When I was diagnosed some 30 years ago, prednisone was the only drug being used. Imuran had just started appearing on the scene. My doctor didn’t know if it was an option, so we tried methotrexate; and that caused too many problems. Being diagnosed with PV at 37 was not something I handled very well. It took time. A lot of it. I was living in Berkeley, California, and alternative medicine was start- ing to become very popular in the ar- ea. I was having difficulty handling prednisone so I started augmenting my healing with Chinese medicine, Bio- feedback, Linseed oil, and soybeans. I tried tea that tasted and smelled horri- ble (my roommates were not too hap- py about that). I drank a tablespoon of Linseed oil every day, and cooked raw soybeans that I made into soybean pat- ties and ate twice a day. I don’t know scientifically if any of the things I did back then worked, but they helped me live better with prednisone. Along the way, I learned some incredibly helpful relaxation techniques I still use to this day. After many years living and learning as much as I could about the diseases, I know it is important for someone with pemphigus or pemphigoid to avoid things that might enhance the immune system, such as alternative medicines, vitamins, or foods rich in spirulina and echinacea. These boost your im- mune system and can negate the ef- fects of immunosuppressive drugs being used to get disease activity under control. Typically a person uses alter- native medicines and diet to try and stabilize the immune system. My per- sonal advice is that if you decide to try Chinese medicine, make sure none of the ingredients will harm you in some way. A specialist should know the right combinations to use but always discuss this with your physician BEFORE mak- ing any changes to your diet. Dr. Sarah Brenner (Israel) conducted small studies several years ago on certain foods and how they affect pemphigus and pemphigoid patients. Even though they were not double- blind studies and were very small in size, she was able to theorize that foods from the Alum group might cause problems for people with pemphigus. These foods included onions and gar- lic. Mediterranean countries tend to eat a lot of onions and garlic, so deter- mining if they pose a problem for you might be challenging. I am very fond of both onions and garlic so I decided I would test myself. At the time I was clear of lesions but still on drug therapy. For three days, I ate two small cloves of garlic a day on “rice cake pizzas”. Three days later I no- ticed I had a couple of new lesions. I stopped eat- ing the garlic and two days later the lesions disap- peared. I waited several weeks (enough time for the garlic to leave my system) and I tried it again. The same thing happened, as well as a third time.
I came to the personal conclusion that eating a large amount of garlic consistently was not a good idea for me. I found the same thing happened with large amounts of onions and, believe it or not, sour- dough bread. I discovered testing foods myself less- ened my overall disease activity and allowed me to identify what foods and approximate what amounts were going to result in lesions. One of the most helpful things I tried was bio- feedback and Transcendental Meditation. Before I was diagnosed with PV, I went to the Transcenden- tal Meditation Center in San Francisco and learned some methods for relaxation. Biofeedback showed I was able to significantly reduce my stress quotient. Because of that early success, I recently became cer- tified in Hypnotherapy. Now, I use hypnotherapy on myself and to help others reduce stress. There isn’t any disagreement that stress reduction is an important part of healing from disease — not just pemphigus and pemphigoid, but any disease. Dr. Terry Wolinsky McDonald (IPPF Board of Direc- tors and a regular Quarterly columnist) has written many articles on coping with stress. I strongly agree with her that it isn’t about the stress itself as much as it is the way we deal with the stress. There are ma- ny different methods of stress reduction so finding one that fits into your life is a key to effective heal- ing and coping. Sometimes being proactive with disease can be difficult. Once the medicine starts to work, many people want to pretend nothing was wrong in the first place. Some simply take their medicine, eat whatever they want, and avoid physical activity. I’m guilty! We just pretend we’re fine hoping a positive attitude will be enough. Having a positive attitude is extremely important, but pretending we don’t have to take care of ourselves can be hurtful. The drugs for these diseases can cause damage to our bodies and a proactive, positive attitude can help promote healing faster. I have been in remission now for about 15 years, 10 drug-free. I watch my eating habits and notice any changes certain foods cause. Taking prednisone off and on for 10 years has had a negative effect on my spine and joints, but I find ways to exercise: mild yo- ga, walking, hot pool aerobics, and stretching — and last but not least — relaxation. I believe a comprehensive approach to healing not only helps us heal our disease, but can help us keep a positive attitude as well.
Here are some Fast Facts from the Registry:
• 73% of all P/P patients are female
• 65% of all P/P patients have PV (11% have BP) • 11% also have thyroid disease (5% have rheu- matoid arthritis)
• 70% see only a dermatologist — even though 41% have current lesions in their throat/mouth.
As you can see, the information is compelling, but if this does not reflect you, then we need your data! The data can be segmented by gender, age, and disease type (see A Look at PV from Inside the Regis- try on the previous page). The goal of 1,000 will make our Registry the largest collection of pemphigus and pemphigoid data available to researchers. Please do your part to help patients everywhere. For more information, or to participate, please visit www.pemphgius.org/registry.
During that time, Isabelle became active in the French support group. I was determined to reconnect with these special people.
The week after visiting Hermien, Berna and I traveled to Paris and to meet with Isabelle and Oceane. I quickly realized that after 8 years, young children really grow up. When I first met Oceane, she had been diagnosed with PV for two years and her doctors were worried about her being on systemic drugs at such a young age. Oceane had written about the difficulties she en- dured, even having to soak in
a tub at night just to remove her clothes. The good news is Oceane just turned 16 and is doing great! Isabelle shared some literature and material from their support group as we walked around Paris (Oceane and I definitely share a sweet tooth). It was a fun adven- ture where time passed too soon for our liking. Oceane gave my friend and me special cups used for relaxation in a Paris bistro- type of atmosphere. Saying good-bye was difficult
and emotional, but I am grate- ful to have met these wonderful people. The “I” in IPPF stands for International making these face- face meetings truly special times for those of us who are able to connect — and reconnect. These friendships carry the same un- common bond that brought us together. Crossing the “pond” was — and will continue to be — a very important part of my life. How lucky am I? My advice to you is if you have an opportunity, grab it!
- Drink liquids through a straw.
- Cook coarse or hard foods, such as vegetables, until they are soft and tender.
- Soften or moisten foods by dipping them in gravies or cream sauces.
- Sip a beverage when swallowing solid food.
- Eat small meals more often instead of one large meal.
- Rinse your mouth with water while eating (or use water, peroxide, or Biotene afterwards).
• Remove food and bacteria to promote healing.
Having oral lesions can present many challenges including; pain manage- ment, oral hygiene, nu- tritional intake, and your overall health. Speak with your doctor about ways to help relieve the pain. Also make sure they monitor your blood sugar levels if you are taking systemic steroids. Don’t for- get to inform your dentist of your condition and ask them to use caution when treating you. If you have difficulty swallowing, or find yourself frequently choking on food, talk to your doctor. You may want to ask to be seen by an ENT to help deter- mine the extent of your disease activity. With pemphigus and pemphigoid, the mouth is one of the most difficult areas to treat and requires due diligence. Changing your behavior and habits can be the biggest “pain” but will eventually pay off. If you need help, encouragement, or suggestions… just “Ask a Coach!”
The information we have is compelling, but more information is better. I ask each of you to take 15 minutes and complete the Registry. We need FEWER THAN 300 more people to surpass 1,000 participants and give researchers a better understanding of P/P. Your contact information is NEVER shared and when we look at the responses, all we see is a number, not a name; a state/province, not an address. Can YOU help us pass 1,000 by the next issue? I think you can at www.pempihgus.org/registry. The 2013 Patient Conference (formerly the Annual Patient/Doctor Meeting) will be in San Francisco, April 26-28, 2013. The Planning Committee is looking to capi- talize on the success in Boston — and we think San Francisco is the place to do it. If you have never been to the Bay Area, there is no better time that April 2013. And if you live in the Bay Area, join us at Stanford September 29, 2012 for the Bay Area Sup- port Group Meeting (11 am – 2 pm, pemphig.us/ippf-basg-201209). The Holiday Fundraiser will be gearing up in a couple of months and I am challeng- ing each and every one of you to find one person to match your donation. Imagine if 2,000 people gave just $50 — and their one friend gave $50. These donations will af- ford us the opportunity to have more Peer Health Coaches to answer your questions, more informational materials for physician offices, more educational materials for patients – and the list goes on … but we need your help to make 2013 bigger and bet- ter than 2012. And don’t forget you can now give a monthly amount that is automat- ically deducted from your credit card. How about giving $25 a month instead of $100 all at once? It’s just as easy and makes a big impact for us – and a bigger tax deduc- tion for you! You can always donate online at www.pemphigus.org/donate. Thank you for your continued support and we hope you enjoy this issue.
Our Gold Sponsor, AxelaCare (www.axelacare.com), has been a sponsor since 2009 and Mr. Brian Cleary has helped many pemphigus and pemphigoid pa- tients with insurance-related is- sues. Our Silver Sponsors were Cres- cent Healthcare, Inc. (www. crescenthealthcare.com), PNC Wealth Management (www.pnc. com), and NuFactor Specialty Pharmacy (www.nufactor.com). Crescent has been an IPPF spon- sor since our 2006 Annual Meet- ing in New York. Both PNC and NuFactor are new sponsors and the IPPF welcomes them to our family. Our Bronze Sponsors were BIOFUSION (www.biofusion. com), Centric Health Resourc- es (www.centrichealthresourc- es.com), and the Massachusetts Eye Research and Surgery Insti- tution (MERSI, www.mersi.com). Biofusion first sponsored the IPPF back in 2003 and has been a valued partner ever since. Cen- tric has been a sponsor since 2006 and helped develop the free IPPF Health Management Program (www.pemphigus.org/hmp). The IPPF welcomes MERSI as a spon- sor this year and looks forward to the future. The Blistering Disease Sup- port Group from Boston con- tributed hundreds of hours of work to help with planning, din- ner, and entertainment arrange- ments; packaging thousands of product samples; and greeting at- tendees to the meeting. A special thanks goes out to the Stillman’s and Peckrill’s for their leadership of this massive project. The Bos- ton Support Group, led by Alan Papert, helped with site surveys,
event planning and A/V coordi- nation. Alan’s wife, Gloria Papert, was her usual self helping with the registration table, answering attendee questions, and making the event an enjoyable one for ev- eryone. Thank you, Alan and Glo- ria, for your support. Bruce Heath, Vicki Garrison, Phyllis MacPherson, Dr. Shawn Shetty, and Dr. Marsha Fearing from Dr. Ahmed’s office helped with everything from sending let- ters and flyers, to attendee and speaker reservations and regis- tration. And the muscle behind the event, Ike Mahmood and Gh- ulam Ali, helped package, load and transport over 2,000 pounds of donated products. Lastly, Kendra Smith, Con- vention Services Manager at the Hyatt Harborside, worked with the IPPF Annual Meeting Com- mittee to make the impossible possible. Thank you everyone for mak- ing this THE meeting that has raised the bar to a new level. The IPPF invites this year’s attendees, and the entire pemphigus and pemphigoid community, to join us April 26-28, 2013, in San Fran- cisco for the 2013 Annual Meeting (information will be sent out lat- er this year).
Attendees included patients in remission for 20+ years, to a patient diagnosed a few days before the meeting started. If you were there, then you already know how incredible this meeting was — and the fireworks were truly a coincidence!
If you were not able to attend, then you missed a doozy, but fear not: everyone can visit www.pemphigus.org/2012am and get copies of the program, presentations, and available audio. I would like to personally thank everyone who helped make this meeting possible – and the success it was! Turn to page 7 and see who made the 2012 Annual Meet- ing THE meeting to top! And don’t forget to mark your calendars: the 2013 Annual Meeting will be in San Francisco, April 26-28! Dr. Terry Wolinsky-McDonald’s “Psychologically Speaking” column will return next issue. If there is a topic you would like her to cover, please email her at firstname.lastname@example.org. As we move into the second half of the year, the IPPF is focusing on in- creasing physician awareness. Remember when you, or someone you know, was first diagnosed with pemphi-what? Were you one of the lucky ones who was diagnosed in less than a month? Or even less than three? Most are not that lucky.
In 2011 the IPPF funded a study to determine how long it takes new patients to be diagnosed. The results indicat- ed patients see an average of 5 doctors over a 10-month span before receiving a proper P/P diagnosis. This does not have to be! With the right information available to physicians, patients could be di- agnosed in days or weeks, not months or years. Our goal is to increase pemphigus and pemphigoid awareness in the medical professions. During March 2012’s National Autoimmune Disease Awareness Month, we raised almost $10,000 towards P/P awareness. In April 2012, the IPPF was awarded a $15,000 grant from the Sy Syms Foundation to help with our Awareness Campaign! And recently, a special do- nor has pledged even more towards our awareness efforts! But we are not there yet! Awareness campaigns DO work! To get the most coverage among dermatol- ogists, dentists, and other specialties, we need your help! This summer we are asking everyone to con- sider a tax-free contribution specifically for our Awareness Campaign. You can donate online at www.pemphigus.org/donate, call our offices at (916) 922-1298 ext 1003, or mail in the form located inside the back cover. Please consider asking a friend or family member to support this very important effort. And don’t forget about Matching Gifts from your employer! Con- tact your Human Resources department for more information. The sooner we can fund this project, the sooner we can increase physician awareness — and the sooner pemphigus and pemphigoid patients will be diagnosed and treated. That means patients can begin their journey to recovery and remission sooner than ever before! Finally, thank you for your continued support and have a safe and happy Summer!!
Next, IPPF President Dr. David Sirois (New York University) began with a cheerful welcome and an update on the IPPF. As an ultra-orphan disease sup- port organization, the IPPF “sees its primary mission to connect together the different parts of the com- munity that together can give [patients] a better ill- ness experience.” Dr. Sirois invited attendees to par- ticipate in Town Halls, Annual Meetings, the Email Discussion Group, Patient Forums, Facebook, the Pemphigus & Pemphigoid Disease Registry, and IPPF studies and surveys. He then discussed the results of a recent IPPF study that showed delays for initial diagnosis, including a patient seeing five physicians over a 10-month pe- riod.
Dr. Sirois al- so discussed the cor- nerstone of the IPPF’s 2012 efforts: the IPPF Awareness Campaign. This program will focus on physicians in training and in practice to increase recognition of these diseas- es, provide guidelines for treatment and care, and bring new clinicians into medical dermatology committed to bullous diseases through fellowships and scholarships. The opening talk was given by Dr. Grant Anhalt (Johns Hopkins University), who provided a brief overview on pemphigus and pemphigoid. Dr. Anhalt was instrumental in helping Janet Segall found the National Pemphigus Vulgaris Foundation in 1994 (today known as the International Pemphigus Pemphigoid Foundation). Dr. Anhalt’s discussion covered how the immune system uses antibodies against antigens. He also mentioned that autoim- mune diseases are the third most common group of diseases behind cardiovascular diseases and cancer. Dr. Anhalt mentioned how none of the drugs used to treat the disease can be used to treat the target area, but must focus on getting rid of the antibody produced by the immune system. Next, legendary pemphigus and pemphigoid phy- sician Dr. Samuel Moschella (Lahey Clinic Medi- cal Center) told attendees what it was like to treat pemphigus before prednisone was available. His stories of varying treatments and therapies of pemphigus vulgaris malignus were met with silence as he mentioned “70-90% of these patients died from this disease” as a result of infection, malnu-trition, or other elec- trolyte and protein problems, and how it was treated much like burn victims. As Dr. Moschella’s story moved along his time line, the introduction of better therapies and an increased understanding by physicians improved treatment. The first dental talk of the day was given by Dr. Sa- dr Kabani (STRATA Oral Pathology Services) on the oral manifestations of pemphigus. Dr. Kabani men- tioned pemphigus may begin with canker sore-like lesions on a localized area of the gums or inside of the cheek, that can become progressively worse. A com- mon site of involvement is the soft palate. Gum in- volvement is common and might be the only manifestation. Diagno- sis is based mostly on clinical presentation, but must be confirmed by a biopsy and immunoflorescence. Dr. Sook-Bin Woo (Harvard Dental School) dis- cussed the clinical presentation and diagnosis of pemphigoid in the oral cavity. Dr. Woo opened with how pemphigoid is of- ten associated with eye and skin lesions, but more often than not it is associated with purely the oral region, and typically female. She mentioned that blisters are not commonly visible because they rup- ture frequently, so peeling mucous membranes are a good indication. Dr. Woo said a biopsy is a must in properly diagnosing pemphigoid in the mouth. She said 95% of her patients are oral only without skin or eye involvement, but does not rule those areas out until af- ter the patient sees a dermatolo- gist and ophthalmologist. Attendees were then free to at- tend one of seven breakout ses- sions, or workshops. The morn- ing’s sessions focused on oral issues, topical management, and coping with pemphigoid. After- wards, everyone enjoyed a scenic luncheon outdoors in the Grand Pavilion before returning for an afternoon of systemic informa- tion. Dr. Kunal Jajoo (Brigham and Women’s Hospital) began with a talk on esophageal involvement of pemphigus and pemphigoid. The diagnosis of esophageal in- volvement involves radiology and endoscopy. In the majority of pa- tients he cares for, the primary disease has been diagnosed, so he focuses on if there is esophageal involvement using tests like the Barium Swallow (a patient drinks a chalky milk-like substance com- prised of a metallic compound that shows up on x-ray). He also mentioned careful consideration must be given before biopsying the esophagus so not to cause fur- ther damage, unless the diagno- sis is uncertain or the results will change disease management. Next was a presentation on oc- ular involvement by internation- ally recognized eye specialist Dr. C. Stephen Foster (Massachusetts Eye Research and Surgery Insti- tute (MERSI) and Harvard Med- ical School). He gave a basic over- view of the eye, and how the bulk of the oc- ular area is not af- fected by the blis- tering, but how it is focused on conjuncti- va (the lining of the eyelids and the whites of the eye) and the cor- nea itself. Unlike the skin, the eye is very unforgiving of chronic in- flammation and, therefore, thera- py should be aggressive for MMP patients with eye involvement.
Vice Chair of the IPPF Med- ical Advisory Board Dr. Sergei Grando (University of Califor- nia – Irvine) spoke about system- ic corticosteroids and if they are “friends or foe.” It is important to learn that prednisone can be your friend. It has reduced the mor- tality rate of pemphigus to 5-12% when used with a steroid sparing agent/reg- imen such as cytotoxic drugs, pro- tein inhibi- tors, or IVIg. Dr. Grando pointed out that prednisone mimics the body’s production of cortisone acetate (the adre- nal gland produces 35-40 mg/ day, which is equal to 7-8 mg of prednisone). However, when doses are too high or too low, or non-responsiveness is not recog- nized, systemic corticosteroids can become a foe.
This can lead to enhanced appetite, fluid and salt retention, emotional disor- ders, diabetes, hypertension, and more Our first international plenary speaker was Dr. Richard Groves (St. John’s Institute of Derma- tology, London). He presented on immunosuppressive agents, and when a physician should choose which one when treat- ing pemphigus and pemphigoid. Dr. Groves said the aim of adju- vant immunosuppression is to achieve great disease control with minimal adverse effects. These steroid sparing agents include azathioprine, mycophenolate mofetil, cyclophosphamide, sulfa drugs, and tetracyclines, among others. Dr. Groves continued with a detailed discussion on effective options, treatment based on the diagnosis and severity, genetics, and how adverse effects are well understood and controllable. Dr. A. Razzaque Ahmed (Center for Blistering Diseases) returned to the stage to discuss IVIg and rituximab use in autoimmune blistering diseases. These treat- ments are an area of controver- sy and lack uniformity of opinion and therapy. Dr. Ahmed talked about the IVIg treatment proto- col developed by 35 experts from the US, Canada, and Europe (pub- lished in 2003). He stressed with IVIg “there is an endpoint to the therapy…there is light at the end of the tunnel.” Dr. Ahmed stated IVIg and rituximab, alone or in combination, have shown signif- icant benefit and newer diseases’ specific biological agents will be discovered once there is a better understanding of the pathogene- sis of blistering diseases.
Next began the afternoon breakout session (workshops) focused on side effects, ocular pemphigoid, IVIg, genetics, treat- ing blistering diseases differently, and coping with pemphigus. This was followed by a short question- and-answer session with the day’s speakers (see page 14 for some of the questions). SUNDAY Sunday brought out the scien- tist in everyone as the speakers discussed updates, advances, and new therapies. Opening the day was IPPF Med- ical Advisory Board Chair Dr. Vic- toria Werth (University of Penn- sylvania) providing an update on the classification of pemphigoid. Since pemphigoid is a sub-epi- dermal blistering disease, there is a need for standard terminology and severity measures. Based on the number of studies and lack of uniformity among terminology, it is nearly impossible to compare therapeutic outcomes using sim- ple meta-analysis. Currently, sev- eral bullous disease experts are working on a Bullous Pemphigoid Disease Area Index (BPDAI) to standardize scoring and termi- nology, thus making it easier for scientists, researchers, and clini- cians to share information. How- ever, further studies are needed to validate this information, as well as validation of eye and ENT scores. Next, Dr. Ahmed introduced Dr. Grant Anhalt as the “Grandfather of Paraneoplastic Pemphigus,” a title unofficially bestowed upon him since he was one of the first to describe it in 1990. During his talk on PNP, and using several index cases, Dr. Anhalt discussed the history, exam, and treatment plans needed to help the patients. He said most PNP cases are mis- diagnosed as chronic erythe- ma multiforme, toxic epidermal necrolysis, and combined lichen planus – and he estimates as many as 75% of cases are still not prop- erly recognized or diagnosed. Dr. Anhalt then discussed why PNP looks and acts differently than pemphigus vulgaris, and how the mortality rate is nearing 90% be- cause it is the most treatment-re- sistant disease. Dr. Peter Marinkovich (Stanford University) discussed laminins in skin diseases. Dr. Marinkovich noted that Laminin-332 is ab- sent in a severe, inherited blis- tering disease. Laminin-332 is an important adhesion molecule and target- ing the en- tire protein would cause widespread blister- ing. Therefore, selectively target- ing Laminin-332’s carcinoma pro- moting regions does not interfere with tissue adhesion. Dr. Marcel Jonkman (Univer- sity of Groningen, The Nether- lands) presented on the patho- genesis of bullous pemphigoid. While pemphigus has document- ed findings dating back to 1768, pemphigoid was not indepen- dently distinguished until the ear- ly 1950s. Dr. Jonkman discussed the histopathology, diagnostic al- gorithm, and autoantigens of BP. He also mentioned the role of IgE and how it is faintly detectable in the epidermal basement mem- brane zone (BMZ) of a BP patient, but strongly detectable in the BMZ of a skin organ culture. Our last international speaker was Dr. Michele Mignogna (Uni- versity of Naples, Italy) who presented on his 20+ years of treat- ing patients with oral pemphigus and pemphigoid in Naples. Dr. Mignogna has used a blend of conventional methods and newer strategies. He talked about the differences between treating pa- tients in the United States vs. Ita- ly.
Generally, Dr. Mignogna prefers to use rituximab (and IVIg in severe cases) where they can only be used in medical facilities. He then men- tioned that the use of rituximab and IVIg do not require insurance com- pany approval in Italy, but the ap- proval of the hospital committee, and are no cost to patients. Our final speaker of the conference was Dr. Sergei Grando, who returned to the podium to discuss new immunosuppressive drugs for blistering diseases. Dr. Grando discussed the therapeutic ladder for PV and the treatment algorithm for pemphigoid before mov- ing on to cytotoxic drugs. He referenced a study that concluded the “most efficacious cytotoxic drug to reduce steroids was found to be azathioprine.” He mentioned that all treatments have side effects, some of which can be serious, and treatments that work rapidly have the most serious side effects. Dr. Grando discussed the current challeng- es and said that current drugs suppresses all immune responses—good and bad—result- ing in unnecessary side effects. Ideally, se- lective immunosuppressive treatments that could suppress only pathogenic responses would be developed.
This year’s Annual Meeting would not have been the success it was without the present- ers who volunteered their time. The IPPF would like to thank each and every speak- er for helping make this year’s meeting the best ever!