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.
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Bone Mineral Density in Pediatric Eosinophilic Esophagitis
Episode 47
Tuesday, June 24, 2025 • Duration 33:16
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 living with EoE who serves on APFED’s Health Sciences Advisory Council, interview Anna Henderson, MD, a pediatric gastroenterologist at Northern Light Health in Maine, about bone mineral density in EoE patients. They discuss a paper she co-authored on the subject.
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 introduces the episode, brought to you thanks to the support of Education Partners Bristol Myers Squibb, Sanofi, Regeneron, and Takeda. Ryan introduces co-host Holly Knotowicz.
[1:17] Holly introduces today’s topic, eosinophilic esophagitis (EoE), and bone density.
[1:22] Holly introduces today’s guest, Dr. Anna Henderson, a pediatric gastroenterologist at Northern Light Health in Maine.
[1:29] During her pediatric and pediatric gastroenterology training at Cincinnati Children’s Hospital, she took a special interest in eosinophilic esophagitis. In 2019, Dr. Henderson received APFED’s NASPGHAN Outstanding EGID Abstract Award.
[1:45] Holly, a feeding therapist in Maine, has referred many patients to Dr. Henderson and is excited to have her on the show.
[2:29] Dr. Henderson is a wife and mother. She loves to swim and loves the outdoors. She practices general pediatric GI in Bangor, Maine, at a community-based academic center.
[2:52] Her patient population is the northern two-thirds of Maine. Dr. Henderson feels it is rewarding to bring her expertise from Cincinnati to a community that may not otherwise have access to specialized care.
[3:13] Dr. Henderson’s interest in EoE grew as a GI fellow at Cincinnati Children’s. Her research focused on biomarkers for disease response to dietary therapies and EoE’s relationship to bone health.
[3:36] As a fellow, Dr. Henderson rotated through different specialized clinics. She saw there were many unanswered questions about the disease process, areas to improve treatment options, and quality of life for the patients suffering from these diseases.
[4:00] Dr. Henderson saw many patients going through endoscopies. She saw the social barriers for patients following strict diets. She saw a huge need in EoE and jumped on it.
[4:20] Ryan grew up with EoE. He remembers the struggles of constant scopes, different treatment options, and dietary therapy. Many people struggled to find what was best for them before there was a good approved treatment.
[4:38] As part of Ryan’s journey, he learned he has osteoporosis. He was diagnosed at age 18 or 19. His DEXA scan had such a low Z-score that they thought the machine was broken. He was retested.
[5:12] Dr. Henderson explains that bone mineral density is a key measure of bone health and strength. Denser bones contain more minerals and are stronger. A low bone mineral density means weaker bones. Weaker bones increase the risk of fracture.
[5:36] DEXA scan stands for Dual Energy X-ray Absorptiometry scan. It’s a type of X-ray that takes 10 to 30 minutes. A machine scans over their bones. Typically, we’re most interested in the lumbar spine and hip bones.
[5:56] The results are standardized to the patient’s height and weight, with 0 being the average. A negative number means weaker bones than average for that patient’s height and weight. Anything positive means stronger bones for that patient’s height and weight.
[6:34] A lot of things can affect a patient’s bone mineral density: genetics, dietary history, calcium and Vitamin D intake, and medications, including steroid use. Prednisone is a big risk factor for bone disease.
[7:07] Other risk factors are medical and auto-immune conditions, like celiac disease, and age. Any patient will have their highest bone density in their 20s to 30s. Females typically have lower bone mineral density than males.
[7:26] The last factor is lifestyle. Patients who are more active and do weight-bearing exercises will have higher bone mineral density than patients who have more of a sedentary lifestyle.
[7:56] Ryan was told his bone mineral density issues were probably a side-effect of the long-term steroids he was on for his EoE. Ryan is now on benralizumab for eosinophilic asthma. He is off steroids.
[8:36] Dr. Henderson says the research is needed to find causes of bone mineral density loss besides glucocorticoids.
[8:45] EoE patients are on swallowed steroids, fluticasone, budesonide, etc. Other patients are on steroids for asthma, eczema, and allergic rhinitis. These may be intranasal steroids or topical steroids.
[9:01] Dr. Henderson says we wondered whether or not all of those steroids and those combined risks put the EoE population at risk for low bone mineral density. There’s not a lot published in that area.
[9:14] We know that proton pump inhibitors can increase the risk of low bone mineral density. A lot of EoE patients are on proton pump inhibitors.
[9:23] That was where Dr. Henderson’s interest started. She didn’t have a great way to screen for bone mineral density issues or even know if it was a problem in her patients more than was expected in a typical patient population.
[9:57] Holly wasn’t diagnosed with EoE until she was in her late 20s. She was undiagnosed but was given prednisone for her problems. Now she wonders if she should get a DEXA scan.
[10:15] Holly hopes the listeners will learn something and advocate for themselves or for their children.
[10:52] If a patient is concerned about their bone mineral density, talking to your PCP is a perfect place to start. They can discuss the risk factors and order a DEXA scan and interpret it, if needed.
[11:11] If osteoporosis is diagnosed, you should see an endocrinologist, specifically to discuss therapy, including medications called bisphosphonates.
[11:36] From an EoE perspective, patients can talk to their gastroenterologist about what bone mineral density risk factors may be and if multiple risk factors exist. Gastroenterologists are also more than capable of ordering DEXA scans and helping their patients along that journey.
[11:53] A DEXA scan is typically the way to measure bone mineral density. It’s low radiation, it’s easy, it’s fast, and relatively inexpensive.
[12:10] It’s also useful in following up over time in response to different interventions, whether or not that’s stopping medications or starting medications.
[12:30] Dr. Henderson co-authored a paper in the Journal of Pediatric Gastroenterology and Nutrition, called “Prevalence and Predictors of Compromised Bone Mineral Density in Pediatric Eosinophilic Esophagitis.” The study looked at potential variables.
[12:59] The researchers were looking at chronic systemic steroid use. They thought it was an issue in their patients, especially patients with multiple atopic diseases like asthma, eczema, and allergic rhinitis. That’s where the study started.
[13:22] Over the years, proton pump inhibitors have become more ubiquitous, and more research has come out. The study tried to find out if this was an issue or not. There weren’t any guidelines for following these patients, as it was a retrospective study.
[13:42] At the time, Dr. Henderson was at a large institution with a huge EoE population. She saw that she could do a study and gather a lot of information on a large population of patients. Studies like this are the start of figuring out the guidelines for the future.
[14:34] Dr. Henderson wanted to determine whether pediatric patients with EoE had a lower-than-expected bone mineral density, compared to their peers.
[14:44] Then, if there were deficits, she wanted to determine where they were more pronounced. Were they more pronounced in certain subgroups of patients with EoE?
[14:59] Were they patients with an elemental diet? Patients with an elimination diet? Were they patients on steroids or PPIs? Were they patients with multiple atopic diseases? Is low bone mineral density just a manifestation of their disease processes?
[15:14] Do patients with active EoE have a greater propensity to have low bone mineral density? The study was diving into see what the potential risk factors are for this patient population.
[15:45] The study was a retrospective chart review. They looked at patients aged 3 to 21. You can’t do a DEXA scan on a younger patient, and 21 is when people leave pediatrics.
[16:03] These were all patients who had the diagnosis of EoE and were seen at Cincinnati Children’s in the period between 2014 and 2017. That period enabled full ability for chart review. Then they looked at the patients who had DEXA scans.
[16:20] They did a manual chart review of all of the patients and tried to tease out what the potential exposures were. They looked at demographics, age, sex, the age of the diagnosis of EoE, medications used, such as PPIs, and all different swallowed steroids.
[16:44] They got as complete a dietary history as they could: whether or not patients were on an elemental diet, whether that was a full elemental diet, whether they were on a five-food, six-food, or cow’s milk elimination diet.
[16:58] They teased out as much as they could. One of the limitations of a retrospective chart review is that you can’t get some of the details, compared to doing a prospective study. For example, they couldn’t tease out the dosing or length of therapy, as they would have liked.
[17:19] They classified those exposures as whether or not the patient was ever exposed to those medications, whether or not they were taking them at the time of the DEXA scan, or if they had been exposed within the year before the DEXA scan.
[17:40] They also looked at whether the patients had other comorbid atopic disorders, to see if those played a role, as well.
[18:03] The study found that there was a slightly lower-than-expected bone mineral density in the patients. The score was -0.55, lower than average but not diagnostic of a low bone mineral density, which would be -2 or below.
[18:27] There were 23 patients with low bone mineral density scores of -2 or below. That was 8.6% of the study patients. Typically, only 2.5% of the population would have that score. It was hard to tease out the specific risk factors in a small population of 23.
[18:57] They looked at what the specific risk factors were that were associated with low bone mineral density, or bone mineral density in general.
[19:12] After moving from Colorado, Holly has transferred to a new care team, and doctors wanted her baseline Vitamin D and Calcium levels. No one had ever tested that on her before. Dr. Henderson says it’s hard because there’s nothing published on what to do.
[19:58] The biggest surprise in the study was that swallowed steroids, or even combined steroid exposure, didn’t have any effect on bone mineral density. That was reassuring, in light of what is known about glucocorticoid use.
[20:16] The impact of PPI use was interesting. The study found that any lifetime use of PPIs did seem to decrease bone mineral density. It was difficult to tease out the dosing and the time that a patient was on PPIs.
[20:34] Dr. Henderson thinks that any lifetime use of PPIs is more of a representation of their cumulative use of PPIs. At the time of the study, from 2014 to 2017, PPIs were still very much first-line therapy for EoE; 97% of the study patients had taken PPIs at some time.
[21:02] There are so many more options now for therapy when a patient has a new diagnosis of EoE, especially with dupilumab now being an option.
[21:11] Dr. Henderson speaks of patients who started on PPIs and have stayed on them for years. This study allows her to question whether we need to continue patients on PPIs. When do we discuss weaning patients off PPIs, if appropriate?
[22:05] Ryan says these podcasts are a great opportunity for the community at large and also for the hosts. He just wrote himself a note to ask his endocrinologist about coming off PPIs.
[22:43] Dr. Henderson says that glucocorticoid use is a known risk factor for low bone mineral density and osteoporosis. In the asthma population, inhaled steroids can slightly decrease someone’s growth potential while the patient is taking them.
[23:10] From those two facts, it was thought that swallowed steroids would have a similar effect. But since they’re swallowed and not systemic, maybe things are different.
[23:23] It was reassuring to Dr. Henderson that what her study found was that the swallowed steroid didn’t affect bone mineral density. There was one other study that found that swallowed steroids for EoE did not affect someone’s height.
[23:51] Dr. Henderson clarifies that glucocorticoids include systemic steroids like prednisone and hydrocortisone.
[23:57] Based on Dr. Henderson’s retrospective study, fluticasone as a swallowed steroid did not affect bone mineral density. It was hard to tease out the dosing, but the cumulative use did not seem to result in a deficit for bone mineral density.
[24:16] Holly shared that when she tells a family of a child she works with that the child’s gastroenterologist will likely recommend steroids, she will now give them the two papers Dr. Henderson mentioned. There are different types of steroids. The average person doesn’t know the difference.
[25:15] Dr. Henderson thinks that for patients who have multiple risk factors for low bone mineral density, it is reasonable to have a conversation about bone health with their gastroenterologist to see whether or not a DEXA scan would be worth it.
[25:56] If low bone mineral density is found, that needs to be followed up on.
[26:03] There are no great guidelines, but this study is a good start on what these potential risk factors are. We need some more prospective studies to look at these risk factors in more detail than Dr. Henderson’s team teased out in this retrospective study.
[26:23] Dr. Henderson tells how important it is for patients to participate in prospective longitudinal studies for developing future guidelines.
[26:34] Holly points out that a lot of patients are on restrictive diets. It’s important to think about the whole picture if you are starting a medication or an elimination, or a restricted diet. You have to think about the impact on your body, overall.
[27:11] People don’t think of dietary therapy as medication, but it has risks and benefits involved, like a medication.
[27:50] Dr. Henderson says, in general, lifestyle management is the best strategy for managing bone health. Stay as active as you can with weight-bearing exercises and eating a well-balanced diet. If you are on a restrictive diet, make sure it’s well-balanced.
[28:12] Dr. Henderson says a lot of our patients have feeding disorders, so they see feeding specialists like Holly. A balanced diet is hard when kids are very selective in their eating habits.
[29:10] Dr. Henderson says calcium and Vitamin D are the first steps in how we treat patients with low bone mineral density. A patient who is struggling with osteoporosis needs to discuss it with their endocrinologist for medications beyond supplementation.
[29:31] Ryan reminds listeners who are patients always to consult with their medical team. Don’t go changing anything up just because of what we’re talking about here. Ask your care team some good questions.
[29:47] Dr. Henderson would like families to be aware, first, that some patients with EoE will have bone mineral density loss, especially if they are on PPIs and restrictive diets. They should start having those discussions with their providers.
[30:04] Second, Dr. Henderson would like families to be reassured that swallowed steroids and combined steroid exposure didn’t have an impact on bone mineral density. Everyone can take that away from today’s chat.
[30:18] Lastly, Dr. Henderson gives another plug for patient participation in prospective studies, if they’re presented with the opportunity. It’s super important to be able to gather more information and make guidelines better for our patients.
[30:35] Holly thanks Dr. Henderson for coming on Real Talk — Eosinophilic Diseases and sharing her insights on bone mineral density, and supporting patients in Maine.
[30:57] Dr. Henderson will continue to focus on the clinical side. She loves doing outreach clinics in rural Maine. It’s rewarding, getting to meet all of these patients and taking care of patients who would otherwise have to travel hours to see a provider.
[32:01] Ryan thinks the listeners got a lot out of this. For our listeners who would like to learn more about eosinophilic disorders, please visit APFED.org and check out the links in the show notes.
[32:11] If you’re looking to find specialists who treat eosinophilic disorders, we encourage you to use APFED’s Specialist Finder at APFED.org/specialist.
[32:19] 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.
[32:28] Ryan thanks Dr. Henderson for joining us today for this great conversation. Holly also thanks APFED’s Education Partners Bristol Myers Squibb, Sanofi, Regeneron, and Takeda for supporting this episode.
“DEXA scan stands for dual-energy X-ray absorptiometry scan. It’s a type of X-ray where a patient lies down for 10 to 30 minutes. A machine scans over their bones. Typically, we’re most interested in the lumbar spine and hip bones.” — Anna Henderson, MD
“We wondered whether or not all of those steroids and those combined risks even put our EoE population at risk for low bone mineral density. There’s not a lot published in that area.” — Anna Henderson, MD
“If a patient is worried [about their bone mineral density], their PCP is a perfect place to start for that. They’re more than capable of discussing the risk factors specific for that patient, ordering a DEXA scan, and interpreting it if need be.” — Anna Henderson, MD
“I think we need some more prospective studies to look at these risk factors in a little bit more detail than we were able to tease out in our retrospective review.” — Anna Henderson, MD
“Just another plug for the participation in prospective studies, if you’re presented with the opportunity. It’s super important to be able to gather more information and to be able to make guidelines better for our patients about these risks.” — Anna Henderson, MD
Common Nutritional Deficiencies that Affect Those with Non-EoE EGIDs
Episode 46
Friday, May 30, 2025 • Duration 41:27
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 living with EoE who serves on APFED’s Health Sciences Advisory Council, interview Bethany Doerfler, MS, RDN, a clinical research dietician specializing in lifestyle management of digestive diseases at Northwestern Medicine. Ryan and Holly discuss managing nutritional deficiencies in patients with non-EoE EGIDs and a study Bethany worked on.
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 introduces the episode, brought to you thanks to the support of Education Partners Bristol Myers Squibb, Sanofi, Regeneron, and Takeda. Ryan introduces co-host Holly Knotowicz.
[1:17] Holly introduces today’s topic, common nutritional deficiencies that affect those with eosinophilic gastrointestinal diseases that occur in the GI tract lower than the esophagus (non-EoE EGIDs).
[1:31] Holly introduces today’s guest, Bethany Doerfler, a clinical research dietician specializing in lifestyle management of digestive diseases, including gastroesophageal reflux disease, motility disorders, and eosinophilic diseases.
[1:45] Bethany currently practices as part of a multi-disciplinary team in a digestive health institute at Northwestern Medicine.
Comparing Pediatric and Adult EoE
Wednesday, April 30, 2025 • Duration 39:29
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 living with EoE who serves on APFED’s Health Sciences Advisory Council, interview Dr. Melanie Ruffner, an Attending Physician with the Division of Allergy and Immunology and the Center for Pediatric Eosinophilic Disorders at Children’s Hospital of Philadelphia. Dr. Ruffner describes her work in clinic and the paper she co-authored about pediatric and adult eosinophilic esophagitis (EoE). She covers the questions they considered in the paper and the conclusions they reached.
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 introduces the episode, brought to you thanks to the support of Education Partners Bristol Myers Squibb, GSK, Sanofi, Regeneron, and Takeda. Ryan introduces co-host Holly Knotowicz.
[1:17] Holly introduces today’s topic, pediatric and adult eosinophilic esophagitis (EoE), and introduces today’s guest, Dr. Melanie Ruffner.
[1:23] Dr. Melanie Ruffner is an attending physician with the Division of Allergy and Immunology in the Center for Pediatric Eosinophilic Disorders at Children’s Hospital of Philadelphia. Holly welcomes Dr. Ruffner to Real Talk.
[1:50] As an attending physician in the Center for Pediatric Eosinophilic Disorders at Children’s Hospital of Philadelphia, Dr. Ruffner sees patients who have eosinophilic esophagitis and other eosinophilic disorders, including eosinophilic GI tract disorders.
Full Circle: An Immunologist’s Unexpected EoE Journey
Episode 44
Tuesday, March 25, 2025 • Duration 39:21
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 living with EoE who serves on APFED’s Health Sciences Advisory Council, interview Dr. John Accarino, an allergist and immunologist at Massachusetts General Hospital and Mass General for Children, on the topic of immunology support for eosinophilic esophagitis (EoE). Dr. Accarino shares his experiences as a person living with food allergies, allergic asthma, peanut allergy, and eosinophilic esophagitis. He tells how his experiences help him in his work with patients. Dr. Accarino shares some education on a variety of allergy mechanisms and the treatments that mitigate them.
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 introduces the episode, brought to you thanks to the support of Education Partners Bristol Myers Squibb, GSK, Sanofi, and Regeneron. Ryan introduces co-host Holly Knotowicz.
[1:14] Holly introduces today’s topic, immunology support for eosinophilic esophagitis (EoE), and introduces today’s guest, Dr. John Accarino, an allergist and immunologist at Massachusetts General Hospital. Holly welcomes Dr. Accarino to Real Talk.
[1:49] Holly notes that Dr. Accarino is her allergist and immunologist.
[2:03] Dr. Accarino works at Massachusetts General Hospital and Mass General for Children. Allergy and Immunology is a field where he can see pediatrics and adults. Originally trained in pediatrics, now Dr. Accarino sees patients of all ages.
The Intersection of Food Allergy and Eosinophilic Esophagitis
Episode 43
Thursday, February 27, 2025 • Duration 50:03
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 living with EoE who serves on APFED’s Health Sciences Advisory Council, interview Dr. Wayne Shreffler, Chief of Pediatric Allergy and Immunology and Co-Director of The Food Allergy Center at Massachusetts General Hospital. Dr. Shreffler is also an investigator at The Center for Immunology and Inflammatory Disease and The Food Allergy Science Initiative. His research is focused on understanding how adaptive immunity to dietary antigens is both naturally regulated and modulated by therapy in the context of food allergy. This interview covers the results of a research paper on The Intersection of Food Allergy and Eosinophilic Esophagitis, co-authored by Dr. Shreffler.
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 introduces the episode, brought to you thanks to the support of Education Partners Bristol Myers Squibb, GSK, Sanofi, and Regeneron. Ryan introduces co-host, Holly Knotowicz.
[1:15] Holly introduces today’s topic, the intersection of food allergy and eosinophilic esophagitis.
[1:26] Holly introduces today’s guest, Dr. Wayne Shreffler, Chief of Pediatric Allergy and Immunology and Co-Director of The Food Allergy Center at Massachusetts General Hospital and an investigator at The Center for Immunology and Inflammatory Disease and The Food Allergy Science Initiative.
Guidelines for Childhood non-EoE EGIDs
Episode 42
Wednesday, January 22, 2025 • Duration 33:41
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 living with EoE who serves on APFED’s Health Sciences Advisory Council, interview Dr. Margaret Collins, a professor of pathology at the University of Cincinnati and a staff pathologist at Cincinnati Children’s Hospital Medical Center. Dr. Collins was a member of the task force that produced the Guidelines on Childhood EGIDs Beyond EoE. In this interview, Dr. Collins discusses the guidelines and how they were created and shares some of the results, including an algorithm for diagnosing non-EoE EGIDs. She shares why she specialized in EGIDs and what her hopes are for the future development of the guidelines.
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 introduces the episode, brought to you thanks to the support of Education Partners Bristol Myers Squibb, GSK, Sanofi, and Regeneron, and co-host, Holly Knotowicz.
[1:27] Holly introduces today’s guest, Dr. Margaret Collins, a professor of pathology at the University of Cincinnati and a staff pathologist at Cincinnati Children’s Hospital Medical Center.
[1:38] Dr. Collins specializes in the pathology of pediatric gastrointestinal disease, especially EGIDs, and is a central pathology reviewer for the Consortium of Eosinophilic Gastrointestinal Researchers (CEGIR), as well as a member of APFED’s Health Sciences Advisory Council.
Eosinophilic Fasciitis (EF), with Dr. Catherine Sims and Jason Ingraham
Episode 41
Wednesday, December 18, 2024 • Duration 43:46
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 living with EoE who serves on APFED’s Health Sciences Advisory Council, interview Jason Ingraham, an adult living with eosinophilic fasciitis (EF), and Dr. Catherine Sims, a rheumatologist at Duke University and a Health Services Research Fellow at the Durham Veterans’ Affairs Hospital. They discuss Jason’s experiences living with EF and Dr. Sims’s experience treating EF. They share Jason’s journey to diagnosis and the importance of working with a group of specialists. They share tips on medication and physical therapy, how to communicate with your medical team, and manage your activity and mindset.
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 introduces the episode, brought to you thanks to the support of Education Partners Bristol Myers Squibb, GSK, Sanofi, and Regeneron, and co-host, Holly Knotowicz.
[1:14] Holly introduces today’s topic, eosinophilic fasciitis, with guests, Jason Ingraham and Dr. Catherine Sims.
[1:25] Jason is an adult living with eosinophilic fasciitis (EF). Dr. Sims is a rheumatologist at Duke University and a Health Services Research Fellow at the Durham Veterans’ Affairs Hospital.
[1:52] Dr. Sims explains what EF is. Patients may present with symptoms of large plaques on their skin, edema of arms and legs, Raynaud’s Phenomenon, contractures of arms or legs, limited mobility, or loss of the ability to do tasks they used to do.
The Evolution of Eosinophilic Gastrointestinal Disorders, with Dr. Dan Atkins
Episode 40
Friday, November 22, 2024 • Duration 48:48
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 living with EoE who serves on APFED’s Health Sciences Advisory Council, interview Dr. Dan Atkins about Children’s Hospital Colorado’s multidisciplinary treatment program for eosinophilic gastrointestinal disorders (EGIDs).
In this episode, Ryan and Holly interview their friend, Dr. Dan Atkins. Ryan was a long-time patient of Dr. Atkins and Holly worked as a feeding specialist with Dr. Atkins at Children’s Hospital Colorado. Together, Dr. Atkins and Dr. Glen Furuta developed the Gastrointestinal Eosinophilic Disease Program at Children’s Hospital Colorado as a multidisciplinary treatment center for pediatric patients impacted by eosinophilic gastrointestinal diseases. They discuss how treatments and medicines have developed over the years. The clinic started with local patients but now also receives referrals from around the United States. Listen in for tips on identifying EGIDs and using multidisciplinary treatment.
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 introduces the episode, brought to you thanks to the support of Education Partners Bristol Myers Squibb, GSK, Sanofi, and Regeneron, and co-host, Holly Knotowicz.
[1:19] Holly introduces today’s topic, the evolution of eosinophilic gastrointestinal disorders, and the guest, Dr. Dan Atkins, a pediatric allergist at Children’s
Navigating Eosinophilic Esophagitis in College with Kate Goncalves
Thursday, October 24, 2024 • Duration 34:24
Co-hosts Ryan Piansky, a graduate student and patient advocate living with eosinophilic esophagitis (EoE) and eosinophilic asthma, and Mary Jo Strobel, APFED’s Executive Director, interview Kate Goncalves about her diagnosis and treatment journey with EoE.
In this episode, Ryan and Mary Jo discuss with Kate Goncalves how she lived with her symptoms for years before finally bringing them to the attention of her primary care physician at age 16, and how she was connected with a gastroenterologist and diagnosed with EoE. Kate discusses her transition from pediatric to adult care, and how she and her care team prepared for her to travel to go to college. They talk about her treatment plan of eliminating milk and using medications, and how it is a challenge to navigate EoE during the long days on her college campus. Kate also talks about the EoE research project she is working on as part of her pre-med studies, and shares advice for people living with EoE and going away to 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:
[:49] Ryan Piansky introduces the episode, brought to you thanks to the support of education partners Bristol Myers Squibb, GSK, Sanofi, and Regeneron, and co-host, Mary Jo Strobel.
[1:31] Mary Jo introduces Kate Goncalves, a 20-year-old living with EoE. Kate is the 2024 recipient of The APFED Abbott College Scholarship, thanks to a grant from Abbott, the makers of the EleCare® brand elemental formula.
[2:05] Kate is from a small town in Connecticut. She is a junior at Vanderbilt University, studying biology and anthropology. She grew up with day-to-day symptoms of EoE but only expressed the symptoms to her doctor when she was 16 years old.
Treating EoE as an Allergist with Dr. Priya Bansal
Episode 38
Thursday, September 19, 2024 • Duration 55:10
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 living with EoE who serves on APFED’s Health Sciences Advisory Council, interview Dr. Priya Bansal about an allergist’s role in treating EoE.
In this episode, Ryan and Holly discuss with Dr. Priya Bansal her career in internal medicine, pediatrics, allergies, and immunology. She emphasizes the importance of patient advocacy and encourages parents to not accept the diagnosis that their child is a picky eater when the child is refusing food. Dr. Bansal talks about the process of reaching a diagnosis and EoE treatment options.
Listen to this episode for more information about living with EoE and how an allergist can 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:
[:51] Ryan Piansky introduces the episode and co-host, Holly Knotowicz. Ryan and Holly will talk about eosinophilic esophagitis (EoE) and how an allergist can help.
[1:25] Holly introduces Dr. Priya Bansal, an internist, pediatrician, and allergist, who is on the faculty of Northwestern Feinberg School of Medicine and practices at the Asthma and Allergy Wellness Center in Illinois.
[2:06] Dr. Bansal does research and consulting and in a private practice. Throughout her 20-year career, she has enjoyed treating chronic and difficult-to-treat diseases.
[2:03] Bethany began working with this disorder almost 20 years ago. She worked with Dr. Gonsalves and Dr. Hirano at Northwestern. Dr. Gonsalves invited her to work with EoE patients. Bethany had not heard of EoE.
[2:59] Bethany says the lens that we’ve used to look at food as the trigger and also a therapeutic agent in the esophagus, we’re looking at in non-EoE EGIDs as well; at the same time, trying to make sure that we’re honoring the other parts of our patient’s lives.
[3:27] Before Bethany started working in GI at Northwestern, she worked in the Wellness Institute, doing nutrition for patients at Northwestern. Bethany has a research background in epidemiology and she wanted to see better nutrition research in GI.
[3:56] Through a friend, Bethany connected with the Chief of GI at that point. Northwestern had never had a dietician working in GI.
[4:08] Bethany is pleased to see a trend in healthcare of thinking about the patient as a whole person, including diet, psychological wellness, physical health, exercise, sleep, and more. Bethany wanted to see more research on GI disorders.
[4:38] Bethany says that eosinophils in the esophagus indicate that something is irritating the tissues, such as reflux, food triggers, aeroallergens, and other things.
[4:58] Eosinophils do belong in the stomach, the small intestine, and the colon. The challenge for researchers has been, how many, where are they supposed to live, and what are they supposed to look like.
[5:10] There is eosinophilic gastritis, where eosinophils can infiltrate the stomach, causing a lot of inflammatory responses that make patients sick. We see that in all parts of the small intestine and less commonly, in the colon, as well.
[5:32] It’s a good reminder for listeners that eosinophils are white blood cells. When they’re in the tissues, they can swell things up and cause the body to have this inflammatory response in these lower GI tract organs.
[5:49] The symptoms patients can experience are vomiting, diarrhea, and abdominal pain, among other things.
[6:14] The nomenclature for this subset of eosinophil-associated diseases has changed and Bethany says to hang tight, there is lots of work underway to nail this down further in the next couple of months to a year.
[6:29] The last guidelines were published by a Delphi Consensus in 2022. The experts in the field got together and voted on the scientific accuracy of certain statements to develop cut points for how to grade.
[6:48] The experts are asking questions like: What counts as eosinophilic gastritis? What do we think are some of the symptoms and the clinical findings so that we all are looking at things through the same lens?
[7:02] To get to these consensus statements, there’s a lot of discussion, agreement, and good collegial discussions about making sure that we’re looking at this accurately.
[7:12] We’re trying to give the right names to the right disorders and give clear diagnostic criteria, so that we’re helping our patients get a diagnosis, and we’re not labeling something incorrectly and sticking someone with an inaccurate diagnosis.
[7:36] The proper terminology is eosinophilic gastritis in the stomach, eosinophilic enteritis in the small intestine, eosinophilic colitis in the colon, and eosinophilic gastroenteritis where the stomach and the small bowel are involved.
[7:53] There’s more to come on the clinical criteria of what makes that diagnosis but we’re getting the names and the numbers right.
[8:03] Holly agrees that having the symptoms given a named diagnosis is important to patients, knowing that researchers are looking into their illness.
[9:00] Bethany notes that the diagnosis also means that there are opportunities for medical therapy, cut points for which medicines or therapies work or not, and billing codes. If we can’t bill insurance companies, patients might not get certain services.
[9:28] Ryan tells how beneficial it was for him to have access to multi-disciplinary teams and see specialists he might not have seen without the proper diagnosis and just thought it was a GI issue. He was fortunate to see a dietician and start dietary therapy.
[9:53] Bethany says the dietician’s priority is the patient’s health and wellness.
[10:13] These disorders carry clinical non-gastrointestinal manifestations: fatigue, concern over what to eat, food access issues, family support, and other food allergies. These are important things for a dietician to consider.
[10:37] Are patients growing as they should? Do they feel like they have enough to eat? Do they feel excluded in social settings? There’s a list of important things that we want to be looking at. That’s why it’s important to have a multi-disciplinary approach.
[11:07] First, Bethany wants to see that her patients are physically and nutritionally well. That’s a priority if we’re going to try to get rid of some of the food triggers that could be exacerbating the disease.
[11:20] Before Bethany takes anything out of someone’s diet, she wants to make sure that they’re getting enough of the good stuff to help them feel good and grow.
[11:29] From a diet therapy perspective, Bethany is trying to apply a food removal or substitution protocol to other spots outside the esophagus. They’re seeing that some of the triggers are very similar, both in the stomach and small intestine.
[12:09] Dr. Gonsalves, Dr. Hirano, and Bethany did a study, The Elemental Study, where they wanted to uncover if food proteins carried the same trigger risk in the stomach and small intestine as they do in the esophagus.
[12:35] They put their patients on a hypoallergenic elemental formula for a period, followed up, and looked at their biopsies of the stomach and small intestine. Fifteen wonderful patients made it through the trial.
[12:56] One hundred percent of the patients achieved disease remission and felt better. There were some genetic alterations in the patients. Then they started the process of reintroducing foods over the year.
[13:15] That was not part of the original grant but was the team’s clinical interest to see what it is that people are allergic to. Some of the common suspects: wheat, dairy, eggs, soy, and nuts, were found to be very common triggers for EoG and EoN, as well.
[13:47] The benefit of working with a dietician as part of your team is, first, we can remediate things the disease has caused nutritionally, and second, we can think about how diet can be a therapeutic tool to use with medications or instead of medication.
[14:15] If you want to use nutrition therapeutically, you don’t have to stay there if it’s not the right time to be taking things out of your diet. We have some good, safe, medical therapies. You can find your food triggers but you don’t have to pick that lane forever.
[14:42] Holly and Ryan relate their experiences with traveling abroad and going on medical therapies when they can’t stay on their diets.
[15:57] Bethany says low levels of vitamins and minerals in the blood can be caused by a disorder or an elimination diet. In the U.S., dairy is the biggest source of protein for young kids. It’s also the biggest source of calcium and vitamin D.
[16:22] Dieticians often say, if we are going to use dietary therapy for EoE or non-EoE EGIDs, we have to think of this as a substitution diet. If we remove something, we have to replace it with something equally nutrient-dense.
[16:39] Bethany and her group look at serum values of Vitamin D, B12, and iron they assess for patients. For kids, instead of drawing blood, they piece together what they’re taking against what they need and see if there are gaps to fill with food or supplements.
[17:32] In patients with non-EoE EGIDs, Bethany says we see the disease intersect with the food supply. When we take milk out, we’re cutting the biggest source of calcium and Vitamin D. We have to replace calcium and Vitamin D.
[17:55] In the 1950s, a public health law allowed wheat to be enriched with folic acid and other B vitamins and iron. When we cut out wheat, our patients aren’t getting enough iron or B vitamins. We have to replace those.
[18:16] For patients who have eosinophils in their stomach and small intestine, their absorption in the small bowel may be directly impacted.
[18:26] People can have low levels of protein in their blood, maybe because they’re eating insufficient protein or maybe because the disease doesn’t allow them to absorb protein sufficiently when there’s swelling in the small intestine.
[18:44] There are other nutrients, like zinc, for people who have diarrhea, and magnesium if you can’t eat a lot of whole grains and nuts, There are quite a few nutrients that Bethany is broadly looking at.
[18:54] Based on the absorption in the small intestine, patients’ doctors need to look at their B12, folic acid, iron levels, and Vitamin D.
[19:12] Holly loves Bethany’s terminology of replacing, not just eliminating, foods. She will use that terminology with her patients to make it feel more supportive for them.
[20:40] A lot of people want to get all their nutrients through their food. That’s not always practical. Vitamin D is hard to get exclusively in your diet if you’re not drinking milk or eating wild-caught fish. You have to rely on fortified foods or add supplements.
[21:15] One, we want to take a look at your diet and ask how are your calories. We want to make sure you’re eating enough. Two, if we suspect there are some vitamin deficiencies, we check your blood or just empirically supplement you.
[21:36] Supplementation should be done carefully. There are some vitamins where you can get too much of a good thing. Vitamins stored in the fat need to be at levels sufficient for repletion, dictated by age and gender. Dieticians know what to recommend.
[22:19] For patients who have non-EoE EGIDs, some have tentative swallowing, so Bethany tries to do as many liquid or chewable safe options for supplements as possible.
[23:46] Holly works with patients who have feeding difficulty, so she appreciates the liquid and chewable supplements for easier swallowing and quicker absorption.
[24:08] Bethany mentions that some fortified oat, corn, and rice breakfast cereals are highly enriched with B vitamins and iron. Look at the labels. It can be a way to layer in more vitamins without purchasing a supplement.
[25:24] Holly doesn’t think patients understand how valuable a good dietician can be. She had one patient with celiac who was taking a supplement with gluten in it! She reminds listeners to always consult your care team before making any changes to your treatment plan.
[25:59] Bethany’s favorite thing to talk about is foods and where to find what. If listeners have questions, she is happy to post answers on the website.
[26:25] The American Academy of Pediatrics says a cup of vitamin-fortified juice a day is not too much sugar and is a good source of Vitamin C and other nutrients. The calcium and Vitamin D you get from a cup of fortified juice is very value-available.
[26:46] In the non-dairy drink world, some are nicely fortified and some are not. If you make your almond milk, you’re missing out on the fortifications.
[27:11] Bethany likes some of the fortified juices and some of the enriched non-dairy milk options. Those are the best ways to get calcium and Vitamin D for people who need calories. Instead of water with meals, substitute an enriched drink with meals.
[27:33] Some people struggle with protein, probably because of their level of food restriction. The typical animal proteins are great. If you can do soy, a cup of soy milk has eight grams of protein. Soy is a complete protein that mimics animal proteins.
[28:04] Cook your cereal in soy milk. Use it as the base of a smoothie. This is before getting into protein powders. Try legume-based proteins, if you can handle legumes. Your supplements have to be personalized. That’s the tricky part.
[28:30] If you have a lot of food allergies or intolerances, it may be worth talking to your gastroenterologist, allergist, or dietician about adding elemental formula as a supplement. Bethany uses it often with food allergy patients as a safe supplement.
[29:31] Bethany primarily treats adults but also young adults transitioning from the pediatric side into the adult world. Sometimes a feeding difficulty follows patients into adult treatment. We need everyone at the table to treat this immune-mediated disease.
[30:32] Patient advocacy groups like APFED have ways to help you find dieticians. Also, the Academy of Nutrition and Dietetics has “Find a Specialist” on their website. Eatright.org. Dieticians can do telehealth if you are not near one.
[31:45] If the practice that you’re in doesn’t have a dietician, you could gently suggest they have one join the practice, or consult with the practice. Patient advocacy is strong.
[33:12] Bethany talks about getting an appointment with a dietician. On the pediatric side, it has to do with the billing code. Ask your insurance if they cover medical nutrition therapy, Billing Code 97802, and for which diseases. Insurance may have stipulations.
[34:14] If medical nutrition therapy is not a covered benefit, ask the dietician if they can do a sliding scale. Holly says she has seen plans in several states where the patient can use the HSA or FSA card to pay for medical nutrition therapy.
[34:49] Bethany believes in the pediatric world, where growth and development are concerns, there’s a little bit better coverage.
[34:59] On the adult side, if Bethany has other diagnoses, like high blood pressure, or diabetes, she is also billing for those because she makes sure what she recommends is also in line with what is good for their heart and wellness in general.
[35:55] Bethany was intrigued to learn food proteins do trigger disease activity for our patients in the stomach and small intestine, just as in the esophagus.
[36:20] In the Elemental Trial, they were surprised to learn people with non-EoE EGIDs had more allergies than expected. They were more likely to have more than just one or two. They were also more likely to have rare food allergies like legumes or grains.
[36:43] A patient may want to learn all their food triggers, but they may be a highly allergic person and it may not be worth trying to remove all their food triggers.
[37:06] Bethany wants to remind listeners that the diet approach should be a substitution diet. If you take things out, you’ve got to replace them with other plants.
[37:18] There’s great crossover nutrition between fruits and vegetables. Seeds are great as a fill-in for nuts. There are plenty of other whole grains out there besides wheat. There are lots of good ways to get that nutritional balance into your diet.
[37:31] For anyone who’s eliminating a food group, even if you’re substituting it, it’s a good idea to talk to your doctor about filling in with a good multivitamin, multimineral supplement.
[37:59] Bethany says it’s fun working with colleagues to look for other ways to look at this nutrition lens for patients with Non-EoE EGIDs.
[38:14] They are looking at noninvasive ways to find eosinophils to go faster with helping people find their food triggers without having to scope them.
[38:28] Bethany is hoping with that research to be able to help people learn how they can cheat, like having pizza once a month if they are allergic to dairy. That’s a question for your care team, but we don’t have a great science-based way to answer that.
[38:53] As we study more noninvasive ways to get at eosinophilic activity, we can give patients a little bit more freedom and quality of life. That’s what Bethany is working on next.
[39:58] Holly thanks Bethany Doerfler for joining us on Real Talk — Eosinophilic Diseases. For our listeners, to learn more about eosinophilic disorders, please visit APFED.org and check out the links in the show notes.
[40:11] If you’re looking to find specialists who treat eosinophilic disorders, we encourage you to use APFED’s Specialist Finder at APFED.org/specialist.
[40:21] 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.
[40:34] Holly thanks Bethany for joining us today. Holly also thanks APFED’s Education Partners Bristol Myers Squibb, Sanofi, Regeneron, and Takeda for supporting this episode.
“The lens that we’ve used to look at food as the trigger and also a therapeutic agent in the esophagus, we’re doing that in non-EoE EGIDs as well, and at the same time, trying to make sure that we’re honoring the other parts of our patient's lives.” — Bethany Doerfler, RD
“We are trying to give the right names to the right disorders and give clear diagnostic criteria so that we’re helping our patients get a diagnosis, and we’re not labeling something incorrectly and sticking someone with a diagnosis that isn’t accurate.” — Bethany Doerfler, RD
“The diagnosis also means that there are opportunities for medical therapy, cut points for which we decide if medicines or other therapies work or not, and billing codes. If we can’t bill insurance companies, patients may not be privy to certain services.” — Bethany Doerfler, RD
“Look at the [fortified cereal] labels. You’d be surprised how much they look like a multivitamin, not only for B vitamins but for iron. … It can be a fantastic way to layer in more vitamins without having to think about purchasing a supplement.” — Bethany Doerfler, RD
“There’s great crossover nutrition between fruits and vegetables. Seeds are great as a fill-in for nuts. There are plenty of other whole grains out there besides wheat. There are lots of good ways for us to get that nutritional balance into your diet.” — Bethany Doerfler, RD
[2:09] Dr. Ruffner also leads a research group that studies how the immune system causes inflammation in response to certain foods, leading to EoE.
[2:20] Inflammation in the esophagus is tied to other diseases like epithelial barrier dysfunction and fibrosis.
[2:28] Our bodies use many different proteins that allow cells to communicate with one another. One type of signaling protein that causes inflammation is called cytokines.
[2:41] Dr. Ruffner’s group is interested in how these signaling proteins called cytokines interact with epithelial cells and how that impacts the oral function of the esophagus in patients with EoE.
[3:02] In training, Dr. Ruffner became interested in eosinophilic esophagitis and other non-IgE-mediated food allergies because we don’t have a lot of clear treatments or clear mechanisms that cause them.
[3:21] Dr. Ruffner felt there was a lot of work to be done in that area. It was rewarding to be in clinical encounters with those patients. Often, patients had spent a long time trying to find out what was happening and to find a treatment plan that worked for them.
[4:31] Dr. Ruffner’s group sees some patients who have eosinophilic gastroenteritis and patients who are referred for hypereosinophilia with impacts of inflammation in other organ systems.
[5:06] Dr. Ruffner co-authored a paper about pediatric and adult EoE published in the Journal of Allergy and Clinical Immunology. It explored if EoE in pediatric patients and adult patients is a spectrum or distinct diseases.
[5:29] EoE is a chronic allergic condition that affects the esophagus. The esophagus carries food from the mouth to the stomach. In people with EoE, the immune system overreacts to foods and causes inflammation in the esophagus.
[5:47] Eosinophils are a type of white blood cell. Eosinophils infiltrate the tissue in the esophagus of people with EoE. Doctors look for eosinophils in the tissue of the esophagus as a sign that inflammation in the esophagus is EoE.
[6:04] The symptoms of EoE can vary in children and adults. That was one of the things the doctors were interested in when they were thinking about this paper. There are no blood or allergy tests that make it easy to diagnose EoE, which requires an endoscopy.
[6:31] An endoscopy is performed by a gastroenterologist. The gastroenterologists look at the appearance of the esophagus and take biopsies.
[6:49] A pathologist counts the eosinophils in the tissue to determine if there are eosinophils present. If there are more than 15 eosinophils in the high-powered field of the microscope and symptoms and clinical conditions are present, EoE is diagnosed.
[7:25] One of the variables Dr. Ruffner considers is that symptoms can be different in children versus adults. In older adolescents and adults, the classic symptom is difficulty swallowing or dysphagia. That is often caused by fibrosis in the esophagus.
[7:54] In younger children this is often not how EoE presents. They may vomit or refuse food. They may experience more weight loss. Symptoms vary over the lifespan. Pediatric EoE symptoms of nausea and abdominal pain can also show up in adults.
[9:54] Atopy refers to allergic conditions. In the paper, a history of atopy means a history of allergic conditions, like atopic dermatitis, IgE-mediated food allergy, allergic rhinitis, or asthma.
[10:37] These disorders tend to cluster together, over time, because they share many common genetic risks. They cluster in families because some of the genetic risks are the same. Not every family member will have the same atopic or allergic conditions.
[11:07] In families, perhaps one person will have atopic dermatitis and allergic rhinitis while another will have atopic dermatitis, allergic rhinitis, asthma, and EoE. They may have inherited different genetics or had different environmental exposures.
[11:50] Ryan says that describes his family. They each have different atopic conditions. Ryan got them all! Dr. Ruffner says it describes her family, as well.
[12:26] Dr. Ruffner says it’s understandable for families to stress about atopic conditions. Unfortunately, right now, there’s no way to predict who will develop which atopic conditions. It’s on the minds of the medical and research communities.
[13:10] IgE is an antibody that binds to food allergens and mediates anaphylaxis, usually within 30 minutes, with hives, vomiting, and difficulty breathing. Not everyone with a diagnosed food allergy will be given an epinephrine auto-injector.
[13:44] IgE-mediated food allergies are influenced by type 2 cytokines. Cytokines are immune system signaling proteins that have been labeled as groups. The group that is involved in allergy most heavily is under the label type 2.
[14:15] These type 2 cytokines are responsible for influencing B cells to make IgE. In the tissue in EoE, we find that there is a large amount of these type 2 cytokines present.
[14:37] This is quite relevant because dupilumab, the monoclonal antibody that has been approved to treat EoE, targets type 2 inflammation by blocking type 2 cytokines.
[16:04] Dr. Ruffner says one of the biggest challenges in the field of EoE is we don’t have a way to stratify who should get which treatment for EoE. Patients have to choose between diet and pharmacologic therapy.
[16:48] We don’t know enough about the inflammatory profiles to give any patient the specific guided information that one therapy would be better than another.
[17:11] Pediatric and adult patients are given the same treatment options. Some dosing, such as proton pump inhibitors and dupilumab, is weight-based so different doses are needed.
[17:36] Over time, people’s needs change. From early school age to when people leave home, they may have very different needs. They may do well on diet therapy when their diet is controlled by parents, but, on their own, that may not be the best option for them.
[18:20] Therapy may change over time to support each patient’s individual goals. It can be challenging because therapies are imperfect. Each therapy has a percentage probability of success. Not every therapy is guaranteed to work for every individual.
[19:01] There is some flexibility and possibility of switching between therapies to support people. Ryan shares one of his experiences in changing treatments.
[20:03] Some patients are stable on a therapy for a time but then see symptoms creep back up. Dr. Ruffner strongly suggests they talk to their care team for an endoscopy and biopsy to see if they need to switch therapy and if their diet has changed.
[21:31] In young children, Dr. Ruffner sees a much higher incidence of feeding refusal. The child may have a preferred food or a preferred texture like puree, long past when that would be appropriate for the age.
[22:41] It can be very difficult to move past this learned behavior even if remission is achieved through therapy. The child may need feeding therapy to help with that.
[22:59] Feeding behaviors in older individuals may be much more subtle. Talk about them with your care team. Needing water to eat, cutting food very small, and fearing to eat around people are common eating behaviors to discuss in older patients.
[23:53] These eating behaviors affect people’s well-being deeply because they affect how social they feel when they are around people. Ideally, you want to be around people and share in social times.
[24:16] Holly has used these eating behaviors herself and notices them in other people. When adults come to her for therapy, she asks how many times they refill their water when they eat, and if food ever gets stuck. They are surprised that those are symptoms.
[26:01] Dr. Ruffner says it’s important to recognize the difference in symptoms in diagnosing EoE. The main risk factor of EoE is fibrosis, over time. The thought is that early in EoE there is an inflammatory phenotype, but later, there is a fibrotic phenotype.
[26:51] The phenotype refers to the presentation or characteristic of disease. What is the appearance at endoscopy? What do we see in the biopsied tissue? Is there fibrosis or not?
[27:15] This is the crux of the paper: Is this on a spectrum, that the inflammation is driving the fibrosis, or are these two different things altogether? There is some evidence to suggest that the inflammation contributes to this fibrosis over time.
[27:40] One thing that is missing is following a group of patients from the start and having that evidence. There is mechanistic evidence from studies to show that inflammation can contribute to fibrosis. That was one of the discussions in the paper.
[28:29] In endoscopies, something that can be seen with fibrosis or fibrostenotic features is more of an appearance of rings and narrowing of the esophagus. A proportion of patients with strictures or narrowing need to have them dilated.
[29:11] For patients who have dilation, it can help with symptoms significantly. When pathologists look at the tissue with fibrosis, they can see changes in the protein structure. There is more collagen and other changes in the tissue, causing fibrosis.
[30:03] Some patients use adaptive eating behaviors to adapt to significant changes in their esophagus and go for many years without being diagnosed until they present with an impaction when food becomes stuck in their esophagus.
[30:46] This makes EoE a challenging disorder for many because it can be very difficult to diagnose. The journey to a diagnosis is very individual. As a group, adults are much more likely to have fibrosis, leading to dysphagia, strictures, or impaction.
[31:25] Statistically, across all patients, you see fibrosis more in adults than in children.
[32:42] In the paper, Th1 cells are mentioned. Th1 is an immune system term referring to a cell that produces interferon-gamma. Studies show there may be differences in interferon signaling in different age groups but it needs to be studied further.
[33:57] Dr. Ruffner’s team had looked at a small group and saw that interferon signaling seemed to be relatively similar between children and adults. Both CD4 and CD8 T cells (types of immune system cells) are potentially producing interferon in the esophagus.
[34:32] More study needs to be done around those immune system cells and their potential significance in EoE, if any.
[35:33] The paper suggests that EoE in children and adults is essentially a spectrum of the same disorder rather than distinct diseases.
[35:42] Aspects of immunology, responses to different treatments across children and adults, the similar responses to diet and different medications, and over time in the same individuals, indicate these are changes and complications over time.
[36:41] Dr. Ruffner suggests that medical researchers need to understand which patients are at the highest risk of complications and work to identify the best treatments to prevent those.
[37:14] Dr. Ruffner is thinking about the response to proton pump inhibitor therapy. One of the things she is looking at is whether or not proton pump inhibitors affect how eosinophils migrate into the tissue.
[37:33] They are finding that it seems that PPIs can decrease the degree of migration of eosinophils into the tissue. They are very interested in looking at that. Ryan says when Dr. Ruffner gets that paper published, she’ll have to come back on the show!
[38:06] Ryan thanks Dr. Ruffner. For our listeners who would like to learn more about eosinophilic disorders, including EoE, please visit APFED.org and check out the links in the show notes.
[38:15] If you’re looking to find a specialist who treats eosinophilic disorders, we encourage you to use APFED’s Specialist Finder at APFED.org/specialist.
[38:24] 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.
[38:33] Ryan thanks Dr. Ruffner for participating in the podcast episode. Holly also thanks APFED’s Education Partners Bristol Myers Squibb, GSK, Sanofi, Regeneron, and Takeda for supporting this episode.
“EoE is a chronic allergic condition that affects the esophagus. The esophagus carries food from the mouth to the stomach. In people with EoE, the immune system overreacts to food allergens and causes inflammation in the esophagus.” — Dr. Melanie Ruffner
“In EoE, there are no blood or allergy tests that make it easy to diagnose EoE without an endoscopy.” — Dr. Melanie Ruffner
“Is EoE on a spectrum, that the inflammation is driving the fibrosis, or are these two different things altogether? There is some evidence to suggest that the inflammation contributes to fibrosis over time.” — Dr. Melanie Ruffner
“When pathologists look at the tissue with fibrosis, they can see the changes in the protein structure.” — Dr. Melanie Ruffner
“There are some folks who have adapted their eating behavior quite significantly and may have quite a number of chronic changes in their esophagus that they have adapted around, and they go for many years without being diagnosed.” — Dr. Melanie Ruffner
[2:23] Dr. Accarino grew up with allergies. He has experienced food allergies since he was young, along with allergic asthma, and some eczema, which he grew out of. Later in life, he was diagnosed with eosinophilic esophagitis. He talks with his patients about his experiences.
[2:47] Dr. Accarino also does research on drug allergies in the context of certain drug interactions that involve eosinophils.
[3:06] When Holly was referred to Dr. Accarino, it was for multiple sclerosis (MS). He told her, “It looks like you have EoE. I have EoE.” It was a huge relief to Holly not to have to explain EoE to her doctor.
[3:41] Some patients start to explain their EoE to Dr. Accarino, and he assures them he understands where they’re coming from. Sometimes, he has to be careful not to think everyone has his symptoms, as there is a large spectrum of presentations.
[4:26] Dr. Accarino wasn’t diagnosed with EoE until he was in his allergy fellowship, after he suspected it when he had a food impaction at a steakhouse at a graduation party from his pediatric residency. He tried to manage the EoE with lifestyle changes.
[5:39] Dr. Accarino didn’t often go to see a doctor during residency, but he realized it was probably a good time to get an endoscopy.
[5:52] Holly shares how she was also diagnosed as a clinical fellow. She was subbing for someone on the GEDP team at Children’s Hospital in Colorado. Listening to all the patients, she realized, “This sounds a little bit like me … What is going on?”
[6:23] Even with his medical background, it took Dr. Accarino some time to decide to get the endoscopy and biopsies. You or your doctor have to have a high level of suspicion to realize this isn’t just reflux. Food doesn’t get stuck in every person’s throat.
[7:01] Thinking back, Dr. Accarino remembers an instance as a child when a dry muffin got stuck in his throat. He stayed calm and waited for it to pass. He thought it was normal.
[7:39] He drank a lot of water and chewed his food a lot. Those are markers of potential esophageal inflammation.
[8:20] Different groups have different management strategies for EoE. Dietary management, topical steroids, biologics. A subgroup of people with EoE are responsive to proton pump inhibitors (PPIs). Finding the best management strategy is a work in progress.
[8:53] With pediatric patients, the parents control the diet, and the children eat what is prepared. He notes that with adult patients, sometimes they let foods slip through.
[9:10] If you want to do a single-food elimination diet with dairy, there’s a lot of dairy in the American diet. Dr. Accarino tried eliminating dairy and wheat, but he still had persistent eosinophils with dietary elimination.
[9:24] Dr. Accarino then tried PPIs. To know if you have PPI-responsive EoE, you might do twice-daily omeprazole at a significant dose. Have the endoscopy after a few weeks pass and see if the eosinophils are still present in the biopsy.
[9:59] Dr. Accarino did that recently and still has the eosinophils. He plans to talk to his gastroenterologist about considering dupilumab, but he feels that he can mitigate his subjective day-to-day experience of symptoms with dietary elimination and PPIs.
[10:24] If you still have the presence of eosinophils on biopsy, there’s still inflammation happening. In the long term, you still have to worry about fibrosis and narrowing.
[10:34] The last treatment Dr. Accarino tried was as a research participant in a study for dissolvable fluticasone. He received either the medication or a placebo; he doesn’t know which.
[11:01] To stay in the study, he had to journal and report his symptoms regularly. He didn’t have enough symptoms to stay in the study. They were looking for a baseline to see how it changed with either the placebo or the medication.
[11:20] In research, you have to have a baseline to start, and then you want to see improvement, plus or minus. With EoE, it’s difficult. You have the biopsy and eosinophils, but there’s a large spectrum of symptoms that people may experience.
[12:40] Holly appreciates Dr. Accarino’s unique perspective as a doctor with EoE who has experienced various treatments and diets. He understands the concerns of his patients.
[12:43] Dr. Accarino says even taking a twice-daily PPI or other medication is difficult for a lot of people, and that’s the most simple of these therapies.
[13:06] Dr. Accarino wants to validate everyone’s experience in terms of how difficult it is to treat this disorder, how it may present in different ways, and how there may be a delay in diagnosis.
[13:16] This isn’t IgE-mediated immediate food allergy, where you eat a food and may have swelling within minutes; you may have flushing or hives. That’s very clear. With EoE, it’s a different mechanism; in many cases, there is a delay.
[14:37] Allergy, in general, is under the purview of clinical immunology. Dr. Accarino is allergic to peanuts and has an IgE-mediated immediate reaction to them. If he eats a peanut, he has symptoms within minutes. He could have anaphylaxis. As a result, he carries an epinephrine auto-injector.
[15:01] If Dr. Accarino has a skin test, it will be positive for peanut. He has IgE antibodies to peanuts. He also has oral allergy syndrome where the body mistakes certain fruits, vegetables, or nuts with certain tree pollens or grass pollens.
[15:23] Oral allergy syndrome is usually a lower-risk condition where it’s a less-stable protein that once cooked might not produce any symptoms. If it’s raw when you consume it, you may have oral itching, a bit of throat discomfort, or tongue itching.
[15:54] Your stomach acid breaks it down so it doesn’t get into your bloodstream and you shouldn’t have a systemic reaction.
[16:01] If Dr. Accarino eats a peanut, his stomach acid doesn’t break down the high-risk, stable peanut protein, it gets into his bloodstream, and he can have a systemic anaphylactic reaction.
[16:20] Chronic EoE symptoms can present with something like a food impaction, or bad reflux or belly pain, and nausea. The reaction may not be immediate. It may be progressive over days or weeks.
[16:38] FIRE is an interesting condition that takes some time to narrow down. It’s an immediate response of the esophagus, but we don’t think it’s histamine-mediated.
[16:56] We don’t know, exactly, the mechanism but it’s in people with eosinophilic esophagitis. They feel differently, and there would be different specific food triggers.
[17:11] It took some time to figure out what was going on. Dr. Accarino felt like he had a lump in his throat, then a lump in his chest, nausea, and belly pain. It felt like a slow progression of EoE symptoms, and it was from specific food triggers, in his case.
[17:30] In some of the FIRE literature, they looked at banana and avocado. For Dr. Accarino, it took a couple of exposures to protein bars and milk protein whey isolate, specific to protein bars he had multiple times, until he figured out that was the trigger.
[17:50] Another protein whey isolate that Dr. Accarino scooped as a powder and made into a shake also led to FIRE.
[17:55] It took that event for Dr. Accarino to figure out it wasn’t just a flareup of EoE or reflux but some trigger that caused this response that wasn’t anaphylaxis but may be due to the recruitment of eosinophils or some immediate process not well understood.
[18:18] FIRE is going to be very hard to research. How would we figure this out? Would we bring someone in and do an endoscopy immediately and see what happens? There’s a lot of descriptive data and case series.
[18:32] Dr. Accarino has had experiences when he knew it wasn’t an immediate anaphylactic reaction, oral allergy, or reflux. He asked what else it could be in the context of EoE. When he looked at different case series, that’s the presentation he had.
[19:17] Dr. Accarino acknowledges that having personal experience with FIRE, oral allergies, and IgE-mediated allergies, on top of EoE, has influenced his work as a medical professional. He can share anecdotes with patients as he explains the available testing.
[19:39] Dr. Accarino says a lot of immunology and allergy is explaining the diagnostic tools and management strategies we have and what we think is going on.
[19:50] The immune system is infinitely complex, and a lot of the practice is making a digestible analogy, not just in the context of allergic conditions but also everything with the immune system. There are so many cells doing so many different things.
[20:04] Dr. Accarino explains false positives in testing. He has positive scratch tests for peanuts, cashews, and almonds, which shows he has IgE for each of them. He is allergic to peanuts, but he can eat cashews and almonds. Those are false positives.
[20:56] When a scratch test is negative for immediate food allergy, it’s a powerful predictive tool. But you may get false positives. How positive is it? There might be room for more discussion.
[21:10] There may be more hesitation for people who do large panels of food testing without any history of reacting to any foods.
[21:31] Some people have EoE triggered by milk or wheat but have negative skin tests. That doesn’t mean they aren’t triggered by these foods. The skin test is an IgE histamine mast cell mechanism, not for eosinophils, which are other immune cells.
[21:58] We go down these steps of thinking about diagnostic triggers and eventually treatment for those immediate symptoms mentioned for EoE.
[22:09] Dr. Accarino doesn’t expect FIRE to be responsive to epinephrine. He doesn’t have to stabilize the mast cells. It’s a chronic disease that’s flaring up. You treat it with a chronic type of treatment.
[24:10] Dr. Accarino says that for a doctor, immunology is rewarding, interesting, and complex, but it’s intimidating until you get your foothold and see patients and clinical experiences.
[25:14] A lot of medical students and residents are a little fearful of immunology. They might not think about it too much. Dr. Accarino loves to talk about it and think about it. He can’t think of anything more complex in terms of systems within our body.
[25:37] Ryan comments on his experiences with IgE-mediated food allergies, some environmental allergies that he has no idea how they work, and EoE, which he believes he has a good grasp on.
[25:55] Ryan imagines that having a physician with a good understanding of the immune system and also personal experience would be helpful for a patient with multiple allergic conditions.
[26:13] Dr. Accarino sees a large overlap of seasonal or year-round environmental allergies and EoE. There are some studies that show that endoscopies on patients with EoE may change at different times of the year if they have underlying seasonal allergies.
[26:33] Some people who have food allergies also have EoE or other eosinophilic disorders. Some discussions with them may be about blood tests that detect eosinophils in the bloodstream versus biopsies of the esophagus, stomach, or colon.
[27:15] It’s thinking about what tests are available, what they tell us, and how to use them to predict the next steps, things like dietary changes or for immediate food allergy, considering challenges versus full avoidance. Each test has its pluses and minuses.
[27:35] People like a clear test, and they like an easy fix, but sometimes there’s a lot of nuanced conversation of shared decision-making and trying things in a supervised setting.
[27:57] Holly speaks as a patient of the investigative testing Dr. Acarino is doing with her immune system trying to figure it out along with her MS and EoE.
[28:14] Dr. Accarino says the words immune system, immunity, and inflammation are used a lot in talking about foods. Dr. Accarino uses the framework of the immune system trying to help you.
[28:42] Sometimes, instead of making helpful antibodies to things like vaccines or viruses, that give you protection, the immune system makes antibodies that attack a certain organ or your joints.
[29:02] Dr. Accarino thinks of treatments that suppress the immune system in certain ways. Some treatments cool down the populations of many different immune cells. Oral steroids and prednisone are used for many conditions for autoimmune flares.
[29:29] Oral steroids, in the long term, may lead to weight gain, bone density changes, and diabetes. The big push for many diseases is toward non-steroidal biologics to target specific cells that cause disease.
[29:59] For Crohn’s disease, a specific monoclonal antibody is used to target TNF-alpha molecules and blocks that inflammation pathway.
[30:14] For EoE, dupilumab, a specifically designed antibody, blocks a specific receptor in a specific pathway so the immune system doesn’t have to be shut down and the patient doesn't have the side effects of steroids. It’s a targeted therapy.
[30:32] What you see in commercials for injectable medications are large, designed antibodies that, if you took them in a pill form, your stomach acid would break down and digest. So they are injections and infusions that go directly into the bloodstream.
[31:22] Medications that end in -mab are monoclonal antibodies. They are very large molecules that would not be stable in stomach acid.
[32:09] Dr. Accarino talks of eosinophil normal function and aberrant function. IgE-mediated reactions are usually related to mast cells, a type of immune cell that shouldn’t be in the bloodstream.
[32:54] Dr. Accarino can do a CBC with differential to see the number of white blood cells and the number of red blood cells. The differential of white blood cells will include neutrophils, lymphocytes, and eosinophils. It shouldn’t show mast cells.
[33:19] If you have mast cells in your bloodstream, that’s mastocytosis, a different problem. Mast cells live in your skin, in your gut, and around your blood vessels. They’re full of granules like histamine and tryptase.
[33:38] Dr. Accarino explains how mast cells release their contents and how he would treat the resulting swelling or itch with an antihistamine or epinephrine. Epinephrine treats systemic reactions and stabilizes the mast cells.
[34:16] Mast cells have many receptors and may be triggered by many things other than IgE. This is a conversation Dr. Accarino has with patients who have chronic hives unrelated to any foods.
[34:29] Some people get hives from non-steroidal anti-inflammatory drugs NSAIDs. Some get hives from vancomycin. Some get hives when the temperature changes, from tight clothing, or from IV contrast. It’s not an IgE-mediated mechanism, but it’s still mast cells being degranulated.
[35:45] Dr. Accarino says people see hives and they think allergy. But, like EoE, it doesn’t involve histamine. There can be hives that aren’t related to allergies. This can be idiopathic urticaria or spontaneous urticaria.
[36:04] Sometimes, when switching from a day shift to a night shift, hormonal changes will trigger hives. Sometimes, the stress of having a family member in the hospital will cause hives. An accumulation of triggers can lead to mast cell degranulation.
[36:38] There are many ways that allergy can have different mechanisms and treatments, with different cells involved. There are different molecules that cause symptoms and manifestations.
[36:50] Navigating that and understanding what might be going on can give people a sense of reassurance. The biggest fear is a life-threatening allergic reaction. People will read about fatal anaphylaxis and wonder if it will happen to them with their condition.
[37:16] Sometimes, thinking of the cells involved and the pathways may give a level of reassurance that this may not be the same thing that they read about.
[37:28] Ryan thanks Dr. Accarino for joining us today.
[37:37] Dr. Accarino says it was nice to reflect on things and to go through different scenarios and experiences he has gone through. It was nice to have the opportunity to share them with Ryan, Holly, and all the listeners.
[37:57] For our listeners who would like to learn more about eosinophilic disorders, including EoE, please visit APFED.org and check out the links in the show notes.
[38:06] If you’re looking to find a specialist who treats eosinophilic disorders, we encourage you to use APFED’s Specialist Finder at APFED.org/specialist.
[38:15] 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.
[38:25] Ryan thanks Dr. Accarino for joining us today for this fun conversation. Holly also thanks APFED’s Education Partners Bristol Myers Squibb, GSK, Sanofi, and Regeneron for supporting this episode.
“Allergy and immunology is a field where I can see pediatrics and adults. I was originally trained in pediatrics, but now I see all ages, from infants up until older adults.” — Dr. John Accarino
“Part of the conversation sometimes is trying not to overly bias myself, where I say, ‘Oh, this is my experience.’ … Like many diseases, there’s a large spectrum of presentations, … different symptoms that people have.” — Dr. John Accarino
“We don’t think [Food-Induced Response in Eosinophilic Esophagitis is] histamine-mediated. We don’t know exactly the mechanism, but it’s in people with eosinophilic esophagitis. They feel differently, and there would be different specific food triggers. It took some time to figure out that was going on.” — Dr. John Accarino
“When a scratch test is negative for immediate food allergy, it’s a very powerful predictive tool. But there are times that you may get false positives. How positive is it? There might be room for more discussion.” — Dr. John Accarino
“There are a lot of ways that allergy can have different mechanisms and different treatments, with different cells involved.” — Dr. John Accarino
[1:43] Dr. Shreffler’s research is focused on understanding how adaptive immunity to dietary antigens is both naturally regulated and modulated by therapy in the context of food allergy.
[1:54] Holly welcomes Dr. Shreffler to Real Talk. When Holly moved to Maine, she sent her patients to Dr. Shreffler at Mass General.
[2:25] Dr. Shreffler trained in New York on a Ph.D. track. He was interested in parasitic diseases and the Th2 immune response. Jane Curtis, a program director at Albert Einstein College of Medicine, encouraged him to consider MD/PhD programs. He did.
[3:31] Jane Curtis connected him to Hugh Sampson, who was working with others to help understand the clinical prevalence of food allergy and allergens.
[3:51] As a pediatric resident, Dr. Shreffler had seen the burden of allergic disease, caring for kids in the Bronx with asthma. His interest in Th2 immunity, the clear and compelling unmet clinical need, and the problem of food allergy guided his career.
[4:31] Dr. Shreffler’s wife has food allergies and they were concerned for their children. Fortunately, neither of them developed food allergies.
[5:21] Dr. Shreffler thinks the food allergy field has a lot of people who gravitate toward it for personal reasons.
[5:53] Food allergy is an adverse response to food that is immune-mediated. There is still uncertainty about this but Dr. Shreffler believes that a large percentage of patients with EoE have some triggers that are food antigens.
[6:27] The broad definition of food allergy would include things like food protein-induced enterocolitis syndrome (FPIES).
[6:47] The way we use the term food allergy in the clinic, there are two forms: IgE-mediated allergies and non-IgE-mediated allergies, including EoE.
[7:40] Some patients have food-triggered eczema, some have FPIES.
[8:04] In 2024, Dr. Shreffler and Dr. Caitlin Burk released a paper that looked at the triggers of EoE, particularly the intersection of IgE-mediated food allergy and EoE.
[8:41] Dr. Caitlin Burk joined the group as they were publishing papers on IG food allergy and EoE. It was a moment where things unexpectedly came together.
[9:17] Adaptive immunity to food proteins comes from antibodies that cause milk allergy, egg allergy, peanut allergy, or multiple allergies. The IgE has specificity.
[9:40] T cells also are specific to proteins. They express a host of receptors that recognize almost anything the immune system might encounter. They have a long memory like B-cells.
[10:09] The overlap in these two threads of research was regarding a population of T cells that are important for mediating chronic inflammation at epithelial sites, including the gut.
[10:36] These T cells have been described in the airways in asthma, in the skin in eczema, and the GI tract. Researchers years ago had also described them as being associated with IgE food allergy. People with IgE food allergies avoid allergens.
[11:13] T cells, being associated with chronic allergic inflammation, now being associated with food allergies which are not having chronic exposures to the allergen, was interesting and surprising.
[11:30] Dr. Shreffler and his group found the T cell subset in patients who don’t do well with Oral Immunotherapy (OIT) and patients who have EoE with immediate symptoms.
[12:01] Dr. Shreffler notes differences. There are immediate symptoms of IgE food allergy. There is a subset of patients with EoE who have immediate symptoms that are not fully understood. Maybe IgE plays a role there.
[12:28] There are different mechanisms for how symptoms are caused and so different ways of making a diagnosis. A food allergy with an IgE antibody can be measured through skin tests and blood tests. This can help identify which foods are the trigger.
[12:57] This common T cell subset that we see in EoE and food allergy, helps to explain why IgE alone is not always a very specific marker for identifying people who will have immediate reactions when they’re exposed to the food.
[13:17] For patients who react at low levels, it’s not just that they have more or better IgE but they also have an expansion of these T cells that are common between EoE and other chronic forms of allergy and IgE food allergy.
[13:41] There’s a lot to learn that might be relevant for patients about this T cell subset.
[14:23] These T cells are a specific subset of the group of Th2 T cells, which are a subset of all CD4 T cells. Some CD4 T cells are important for responding to viruses and tumors. Others are important for responding to outside allergens.
[15:01] In an allergy or a parasite infection, Th2 T cells are important. There is a subset of T cells that is driven by repetitive and chronic exposure to the triggering protein, antigen, or allergen.
[15:47] Most antigens are proteins that trigger an immune response. An antigen that elicits an allergic response is an allergen.
[16:30] A food trigger is a protein antigen that is an allergen. In IgE, food allergies, milk, and eggs are prevalent triggers early in life. For reasons not well understood, a lot of people outgrow them. In older patients, peanut and tree nut allergies are prevalent.
[17:01] In EoE, milk is one of the most common dietary triggers into adulthood. Some patients with IgE allergy to milk can tolerate it if it’s well cooked. Patients with EoE are less likely to be able to get away with regular and ongoing exposure to milk protein.
[17:54] Milk, eggs, and nuts are common triggers in both conditions. There can also be rare food allergy triggers. That’s part of the early evidence that the adaptive immune response was likely to be involved. It can be so specific for some people to rare things.
[18:20] Hallmarks of something being immune-mediated are that it is reproducibly demonstrable as a trigger. It’s going to be long-lived. It’s going to be generally relatively small amounts. The immune system is good at detecting small exposures.
[19:07] EoE is tricky because there’s not that clear and easy temporal association between an offending allergen exposure for most people and their symptoms. People don’t associate the symptoms with the triggers.
[20:14] A history of having blood in the stools can be milk-allergen-driven and was associated with a diagnosis of EoE in those kids when they’re older.
[20:26] There are a lot of commonalities in the allergens but it’s not always obvious clinically.
[22:40] A challenge in diagnosing EoE is that providers have to be on guard against their biases. They have to give a patient good advice. In EoE there is no test to identify triggers, except rigorous introduction, elimination, reintroduction, and endoscopies.
[24:18] For some of Dr, Shreffler’s patients, it becomes less important to know their dietary triggers. They gravitate toward an approved form of treatment that may, if successful, allow them to have a more normal diet because of effective medication.
[24:50] Dr. Shreffler thinks there are other triggers, including pollens. There is evidence of seasonality of active EoE in patients shown to have allergic sensitization to pollens. That’s indirect evidence. If the body is making IgE, it’s likely making other responses.
[25:32] There are questions about how large the population of patients is who have EoE that may be more intrinsically than extrinsically driven because of genetic variations.
[25:54] Dr. Shreffler believes that EoE in some patients is allergen-driven and in some patients EoE is food-driven. Food is a trigger for the majority of pediatric patients and a large percentage of adult patients but not necessarily the exclusive trigger.
[27:04] If a patient is motivated to learn what dietary triggers may be at play, Dr. Shreffler often makes assessments outside of pollen season for allergens to which the patient has demonstrated positivity.
[28:09] Looking at the epidemiology, both EoE and food allergy are atopic disorders. You see an increased prevalence of asthma, hay fever, eczema, and even allergic proctocolitis in infancy. You see an enrichment of one disorder to another.
[28:29] The overlap of food allergy to EoE is stronger than you might expect. About 30 to 40% of patients with EoE will also have IgE food allergy. A higher rate will have IgE positivity, whether or not that food is a trigger of immediate symptoms.
[28:48] Patients with food allergies are about four times more likely to have EoE than the general population. That’s a stronger association than the risk of eczema or other atopic conditions to EoE.
[30:09] There are differences between IgE food allergy and EoE. The presence of IgE gives a useful tool for identifying the food trigger in food allergy, but not in EoE. Identifying rare triggers in EoE patients is done by clinical observation.
[31:46] Epinephrine and antihistamines are not useful in treating EoE. Blocking IgE with Omalizumab has not been effective in trials in treating EoE. PPIs, topical steroids, and dupilumab are helpful for many EoE patients.
[32:38] Dupilumab has been evaluated a bit in food allergy in combination with OIT, and there was no statistically significant benefit from dupilumab in food allergy.
[33:25] A group in Pennsylvania has been evaluating epicutaneous immunotherapy as a modality to treat EoE. It’s also being evaluated for IgE food allergy. Dr. Shreffler thinks it’s something to keep an eye on.
[33:40] The oral route for immunotherapy can drive EoE for patients. As they become less sensitive from an immediate reactivity viewpoint, a significant percentage of patients develop GI symptoms. This has also been observed with sublingual therapy.
[34:14] Iatrogenic EoE, caused by the treatment, may resolve on the cessation of the immunotherapy treatment.
[36:25] Dr. Shreffler says in some cases, the shared decision is a decision where he has a strong evidence-based opinion. In some cases, there’s a lot more room for a range of clinical decisions that could be equally supported by what we know right now.
[36:57] We’ve said that EoE is a contraindication for OIT. There is a shift happening. Dr. Shreffler sits with families and has a conversation about restricting diet or trying chronic therapy and keeping an ad-lib diet.
[37:38] What about doing the same thing by treating the immediate-type food allergy with chronic allergen exposure and then ameliorating the effects of EoE if it emerges, with another therapy? A hundred providers would have a diversity of responses.
[38:19] When there is a history of EoE in a family, Dr. Shreffler advocates for getting a baseline scope. It becomes an important “ground zero.”
[38:28] The goal is to have less invasive ways to monitor these conditions.
[39:32] Chronic inflammation, which is the hallmark of EoE, is well-targeted by therapies like PPIs and steroids. Steroids don’t help with IgE-related food allergies. They’re not effective at blocking the IgE-driven immediate response.
[41:13] Until recently, IgE food allergy has only been managed with avoidance. We have some other tools now. Xolair is not effective in EoE but is effective in two-thirds to three-quarters of patients with immediate-type food allergies for preventing anaphylaxis.
[41:45] Dr. Shreffler refers to an upcoming study on the effectiveness of Xolair in treating people with food allergies. Those who were able to tolerate a minimum amount were allowed to begin consuming allergen. We’ll get insight into how those patients did.
[43:08] Food-induced immediate response of the esophagus (FIRE) is immediate discomfort with exposure to some allergens. Dr. Shreffler explains it. Data supports that these patients are experiencing an IgE-mediated but local response to those triggers.
[44:59] If FIRE is IgE-mediated, it may be that Xolair would help suppress it in these patients. It’s worth looking at Xolair for this subset of EoE patients.
[45:20] Ryan invites any listeners who want to learn more about FIRE to check out episode #34 with Dr. Nirmala Gonsalvez.
[45:37] In the paper, Dr. Shreffler wrote about what he hopes will be the practical usefulness of the finding, the intersection between IgE food allergy and EoE.
[45:56] A subset of Th2 T cells express a protein called GPR15. It appears to be a marker for the subset of cells that are playing a role in the EoE.
[46:36] Caitlin Burk’s work now is looking at their activation status in active disease and post-diet elimination and remission. She is developing a data set that is leading us toward the possibility of focusing on that cell subset and techniques to adopt in clinics.
[47:12] She is also working out more advanced techniques to look at the receptors. Dr. David Hill at CHOP is working on similar research. This research has the potential to lead to the development of better tests for EoE.
[47:44] Holly tells Dr. Shreffler this has been such an informative episode with so many tidbits of things to help patients advocate for themselves. Holly thanks him for sharing all of that.
[48:12] Dr. Shreffler is trying to see what can be utilized from their research to make non-invasive tests to identify food allergen triggers for patients so they don’t have to go through so many endoscopies. He sees it as a huge unmet need.
[48:31] Ryan thanks Dr. Shreffler for joining us. For our listeners who would like to learn more about eosinophilic disorders, including EoE, please visit APFED.org and check out the links in the show notes.
[48:41] If you’re looking to find a specialist who treats eosinophilic disorders, we encourage you to use APFED’s Specialist Finder at APFED.org/specialist.
[48:50] 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.
[49:00] Ryan thanks Dr. Shreffler for joining us today for this interesting conversation. Holly also thanks APFED’s Education Partners Bristol Myers Squibb, GSK, Sanofi, and Regeneron for supporting this episode.
“This fascinating problem of food allergy: why does the immune system do that for some people — recognize what should be nutritive and innocuous sources of energy as an immunological trigger? ” — Dr. Wayne Shreffler
“A food allergy; because there is this IgE antibody, we can do skin tests. We can measure that in the blood. It’s a useful marker for helping to identify which foods are the trigger.” — Dr. Wayne Shreffler
“EoE is tricky because there’s not that clear and easy temporal association between an offending allergen exposure for most people and their symptoms. People don’t associate the symptoms with the triggers.” — Dr. Wayne Shreffler
“Everything is shared decision-making. In some cases, it’s a shared decision where I have a strong evidence-based opinion. In some cases, there’s a lot more room for a range of clinical decisions that could be equally justified.” — Dr. Wayne Shreffler
“Steroids don’t help with IgE-related food allergy. They’re not effective at blocking that IgE-driven immediate response.” — Dr. Wayne Shreffler
“I’m trying to see what we can utilize from our research to make non-invasive tests to identify food allergen triggers for patients so they don’t have to go through so many endoscopies. I think that’s a huge unmet need.” — Dr. Wayne Shreffler
[2:11] As a pathologist, Dr. Collins examines biopsies microscopically. For EGIDs, she determines the peak count of eosinophils per high-power field, or reports the numbers of eosinophils in multiple high-power fields, and analyzes the tissue for additional abnormalities.
[2:33] Dr. Collins then issues a report that becomes part of the patient’s medical record and is provided to the patient’s doctor.
[2:41] The biopsies Dr. Collins examines may be the first biopsies for a diagnosis, or follow-up biopsies to determine response to therapy, or as part of ongoing monitoring to determine if inflammation has returned even if the patient has no symptoms.
[3:07] Dr. Collins was inspired to specialize in EGIDs after speaking with patients with EGIDs. She used to give tours of the pathology lab at Cincinnati Children’s Hospital. She met affected children and their caregivers. Their courage and gratitude moved her.
[3:43] Ryan mentions the wonderful patients and their families in the APFED community. Holly says that as a patient, it’s fascinating to meet a pathologist. Pathologists are generally behind the scenes.
[4:42] Dr. Collins specializes in GI pathology, including eosinophilic-related conditions in the GI tract. EoE, eosinophilic gastritis, eosinophilic enteritis, and eosinophilic colitis.
[5:16] In January 2024, “Guidelines on Childhood EGIDs Beyond EoE” were published in the Journal of Pediatric Gastroenterology and Nutrition. Dr. Collins served on the task force that prepared the guidelines.
[5:35] Non-EoE EGIDs affect all sites of the GI tract except the esophagus. All sites of the GI tract except the esophagus normally have eosinophils in the mucosa, which complicates the diagnosis.
[6:03] Like EoE, the diagnosis of non-EoE EGIDs is made after known causes of tissue eosinophilia are excluded.
[6:28] Consensus guidelines help bring attention to best practices and encourage uniformity of practices.
[6:50] This is especially important for rare diseases and for centers that see fewer patients with rare diseases than the more specialized centers. Guidelines based on the best information available help these centers.
[8:03] The best distribution of guidelines is to publish them in the medical literature and sometimes in multiple journals to target audiences of allergists, gastroenterologists, and pathologists. Guidelines may be presented at national meetings to increase awareness.
[8:36] Several specialties are involved in the care of patients who have EGIDs. If patients or caregivers learn of published guidelines, they can also inform their providers.
[9:23] Insurance is a big issue for so many patients. Getting coverage for both diagnostic and treatment options can be complex.
[9:50] The guidelines may be helpful to insurance companies to accept that a certain drug is needed by a patient with a certain condition. However, if the sequence suggested in the guidelines is not followed, there may be difficulty getting coverage in the U.S.
[11:11] Patients can advocate for themselves with insurance companies by explaining that the order of testing is not important but getting the recommended tests done is important.
[11:55] The greatest challenge the task force faced was the lack of large clinical studies and quality research reports. We’re making progress in this field but we’re at the beginning. Dr. Collins is hopeful that progress will be made in the next two to three years.
[12:24] When there were knowledge gaps, the task force filled them in with their published research and their own experiences. It’s always reassuring to have a well-conducted clinical study that verifies that your thinking is correct.
[13:29] How long did it take the task force to create these guidelines? Longer than they wanted it to take! The years they put into composing these guidelines were greater due to the interruption caused by the [COVID] pandemic. They all felt good when they finished.
[14:18] The guidelines were written by 26 authors from five continents. These are international guidelines.
[14:44] Dr. Collins highlights the pathology. The guidelines state that non-EoE EGIDs should be considered clinicopathologic diagnoses, as EoE is, meaning that biopsies from the affected site in the bowel must show excess eosinophils.
[15:10] The guidelines, for the first time, recommend threshold eosinophil values for a diagnosis in the parts of the GI tract other than the esophagus. For a diagnosis of EoE, a threshold value of greater than or equal to 15 eosinophils per high-power field.
[15:36] The guidelines now recommend that for a diagnosis of eosinophilic gastritis, a threshold value of greater than or equal to 30 eosinophils per high-power field is present.
[15:48] For a diagnosis of eosinophilic duodenitis, a threshold value of greater than or equal to 50 eosinophils per high-power field. For a diagnosis of eosinophilic ileitis, a threshold value of greater than or equal to 60 eosinophils per high-power field.
[16:03] For a diagnosis of eosinophilic colitis in the right colon, a threshold value of greater than or equal to 100 eosinophils per high-power field. For a diagnosis of eosinophilic colitis in the transverse and descending colon, a threshold value of greater than or equal to 80 eosinophils per high-power field.
[16:12] For a diagnosis in the rectosigmoid, a threshold of greater than or equal to 60 eosinophils per high-power field.
[16:18] These numbers may change over time. One or more thresholds will likely change as we gain more experience with these diseases. The pattern won’t change.
[16:29] Several studies have shown that the normal pattern of eosinophil presence in the mucosa in the GI tract is that the number increases from the stomach to the right colon and then decreases throughout the colon to the rectosigmoid.
[17:40] When giving tours of the hospital, Dr. Collins found that people understood better when they knew the numbers and could see the slides of their biopsies.
[18:48] Dr. Collins found literature reviews that suggested that the GI mucosa was often normal in non-EoE EGIDs. She believes that in the next few years, as we publish more and gain more experience, we will realize that is not the case.
[19:14] There is already a method for scoring the mucosa in the stomach in eosinophilic gastritis (EoG) and there are abnormalities found in a majority of patients. We have to work on the rest of the GI tract.
[19:35] Dr. Collins was surprised that there’s not very good information about the use of proton pump inhibitors (PPIs) in eosinophilic gastritis and eosinophilic duodenitis. There haven’t been studies about that. We need to work on that, too.
[20:47] Dr. Collins isn’t sure we can recognize misconceptions about non-EoE EGIDs at this point. It might be premature to label any belief as a misconception. We thought that eosinophils were responsible for all symptoms in EoE, but we know now that is not true.
[21:10] Dr. Collins thinks we need to wait a bit before we decide that we know for sure all about non-EoE EGIDs. Ryan is excited to learn what the research will show us next.
[21:44] Holly loved learning about the algorithm in the guidelines.
[22:01] Dr. Collins says this is the first effort to create uniformity in the way in which non-EoE EGIDs are diagnosed. This algorithm can change over time. It provides signposts for the diagnosis, based on the information we have currently.
[22:20] The diagnosis of non-EoE EGIDs should rest on symptoms and the detection of dense eosinophilic inflammation in the mucosa by biopsy and the absence of evidence of other diseases, such as parasitic and other diseases, that might cause dense eosinophilic inflammation in the GI tract.
[22:46] The algorithm suggests that the particular anatomic site or sites in the GI tract responsible for the symptoms should be determined, for example, eosinophilic gastritis or eosinophilic colitis.
[23:03] The algorithm also suggests that the involved part of the wall in the involved anatomic site should be identified.
[23:13] For example, if the symptoms are suggestive of mucosal disease, without deeper mural or wall involvement, the clinical investigation can proceed directly to endoscopy.
[23:26] However, if symptoms suggest partial or complete bowel obstruction, which is typical of deep muscular involvement, then imaging studies should be considered before proceeding to endoscopy, to confirm or refute that there is a bowel obstruction.
[23:47] If the obstruction is identified, a full-thickness biopsy of the bowel wall may be indicated, possibly requiring a non-endoscopic surgical procedure. If obstruction is not identified, then the investigation can proceed to endoscopy.
[24:05] If there is abdominal distension, suggestive of fluid accumulation, consideration should be given to sampling the fluid, using a needle to pull some fluid out to determine if there are numerous eosinophils in the fluid that would indicate eosinophilic ascites, with the eosinophilic inflammation involving the outer lining of the bowel wall.
[24:41] The signposts are a little involved. They are a reasonable way to approach working up a diagnosis of non-EoE EGIDs.
[25:34] How is EoE ruled out before using this algorithm? It’s sometimes difficult to distinguish symptoms that are relevant only to the esophagus and symptoms that are relevant only to the stomach.
[26:26] Someone with upper tract symptoms only will have an upper tract endoscopy, especially if that person has mucosal symptoms that seem to be relevant to the mucosa only. The best thing is to take biopsies of the esophagus, stomach, and duodenum to be sure where the eosinophil infiltrate is.
[27:06] If the person has lower tract involvement only, such as diarrhea and lower abdominal pain, and no upper tract symptoms, a transnasal endoscopy could be used to determine if there is EoE in addition to the non-EoE EGIDs. Each case is different.
[29:12] As a pathologist, Dr. Collins has seen the guidelines for treating eosinophilic conditions evolve. They’ve become more specific as our knowledge of the data concerning the disease has increased. PPIs are now considered a treatment for EoE.
[30:12] Dr. Collins says we need clinical trials testing therapies in children and adults with non-EoE EGIDs. We need to determine which patients have single-site disease and will only have single-site disease, and which patients may develop multi-site EGIDs.
[30:42] Those aspects will be addressed in the next version of CEGIR, if it’s funded.
[30:49] Ryan tells Dr. Collins it’s been fantastic having her on the show. This has been a good overview of non-EoE EGIDs and the new methods clinicians are looking into to help people get a better diagnosis and treatment.
[31:11] Dr. Collins says we don’t have validated instruments yet to measure symptoms, evaluate the mucosa, and evaluate the biopsies under the microscopes. We need to create those validated tools to help us determine the significance of our findings.
[31:31] Some data strongly suggest that eosinophilic colitis is different from the rest of the EGIDs; certainly from the upper tract EGIDs. We need to move more deeply into what eosinophilic colitis actually is.
[32:05] For our listeners, feel free to check out the article we’ve been mentioning in the show notes. We’ll include a link to it.
[32:11] For those of you who would like to learn more about eosinophilic disorders, please visit APFED.org and check out the links in the show notes.
[32:18] If you’re looking to find a specialist who treats eosinophilic disorders, we encourage you to use APFED’s Specialist Finder at APFED.org/specialist.
[32:27] 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.
[32:36] Ryan thanks Dr. Collins for joining us today for this great conversation. Holly also thanks APFED’s Education Partners Bristol Myers Squibb, GSK, Sanofi, and Regeneron for supporting this episode.
“The best distribution of guidelines is accomplished by publishing them in the medical literature; sometimes in multiple journals to target audiences of allergists, gastroenterologists, and pathologists.” — Dr. Margaret H. Collins
“Guidelines may be helpful to insurance companies to accept that a certain drug is needed by a patient with a certain condition.” — Dr. Margaret H. Collins
“It’s always reassuring to have a well-conducted clinical study that verifies that your thinking is correct.” — Dr. Margaret H. Collins
“This is the first effort to create uniformity in the way in which non-EoE EGIDs are diagnosed. This algorithm can change over time. It provides signposts for the diagnosis, based on the information we have currently.” — Dr. Margaret H. Collins
“We don’t have validated instruments yet to measure symptoms [for non-EoE EGIDs], evaluate the mucosa, and evaluate the biopsies under the microscopes. We need to create those validated tools to help us determine the significance of our findings.” — Dr. Margaret H. Collins
[2:42] EF, as with most eosinophilic disorders, doesn't follow the textbook. Some people will present with one symptom and some with multiple symptoms. There is a disconnect between how we diagnose conditions like EF and how patients present.
[3:01] There are major and minor criteria for the diagnosis. As in Jason’s case, it takes time for the symptoms to present. Things develop over time. It took multiple specialists to diagnose Jason.
[3:38] Eosinophilic conditions are incredibly different from each other. When Dr. Sims sees a patient with high eosinophils, she thinks of three major buckets: infection, autoimmune diseases, and cancer.
[4:12] Patients will often see many different specialists. In Jason’s case, they had done a skin biopsy that wasn’t as helpful as they hoped. That led him to get a deep muscle biopsy to collect the lining of the muscle.
[4:47] Fasciitis is the inflammation of the muscle lining or fascia. A sample of the fascia can demonstrate under the microscope if there is a thickening, swelling, or inflammation of the lining of the muscle.
[5:24] Dr. Sims as a rheumatologist treats a number of rare diseases. Eosinophilic fasciitis is an ultra-rare disease.
[5:43] Jason had a local primary care doctor and a rheumatologist who both did a really good job and referred him to Dr. Sims. She had the benefit of their hard work to guide her next steps. Because EF is so rare, she has pitched Jason’s case twice in rheumatology grand rounds sessions.
[6:18] During one of these sessions, Dr. Sims was advised to get the fascial biopsy that ultimately led to the diagnosis. She benefited from the intelligence and input of dozens of doctors.
[6:59] In the Fall of 2022, while hiking on vacation with his wife, Jason was extremely fatigued, and his forearms and lower legs swelled. His socks left deep impressions. It was difficult to reach his feet to put socks on. He spent a lot of time uncharacteristically resting.
[8:09] Jason’s primary care doctor ran lots of blood tests. He thought it might be a tick bite. Jason started seeing specialists, having tests and hospital visits.
[8:57] Jason worked with a rheumatologist in Wilmington, an infectious disease doctor, and a hematologist/oncologist who reached out to a Duke expert. He also saw a pulmonologist and a dermatologist. He got the referral to Dr. Sims for March of 2023.
[9:57] The first diagnosis Jason received was after his first hospital stay in January of 2023, when he had bone marrow biopsies, CT scans, ultrasound, and other tests. He was deemed to have idiopathic hypereosinophilic syndrome (IHES).
[10:30] It was only a few weeks before his local rheumatologist said his panels were back and one tipped it from an IHES diagnosis to eosinophilic granulomatosis with polyangiitis (EGPA). He joined the Vasculitis Foundation and researched EGPA.
[11:03] Dr. Sims told Jason that EGPA was a working diagnosis but he didn’t check all the boxes. There was the underlying thought that maybe it was something else. He had a second flare when he came off of prednisone in June of 2023.
[11:48] Dr. Sims scheduled Jason for a muscle biopsy while he was off steroids. That’s how he got the diagnosis of eosinophilic fasciitis (EF). Jason says the disorder is hard for him to pronounce and he can barely spell the words.
[12:52] Jason’s wife Michelle encouraged Jason to track his symptoms and medications and keep track of data. Going from specialist to specialist, the first thing he did was give the history.
[13:31] Jason found it helpful to create a spreadsheet of data with blood test results, meds, how he was feeling each day, his weight, and even notes about when he had difficulty putting his socks on. Jason is an advocate of owning your continuity of care as you see different doctors.
[14:42] Jason says the doctors at Duke talk very well between themselves.
[14:49] Jason likes to look back at that spreadsheet and see how far he’s come, looking at the dosage he was on during and after flares and the dosage he’s on now, or zero, on some of the medications. That’s a little bit of a victory.
[15:16] Holly works at a private hospital without Epic or CareEverywhere so she gives physical notes to her patients to give to their doctors. She comments that a great PCP, like the one Jason had, can make all the difference in the world.
[16:18] Jason’s PCP, Dr. Cosgrove, referred Jason to Duke for a second opinion. That was where he met Dr. Sims. He’s glad to have both Dr. Sims and his PCP accessible.
[17:35] Jason says the number of questions you have with this type of thing is immense. When you look up EF, you find very little and the literature isn’t easily digestible by patients. Being able to reach out to your doctors for a quick question is super helpful.
[17:56] Jason has been able to do telehealth follow-ups and not always have to travel or take off work, which has been extremely helpful. He has been at Duke a good handful of times for various things but remote follow-ups are helpful.
[18:52] Dr. Sims says people just don’t know about EF as it is an ultra-rare diagnosis. Even physicians don’t understand what causes it. It’s lumped in with all other eosinophilic conditions but these disorders don’t all present the same way.
[19:19] EoE doesn’t look like EF, even though they’re both driven by the same immune cells. Dr. Sims says the first need is educating providers and patients on what the diagnosis is; awareness in general when a patient is having this swelling of extremities.
[19:44] Dr. Sims says at his baseline, Jason is very active with multi-mile hikes. When Dr. Sims met him, he was off from the baseline of what he was able to do. Being aware of your baseline and changes from that is very informative for doctors.
[20:07] Dr. Sims talks about the patient being a liaison between multiple specialists. Bringing data to your subspecialist always helps facilitate care and come up with a bigger picture of what’s happening.
[20:23] Jason first went to Dr. Sims with the diagnosis of EGPA. She said, let’s treat the EGPA and see what happens but they kept an open mind. With ultra-rare diseases, sometimes it’s difficult for patients not to have a label for their condition.
[20:45] Dr. Sims explains to her patients that sometimes we live in the discomfort of not having a label. She keeps an open mind and doesn’t limit herself to just one diagnosis. She seeks feedback from providers who have seen this before and know what works.
[21:07] Just as Jason described, you will go through multiple diagnoses. Is this cancer? Is it a parasitic infection? Where did you travel? You will see many subspecialists. It’s extremely anxiety-provoking.
[21:31] When Dr. Sims did her grand rounds, she gave a third of the presentation, and the other two thirds were presented by an infectious disease doctor and a hematologist. In these cases, you need more than one subspecialist to complete the workup.
[22:10] Dr. Sims says there are a lot of misconceptions that the patient will get the diagnosis right away and the right therapy and get better. There are multiple therapies, not just medications. There are lifestyle and work modifications; it’s a gradual process.
[22:22] One of Dr. Sims’s goals for Jason and Michelle is to get back to doing the things that they enjoy, tennis and hiking. That’s a measurement of the quality of life that a patient has.
[22:34] Talking to your doctors about how you’re feeling and how you’re functioning is huge. It may be that this is your new normal, but it may also be that we can make adjustments to maximize your quality of life.
[23:00] There are misconceptions about the journey of diagnosis and treatment. Have a close relationship with your subspecialist. PCPs have a high burden of expectations. As a rheumatologist who treats rare diseases, it’s helpful to take on a part of that burden.
[22:31] If you don’t have good communication with your providers and they aren’t listening to you, you can always go get another opinion. The provider relationship is life-long.
[23:43] It’s important for your provider to take what’s important to you into consideration when they make treatment decisions.
[25:00] As a rheumatologist, steroids are a first-line therapy for Dr. Sims. Their role is the quick control of inflammation. The goal is always to get you off of the steroids as soon as possible, in the safest way possible.
[25:17] When Jason came to Dr. Sims, he was on mepolizumab for the working diagnosis of EGPA. Mepolizumab is one of the primary therapies for EGPA. They talked about not making treatment changes as they were navigating what was happening.
[25:40] They didn’t want to make a change of medication and then have that be mistaken for disease activity. They didn’t want too many variables moving at once.
[25:47] Typically, the first-line therapy is steroids, meant to help with the swelling, pain, and tightness that patients will get lining their muscles and give them a bit more functionality and decreased pain.
[26:00] Long-term, Dr. Sims gives immunosuppressant medication. She prescribed methotrexate for Jason. In EF, the immune system is overly activated, attacking the lining of the muscles and causing the symptoms.
[26:51] If you suppress the immune system activity, that leads to decreased inflammation and symptoms in the patient. Steroid use, over a few months, is detrimental, with low bone density, weight gain, high blood pressure, and diabetes.
[27:14] Dr. Sims starts with prednisone and folds in medications like mycophenolate or methotrexate.
[27:19] Mepolizumab is an interleukin 5 blocker. Interleukin 5 is part of the immune system and is necessary for eosinophils to grow, function, and multiply. The goal of using mepolizumab is to lower the eosinophils that are contributing to the disease symptoms.
[27:48] Methotrexate, prednisone, and mepolizumab can work synergistically or independently. Most rheumatologists start with methotrexate or mycophenolate which have fewer side effects and have been around longer. We know how to manage those.
[28:08] If there is no response, we may add something like mepolizumab. As Jason was already on mepolizumab, Dr. Sims added methotrexate.
[28:20] IVIG, an infusion of immunoglobulin, has also been used as a quick way to control inflammation. It is used in other autoimmune diseases like myositis, which is inflammation of the muscle itself.
[29:08] With untreated eosinophilic fasciitis, the lining of the muscle may continue to be inflamed and can lead to fibrosis, damage that cannot be reversed. The patient can become very disabled. Contracture is one result of this.
[30:16] Jason says when he tried a new medication, he monitored if it was a good fit and if the side effects were less impactful than the underlying disease. Dr. Sims adjusted his dosages or tried to get off certain medicines as needed.
[30:59] After his muscle biopsy from his left calf, it took about a month to get back to walking easily. He was already in physical therapy, going many times for a variety of things. He had back pain, potentially related to his EF. His physical therapist was great.
[31:56] The stretches alternated between upper and lower body. Jason bought tools to do the stretches at home. When he’s not feeling as well, he goes back to some of those same stretches. When he was on steroids, he took long walks to strengthen his bones.
[32:39] Jason started making phone calls to supportive family and friends on his walks and started listening to podcasts related to his condition or medications. Getting back to tennis and hiking is important to Jason. He’s happy to be out there.
[33:20] Jason was open with his employer about his condition. Some of the weekly meds can make him not feel well. His employer gives him some flexibility. He has good days that far outnumber the bad days. He doesn’t have to think about EF too much now.
[34:33] It’s nothing like when he was in a flare, especially when he was in a flare before being diagnosed. What gets him through a bad day is giving himself some grace and understanding while he waits for his meds to catch up. He rests more than he wants to.
[35:33] Low-impact exercises like walking help Jason. He’s trying to find a support network that gets EF. That led him to APFED, to find anyone experiencing something like what he was. He saw a conference that included a session on EF.
[36:09] Jason signed up for the conference and there he met Ryan’s mother who has EF. They were each the first person the other had met with EF. They decided to connect after the conference. They talked on the phone for about an hour.
[36:39] She told Jason how she got into APFED and talked a lot about her son who had eosinophilic diseases. Soon after, Jason talked to Ryan as a primer for this podcast.
[38:15] Having a community to relate to, even if it’s one person, is massive. It can make you feel less isolated.
[38:42] Holly says it’s hard having a chronic illness. She thanks both Jason and Dr. Sims for sharing so much information and their journey and she asks for last words.
[38:58] Dr. Sims believes finding a community is critical. She interviews a lot of patients for research and isolation is a frequent theme. Even the doctor doesn’t know what it’s like to live with the condition you live with daily. As Jason said, give yourself grace.
[39:33] Dr. Sims tells her patients that they’re different from the general population because they have to spend so much time and energy managing their condition that they can’t do x, y, or z today, and that is OK. She says to stay motivated and positive.
[40:12] Find what works for you. Walking is good for your physical and mental health. Have the goal of getting back to what makes you happy. Take initiative and find non-medication ways to recuperate. You have control over ways you can feel better.
[40:43] Connect with others and share your story, like Jason did today. It may make someone’s journey a little easier and make them feel less alone. Utilize your condition for good, for a bigger purpose.
[41:04] Jason had wished he could meet someone who could tell him what EF would be like over the years. He says to stay positive and find out what you have control over. Jason believes the future is bright for being able to do many things for a long time.
[42:26] For our listeners who would like to learn more about eosinophilic fasciitis, please visit APFED.org and check out the links in the shownotes.
[42:33] If you’re looking to find a specialist who treats eosinophilic disorders, like Dr. Sims, you can use APFED’s Specialist Finder at APFED.org/specialist.
[42:43] 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/.
[42:55] Ryan thanks Jason and Dr. Sims for joining us for this excellent conversation. Holly also thanks APFED’s Education Partners Bristol Myers Squibb, GSK, Sanofi, and Regeneron for supporting this episode.
“EF patients may present with large plaques on their skin, edema of arms and legs, Raynaud’s Phenomenon, contractures of arms or legs, limited mobility, or loss of the ability to do tasks they used to do.” — Dr. Catherine Sims
“Steroids are … first-line therapy. Their role is the quick control of inflammation. The goal is always to get you off steroids as soon as possible, in the safest way possible.” — Dr. Catherine Sims
“Methotrexate, prednisone, and mepolizumab can work synergistically or independently. Most rheumatologists start with methotrexate or mycophenolate which have fewer side effects and have been around longer.” — Dr. Catherine Sims
“Stay positive and find out what you have control over. The future is bright for being able to do many things for a long time.” — Jason Ingraham
Hospital Colorado.
[1:32] With more than 40 years of experience as an allergist, Dr. Atkins has seen the evolution of eosinophilic disease patient care first-hand and helped establish the Gastrointestinal Eosinophilic Disease Program at Children’s Hospital Colorado.
[1:46] The Gastrointestinal Eosinophilic Disease Program is a multi-disciplinary program designed for the optimal evaluation and treatment of children with eosinophilic gastrointestinal disorders.
[2:10] Dr. Atkins thanks Holly, Ryan, and APFED for programs like this podcast to help educate the population of patients with eosinophilic gastrointestinal diseases.
[2:39] Dr. Atkins chose a pediatric residency. The last rotation was with an incredible allergist, Dr. Lenny Hoffman, in Houston. Dr. Atkins loved seeing patients with asthma, eczema, allergic rhinitis, food allergies, and anaphylaxis.
[2:59] The thing Dr. Atkins liked about it was he could take kids who had potentially life-threatening conditions, work with them, and stabilize things, and they did really well. He could see a change in their quality of life. That got him started.
[3:22] Dr. Atkins did an allergy and immunology fellowship in Buffalo, New York with Dr. Elliott Ellis and Dr. Elliott Middleton who had just written the Allergy: Principles and Practice text. They were incredible, brilliant mentors and wonderful people.
[3:55] Dr. Atkins went to the National Institutes of Health to do basic science research after learning of a double-blind, placebo-controlled food challenge by Dr. Allan Bock and Dr. May in Denver.
[4:27] Dr. Atkins did a clinical project on food allergy with Dr. Dean Metcalf, which was one of the first double-blind, placebo-controlled food challenges in adults. They published two papers on it.
[4:47] Then Dr. Atkins went to work on the faculty of National Jewish Health. He was there for 25 years.
[5:04] Dr. Atkins was invited to join the Children’s Hospital of Colorado because they wanted to start an allergy program there.
[5:21] Dr. Atkins got interested in eosinophilic gastrointestinal diseases in 2006 after he saw a patient who had had difficulty eating, eosinophils in his esophagus, and food allergies. In another state, a doctor had put him on an elimination diet and he got better.
[5:42] The patient moved to Denver. Dr. Atkins saw him and found the case to be interesting. He looked in the literature and found an article on eosinophilic esophagitis by Dr. Alex Straumann.
[5:53] A gastroenterologist, Dr. Glen Furuta, came to Children’s Hospital, looking to work with an allergist. Dr. Atkins met with him and they hit it off. They saw the need for a multidisciplinary program to take care of these patients.
[6:31] Dr. Atkins has always been interested in diseases that led to eosinophilia. Most of them were allergic diseases. Eosinophilic esophagitis and other eosinophilic gastrointestinal diseases came along with much more of a focus on eosinophils in the gut.
[6:45] The first case of eosinophilic esophagitis that Dr. Atkins treated was a patient in 2006. He believes he probably missed earlier cases just by not asking the right question, which is, “Does your child eat slower than everybody else?”
[7:18] Once Dr. Atkins started asking patients that question, it stunned him to find out how many patients said that was part of the issue. He followed up with testing and, sure enough, they had eosinophilic esophagitis.
[7:45] Dr. Atkins says that, in general, eosinophils are present in allergic conditions. If you look at “allergic snot,” and stain it up, it’s full of eosinophils. If you have eosinophilic esophagitis and allergic rhinitis, if you swallow snot, it will impact your EoE.
[8:40] Dr. Atkins tends to be more aggressive with using a topical nasal steroid spray with patients who have allergic rhinitis because that decreases the eosinophils in the nose.
[8:50] Dr. Atkins says since eosinophils are on their way to areas that are involved in allergic inflammation, that’s how Dr. Atkins got interested in eosinophils. It played out with the multidisciplinary group and eosinophilic esophagitis.
[9:07] Ryan went to see Drs. Atkins and Furuta when he was eight. Ryan is so thankful he was able to go to their clinic and is grateful that the doctors helped to create one of the first programs dedicated to treating EGIDs in the U.S.
[9:40] Dr. Atkins credits Dr. Furuta with the multidisciplinary program. They discussed who needed to be part of it. A gastroenterologist first but Dr. Atkins thought an allergist was also necessary. Treating other allergies helps the patient’s eosinophilic condition.
[10:51] They decided they needed excellent nurses who loved working with kids. A lot of the kids had trouble eating, so the group needed feeding therapists and dieticians because these kids have a limited diet. They also needed a pathologist to read the slides.
[12:59] Because this is a burdensome disease, they needed psychologists for the child and the family. Learning coping mechanisms is a big part of the experience.
[14:20] As the program progressed, they saw they needed an endocrinologist to look at the children who weren’t growing as expected. In clinic, they needed a child health person who could play games with the kids and keep them engaged during the long visits.
[14:44] That was how the program evolved. They had an idea but they had to show people it would be an active clinic that would grow and they had enough patients to warrant the program. It happened quickly.
[14:54] The program is fortunate to have a wonderful group of people who get along well and check their egos at the door. There are lots of conversations. Everybody’s willing to listen and put their heads together. They compare notes and histories.
[15:46] Holly had been working as a feeding specialist at the hospital. Someone was on leave so Holly was put into the clinic. She had never heard of EoE; she didn’t know she had it! Her first meeting was a roomful of professionals comparing notes on patients.
[16:31] Holly was in disbelief that these medical professionals met together for an hour weekly to discuss their patients with each other. Later, Holly followed a patient with Dr. Atkins, then Dr. Furuta, then a dietician, and then a nurse.
[17:01] As she followed the patient, Holly listened and recognized the symptoms. She thought that she might have EoE! She introduced herself to Dr. Atkins and asked for a referral for a diagnosis. She was diagnosed that year with EoE.
[17:25] Holly sees many unique things about the program. She was impressed that they had the foresight to include a feeding specialist, not a common specialty at the time. Holly also thinks it’s neat that the clinic sees patients from all over the country.
[18:01] Dr. Atkins says the availability of care is improving across the country. When the program began, people had not heard of eosinophilic esophagitis, not even the local pediatricians. Allergists were just becoming aware of it. They had to be educated.
[18:29] There were people in other communities who didn’t have access to multidisciplinary care. Over time the word has spread. Pediatricians are referring patients to the clinic for diagnosis. Care availability has improved.
[18:55] Not every patient needs a multidisciplinary program. If you have mild to moderate eosinophilic esophagitis and you’re responding to a current therapy, are doing well, and are communicating well with your provider, that’s great!
[19:10] If you need a second opinion or if you have a complicated case, there are some benefits to multidisciplinary care.
[19:33] The providers at the clinic listen to the children as well as to the parents. When a food is removed, a dietician can suggest an alternative the child might like.
[20:07] The clinic wanted to treat local patients but go beyond that, as well. They learn a lot from seeing patients from all over the country with different exposures and being treated by different doctors.
[20:17] When Ryan was young, he would go from his home in Georgia to Denver, yearly. He reflected it felt like summer camp. He got a scope one day and saw the full team of specialists the next few days. It was different from how he was treated before.
[21:06] Ryan says he was listened to and heard, and it was such a great experience for him as a patient to be seen in Dr. Atkins’s clinic.
[21:46] Dr. Atkins says they are trying to teach children to be their own advocates. If the doctor does not listen to what the patients have to say, why should they be involved?
[22:50] A patient experience at the clinic starts with somebody deciding they need to go there and get a second opinion or a diagnosis. They get a referral to the clinic. Dr. Atkins mentions the need for administrative staff as part of the clinic team.
[23:40] The patient fills out forms and gives their records to be reviewed by a physician before being seen to see if the clinic is a good fit for them. If it is, the patient is scheduled with an appointment for each doctor and professional in the clinic.
[24:02] The patient records are seen by each professional on the team for how they relate to the professional’s specialty. They decide what tests need to be done and if they are covered by insurance.
[24:27] The care team meets before clinic to talk about all new and follow-up patients. Then the patient comes in to see the providers, one after another. Patients don’t see all the providers in the same order. The endocrinologist and psychologist are not in the clinic.
[25:39] The clinic visit takes three to four hours. It may involve skin testing for allergies or spirometry for lung function. The patient is scheduled for an endoscopy. When appropriate, they offer transnasal endoscopy, which takes only eight minutes and does not require anesthesia.
[27:11] If the patient has a stricture and the esophagus needs to be dilated, the patient is asleep for that. There is also the esophageal string test, developed by Dr. Furuta. It takes a little over an hour and tells whether there is active disease or not.
[28:02] Care has been made easier. Patients have different options for testing. Holly points out that the family is a part of the team and they are involved in every process and decision. Dr. Atkins says that shared decision-making is a cornerstone of care.
[29:31] Dr. Atkins says what happens in the room is the care provider and patient connect and the patient talks about their problems with somebody they trust. To get the patient to do what they need to do, they have to understand and feel understood.
[31:03] When Dr. Atkins started treating patients with asthma at National Jewish, patients came for a long evaluation, sometimes months. The only treatments were theophylline and steroids. There were side effects to those medications.
[31:39] Dr. Atkins says it has been wonderful to be involved while new treatments have evolved. For eosinophilic esophagitis, when diet works for people, it works. He shares the experience of a teen who is doing great on a diet eliminating milk and eggs.
[32:56] Don’t discount diet. It’s still up front. On the other hand, that doesn’t work for some people. A metered dose inhaler with the puff swallowed may work for some. That’s ideal for teenagers. There are other treatment choices like budesonide.
[34:14] Swallowed steroids go to the liver, where they are metabolized. Now biologics are revolutionizing treatment. Not everyone needs biologics but they’re a great choice for some.
[35:30] A patient starting out doesn’t need biologics as a first treatment. Other therapies may be effective and cheaper. If a patient doesn’t respond, they can go to a biologic. More treatments are being developed.
[36:42] Dr. Atkins wishes for a way to determine the food trigger with a simple test.
[38:00] Dr. Altkins remembers Ryan as a little kid who should have gotten off of milk but he just wouldn’t do it. He also recalls a patient who thrived when he was put on the right elimination diet, giving up only a couple of foods.
[38:33] Dr. Atkins doesn’t want to diminish any of his patients. Every patient is an individual. It’s so much fun working through the problems, the goal, and the adjustments to get there and how the patient is dealing with it, and then watching them do better!
[40:35] The hard part about eosinophilic esophagitis is that very few people outgrow it. It tends to be lifelong. But in the lifetime of patients he is seeing now, Dr. Atkins thinks we will see a cure, or at least, much easier, better treatments.
[40:56] In the population Dr. Atkins treats, they start treatment and all of a sudden, they’re not having trouble swallowing. Everyone who had a dilation said they would do it again when needed. They can swallow better.
[41:44] But then, they have to maintain control of the inflammation. When people feel better, their impetus to take the medication drops off. If they stop taking their medications, a month later, they can’t swallow their bagels.
[42:18] There are holidays, such as Halloween, that are challenging for kids who are on elimination diets. If they collect candy but can’t eat any of it, that may be a problem.
[42:48] People who don’t have EoE don’t understand not being able to swallow. Dr. Atkins sees dads who have this but don’t want to go get checked out. He tells them they need to be examples for their children and go get endoscopies to know if the condition is familial.
[43:53] Dr. Atkins says there are a number of other excellent programs in the country.
[44:24] Holly thanks Dr. Dan Atkins for sharing his expertise to help others and continually teaching the medical community how to recognize eosinophilic diseases and optimize care for all patients.
[44:40] Eosinophilic diseases are not going away. Allergists need to learn the ins and outs of all different eosinophilic disorders. The medications available to treat those disorders are increasing.
[45:02] Early in your career as an allergist, learn as much immunology as you can and how the biologics work and the newer medications coming out. Follow side effects so you know what to say to your patients and what to look for.
[45:20] A lot of EoE patients get picked up in the allergist’s office because they have other allergic diseases. As an allergist, ask if the child or parent eats slower than everybody else.
[45:57] Ryan thanks Dr. Dan Atkins for joining today and personally, for all he has done to treat Ryan over the years. He thanks Dr. Atkins on behalf of APFED and for being instrumental in many APFED conferences and educational materials.
[46:30] Dr. Atkins’s biggest hope is that people sort out the pathways that lead to eosinophilic esophagitis and that we will have an array of targeted treatments for individual patients to cure that disorder for that patient without side effects.
[47:50] Dr. Atkins thanks Ryan and Holly again for the opportunity to join them. It’s been such a pleasure. He thanks APFED again. He has been a big fan for years. Giving patients a voice to share their stories is incredibly important.
[47:34] For our listeners who would like to learn more about eosinophilic disorders, please visit APFED.org and check out the links below.
[47:41] If you’re looking to find a specialist who treats eosinophilic disorders, you can use APFED’s Specialist Finder at APFED.org/specialist.
[47:51] 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/.
[48:07] Holly thanks Dan and also thanks APFED’s Education Partner Bristol Myers Squibb, GSK, Sanofi, and Regeneron, who supported this episode.
“I loved helping patients with asthma, eczema, allergic rhinitis, food allergies, and anaphylaxis. You could take these kids who had potentially life-threatening conditions, work with them, and stabilize things, and they did really well.” — Dr. Dan Atkins
“With patients who have allergic rhinitis, we tend to be more aggressive with using a topical nasal steroid spray because that decreases the eosinophils in the nose.” — Dr. Dan Atkins
“We are trying to teach children to be their own advocates. … If you don’t listen to what the patients have to say, why should [the patients] be involved?” — Dr. Dan Atkins
“The hard part about eosinophilic esophagitis is that very few people outgrow it. It tends to be a lifelong phenomenon.” — Dr. Dan Atkins
[2:23] At that point, she underwent testing and was diagnosed. Since then she has been trying to find a treatment plan that works for her. She has navigated changes in her treatment plan while going away to college. She comments that, in college, you don’t always have control over the food you eat.
[2:42] It has been a challenge but she has a great support system and care team.
[3:15] In middle and high school, Kate got a food impaction nearly every day at lunch. She thought it was normal. When she finally brought it up to her primary care physician, the doctor suspected EoE. She also had the symptoms of heartburn or acid reflux.
[4:15] Kate immediately had an endoscopy scheduled and was then diagnosed with EoE.
[4:22] Prior to a diagnosis, Kate coped with symptoms by drinking “a ton” of water with foods to help them go down her esophagus, which was narrowed from scarring. She also avoided bread and meats. She only recognized these as adaptive behaviors after she was diagnosed.
[5:32] Kate was referred to a gastroenterologist. That doctor is still part of Kate’s care team. She also saw a nutritionist when looking for a treatment plan that worked for her.
[6:02] Kate avoids anything with dairy in it. Her elimination diet removed anything made with butter, milk, yogurt, and more. At her follow-up endoscopy, her eosinophils were way down so she didn’t have to go through the six-food elimination diet. She was very grateful.
[6:43] Mary Jo clarifies for listeners who may not be familiar with the six-food elimination diet, that the foods removed from the diet are typically milk, egg, wheat, soy, peanut/tree nut, and fish/shellfish.
[7:05/] Kate says it was a tough transition because more foods than you might expect have milk in them. She is grateful she didn’t have to cut out gluten or nuts.
[7:32] Kate admits that sometimes she knows there is dairy in the food and eats it anyway. Then the symptoms are most often severe acid reflux and heartburn. She’ll get a food impaction if she’s been eating dairy for days or a lot of it at once.
[8:35] At school, sometimes the dining hall workers aren’t aware if a food has dairy in it. Some foods are mislabeled as vegan. So Kate often has dairy without knowing it until symptoms appear later.
[9:12] Kate is on a proton pump inhibitor twice a day and a steroid medication. Her first treatment was the elimination of dairy. The summer before going to college, she was also put on these two medications, in case she was exposed to dairy.
[10:50] Kate had to switch from one steroid medication to another because the first one wasn’t working for her varying schedule. She asked her doctor for another medication that fit her schedule better.
[11:31] Kate is very grateful to feel autonomy over her treatment plan. She feels her gastroenterologist is amazing. Kate is always free to give feedback about how she is feeling.
[11:46] Kate feels a more present role in her medical appointments now, versus before she was diagnosed. Both with her gastroenterologist and her primary care physician, she has learned to be comfortable being forward and truthful with her doctors.
[13:16] For patients who have not learned to speak up to their care team, Kate suggests being patient. It takes time to get used to the structure of appointments, follow-up appointments, and appointments with a new doctor.
[13:25] When Kate transitioned from her pediatrician to her adult doctors it took her a while to become comfortable with her gastroenterologist and her primary care physician. Don’t be afraid to pose questions and concerns, even questions about other treatment plans.
[13:56] Your doctors are there to listen to you and to help you, and you can only gain from bringing something up to your doctor that you are afraid to bring up or that you aren’t sure how the conversation is going to go.
[14:47] Kate explains how she tells her friends about her EoE triggers and symptoms and what eosinophils do to her esophagus. Most of her friends study biology and the immune system, so they can understand her condition and needs.
[16:12] EoE impacts Kate’s social life. Kate sometimes sits out of activities involving dinners or events with food present. She doesn’t want to be exposed to a trigger food. She has also missed events and activities due to hospital visits and appointments.
[16:37] If Kate is having a flare-up of her symptoms, she will also miss activities. All these things lead to feelings of exclusion. No one she knows has EoE.
[16:55] Because of her treatment plan and her medications, Kate’s symptoms are somewhat limited so she can participate in activities as she chooses.
[17:14] Kate’s advice for people who know someone with EoE is to be open-minded. EoE is kind of an invisible disorder. People often think that being allergic to dairy is lactose intolerance, which it is not. Do some research about EoE and its effects. Offer support.
[17:42] A patient has a care team whose job is to care for them. When family and friends include you and care for you, it’s a very different feeling. As a friend, be patient and offer any support you can.
[18:34] When Kate travels, her best tool is communication. Informing food service staff about your allergy is the easiest way to ensure that the food you eat is safe. Kate does lots of research when she travels. This includes looking on restaurants’ websites for dietary restrictions.
[20:43] Kate prepared for college by making sure she had a full semester supply of medications and ensuring that she was in close contact with her medical care team over the phone and through MyChart.
[21:19] She made plans with her gastroenterologist to make sure that if anything did come up or if the treatment plan wasn’t working in the college setting, they would talk it through when it was needed and not wait until she was home for Thanksgiving.
[21:54] Kate is thankful she chose a school that was connected to a hospital. She feels more safe and prepared for a bad food impaction. She knows she can seek medical attention and have a team on-site very soon.
[22:43] The quality of the dining at the college was a factor in Kate’s choice of schools. Vanderbilt has a dining hall completely free of the eight main allergens. When she eats there, she knows that the food she is eating will be safe, without having to ask.
[23:34] For people living with an eosinophilic disorder like EoE, Kate has two tips about preparing to go away to college. First, don’t feel limited by your disorder. At first, Kate was afraid of being far away from her care team, but she couldn’t be happier now.
[23:56] At college, there are resources and counseling to help you. Being away from home has taught Kate to be responsible as an adult for the treatment of her disorder. So, second, use college as an opportunity to gain responsibility in treating your disorder.
[25:11] Kate is working in a biomedical engineering lab at Vanderbilt University that is researching a way to diagnose and monitor EoE using saliva. They use Raman Spectroscopy to look for biomarkers in saliva from people with EoE, not found in the saliva of healthy people.
[25:51] A successful test would be less costly and quicker than an endoscopy with anesthesia. It would provide a point-of-care diagnostic that would lower the cost and increase the efficiency for patients.
[28:09] Raman Spectroscopy is a form of spectroscopy that measures the vibrational frequencies between chemical bonds. It reads the composition of biological samples. It can tell the amounts of lipids, proteins, or amino acids.
[26:37] The lab is researching the differences in the saliva of people with EoE from the saliva of healthy people. If they can find a difference, and if that difference is universal, we might be able to diagnose EoE using Raman Spectroscopy.
[26:55] Kate says that would be awesome because the anesthesia of endoscopy is “a lot”, every time.
[27:09] Kate is on the pre-med track, which includes doing research. She wanted her research to be on something that she cared about. She is happy to be at a school that is a top research institution.
[27:48] When she learned of this biomedical engineering lab researching ways to diagnose EoE, she immediately reached out to the Principal Investigator and was invited to join. She has been a part of the lab for over a year and it’s been an amazing experience!
[28:15] Before going to medical school, Kate plans to take one or two gap years after she graduates so she can further her research with EoE or with other eosinophilic disorders. She would love to see advancements in the field.
[29:16] Kate finds that the most challenging part of living with EoE is feeling lonely, She has never met anyone else with EoE. No one knows what it is when she first explains it to them. It’s not a super common disorder.
[29:35] Because EoE is a rare condition, Kate doesn’t have much of a community around it in her life. She was excited to learn about and connect with APFED through social media. Even so, it is easy to feel alone in her personal life.
[29:54] Kate doesn’t want to have to worry constantly about her food or when to take her medications. She wants to live like a normal, healthy college student, but she can’t. Sometimes she doesn’t take her disorder as seriously as she should.
[30:25] Sometimes Kate will have that ice cream because everyone else is having it. She wants to live as though she doesn’t have EoE. Navigating that without a community that relates to her struggle has been a challenge.
[30:51] Kate’s parents, siblings, and friends support her, but it’s not the same as having someone who completely understands and is going through it. She feels lonely.
[32:24] Kate says that awareness is super powerful. She went 16 years without knowing she had EoE. She wasn’t educated on it. She is sure there are many living with untreated EoE just as she was.
[32:42] Kate encourages anyone listening to spread awareness for eosinophilic research. People are struggling with it. Kate was super excited to come on the podcast today to share her story with anyone in college who might be struggling with EoE.
[33:05] Kate says, if you’re struggling with eosinophilic disorders, you’re not alone. I’m there, too! She encourages listeners to continue to spread awareness and advocate for eosinophilic disorders.
[33:13] Ryan and Mary Jo thank Kate Goncalves for joining the podcast episode to share her story and help spread awareness.
[33:19] For our listeners who would like to learn more about EoE, please visit APFED.org/EOE. If you’re looking to find a specialist who treats EoE, you can use APFED’s Specialist Finder at APFED.org/specialist.
[33:40] Kate thanks Ryan and Mary Jo for this opportunity.
[33:43] Mary Jo also thanks Bristol Myers Squibb, GSK, Sanofi, and Regeneron, APFED’s Education Partners who supported this episode.
“I go to school halfway across the country so I don’t have close access to my care team and support. … Knowing I was going to be exposed to some dairy, my GI doctor thought it was best to put me on the steroid medication.” — Kate Goncalves
“Your doctors are there for you. They're there to listen to you and to help you and you can only gain from bringing something up to your doctor.” — Kate Goncalves
“Feeling support from family and friends… you feel so included and cared for. Feeling that from friends versus feeling that from your care team… they’re very different. Your care team cares about you but that’s their job.” — Kate Goncalves
“I think anyone with a chronic illness would relate to this. I just turned 20 and I’m in college. I don’t want to constantly worry about the food I’m eating and when to take my medications. I want to live like my friends.” — Kate Goncalves
[3:06] Now, she enjoys patient advocacy, advocating for EoE, and working with the American College of Allergy, Asthma, and Immunology House of Delegates and the American Academy of Allergy, Asthma, and Immunology.
[3:38] For new listeners, EoE is an allergic and immune disease of the esophagus. With EoE, eosinophils are found in the lining of the esophagus, causing inflammation, and inviting more eosinophils. EoE is a chronic inflammation of the esophagus.
[4:30] A child might have symptoms of food refusal, regurgitation, and abdominal pain. Adults may feel food sticking. The standard for finding eosinophils is endoscopy with six biopsies; two proximal, two mid, and two distal.
[5:24] It is a patchy disease, so the allergist will want to partner with a gastroenterologist who will do the biopsies at the three different levels.
[6:10] Holly tells how she didn’t get diagnosed until she was in her mid-twenties. Her doctors thought she was vomiting for attention. They were not familiar with EoE. She was diagnosed as an adult when working at a major hospital.
[7:00] An allergist at a big academic center, working together with a team of doctors from multiple disciplines, may find it easier to diagnose EoE. Many allergists are not working on multi-disciplinary teams.
[7:34] An allergist can look at the resources in the community. Dr. Bansal has several gastroenterology centers that refer patients to her. They partner with Dr. Bansal on the diagnosis and treatment.
[8:02] In other clinics, it may be the gastroenterologist who takes the lead in treatment and refers to the allergist to manage the allergic disease that exists on top of the EoE.
[8:21] As a patient, the diagnosis process depends on where you live. If you have symptoms, don’t give up. When Dr. Bansal started, she had to ask pathologists to stain biopsies for eosinophils. Today, staining is done routinely; she doesn’t need to ask for it.
[9:07] You are your best advocate. If somebody doesn’t believe you, that doesn't mean somebody else won’t.
[9:22] Holly is a feeding specialist in Maine. In the three years she has been there, more than 100 people whom she has referred have been diagnosed with EoE.
[10:24] Dr. Bansal says that whether you see an allergist or a GI doctor, depends on which specialty takes the lead on EoE in your community. If you do not have a good rapport with your doctor, make a change. Partner with a doctor who advocates for you.
[11:59] Dr. Bansal advises that when seeing a different doctor, to print out your biopsies to bring. If you are using a biologic therapy, print out the scope that you had right before your biologic therapy, too. That biopsy is your golden ticket if you switch insurance companies.
[13:02] Dr. Bansal uses Care Everywhere, but she wants to see prints of your labs anyway. Not everyone opts into Care Everywhere. She likes the Patient Portal and she is looking forward to someday having a universal portal.
[13:43] Dr. Bansal recommends patients sign up with the Portal for their allergist and GI doctor to facilitate communication.
[15:14] Patients can scan their printed scope reports and share them with their care team, such as by putting them on a keychain USB drive, in a photo album on a phone, or a scanning app on a phone. There are different ways to store the data.
[15:39] Ryan describes how his mother organized his medical history, including all prescriptions, every procedure he has had, and all the doctors he has seen. Ryan was diagnosed at two-and-a-half years old, after visits to six or seven doctors.
[17:30] Dr. Bansal participated in an ACAAI video about the difficulty of diagnosing EoE. The video is linked in the show notes.
[17:41] It is a challenge to diagnose EoE. Some children have milder forms of the disease. When it’s familial, some patients think it’s normal for the family. Food sticking is not normal for everyone.
[20:08] The patient may live in an area with a lack of access to a knowledgeable specialist. Sometimes the patient just doesn’t know who to see. When you make an appointment, call ahead and ask if they treat EoE at that office. Not all allergists do.
[22:18] A podcast episode with Dr. Emily McGowan told of her research study on the prevalence of EoE in urban vs. rural areas. It’s not about rural vs urban but about having access to a specialist. Rural patients couldn't find a specialist.
[22:44] Ryan notes that you can use the Specialist Finder at APFED.org/specialist to see if there is a specialist in your area.
[23:12] With younger patients, Dr. Bansal finds that food refusal is a sign of EoE; that, and abdominal pain. By age 10, patients can say they are having trouble swallowing or that food is getting stuck.
[24:28] Holly finds that pediatricians are often not aware that picky eating and food aversion or food refusal are different situations. Food aversion comes from painful swallowing. Dr. Bansal is trying to create an understanding of EoE among pediatricians.
[26:15] Another sign Dr. Bansal watches for is FIRE (Food-induced Immediate Response of the Esophagus), which is different from PFAS (Pollen Food Allergy Syndrome). FIRE is a narrowing of the esophagus; PFAS is an oral issue.
[27:13] As we get more research and learn more about the disease state and the nuances, the hope is that we could educate pediatricians on how to differentiate a picky eater from a patient with food aversion, using compensation mechanisms to swallow.
[28:15] Ryan compares this episode to a “greatest hits” episode, referring to FIRE and compensation mechanisms in the pediatric patient population. Those are great episodes for our listeners to go back and hear again.
[29:01] When a patient has an EoE diagnosis, Dr. Bansal practices shared decision-making with the patient. She outlines four options for the patient. The first option is eliminating dairy and known food allergens from the diet.
[31:01] With a food-elimination diet, ask your nutritionist what you need to eat more of because of the foods you are eliminating. If you’re taking away dairy, you want to make sure you’re getting calcium and Vitamin D. If you’re taking away wheat, you need zinc.
[31:20] The second option Dr. Bansal talks about with her patients is a high-dose proton pump inhibitor (PPI). The third option is topical budesonide, swallowed with honey. Budesonide is a 12-week prescription. Flovent can also be swallowed.
[32:46] The fourth option is dupilumab. Insurance companies may require you to fail option 2, the PPI, for eight to 12 weeks before paying for this.
[33:40] Dr. Bansal wants patients to understand that they can change between treatment options but she wants a patient to stay on an option for eight to 12 weeks and get a scope to see how it works before switching to another option. Follow-through is necessary.
[34:42] Listeners can watch the video of Dr. Jonathan Spergel’s presentation at EOS Connection 2024 to learn more about eliminating milk for EoE and PPI-responsive EoE patients.
[35:14] Dr. Spergel also touched on nutritional deficiencies from some diet therapies. APFED just recorded a great podcast episode on that, as well.
[35:31] Dr. Sara Bluestein made a presentation at EOS Connection on eosinophilic asthma which included an overview of biologic treatments, not just for EoE but for many eosinophilic disorders.
[36:09] Holly loves Dr. Bansal’s team approach with patients, where she acts as the coach, helping guide them toward the decision that will work best for their lives.
[36:20] As a feeding specialist, Holly is excited about the dupilumab option for patients who are on feeding tubes for severe inflammation in the esophagus.
[38:18] Budesonide oral is for 11 and up and dupilumab is for ages one and up. Any doctor who will give a patient the proper care may prescribe these to the patient. For dupilumab, the success rate goes up over time, starting at around 60% to around 80%.
[39:27] You want to get the biologic prescribed and approved for EoE. If it is approved for atopic dermatitis, the dosing is too weak for EoE and it fails at two-week dosing.
[39:56] Biologics need to be continued even when symptoms go away, as the symptoms will return. These two medicines are immunomodulators, not immunosuppressants so they don’t increase the risk of other diseases.
[41:50] Patients need biopsies to get therapy.
[42:13] Ryan reminds listeners about the trans-nasal endoscopy podcast episode and the episode on the string test, which don’t require sedation.
[43:06] Dr. Bansal notes that in trials, benralizumab failed as a treatment for EoE. It brought down the eosinophil count but it didn’t treat the patient’s dysphagia symptoms. Some specialists are wondering if there’s more to EoE than just the eosinophils.
[44:30] Mepolizumab also reduces eosinophils. It is not known if it is effective against EoE. Tezepelumab is undergoing EoE trials now. It reduces eosinophils in the first two weeks. The data is not out on its effectiveness as an EoE treatment.
[44:51] At EOS Connection 2024, Dr. Bluestein gave a great talk on eosinophilic asthma, including information about biologic treatments. More information is coming out about them and their trials for other eosinophilic diseases.
[45:27] Dr. Bansal talks about how great it is to have new biologic options for people who tried an elimination diet, PPIs, and budesonide without relief from dysphagia. There was a void in the space, which is what dupilumab filled.
[46:27] Allergists think that at least 70% of EoE patients have at least one other atopic disease, such as eczema, allergic rhinitis, polyps, or asthma. In pollen season some patients’ EoE gets worse.
[47:11] Dr. Bansal tells patients she doesn’t want them dripping and draining into the esophagus. She doesn’t want them to add inflammation where there is already inflammation. So she treats their allergies.
[47:44] After a dilation, a patient may feel cured. It’s just a stopgap; they’ll be back if they don’t treat their EoE. Dr. Bansal hopes that allergists treat any allergies as well as the EoE, so the allergies don’t have a negative impact on the EoE.
[49:41] There’s no harm in getting an evaluation and seeing if there is something you could be doing to minimize the overall disease impact on your body and keep you healthier overall. Inflammation anywhere is never a good thing.
[50:41] Ryan and Holly thank Dr. Bansal for joining the podcast today.
[51:01] Dr. Bansal’s last word: “I would advise parents to trust your instincts. If you think that something’s wrong, even if somebody’s telling you it’s not wrong, get to the right people. Some insurances allow you to make an allergist appointment without a referral.”
[52:18] If an allergist tells you there is a problem, know that they have years of experience with allergy patients. Dr. Bansal has hundreds of patients with EoE in the clinic. Create a partnership with an allergist.
[53:12] If you would like to learn more about EoE, please visit APFED.org/EOE. If you’re looking to find a specialist like Dr. Bansal, you can use APFED’s Specialist Finder at APFED.org/specialist.
[53:29] 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.
[53:39] Ryan thanks Dr. Bansal again for joining us on Real Talk: Eosinophilic Diseases. Dr. Bansal thanks Ryan and Holly. Holly would like to clone Dr. Bansal with all her enthusiasm. Holly also thanks Education Partners, Bristol Myers Squibb, GSK, Sanofi, and Regeneron for supporting this episode.
“We have other things that we’re working with. We’re trying to find biomarkers and other tests that are not as invasive but for right now, it’s the endoscopy with six biopsies; two proximal, two mid, and two distal.” — Dr. Priya Bansal
“As we get more research and learn more and more about the disease state and the nuances and some of the things that we’re seeing, obviously then, the hope is that we could educate people.” — Dr. Priya Bansal
“You’ve got to have your biopsies. I know everyone hates them, everyone hates the scope, it’s uncomfortable, it’s annoying. I get it, but you need it to get therapy.” — Dr. Priya Bansal
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