The 2007 APFED conference was an amazing and informative experience. Held in scenic Snowbird, UT, the attendees were able to enjoy mountain views, tram rides, and many activities for the kids. On Friday we had a short session in the afternoon. Speaking were Dr. James Lee, Dr. Marc Rothenberg, and Dr. Gerald Gleich. For those new to the diagnosis it was a good overview of the eosinophil and how the disease works. We learned that eosinophils originate in the bone marrow and then migrate to the intestines in their mature state, where they normally reside in small numbers. The just "hang out" waiting for parasites and other pathogens. There are a small number of them that also circulate in the blood. The chemicals that make the eosinophils function are many, but one of the main ones focused on during the conference was Interleukin-5 or IL-5. This chemical serves to start the growth process of new eosinophils in the marrow. It also plays a role in attracting large numbers of eosinophils to a particular area of the body.
We were introduced to the symptoms of EE and how it changes with the age of the patient. Generally infants show feeding problems and preschoolers have vomiting. By school age the main symptoms are abdominal pain and swallowing problems. Towards adulthood food impaction becomes a more common occurrence. Many adults who are treated for food impaction end up being diagnosed with EE. EE seems to have a genetic basis. Dr. Rothenberg said that in the future we should be able to study a patient's genetic material (DNA) to determine if they have the genetic profile for EGID. Lastly, we were given a history of the study of eosinophils from their discovery to the present. Asthma and eczema are eosinophil associated diseases as well.
Saturday's session was packed with information and I think we all left with honorary college degrees! Dr. Peter Weller introduced eosinophils as associated with "worms, wheezes, and weird diseases." The weird diseases include EGIDs. He went into detail about the various chemicals involved in the function of eosinophils, and how there is potential to interrupt the functions of these chemicals in order to treat EGIDs. The eosinophil cell is packed with "bubbles" called granules. In the granules are cytokines, various toxins and chemicals that serve to attack pathogens and parasites. When an eosinophil is active there are not many intact granules present in biopsy. When they are inactive the granules can be easily seen through the microscope. We were given the latest info about the anti IL-5 drug that is in development. Four adults with EE responded well to the drug. Rats have shown no side effects and appear to be healthy after several generations.
Dr. Alex Straumann presented some interesting case studies during his talk. He explained that it is important to follow the natural history of EE to determine the course of the disease if left untreated. Based on his extensive experience, he is able to determine that: EE is a chronic disease, there is no cure and it does not resolve. EE is restricted to the esophagus, it does not lead to cancers, and that it is a non-fatal disease. However it substantially impairs quality of life. Dr. Straumann then shared slides and case studies of previously healthy adults who were found to have EE secondary to the presenting problem (such as food impaction or esophageal perforation). What he concluded from these cases was that EE may persist in "stand-by" mode for years. It leads to remodeling of the esophagus over time that may lead to thickening and tearing, and that it is a risk factor for perforation from forceful vomiting or endoscope procedures (for example to remove impacted food). He emphasized that if food impaction occurs, the doctor must use a flexible scope instead of a rigid one to prevent perforation in someone with EE.
Next was Dr. Seema Aceves, who spoke on the ways to diagnose EE and how it differs from GERD. Dr. Aceves said that there was no current clear-cut guideline in how many eosinophils are too many. Eosinophils do not normally reside in the esophagus at all, but it's normal to find small numbers of them in other parts of the digestive system. The current recommendation to diagnose EE is 15 eosinophils per high powered field at a 400x magnification, in conjunction with symptoms and clinical findings suggestive of EE. For research purposes they recommend 20-24 eosinophils per high power field. People who have reflux often do have some eosinophils present but the numbers are much lower. With reflux, the eosinophils are usually only in the distal esophagus (closer to the stomach). Reflux symptoms get better with acid-reducing medicines. If examined under a microscope, the eosinophils are granulated in people with reflux, but degranulated in EE. People with EE are constantly making epithelial (surface) cells in the esophagus, and there are more chemicals present that cause fibrosis. There is a greater concentration of Eotaxin-3 which calls cells to that area from other parts of the body. People with EE have more blood vessels, and they are "stickier", meaning they can transport and hold eosinophils in the area much more effectively.
It has also been found that fibrosis, or remodeling of the esophagus that makes the tissue less flexible and thicker, can completely resolve in children; however in adults the remodeling is somewhat permanent. She explained the differences between IgE mediated conditions-- including anaphylactic responses and oral allergy syndrome-- and non-IgE conditions such Celiac disease and food protein intolerance. EGIDs fall somewhere in between with characteristics of both. In allergy testing, the RAST is not very effective for EE purposes. The skin prick test is somewhat more useful. Patch testing is the best. The most useful of all is using both patch and prick testing to determine elimination diets.
Dr. Putnam spoke about EGID classification. He shared that there are two types: allergic and non-allergic. The allergic type is associated with asthma, environmental allergies, eczema, and rhinitis. In fact 75% of patients with EGID also have a diagnosis of the associated conditions. The correlation between the severity of inflammation and the symptoms is poor. Many patients have mild or no symptoms and still scope poorly, while others may have a low eosinophil count and have severe symptoms. The best way to determine the current condition of a patient is to biopsy rather than relying on symptoms. Using research from several sources, it was found that up to 95% of children with EE got better on formula only, even when they did not have positive allergy tests. Those who chose an elimination diet of the top 8 foods had up to 88% improvement. Dr. Putnam distinguished between different EGIDs and their symptoms. For example the esophagus and stomach had the symptom of vomiting, while diarrhea signaled that the disease was in the intestines and colon.
Dr. Glenn Furuta shared how EE and EGIDs are getting increased attention in medical journals over the last ten years. He presented several covers of journals with EE featured on them emphasizing the fact that this is a disease that the medical community if very interested in. Dr. Furuta said that the intestines secrete natural antibiotics. Eosinophils may participate in disrupting normal intestinal function. Mouse experiments suggest that PPIs may increase the IgE levels. This emphasizes the fact that all medications must be considered for both the risks and benefits they offer. The primary ideal treatment for EGIDs is an elemental or elimination diet. He emphasized that patients should work with a nutritionist when eliminating foods to make sure they still get proper nutrition. Steroids (particularly corticosteroids) provide only temporary remission. Cromolyn and Leukotriene inhibitors (Singulair) are not effective in EGID treatment. In deciding a treatment plan for a patient, a doctor must "first do no harm." This is why many doctors prefer dietary treatment over the use of steroids.
Dr. Nirmala Gonsalves gave a presentation on the differences between adult and childhood EE. Many doctors viewed them as separate but related disease. Dr. Putnam presented a slide earlier showing the range of symptoms. This included feeding problems in infants, vomiting in preschoolers, and Dysphagia in older children. The most common symptom for adults is food impaction. It is important for patients of all ages to try to keep the eosinophils in remission. Prolonged EE, even without obvious symptoms, can cause permanent damage. In adults with EE, 96% were found to have environmental allergies and 72% had food allergies. Dilation to correct swallowing difficulty (dysphagia) works, but there is a risk of perforation, and the problems can come back after 3-6 months in most patients. The best strategy is to prevent inflammation.
There were some important points to remember from the presentations to keep in mind when working with doctors. First, is that in order to get an accurate count of eosinophils, at least four to five biopsies should be taken in each of the distal and proximal areas of the esophagus. Secondly, airborne allergens may cause a rise in eosinophils. If a person who has both food and airborne EE they should avoid being scoped during allergy season when undergoing food trials.
Several of the physicians and scientists emphasized how important it is to become in involved in a group like APFED, that we can make a difference if we work together towards a common goal. There are many ways families help out- from fundraising, to education to volunteering. “Get involved!” was a clear message heard throughout the conference.
The program ended with several panels of parents and teens with EE. The teens were especially impressive with their great attitude about living with EGIDs and being able to suggest helpful tips for the parents of children who are affected by them. By hearing directly from the experts and being able to ask them direct questions, we become empowered to be better advocates for our children and ourselves.
Many thanks to all of the volunteers and attendees who made this year’s conference a reality! We had a record number of volunteers this year working on pre-planning, fundraising and numerous areas during the conference. A big THANK YOU to everyone who participated!
Eos Connection 2008 is planned for next summer in San Diego. We need local volunteers! Please contact us at mail@apfed.org if you would like to help out.
Conference programs will be sold on the website with conference bag and ‘Got Eos?’ T-shirt for $29. Please see the on-line Gift Store for details.
