Yearly Archives: 2010

MedWire News: Having a neurological or psychiatric disorder, being bedridden, or being a chronic user of various drugs significantly increases the risk for bullous pemphigoid (BP) in elderly individuals, say researchers.

“A rise in the incidence of BP was documented recently in Europe, and the main risk factors for BP remain unknown,” write Sylvie Bastuji-Garin (Université Paris-Est, Créteil, France) and co-workers. This condition is also much more common in people over the age of 60 years than in younger individuals.

To investigate possible reasons for this, the team selected 201 individuals (64.7% female), aged 84.2 years on average, with incident BP and 345 controls who were matched for gender, age, place of residence, and center.

Diagnosis of BP was based on identification of typical clinical features and on direct immunofluorescence showing linear deposits of immunoglobulin G and/or C3 along the basement membrane zone.

Drug use over 3 months, comorbidities, and physical and cognitive impairments were compared between cases and controls.

As reported in the Journal of Investigative Dermatology, multivariate analysis showed that major cognitive impairment, being bedridden, having Parkinson’s disease or unipolar/bipolar disorder, or chronic use of spironolactone or phenothiazines with aliphatic side chains increased the risk for BP a significant 2.19-, 2.19-, 2.16-, 5.25-, 2.30, and 3.70-fold, respectively.

In contrast, chronic use of analgesics reduced the relative risk for BP by a significant 51%.

“These findings may have implications for the management of BP patients,” write the authors.

“Moreover, they indicate a need for further investigations into the association of BP with neurological disorders,” they conclude.


Pemphigus vulgaris (PV) is the most severe autoimmune blistering disorder of the skin that is mediated by circulating autoantibodies against desmoglein 3 (Dsg3). It has been reported that in Jews the associated haplotype in PV is human leukocyte antigen (HLA) B38, DRB1*0402, DQB1*0302. Significant associations with HLA were observed also in non-Jews. Dsg3-specific T-cell responses were detected in PV patients but also in healthy individuals who were either carriers of the PV-associated DRB1*0402 allele or alleles that share similar or identical peptide binding motifs to DRB1*0402. This suggests that genes other than the classical major histocompatibility complex (MHC) genes are associated with the development of the autoimmune response. We used 16 microsatellite probes that span the entire MHC region to screen DNA samples from 38 PV patients and 76 healthy controls. Results demonstrated that some markers were associated with class II region including a TAP associated marker. However, four probes, D6S265, C_527, D6S510, and MOGC, which are all mapped to the region of HLA-A, were highly associated with PV. These results suggest that a gene, or genes in the class I region are important in the initiation of the autoimmune cascade. Activation/suppression of these genes might act as the trigger mechanism that starts the autoimmune destructive process.


Terry Wolinsky-McDonald, PhD (PV patient and IPPF Board Member)

The holidays and end of year are nearly upon us as I write this article.  This time of year brings so many different and conflicted feelings for most people, usually very personal and emotional.  As noted previously, while so many look forward to so much, many people have bittersweet memories and difficult standards and goals for which to strive during the holidays and into the New Year.  It is a time for remembering activities and traditions of previous holidays but also the losses (health restrictions, jobs, life styles and people). For some there have also been improvements in health and treatments and new traditions.  Others hold onto the more troubling thoughts and feelings and have great difficulty moving on.  Sometimes the illness itself is not responding to treatment yet or is being stubborn and brutal.

Tears often represent unresolved sadness and grief, in addition to actual clinical depression.  When living with chronic illness patients and caregivers can’t help but experience, a mix of depression and anxiety from changes in the status quo.  After all, chronic illnesses don’t go away, which is why they are called chronic to begin with.  Seeing family and friends, not seen often, during the holidays often brings people into the position of being seen as SICK and HELPLESS or being told they look BETTER and so must be doing/feeling better.

And, don’t forget that tears can be positive, too.   I often tell patients that tears are allowing toxins to leave the body; who doesn’t want a less toxic body?  People also cry when happy or just plain sentimental.  I have one friend who never ceases to make me laugh.  I keep telling him to take his act on the road!

Sometimes it is just easier to not discuss at all since “chronic illnesses” seems to be such a mystery to so many, especially a rare or ultra rare disease. One person I know actually carries around a few copies of the dictionary definition of chronic in order to avoid explanations. If there is loss of energy it may feel like running a marathon just getting together with family and friends, even when not trying to “host” the holidays or a get-together.  Travel can feel like a triathlon; sometimes just getting out of bed, let alone the home, can be excruciatingly difficult.

The essential ingredient in getting through this is acceptance; think of acceptance as the main ingredient in the recipe.  If unable to get to acceptance, try putting hurtful feelings on hiatus in order to get through difficult periods.  I do not advocate pushing yourself beyond your limits, but one thing psychologists learned from the “behaviorists” (Skinner, Watson, et al) of the 1960’s is that one does not have to feel wonderful or have tremendous insight to effect changes:  making changes, even small ones, can actually make you feel better.  For example, just going to a gathering may make a person feel better, even if there is a need to leave early.  Getting more dressed up, and seeing oneself in a mirror, allows the person to see themselves more as they once were and may again be.

Fears of the uncertainty of it all are real and often keeping anxiety high or ever-present. With chronic illness one never knows what the next day will bring.  Expectations of what one can or cannot do may be unrealistically high or low.  In either case, anxiety may remain a constant.  Some people even have actual panic attacks, where they have trouble breathing and chest pain, simulating a heart attack.  If on high doses of a medication like prednisone, life will feel like a roller coaster . . . because it is!

Medications can help, and are often necessary. Therapy can teach new ways of coping that will be absolutely necessary to get back in (limited) control and to accept to help move on.
Sometimes techniques can be learned in therapy or from self-help books.  I am not a great fan of most self-help books, but new ideas and ways of handling things will be helpful, however they are learned.  When new circumstances take people back to old unresolved events or issues that is when psychotherapy will be absolutely necessary.

Fortunately the stigma once attached to the need for psychologists and psychiatrists has changed for most of the population.  Asking for help in just understanding why one feels as they do is the first step in taking this course.  The support and understanding of others, your support system or lack of one, is never to be underestimated.

So, where are the cheers to come from?  How about improvement in the illness’ impact, adjustment to this new life, just being able to sit comfortably or to eat and enjoy food again, feeling more stable, being in partial or full remission, fewer or less harsh drugs?  Any or all of the above and so much more are cause for celebration.

What are my recommendations for the holidays and New Year?  Take a lot of pictures to help create new memories; try at least one new thing; let the people you love know how much you love and appreciate them in your life; smile more; let go of small disturbing things; say hello to someone you don’t know.

Cheers to all for a happy and healthier New Year.  Remember, the IPPF Annual Meeting will be in Detroit in 2011 – May 20-22 – a time to learn from the experts, meet or reunite with others, and to feel connected.

I hope to see you there!

Dosage and indication “creep” with the new biologics to treat inflammatory diseases have payers enforcing strict utilization policies. Physicians counter that payers can’t dictate treatment. With even more biologics on the horizon, and drug spend spiralling upward, both sides need to seek a middle ground. The question is how?

Read more at:

In the latest effort to break up the often cozy relationship between doctors and the medical industry, the University of Michigan Medical School has become the first to decide that it will no longer take any money from drug and device makers to pay for coursework doctors need to renew their medical licenses.

If you turn on a light switch for a room to illuminate it and then the television, do they both use the same amount of electricity? The answer is no, they do not.

It felt as if a small village illuminated with full power at the Annual Patient/Doctor Meeting held at the Sheraton Society Hill Hotel in Philadelphia. When I arrived, my room was ready. So I did some quick sightseeing with Janet [Segall, IPPF Founder]. Philadelphia is historic on its own; the crack in the liberty bell like the crackle of electricity was something of which to be in awe. When you combined the location, the group of rarities that we all are, and the wonderful speakers and sponsors, I have to tell you, popcorn was popping!  What a way to get in the mood!

Speaking of sponsors, a heartfelt “Thank You” goes out to Centric Health Resources, AxelaCare, Crescent Healthcare, National Rehab, and Alwyn Cream! Their continued support helps meetings like this one reinforce the Community concept!

Friday night’s mixer was very relaxing.  Those in attendance could mingle with each other, the doctors, the sponsors, Molly [Stuart, CEO] and Will [Zrnchik, Director of Communications]. I chose to sit with a small group of women for a bit, then went from table to table and began just asking people who they were.

One big table had me repeat the names, and I did pretty well until the last one. I blamed it on the prednisone. I introduced some to others that were in search of answers to some questions. I sat back and observed the camaraderie of the group. Intense conversations and laughter filled the air. Will was on spot when I whipped out my camera – as if on cue, he turned his head and smiled.

Saturday’s breakfast was magnificent, and I felt so much better once I had my café mocha with a shot of hazelnut. Oh, I was a happy little camper and ready for anything with a big old smile on my face. Everything seemed as if it was in its own perfection. The booklet with the weekend layout was great in the sense that you could follow along. I sat in the back, listened to Dr. Grant Anhalt’s presentation, and understood it.  Please allow me to take a moment and thank whomever it was that came up with the idea of espresso in the morning before medical doctors and panels spoke. I believe Gloria Papert did an excellent job of keeping the times allotted for speaking. She is such a jovial woman; you could not help but smile when she spoke to you.

The breakout sessions were clearly marked, and I chose to listen to NDRI, which stands for National Disease Research Interchange. A lot of interesting information on donating tissue and blood samples that is extremely important in order to one day have a cure. I grabbed a booklet, but have not had time yet to fill out the information. I, for one, am totally for this. My skin comes off with the PV anyway, so I am not missing anything.

The Awards Luncheon was tasty! Dr. Jean-Claude Bystryn received the Founder’s Award and Janet accepted it on his behalf. I personally would like to wish him a full recovery from his illness. I have only met him twice,  but can see that he has dedicated his life in hope of controlling these complicated diseases.

I have to admit I was floored when Molly announced an award for my writings. I was not expecting anything of the sort, but it meant a lot. I just have a gift of gab. Somewhere I can hear my mother telling me to keep quiet, but she is smiling in her heart.

When lunch was through, Dr. Ani Sinha gave a very informative lecture on Why Do Patients Have Flares?  My second cup of espresso kicked in during his question-and-answer session. Dr. Dave Sirois had a breakout on Oral and Dental Care. Something interesting with Dr. Sirois: During the raffle, he whipped out about a dozen tickets. That was ok, except I noticed he did not put the other halves in the boxes. I informed him of this and his statement was, “Well that explains why I never win anything!”

Saturday evening was “on your own” or a movie night and many people decided to go out, which was nice. I ate dinner with a small group and enjoyed their company immensely. I love to hear stories as well as tell them. Brian Cleary from AxelaCare was like a personal map/tour guide because Philly is where he lives.

I felt for Will, he was running around like a mad man all weekend and his coffee was usually cold. He gave up his animal printed tie to me to add as a band for a sundress I am making. He just had to explain it to his other half. Wonder how that went… 😉

Anyway, bottom line is that the lights did not go out. With a walk to Penn’s Landing to see the old masted ships in the harbor, I went back to the hotel and had a “special” decaf nightcap. Unfortunately, I had to leave early Sunday to come back to my family. It was nice to run away for a weekend  and share something that so few of us know.

To my other family, thanks for the laughs, the conversations and the fun. You are the entire “crack in my bell” — but the electricity is what keeps us connected.

We’re fresh off of the delight of connecting with everyone available at the Annual Meeting (AM) in Philadelphia. Read more in this issue and visit our website at for additional materials.

The IPPF capstone project for 2010 is documenting 1,000 pemphigus/pemphigoid patient medical data records in our Registry — launched at the AM. This many records in the registry places us in position to ask for funding to support a research grant, inspire a scientist to conduct research, or provide data to move drug company developments in treatments.

Last year we addressed personal patient support needs with the Health Management Program (HMP). If you need personal support managing your disease, have specific questions, or want to discuss options with someone who’s been there, you need the Health Management Program and a relationship with one of our Peer Health Coaches (PHC). (See Dr. Sirois’ article on the demonstrated benefits in improved heath from participation on page 4)  Let us know and we can set you up.

The Registry is an animal of a different stripe. In the Registry, instead, we need something from each one of you  — we need your data — all confidential, of course (read more about our Registry’s privacy policy at Not “we” so much, but P/P patients of the future. For the benefit of the future collective of P/P patients, we need you to complete the registry so your challenges can be counted and addressed.

We will be sending every patient that we have email contact with a unique log-in to privately enter your information from your personal computer. Please, please take the 15 minutes or so to document your experience and illuminate the urgent needs of this community — for awareness, resources, scientific discovery and everyday solutions. Last year we asked you to dig deep to help us leverage the opportunity for a matching grant of financial support. This year we need to dig deep to make the collective of your voices and experiences heard — for everyone’s progress.

We are at an exciting milestone, providing proof of the burden of disease and the path to discovery of effective new treatments. Please dig deep, once again — this year with your time — to support this community.

If you don’t, who will?

Three years ago a joint task force of the IPPF comprised of expert members of our Medical Advisory Board, Janet Segall (IPPF Founder) and Centric Health Resources (a Missouri based company dedicated to improving the quality of life for people nationwide with rare, orphan, ultra-orphan, and chronic genetic disorders), conceived and developed a Health Management Program (HMP) with the goal of improving illness experience by providing patients with educational resources, personal support and improved access to doctors and medicines. How did they decide to make all that happen?