus of the study was performing molecular testing that was very helpful in diagnosing EoC. They found that nearly 1,000 genes were differentially expressed in EoC compared to normal participants or Crohn’s disease subjects. [14:30] They found differences in gene expression between EoC and other types of EGIDs, such as eosinophilic esophagitis or gastritis. [15:02] The main chemotactic factor in EoC seems to be CCL11 (Eotaxin-1). CCL11 is a molecule that attracts eosinophils in the tissue. In eosinophilic esophagitis or gastritis, the main chemotactic factor appears to be CCL26 (Eotaxin-3). All these findings support the idea that EoC is a distinct entity, which is different from other EGIDs and IBD. [15:37] EoC seems to be driven by a mechanism that does not involve an allergic inflammation. The therapeutic strategy may be much different now that we have a better understanding of EoC. [16:23] Eosinophilic esophagitis has been shown to run in families and it would be beneficial to test family members with a blood test, allergy testing, or endoscopy if they develop symptoms. As eosinophilic colitis has a different epigenetic mechanism, it is not clear that the same testing is needed for EoC patients or family members. [17:26] Besides endoscopies and colonoscopies, blood tests looking for high IgE levels, allergy testing, and CT can be useful in testing for various eosinophilic disorders. [21:07] Dr. Arva explains how many eosinophils are seen in a high-power field of a slide from a colon biopsy to diagnose EoC. They established a normal count of eosinophils for the segment of the colon and the abnormal count would be twice the normal count. [22:20] Now that a different pathogenic mechanism has been discovered for EoC than for EGIDs and IBD, new treatments can be explored or developed. We are just beginning the research. CLC protein (galectin-10) is upregulated in EoC and may be a target for treatment development. Antibodies may be effective for relieving EoC inflammation. [23:34] It is challenging to treat EoC because the newly-discovered pathogenic mechanism shows that EoC is unlikely to be allergic in nature, making elimination diets and steroid treatments ineffective. New therapies will be required. [24:05] All EGIDs can have a significant impact on quality of life. People with EoC can develop serious complications, such as dehydration, malnutrition, intestinal strictures, and bowel obstruction. [25:04] Dr. Arva considers the difficulties pathologists face in analyzing eosinophilic diseases. There are few patients with EoC. Dr. Arva describes additional challenges. [26:25] Dr. Arva looks ahead. Most of her research goes in the direction of pediatric gastroenterology. She is working with a clinical colleague, Dr. Josh Weschler, to analyze the role of mast cells in EGIDs. They are finding that eosinophils are not the only offenders in EGIDs and are looking to establish a cohort of EGID patients to study. [27:43] Holly thanks Dr. Arva for taking the time to talk with us today. [27:51] Dr. Arva says we are just at the beginning of understanding the etiology of EoC. This study had a small sample size and the results will have to be validated with larger cohorts of patients. The analyses were performed on whole biopsies that contained all types of cells. Future studies using single-cell preparations will be important. [28:55] Ryan invites listeners to look at apfed.org/eoc to learn more about EoC. Ryan also encourages you to connect with the APFED online community at apfed.org/connections. [29:25] Dr. Arva thanks Ryan and Holly for having her on the podcast. She is grateful for the opportunity to research EGIDs and benefit patients suffering from these conditions. Mentioned in This Episode: APFED on , , , , This episode is brought to you thanks to the support of our Education Partners , , , , , and . Tweetables: “I have always wanted to better understand the mechanisms of diseases because I considered that if we know how an illness occurs, we can treat that entity more efficiently.” — Dr. Nicole Arva “As testing increases, more and more diseases are found to have recurrent genetic abnormalities. … Molecular testing can help the pathologist to render the correct diagnosis.” — Dr. Nicole Arva “There are similarities and dissimilarities between EoC and other EGIDs and IBD.” — Dr. Nicole Arva “Findings support the idea that EoC … seems to be driven by a mechanism that does not involve an allergic inflammation.” — Dr. Nicole Arva /episode/index/show/eosinophil/id/24316359

Aeroallergens and EoE with Dr. Amal Assa’ad 05/31/2022

Aeroallergens and EoE with Dr. Amal Assa’ad Host Ryan Piansky and co-host Mary Jo Strobel, talk with guest Dr. Amal Assa’ad about aeroallergens, food allergies, and eosinophilic esophagitis. Dr. Assa’ad is a pediatric allergist. She is a Professor at the University of Cincinnati in the Department of Pediatrics, as well as the Director of Clinical Services and Associate Director of the Division of Allergy and Immunology at Cincinnati Children's Hospital Medical Center. She is an accomplished, award-winning researcher who has published more than 100 peer-reviewed manuscripts and book chapters. In this episode, Mary Jo and Ryan invite Dr. Assa’ad to share her academic path and the research she has done on eosinophilic esophagitis (EoE) as well as current research directions. Dr. Assa’ad discusses how environmental allergens and EoE connected and why treatments for allergies are ineffective at preventing EoE. She also discusses a study on the demographic differences in the diagnosis of EoE and shares her optimism for biological treatments in the pipeline for eosinophilic disorders. Disclaimer: The information provided in this podcast is designed to support, not replace the relationship that exists between listeners and their healthcare providers. Opinions, information, and recommendations shared in this podcast are not a substitute for medical advice. Decisions related to medical care should be made with your healthcare provider. Opinions and views of guests and co-hosts are their own. This podcast was recorded prior to the FDA approval of DupixentⓇ for indicated treatment of eosinophilic esophagitis for people aged 12 and older. Key Takeaways: [1:11] Mary Jo introduces today’s guest, Dr. Amal Assa’ad, Professor at the University of Cincinnati’s Department of Pediatrics, Director of Clinical Services, and Associate Director of the Division of Allergy and Immunology at Cincinnati Children’s Hospital Medical Center. [1:45] Dr. Assa’ad is an accomplished award-winning researcher. Dr. Assa’ad has published over 100 peer-reviewed manuscripts, book chapters, and research publications. She holds several committee appointments and is an engaging speaker with a gift for teaching difficult concepts in an easy-to-understand way. [2:29] Dr. Assa’ad is not college-educated! In Egypt, she went directly from high school to medical school. She is the first medical doctor in her family. She wanted to relieve patient suffering and study medical disorders. She started medical school at age 16 and stayed for seven years. At the age of 23, she was a physician. [3:40] After receiving her MB BCh medical degree, Dr. Assa’ad entered a Master’s program for a degree in testing for food allergies. In the United States, Dr. Assa’ad did a visiting fellowship in San Antonio, Texas, and an internship and residency in pediatrics in Michigan and Dayton, Ohio. [4:38] From there, she joined Cincinnati Children’s Hospital where she started her work in food allergy and other allergic disorders. She developed an interest in eosinophilic disorders. Dr. Assa’ad was always thrilled to be making science. With other doctors, she worked on several of the initial clinical trials for hypereosinophilic syndrome patients. [5:51] From these trials, Dr. Assa’ad learned how patients present differently; at different ages, different organ systems affected, and with different quality of life. [6:07] Dr. Assa’ad started working with eosinophilic esophagitis and eosinophilic gastroenteritis and contributed to the descriptions of these disorders and the study of the pathogenesis, presentations, and epidemiology. She continues to be very interested in the overlap of these disorders with food allergy and environmental allergy. [6:55] Is EoE always triggered by food? Patients usually say if they take certain foods, they have symptoms that are associated with EoE. The symptoms of EoE are also very difficult to pin down. With infants, the symptom is feeding difficulties. In toddlers, it is vomiting. At school age, stomach aches. In adults, dysphagia, or difficulty swallowing. [8:52] Quite often, EoE is not diagnosed until a major event happens, like a food impaction, like something getting stuck in the esophagus at dinner. Is it that the food instantly made the EoE worse, or is it the texture issue and the difficulty in the motility of the esophagus that cause the impaction? [10:09] Are foods the only thing that can increase your EoE symptoms? What about environmental allergens that can contribute? Dr. Assa’ad cites a study in mice where allergens were put into the esophagus or the nose. The observation was that inhaled allergens may cause symptoms to worsen. [11:00] Patients have been observed with symptoms that cycle with the seasons. Certain seasons were associated with either diagnosing EoE or flare-ups of EoE. There are common proteins between pollen and foods, so the question is: Are these kinds of proteins, whether in foods or inhalants, causing a flare-up of symptoms? [11:57] When you have a flare-up of symptoms, you assume that you have a worsening of the pathology in the esophagus, so you have more eosinophils. Of course, you don’t get an endoscopy every season, but you could assume the esophagus isn’t working as it should. [12:25] Dr. Assa’ad thinks that EoE is multifactorial. Different patients have different triggers. In the 1990s, when EoE was being described, researchers did skin tests for environmental and indoor allergens. In Dr. Assa’ad’s published research, some EoE patients had no or few allergies, and others had many reactions to allergens. [15:38] Dr. Assa’ad believes that patients with large numbers of allergies are patients who are likely to develop eosinophilic esophagitis because they tend to be allergic. She believes that the lining of the esophagus is primed and prone to respond to these food and environmental allergens. [16:14] How can you determine which foods are problematic for your EoE? Dr. Assa’ad describes the difference between sensitivity and allergy. Many studies were looking for connections between food sensitivities and EoE. Researchers used to try drastic dietary elimination, but now they limit the eliminations to a few foods. [18:29] In a new study, not yet published, removing a few foods can significantly reduce the EoE symptoms. Some patients know that if they eat a certain food they are going to have symptoms, so they don’t eat it. But don’t restrict your diet to extremes. Maintain a good quality of life. [20:50] What about seasonal EoE flare-ups? Is that only related to airborne allergens? There are no definitive population studies on this question. It is also very hard to filter out airborne allergens. But what other changes in the environment may cause EoE flare-ups during these cycles? They may be pollution, toxins, dust, and particulates. [23:15] Dr. Assa’ad was surprised in her research by the number of positive tests she found in the larger part of the patients, who were highly sensitized to a large number of things. These patients were being treated for their symptoms and still got diagnosed with eosinophilic disorder. They were not being treated for the immunologic mechanism. [24:16] Dr. Assa’ad was excited about a study where they looked at mepolizumab in eosinophilic disorders. The study showed it did decrease the eosinophils in the biopsies but it didn’t meet the primary goal endpoint of ameliorating symptoms, which is something that has plagued a lot of the eosinophilic disorders studies. [25:39] These study patients came having already had treatments for allergies but they still developed the disorder. Mepolizumab went on to be developed for eosinophilic asthma and eventually for hypereosinophilic syndrome as well, but not for eosinophilic esophagitis or gastroenteritis. Dr. Assa’ad is hopeful it will be developed for EoE. [26:41] Dr. Assa’ad recommends patients with EoE symptoms that are caused by environmental allergens to treat the allergic symptoms. It’s important for patients to manage their quality of life. [28:28] It has been noted that epidemiologically, eosinophilic esophagitis and the disorders are more common on the east and west coasts but less common in the middle of the country. Is it the stresses and rapid pace of life on the coasts? [29:38] Dr. Assa’ad has seen improvement with puffed and swallowed fluticasone. They have published papers on that. [30:25] The availability of endoscopies and biopsies in the United States is far greater than in other countries, even European countries. The diagnosis of EoE in some countries relies on counting eosinophils in blood samples instead of biopsies. Flare-ups and eosinophil counts do not necessarily correlate. Other countries go by symptoms. [32:21] Environmental allergens are very different in different countries so the influence of environmental allergens on EoE from country to country cannot be easily compared. [33:13] What research is next for Dr. Assa’ad? Food allergies and working with colleagues at other institutions on a grant following a cohort of situationally diverse African-Americans and Caucasians with food allergies. They are looking into the diagnosis of eosinophilic esophagitis and how being diagnosed has differed by race. [34:26] Through this cohort, they have found that EoE is infrequently diagnosed in African-American or Latinx populations that have symptoms that are very consistent with EoE. [34:45] Dr. Assa’ad is also working on looking at the genetics of food allergies with regard to races and what makes a difference. She is also working on establishing a biobank for food allergy biomarkers for researchers around the country. [36:04] Another project Dr. Assa’ad is working on is immunotherapy for food allergies. Patients with EoE have previously been excluded from studies with immunotherapy because it might provoke the disorder. [37:30] Dr. Assa’ad focuses on where the patient is coming from and the patient’s wants and needs. She is working on clinical trials with biologics and is optimistic about the results with acute food allergies and eosinophilic disorders. [38:07] Mary Jo thanks Dr. Assa’ad for taking the time to talk with us. [38:16] Dr. Assa’ad shares the optimism that the whole scientific community is making strides. A lot is coming down the pike and things are going to get better from the diagnostic and therapeutic points of view and improving the patients’ quality of life and possibly helping other countries. [39:19] Ryan invites listeners to look at apfed.org for additional resources to help with the day-to-day management of EoE. Ryan also encourages you to connect with the APFED online community and listen to past podcast episodes. [39:59] Dr. Assa’ad thanks Ryan and Mary Jo for having her on the podcast and thanks APFED and other patient organizations. Mentioned in This Episode: APFED on , , , , This episode is brought to you thanks to the support of our Education Partners , , , , , and . Tweetables: “I was always very thrilled to be in a room where we are making science. We are making observations on these eosinophilic disorders and specifically, eosinophilic esophagitis and hypereosinophilic syndrome.” — Dr. Amal Assa’ad “I would [consider], for each particular person: What is it that makes their symptoms flare up?” — Dr. Amal Assa’ad “There were first case reports, and then there were retrospective population studies that found that certain seasons were more associated with either diagnosing EoE or flare-ups of EoE.” — Dr. Amal Assa’ad “What the patient wants and what the patient needs should be our focus.” — Dr. Amal Assa’ad /episode/index/show/eosinophil/id/23267885

Growing Up with EGIDs with Kayla Abramowitz 04/26/2022

Growing Up with EGIDs with Kayla Abramowitz Growing up with an eosinophilic gastrointestinal disorder (EGID) can be a challenge. In this episode, host Ryan Piansky, and guest host Mary Jo Strobel, talk to 19-year-old Kayla Abramowitz to discuss her journey living with eosinophilic colitis. Kayla Abramowitz started having symptoms of an EGID at the age of six but was not diagnosed until many years later. Today, she is a sophomore in college majoring in Public Advocacy at the University of Miami. She is also the Founder, Chief Kid Officer, and Vice Chairperson of the non-profit “Kayla Cares 4 Kids.” Kayla founded the organization at age 11. Motivated by her own experience with hospital stays due to chronic illness, she collected entertainment and educational items to donate to children’s hospitals. Kayla has received recognition and awards for her work from various organizations. In this episode, Kayla shares her journey as a child growing up with multiple medical conditions, including eosinophilic colitis, and how she navigates the challenges of living with chronic diseases. She explains her diagnostic journey, how important community support was and continues to be for her, and what inspired her charity work. Tune in to listen to Kayla’s inspiring story and learn more about her motivation to make life better for other children with chronic disease. Disclaimer: The information provided in this podcast is designed to support, not replace the relationship that exists between listeners and their healthcare providers. Opinions, information, and recommendations shared in this podcast are not a substitute for medical advice. Decisions related to medical care should be made with your healthcare provider. Opinions and views of guests and co-hosts are their own. Key Takeaways: [1:10] Mary Jo introduces the guest for this episode, Kayla Abramowitz. [2:06] Kayla shares her background about her journey with an EGID. [3:28] How did Kayla juggle school responsibilities alongside going to doctors’ appointments and trying to get a diagnosis for her condition? [4:33] What are some common misconceptions that people might have about eosinophilic colitis? [5:38] Kayla has a sibling with an eosinophilic disorder as well. What has that been like? [6:56] Kayla’s brother’s diagnosis helped guide her toward exploring eosinophilic disease as a possibility to explain her condition. [7:30] Kayla shares how she and her brother were able to help each other in their journey living with eosinophilic disorders. [10:10] Kayla explains what some of the day-to-day management of her condition looks like and how she’s trying to manage it in different ways. [12:10] How did Kayla initially get connected with the APFED community? [14:37] How is Kayla’s chronic disorder impacting her time at college and her ability to participate in social events? [16:43] How much did Kayla have to prepare to go away to college? [18:09] Kayla shares more about her college experience and how studying public advocacy is helping her speak up about topics that are important to her. [18:31] How does Kayla explain her condition to new friends at college? [20:40] Kayla’s experiences with her chronic health condition inspired her to establish Kayla Cares for Kids. She explains more about this nonprofit that she started. [23:37] What are some of the donations that Kayla's nonprofit receives and distributes? [26:05] What are some preparations that Kayla makes to travel? [26:56] Kayla talks about some of the recognition she has received for the work that she does. [28:13] Find out more about Kayla and the work she does via her website or reach out to her via email. [29:36] Kayla shares some advice for kids growing up with chronic illness. Mentioned in This Episode: APFED on , , , , This episode is brought to you thanks to the support of our Education Partners , , , and . Tweetables: “It’s really hard to explain that I’m not feeling well even though I look fine.” — Kayla “It made me realize how lonely he was feeling and how much support you can get by going to a conference like that.” — Kayla “Community is so important.” — Mary Jo “It’s do or don’t with this medical issue and I have to really evaluate everything on a daily basis.” — Kayla /episode/index/show/eosinophil/id/22825952

Co-morbidity of Eosinophilic Esophagitis (EoE) and Food Allergy with Ruchi Gupta, MD, MPH 03/22/2022

Co-morbidity of Eosinophilic Esophagitis (EoE) and Food Allergy with Ruchi Gupta, MD, MPH Many patients diagnosed with eosinophilic esophagitis (EoE) are initially diagnosed with food allergies. What is the relationship between food allergies and EoE, and what is the research available on this topic? In this episode, host Ryan Piansky, guest host Mary Jo Strobel, and Dr. Ruchi Gupta discuss the co-morbidity of EoE and food allergies. Dr. Ruchi Gupta is a Professor of Pediatrics & Medicine at the Northwestern University Feinberg School of Medicine, Clinical Attending at Ann & Robert H. Lurie Children’s Hospital of Chicago, and Director at the Center for Food Allergy & Asthma Research (CFAAR). She is world-renowned for her research on the epidemiology, economic impact, prevention, and management of allergic conditions, and strives to find answers and shape policies surrounding these conditions in order to reduce the burden and improve health equity. As a mother of a child with food allergies, Dr. Gupta also brings a personal perspective to the discussion. In this episode, Dr. Gupta shares information about food allergies and EoE, the similarities and differences between them, and some of the available treatment options. She also highlights how treatments may differ for food allergies vs EoE, and the cases in which oral immunotherapy can cause or exacerbate EoE. Tune in to find out more about allergic diseases and their relationship to EoE, and to get advice on managing food allergies and EoE. Disclaimer: The information provided in this podcast is designed to support, not replace the relationship that exists between listeners and their healthcare providers. Opinions, information, and recommendations shared in this podcast are not a substitute for medical advice. Decisions related to medical care should be made with your healthcare provider. Opinions and views of guests and co-hosts are their own. Key Takeaways: [1:22] Ryan introduces the guest for this episode, Dr. Ruchi Gupta. [2:05] What is Dr. Gupta’s experience with allergic conditions? [3:30] How do food allergies and EoE differ? [4:51] EoE is more challenging to diagnose than food allergies. [6:25] What is the difference in treatments for EoE and food allergies? [9:47] Ryan shares some of his experiences with EoE and being on an elimination diet. [10:37] Eliminating foods and reintroducing them again slowly is a treatment option for EoE, as is oral immunotherapy for some people with food allergies. What is the difference in how these treatments work for EoE vs. food allergies? [14:14] There is some indication that EoE can develop among some patients who undergo peanut oral immunotherapy (OIT). How common is it? [15:38] Is this phenomenon something that is specific to peanut OIT, or does it happen with OIT for other allergens as well? [17:31] Is there enough of a risk of developing EoE from OIT that people are routinely counseled before starting the treatment? [19:22] What are some of the associations Dr. Gupta discovered in her research? [22:37] A number of different conditions came up as being associated with EoE. Which of them were surprising to Dr. Gupta? [23:37] Ryan shares a little about his dietary restrictions and food allergies currently. [25:38] Medical diets can be pretty effective for people with EoE but sometimes it’s hard for families to adhere to. What are some challenges Dr. Gupta’s family has had with medical diets and what is her advice for people who may be new to that kind of food avoidance? [28:02] Dr. Gupta speaks to the importance of building a community around you. [32:54] Dr. Gupta recently published a new book, Food Without Fear. What is the book about, and what was Dr. Gupta’s inspiration for it? [35:49] What’s next for Dr. Gupta? [37:24] Dr. Gupta shares some final thoughts for listeners. Mentioned in This Episode: APFED on , , , , This episode is brought to you thanks to the support of our Education Partners , , , and . Tweetables: “EoE is a little bit more challenging to diagnose [than food allergies].” — Dr. Gupta “The type of EoE that a lot of people developed when they started oral immunotherapy tends to disappear once they stop.” — Dr. Gupta “How do you standardize treatment options for patients because they all have different pieces that make it really challenging?” — Dr. Gupta “Medical diets can be pretty effective for people with eosinophilic esophagitis. But sometimes it’s hard for families to adhere to.” — Ryan “It has allowed them to become better advocates for themselves and that’s not only for their condition but for life.” — Dr. Gupta “One of the most inspiring ones that I’ve heard more than once is ‘This is one part of me, this does not define me.’” — Mary Jo /episode/index/show/eosinophil/id/22496351

Drug Development and Approval: A Conversation with the FDA 02/23/2022

Drug Development and Approval: A Conversation with the FDA For many subsets of eosinophilic associated disease, there is not yet an FDA-approved therapy available. There are several therapies in various stages of development for eosinophilic esophagitis, but what exactly are the steps that need to be taken to bring a therapy to market and what is the role of the U.S. Food and Drug Administration (FDA) in medications coming to market? In this episode, host Ryan Piansky, guest host Mary Jo Strobel, and Dr. Scott Winiecki discuss this topic. Dr. Scott Winiecki is the Director of the Safe Use Initiative at the FDA. He completed his M.D. at the University of Maryland and his pediatric training at the Children’s Hospital of Philadelphia. After 12 years in private pediatric practice, he joined the U.S. Food and Drug Administration in 2011. After working on immune globulin products and vaccines for five years, he joined the Center for Drug Evaluation and Research (CDER) in September 2016. In his current role at the FDA, he is working to reduce preventable harm from medications by collaborating with both public and private groups within the healthcare community. In this episode, Dr. Winiecki explains the role of the FDA in getting a drug to market. He talks about the different stages a drug has to go through before it reaches the FDA and the phases of clinical trials in assessing a drug’s suitability for public use. Dr. Winiecki gives us an inside look at the intricacies of ensuring the safety and efficacy of a drug, as well as how patients can work with the FDA in terms of drug development. Tune in to find out more. Disclaimer: The information provided in this podcast is designed to support, not replace the relationship that exists between listeners and their healthcare providers. Opinions, information, and recommendations shared in this podcast are not a substitute for medical advice. Decisions related to medical care should be made with your healthcare provider. Opinions and views of guests and co-hosts are their own. Key Takeaways: [1:51] Education partner: Bristol Myers Squibb [1:54] Mary Jo introduces the guest for this episode, Dr. Scott Winiecki. [3:14] Dr. Winiecki shares a bit about his background and his role at the FDA. [3:58] What are some of the steps a drug typically goes through before it reaches the FDA for approval? [5:04] What are the phases of a clinical trial and what is being assessed in each phase? [8:01] How does the process differ if the drug is for rare diseases, or if the drug is already approved for some other use case, or approved in other countries? [9:15] How does the FDA engage with patients that are enrolled in a clinical trial? [9:45] What protections are in place for people participating in a clinical trial? [10:46] One concern patients may have in deciding whether to participate in a clinical trial involving investigational drugs is whether they will be able to access that medicine after the trial ends. Dr. Winiecki explains how decisions are made regarding post-trial access to a drug. [11:50] The FDA has a variety of different designations it gives to drugs. What does each of these designations mean? [16:55] What are some circumstances that might cause a delay in the approval process? [17:56] On average, how long is the development timeline for a drug to come to market? [18:25] Who decides which disorder a drug will be approved for? [20:20] How does it determine if a medicine would be made available over the counter or by prescription? [20:58] What is the FDA involvement after a drug is approved? [22:27] How would patients report side effects that they believe might be caused by a drug? [23:19] What are some considerations in the labeling of allergens used as inactive ingredients in drugs? [24:10] What is the role that patients play in drug development? What is the value of having the patient and families engaged and involved? [26:41] What are some ways patients and families can engage with the FDA, apart from the PFDD meetings? [27:40] What are some FDA programs that might focus on rare disease? [29:23] For more information and resources, visit the FDA website. Mentioned in This Episode: APFED on , , , , Call FDA — 1-888-INFO-FDA (1-888-463-6332) This episode is brought to you thanks to the support of our Education Partner . Tweetables: “Pre-clinical research can answer basic questions about drug safety, but they’re not a substitute for ways the drug can interact in the human body.” — Dr. Winiecki “The FDA continues to look at benefits and risks of a therapy throughout its lifecycle even after a drug is approved.” — Dr. Winiecki “It’s also noteworthy that most rare diseases do not have any FDA approved treatments.” — Dr. Winiecki “The FDA has a really important role in protecting patients enrolled in clinical trials.” — Dr. Winiecki “The FDA estimates it takes about 8.5 years to study and test a new drug before it can be approved for the general public.” — Dr. Winiecki “Patient involvement is very important and perhaps even more important in the context of rare diseases because obviously, no one knows the disease as well as the patient and what sort of outcomes or benefits would be important.” — Dr. Winiecki /episode/index/show/eosinophil/id/22195241

History of Eosinophils with Dr. Gerald Gleich 01/25/2022

History of Eosinophils with Dr. Gerald Gleich Eosinophil-related diseases are a relatively recent discovery in the medical field, and patient advocacy in this area has an even shorter history. In this episode, host Ryan Piansky, guest host Beth Allen, and world-renowned expert Dr. Gerald Gleich discuss eosinophils, the evolution of patient advocacy, and progress within this field. Dr. Gerald Gleich is a Professor of Dermatology and Medicine at the University of Utah and has had a lifelong professional commitment to understanding eosinophils. During his tenure, he established a research laboratory for allergic diseases at the Mayo Clinic and Foundation where he was a professor of medicine and immunology. He has contributed to more than 600 scientific articles and is a member of APFED’s Board of Directors. Co-host Beth Allen is the parent of a child who was diagnosed with eosinophilic gastrointestinal disease at a time when very little information about these conditions were available. She co-founded APFED in 2001 and is now serving as Senior Director of Strategic Sales at EvoEndo, which develops systems that enable unsedated endoscopic procedures. In this episode, Dr. Gleich shares more about what sparked his interest in researching eosinophils and how he has contributed to various related research studies. He sheds light on his discovery of Gleich syndrome, some of the studies that have contributed to a better understanding of eosinophilic conditions and their treatment, and his hopes for further research in this area. Beth Allen also talks about the history of APFED and patient advocacy in relation to eosinophil-associated diseases, and how patients have contributed to research. Tune in to find out more. Disclaimer: The information provided in this podcast is designed to support, not replace the relationship that exists between listeners and their healthcare providers. Opinions, information, and recommendations shared in this podcast are not a substitute for medical advice. Decisions related to medical care should be made with your healthcare provider. Opinions and views of guests and co-hosts are their own. Key Takeaways: [1:08] Ryan introduces his co-host for this episode, Beth Allen. [2:47] Ryan introduces the guest for this episode — Dr. Gerald Gleich. [3:15] As a specialist in dermatology, how did Dr. Gleich get interested in researching eosinophils and related diseases? [6:34] Where did Dr. Gleich’s research begin and how did his initial research play out? [9:08] At what point did Dr. Gleich start seeing other eosinophilic cases or associated disorders? [12:12] Dr. Gleich shares more about why eosinophils are named as such. [16:21] Dr. Gleich discovered an eosinophil-associated disease called Gleich syndrome. How did he discover this condition? [22:03] What did patient advocacy look like 20 years ago? Beth Allen shares how APFED got started and what things were like in the early days. [31:03] How did Dr. Gleich get involved with APFED? [34:08] Dr. Gleich shares a little about some of the studies that have been done around granule proteins and eosinophils. [39:45] Dr. Gleich shares an anecdote of a patient who had a child while on eosinophil suppressing treatment. [42:24] Despite advances being made in research in this area, there is still a lot that we don’t know and a lot more to learn. Patients have a huge role to play in contributing to research about eosinophils. [46:43] What are Dr. Gleich’s biggest hopes for research in the field of eosinophil-associated diseases going forward? [52:32] What is Dr. Gleich currently working on? [54:08] Dr. Gleich reflects on the importance of being ready and prepared for things we may not predict or expect in the future. Mentioned in This Episode: APFED on , , , , This episode is brought to you thanks to the support of our Education Partners: . Tweetables: “The eosinophil has its roots in Greek mythology, in German chemistry, and then in the wonderful insights that Ehrlich had to apply these dyes to tissues.” - Dr. Gleich “The drive to discover the syndrome was really a matter of necessity.” - Dr. Gleich “Advocacy in this community actually started with just trying to bring together education in a more uniform way [and] finding other avenues of support between us.” - Beth Allen “The amount of support that came back from the simple asks that we had was surprising.” - Beth Allen /episode/index/show/eosinophil/id/21887534

Research Advances in Identifying EoE-causal Foods with Dr. David Hill 12/28/2021

Research Advances in Identifying EoE-causal Foods with Dr. David Hill Recent findings from research co-funded by APFED and the American Academy of Allergy, Asthma & Immunology could have a major impact on food allergy testing for people with eosinophilic esophagitis (EoE). In this episode, co-hosts Dawn McCoy and Ryan Piansky discuss the research that could better identify food triggers that cause EoE symptoms with the principal investor of the study, David Hill, MD, PhD. Dr. Hill is an Assistant Professor of Pediatrics at the Perelman School of Medicine at the University of Pennsylvania and an Attending Physician in the Division of Allergy and Immunology at the Children’s Hospital of Philadelphia (CHOP). In addition to being a practicing allergist, Dr. Hill runs a basic and translational research laboratory that studies the role of the immune system in allergy and obesity, with a particular focus on eosinophilic esophagitis (EoE). He is the author of more than 50 research articles and abstracts, is on the Editorial Board of BMC Pediatrics, and has been the recipient of several awards including the Young Faculty Award from the American College of Allergy, Asthma, and Immunology and the Young Physician-Scientist Award from the American Society for Clinical Investigation. In this episode, Dr. Hill shares more about his interest in studying EoE, and how he became involved in research related to food triggers and EoE. He explains how the new tests he and his team have created compare to traditional food allergy tests, and why the new tests are much more effective at identifying EoE food triggers. Dr. Hill also sheds light on the impact these tests could have on clinical management of EoE, and the impact they could have on people with EoE. Tune in to find out more. Disclaimer: The information provided in this podcast is designed to support, not replace the relationship that exists between listeners and their healthcare providers. Opinions, information, and recommendations shared in this podcast are not a substitute for medical advice. Decisions related to medical care should be made with your healthcare provider. Opinions and views of guests and co-hosts are their own. Key Takeaways: [1:34] Ryan introduces the guest for this episode — Dr. David Hill. [2:15] Dr. Hill shares information about his background and what led to his interest in studying eosinophilic esophagitis. [3:38] How does traditional food allergy testing work? [6:37] Dr. Hill is involved in research to create a special test to help quickly and easily identify foods that could be triggering symptoms of EoE. He explains how he entered this research area. [10:03] Dr. Hill recently published a paper describing a new form of testing for EoE. He explains more about the test and how it fits in clinical management. [11:34] Are Dr. Hill's tests similar to the food sensitivities tests available online? [12:39] What are T-cells? [15:36] Traditional food allergy tests are not much better than guessing for diagnosing allergens. Dr. Hill’s new test is much more accurate. [18:51] These same types of tests can also be used to determine if new triggers have developed in a patient with EoE. [20:23] Could this test also be applied to non-food triggers such as environmental allergies? [21:23] Would this test be beneficial for other sorts of eosinophil-associated diseases that aren't purely focused on the digestive tract? [22:56] Are clinicians already using this test in daily practice? [24:35] Is Children’s Hospital of Philadelphia (CHOP) the only center currently recruiting for this study? [25:50] What are some things that have surprised Dr. Hill in his research? [27:25] What are some of Dr. Hill's upcoming research projects related to EoE? [30:00] Dr. Hill shares some final thoughts. Mentioned in This Episode: APFED on , , , , This episode is brought to you thanks to the support of our Education Partners: , , and . Tweetables: “We study EoE using animal models, and we've actually shown that IgE is not necessary to develop an EoE-like disease in a mouse, for example.” — Dr. Hill “We developed a new test that instead of looking at the IgE molecule, looks at the T-cell, and what we're hoping is that by looking at the food response that the T-cell makes, that we can then figure out which foods are causing the disease.” — Dr. Hill “[The tests] could be used to determine whether or not new triggers or previously undiagnosed triggers were contributing to EoE.” — Dr. Hill /episode/index/show/eosinophil/id/21606290

Understanding and Managing Eosinophilic Granulomatosis with Polyangiitis (EGPA) with Dr. Peter Merkel 12/17/2021

Understanding and Managing Eosinophilic Granulomatosis with Polyangiitis (EGPA) with Dr. Peter Merkel Eosinophilic Granulomatosis with Polyangiitis (EGPA), formerly known as Churg-Strauss Syndrome, is a rare condition that can be challenging to diagnose. In this episode, co-hosts Dawn McCoy and Ryan Piansky explore the topic of EGPA and how it can be managed with expert Peter A. Merkel, MD, MPH. Dr. Merkel is the Chief of Rheumatology and a Professor of Medicine and Epidemiology at the University of Pennsylvania. Dr. Merkel is an internationally recognized research and clinical expert in vasculitis and scleroderma and is an author of over 350 scientific publications. He is the Principal Investigator of the NIH-Sponsored Vasculitis Clinical Research Consortium (VCRC), a leading international research infrastructure for vasculitis clinical investigation. Dr. Merkel’s research focuses on clinical trial design and conduct, outcome measure development, clinical epidemiology, genetic epidemiology, and biomarker discovery. In this episode, Dr. Merkel shares information about what EGPA is, and why diagnosing it can be a challenge. A rare disease that mimics other conditions, EGPA is often misdiagnosed and confused with other similar conditions. Dr. Merkel explains the key identifying features of EGPA, current treatment options, and how patients can manage their symptoms by working closely with their healthcare team. He also talks about research advances in this area, and how patients can help in the understanding, treatment, and management of EGPA. Tune in to find out more. Disclaimer: The information provided in this podcast is designed to support, not replace the relationship that exists between listeners and their healthcare providers. Opinions, information, and recommendations shared in this podcast are not a substitute for medical advice. Decisions related to medical care should be made with your healthcare provider. Opinions and views of guests and co-hosts are their own. Key Takeaways: [1:31] Ryan introduces the guest for this episode — Dr. Peter Merkel. [2:15] Dr. Merkel shares a little about his background and his experiences treating people with EGPA. [3:42] EGPA can be difficult to diagnose because the symptoms are similar to other diseases. What are some common conditions that EGPA is misdiagnosed as? [7:30] How well is EGPA understood among doctors? [9:11] There is a lot of overlap between EGPA and other conditions. What makes EGPA unique? [10:11] How likely are doctors to come across a situation where it is impossible to distinguish between EGPA and other conditions? [11:39] What are some indications or symptoms that would prompt a doctor to start exploring EPGA as a diagnosis? Specifically, how do doctors make that diagnosis? [13:01] How would doctors determine if a person’s asthma is related to EGPA or eosinophilic asthma? Can a person have both conditions? [14:23] Do most people with EGPA end up seeing a number of different specialists before they get to diagnosis? [15:50] Who are some of the specialists that a person with EGPA might want to have on their care team? [17:14] With so many different things to juggle at once, how often should patients see their care team? [19:24] What kinds of research is being conducted to develop a proper diagnostic test for EGPA? [20:54] Dr. Merkel explains more about the different treatment options available and how they work. [25:54] The other aspect of treating EGPA is addressing the airway disease. [27:05] What is considered well-controlled EGPA? [28:05] What is the typical progression of EGPA? [29:00] EGPA is a fairly rare condition. Is it more common in children or adults? [30:57] What are some key findings from recent research about EGPA? [32:17] How has Dr. Merkel seen patients make a difference in research discoveries? [33:26] Dr. Merkel shares a few resources for listeners. Mentioned in This Episode: APFED on , , , , This episode is brought to you thanks to the support of our Education Partner: . Tweetables: “EGPA is a form of vasculitis but really, it’s a combination of multiple different organ system problems. The commonality among patients who have EGPA is over 90% of patients will have asthma.” — Dr. Merkel “[EGPA] is a rare disease. It’s unusual and it can mimic other things and it hides in plain sight.” — Dr. Merkel “The patient needs to be part of that captaincy, if you will, and he or she should help take control of their own disease. I think that’s very important for patients to be as aware as they can so they can help work together.” — Dr. Merkel “You can’t do research without patients. They’re at the center of what we do.” — Dr. Merkel /episode/index/show/eosinophil/id/21512003

Understanding and Managing Eosinophilic Asthma with Dr. Ravi Viswanathan 11/19/2021

Understanding and Managing Eosinophilic Asthma with Dr. Ravi Viswanathan There has been a growing awareness of eosinophilic asthma in recent years, and thereare increasingly more treatment options for people with this type of asthma. In thisepisode, co-hosts Ryan Piansky and Mary Jo Strobel explore the topic of eosinophilicasthma, triggers, treatment options, and more with Ravi Viswanathan, MD. Dr. Viswanathan is a board-certified Allergist & Immunologist, and Assistant Professorin the Section of Allergy, Pulmonary & Critical Care at the University of WisconsinSchool of Medicine & Public Health, as well as the Medical Director of the Allergy Clinic.His research and clinical interests include severe asthma, mast cell disorders, and drugallergy. Dr. Viswanathan has authored and co-authored multiple publications and bookchapters, including the chapter on “Management of Asthma in Adolescents and Adults”in Middleton’s Allergy Textbook. He is also involved with teaching at the University ofWisconsin School of Medicine for topics on asthma, drug allergy, and muscle disorders. In this episode, Dr. Viswanathan shares more about the different types of asthma, andthe differences between them. He explains more about the diagnostic journey for aperson with eosinophilic asthma, the different triggers that may exacerbate symptoms, and treatment options available to patients. Dr. Viswanathan also sheds light onresearch being conducted in this area, and how patients may better manage theirasthma by working closely with their healthcare team. Tune in to find out more abouteosinophilic asthma and how patients can get better outcomes and improve their qualityof life. Disclaimer: The information provided in this podcast is designed to support, not replace therelationship that exists between listeners and their healthcare providers. Opinions, information,and recommendations shared in this podcast are not a substitute for medical advice. Decisionsrelated to medical care should be made with your healthcare provider. Opinions and views ofguests and co-hosts are their own. Key Takeaways: [1:43] Ryan introduces the guest for this episode — Dr. Ravi Viswanathan. [2:20] Dr. Viswanathan shares a bit about his background and experience treating people who have asthma. [3:57] What do the terms “phenotype” and “endotype” mean? What are the differences between them? [5:25] What are some examples of different phenotypes of asthma? [6:03] There has been a growing awareness of eosinophilic asthma. What has led to this awareness? [7:34] What makes the phenotype in eosinophilic asthma different from other types of asthma? [8:27] Ryan shares his experience about having asthma as a child and having high eosinophil counts, but only being diagnosed with eosinophilic asthma much later. Is this a common occurrence? [9:25] At what point do doctors start to establish a diagnosis for a specific phenotype of asthma, instead of just asthma in general? [10:55] What are some common tests that can help diagnose between specific phenotypes of asthma? [13:08] Dr. Viswanathan shares more about sputum samples and bronchial washing. [14:35] What is considered well-controlled asthma, and how can people know if their asthma is indeed well-controlled? [15:56] What are some common asthma triggers, and are these triggers generally consistent across different types of asthma? [18:15] What are some things beyond medicine that can help control asthma? [20:25] What are some treatment options available to people with asthma and what are the differences between these treatments? [24:00] How do these treatment options work to help people with asthma? [25:49] Having a combination of therapies may help patients better deal with their symptoms and improve their quality of life. [27:02] Is immunotherapy or a gradual increased exposure to triggers to build up tolerance something that is applicable to EOS asthma? [28:48] How quickly would symptoms return for someone whose treatment has been stopped or interrupted? [30:02] How often should patients visit their doctor to monitor all the factors involved in EOS asthma? [31:07] What is the benefit of having different treatment options available? [33:43] Are there large disparities in efficacy between different drugs used in treatment? [35:21] From a research perspective, what are some areas that are being looked at in regard to eosinophilic asthma? [36:48] Dr. Viswanathan shares some final closing thoughts. Mentioned in This Episode: APFED on , , , , This episode is brought to you thanks to the support of our Education Partner: . /episode/index/show/eosinophil/id/21196229

EGID Elimination Diets: Creative Strategies to Make Mealtimes More Engaging with Holly Knotowicz 09/17/2021

EGID Elimination Diets: Creative Strategies to Make Mealtimes More Engaging with Holly Knotowicz There is no denying that mealtime can be a challenge for those with eosinophilic gastrointestinal diseases (EGIDs) who are on elimination diets. In this episode, co-hosts Dawn McCoy and Ryan Piansky discuss some creative strategies that can help make mealtimes easier with Holly Knotowicz, MS, CCC-SLP. Holly Knotowicz is a Speech-Language Pathologist and a Feeding and Swallowing Specialist. Her background is in Communication Sciences and Disorders, and her areas of clinical interest include behavioral, oral motor, and oral sensory feeding disorders, including mealtime dynamics. Holly specializes in working with children who have eosinophilic gastrointestinal diseases (EGID), including eosinophilic esophagitis (EoE), and working with children who have food allergies, FPIES, and neurogenic disorders. She is an active participant in research, has been published in Gastroenterology Journal, and is an international speaker on feeding. In this episode, Holly shares a little about her own journey with EoE, how that influenced her to dedicate herself to this field, as well as how feeding therapists can help care for those with these conditions. Holly draws on her years of experience to share tips, strategies, and creative coping mechanisms to improve people’s experiences with food, as well as how to navigate the social norms around food and eating. Tune in to find out more about how a feeding therapist can become an invaluable part of a healthcare team for someone with an EGID, such as EoE. Disclaimer: The information provided in this podcast is designed to support, not replace the relationship that exists between listeners and their healthcare providers. Opinions, information, and recommendations shared in this podcast are not a substitute for medical advice. Decisions related to medical care should be made with your healthcare provider. Opinions and views of guests and co-hosts are their own. Key Takeaways: [1:40] Ryan introduces the guest for this episode — Holly Knotowicz. [2:40] Holly shares a little about how she got started in her field. [3:11] What is the role of feeding therapists in the care of someone with Eosinophilic Esophagitis (EoE)? [4:08] How does Holly collaborate with other specialists, specifically pediatricians and gastroenterologists? [5:27] What are some of Holly's tips for people who are just starting an elimination diet? [9:42] Ryan relates his personal experience of having limited foods in his diet. [10:37] What are some coping mechanisms that Holly recommends for people who have trouble with a new food? [13:30] Holly touches on the social aspect of food in our culture. How might someone with dietary restrictions navigate these social activities? [18:48] What are some of the long-term benefits of parents and children working with feeding therapy services? [21:17] How does Holly help children and families who have a reluctance to reintroduce certain foods back into their diet, and how can they work with their healthcare provider to do that? [24:50] What are some creative tactics and tools that Holly gives the children she works with to help them in the long term after they leave the treatment session? [29:20] How can someone locate a feeding specialist? Mentioned in This Episode: APFED on , , , , This episode is brought to you thanks to the support of our Education Partners: , , and . /episode/index/show/eosinophil/id/20490611

Real Talk: Eosinophilic Diseases

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