Many of you already know that my garden is a fundamental aspect of my life. I find each dimension of the garden — the change of seasons, the plants that open in the morning and close at night, the patch of sunlight created when I lost a branch in the storm — to bring its own opportunity for grace. I noticed it this morning in particular, as this bouquet of lovely peach roses dropped its petals after a couple days in our 100 heat. The petals that lay on the counter top however, were the most intense yellowy-pink with deep magenta edges. It was magnificent, the dried, dying petals. Part of the grace that is delivered to me from the garden is a (sometimes forced) appreciation for what is unfolding. So, I’m excited to highlight here, for you, some of the changes that we’ve been putting in place at the IPPF and some of the dreams we hope to make into realities over the next year. I hope you’ll find them to be of benefit to you personally; that you’ll see how they help the IPPF serve more and better; and that you will consider sharing with me your expertise, provided from your perspective as a participant in this organization, so that we may serve in the most meaningful way possible. I know none of you would miss it, but please consider this a jumpstart encouraging you to read Janet’s letter on page 3. There she describes our Health Management Program, the most critical project we are engaged in for 2008. Please offer 30 to 45 minutes of your time to help us document a disease registry that will be the foundation for improved diagnosis, improved treatment protocols, and more extensive research. It’s a small legacy of time that you can leave which will make a huge difference for generations to come. Also, if you do business on the Internet, take a look at the more than 30 hours Will has spent organizing and simplifying our web site at (www.pemphigus.org). All the informational content remains, however the layout is expected to be more clear and intuitive. In addition, I hope you come to enjoy the brighter colors and our new logo, designed to prepare us to catch the attention of grantmakers and corporate partners and pharmaceutical contributors. Finally, we have already started preparations for the 2009 Annual Meeting which will be in Los Angeles April 25, 2009, it’s a great chance to learn, share, console, and laugh. Please continue to join us as we move forward to use the registry as a resource to pursue NIH funding, to train current practitioners in earlier diagnosis and more effective treatments, to support the next generation of dermatologists to gain experience, and to fund and support scientific advances in the effective treatment protocols. And continue to let us know what changes you’d like to see! Warm regards,
With the spectacular view from the 14th floor of the T. Boone Pickens Biomedical Building on the UT Southwestern’s campus, Dallas, the IPPF Annual Meeting opened with Dr. Grant Anhalt, Johns Hopkins. Dr. Anhalt helped new patients learn about their disease, giving an overview of autoimmunity, pemphigus, pemphigoid, treatments, and genetic triggers. Dr. Anhalt’s starts our program with his New Patient Orientation lecture, always with great success. It was an eye opener to many that autoimmune diseases are the 3rd most common group of diseases after heart disease and cancer, with billions of dollars being spent each year on treatments. He talked about treatment in general terms, how PNP’s disease looks and acts differently from PV and how all these diseases are clinically different, even though they seem so similar. He described the differences between the pemphigoid diseases as well; how bullous pemphigoid affects people of ages 60+ and that mucous membrane pemphigoid is considered more serious requiring early intervention for success.
Dr. Amit Pandya, our UT Southwestern host, officially opened the meeting at 9:15am, introducing David Sirois, IPPF Board President. Dave gave a brief introduction and thanked everyone — participants and speakers — for their enthusiastic participation. He talked about the new Centric/IPPF Health Management Program (HMP), and introduced two new Board members Rebecca Albrecht and Badri Rengarajan. In celebration of all her efforts for the IPPF Dave presented Janet Segall a certificate from the Texas Governor’s Office for celebrating the Annual meeting in Dallas.
The next speaker introduced by Dr. Pandya was Dr. Victoria Werth, University of Pennsylvania, Philadelphia; Dr. Werth discussed the project funded by the IPPF in 2007 which created the International Pemphigus/Pemphigoid Definitions Committee. The committee consisted of experts on pemphigus/pemphigoid from around the world, who worked together to establish standardized disease descriptions and definitions to allow worldwide documentation and to support a registry and better diagnoses. She explained how important it was to develop common language among researchers when describing disease what is complete remission, or what the definition of partial remission might be. The first results were described in review, and will be published later this year.
Dr. Werth went on to explain how the use of drug trials are important in helping doctors evaluate and recommend what drugs would be most appropriate to give to patients, in addition to finding new, less burdensome treatments. Recent trials showed that using IV steroids instead of oral steroids did not change outcomes. It has also been shown that that CellCept and Imuran are similar treatments; and new with treatments trials like remicade (infliximab) and the new P38 inhibitor can help evaluate how well a drug might work. Rituximab is now being used to treat pemphigus and pemphigoid, but a trial for PV has not yet been approved. Finally, trials also showed that in the Dapsone study with 9 patients, 73% of people got better and were able to reduce steroids in maintenance.
Patients did their part too, signing up for the new Centric/IPPF HMP (registry), and literally offering up their blood to help the IPPF and its research doctors aid the pursuit of more clinical trials.
Dave Sirois, and Craig Kephardt, CEO of Centric Healthcare, discussed how the questions were developed by the IPPF Medical Advisory Board to methodically register the parameters of disease and the results of treatment protocols, and how the HMP has the added component of providing resource materials for patients along with the opportunity to work with Janet as the peer health coach. This collective (and private) data will help measure the burden of our diseases and treatment outcomes. These data will help doctors and patients manage the disease in the future by minimizing the cost of care, increasing protocol compliance and allowing better communication between the individual and his/her physician.
With the IPPF’s movement into the future, the next to speak was the IPPF’s new CEO, Molly Stuart. Molly is a lawyer by trade, and much of the work she has been involved with was in the non-profit world, including as President of the Random Acts of Kindness Foundation. She asked the participants to educate and inspire her on the specifics of what they (you) see as the programs most meaningful to all.
Next, Dr. Animesh Sinha, Michigan State University, East Lansing, one of the only core researchers in the U.S. on pemphigus and pemphigoid discussed how relevant and important research is. He discussed how antibodies attack protein that holds skin cells together, which causes a split and blisters. He explained that they don’t know all the genes involved, but do know that HLA genes are important genes in regulating the immune system and balance. They found over 91% of patients had either the DR4 (seen in Ashkenasi Jews) or the DQ1 (found mostly in Asian ancestry patients). They looked at different genes and found that another marker Class 1 B and saw that protein HLA E might also be involved in disease development. These are just one set of genes and there are probably many, so they are still looking!! Given this genetic tie, the blood drive became a critical component of the weekend. The goal is a better understanding of the genetics so we can determine who might be at risk.
Although little known about why pemphigus and pemphigoid happen, we do know that there are also environment factors that work together to cause disease process. Researchers are looking for several different trigger factors and believe that investigating different phases of activity will help define how they might find ways from activity to remission. Much of this data will be provided with blood and tissue donations and with trends observed from the registry.
Dr. Pandya explained how because physicians must rely on evidence-based data to make decision on treatment, the decisions that are made in treatment are often based on the experiences of other physicians or from looking at trials with small numbers of patients. Because our diseases are so rare, the clinical data is limited due to lack of patients for clinical trials.
Outlining the steps to effective care, Dr. Pandya noted that it is important that the physician first make the right diagnosis and that it is best if a patient has a doctor who knows about the disease, so that the biopsies are done properly. He recommends that everyone have an internal medicine doctor to help monitor side effects. Other tips included:
- have good hygiene to keep bacteria away;
- remember infection is the greatest enemy of people with pemphigus;
- exercise is important to main muscle mass since prednisone causes weight gain;
- use roll gauze in wound care and avoid bandages with tape;
- use antibiotics and, if necessary, consider pain control as well.
The goal of treatment is to stop lesions and clear up the ones there. Some good news is that we do not see high mortality rates any more because there are so many better ways to treat drug side effects. He discussed management of drug therapies, and suggested that side effects will usually occur in patients taking steroids for more than one month. He made it clear that not everyone gets the side effects that are listed on medicine inserts. From a worldwide survey, the immunosuppressive azathioprine (Imuran) is used most often around the world.
Dr. Victoria Werth took the podium again discussing the risks of immunosuppressives, the side effects of drugs and how to minimize complications. Dr. Werth explained that taking steroids once a day may help with side effects and that the longer we take the drugs, more probable it is that we will have side effects. She believes that alternate-day dosing is best and a goal. She emphasized compliance, and said the best way to minimize side effects is with interventions.
- measure bone density, treat bone loss, and watch lipids and use medication that can help with side effects
- check glucose, cholesterol, triglycerides, and blood pressure
- visit the eye doctor and check for increase in eye pressure and watch for cataracts
- watch for signs of anxiety and depression — commonly experienced as a result of the medications
- do not stop prednisone without tapering
- do not get live vaccine if you are on 20 mg of steroids or more
- avoid activities that can contribute to osteoporosis alcohol, smoking, etc.
- if you are on immunosuppressives then get monthly blood tests
- check liver and blood functions
She encouraged all of us to eat a healthy diet low salt, low cholesterol, that exercise was critical, and that we should try to minimize weight gain and stress. She was adamant about keeping an eye on herbs that would enhance the immune system and possibly cause flares. Finally, she reminded that it is important to have a medical ID since emergency responders need to know you are on steroids, or have been on steroids within the past few months. Check with your doctor if you notice anything different or unusual happening.
We ate lunch, rested, connected. Door prizes were awarded by our new board member, a very enthusiastic Rebecca Albrecht-Oling. The Q&A followed lunch (see page 12).
Our next speaker, Dr. Kim Yancey, Chairman and Head of the Dermatology Department at UT Southwestern, Dallas, opened the session with a little history explaining that the distinction between pemphigus and pemphigoid was not really made until the 1940s & 1950’s. The focus of his session was Clues Regarding Ocular, Nasal, Laryngeal, Esophageal, and Anogenital Involvement. He gave us an in-depth look at eye involvement with OCP.
He explained that it often feels like sand or grit in the eyes. The eyes would sometimes be shut, and secrete mucous. He said the eyes would be sensitive to sunlight, and there is usually blurred vision. He explained that it usually will start in one eye, but will usually also affect the other as well. One should check for inward growing eye lashes, but if there are questions, a qualified ophthalmologist must look closely at the eye to decide whether the eye is involved.
Dr. Yancey then discussed the nose and nasal passages and that one of the signs of possible involvement was consistent nose bleeds and/or pain. He suggested regular nasal irrigation to keep the area clean, and recommended that the nurses were great resources for all our diseases and the areas affected. He then mentioned the other areas of possible involvement: throat, voice box, and esophagus. Signs to look for in MMP were hoarseness all the time, loss of the voice, severe pain, difficulty swallowing, or feeling like something was stuck in the throat. Sometime reflux could be a symptom, and a person with MMP should watch for problems with respiration. Dr. Yancey then discussed one of the more difficult areas of involvement genital and anal lesions. The signs to look for were pain and difficulty with bowel or urination. Bleeding could also occur. Erosions are more often found then blisters. Most patients with pemphigus or MMP will not have eye involvement, but some will.
Dr. David Sirois, Professor of Dental Medicine at NYU, New York, discussed the impact of oral lesions. His slides showed how the ulcer on the cheek would be white and not blistered. He explained how important correct biopsies were because if the site for biopsy was incorrect the results can be mistakenly negative for pemphigus or pemphigoid.
He emphasized dental care because gum disease can cause root degeneration and if left untreated can cause a patient to lose teeth. Complications from the drugs can affect the gums and teeth as well. He encouraged patients to watch for candidsis and to use high fluoride if root of tooth is showing. He mentioned that the best toothpaste for oral problems was Prevident 5000, a prescription toothpaste which can be helpful. He encouraged all of us to be educated on what to look for and that prevention is very important. The only solution to restoring teeth is implants and false teeth sitting on mucosal tissue is not good when a person has oral pemphigus or pemphigoid.
Our final speaker of the day was Terry McDonald, Ph.D., a psychologist who talked with us about living and coping with pemphigus and pemphigoid. As a pemphigus patient herself, Terry knows the emotional toll these diseases can have. She discussed how being diagnosed with pemphigus or pemphigoid raises feelings of loss and explained the different stages of loss that one goes through when diagnosed.
Terry talked about her own experience with pemphigus vulgaris and how critical it was to set small goals and to try looking at things differently. There are choices and changes one can make that would impact life. With a diagnosis like ours, we all need to make adjustments. Many need to work to deal with depression, need to find safety, or need to know you belong somewhere. For many, medication can be very helpful. Also, meditation, exercise, and nature can be helpful.
The day’s program ended with a final question and answer session with the doctors. The day was informative and interesting. All the speakers were wonderful, gearing their talks so that we all could understand the information. We left for the day, returning on Sunday.
Sunday opened with the final Q&A session. Caregivers went off together to talk about how they coped living with someone with pemphigus and pemphigoid, and patients got together to give each other support.
The IPPF would like to thank all the patients and patient family members who donated blood at the meeting to Dr. Ani Sinha’s ongoing research into our diseases. By being a part of this project we have a much better chance of finding answers. We hope to continue to support Dr. Sinha’s research at subsequent meetings. Also, cheers to everyone who signed up for the HMP registry. Don’t forget to get your data in!
The 50/50 drawing was won by Dr. Kim Yancey who, in turn, gave his winnings back to the IPPF. We thank Dr. Yancey for his generosity.
The dinner program began with drinks, hors d’oeuvres, and good conversation, then a terrific (big) Texas-style meal. Certificates of Appreciation went to Joyce Korn for her help in finding the hotel for our dinner and the rooms at great prices, and to Dr. Amit Pandya and Dr. Kim Yancey for the major roles they played in making this meeting a success.
Janet Segall presented a Lifetime Achievement Award to Siri Lowe, PV Network founder from the United Kingdom for her dedication to patients and patient advocacy for more than 10 years in the UK. Unfortunately Siri could not be present to accept her award. Janet, to her surprise, was presented with the inaugural Founder’s Award for her efforts over the years on behalf of patient care. A rousing standing ovation followed. Janet was very humbled and still passionate about contributing more. The final award was given to Dr. Robert Jordon, UT Southwestern-Houston. Dr. Jordon along with Dr. Ernest Beutner, SUNY Buffalo pioneered research on immunofluoresence and pemphigus. The IPPF honored Dr. Jordon’s contribution to Immunodermatologic research and his many years of treating and helping blistering disease patients.
Dallas lived up to expectations and has been one of our best meetings ever. Remember to mark your calendars for April 25, 2009 as Los Angeles will be the location for the 12th Annual Patient/Doctor Meeting. We look forward to seeing you there!