Yearly Archives: 2011

After months of uncertainty waiting for a diagnosis and finally finding a physician who can treat your rare skin disease, you return from the mailbox with a denial letter from your insurance company. After the shock and frustration has subsided you are now faced with the decision of whether it is worth appealing this insurance determination or should you start back at “square one”.

Chances are that the insurance company is counting on you not appealing but according to the U.S. Government Accountability Office, one report revealed that in the four states that tracked such data, 39 to 59 percent of private health insurance appeals resulted in reversal. Those are pretty good odds!

Here are some tips on how to appeal:

1.      Read the denial letter carefully. Make sure that you learn about your insurer’s appeals process. In the coverage documents and summary of benefits, insurance companies are required to give all the tools needed to properly make an appeal. There are often deadlines to meet, so act quickly and send it to them as soon as possible.

2.      Ask for help from your doctor. Check the medical policy and ask your doctor to review it to prepare something called a letter of medical necessity to support your case. If your doctor is not able to help, be prepared to handle it yourself. You are your best advocate!

3.      Contact the IPPF. The foundation can help provide you with resources about the disease and publications citing the use of treatments used for your condition. These documents can help support your case to the insurance company.

4.      Write a testimonial letter. You have a rare disease and it is likely that the person making the decision knows nothing about your disease. A letter with pictures detailing your story and exactly what happened will make it personal. Tell them that you are seeking approval for treatment, note any supporting science, clinical evidence, expected benefits, etc. Be clear, firm and concise. Make it clear that you plan to pursue the appeal until it is resolved and is approved.

5.      Follow up. Many appeals take weeks, even months, so call often to check the status and take notes of each call. When you speak to the insurance company, write down the time and date, length of the call, the name and title of the person you speak with and all the details of the conversation. Make note of any follow-up activities and next steps to be taken.

Remember, many insurance companies have a tiered appeals process. The first level is processed by the company’s appeals staff or medical director responsible for the denial. Second-level appeals are reviewed by a medical director not involved in the original claim. The third level involves an independent, third-party reviewer. If your insurance company continues to deny the claim; you can then take the appeal to your state’s insurance department, state insurance commissioner or even your local legislators who have staff to assist you.

This process may seem overwhelming but it is worth it. Your health and the health of other pemphigus and pemphigoid patients may be impacted by the awareness you create with the insurance claim.

If you need assistance, just “Ask a Coach!” Remember, when you need us, we are in your corner!

Dear pemphigus & pemphigoid Community,

As you know, the IPPF and the rare disease community have been working with Congress to advance the 21st Century Cures Act (HR 6). This legislation contains billions in new funding for research along with critical incentives to develop treatments. In July, the 21st Century Cures Act passed the House with wide bipartisan support and it is now up to the Senate to pass their own version of the legislation, called “Innovation for Healthier Americans“. Unfortunately, the Senate initiative has stalled and we are concerned that additional delays could derail the entire bill.

The IPPF is partnering with patient advocacy groups across the country to hold a day of action to call the Senate and urge them to prioritize Innovation for Healthier Americans – a delay in legislation is a delay in treatment for patients.

We are asking you to call you Senators today to convey this message and to let them know that patients can’t wait! Thank you for your all of your support and for taking action. The entire pemphigus & pemphigoid is counting on you!

Please use this sample script to help you with your call and the attached list to contact your Senators.

Sample Script:

My name is XXXX from [your city/state] and I represent the [IPPF and/or pemphigus or pemphigoid].

[Briefly explain the IPPF and/or your disease].

I’m calling today to urge Senator XXXX to prioritize Innovation for Healthier Americans. Its companion legislation in the House includes vital funding for the NIH & the FDA and incentives that will dramatically boost the number of treatments for rare disease patients.
Please support the passage of the Innovation for Healthier Americans legislation this year. A delay in legislation is a delay in desperately-needed treatments. Patients simply cannot wait. [Provide your contact information if you wish for a response from your Senator].
Thank you so much for your consideration and your leadership.

*Senate phone numbers are listed below:

Alabama                SHELBY, Richard @SenShelby (202)224-5744                     SESSIONS, Jeff @SenatorSessions (202)224-4124

Alaska                     SULLIVAN, Dan @sendansullivan (202)224-3004                *MURKOWSKI, Lisa @lisamurkowski (202)224-6665

Arizona                  FLAKE, Jeff @JeffFlake (202)224-4521                                  McCAIN, John @SenJohnMcCain (202)224-2235

Arkansas                BOOZMAN, John @JohnBoozman (202)224-4843              COTTON, Tom @SenTomCotton (202)224-2353

California               BOXER, Barbara @SenatorBoxer (202)224-3553                FEINSTEIN, Dianne @SenFeinstein (202)224-3841

Colorado                *BENNET, Michael @SenBennetCO (202)224-5852           GARDNER, Cory @SenCoryGardner (202)224-5941

Connecticut          BLUMENTHAL, Richard @SenBlumenthal (202)224-2823 *MURPHY, Chris @SenMurphyOffice (202)224-4041

Delaware               CARPER, Thomas @SenatorCarper (202)224-2441             COONS, Christopher @SenCoonsOffice (202)224-5042

Florida                    NELSON, Bill @SenBillNelson (202)224-5274                       RUBIO, Marco @SenRubioPress (202)224-3041

Georgia                  *ISAKSON, Johnny @SenatorIsakson (202)224-3643        PERDUE, David @sendavidperdue (202)224-3521

Hawaii                    HIRONO, Mazie @maziehirono (202)224-6361                   SCHATZ, Brian @SenBrianSchatz (202)224-3934

Idaho                      CRAPO, Mike @MikeCrapo (202)224-6142                         RISCH, James @SenatorRisch (202)224-2752

Illinois                    *DURBIN, Richard @SenatorDurbin (202)224-2152          *KIRK, Mark @SenatorKirk (202)224-2854

Indiana                   COATS, Daniel @SenDanCoats (202)224-5623                    DONNELLY, Joe @SenDonnelly (202)224-4814

Iowa                       ERNST, Joni @SenJoniErnst (202)224-3254                          GRASSLEY, Chuck @ChuckGrassley (202)224-3744

Kansas                    MORAN, Jerry @JerryMoran (202)224-6521                      *ROBERTS, Pat @SenPatRoberts (202)224-4774

Kentucky               *McCONNELL, Mitch @SenateMajLdr (202)224-2541      *PAUL, Rand @RandPaul (202)224-4343

Louisiana               *CASSIDY, Bill @BillCassidy (202)224-5824                         VITTER, David @DavidVitter (202)224-4623

Maine                     COLLINS, Susan @SenatorCollins (202)224-2523                KING, Jr., Angus @SenAngusKing (202)224-5344

Maryland               CARDIN, Benjamin @SenatorCardin (202)224-4524           *MIKULSKI, Barbara @SenatorBarb (202)224-4654

Massachusetts      MARKEY, Edward @SenMarkey (202)224-2742                  *WARREN, Elizabeth @SenWarren (202)224-4543

Michigan                PETERS, Gary @SenGaryPeters (202)224-6221                   STABENOW, Debbie @SenStabenow (202)224-4822

Minnesota             *FRANKEN, Al @alfranken (202)224-5641                          KLOBUCHAR, Amy @amyklobuchar (202)224-3244

Mississippi             COCHRAN, Thad @SenThadCochran (202)224-5054          WICKER, Roger @SenatorWicker (202)224-6253

Missouri                 *BLUNT, Roy @RoyBlunt (202)224-5721                             McCASKILL, Claire @McCaskillOffice (202)224-6154

Montana                DAINES, Steve @SteveDaines (202)224-2651                      TESTER, Jon @SenatorTester (202)224-2644

Nebraska               FISCHER, Deb @SenatorFischer (202)224-6551                  SASSE, Ben @SenSasse (202)224-4224

Nevada                  HELLER, Dean @SenDeanHeller (202)224-6244                 *REID, Harry @SenatorReid (202)224-3542

New Hampshire   AYOTTE, Kelly @KellyAyotte (202)224-3324                       SHAHEEN, Jeanne @SenatorShaheen (202)224-2841

New Jersey           BOOKER, Cory @CoryBooker (202)224-3224                      MENENDEZ, Robert @SenatorMenendez (202)224-4744

New Mexico         HEINRICH, Martin @MartinHeinrich (202)224-5521           UDALL, Tom @SenatorTomUdall (202)224-6621

New York              GILLIBRAND, Kirsten @SenGillibrand (202)224-4451         *SCHUMER, Charles @SenSchumer (202)224-6542

North Carolina     *BURR, Richard @SenatorBurr (202)224-3154                   TILLIS, Thom @SenThomTillis (202)224-6342

North Dakota       HEITKAMP, Heidi @SenatorHeitkamp (202)224-2043        HOEVEN, John @SenJohnHoeven (202)224-2551

Ohio                       BROWN, Sherrod @SenSherrodBrown (202)224-2315     PORTMAN, Rob @PortmanPress (202)224-3353

Oklahoma              INHOFE, James @jiminhofe (202)224-4721                          LANKFORD, James @SenatorLankford (202)224-5754

Oregon                  MERKLEY, Jeff @SenJeffMerkley (202)224-3753                WYDEN, Ron @RonWyden (202)224-5244

Pennsylvania         *CASEY, Jr., Robert @SenBobCasey (202)224-6324          TOOMEY, Patrick @SenToomey (202)224-4254

Rhode Island         REED, Jack @SenJackReed (202)224-4642                     *WHITEHOUSE, Sheldon @SenWhitehouse (202)224-2921

South Carolina      GRAHAM, Lindsey @GrahamBlog (202)224-5972               *SCOTT, Tim @SenatorTimScott (202)224-6121

South Dakota        ROUNDS, Mike @SenatorRounds (202)224-5842               *THUNE, John @SenJohnThune (202)224-2321

Tennessee             *ALEXANDER, Lamar @SenAlexander (202)224-4944      CORKER, Bob @SenBobCorker (202)224-3344

Texas                      *CORNYN, John @JohnCornyn (202)224-2934                   CRUZ, Ted @SenTedCruz (202)224-5922

Utah                      *HATCH, Orrin @SenOrrinHatch (202)224-5251                LEE, Mike @SenMikeLee (202)224-5444

Vermont               *SANDERS, Bernard @SenSanders (202)224-5141            LEAHY, Patrick @SenatorLeahy (202) 224-4242

Virginia                  KAINE, Tim @SenKaineOffice (202)224-4024                      WARNER, Mark @MarkWarner (202)224-2023

Washington          CANTWELL, Maria @SenatorCantwell (202)224-3441       *MURRAY, Patty @PattyMurray (202)224-2621

West Virginia        CAPITO, Shelley Moore @SenCapito (202)224-6472         MANCHIN III, Joe @Sen_JoeManchin (202)224-3954

Wisconsin              *BALDWIN, Tammy @SenatorBaldwin (202)224-5653    JOHNSON, Ron @SenRonJohnson (202)224-5323

Wyoming               *BARRASSO, John @SenJohnBarrasso (202)224-6441      *ENZI, Michael @SenatorEnzi (202)224-3424
*indicates Senator is a Member of the Senate Health, Education, Labor, & Pensions (HELP) Committee or holds a leadership position


Pemphigus and pemphigoid (P/P) can take a long time to improve or reach remission. The process can take months or even years. Although it may seem that this disease appeared overnight, in fact, it has taken a long time to present itself, and it will most likely take just as long to resolve. So the question often arises, “How do I know if I am improving?” Improvement can be measured in a couple ways, so here are some tips to measure whether your disease is improving:

1.      Keep a log of disease activity – It’s easier to measure if you can see it on paper.

2.      Amount – Count the number of blisters, their location and write them in your log. If you have less blisters than before then you are improving.

3.      Frequency – Take note of the blisters and how long they take to resolve. If they are clearing up quicker than before then it is showing improvement.

4.      Fatigue – Pemphigus and Pemphigoid cause fatigue. A clinical sign of improvement should be less overall fatigue.

5.      Talk to you Doctor – Ask your doctor what signs they look for that indicate improvement.  Working together with your physician to reach remission should be your biggest priority.

Improvement is a step-by step process and can be slow. If you follow these tips you may just find that you are improving, even if it’s just a little at a time. Knowing that you are improving will help you realize that you are in control and on the road to remission.

Have other questions about measuring improvement? Just “Ask a Coach! Remember, when you need us, we are in your corner!

The main physical manisfestation of the P/P diseases is the presence of blisters on the skin and mucous membranes. Underlying those blisters are numerous molecular processes including recognition of keratinocyte cells of the skin and cell death. But how these blisters actually form, that is, what is the order of events leading up to their formation, has not been clear. A recent study by scientists Parviz Deyhimi and Payam Tavakoli suggests that in pemphigus vulgaris (PV), cell death comes first, then the formation of blisters (Journal of Oral Pathology and Medicine, doi: 10.1111/jop.12022).
The blisters that form in PV are referred to as lesions, or suprabasal vesicles, because of where they are found within the layers of the epidermis (supra meaning above, so above the basal layer, see Figure 1a).
Because they are found so deep within the tissue, the blisters formed and PV disease itself is considered more severe than pemphigus foliaceus, where the blisters appear within the granular layer. The lesions formed during PV and in other mucocutaneous autoimmune blistering diseases are formed when the rogue antibodies formed during disease recognize proteins found at junctions formed by keratinocyte cells interacting with one another. The loss of these junctions that generates the tear in the skin is called acantholysis.
Acantholysis is more than a tearing of the skin. There is also cell death (also called apoptosis) within the lesions. But it has been unclear when and where apoptosis occurs with relation to acantholysis and to recognition of the junctions by antibodies generated by the immune system of the patient.
Besides the ordering of events, it has been unclear which of the various types of apoptosis are at play. In the intrinsic pathway of apoptosis, a cell essentially commits suicide because of an internal trigger, perhaps as part of a genetic program as occurs during cell or tissue development. In the extrinsic pathway, the trigger to commit suicide is external. Perhaps this is where the antibodies of PV patients play a role, then?
At least two models, both with excellent experimental support, exist for the ordering of events. The first suggests that apoptosis is a late event in pemphigus and that it is not required for acantholysis and blister formation, while the second suggests that apoptosis occurs early, before significant acantholysis. A related viewpoint to the second is that the two occur simultaneously, though independently, though evidence exists for apoptosis actually causing acantholysis. For instance, chemical inhibitors of apoptosis have been shown to prevent lesion formation and a time-course study has shown that apoptotic cells were present before blisters in pemphigus foliaceus.
The current authors looked at tissue samples from 25 patients with oral lesions due to PV. They used immunohistochemistry, the same technique that is used to diagnose PV (Figure 1b). Looking closely for regions where normal lesion-free tissue was adjacent to lesions, so-called peri-lesional regions, they found that 100% of the cells within lesions had fragmented DNA, the hallmark of apoptosis. In the adjacent normal tissue (in the parabasal region) of most of the samples, 75% of the cells had the marker of apoptosis. Looking at the acantholytic cells within the lesion, the result was strikingly close to 75%, at 76% and at the roof of the vesicle, it was even higher, at 80%. Given the presence of apoptotic cells in the lesion-free patient tissue, the authors concluded that apoptosis is not a late event, but an early one that may cause acantholysis.
Recognizing that the structural damage (acantholysis) and death (apoptosis) of keratinocytes are mediated by the same molecular players – the caspase enzymes. Research led by Sergei Grando has proposed a novel theory of “apoptolysis”, combining the two terms. The work of Deyhimi and Tavakoli supports this model and suggests that once a threshold level of apoptotic cells exist in the basal cell layer, somewhere north of 80%, then a lesion will form. According to the authors, conventional therapy of PV consisting of high-dose corticosteroids is based on the hypothesis that acantholysis leads to apoptosis, so it will be critical to unravel the current results and to determine if treatments might be tailored differently in the future.
How apoptosis leads to formation of blisters and how antibodies to desmogleins may promote apoptosis is still under investigation, but one additional piece of information from the current work is that based on the absence of another cell death marker, Bax, the authors suspect the extrinsic cell death pathway.
The pieces to the pemphigus puzzle are beginning to be unraveled. Driven by the fact that the more we learn about the molecular events leading up to blisters, the more chances there will be to intervene before debilitating blisters can occur.
The most common complaint I, as a psychologist, hear from chronic pain or illness patients is not, “why me?” or “poor me” but rather “other people just don’t get it.” And, often the hardest part is that the people to whom the patient has felt closest to previously are often the very ones who ‘just don’t get it.’ This can include immediate or extended family members, co-workers/ colleagues, or friends. It often includes primary care physicians, as well.
Additionally, being part of a not-well-known or rare chronic illness population has a lot of very real medical and life challenges. Being misunderstood can lead to even more frustration and feelings of loneliness, low self- esteem or depression.
Because of the groundbreaking work of the IPPF and its primary focus on patients, there is a pemphigus/pemphigoid (P/P) community, and no one has to feel alone again. But, because they are rare, P/P isolates patients from the people they spend most of their time with — and those people don’t fully understand. Simple comments or true answers to “how are you feeling?” can be misconstrued by some as incessant complaining. Others don’t want to hear about it. If someone looks okay, they must feel okay. Right? In a word, “No.”
How should people with chronic pain or illness present themselves in order to achieve an understanding from others around them? There is no “correct” answer that works for everyone. But there are choices. And different choices will work better in different situations. If someone is in a wheelchair, using a walker, limping, or having breathing problems, for instance, they will be seen as handicapped or sick.
Most people do not want to be seen as sick, but being seen as totally well, while suffering the debilitating symptoms of P/P, has expectations. If you are not sure where you stand on this, there is an easy way to determine your position.
Draw a horizontal line on a piece of paper: On the far left end write “human being”; in the middle write “human being with a (or several) medical problems”; on the far right, write “walking disease.” You can then think about 1) where you see yourself on this continuum, and 2) how you want others to perceive you. This is an exercise that can be done daily, weekly or any way that helps you. Don’t be surprised if it changes over time. It will affect your mood. It will also affect the way you both “think” AND “feel” about yourself and others. Also, be aware of how YOU perceive yourself; this will directly affect how others perceive you — unless you are a very good actor.
As someone with multiple autoimmune diseases, I sometimes hear disconcerting comments from colleagues and professionals who I suspect actually know better. One colleague I respect, scratched his bearded chin a few years ago and said to me, “Do you know what your problem is? You don’t look as sick as you are.” How does one respond? Whose ‘problem’ is it exactly? It was more than a comment; it was a total misunderstanding of my world.
All I said at the time was “Thank you.” It seemed clear to me that a true response/explanation would take more energy than I wanted to expend. I also didn’t think this person would understand or that he really wanted to at that time. I had to let it go and move on. This caused some more chin scratching on his part. The comment all of us hear is, “ You look good; you must be feeling better.” Where is there a direct link between how chronic illness patients look and how they feel?
These people mean well, which makes the situation difficult. I usually don’t bother to explain things; I do when there is a sincere concern or interest on their part. I don’t want to waste any energy; it is a personal choice. For me, carrying around cards with the IPPF website has been the right choice. I can easily hand it to someone. The ball is now in their court, and they can decide whether or not to take the next step.
Because of my particular profession, more people I know tend to look it up; however, most people will never bother. I am actually fine with it since I have no expectations they will take that next step.
And, I absolutely do not want to look sick. Being understood is always better than being misunderstood, but why would anyone actually want to look sick? Well, believe it or not, there are people who thrive on being ill and helpless. Sometimes it is because of a “Dependent Personality Disorder”, and sometimes it can go back to having had “special” treatment when ill as a child. What are the criteria anyway?
I can have a bit of a “Far Side” sense of humor, and I love to observe other people’s reactions. I tend to listen more than talk. Since I lose my voice easily, this is probably a positive thing for me! I often assign people the task of being a “participant observer” in uncomfortable situations. This can be an eye-opening experience.
Letters and comments that I receive from people and many patients I meet at meetings and presentations say that this is the hardest part for them on an emotional and psychological level. I often ask how they themselves feel when they see someone “who looks totally healthy” using a handicapped space; this is something most people can relate to at some level. This is how others view a chronically ill person with invisible symptoms.
An excellent story and innovative (copyrighted) rationale can be found at: www.butyoudon’ theory. It is about one woman’s spur of the moment way to explain to a good friend who wanted to understand her illness, but could not.
At our upcoming Annual Patient Conference in Chicago (last weekend of April 2014), you will have the opportunity to see and talk with both expert professionals and others going through physical and emotional changes. You will learn how others have not only coped, but also flourished.
And don’t forget, you can always ask your Peer Health Coach, the Email Discussion Group, or the IPPF Forums and someone will help find an answer for you.
“13 months, 6 doctors”
“4 months, 4 doctors”
“7 months, 4 doctors”
These statements are from three patients describing how long it took, and how many doctors they saw, before receiving a P/P diagnosis. I could put this data into a spreadsheet. I could tell you that, on average, it takes P/P patients 10 months and five doctors before receiving a proper diagnosis. But, I think we would be missing something.
Numbers are more than just data points: they tell a story. Between the digits we see frustration, anxiety, and confusion. We see seven months on the wrong medication, six physicians making misdiagnoses and 13 months in search of answers. For many P/P patients, the pathway to a diagnosis is quite grueling — both physically and mentally. It doesn’t have to be this way.
This is where the IPPF Awareness Campaign comes in!
Over the next three years, the IPPF will unleash a national Awareness Campaign focusing on early diagnosis and best treatments of P/P. The ultimate goal of the campaign is to achieve better patient outcomes by reducing the time it takes and the number of doctors seen along their pathway to diagnosis. This will be accomplished through five objectives:
1. Media and Marketing. Be on the lookout for a new website, patient and scientific videos, social media, ad placements and educational technology.
2. Dental Student Education. Patient educators will travel to 19 dental schools throughout the US to provide an emotional appeal to dental students. Topics include: pathway to diagnosis, symptom recognition, and patient care and understanding.
3. Dental Provider Education. The IPPF will provide tuition-free continuing education credits to train primary care dentists and oral specialists on P/P symptom recognition, diagnosis, and best treatments.
4. Awareness Ambassadors. The IPPF will develop an Awareness Ambassador program where IPPF community members provide outreach to dental providers in their region, build rapport with local media outlets, and plan and/or participate in community outreach and awareness activities. Stay tuned for information on how YOU can become an Awareness Ambassador!
5. Best Practices Conference. The IPPF will hold a Best Practices Conference of clinicians and scientists to develop a clear message and consensus regarding the most effective P/P treatments.
Dentists are the primary focus of the Awareness Campaign because the majority of P/P patients develop oral symptoms first and see dentists early on, often well before other healthcare providers. Primary care dentists have the unique opportunity to shorten diagnosis times by recognizing P/P symptoms and making referrals to oral specialists. Dentists could lessen the uncertainty of a patient by educating them about P/P, providing clear information, and showing confidence in treating P/P.
Delayed diagnosis can increase patient pain and suffering, heighten risk for worsening illness and poor response to treatment, result in loss of productivity and reduced quality of life, cause anxiety and fear, and increase patient healthcare expenses. The Awareness Campaign aims to significantly reduce patient uncertainties and increase positive patient outcomes by shortening the pathway to diagnosis and providing patients with best treatments sooner rather than later.
We encourage our Community to get involved with the Awareness Campaign to help spread awareness. If you are interested in learning more about the campaign or getting involved, please contact Kate Frantz at
I came down with my first symptoms of pemphigus in the early part of 1994. I have been in remission since 1998 — 16 years without medications or blisters. My life is so different now and I rarely think about it. But it wasn’t always this way.
After I bought my first computer I started searching for information about pemphigus. I came across the IPPF’s online discussion group. I cringed as I read each email and was reminded of how uncomfortable and unnerving this disease can be. I thought about how I managed to go into remission and remain blister-free for so many years, and what it was I might say to the group that could provide some hope of a similar outcome.
I kept journals during the time I had pemphigus, and wrote in them nearly every day. I recorded my medications and my physical state, thoughts and feelings. It was comforting to spell out my changing moods, from hope to despair, and vice versa.
My journals became a confidant of sorts, where I could bitterly complain about my condition, or exalt over the slightest sign of improvement. They also became a valuable source of insight when I looked back through them in my second year of pemphigus and found a pattern of ups and downs that seemed to correspond to those times when I had experimented with various vitamin and mineral supplements.
I could see after adding supplements for a few weeks my entries were more upbeat and optimistic; and when I quit taking them, I became discouraged and frustrated. I tried the vitamins several times to see if I continued to improve. Within 6 months I saw my blisters healing, I tapered off of prednisone, and entered remission. I can’t say for sure taking supplements led to my remission, but I think it’s safe to assume improving my overall health increased my chances.
One key element that could have helped is the mineral zinc. Zinc stimulates the adrenal glands to produce natural corticosteroids. Prednisone is a synthetic corticosteroid. It’s possible increasing zinc levels lowered my need for prednisone while restoring the natural secretion of corticosteroids helping the immune system distinguish between healthy tissues and foreign “invaders.” Zinc also plays a role in the Thymus gland, which produces regulatory T-cells (or T-regs), responsible for secreting immunosuppressants.
You should know I never considered supplements a substitute for prednisone or any other medication prescribed by my doctors. I never deviated from my doctor’s orders. I learned certain key vitamins and minerals are depleted by prednisone and other drugs, including Folic Acid, Magnesium, B-6, B-12, Calcium, and Vitamin D.
I chose a multi-vitamin supplement containing 23.5 mg. of zinc and the recommended levels of other nutrients to counter that effect. After several weeks I reached a relatively normal balance between the two. At least I was able to get through the disease and function without experiencing severe side effects drugs can produce.
Adding a supplement to your diet is something you should to discuss with your doctor. Finding the right multivitamin for you is easy. What was once unique to the health food market is now a common formula found in most pharmacies under a variety of brand names.
The farther away I get from my experience with pemphigus, the less I think about it. The years have gone by quickly. Now that I am retired, I find myself working quietly at home doing things I love, and feeling very grateful for having come through it without any long-term effects.
I no longer take the supplements I feel helped me to recover, or any medications at all. I believe I am living my life the way it was meant to be, but I still keep a journal of sorts. You never know when you might want to look back and remember the good days, as well as the bad.
It all started with me — a true foodie — and a scrumptious kettle chip I sampled at a barbecue in late August 2012. As an executive chef and owner of two restaurants/bars, I’m one of those people whose world revolves around great food and friends, cooking and entertaining. I used to throw dinner parties for 14 people in my home and served up multiple courses every Wednesday night. I was even written up in Time Out Chicago. . .
Back to the barbecue. When I bit into that perfectly cooked kettle chip on that late summer day, I savored its salty thickness at first. But then, I said — ouch.
The sharp edge of the chip cut me on the area between my gums and my cheek. It didn’t really phase me. I thought to myself the mouth heals quickly and it would be gone in a couple of days.
Days passed and the cut remained. I went online to research homeopathic remedies. I rinsed with honey, gargled with a hydrogen peroxide/water mix, chewed basil leaves. If I came upon a remedy — I tried it. Weeks passed by, and there was no change. In fact, my condition grew worse. I instinctively knew something wasn’t right.
When my dentist examined me, she commented on how bad the sore in my mouth looked, but had no idea what was causing it. She surmised it might be Herpes and I completed a prescription meant to heal it. When I went back to see her there was no change — and no ideas what this might be.
I visited my uncle in New York who was a practicing dentist of forty years. He suggested I gargle with salt water and restrict my diet to soft foods. I was diligent in following his advice, but again, nothing changed.
From there, I saw many doctors: my primary care physician, oral surgeons and oral pathologists, rheumatologists and allergists. The feedback was always the same – the inside of my mouth looked horrible, but the cause was unknown. All I knew was I couldn’t eat anything I used to love.
My diet was eventually restricted to egg whites, oatmeal and protein shakes. Any food harder was impossible for me to eat. Spices of any kind were out unless I wanted to be brought to my knees in pain. I began an investigation of my own.
I spent countless hours on the Internet looking up anything that could help me find the answer. . .what was wrong with me? I became so frustrated, I would leave doctors’ offices and picture them closing my file and moving on to the next patient – possibly shrugging their shoulders. Depression began to set in. This was my health, and I was grasping for answers.
I saw another oral surgeon who sent a biopsy sample to University of Chicago. I waited three long and excruciating weeks for the biopsy to come back inconclusive. More frustration.
One day I was having lunch with my brother and ordered a salad. Soon I felt a horrible burning sensation, one I likened to drinking a Molotov cocktail. I realized it was a red onion.
That onion eventually lead me to my answer. . .
I searched online for ‘mouth sores’ and ‘onion.’ The words ‘Pemphigus Vulgaris’ popped up, front and center. As I read more about this disease, I discovered five documented symptoms and I suffered from all of them. I learned a number of foods, including onions, aggravate the disease and its symptoms. Though PV can occur in individuals of all ethnicities, I discovered people of Mediterranean ancestry, Ashkenazi Jews and people from Columbia and Brazil are more likely to have it.
The most damning information I came upon demonstrated what was believed to be the severity of this condition in a 2007 publication.
“Unfortunately, if you have been diagnosed with the autoimmune blistering disorder known as Pemphigus Vulgaris, it is very likely that your life expectancy will be significantly reduced, often to just within a few years of the onset of symptoms. Most early deaths associated with autoimmune blistering disease are the result of secondary infections, such as that of the genitourinary system.”
That was one of the first published articles I read about the disease and I was horrified. Though I could not give in to the idea it would kill me in a few years, I did believe I was in for a miserable life ahead.
Despite the doom and gloom, I finally had an answer after three months of living in the dark. Finally. Now I had to do something about it.
My primary doctor suggested I see an ear, nose and throat specialist. I reached out to a dear friend who is a Harvard educated physician, Dr. Scott Smith, who was practicing dermatology at Northwestern. He said he had attended a seminar years ago about this disease, and agreed to help me. I learned about a blood test for Desmoglein, which are proteins that join human cells together. People who test at levels of 0—9 don’t have PV, or express any symptoms; levels of 9—20 are classified as ‘undetermined’; levels of 20+ indicate a person has the disease. When I was tested, my level was at 75.
The very next day I couldn’t even talk; I was certain this disease had already begun to take a terrifying toll on me.
Dr. Joquin Brieva, a specialist in Chicago, explained how different parts of the mouth, throat and eyes could be attacked. Basically, it is the good cells attacking other good cells in the body’s mucous membranes. The glue that held my skin together was actually being attacked by my own body.
What I also learned some people respond positively to Rituxan® (rituximab), a type of chemotherapy. The good cells doing harm to the body are tagged by this treatment and stopped from doing more damage. Monoclonal antibodies (clones of original cells) help to strengthen the immune system.
I told people the most dangerous thing I did every day was to brush my teeth; it took one slip of the toothbrush from the front to back and I ripped the lining of my cheek like stripping wet drywall of the wall with a putty knife.
When I began Rituxan® in January 2013, my Desmoglein levels had jumped from 75 to 128. Every day after I finished my treatment I would go shopping. There really is something to be said for retail therapy to get your mind off of things. I would push myself to go to the gym for at least ten minutes and ended up working out longer. It made me feel more positive. I never missed a day of work. I would listen to motivational tapes on my drive. I got dressed up and wore a suit and tie every day. I even got dressed up for my treatments.
Besides the exhaustion, and severe depression at times, I became very moody because of the steroids. I got infections in my nails, sties in my eyes, and rashes. It was just awful.
I couldn’t get a good night’s sleep and was in constant pain. Then I met Dr. Judy Paice. She walked into my life at just the right time, and was so compassionate and caring. I was concerned about being addicted to pain medication after reading blogs about the disease. Dr. Paice worked with me to form a strategy to reduce the pain to help me not become addicted to pain medication.
During my chemotherapy treatments my levels of the disease fluctuated. I first dropped from 128 to 98, then from 78 to 58, but then spiked during my third month of treatment to over 100. I became discouraged, but I knew I had to stay positive. Within the year, my levels went down to 12 before my last results were classified as undeterminable.
I learned through my initial research that many people dealing with rare diseases which are a mystery to the medical community. It normally takes about a year and 12 doctors to determine what ails them.
It took me three months, ten doctors, and ultimately my diagnosing myself. It also took tremendous faith, determination, hope and belief that I would find my answer. I didn’t give up on finding a way to heal myself once I learned what I was facing.
Along the way, I wasn’t able to connect with anyone who was going through what I was. I know that could have been healing on its own. My hope is that my story can be an inspiration for others, and I am open to anyone who is going through a similar circumstance to reach out to me.
The larger message I’d like for others to take away is that you must be a true and unwavering advocate for your own health. I told myself at the start of this life—changing journey that I was going to overcome this. And I did, with a steadfast determination and the support and knowledge from a fantastic team of doctors who I was fortunate enough to encounter.
Today, I know there is no playbook for this disease and that it can reappear at any time. I also know I will stay positive and continue my treatment. As Dr. Mehta, my chemo doctor says, “we kill big cancer and we will kill this.” This, and my entire medical team at Northwestern were simply amazing and key in helping me to remain positive.
I never cried while going through this trial of not knowing. I learned what I was up against and did what was necessary to bring about my own healing. However, now when I tell my story to others it is extremely emotional. I hope I can be a beacon of hope for others who might otherwise not know where to turn.
Can you believe it’s already March? I can’t! Rare Disease Day came and went, Autoimmune Disease Awareness Month is upon us (see page 13 for a special offer), and the Patient Conference is nearly upon us! If you ever wanted to attend — this is the year! Chicago? Need I say more!
New to the Office. I’d like to welcome Noelle Madsen to the IPPF family as Patient Services Coordinator. Noelle has a background in health education, event coordination, and working with patients and physicians. She will join Marc Yale and the Peer Health Coaches helping new and existing patients, caregivers, and medical professionals every step of the way! If you will be in Chicago at the Patient Conference, stop by and welcome her aboard!
2013 Holiday Fundraiser. Wow! Let me say that again: WOW! Our 2013 goal was $50,000 and we not only surpassed it, we crushed it! This Holiday Season we raised over $64,000 that goes directly to supporting our patient services and education programs. Great job, everyone!!
Awareness. The IPPF was in full Awareness-mode at the California State Capitol and the United States Capitol. In Sacramento, I was joined by Kate Frantz, Monique Rivera, and Janet Segall for an afternoon of rare disease advocacy and awareness. The California State Assembly unanimously passed Assembly Resolution 32 recognizing February 28th as Rare Disease Day in California.
Meanwhile, Marc Yale was in Washington, D.C. advocating for support of HR 3116, The MODDERN Cures Act. HR 3116 provides incentives for development of cures and treatments for unmet medical needs. We ask you to contact your Representatives ( and ask them to support HR 3116 (
IPPF joins RareConnect. RareConnect is a growing online network for rare disease communities, bringing together thousands of patients, families, and groups who might otherwise be isolated. Through RareConnect, patients and those who care for them can communicate, sharing experiences and information in a safe, moderated online forum. RareConnect allows patients from different countries to interact in English, French, German, Italian, or Spanish.
Join us at and being connecting with others around the globe. . .for FREE!
That’s it for now. From all of us at the IPPF, we hope you have a great Spring and we’ll see you in Chicago!
To Anna, the IPPF means hope when all hope was gone. For Steve, the IPPF means discovering hundreds of other patients when he thought he was alone. And Jack — Jack says he owes the IPPF his life. To me, the IPPF means helping my ‘pem-family’ find comfort, strength, and support through our dedicated staff and volunteers.
Phone calls and emails come in to our offices every day. Between the new patient calls and flare-up questions, there are little rays of sunshine that bring hope. Thousands of people around the world remember their first call, how the IPPF was there for them, and what the IPPF means to them. Now it’s your turn.
Please contribute to the IPPF this Holiday Season. Your donation funds essential, life-saving programs and services such as the Registry, Peer Health Coaches, Awareness, and more. And now you can be a Sustaining Donor! Our organization is lean, so a higher percentage of your money goes directly to the programs we offer and the research initiatives we sponsor.
You can safely donate online at or use the form below.
As I look back over the past year, I remember how many members of my pem-family I have talked with and helped with the gift of hope. I know for our Peer Health Coaches that number is even higher. This holiday season give the gift of knowledge, support… and hope.
Happy Holidays and Happy New Year!