Every day, our patient services team hears stories from our community about what it’s like to live with pemphigus and pemphigoid. From getting diagnosed to finding the right doctor to thriving post-treatment, many patients express similar frustrations. And yet, there’s a common hope that runs through many of the stories we hear at the IPPF.

Each week through August and September, we’re featuring a story that highlights a specific part of the patient journey. OUR HOPE is that by sharing stories from our community, more patients and caregivers will realize they are not alone.

Our third story in the Patient Journey Series comes comes from IPPF Peer Health Coach, Mei Ling Moore:

I’ve been a peer health coach (PHC) with the IPPF since 2012. However, if you had told me back in early 2001 that this is what I would be doing today, I never would have believed you. I had never even heard of pemphigus vulgaris (PV) then, much less known about rare autoimmune diseases.

I’ve always believed that everything happens for a reason and that no matter how good or bad it is, there is a destiny in the event. When I finally was diagnosed in February 2002, I didn’t think, “Why me?” Instead, I thought, “Why not me?” I also couldn’t figure out why I thought that! When I reached remission 10 years later in 2012, I was asked by the IPPF to become a PHC.

My journey with PV started in early October 2001. A friend and I were out to dinner one night. While we were waiting for the valet to bring us our cars, I felt an itch and a tiny bump on my upper back. I asked my friend to look at it and whether it looked like a bite. She thought it did. When I went home, I put some aloe with vitamin E on the bump and covered it with a small bandage. The next day there were two more bumps. In the ensuing weeks, I would find about 10 more bumps. Pretty soon, the bandages weren’t large enough to cover them. They grew so big that even a gauze square wasn’t enough to cover them. I bought Telfa pads and paper tape, because it turned out I was allergic to adhesive as well. These “bites” kept growing. My upper back was completely covered, it was raw, oozing, and extremely painful. It looked like I had open wounds from being hit by shrapnel.

Clothing hurt. A strand of hair touching my back felt like a razor blade was cutting into me, so I had to cut off all my hair. I couldn’t shower because the water hitting my skin hurt. Positioning myself on my mattress was painful and took fifteen minutes. Sleeping was a challenge because of the pain and my skin sticking to the sheets. Three months of misdiagnoses and money spent on the wrong medications prompted anxiety, fear, and anger.

My dermatologist, Dr. David Rish in Beverly Hills, was out of town for the holidays, so I saw three of his colleagues who, month after month, kept diagnosing me without success. When Dr. Rish returned at the beginning of January, he said, “I think I know what you have,” and he sent me to a phlebotomist to have a blood test. The phlebotomist had to make a special phone call to her boss asking what to look for since she had never done that type of blood draw before. Dr. Rish also told me not to go on the internet. Who would listen to that? When I searched for pemphigus, I panicked. I read that there was a five-year mortality rate. Did that mean that I had five years left to live? I went into an immediate emotional dive.

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I was finally diagnosed by an associate dean of dermatology at UCLA in February 2002 and was started on 100mg of prednisone. Sadly, this doctor passed away two months after seeing me, and I no longer had a doctor to treat me for this scary condition. Dr. Rish kept refilling my prescription while I went on the hunt for a doctor who could treat me. I found the IPPF online, joined the email discussion group, and went to a local support group meeting with a dermatologist as the guest speaker. I also met Janet Segall, the founder of the IPPF.

I started seeing the doctor from the support group meeting, and she kept me on prednisone. The blisters had spread from my upper back to my scalp, and then to my mouth. Three or four months passed before my gums started to peel back. I was scared I was going to lose my teeth. Then the blisters started under my tongue and inside my cheek. Eventually, blisters were in my larynx. I sounded like a frog, and I couldn’t swallow without pain. Ensure was my breakfast, lunch, and dinner for a few weeks. To this day, I still have to cut out clothing labels from new clothing as they irritate my skin, and I hardly ever go out in the sun as too much exposure can lead to a disaster.

In the beginning, my dentist said he had vaguely heard of PV. He didn’t know very much about it and didn’t seem interested to learn more. My dental hygienist had never heard of PV either, and I explained it to her. I asked her to be very careful with the instruments. She tried, but there was quite a bit of flinching. I kept educating each new dental hygienist, as they rotated and worked various clinics at once.

Because I was hurting from PV, I rarely went out and cut back on seeing friends or going to temple. I became quite isolated. However, I stayed active online with the PV discussion group, and whenever there was a support group meeting, I attended and helped out.

I attended my first IPPF Patient Education Conference in Los Angeles in 2007, followed by the conference in San Francisco in 2013, and I was very involved with the IPPF whenever I could help out. I have been in remission since September 2012 and have not had an episode since.

I love what I do as a PHC. I remember what it was like for me when I was first diagnosed with PV, so I can empathize with the patients who contact the IPPF. Along with IPPF Executive Director, Marc Yale, I also organize patient support group meetings for Southern California patients, and I regularly give a workshop at the Patient Education Conference on how to de-stress. I feel blessed to be able to help others and give back in service the same kind of support I was given. It helped me survive this rare autoimmune disease. I can’t imagine doing anything else with my life right now. I am blessed to be a part of the excellent IPPF team.

Your donation helps patients like Mei Ling connect with the resources they need to live—and thrive—with pemphigus and pemphigoid.

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Check out the rest of the Patient Journey Series:

On Thursday, June 7th, the FDA approved Rituxan for the treatment of adults with moderate to severe pemphigus vulgaris (PV).

Rituxan is the first biologic therapy approved by the FDA for PV and the first major advancement in the treatment of PV in more than 60 years. The FDA previously granted Priority Review, Breakthrough Therapy Designation and Orphan Drug Designation to Rituxan for the treatment of PV. With this decision, Rituxan is now approved to treat four autoimmune diseases.

“It is our hope that this announcement will open the door to approval for other indications in our diseases and usher in a renewed focus on available treatments,” said Marc Yale, Executive Director of the International Pemphigus & Pemphigoid Foundation.

The FDA approval is based on data from the Ritux 3 trial, a Roche-supported, randomized, controlled trial conducted in France that used Roche-manufactured, European Union (EU)-approved rituximab product as the clinical trial material. The study compared the Ritux 3 regimen (EU-approved rituximab product plus short-term corticosteroids [CS]) to CS alone as a first-line treatment in patients with newly diagnosed, moderate to severe pemphigus. The primary endpoint of the study was complete remission at month 24 without the use of steroids for two or more months. (Complete remission defined as complete epithelialization and absence of new and/or established lesions.)

Results of the study showed that 90 percent of PV patients treated with the Ritux 3 regimen met the endpoint, compared to 28 percent of PV patients treated with CS alone. These results supported the efficacy of Rituxan in treating patients with moderate to severe PV, while tapering off of CS therapy. These results were published in The Lancet in March 2017.

An international panel of experts called the International Bullous Disease Consensus Group recently provided new recommendations on the diagnosis and management of pemphigus in the Journal of the American Academy of Dermatology. Based on existing European treatment guidelines, a Delphi survey process was used to help achieve international expert consensus. The consensus includes the recommendation to use an anti-CD20 monoclonal antibody (Rituxan) and corticosteroids as first line therapy options for moderate to severe pemphigus.

The Role of the IPPF

The IPPF aims to serve as a primary source of information for you regarding this approved treatment and is available to help answer your questions in the upcoming months. If you are considering Rituxan as a potential therapy, please consult your healthcare provider. Inform them of your medical history, and ask about the potential side effects.

The IPPF’s Peer Health Coaches (PHC) are pemphigus and pemphigoid patients who help more than 1,200 patients and caregivers each year. These specially trained PHCs reduce patient anxiety and uncertainty while providing unbiased disease and treatment knowledge. You can find our PHCs engaging the community through social media, emails, phone calls, and in-person support. The goal of our PHC program is to ensure we help every person who needs assistance in the shortest amount of time possible.

Genentech Access Solutions

Genentech is the drug company that produces Rituxan (rituximab). Genentech Access Solutions is a resource for people considering Rituxan as a treatment option. It may be worth contacting Access Solutions directly regardless of whether or not you have health insurance.

Access Solutions may be able to help by:

  • Checking your insurance coverage and costs
  • Helping you find ways to pay for your medicine
  • Working to get your medicine to you

Visit Access Solutions to learn more.

Having a flare after being in remission can be a scary and frustrating experience. Thoughts run through your head about your previous experiences and you may wonder if your disease will be as bad as it was before. When you have the flare, it is important to recognize it and take the challenge head-on. It’s easy to become stressed from the uncertainty and lack of control, but remember that stressing will only make things worse. Here are some tips to reduce the intensity and time that you may have the flare.

1.      Schedule an appointment with your doctor immediately.

2.      Have your doctor give you a clinical diagnosis or get a biopsy done to confirm the flare. There are many differential diagnoses for your disease so you want to be sure it is what you suspect.

3.      Discuss with your doctor a treatment strategy and begin right away.

4.      Track your disease activity in a log, this will help you determine if you condition is improving.

5.      Follow up with your doctor regularly and advocate for yourself. Seeing your doctor every 4-6 weeks is recommended. If you have an aggressive flare you may need to see your doctor more frequently.

6.      If you need support, contact the IPPF and talk with a Peer Health Coach. Coaches are available to answer questions and help you decide how to best handle your flare.

It is common for flares not to be as intense as your first experience with the disease, but all patients have different experiences. The important thing is to be proactive and stabilize the disease activity as soon as possible. Flares are part of living with pemphigus and pemphigoid but if they are handled quickly and with a positive attitude you can eliminate them sooner.

Remember, if you have questions to “Ask a Coach” because when you need us we are in your corner!

All it takes is the slightest bump up against an object, just a few too many minutes in the sun, eating something that is hard and sharp or even the force of water pressure coming out of your shower head to cause trauma to your skin tissue.  This trauma creates a reaction in your body’s immune system and before you know it a blister or lesion has appeared. So does this mean that you can go out in the sun or do normal activities that most people do? No, but as a patient with pemphigus or pemphigoid it is recommended that you be more aware of any activity that may cause trauma to your skin tissue.  If you have to ask, then you probably already have the answer and you should avoid it and if you are not sure…“Ask a Coach!

Remember, when you need us, we are in your corner!

Marc Yale

Certified Peer Health Coach

Prednisone Tips

  •  Take as early in the morning as possible so as to avoid sleep problems at night.
  •  Supplementing a healthy diet with calcium will help to keep bones healthy through a course of prednisone.
  •  Reducing salt intake can prevent side effects associated with fluid retention.
  •  Taking it with meals could prevent stomach upset.

If you find that you are too energetic, you could try to:

  •  Do some deep breathing, yoga or listen to meditative music.
  •  Avoid caffeine after 4:00 or 5:00 P.M. to help avoid sleeplessness.
  •  Weight bearing exercises will help keep bones strong. If too difficult, stretching exercises in a swimming pool is also good.

When you need us, we are in your corner.