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- Giving Back
- As I am writing this, I am trying to hold onto a delicate balance of my clinical psychology practice and a special birthday party/fundraising event. The expression “it is better to give than to receive” means much more to me, and when I see a window of opportunity, I go for it.
Normally, I skip having an actual birthday party, opting instead for a small group of close family and maybe a few friends, a casual evening, and cake. This year was different.
Initially I considered a nice vacation for my husband and me because this birthday was a bit of a landmark for me, and I did want to do something special. However, the more I thought about it, the more I realized that I really wanted to celebrate the fact that I was still alive. This reason was more evident as I recalled the past 12 years and my diagnosis with two very rare autoimmune diseases, amidst the losses of my parents and some family and friends. The question was: How could I celebrate my special birthday and life, AND use this as an opportunity?
A seed was planted.
When we think of planting seeds, many people immediately think about the Spring. Here I am in early mid-Fall having my "a-ha" moment. Interestingly enough, my favorite flowers have been tulips since my first visit to The Netherlands more than 40 years ago. Unlike most flowers, tulip bulbs are planted in the Fall and then come up beautifully in the Spring. I love how these flowers normally bloom earlier than most, and at night or in darkness, they close to protect themselves, reopening when it is once again safe.
A seed was beginning to metaphorically grow in my mind. Why not use my birthday to have a special party/fundraiser/event where guests would be asked to not bring me a personal gift, but rather contribute to a charity of my choice?
Great! Now I was going to have a big party and not just a small, intimate gathering. What would be my first step?
I began to assemble a list of family, friends, acquaintances, colleagues, and the physicians who have been helpful during my journey. The list was a lot longer than I expected.
I kept going through the list. I couldn't find anyone I didn't want to include in my celebration. And to compound the issue, I found myself adding more and more names. I even added some out-of-town people, including my life-saving physician, Dr. Razzaque Ahmed from Boston. My original dermatologist in Pittsburgh, Dr. Judy Small, had worked closely with Dr. Ahmed over the phone during the first six years of my treatment (until I had to change doctors because of insurance and facility/provider changes). How could I have a celebration of LIFE and not invite the doctor whose ‘alternative’ treatment of IVIg was the reason I was alive? Remember, IVIg was very controversial back in 2001.
Since it was for charity, I was willing to go that extra mile. I am not sure I was using my old and trusty cognitive model -- without emotional interference -- but I was determined to stay on track. Finally, I decided on a private club where a dear friend (and a Vice President at PNC Financial Services, a 2012 Annual Meeting sponsor), had generously offered to sponsor my birthday event.
I enjoyed creating the menus, invitations, and RSVPs. Thanks to some technical help from my husband, everything got printed and mailed. Will Zrnchik, our CEO, set up a special Web page for the party so people could donate online.
The RSVPs started coming in and more people than I expected were planning on attending! This was getting big. What about entertainment? I realized I know a lovely and accomplished young woman who is the founder and creative director of a local ballet company whose studio was originally leased to Gene Kelly before he went to Hollywood! She had choreographed a perfect piece just for my celebration that could be performed by two ballerinas.
This effort took a lot of time and effort, but it was a night to remember. The party brought together people who did not know each other, and might not see each other again, but who kept telling me they had been at the "fun" table. The funny part is they were all at different tables!
I was blown away by Dr. Ahmed's attendance and the speech he gave to educate the important people in my life about the seriousness of my particular diagnoses (many were not aware of my conditions). Even my normally quiet, engineer-minded husband gave a very beautiful and emotional toast in my honor. I swear that if I didn't also have Sjogren's Syndrome I would have cried my eyes out!
Many of my guests asked me to make it a yearly event! While flattered, it probably won't happen at the level of this one, but I will definitely have smaller cocktail-like parties at home and continue to request donations in lieu of gifts.
It feels so wonderful to have both a landmark birthday and a memorable evening and celebration with friends and family who donated to IPPF. The reality is anyone can do this on their own comfort level.
The bottom line here is from that original "seed" came an evening I will never forget and will help others because of generous donations. The icing on the cake is most of my doctors got to meet each other face-to-face for the first time. And, it looks like Dr. Ahmed will be invited to talk to Pittsburgh doctors, residents, and others in the near future with a hope that it could drive some to study and research pemphigus and pemphigoid.
I am thankful to the generous local merchants who provided many items, services and gift certificates for the Silent Auction.
Knowing that this fundraiser can be repeated in small ways over the coming years gives me far more happiness than any one-week vacation could possibly have given me. I am not special for having planned this event, but I get a warm and wonderful feeling just knowing that I was able to embrace a new way of giving. I can absolutely validate that it is far better to give than to receive.
If you have an idea, let that seed evolve. See where it takes you. We can find new ways of giving -- whether with special skills, money, frequent flyer miles, volunteering time and services for one of the Foundations' many committees, educating people, or supporting others emotionally. The list is endless, and you will feel better knowing you are making a contribution that makes a difference.
I am writing this shortly before Thanksgiving, and I will appreciate it more than usual. In recent years I, like many of you, have experienced many losses (people, pets, previously taken for granted good health).
But in many ways my life is now fuller. I have met amazing people and physicians whom I never would have met. As 2012 comes to an end, and the new year starts, move forward in your own way and make a difference. Your life will be fuller for it. That is a promise.
Just plant a seed.
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to let people know as soon as
possible, and maybe, by chance,
someone contacts us saying that
they know somebody who needs
help.”
This goes to show no volunteering
act is too small. We all have it
in us to spread awareness and can
start by looking for opportunities
in our own communities.
ADA Presentation
On October 9, 2014, Dr. Vidya
Sankar presented at the American
Dental Association’s (ADA)
Annual Meeting in San Antonio,
Texas. Her presentation, “The Dental
Detective: Investigating Autoimmunity,”
addressed the common
symptoms and referral patterns
for several different autoimmune
diseases, including PV and MMP.
This served as an excellent way to
provide continuing education on
these illnesses to the entire dental
community.
“The oral health care professional
will commonly encounter
patients with an array of oral and
systemic health needs,” Dr. Sankar
said. “The ADA offered us a
platform to review some of these
lesser known conditions in order
to identify patients with potentially
undiagnosed needs and act as
a conduit to aid in diagnosis and
management. Additionally, linking
up the dental professionals
with professional societies such
as the IPPF will help to increase
awareness and access to care and
potentially increase our patients’
quality of life.”
The IPPF thanks Carlos, Dell,
and Dr. Sankar for their collaboration
on this project and their devotion
to raising awareness.
Together we can raise awareness
and promote early diagnosis
of PV and MMP. We all have ways
we can contribute. These are just
three of the many more tales of
awareness there are to share.
What’s yours? The IPPF encourages
our Community to get
involved with the Awareness
Campaign. If you are interested in
learning more about the campaign
or getting involved, please contact
Awareness@pemphigus.org.
Kate E. Frantz, MPH, CTTS, is
the Awareness Program Manager
at the IPPF living in Dixon,
CA. She is a contributor to
the Quarterly newsletter in her
“Awareness and You” column.
Kate can be reached at awareness@
pemphigus.org.
. . .continued from AWARENESS, page 6
Vidya Sankar, DMD, MHS, University of
Texas Health Science Center, San Antonio
generous. It has been overwhelming and we have
done really well so far. The IPPF has helped us, too, by
providing promotional material that we handed out.
We also had T-shirts and a banner made, which have
definitely helped raise awareness of PV. Please check
out the IPPF Facebook page to see photographs of
the event.
Thanks entirely to the funds we raised, I received
my first rituximab infusion two weeks ago, and it went
well. I am due for my second infusion next week, and
while I know I have a long wait, I am feeling very positive
about the outcome.
I just hope and pray it will work for me. I have been
advised that it normally takes at least three months
to determine whether or not rituximab is effective,
and I understand it is likely I will need further infusions.
Over the next few weeks, I will hopefully be
reducing the steroids and immunosuppressents.
As you can imagine, trying to cope with this disease
over a number of years has taken its toll. I have gone
from being a very fit and healthy man to being overweight
with a painful, incurable disease. This whole
period has been a depressing and difficult time for
me and my whole family. The most difficult aspect of
living with this disease is the excruciating pain I have
had to endure, and therefore I am looking forward to
returning to a life free of pain where I can enjoy keeping
fit and healthy and return to my weekly cycling
event with my brothers.
. . .continued from ROAD TO REMISSION, page 13
Scott is an Operations/Call Center Manager
who lives and works in West Midlands, United
Kingdom. Scott is happy to raise P/P awareness
by telling his story in the Quarterly. Once he is
feeling better, he hopes to continue to raise
funds for other PV sufferers. If you would like
to support Scott’s treatment fund, please visit
http://www.youcaring.com/medical-fundrais
a time . . . what else could I do?
In December one of the doctors
noted that along with the
predominantly oral presentation,
I had some skin symptoms, but it
wasn’t until July 2011 as a result of
my skin condition worsening with
blisters on my scalp and face that
I was referred to a dermatologist.
In September 2011, as my dermatologist
had suspected, my
PV diagnosis was confirmed and
refined as pemphigus with predominant
mucous membrane
involvement. Unfortunately, my
dermatologist moved to another
country and I was referred to a
new dermatologist. I was now being
seen by both an oral consultant
and a dermatologist. I continued
a variety of different treatments
(Dermovate, Protopic ointment,
Elidel, Betnesol nasal spray, and
prednisolone mouth wash) and
drugs (prednisolone, azathioprine,
mycophenolate). None of them
were particularly effective.
During this time, the erosions
had spread all over my face and
nose and were particularly severe
on the top of my head. I was then,
and I still am, obese due to the
large amount of steroids. The disease
is extremely painful; so much
so it affected my sleep because I
cannot rest my head on the pillow
at night. I remember visiting
the hospital one day, when my lesions
were particularly bad and the
nurse thought I had been in a car
crash!
Every time I washed my hair
or face I would lose a piece of
skin. Every time I ate anything my
mouth would start to bleed. I can
honestly say at this point I was a
broken man with little quality of
life. I would hide the pain and discomfort
from my family. Many
times I said I was okay, but inside I
felt so depressed and despondent:
there did not seem to be any light
at the end of the tunnel. It felt like
no one could help me and there
was nowhere to go.
In an attempt to seek help and
get yet another opinion, my dermatologist
arranged for me to
see a world expert in dermatology
at Guys Hospital who determined
that my high daily steroid
requirement could not continue.
He recommended a drug called
rituximab and initiated a request
for funding via National Health
Service (NHS) of England. Unfortunately,
the funding request was
declined. I was devastated.
My wife wrote to our local member
of Parliament hoping his support
would sway NHS to reconsider
their decision, but it didn’t. My
wife and family decided that we
had no alternative but to try and
raise the money ourselves, and as
a family we began our mission.
In preparation to receive rituximab,
I was advised to start weaning
off the drugs I had taken for so
long. This had disastrous consequences
and led to a massive flare
up — the worst I have ever had. I
had to be taken into hospital as an
emergency case, where for over
four days I received three pulsed
doses of IV methylprednisoloine
and IVIG infusions.
This made an enormous improvement
to my pemphigus and
for the first time in years I felt so
happy and such a sense of relief as
the pain had largely subsided.
I was aware IVIG is meant to be
an interim treatment and a temporary
solution. Generally patients
remain lesion-free for up to 30
days. In my case only two weeks
passed. I continued to receive IVIG
every two to four weeks, and it
seemed to stop disease progression.
Because I had to be hospitalized,
my consultant submitted
additional clinical information to
the NHS explaining my new circumstances.
Sadly, this renewed
request for funding of rituximab
was declined again.
One of my family’s first fundraising
events was a 100-mile
bicycle ride. I am so proud of my
son, brothers, and friends who
took part. Everyone who donated
tween the winter holidays and early January is the
most busy time of the year for mental health professionals,
in part because it is difficult during these
times of reflection not to become depressed, if that
is a struggle.
It is important for those who are affected in this way
to make a special effort to practice positive thinking
(mindfulness) and reflect not only on the unpleasant,
but also pleasant memories. I’ve suggested imagining
that you’re putting negatives into a box and taping it
up; then seeing yourself locking that box into a trunk;
then imagining wrapping chains around the trunk
and leaving it for a later date. You will know where the
trunk is and how to retrieve what is inside, but you
can also choose to keep all or most of the contents
locked up for a while. At this point you may be willing
to accept offers of help from others, which is among
the healthiest of escapes.
The holidays are also a good time to reach out to
others. We’ve all heard that it is truly more satisfying
to give than to receive. Inviting lonely people to an
event or volunteering at a nursing home or other facility,
for instance, will have a positive effect on your
mood. As a way to track your progress through the
difficult holiday period, keep a record of your mood
and activities or lack of activities. As regularly as you
can, rate your mood on a 1-to-10 scale with 1 being
the worst and 10 being the best. Also track your activities
or lack of them as a record of the patterns and
both positive and negative triggers. Everyone is different,
and how you are in this world and during this
time of year will not be the same as for others. Hold
onto necessary traditions, but allow yourself to have
new experiences. You may surprise yourself.
Besides these mindfulness approaches and techniques
related to reaching out to others, there are
a number of physical solutions that may help with
dealing with SADS. Vitamin D supplementation has
been found to be useful. As well, daylight spectrum
lamps (or just special bulbs put into existing lamps)
can also help counteract the seasonal lack of natural
sunlight. Still, many people will need different medications
or increased doses of their current medications.
Checking with your primary care physician or
having an evaluation by a psychiatrist or psychologist
can be helpful and may greatly increase the quality of
your holiday season.
After eight months of oral symptoms and appointments
with doctors and dental specialists, I received
my own PV diagnosis right before Thanksgiving. One
of the first posts I read on the P/P online discussion
group forum was from someone (Hi, Skip!) who recounted
feeling lucky to swallow mashed potatoes in
his first symptom-filled year. That really helped me
to put things in perspective.
I focused on what I could do, not what I could not
do. Unless you’re having a flare or going through a
particularly rough time physically, get out and do
things. Happiness is a choice, and all humans have
the ability to be happy.
Happy holidays and my best wishes for a terrific
new year.
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- of COL17 to test the effect of BP (human) patient-derived
antibodies. Generally, mice are a great experimental
model for studying the human immune system
since the mouse and human systems have been
found to be mechanistically very similar.
The authors genetically removed C3 from the humanized
COL17 mice and showed that indeed, they
lack the complement system. The authors also isolated
four different autoantibodies from four different
BP patients and found they vary in the degree
to which they activate the complement system. All
of the BP antibodies could induce skin detachment
(characteristic of blisters) when injected into either
the normal mice or in the complement-deficient
mice, demonstrating the complement system is likely
not at play in BP blister formation.
They next developed new antibodies that recognize
the exact same portion of COL17 and found a
correlation between the level of COL17 recognized
by the autoantibodies and blister formation. Recent
studies have shown COL17 antibodies not only recognize
and bind COL17 but also deplete it from cultured
cells. Ujiie and colleagues repeated that result show it
is complement-independent. As well, they find the
same effect of COL17 depletion in the COL17-humanized
mice - the antibodies caused blisters and
simultaneously reduced the amount of COL17.
Finally, the authors found that this was due to an
induction of the ubiquitin-proteasome system, the
machinery of cells that acts as a garbage disposal for
unwanted proteins. In this case, the COL17 autoantibodies
somehow mark the otherwise normal COL17
for destruction, possibly setting the stage for BP
symptoms and disease.
Several mechanisms may still be at play to mediate
the effects of COL17 autoantibodies generated
by BP patients (see figure). These include a degradation
system, as suggested from the current work or
COL17 may be internalized into cells upon binding of
the autoantibodies, as has been seen in studies from
other labs. It is also possible that COL17 gets internalized
first and then the intracellular proteasome
system degrades it. In any case, COL17 targeting by
BP autoantibodies is a probably occurring by a more
direct mechanism than if it involved the complement
system.
It is possible that BP shares a mechanistic basis with
other autoimmune mucocutaneous diseases such as
pemphigus vulgaris, where autoantibodies recognize
desmoglein proteins Dsg1 and Dsg3 in keratinocytes
of the epidermis. Therefore, understanding the underlying
mechanisms at play in blister formation in
the various P/P diseases will be applicable to all patients
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- The Power of Giving Back
- The IPPF is looking for volunteers to help us with many of our education and support projects and programs, including in several newly-created roles. The three main types of positions we are looking to fill are Executive Board (CEO’s senior staff), Special Projects Contributors, and General Volunteers.
The Executive Board heads up a “department” or initiative under the CEO. This could include efforts like managing our patient support programs, planning and running our annual Patient Conference, running fundraising events, volunteer management, and research and development. Other areas include accounting, collaborations, and project management.
- The commitment would be for one year or the duration of the initiative, whichever is shorter. And we are open to ideas for new “departments” and initiatives, and will even tailor a position to an individual’s talents and interests. This role is ideal for people that are operationally-oriented and want a significant role within the foundation.
Special Projects Contributors (SPC) have special expertise, resources, and access to give the IPPF a short-time commitment revolving around a specific area. This allows individuals to make a substantive contribution that fits their interests and lifestyle. Example projects include:
- competitive landscape analysis
review of clinical guidelines
website and/or graphic design
exploration of a specific clinical care question
legal analysis of a specific issue
accounting and investments
technical writing and reporting
building a data management platform for our patient registry
development strategy
helping set up and negotiate an external collaboration
developing a market access/reimbursement advocacy strategy
The SPC role is great for people that have a special expertise they would like to use for a short-term project. Volunteers are encouraged to propose their own projects as well.
And equally important are the General Volunteers. General volunteers contribute across a wide range of initiatives and events. Example include newsletter and website contributions, print and graphic design, Patient Conference preparation and on-site help, event planning, peer support, and community fundraisers (e.g., 5K run, bake sales, wine and cheese tasting, golf outings, etc.). This role provides an opportunity for anyone to contribute with as much or as little time as they can.
We encourage those interested to tell us about their talents and interests, and we are happy to find a way for them to contribute.
These are great opportunities for anyone that feels strongly about our community, wants to give back to the Foundation with their time and expertise, and wants to help grow our organization to deliver greater impact.
All volunteers receive acknowledgment in our newsletters and website, and they can list the experience or project in CVs, resumes, and related documents.
If you or someone you know would like to get involved, please contact me at will@pemphigus.org or by calling the office at 855-4PEMPHIGUS (855.473.6744) extension 103.
Helping the IPPF through volunteering is a great way to help us help others. That’s the Power of Giving Back!