The PBCers Biannual Conference recently took place in Houston, Texas. The 2018 PBCers Conference was highly informative with topics ranging from the basics of Primary Biliary Cholangitis, dietary tips, updates in research/drug development, coping with symptoms such as fatigue and pruritus. It also gave PBCers from across the country the chance to have some face time with each other and with some PBC experts. Two physicians who couldn’t be there in person due to prior commitments provided in depth videos for attendees.
Here are a couple highlights:
Dr. Gish was the first presenter at the event. He provided a detailed overview of PBC and described the necessity for a comprehensive, individualized treatment plan for those living with PBC. It is a liver condition, but that does not mean that the liver itself should be the only concern when patients are assessed and treated. It was emphasized that PBC can affect many aspects of people’s lives and that that should be taken into account. Dr. Gish also ventured outside a strictly biomedical perspective, suggesting that coffee can be beneficial to those with PBC and that the use of topical primrose oil could help lesson the severity of pruritis (itching). He also recommends that PBCers get three hours of a mix of aerobic exercise and weightlifting per week. For more info check out his website.
Eric Gershwin, MD, provided a video all the way from Lisbon, Portugal where he was attending a conference on autoimmune disorders. He echoed Dr. Gish’s sentiment that “The treatment of PBC should be individualized referencing that we all have unique genetic components and a unique immune signature. He also believes that different treatments will be used for different stages of the disease. Dr. Gershwin also mentioned a new mouse model; “designer” mice that develop PBC and that he believes, will help answer some questions about the disease.
“Early intervention is key for autoimmune diseases.” – Eric Gershwin, MD
A video presentation by Chris Bowlus, MD, outlined autoimmune liver diseases; delineating how PBC targets the small bile ducts, PSC targets the large bile ducts, and AIH targets the main liver cells. Dr. Bowlus also detailed clinical endpoints of PBC and provided a brief history of treatment trials for the disease as well as some basic info regarding where to start with treatment(typically beginning Urso at 15mg/kg/d).
Alicia Gilmore, a Registered Dietitian, led a breakout session answering questions and providing advice about what PBCers should and shouldn’t eat. PBCers were advised to avoid diets high in salt and fat and to avoid alcohol. She addressed how difficult it can be for many people to realize how much sodium, etc, is actually in their food, and suggested using an app, like Fooducate to help keep track of it all. For those who want to try a specific type of diet, she recommended they stay away with ketogenic diets and aim for a Mediterranean diet(high in vegetables and anti-inflammatory foods).
A Ursodial breakout session with a Q & A and group discussion led by Thomas Draime. PBCers discussed what time of day they take their medication, what food they take it with, and how some may have allergies to fillers used in medication, rather than the medication itself.
Sponsors of the events were presented with appreciation awards and PBCer of the year was announced (congrats Carol Roberts!)
Jennifer Pate, MD (AND a PBCer!) presented “Living as a Princess with PBC” in which she outlined how chronic illness can result in psychological issues that take a toll on people. PBC in specific, is difficult for people to explain to their peers, and when not understood, it might be assumed that PBC is a result of a drinking problem.
Fatigue can make some social activities limited and leave friends and yourself disappointed. Dr. Pate recommended some ways to manage fatigue; “Wellbutrin XL, Nuvigil or Provigil, stimulants (such as those used to treat ADHD, and if possible, changing when you take beta blockers to the evening can help. Plan activities for the best part of the day for you. Avoid napping so you don’t disrupt your sleep at night and optimize sleep hygiene. Exercise during the week, and avoid supplements and energy drinks.”
Dr. Pate mentioned that depression is “one of the most common complications of all chronic illness” but stressed that that doesn’t make it a “normal part of illness” or another symptom of having the illness itself. It is treatable and not something that should be accepted. treatment can improve quality of life. It can be hard to diagnose; possibly overlooked by doctors, possibly due in part to a medication, but Dr. Pate recommends seeking professional help when;
- Symptoms impair functioning
- Symptoms cause significant sleep disturbance or appetite change
- Depression interferes with compliance with medical treatment
- You attempt to self medicate through use of drugs or alcohol
- Suicidal thoughts
Some psychological treatments for depression that Dr. Pate recommended include reading, workbooks such as “mind over mood”, journaling, support groups, writing, pets, and exercise, among others.
“Depression is not a weakness.” – Jennifer Pate, MD
John M. Vierling, MD presented “PBC-AIH Cholestatic Variant or “Overlap Syndrome”. He cautioned that misdiagnosis of “overlap syndrome” are “rampant in clinical practice” and that “Diagnostic criteria based on disease specific pathogenesis is urgently needed.”
Sudhakar Venkatesh, MD, presented “MR Elastography of Liver”, informing the audience that an MRE provides a reliable evaluation of liver stiffness and is the most accurate test to detect fibrosis.
Marlyn J Mayo, MD discussed Pruritus in PBC. Pruritus tends to be worse in the evening and exacerbated by heat. For most who experience it, it disturbs their sleep. Pruritus does not necessarily correlate with disease severity. Trimming your nails can help reduce the damage you do while scratching and wearing loose, absorbent, clothes can help keep the itching from worsening as much in warm weather. Keeping your home environment cool and opting for cooler showers can help. In terms of medication, it was suggested that after trying Cholestyramine, Clestipol, patients investigate Rifampin, Naloxone or Naltrexone, and then Sertaline or Paroxetine.
John Campbell, PhD from GSK shared information about rare disease clinical trials and how they can differ from trials performed outside the rare space(smaller number of participants, different risk/benefit assessments etc) .
Kidist Yimam, MD, presented “Autoimmune Liver Disease and Associated Diseases” discussing autoimmunity in the families of PBC patients. In one study it was found to be, “common, with about ~14% first degree relatives suffering from autoimmune disease other than PBC, further supporting the autoimmune hypothesis (genetic predisposition).” Yet, ” individual autoimmune conditions however, generally seen at a markedly low frequency in first degree relatives than PBC patients themselves.”
Silvia Hafliger, MD, gave a presentation delineating what the “transplant journey” really entails and how to prepare for it. Patients all need a “transplant buddy” who attends appointments and helps them through the process. Patients need to be fully aware of what to expect and there is a lot to handle after the transplant itself has been performed; there are a lot of appointments the first few months after transplant, you will be on immuno-suppressants post-transplant, and you’ll be dealing with bills on top of it all!
Dr. Hafliger mentioned how beneficial support groups and peer counseling can be helpful in the context of transplant.
This is true within the context of chronic illness itself as well, and events such as the PBCers Conference can help facilitate some of those connections. You can see tweets from the conference by searching the hashtag #2018PBCConf on twitter. Check out PBC.org and connect with PBCers on facebook!
For info on clinical trials for PBC go to clinical trials.gov
Presentations will be available on PBCers.org in the future. For more info or any questions please contact me at [email protected] and I will do my best to get you in contact with the person who can help. Please remember Patient Worthy content is not meant to substitute guidance from your health care team.