DEBRA CURRENTS - Spring 2010

SPRING 2010
CURRENTS
2010 Patient Care
Conference
WHAT IS EB?
Epidermolysis Bullosa (EB) is a
genetic disorder characterized
by chronic, painful blistering
outside and inside the body.
June 16 -June 19, 2010
Cincinnati, Ohio
INSIDE:
at the Hyatt Regency Cincinnati
Message from the
Executive Director ..............3
Platinum Sponsor
Silver Sponsor
2010 Patient Care
Conference Agenda............4
Silver Sponsor
Ask the EB Nurse................5
New DebRA Board
Member.....................................6
Learn more about the event agenda, registration, and other
activities on page 4
DebRA Partners With Make-A-Wish
In Her Own Words: Nine Year Old Sophie Schulz’s Wish Story
EB in the News.....................6
Local Events Schedule.......7
How You Can Help.............8
Debra is now on Facebook!
Visit our page to become a fan:
facebook.com/DebraofAmerica.
Summer of 2009 rocked! My brother Sam and I
were granted a "Make a Wish." It was so exciting!
With our wish granter’s help my brother and I both
chose travel wishes. I chose to fly to Los Angeles
and have a shopping spree at the American Girl
Doll store. I've never been able to go into a store
and pick out anything and everything I wanted. It
was awesome.
We stayed in a beautiful hotel for about a week.
My whole family (my Dad, Mom, two brothers and
sister) was able to go. We visited the beach and saw
the Hollywood sign. To top it off, I met lots of cool
celebrities: Selena Gomez, Dylan and Cody
Sprouse, (Zac and Cody) and many more. I truly
got the rockstar experience. But wait that's not all!
Continued on page 3
Spring 2010
L to R: Sophie Schulz, Selena Gomez and Sadie Schulz
1
Virtually Painless Removal
Dressing changes at the Escobar home are a lot
more relaxed now that they’re using Hollister
Wound Care products with TRIACT® technology.
TRIACT dressings encourage wound healing and
are virtually painless when removed—exceptional
for your “Butterfly” child.
hollisterwoundcare.com
1.888.322.4348
Visit us online to request product samples today.
Proud supporter of:
www.debra.org
search “EB Resource”
Hollisterwoundcare and wave logo are trademarks of Hollister
Incorporated. ©2010 Hollister Wound Care LLC. Facebook is a
registered trademark of Facebook, Inc.
DebRA of
America
Board of
Trustees
President
Faith Daniels
Vice-President
Warren T. Buhle, Esq.
Treasurer
Richard Gallagher, Jr.
Secretary
Charlee Miller
Chairman SAB
Alan Shalita, M.D.
Kathleen Brown
Angela Christiano, Ph.D.
Gregg Cuvin
Tom Gillespie
Frank Kacmarsky
Brett Kopelan
Robert Meirowitz, M.D.
Laurence Miller, M.D.
Thomas Misisco
Paul Nunnari
Leslie Rader
Jeanne Roccon Rohm
Randy Schwartz
Daniel Siegel, M.D.
Alexander J. Silver
Jouni Uitto, M.D., Ph.D.
Timothy Wiebe
Sonya Wilander
Message from the Executive Director
We are often asked
how research is funded at
DebRA. DebRA
of
America contributes to
an International EB
Research Consortium
that
represents
EB
organizations all over the
world. EB researchers
and clinicians apply to the Consortium to fund
therapies that can increase quality of life for EB
patients and promising work toward a cure for EB.
The applications are reviewed by an international
Medical Scientific Advisory Board (MSAP). Two
members of DebRA’s Scientific Advisory Board sit
on this prestigious panel. There are usually two
rounds of applications annually with fifteen to
eighteen applications each round. In the last round,
five were awarded funding. The amount of the
award depends on the quality of the proposal and
the available funds for each round. The Consortium
is rigorous in selection, reporting, and tracking grant
outcomes. We believe this structure offers the best
use of funds. It is objective, accountable, and
dedicated to finding a cure for Epidermolysis
Bullosa.
Sophie’s Wish Story
I’ve been thinking a lot about how challenging it is
to raise awareness about rare/orphan diseases,
such as EB. An orphan or rare disease is generally
considered to affect fewer than 200,000 people in
the United States. There are more than 7,000 rare
disorders that impact the lives of 25,000,000
Americans and their families. Epidermolysis Bullosa
is one, and its impact is physical, emotional, and
financial. Over its 30 year history, DebRA has
offered support to the 30,000 patients, their
families and caregivers with knowledge, comfort
and care. We are also an important resource for
the medical community who may never have seen
an Epidermolysis Bullosa case.
This June, DebRA will host the 2010 Patient Care
Conference. We have a sparkling agenda–the top
researchers, the best in care and treatment, and fun
events. Hope to see you in Cincinnati from June
16th through June 19th!
Warmest Regards,
Mary Sprague
Executive Director of DebRA
Continued from page 1.
The very next month we all flew to New York. It
was so cool to see all the big buildings. My brother,
Sam, who is five years old, and a huge baseball fan,
chose to meet his favorite player Derek Jeter and
the whole Yankees team. Sam got to go into the
dugout and meet the players during batting
practice.
He got lots of players’ autographs and his picture
taken. He was so excited to meet Derek Jeter. The
boys were in heaven. We also went to the Central
Park Zoo and saw Mary Poppins on Broadway. It
was amazing. I said to my Dad as we were coming
home," We went to LA and NY in the same
summer, and we are all eight and under."
For a brief moment in time the answer was always
yes. All our dreams came true, except for a miracle
that is. My summer was very uplifting, filling my
heart with hope, joy and strength. My family and I
were truly blessed. We made memories that will
last a lifetime.
Spring 2010
Schulz Family Left to Right: Sadie, Simon,
Sam (Front), Mom, Lori, Dad, Patrick and Sophie.
3
2010 Patient Care
Conference
Save the Date!
The 12th Annual
Mats Wilander Celebrity
Tennis & Golf Classic
Monday October 18, 2010
Westchester Country Club
Rye, New York
Sponsorships Available
To reserve your foursome
and/or your place on
the court:
Call: 212.868.1573
Email: events@debra.org
This year DebRA’s Patient Care Conference will be
co-hosted by the excellent team at the Cincinnati
Children’s Hospital Medical Center. The most
current information on EB research will be
presented by four of the top researchers in the
USA. They each have different approaches to
solving the same puzzle…how to cure EB.
Wednesday, June 16 through Saturday, June 19
at the Hyatt Regency Cincinnati (151 West Fifth Street Cincinnati, OH 45202)
We Look Forward to Seeing You There!
Registration
Topics
All registration materials are on the DebRA
website: www.debra.org. When you register
through our site you are guaranteed our special
room rate of $99 per night, plus tax. Special needs
accommodations should be arranged with the
hotel when you call to book your rooms.
The agenda has a range of interesting topics over
the three day conference. Some topics include:
In addition to reserving your room, please register
online at Debra.org. Indicate your name, number of
children attending, special meal requests, and if you
will participate in any planned activities.
Activities and Other Information
Did You
Know?
The Kakkis Everylife
Foundation
(curetheprocess.org) is a
new colleague.
On Thursday evening, Hollister Wound Care is
planning an Aquarium adventure for children and
their families. They will provide a light dinner and
transportation to and from the Aquarium.
This year we are also adding short private medical
consultations. Families can sign up to discuss topics
that are of special interest to them.
National Rehab will be taping the PCC for those
who can’t join us in June.
Latest Updates on Research
Getting the Dressings You Need
Everyday Wound Care and Special Dressings
Early Childhood, Adolescence and School
Surgical Issues: Hand Release Surgery,
G – Tubes, Trach Tubes, Dilatations and GI Issues
Financial Issues, Orphan Disease Funding
Insurance, Medicare, SSI, Make-A-Wish
For Adults Only - Emotional Issues, Marriage,
Genetic Counseling and Pregnancy
The foundation is a non-profit
organization created by Dr. Emil
Kakkis, M.D., Ph.D. to improve
diagnosis and treatment of rare
diseases. The organization
believes that no disease is too
rare to be treated. DebRA is
fortunate enough to have a
representative from the Kakkis
Every Life Foundation joining us
at the Patient Care Conference.
4
Spring 2010
Ask the EB Nurse
Dear DebRA Nurse,
My 10 year old son is having a hard time taking in
enough nutrition, and we are looking for ideas to
increase his daily calorie intake. Any suggestions?
-Nutritionally challenged
Butter or Margarine (45 Calories/Tsp.)
Add to eggs, casseroles, vegetables, rice, cooked
cereals, and pastas.
Mayonnaise (45 Calories/Tsp.)
Use liberally, add extra to salads.
Dear Nutritionally Challenged,
Eating and taking in enough calories to promote
growth and development and wound healing can
be a difficult task in EB. Many families depend on
soft diet options and liquid nutrition (i.e., PediaSure,
Boost) because of structural issues with chewing
and swallowing. Working with a registered dietitian,
you can maximize calories with some of the
following ideas that were put together by the
Nutrition team at Cincinnati Children’s Hospital
Medical Center.
Salad Dressing/Dips (45 Calories/Tsp.)
Use liberally on salads, as a dip for raw vegetables
or sauce on cooked vegetables.
Sour Cream (26 Calories/Tsp.)
Add to potatoes and casseroles.
Gravies (40 Calories/Tsp.)
Use liberally on mashed potatoes and biscuits.
Whipped Cream (30 Calories/Tsp.)
I hope some of these suggestions work for you.
Use on pudding, jell-o, pies and other desserts.
Use as a dip with fresh fruit.
Geraldine Kelly-Mancuso, RN
Wheat Germ (25 Calories/Tsp.)
DebRA’s EB Nurse Educator
866-322-7276, debranursec@yahoo.com
Add a tablespoon or two to cereal.
Mix into cookie batter, casseroles, etc.
Spring 2010
Meet Debra’s EB
Nurse
Geri Kelly-Mancuso has
extensive experience as a
pediatric nurse. As
DebRA's Nurse Educator,
Geri works at Cincinnati
Children's Hospital Medical
Center, where she responds
to over 3,000 inquiries
annually for EB patients
and their families. Geri is
also fluent in Spanish and
helps DebRA meet the
needs of Spanish-speaking
families.
5
EB in the News: New York Times Article
Excerpt – Originally Published March 14, 2010
Rays of Hope in Battling an Agonizing Disease
By Sabin Russell
New Board Member
DebRA welcomes Ronald "Randy" J.
Schwartz to the Debra of America
Board of Directors. Randy is the
Vice President of Marketing at
Molnlycke Health Care, US, LLC.
Prior to joining Molnlycke in 2007,
Randy served as Director of
Marketing/ Commercial Development at COLOPLAST Corporation
Skin Health Division and has held
several key positions with top
healthcare firms.
His extensive charitable work
includes: Becca's Closet, the
Phoenix Society and Burn Summer
Camps and he is a long standing
member of the Association for the
Advancement of Wound Care
(AAWC).
Randy's experience is invaluable
to DebRA as we continue to
strengthen our capacity and
commitment to finding a cure for
EB. In his words, "I am delighted and
excited to join the board of DebRA.
I have seen the devastating effects
of EB and understand the pain and
frustration patients and family
members feel when a child is
stricken with EB. With over 25 years
of wound care marketing and sales
experience, I look forward to
helping the organization in any way
possible to ensure that we meet the
mission and values of DebRA."
It wasn’t until Ileana Peralta was in junior high
school that she summoned the courage to Google
her own disease.
The teenager from Livermore knew almost
everything about her inherited condition,
Epidermolysis Bullosa, a tongue twister even
doctors call just EB. The disease is caused by the
lack of the collagen gene that makes the glue that
tethers layers of skin together. It makes her skin
seem as fragile as a butterfly’s wing.
Ileana and other children with EB go through life
with much of their bodies wrapped in gauze, like a
burn patient. Their skin, at the slightest trauma, is
prone to form blisters, which are vulnerable to
infection. Scarring eventually curls fingers and toes,
fusing them. To varying degrees, these children are
condemned to a life of pain and disfigurement.
EB is rare. Ileana’s severe form of it, called recessive
dystrophic, affects between one and four per one
million in the United States, said Dr. Alfred T. Lane,
a pediatric dermatologist. Dr. Lane was recruited
to Stanford University Medical School 19 years
ago to search for treatments for EB, and has been
working on it ever since.
most advanced technologies in medicine — gene
therapy, stem cell therapy and bone marrow
transplantation — as a means of eventual
treatment.
Last fall, the California Institute of Regenerative
Medicine, the state agency that runs the voterapproved $3 billion stem cell research program,
allotted $11.7 million to Stanford to find a way to
harness the newest stem cell technologies against
EB. The goal is a clinical trial by 2014.
Under Dr. Lane’s direction, the California stem cell
money will pay for an approach using new
techniques that transform ordinary skin cells into
all-purpose stem cells. In theory, these stem cells
can be engineered to carry the missing collagen
gene, and can then be coaxed to grow large
amounts of healthy skin grafts, immunologically
compatible with each patient, like Ileana.
When Ileana trolled the Web for information
about her disease, she discovered that children
who have it may not survive to adulthood. “The
part that scares me is that some kids died from it,”
she said. “I’m glad I’m still here.”
“It’s the worst skin disease that I know of,” he said.
“This disease just cries out for help.”
To read the full article visit
The handful of doctors in the United States who
deal with EB are experimenting with some of the
You may also view a link to the article on DebRA’s
Facebook page: www.facebook/DebraofAmerica
http://nytimes.com/2010/03/14/health/14sfblister.html
Did You Know?
DebRA partners with NORD–National
Organization of Rare Diseases www.rarediseases.org.
NORD is committed to the identification, treatment,
and cure of rare disorders through programs of
education, advocacy, research, and service. Mary Sprague
is a member of the NORD Board of Directors.
6
Spring 2010
2010 Local Events Schedule
4th Annual Grant Taylor
Fishing Rodeo
Waterville Baptist Church
Trion, GA
February, 2010
Emma Kinley Mack Chasing
A Cure for EB
Taylor Armstrong, GA
February 6, 2010
Ella Furjes Trivia Night
Gwen Furjes, Ballwin, MO
February 13, 2010
Butterfly Benefit
“Pub Fundraiser”
EB Walk/Run
Leslie Rader, Louisville, KY
March 27, 2010
Colleen Schindledecker
Harrisburg, PA
May 2, 2010
Jennifer Searle, Syracuse, NY
October 10, 2010
“Cookbook Fundraiser”
Weston Zucha’s
Walk-A-Thon
Winos and Dinos
Darlene Vantine, Crosby, TX
April 10, 2010
Lindsey Gregg, Eaton, OH
May 22, 2010
Butterfly Wishes for Ellie
Lancaster Golf Fundraiser
Shawn and Andrew Tavani
Atlanta, GA
April 22, 2010
Dave Cornman, Lancaster, PA
June 9, 2010
“Birthday Fundraiser”
Eagles’s Bowl-A-Thon
Elizabeth Largay, Westbrook, ME
July 24, 2010
Katie McNalley,
February 23 - 27, 2010
Helen Barnhart, Hagerstown,
MD
April 24, 2010
“Applause for A Cause”
EB Awareness Walk
One in A Million - University
of Miami, Miami Florida
March 22, 2010
One in A Million - University
of Miami, Miami, FL
April 25, 2010
Jonah’s Online EB Auction
Spring 2010
Tyler Fisher EB Awareness:
Open House and Spaghetti
Dinner
Dawn Kuznik, Buffalo, NY
Fall 2010
If you would like to host a Local
Event to benefit DebRA and
help raise awareness for
Epidermolysis Bullosa, please
contact the office at 212-8681573 or email
events@debra.org.
DebRA thanks all of our
dedicated supporters for their
time and generosity.
Trisha Wirick, Lancaster, OH
August 24, 2010
7
Until there is a cure...
There is...
Make a Difference
Purchase a Limited
Edition Butterfly Pin
Only 30 left!
Dystrophic Epidermolysis Bullosa
Research Association of America
Go to www.debra.org
16 East 41st Street
3rd Floor
New York, NY 10017
Phone: 212-868-1573
Fax: 212-868-9296
E-mail: staff@debra.org
The DebRA Team
How You Can Help
Mary Sprague
DebRA of America not only supports research
towards finding a cure for EB, we also provide
direct services to families coping with the daily
hardships of the disease. Your donations to DebRA
help to brighten the lives of these families and ease
some of the emotional and financial hardships of
living with EB. Your dollars support:
Executive Director
Alecia Baker
Business Manager
Pennie Cannon
Project Manager
Development Assistant
Provides emergency assistance to alleviate the
difficulties of unreimbursed medical costs.
To make a donation today,
send a check payable to DebRA of America or you
can make a gift online at www.debra.org.
Maxine Silent
Nurse Educator
We deeply appreciate your support!
Development Manager
Resident EB expert who manages over 3,000
inquiries annually, answers medical questions and
makes referrals.
Combined Federal
Campaign #11990
Family Crisis Fund
Ro-Ann Rabanillo
New Family Advocate Program
Provides a critical support system for families
learning to care for a newborn with EB.
Maxine, Alecia, Pennie, Mary, Ro-Ann
(Left to Right)
Did you know?
Wound Care Clearing House
89.4¢
Provides free medical supplies to EB patients.
Each year, over $100,000.00 worth of products
are sent directly to EB families from DebRA.
of every dollar donated to DebRA is used to
support EB patients and their families, and fund
research towards a cure.