Events

“I truly felt that our representatives were listening closely when I told them my story of being diagnosed with and treated for pemphigus vulgaris.” On Thursday, September 22, I joined 350 advocates on Capitol Hill for the Rally for Medical Research. Our main goal was to encourage members of Congress to continue robust, sustained, and predictable funding for the National Institutes of Health (NIH). Because I live in Washington, DC, a city without a vote in Congress, I was assigned to the group from Montana.

Along with a University of Montana biologist and a cancer research advocate, I met with Sens. Jon Tester and Steve Daines, and Rep. Ryan Zinke. All three members were supportive of our efforts to maintain NIH funding, especially Sen. Tester, who seemed to have a vested interest in medical research. In addition to our general request for NIH funding, I stressed the importance of cutting edge research on rare diseases and emphasized the significance of the Open Act, which incentivizes pharmaceutical companies to make treatments available off-label.

Of my three visits to Capitol Hill as a rare disease advocate, this is the first time I met with the members themselves rather than only their staff. It was a humbling experience but also a powerful one. I truly felt that our representatives were listening closely when I told them my story of being diagnosed with and treated for pemphigus vulgaris. Meeting our lawmakers in person and telling our stories makes all the difference!

In the beginning of treating a bullous skin disease like pemphigus, prednisone is usually prescribed.  A high dosage over time, can be tapered down. While we are thrilled that the dosage is being lowered, the side effects of tapering can be debilitating.

It is safer to decrease the dosage by no more than 5mg per week. Tapering too quickly can either cause a flare-up or have you feeling like your muscles are rebelling.

If you abruptly stop taking prednisone or taper off too quickly, you might experience prednisone withdrawal symptoms: A gradual reduction in prednisone dosage gives your adrenal glands time to resume their normal function.

(1) http://www.mayoclinic.org/prednisone-withdrawal/expert-answers/faq-20057923

Essentially this is a drug that mimics your body’s natural hormones produced from the adrenal glands. When prescribed in significant doses, Prednisone works to help suppress inflammation. In the event that a person’s immune system is attacking its own tissues (as is the case with autoimmune diseases), this drug can help reduce activity by suppressing immune system functioning. It affects the “HPA” or hypothalamus-pituitary-adrenal axis when taken longer than 7 days.

Side effects can include the following:  Abdominal pain, anxiety, body aches, decreased appetite, depression, dizziness, fatigue, fever, joint pain, mood swings, muscle soreness, nausea, weakness.  Not everyone experiences the same side effects, but these are the most common.

Taking an OTC (Over The Counter) pain relief can be helpful as well as taking in more salt and sugar that helps with low blood pressure and blood sugar.

(2) http://mentalhealthdaily.com/2014/06/10/prednisone-withdrawal-symptoms-how-long-do-they-last/

The body needs to keep moving although body aches can make you feel as if you don’t want to move!  What you can do is take walks at a leisurely pace until you are comfortable enough to step up the pace to a brisk walk.  Stretching every day is essential to keep your muscles flexible. Hurts,

But do what you can.  The price of being motionless is worse

if you don’t.  Your muscles will tighten and you’ll lose mobility.  A beginners yoga DVD can help you move in the beginning. Exercises in a pool help because the weight of the water pushes against you gently and cushions movement.

Meditation can help to sooth the nerves…keeping calm is very important anyway but especially when tapering because you more anxious than ever.  Listening to meditation music (Youtube if don’t have any) helps.  Talking to family members and closest friends helps too as they will understand if you seem moody or nervous.  The more you communicate, the more they can empathize and realize you need patience and humor from them!  Yes, laughter helps!

If you find that you are still having difficulty with body aches and pains and muscle weakness, consider asking your dermatologist for a referral for physical therapy.  Six visits are all you need to help you with exercises to keep you in motion and help you to get to feel better.

Remember, when you need us we will be in your corner!

You have heard this message time and time again: It’s important for family caregivers to take care of their own health so they can stay strong enough to take care of their loved ones! But as a family caregiver, it’s difficult to find the time because caregiving takes so much work and energy. Being a family caregiver can be like having another full-time job.

Research confirms what we all know – caregiving is stressful. It challenges caregivers physically and emotionally. No one can expend their time, energy, and strength caring for someone else without having to replenish their own reserves. Sooner or later, something has to give. Over time, caregiving takes a toll on body and mind; on personal finances, family relationships, and work life. It can erode the immune system and actually increase susceptibility to illness.

Two out of every five adults provide care for a chronically ill, disabled or aged family member or friend. They typically spend 20 hours per week providing care for their loved one, and 13% are providing 40 hours of care a week or more. And for six in ten family caregivers, this is in addition to already working a full- or part-time job!

Family caregivers face many challenges. Caregivers have a higher incidence of major health conditions (such as depression, hypertension, and diabetes) than non-caregivers – directly as a result of the stress from their caregiving challenges. And ignoring their own health (sleeping, eating, exercising, visiting a doctor) further undermines caregivers’ health. There are many indications that caregivers are at risk of burning out such as feelings of anger, anxiety, depression, or fatigue. Seeking help is the most important step, but admittedly it is not always easy. Thankfully, there are tips and tools available to help avoid burnout and maintain some sense of balance in your life while caregiving!

Washington, DC-based Caregiver Action Network works tirelessly to advance resourcefulness and respect for family caregivers across the country. CAN’s free educational resources assist in learning to develop effective caregiving coping skills. CAN has tips and tools that can help caregivers avoid burnout and stay healthy themselves while caring for their loved ones.

CAN Recommends that Caregivers:

  • Seek support from family and friends.
  • Accept offers of help and suggest specific things people can do to help.
  • Learn how to communicate effectively with doctors.
  • Take respite breaks often.
  • Watch out for signs of depression and don’t delay in getting professional help when they need it.

Connecting caregivers to one another is also vitally important because caregivers need to know that they are not alone. CAN maintains the Family Caregiver Forum so caregivers can connect and help each other. This online forum is a safe place where any caregiver can ask a question of others dealing with similar circumstances and get thoughtful and helpful responses.

Nothing is as important to avoiding burnout as “respite” – the chance to take a breather, the opportunity to re-energize. It is as critical as any other item on a caregiver’s to-do list. Respite is the key to your own well-being. Respite protects your own health, strengthens family relationships, and allows loved ones to stay at home up to three times longer. It should come as no surprise that respite is one of the most frequently requested support services for family caregivers. There are seven essential elements of respite, easily remembered by the acronym “RESPITE”

“R” is for “Rest and Relaxation.” Everyone needs a little “R and R” – especially family caregivers. Relaxing is the best way to return refreshed to handle your many responsibilities as a caregiver.

“E” stands for “Energize.” Caregiving is often round-the-clock. Respite isn’t simply getting a few hours off. It’s necessary to help you reenergize and reduce your stress

“S” is for “Sleep.” Understandably, caregivers often have difficulty sleeping. Address sleep problems and insomnia before they take too great a toll on your health.

“P” is for “Programs that can help you.” Find these programs!

“I” is for “Imagination.” Let your mind run free. Read a book. See a movie. You have been so occupied with the nuts-and-bolts of caregiving that refreshing your mind will actually help you be a better caregiver.

“T” is for “Take Five.” Don’t feel guilty. You need a reprieve – a few minutes to temporarily disengage, so step back and take a breather for your own good.

“E” is for “Exhale.” A simple breath in and then a long exhale can help you focus and increase your vitality. A few deep breaths can give you more energy, reduce stress, and lift your mood. So if you need help managing any of the many challenges that come from caregiving – such as isolation, feelings of anger or depression, or communicating with doctors – feel free to check out CAN’s free resources at CaregiverAction.org. And if you are a patient who relies on a family member for help, do them a favor and send them to CAN’s resources because they need someone to lean on, too!

Although the scalp can be frequently involved with pemphigus/pemphigoid, associated hair loss has only rarely been described. In one case, treatment with oral and topical corticosteroids combined with mycophenolate mofetil resulted in clinical remission, with regrowth of scalp hair. (1)

The main connection between prednisone and hair loss seems to be that, as a side effect of using of the drug, some users have complained of thinning hair.  High doses of the drug can make hair more brittle. This brittleness can make hair more fragile and increase shedding and breakage. Hair thinning may become more severe in individuals taking prednisone who also choose to use a chemical process on their hair such as hair dye or a perm. (2)

The connection between prednisone and hair loss is still being explored.  If you are experiencing hair loss, it is best to discuss with your dermatologist and a change in dosage or the use of additional medications may be used to help avoid this side effect.

I do know of one patient who experienced patches of hair loss.  She wore baseball caps all the time.  It did take a few years, but as she tapered off of her medications, her hair did grow back.  There is no timetable for this as each patient has a different level of antibody activity.  However, do not give up hope.  The hair will return.

(1)      http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3927177/

(2)     http://www.wisegeek.org/what-is-the-connection-between-prednisone-and-hair-loss.htm

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

Pemphigus and pemphigoid are a group of rare autoimmune diseases that affect the skin and mucous membranes causing painful blistering and scarring. For some patients, the noticeable and sometimes life-threatening, symptoms are too much to work with. The Social Security Administration’s (SSA) disability benefit programs are for patients like those who can’t support themselves because of their disability.

Social Security Disability

If you cannot work enough to earn the SSA’s monthly Substantial Gainful Activity (SGA) limit of $1,130, then you may be eligible for Social Security Disability Insurance (SSDI). Generally, you need to have worked five of the last ten years in a job that pays into Social Security, and additional years depending on your age. Benefits average $1,000-$1,500 each month, but the SSA calculates your payment by averaging your previously reported income, so it may be higher or lower.

After you are approved, you will not receive your first check until five months after the date your pemphigus or pemphigoid started, or your reported onset date. Be aware the SSA may take up to two years to approve the claim; however, you will be given any back pay in a lump sum check. Two years after your reported onset date, you will be eligible for Medicare.

Supplementary Security Insurance (SSI) is not paid from taxes, so it is best for those with little or no work history, or parents applying for children. In most states, you will also be automatically enrolled in Medicaid once approved for SSI. You are allowed to work while on SSI, but individuals cannot earn more than $733 a month in countable income, and couples cannot earn more than $1,100 ($1,100 is the total countable income limit for a couple with SSI, if an SSI-eligible individual is married to someone who is not eligible, then the SSA does a process called “deeming” where they factor in the ineligible spouse’s income and resources to determine payment amounts.). Individuals and couples also cannot have more than $2,000 and $3,000 worth of assets. If a person/couple owns a home, that home is not counted toward their income or resources as long as it is their primary residence.

Requirements for Pemphigus and Pemphigoid

The SSA evaluates all applications they receive first with the Blue Book, their official list of impairments and medical requirements. If you meet or equal a condition in the Blue Book, you will be automatically medically eligible for benefits This means that an individual can qualify for benefits based off of their condition, but the decision is ultimately up to a disability examiner, so an individual may have to file an appeal if the examiner denies their application. .

Pemphigus and pemphigoid can be found in Section 8.00—Skin Disorders of the Blue Book. You need to provide medical evidence of the following:

  • Bullous disease (pemphigus, bullous pemphigoid), with extensive skin lesions that persist for at least three months despite continuing treatment as prescribed;
  • Chronic infections of the skin or mucous membranes, with extensive tissue death or extensive ulcerating skin lesions that persist for at least three months despite continuing treatment as prescribed.

If you do not meet a Blue Book listing but feel your conditions restricts you from performing SGA, you may still be approved with a medical-vocational allowance based on your limitations. Applicants do not need to do anything for a medical-vocational allowance. The medical-vocational allowance is a determination made by the SSA during the application process after they determine that someone  is not medically eligible by the Blue Book criteria. This determination takes into account a person’s ability to do the work they have been doing, whether they would be able to be re-trained for another job, whether they can perform any type of work that they’ve done in the last 15 years among other factors.

Applying for Social Security Disability

Talk to your doctor before completing the SSI application. Because the claim can take so long to process, be reviewed, and a determination made, it may not be worth applying if your doctor does not think your chances are good enough. If you do decide to apply, a detailed statement from your doctor about the limitations caused by your pemphigus or pemphigoid is crucial to the claim.

The SSA offers a convenient application on their website for SSDI. SSDI is Social Security Disability Insurance – meaning that it is based off of your work history and income, and its premiums are paid with payroll taxes. SSI is a needs based program and because of that has income limits, restrictions on resources, and a lower maximum benefit amount. If you’re not comfortable applying online or you are applying for SSI, you will need to make an appointment at your local SSA office. You can find a list of documents (such tax information and a birth certificate) you will need, in addition to important medical evidence (labs, imaging tests, and surgical reports) on the SSA’s website.

On March 2nd, 2016, six of my fellow pemphigus and pemphigoid (P/P) advocates and I visited my Congressional members to ask them to co-sponsor legislation that would make it easier for Medicare patients to have IVIg infusions done at home. IVIg therapy is a treatment that many patients such as myself use in conjunction with immune suppressant medication to control our diseases.

Unfortunately, getting IVIg home infusions under Medicare can be complicated because there is a gap between Medicare Part B and Medicare Part D. Infusion therapy is fully covered by Medicare in hospitals, skilled nursing facilities, hospital outpatient departments, and physician offices, but not in a patient’s home. However, if a patient wants to get the infusion done at home, Medicare pays for the infusion drugs (under Medicare Part D), but not the medically necessary services, supplies, and equipment used in the provision of infusion therapy (under Part B). This makes access to these much needed services costly or impossible for many home infusion patients. As a result, most Medicare patients in need of infusion therapy often receive their treatments in health care facilities instead of in their homes, although home may be the setting that is the most desirable, safe, convenient, and by far the most cost effective.

Congress has been concerned with this issue for many years, but has yet to approve any legislation. In January, Congressman Eliot Engel, Representative for New York’s 16th congressional district, and Senator Johnny Isakson, Senior Senator from Georgia, introduced the Medicare Home Infusion Site of Care Act of 2015 (H.R. 605 and S. 275) that would remedy the problem. As we shared our stories on behalf of the entire P/P community, I could see that many of the legislators’ offices we visited began to understand the dilemma. However, it was clear this issue would take an effort by the entire P/P community for our voices to be heard.

I urge you to contact your congressional members and ask them to co-sponsor this crucial legislation. Reach out to the senators on the Senate Help Committee and the representatives on the House Committee on Energy and Commerce who control whether this legislation will move forward. Let’s start a conversation by using the hashtag #medicarehomeinfusionact on Facebook, Twitter, and Instagram. Together we can make a difference!

The biggest problem with dental implants in pemphigus and pemphigoid (P/P) patients is that P/P are often treated with corticosteroids or other immune suppressants for prolonged periods of time. This makes these patient poor candidates for dental implants because the implants will usually not integrate into the bone.

Failures can also occur if the patient already has implants before they have P/P. They may also be more susceptible to periodontal disease because of their altered immune responses and altered bone metabolism.

Insurance

It is at least theoretically possible that steroids could facilitate the loss of some or all of the teeth, especially if the patient has severe P/P requiring a lot of treatment. However, I doubt insurance companies would be willing to reimburse for implants because of the extremely guarded prognosis. They might consider reimbursing for conventional dentures, though.

More Detail

We consider prolonged steroid therapy as a contraindication for dental implants. There are a couple older case reports (1990, 1991) describing successful implant placement in these patients, but the papers are quite old and probably did not really confirm success.

A recent 2013 case report* described success in a patient who had only oral pemphigus vulgaris (PV) and who had steroid therapy for a relatively short time. In this report the patient had two implants placed under a full denture, and the case was followed for 32 months. The authors described very gentle but conventional implant placement and denture management but nothing out of the ordinary.

If an oral PV patient is interested in dental implants, it might be of value to have their doctor review this paper. However, I am not convinced that this small case report indicates that implant placement is highly predictable and cost effective in PV patients, and we have not elected to place implants in anyone on long-term steroid therapy regardless of the disease or disorder being treated.

*Altin, N., Ergun, S., Katz, J., Sancakli, E., Koray, M. and Tanyeri, H. (2013), Implant-Supported Oral Rehabilitation of a Patient with Pemphigus Vulgaris: A Clinical Report. Journal of Prosthodontics, 22: 581–586. doi: 10.1111/jopr.12050

My name is Marlis Lippow, and I reside in Northern California. I participated in a randomized, double-blind, double dummy study evaluating rituximab infusions vs. 2,000 mg of mycophenolate mofetil (Cellcept®).  I had previously received rituximab infusions so I had a pretty good idea of what to expect if I was to again receive it. I had three previous rounds and my doctor said the effects should last about six months, after which I would probably need another round. I was lesion-free for about seven months before the lesions started to return. I also was on CellCept and prednisone, so I know how those affect me.

Learning About the Trial

My doctor mentioned a clinical trial, answered my many questions, and asked me to think about it.  I returned a month later for a followup appointment and there was another doctor present. She was talking about the trial and it seemed she expected me to be a part of it. I was still unsure and had more questions. She did explain that before I could be accepted, I’d undergo a screening (ECG, chest x-ray, and blood work). That was great, I would find out how I am doing. If I passed the tests, I could decide if I wanted to participate. The trial included a stipend, $50 for each session for gas and parking. That sounded good since I live about 45 miles away.

Making the Decision

My doctor and I discussed the pros and cons of the assorted medications and what I’d need to take if I did not participate in the trial but still needed rituximab. Either way, the side effects are not pleasant.  Basically, we talked about the lesser of the two evils.

I learned my doctor is referred to as the Principal Investigator (PI) and the other doctor is the Sub-Investigator (SI). The SI would be seeing me every month. Since the SI is not my primary doctor, it is very important she have a complete grasp of my medical history. During my visits I would have blood work and urinalysis done. The PI would get the test results and be aware of my progress and any possible problems. If he felt there was a concern, he would end my participation in the study.

I also learned I could opt out of the study at any time if I became uncomfortable.

In the end, I chose to be in the trial.

My Trial Experience

Throughout the study, I felt my doctor was most concerned about my well-being, as he should be. He even called me in between visits and that gave me a good, positive feeling.

I received two initial infusions two weeks apart. After about five months, I received two more, also two weeks apart. By the third round I no longer had any sores.  I was told that I was “controlled” and after the last infusion, I’d be in remission! My doctor told me this remission should last anywhere from six months to three years. I am hoping it will be longer!

Don’t be worried about the infusions.  The infusion nurses are angels and take wonderful care of you! They are kind, let you know what to expect, and give you an idea of how you will feel.  If you have any questions, you will have the phone numbers for the PI and SI and are encouraged to use them.

Clinical trials are not for everyone. In fact, there are many qualifying and disqualifying criteria set by the drug manufacturer. I encourage everyone to consider participating in a trial to help advance research on new and emerging pemphigus and pemphigoid treatments. While the short-term benefits help us now, the long-term benefits may change the lives of patients for years to come.

The purpose of advocating in Washington, DC is to spread awareness and lobby for favorable legislation that affects the entire IPPF community. At Rare Disease Week on Capitol Hill 2016, we had 7 members of the IPPF community advocating for:

  • HR 971/S1421 (OPENACT): legislation that will repurpose “off label” drugs for rare disease indications that are not currently covered by the FDA
  • HR 605/S275 (Medicare Home Infusion Act): legislation that will make it easier for Medicare patients to get infusions done in their homes
  • HR 1600 (Patients Access to Treatment Act): legislation that would cap the amount that insurance carriers can charge patients for more expensive medications
  • S 2030 (Advancing Targeted Therapies Act): legislation that would allow information learned in clinical trials to be used in the development of new therapies
  • All Congressional Members to join the Rare Disease Caucus

From the Advocates:

Sarah Gordon

This was my second time advocating on behalf of the pemphigus and pemphigoid community, having visited Capitol Hill with Marc and Kate in the fall. I expected it to be quite similar, and in many respects it was. However, being part of a larger group of rare disease advocates participating in Rare Disease Week made a big difference. Hearing from two congressmen and the newly appointed head of the FDA at the lobby day breakfast emphasized the importance of bringing together advocates from across the rare disease spectrum – not only the power in numbers but also the variety of knowledge and experiences that we could bring to the table.

Mary Lee Jackson

When I received an email about going to Capitol Hill, I got excited and thought it will be easy. But I was wrong it was hard work. I hadn’t walked so much in all of my life. I felt good about what was done on Capitol Hill. It was worth all I went through to get the word out about rare diseases.

Debra Levinson

There’s plenty we can do this election year: Consider writing letters and making phone calls to bolster these pieces of legislation; email your member of Congress, which is made easy utilizing the automatic draft letters on rareadvocates.org; or take advantage of in-district lobby day opportunities to build relationships with your members of Congress. I am hopeful now with so much support we can help ourselves heal. We are the voices of the rare disease community with a common role.

Paula Halicki

Not only was I representing myself, the IPPF, and the pemphigus/pemphigoid community, but I was also representing a lot of other people with healthcare issues who could not represent themselves. And quite a few who never will. Plus I told my story, and a Congressman listened.

Doris Chenier

We may have a rare disease but no one will know about it unless we use our voice to speak to people that can make a change.

Angélica N. García Romero

I had great meetings with the staff of the representatives and felt very accomplished. But to top it all, when I was taking my flight back to Puerto Rico, my representative was on the plane too! When all the turbulence stopped, I went up to him, and had the meeting right there.

It’s no secret among the pemphigus and pemphigoid (P/P) community that treatments to help these rare diseases are not always ideal. It’s also common for patients and caregivers to reach out to the IPPF seeking advice on holistic/natural/eastern medicines to treat P/P before the typically used western medicines recommended by physicians.

The IPPF understands that the potential side effects of steroids, immunosuppressants, and biologics can be very concerning. You already have a lot going on with your body and immune system. Why subject your immune system to these medications if you don’t have to? The IPPF is not against patients trying holistic or more natural remedies. We truly want P/P patients to get into remission by whatever means work for them. However, you need to know one very important fact before trying any natural treatments.

Many natural or holistic treatments work by “boosting” or “enhancing” your immune system. When you have an autoimmune disease like pemphigus or pemphigoid, that means you have a compromised immune system. Therefore, if you “boost” or “enhance” your immune system with an active autoimmune disease, you may be putting yourself at risk of “boosting” or “enhancing” your disease activity.

The IPPF highly recommends that you do your research before you try any natural or holistic treatments. Make sure the treatment you are interested in won’t boost your immune system. The IPPF also recommends that you consult your doctor before trying any treatments. While your intention of trying this treatment will be to make you healthier and improve your disease activity, you may be inadvertently making your disease worse. That is the last thing we want for you, and we know it’s the last thing you would want for yourself.[/vc_column_text][/vc_column][/vc_row][vc_row][vc_column width=”1/6″][/vc_column][vc_column width=”2/3″][vc_empty_space height=”32px”][vc_separator style=”thin”][vc_custom_heading text=”Therefore, if you “boost” or “enhance” your immune system with an active autoimmune disease, you may be putting yourself at risk of “boosting” or “enhancing” your disease activity.” The IPPF doesn’t want to discourage you from trying something that could alleviate your disease symptoms or make you feel more comfortable. We just want you to thoroughly research the product and consult your doctor before doing so.

Going off of prescribed medication in order to try a holistic treatment is not recommended. Patients who go off of treatments to try natural therapies without consulting their doctors can put themselves in a potentially dangerous situation. Let your physician be on this journey with you.

The IPPF does recommend you take your vitamins, exercise, and eat a balanced diet. We also recommend consulting your doctor about ALL treatments that could help treat your pemphigus or pemphigoid.

It has been a busy few months for the Awareness Campaign. With outreach to dental students in full swing, it was time to expand our reach to dental practitioners. And where do we find large groups of dentists, dental hygienists, specialists, and assistants? Dental conferences! We rent a booth on the exhibitor floor and talk with dental practitioners as they walk through the exhibit hall. So far, we’ve reached nearly 2,000 dental professionals.

In November, we attended the American Dental Association’s Annual Meeting in Washington, DC. December found us at the Greater New York Dental Meeting in New York City, and in January we kicked off the new year at the Yankee Dental Congress in Boston.

Our current plans call for us to have a booth in Atlanta at the Hinman Dental Meeting in March, followed in April by the Oklahoma Dental Association Meeting in Oklahoma City.

Many of the larger meetings have 20,000+ people attending. There are rows and rows of exhibits and the halls often seem endless! We usually meet with around 600 people per conference, which is pretty good for a small non-profit. We don’t have large hanging signs, bright lights, or fancy electronic displays. But we do have many free resources and great educational materials!

These conferences are usually three or four days long, and we spend eight or more hours each day at the booth. We couldn’t do what we do without the great help of local volunteers, many of whom are pemphigus or pemphigoid (P/P) patients or friends and family of patients. Patients are encouraged to share their diagnosis stories and reinforce the unique role dental professionals play in getting patients diagnosed sooner.

How do we encourage dentists to stop by our booth? We stand near our booth table, smile, and ask, “Have you heard of pemphigus?” or “Is pemphigoid on your radar?” It is that simple! Many people look at us like, “Pemphi-WHAT?” almost as if we are speaking a foreign language. Once we hand them Awareness Postcards, brochures on the campaign with scientific information, and our Quarterly newsletters and they see the names in print, some recognize them because they remember them from school, saw them in a textbook, or have previously diagnosed a patient. For others, it’s the first time they’ve ever heard or seen the words “pemphigus” and “pemphigoid.” It is truly rewarding to be the person who educates someone about these rare diseases.

“Pemphi-WHAT?” In addition to sharing stories and passing out materials, we also ask dental professionals to take a short survey describing their experience and confidence in diagnosing P/P. We asked, “On a scale from 1 to 5, how confident are you in your ability to recognize the clinical presentation of pemphigus and pemphigoid?” About a third (27/85) of dentists and half of hygienists (14/28) reported low levels of confidence in their ability to recognize P/P. We also asked 141 people if they had heard of the IPPF before they stopped by our exhibitor booth; 81% had not.*

These figures support the need for the Awareness Campaign and increased outreach to the dental community. Since such a large percentage of dental professionals had never heard of the IPPF, we also recognized the need to spread awareness of our Foundation in general. We encourage dental practitioners with P/P patients to inform their patients about the IPPF and all of our patient support services.

These conferences are an excellent way for us to meet dental professionals and spread awareness of P/P and the IPPF. Because of the connections we’ve made at these gatherings, we’ve received requests for Continuing Education presentations and dental student lectures; learned about some great media opportunities; had an article written about us; and have secured some awesome product donations for our Annual Conference goodie bags!

Our goal is that one day dental practitioners will pass by our booth and say, “Yes, I already know about your Foundation.” Until then, we will continue setting up our booth and repeating over and over, as many times as it takes, “Have you heard of pemphigus and pemphigoid?”

*2015 data collected by the IPPF at two dental conferences

“Is there a diet I can go on to help treat my disease?”

It’s one of the most common questions that I receive at the foundation. The answer is, unfortunately, no.

There is currently no diet that will help to put your disease into remission. However, there are certain foods that may exacerbate your condition.

Pemphigus and pemphigoid are very patient-specific diseases. Everyone’s disease activity varies. Well, the same thing goes for diet and these diseases. The foods that negatively affect one person’s disease activity may do nothing to another individual. It is about becoming an expert on you.

We recommend keeping a food calendar or journal. Write down all of the foods that you eat each day, along with your disease activity. Over time you may begin to see patterns form. For example, you may see that every time you eat onions, new lesions appear or current lesions worsen. You then can try to omit onions from your diet to see if it helps.

Patients have reported improved disease activity after changing their diets or eliminating certain foods. It should be noted that other patients have reported no change from adjusting their diet. Again, it is about becoming the expert on you and working with your treating physician every step of the way.

Foods that patients have reported to be bothersome (you may want to talk with your doctor about avoiding these):

  • Citrus
  • Acidic Fruits
  • Bagels
  • Garlic
  • Potato Chips
  • Barbeque/cocktail sauces
  • Horseradish
  • Relishes
  • Chili
  • Onions
  • Red Sauces
  • Chocolate
  • Pickles
  • Tomatoes
  • Creole
  • Popcorn
  • Worcestershire sauce
  • Pretzels
  • Pizza
  • Tortilla Chips
  • Red wine
  • Coffee

Some patients’ oral disease activity is so bad that it is hard for them to consume any food at all. Lesions in the mouth can be painful and cause severe discomfort when eating. The result is a poor nutrient intake, which can result in weight loss and loss of the body’s protein stores. The resulting malnutrition causes fatigue, impairs wound healing, and decreases the body’s resistance to infection.

Suggestions to help prevent malnutrition:

  • Eat a variety of foods daily.
  • Take a multivitamin with minerals if you feel you do not eat the recommended serving sizes of each food group.
  • Weigh yourself weekly. If losing weight, investigate ways to increase calories and protein in your diet.

Soft foods which may be easier to swallow:

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

Take a swallow of a beverage with solid food

Eat small frequent servings rather than a large amount of food at one time

Rinse your mouth with water, peroxide, or Biotene during and after eating to help remove food and bacteria and to promote healing

  • Soft fruits, such as applesauce
  • Nectars, such as peach, pear, or apricot; no fresh juices, like orange or grapefruit juice
  • Apple juice (diluted with water if necessary)
  • Canned fruits
  • Pureed meats and vegetables
  • Milk shakes (add protein powder or egg whites for additional calories and protein)
  • Custard and puddings
  • Macaroni and cheese
  • Pasta with margarine or butter
  • Scrambled eggs, egg beaters, omelets, egg salads
  • Oatmeal and Farina (cool to room temperature)
  • Whipped potato (sweet potato or yams)
  • Mashed vegetables (carrots and peas)
  • Cottage cheese
  • Yogurt
  • Cheesecake
  • Meatloaf and tuna casserole
  • Ensure drinks
  • Soups
  • Casseroles
  • Pastas
  • Smoothies

The First Amendment to the United States Constitution guarantees the right of all citizens to communicate with their elected officials. So you might be asking yourself, “Does my Congressional Representative really care what I think”? Well, the answer is YES!

A recent study showed that if a Member of Congress has not arrived at a firm decision on an issue that a constituent contacting their Washington D.C. office will influence their decision. The study illustrated the degree of influence that the following types of communication will have on a congress person’s decision: phone calls 72% influence, individualized postal letters 70% influence, and individual email messages 69% influence.[/vc_column_text][vc_custom_heading text=”The study illustrated the degree of influence that the following types of communication will have on a congress person’s decision: phone calls 72% influence, individualized postal letters 70% influence, and individual email messages 69% influence.”

Writing your Congressional Member may seem like a small thing, but they need to know that their constituents are paying attention. They want to hear about what issues are important to you. When writing your letter/email to your Congressional Member, you may want to share your story with them and how it relates to your issue. Personalizing your letter/email will grab their attention and give them more reason to consider your issue. It is recommended that you keep your letter/email simple and no more than three paragraphs. Provide facts about the issue you are addressing and try to be as specific as possible. Be sure that you cite the name and number of the Bill or Legislation that you are asking them to support. If they are already a supporter of the Bill, thank them.

Don’t forget to provide your contact information so they can respond directly to you. Remember, your voice can go a long way towards helping your Congressional Member know where the public stands on your issue. The more you correspond with your representative, the more it will help you build a long-lasting relationship with him or her. That relationship is not only your right as a citizen, but a privilege you should exercise.

Many individuals with pemphigus and pemphigoid (P/P) present with chronic mouth sores, often well before lesions appear on the skin or other parts of the body. These painful mouth sores tend to be persistent and present as red, ulcerated areas. In some cases — especially those with mucous membrane pemphigoid — lesions may predominantly involve the gum tissue. However, most people with P/P suffer lesions in multiple areas of the mouth, including the tongue, cheeks (buccal mucosa), wet surface of the lips, floor of the mouth, hard and soft palate, and throat.

Oral lesions are often subtle, especially in early stages of P/P. As a result, P/P are often misdiagnosed by both medical and dental practitioners as thrush, food or toothpaste “allergies,” poor oral hygiene, viral infections, or erosive lichen planus. Many patients with P/P are first treated on an empirical basis (treatment based on observation and experience without a definitive diagnosis), often with multiple medications, until one approach offers some relief. The relative rarity of these conditions mean they are often not on a medical or dental practitioner’s “radar” when assessing a patient’s oral lesions.

So if you suffer oral lesions, how can you partner with your dental health care provider to come up with a definitive diagnosis? A thorough review of your symptoms is critical. Make sure your dentist or dental hygienist listens carefully and asks detailed questions about your concerns, such as:

  • How long have you had lesions?
  • What areas are involved?
  • Do you have any skin, eye, vaginal, or rectal involvement
  • What do the lesions look and feel like?
  • Do the lesions move?
  • Does your pain level and disease activity vary over time?
  • Do you currently have any active lesions?

Biopsy confirmed early gingival and mucosal lesions of mucous membrane pemphigoid. These lesions were managed as “non-specific gingival irritation, suspect allergy” for several months prior to a diagnostic biopsy being obtained.

The saying “diagnosis dictates treatment” is particularly relevant when it comes to treating oral ulcerative conditions.”

While early disease symptoms can be subtle, most of the conditions for which P/P are misdiagnosed are not typically chronic (with the possible exception of erosive oral lichen planus or chronic ulcerative stomatitis). Moreover, at least to a clinician with experience diagnosing and managing these conditions, the clinical appearance is quite distinct — toothpaste allergies and poor oral hygiene do not lead to widespread, chronic oral ulcers!

The saying “diagnosis dictates treatment” is particularly relevant when it comes to treating oral ulcerative conditions. Therefore, the best advice I can offer is threefold:

  1. Your dentist or physician needs to take your complaint seriously and needs to thoroughly investigate your symptoms.
  2. A diagnostic tissue biopsy is essential before your dentist or physician treats you empirically with antifungal, anti-viral, or corticosteroid medication.
  3. If the clinician assessing you does not insist on performing a diagnostic biopsy, insist on being referred to a clinician with extensive experience in the diagnosis and management of oral lesions (e.g an oral and maxillofacial pathologist, periodontist, oral and maxillofacial surgeon, or dermatologist).

Bill Starrels (l), Marc Yale (c), and Sarah Gordon (r) outside the Washington, DC office of Congresswoman Julia Brownley of California.

When the email landed in my inbox last month asking for pemphigus and pemphigoid patients and caregivers to advocate on Capitol Hill, I didn’t hesitate. I live just miles from the Hill, and it was time to share my story and encourage lawmakers to take a stance on research and treatment for rare diseases like pemphigus and pemphigoid.

When my pemphigus was diagnosed eight years ago, I had just given birth to my first son. It was a difficult time, combining the exhaustion and stress of new motherhood with the pain, confusion, and anxiety of an undiagnosed illness. Once I was diagnosed, the first line of treatment, Cellcept, caused neutropenia, a white blood cell condition making me susceptible to infection. So with much trepidation I used the off-label treatment, Rituxan. In retrospect, I see that despite the challenges of my illness and treatment, I was incredibly fortunate. I saw Dr. Grant Anhalt, who was a true lifesaver, and the Rituxan worked beautifully! I have now been in remission for nearly five years. Most remarkably, I was able to give birth to another healthy and happy boy four years after the first. Yes, when I received the email last month asking for advocates on the Hill, I knew it was time. And what a time it was.

Before heading up to the Hill, I discussed the various bills before the House and Senate with Marc Yale, Certified Peer Health Coach at the IPPF. Unfamiliar with congressional practices and language, I was still easily able to grasp the reasons these bills were so crucial for people with rare diseases like pemphigus and pemphigoid. They dealt with incentivizing drug companies to repurposes medications like Rituxan to make them more accessible; they defined the terms of telehealth, a crucial first step toward allowing highly specialized physicians to treat patients remotely; they aimed to maintain funding for vital NIH research on rare disease treatments. Through my conversations with Marc, I found that my personal story could speak directly to the importance of several of these bills.

On October 20, I met Marc, Kate Frantz, Awareness Program Manager at the IPPF, and Bill Starrels, whose wife has pemphigus, at the Capitol. Entering the congressional office, I had the uneasy sensation of being on an episode of the HBO comedy Veep. But I quickly found that the staffers with whom we met were not snarky and self-involved, but rather knowledgeable, compassionate, and devoted to their work. We met with staffers from the offices of Julia Brownley, Doris Matsui, Barbara Boxer, and Dianne Feinstein from California, as well as my own representative, Eleanor Holmes Norton, of Washington, DC. Speaking to them about the bills at hand through the lens of my personal experience made me feel at once vulnerable—presenting myself for the first time as a person with a rare disease—and empowered—taking the opportunity to advocate on behalf of myself and so many others. I learned a tremendous amount about how Congress functions and also quite a bit about my own potential as an advocate.

In the end, I was correct: the time was right for me to share my story with lawmakers and become an advocate for persons with rare diseases such as pemphigus. Keep an eye on your inbox for messages from Mark and Kate. You might just find that soon the time will be right for you as well.

Kate Frantz (left), Awareness Campaign Manager, with Marcy Syms (center) and Mindy Unger (right) at the Greater New York Dental Meeting. The Awareness Campaign is generously funded by the Sy Syms Foundation and the Unger family.

The IPPF Awareness Campaign seeks to reduce pemphigus and pemphigoid (P/P) diagnosis times by increasing symptom recognition among dental professionals. It’s important for the Awareness Campaign to connect with dental communities, and one effective way to do this is by exhibiting at major dental conferences.

Recently, the Awareness Campaign was an exhibitor at the American Dental Association’s (ADA) Annual Meeting in Washington, DC from 11/5-11/10 and the Greater New York Dental Meeting (GNYDM) from 11/29-12/2. Campaign staff and volunteers connected with over 600 dental professionals at each meeting, handing out brochures and post cards specifically designed to help these dental professionals recognize P/P symptoms.

An important feature of the IPPF exhibitor booth was the “Meet a Patient” area, where conference attendees were encouraged to ask a volunteer with P/P about their diagnosis story. This kind of personal appeal goes a long way toward helping dental professionals remember to “Put It On Your Radar.”

Another result of attending these conferences is the opportunity to network with others in the dental community who can help spread the word about P/P. At GNYDM, Awareness Campaign Manager Kate Frantz was interviewed for an article in Dentistry Today. The article was published the day after the conference ended, and it featured both an overview of the Awareness Campaign and key information about P/P.

With the success of the ADA and GNYDM exhibitions, the Awareness Campaign will continue to pursue these valuable opportunities to educate dental communities about P/P in 2016. The next event is the Yankee Dental Congress in Boston, MA from 1/28-1/30.

Stress can play a huge role in health conditions, especially autoimmune diseases.

Symptoms of stress include:

  • Poor or inadequate sleep (insomnia)
  • Anxiety
  • Depression
  • Panic attacks
  • Headaches
  • Poor concentration
  • Muscle aches
  • Skin inflammation (eczema)
  • Inflammation of the joints (arthritis)
  • High blood pressure (hypertension)
  • Stomach ulcers
  • Asthma attacks
  • Exacerbation of disease activity
  • Relapse of disease activity
  • and more

For those of you who have any one of the pemphigus/pemphigoid (P/P) related skin diseases, stress is the number one factor in flare-ups occurring. The mind-body connection is very strong and stress encourages the antibodies to act up and give you more blisters. Trying to stay even-minded is a big challenge. Easier said than done, but with practice, it can be accomplished! Any kind of exercise like yoga, swimming, or taking a brisk walk can be calming and fun at the same time! Meditation is also great tool for relaxing and can be done in the privacy of your home. If your mind wanders…just focus on your breath.

Deep breathing can help to calm down the feeling of anxiousness that stress can cause. You can inhale to the count of 10 (working up to 20), hold the breath for 10 and then exhale at the count of 10. Doing this with your eyes closed should help you to feel more relaxed.

Listening to music can be very relaxing. Dim the lights and lie down, breathe and just let yourself float away! YouTube has great choices if you do not already have your own easy listening library. Examples of music that you could search for are “yoga music,” “relaxing music for stress,” or “calming music for stress and sleep.” There many options available! Just type in the kind of music you want and YouTube has it.

Be creative in your de-stressing activities! Everyone finds activities to be relaxing, and it is important to find what best calms you. Some suggestions for de-stressing activities are:

  • Going for a walk with your dog
  • Going to the movies – especially to see a comedy (laughter can be the best medicine!)
  • Go for a hike
  • Adult coloring books
  • Photography
  • Fishing
  • Gardening
  • Cooking
  • Spending time with friends
  • Turning off all technology
  • Crafts
  • Go to the gym
  • Take art lessons
  • and more!

Anything you can do to take your mind off of the stress of having P/P will be helpful, and is your best tool in addition to what your physician prescribes.

There are two things I can specifically recall never wanting to do: public speaking and participating in politics. This is exactly what I find myself doing recently though when the IPPF asked if I would be interested in meeting with the staff of my Senators and Representatives in their local officers to advocate for rare diseases like Pemphigus. I had no idea what this would mean, but I knew this is exactly what I and my rare disease community needed. Having a rare disease has taught me about the beauty of life and not wanting to miss out on any opportunities before me. It has also taught me about helping others and self-growth.

At first, I did not think it was possible to have a full time job as a nurse and meet with politicians, but the IPPF worked with me over the phone to prepare for my meetings and to discuss bills pertaining to rare diseases. I also immediately went online to research my members and their voting records concerning rare diseases.

I met with the staff of Oklahoma Representative Steve Russell, Senator James Inhofe and Senator James Lankford. I found speaking with Congressional staff much less intimidating than speaking with the Congresspersons directly might have been. I was prepared to ask that Representative Russell to join the Rare Disease Caucus in the House of Representatives and to vote for bills allowing patients participating in clinical trials to be exempt from taxing their financial compensation. I was surprised to discover that there were no coalitions in the Senate for rare diseases. When I met with my Senators’ aides I asked that the Senate form such a coalition. I also asked for support of a bill that would allow off-label drugs to be used for multiple purposes.

I am neither brave nor eloquent with words but the knowledge that I could encourage the passage of bills to find cures or treatments for people all over the world was inspiring. I quickly found my courage and knew my purpose. The meetings that had initially filled me with anxiety ended up being great. The Congressional aides I spoke with and shared my heart with actually listened! I shared my personal story and asked for their support helping our cause. There was even an emotional moment with a senator’s staff member when our passion spilled over. I am now open to whatever is in store for me as a rare disease patient and advocate. I hope that our combined effort takes the rare disease community closer to a bright future of hope and cures.

Dealing with insurance is not fun, especially when you’re in pain, and don’t know what the future holds.

My experience has shown, that the first place to start when you have a problem is with your insurance company’s customer service. What I have found, is that when I call customer service, I can get very different answers for the same question depending on who I talk to. So depending on the complexity of your issue, I would encourage you to make more than one call to see if you do get a consensus. We hope that we will, but if information wasn’t understood, or given incorrectly, you are on your own to straighten it out.

When I had my Rituximab treatment, what I actually got from my insurance was  in writing before the treatment. Customer service sent me a document that indicates what charges I could expect.

Labs are another thing to keep an eye on. When seeing several doctors, many of them want their own labs. It’s very easy to get duplicate lab work. Make sure to check your lab records, and what labs your doctor is ordering. For example one complete blood count (CBC) will do. In many cases your doctor will accommodate not duplicating labs, but it is up to you to let them know if you’ve already had recent lab work by another doctor.

Generally it’s pretty easy to get that lab work to the other doctor. I actually had a doctor who  ordered a CBC when I had just had one recently from another doctor in the same clinic. When I brought it to her attention shortly thereafter, she was very accommodating letting billing know so I wouldn’t get charged. She wouldn’t have done this if I hadn’t brought it to her attention.

There are many other areas where being alert and not afraid to ask questions can pay off.

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

Most individuals have a primary care physician. Many have a dentist. Many women have an OB/GYN for women’s health.  There are a variety of specialists’ that one may have: Internists, Ear/Nose & Throat (ENT), Ophthalmologists, Rheumatologists, and more.

When you are being treated for any form of pemphigus or pemphigoid (P/P) and are on any of the various treatments it is best to keep all of your doctors in the loop.  Each one should know of your present condition and the medications that you are taking as this can affect your diagnosis with each specialty examination.

If you will be considering an invasive surgical procedure of any kind, (including dental work) both your surgeon (and dentist) and your dermatologist need to be in touch.  Usually, your P/P medications can be increased for a few days prior and a few days afterwards to prevent any flare-ups.

Also advise your dental hygienist when you are having your teeth cleaned, so that he/she can be extra gentle with your gums.  (Many Hygienists are just now learning about P/P through the IPPF Awareness Campaign!)

When you are a new patient at a doctor’s office and filling out the information sheet, be sure to list all the medications you are taking for your P/P condition.  Your new doctor will then ask you about them and be aware of your situation.

Remember, when you need us we will be in your corner!

What are Pemphigus and Pemphigoid
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