Yearly Archives: 2007

My pain started four years ago, in April 2010. I distinctly remember the raw feeling in my mouth that appeared unexpectedly as I struggled to eat a meal with my family. Over the next few days, painful sores that were larger than usual mouth ulcers began to form in my mouth. I couldn’t remember ever having mouth sores, ulcers, or any mouth condition in my lifetime. The constant pain and the fact that I was finding it increasingly difficult to eat or drink made me concerned, so I booked an appointment with my general practitioner (GP).

Initially, my GP thought it could be thrush and prescribed basic antibiotics, mouth gels, and other remedies. They seemed to work for a while and eased the pain a little, but once I had completed the treatments, the sores reappeared, only more aggressively.

It was a toothache that led me to book an appointment with my dentist. She strongly suggested I see an oral consultant. At this point I was even more worried about my condition, as the sores were not going away and I was in constant pain. I began to research possible causes for my condition on the Internet, which I am not sure was a good thing to do as it made me imagine I had all sorts of possible diseases.

Near the end of June, at my first appointment with an oral consultant, I was diagnosed with possible geographic tongue. As the condition of the rest of my mouth became progressively worse, I decided to get a second opinion. The diagnosis from the second oral consultant was stomatitis vegetans, and I was prescribed metronidazole, Biotene® Gel and fluconazole. This treatment initially seemed to help, but after a while the oral lesions were widespread in my mouth, which led to my having a biopsy of my tongue.

Eventually, a biopsy confirmed I had pemphigus vulgaris (PV). Like many others, I had never heard of this condition. My oral consultant explained that PV was a rare autoimmune skin blistering disease for which there was no cure, but it was treatable. I was stunned and frustrated, but I also felt some relief that I finally knew what my disease was. The most disappointing news was that there was no cure, but I had faith in my oral consultant and the professor at Guys Hospital in London and believed that they would do everything they could to help me. I am also extremely lucky to have such a wonderful family and friends who have supported me during my suffering, and I cannot thank them enough. Unfortunately, I was unaware that worse was yet to come.

In a strange way, I seemed to be getting used to having this pain on a daily basis and it soon became the norm.

Between November 2010 and March 2011, I was prescribed a steroid mouthwash and continued to take metronidazole with some success. I also had regular reviews with my oral consultant. I continued to suffer on a regular basis with major flare-ups in my mouth and difficulty eating and sleeping. In a strange way, I seemed to be getting used to having this pain

 

 

 

 

 

 

 

Will-Web-headsotWelcome to the final issue of 2014. It’s been an exciting year for the IPPF and we hope 2015 brings more of the same. I’d like to welcome Todd Kuh to the IPPF Board of Directors. Todd is a PV patient from Southern California and avid cyclist.

Our 2015 Patient Conference will be in New York City at Mount Sinai Hospital, April 25-26, 2015. Dr. Annette Czernik is helping plan the event and Biofusion is sponsoring our Friday Night Conference Kickoff, April 24, 2015, with dinner and refreshments at Yankee Stadium.

You may have seen a letter in the mail from my good friend Dave Baron asking you to support the IPPF. I met his father and sister at our 2006 Annual Meeting because he couldn’t make it (his PV made travel impossible). The IPPF helped him find a doctor, educated him on treatments, and supported him every step of the way. Now in remission, Dave doesn’t want patients to suffer like he did. Join him and his family with a generous gift to the IPPF this Holiday Season at www.pemphigus.org/donate.

A Quick Recap

In 2014 the IPPF celebrated our 20th Anniversary. Noelle Madsen and Patrick Dunn joined our team (and Todd with the BOD). The Patient Education Series launched revamped Patient Education Calls averaging 75 registrations each. Our Patient Conference had 125 attendees and raised over $18,000. Social media grew nearly 1,000%. 450+ patient cases were closed. We supported 8 pieces of legislation benefiting patients. A dozen local support group meetings were held. The Physician Referral List grew 5%. The Awareness Campaign (AC) formed a Dental Advisory Council. We finalized patient and physician videos. We are supporting clinical trials. The AC presented at the American Dental Association and to dental students at Indiana University, conducted focus groups, and was published in the American Dental Hygienists’ Association’s Access Magazine. The Awareness Ambassador Program launched. We unveiled our new website in August and the Awareness Campaign’s in December. And I could go on!

What’s in store for 2015?

Rare Disease Day. National Autoimmune Disease Awareness Month. Patient Conference. Patient Education Calls…and videos! New printed and downloadable information. Awareness Ambassadors. Patient Advocacy tools. New ways to support the IPPF. Dental Professional and Student Education. And much, much more!

It’s an honor to serve this wonderful community of friends and family.

My family, and the entire IPPF family, wishes you and yours a safe and Happy Holiday Season!

 

 

When you experience disease activity in your mouth it can be quite uncomfortable.  Patients may experience blisters anywhere inside the oral area: inside of cheeks, upper and underside of tongue, roof of mouth, and as far back as where the uvula is. The gums can peel as well.

Swallowing can be difficult. If this occurs for you, having anything soft is advised. For example, smoothies, yogurt, mashed potatoes, cream of wheat, etc. Avoiding citrus fruits is recommended, as that can agitate your oral lesions.

If your gums are peeling, ask your dermatologist if he/she can prescribe to you a topical corticosteroid. A ‘Magic Mouthwash’ can also be prescribed.

Try not to use alcohol-based mouthwashes as it can be uncomfortable to your lesions. Gentle toothpastes such as Sensodyne or Toms of Main can still be too harsh. If those products are irritating your lesions try going the old-fashioned route of using a paste of baking soda and water.

The use of straws is not recommended if you have flare-ups in the mouth as this can irritate them.

The IPPF suggests that you keep a food journal, so that if a flare-up occurs you can look at the list of foods you have consumed prior to the flare-up and determine which food or spice could be the culprit.

Keep your gums as healthy as possible by using a waterpik on a low speed, and use a very soft toothbrush. Regular dental checkups should be continued as normal, and if you’re going to have any dental work done advise your dermatologist. Depending on the level of activity you have and the medications you are taking, your dosage may be increased a few days prior and a few days after the procedure.  Advise your dentist of this, as well.

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

Mei Ling Moore – Peer Health Coach

If you’re like me you do a lot of shopping online. It’s convenient. It’s safe. And sometimes the prices just can’t be beat. To make sure I get a great deal from a reputable company, I do most of my shopping through AmazonSmile (smile.amazon.com, part of Amazon.com).

My AmazonSmile homepage

My AmazonSmile homepage

AmazonSmile (smile.amazon.com) is a simple and automatic way for you to support the IPPF every time you shop, at no cost to you. When you shop at smile.amazon.com, you’ll find the exact same low prices, vast selection and convenient shopping experience as Amazon.com, with the added bonus that Amazon will donate a portion of the purchase price to the IPPF.  To shop at AmazonSmile simply go to smile.amazon.com from the web browser on your computer or mobile device. You may also want to add a bookmark to smile.amazon.com to make it even easier to return and start your shopping at AmazonSmile.

There are tens of millions of products on AmazonSmile are eligible for donations. You will see eligible products marked “Eligible for AmazonSmile donation” on their product detail pages. Recurring Subscribe-and-Save purchases and subscription renewals are not currently eligible.

The circled area lets you know the product is an AmazonSmile participating product.

The circled area lets you know the product is an AmazonSmile participating product.

It’s easy to use because you use the same account on Amazon.com and AmazonSmile(smile.amazon.com). Your shopping cart, Wish List, wedding or baby registry, and other account settings are also the same. When you visit AmazonSmile (smile.amazon.com) for the first time you need to select the International Pemphigus Foundation to receive donations from eligible purchases before you begin shopping. Amazon will remember your selection, and then every eligible purchase you make at smile.amazon.com will result in a donation to the IPPF.

amazonsmile

You just left your doctor’s office, and you’ve been told that they want to try Rituximab (Rituxan/Mabthera). Most likely, the doctor explained how the treatment is a B-Cell inhibitor, and that it is a very targeted therapy designed to eliminate those cells in your body that are attacking the proteins in your skin. Your doctor has informed you that it is an infusion, reviewed the treatment schedule and protocol with you, possible side effects, and answered any other questions or concerns that you have. Your doctor most likely forgot to tell you about one very important aspect: How will you know if the Rituximab is working?

If the Rituximab is supposed to eliminate your B-Cells then it should be measured exactly that way. Ask your doctor to perform a baseline test to determine you B-Cell (CD20) count prior to your first infusion. A follow up test should be done at the conclusion of each treatment cycle to measure the decrease in your B-Cells. Remember, it may be necessary to have several cycles to eliminate these cells but the best way to check is a simple blood test.

Along with this B-Cell baseline test, it is recommended that your physician performs a thorough pre-treatment screening including:

Your Medical History – Any history of cardiovascular or pulmonary disease, recurring infections or allergies.

Physical Examination – Review all medications and possible contraindications.

Other tests should include; Chest X-ray, routine blood test, Hepatitis B screen, and immunoglobulin levels

Our disease and the treatments can be confusing, so if you’re not sure just “Ask a Coach”!

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

Marc Yale – Certified Peer Health Coach

While you are seeing a qualified dermatologist who is treating you for your Pemphigus Vulgaris, Bullous Pemphigoid, Pemphigus Foliaceus, Mucous Membrane Pemphigoid, etc. you might also be seeing your own dentist, OB/GYN, internist, ophthalmologist or ear/nose/throat specialist.

Please be sure that all of your doctors are aware of your condition and that they have access to your dermatologist.  It is important that they know the medications and dosage that you are taking for each medication.

All of your doctors need to be able to communicate with one another if necessary.  Being left in the dark will leave you at a disadvantage.  Also, if you are going to be scheduled for any major dental work, advise your dermatologist.  Depending on the procedure, your medications may be adjusted for a few days prior and a few days following to prevent any flare-ups.

Remember when you need us we are in your corner!

Many times when seeing a physician for pemphigus or pemphigoid they are quick to prescribe a systemic treatment that will hopefully help you reach remission. This can be a good thing. However, sometimes the obvious may be overlooked.  For example, if you are in pain,  having trouble eating or swallowing, your clothes are sticking to your lesions, the blisters on your scalp make bathing and showering difficult, or perhaps you are having chronic nosebleeds. These symptoms can be managed with topical treatments, but they are often forgotten. There are different options available for different body locations in many different strengths. Be candid with your doctor and let them know where you are having disease activity and how severe it is. Although, ultimately, the systemic treatment is going to make the difference in the long run.  Topical treatment can help relieve many of your symptoms along the way!

If you’re not sure which medications to ask for or their strengths, just “Ask a Coach”!

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

On September 9, 2014, members of the IPPF traveled to Capitol Hill in Washington D.C. to talk to their local congress members about legislation affecting the pemphigus and pemphigoid community.

Senior Peer Health Coach Marc Yale, and Patient Services Coordinator Noelle Madsen spoke with six California members of the House of Representatives, and Senators Barbara Boxer and Diane Feinstein.  Marc and Noelle sought support for the Medicare Advantage Participant Bill of Rights of 2014 (H.R. 4998/S. 2552).

Medicare Advantage Plans are removing dermatologists (and other specialty physicians) from their networks. This gives insurance companies to ability to eliminate doctors who prescribe vital, but expensive treatments to pemphigus and pemphigoid patients.  As a patient or caregiver, you already know how difficult it can be to find a doctor that can treat P/P.  Imagine having that physician removed from your insurance. This would be extremely harmful to a patient’s current quality of care.

Marc and Noelle also discussed the Patients Access to Treatments Act of 2013 (H.R. 460).  This bill would increase National Institutes of Health (NIH) funding by $1.3 billion.  The more funding the NIH gets, the more research can be done for rare diseases like pemphigus and pemphigoid.  This bill would also prevent insurance companies from increasing “tier four” treatment costs.  Many pemphigus and pemphigoid treatments are considered tier four, and increased costs to these treatments could negatively affect quality of care.

The IPPF feels these pieces of legislation are extremely important to the pemphigus and pemphigoid community. We urge you to contact your representatives and senators to ask for their support of these bills.

If you have questions about these, or other legislative affairs, please contact the IPPF at advocacy@pemphigus.org, or call Noelle Madsen at 855-4PEMPHIGUS extension 105.

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Our immune system is a killing machine. It consists of various types of specialized cells and proteins that function to destroy invaders and “non-self” or mutated “self” proteins, such as those that come from virus78_RH image_optes, bacteria, and cancer cells. In the autoimmune diseases such as the P/P diseases, this mechanism has gone awry and the immune system actually attacks its own cells.

In P/P patients, antibodies generated by B cells of the immune system block the function of desmoglein proteins Dsg1 and Dsg3 known to be important in binding together keratinocytes of the skin and mucous membranes, but it is not known how the rogue antibodies are generated by the immune system, how they escape the quality control mechanisms in place that only allow B cells with non-“self” specificities to survive, and why P/P patients are so rare.

New research led by Dr. Aimee Payne in the Department of Dermatology at the University of Pennsylvania (Nature Communications, http://www.nature.com/ncomms/2014/140619/ncomms5167/abs/ncomms5167.html) helps us begin to understand why.

In previous work, Dr. Payne and colleagues have identified antibodies that recognize Dsg1 and Dsg3 (so-called anti-Dsg1 and anti-Dsg3 antibodies) and have also identified regions of those antibodies that are important for the ability of those antibodies to be pathogenic — that is, to recognize their Dsg targets in pemphigus vulgaris (PV) and pemphigus foliaceus (PF) and to disrupt their function. To extend this work and to better understand how PV autoantibodies arise, Dr. Payne and colleagues have performed a similar analysis of PV patients.

PV patients can present as either mucosal-dominant, where only the mucous membranes are affected or as mucocutaneous, affecting both the mucous membranes and the skin. Almost all mucosal-dominant PV patients have anti-Dsg3 autoantibodies, while the mucocutaneous patients have anti-Dsg3 autoantibodies as well as anti-Dsg1 autoantibodies. Since it is thought that Dsg1 and Dsg3 can compensate for each other’s function, the presence of functional Dsg1 in the skin in the presence of anti-Dsg3 autoantibodies can explain why mucosal-dominant patients do not have skin lesions.

The authors first isolated the full antibody repertoire from four different untreated PV patients, all with mucocutaneous disease. They isolated and characterized these in a multistep process that ultimately allowed them to determine the amino acid compositions (by cloning and DNA sequencing methods) of the patient’s PV antibodies. This led to the assignment of six unique antibodies from Patient 1 and five additional unique antibodies from the three other PV patients.

In total, the sequencing efforts identified 21 unique heavy chains among the four patients.

All 11 antibodies could bind to Dsg3 and this was mediated via a domain (called EC1) in Dsg3 that is known to be important for its adhesive interactions, suggesting that anti-Dsg3 autoantibody binding to Dsg3 leads to a direct block in Dsg3 function in keratinocytes (and subsequent skin blistering).

Curiously, not all of the antibodies that the authors identified that bound Dsg3 could cause blistering when added to human skin tissue samples; the VH1-46-containing antibodies did. They determined that these differences in functional effects were due to the inability of the nonpathogenic antibodies to bind to the functional domains of Dsg3.

Even more curiously, the authors found that all four patients had at least one PV antibody that consisted of an identical variable region termed VH1-46. They also found very little change in the VH1-46 amino acid sequence in the patient antibodies compared to the known sequence of VH1-46 that also exists in unaffected patients (considered the “wild-type” or germline sequence).

As noted by the authors, this is a pattern typical of a somatically mutated antibody sequence, meaning that very few changes were generated during the development of the B cells (each with its own single antibody that it makes, see Figure).

They did some additional experiments to define the ability of those amino acid changes to affect the binding to Dsg3. They conclude that VH1-46 autoantibodies in PV are generated during B cell development and require very little mutation to become pathogenic. This suggests that they appear early during the development of the disease and explains their prevalence in all of the patients tested here.

These autoantibodies may not be the most common later on (during full-blown disease), but they may provide a clue to why and how pemphigus arises. The ability of these autoantibodies to escape the quality control machinery at play during B cell development is likely due to the low levels of Dsg3 antigen available that would distinguish these antibodies as “self” antibodies and therefore the ability of the machinery to mark the cells and their rogue autoantibodies for destruction.

These data led the authors to speculate that the five pathogenic (disease-causing) VH1-46 anti-Dsg3 mAbs that they’ve identified in this study are among the earliest autoantibodies formed in PV patients, caused only by how simple they are to generate from germline sequences. They also define a mechanism for how these autoantibodies are made and most importantly, how they are missed by the quality control machinery – all low probability events that likely account for the rarity of PV.

One of the first things to remember about illnesses and the side effects of medications is the effects of illness are not just physical. There is an emotional component as well.

For example, the prednisone roller coaster is both physical and emotional. The ups and downs often have patterns and triggers, and these are not always predictable. The mere fact of having an illness can lead to depression, with or without side effects from medications.

Psychologists have been called “an angry bunch of shrinks” (Newsweek, December 2013) because of their collective response to new and unsettling upcoming changes in current diagnostic criteria and standards. The Diagnostic and Statistical Manual (DSM-IV) of the American Psychological Association has been the “bible” of the psychiatric profession for more than a decade, with the new version (DSM-V) going into effect in October 2015. The physicians’ ICD-10 (or International Statistical Classification of Diseases and Related Health Problems) will also be issued at that time.

In this article I will review some current diagnoses and criteria related to depression. With the aforementioned changes more than a year away, now is a good time to go over the diagnostic criteria for depression as outlined by the DSM and ICD standards. Lenore Sawyer Radloff’s Screening Test for Depression (see p. 17) can be used to monitor your own symptoms and patterns.

One mood disorder in the current DSM-IV is simply called “Mood Disorder Due to ____________.” The blank is filled in with a specific general medical condition, such as pemphigus vulgaris. The diagnosis may develop into a clinical depression over time, which has a different etiology. The diagnostic criteria for these generic mood disorders include:

A prominent and persistent disturbance in mood predominates in the clinical picture and is characterized by either (or both) of the following: Depressed mood or markedly diminished interest or pleasure in all, or almost all, activities. Elevated, expansive, or irritable mood.

There is evidence from the history, physical examination, or laboratory findings that the disturbance is the direct physiological consequence of a general medical condition.

The disturbance is not better accounted for by another mental disorder so as to distinguish this general mood disorder from “Adjustment Disorder With Depressed Mood” in response to the stress of having a general medical condition, another clinical diagnosis.

The disturbance does not occur exclusively during the course of a delirium.

The symptoms cause clinically significant distress or impairment in social, occupational, or important areas of functioning.

Common symptoms of depression look different in each circumstance and with each individual. A diagnosis may be given if there is a prominent and persistent disturbance in mood that predominates in the clinical picture, and it is further characterized by five or more of the following:

Persistent feelings of sadness

Difficulty sleeping or excessive sleeping78_psychspeaking_opt

Poor or increased appetite

Weight loss or weight gain

Anxiety, restlessness and agitation

Inertia: feeling “slowed down” or low in energy

Tearfulness or an inability to cry

Difficulty concentrating, remembering, or making decisions

Loss of interest in sex and other normal activities

Social withdrawal

Difficulty functioning at work, at home and/or in social situations

Irritability

Suicidal thoughts or passive thoughts of death.

Ill people will often try to hide their symptoms until they lose the energy necessary to keep up the act. After all, the last thing most people want is more prescription medications or treatments. This is more so when their bodies have already “betrayed” them and medications are necessary just to not get sicker. It is important to understand what is happening emotionally and to get a proper diagnosis. With a diagnosis can come appropriate treatment.

The simple 20-question screening test for depression can be self-administered. I often recommend that anyone who is concerned or has symptoms they do not understand make copies and re-test themselves roughly every two weeks. This particular screen looks at the feelings and thoughts for the previous seven days, so you could use it weekly if you wanted to.

I often use this tool as a handout at presentations. Patients (and caregivers) usually come up to me and express surprise at how many statements they have endorsed. Many have no idea these particular feelings and thoughts were actually signs of depression. As I noted above, with diagnosis there is treatment.

My philosophy is to refer patients to a knowledgeable psychiatrist for evaluation for possible psychotropic medication. The psychotherapy component may be a fairly short-term cognitive-behavioral model, or a more lengthy psychodynamic approach. The bottom line is that everyone is unique, and no one needs to feel worse than absolutely necessary. For some people this means medication, especially in the beginning, or more frequent therapy appointments. The doctor will monitor and make changes as necessary. Having said that, the sooner the emotional diagnosis and the sooner treatment begins, the better and faster the positive effects will be in stopping any potential downward spirals.

It is often easier to speak with a professional than with someone in your personal network. The key is to identify any problem areas and to address them, not just put on a band-aid when emotional surgery is necessary.