DEBRA CURRENTS - Spring 2010

2010 Patient CareConference

Spring 2010

DebRA Partners With Make-A-WishIn Her Own Words: Nine Year Old Sophie Schulz’s Wish Story

WHAT IS EB?Epidermolysis Bullosa (EB) is agenetic disorder characterizedby chronic, painful blisteringoutside and inside the body.

INSIDE:Message from theExecutive Director ..............3

2010 Patient CareConference Agenda............4

Ask the EB Nurse................5

New DebRA BoardMember .....................................6

EB in the News.....................6

Local Events Schedule.......7

How You Can Help.............8

C U R R E N T S

C U R R E N T SSPRING 2010

Debra is now on Facebook!Visit our page to become a fan:facebook.com/DebraofAmerica.

at the Hyatt Regency Cincinnati

June 16- June 19, 2010Cincinnati, Ohio

Platinum Sponsor

Silver Sponsor

Silver Sponsor

Learn more about the event agenda, registration, and other

activities on page 4

Summer of 2009 rocked! My brother Sam and Iwere granted a "Make a Wish." It was so exciting!With our wish granter’s help my brother and I bothchose travel wishes. I chose to fly to Los Angelesand have a shopping spree at the American GirlDoll store. I've never been able to go into a storeand pick out anything and everything I wanted. Itwas awesome.

We stayed in a beautiful hotel for about a week.My whole family (my Dad, Mom, two brothers andsister) was able to go. We visited the beach and sawthe Hollywood sign. To top it off, I met lots of coolcelebrities: Selena Gomez, Dylan and CodySprouse, (Zac and Cody) and many more. I trulygot the rockstar experience. But wait that's not all! Continued on page 3 L to R: Sophie Schulz, Selena Gomez and Sadie Schulz

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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.

Visit us online to request product samples today.

Virtually Painless Removal

hollisterwoundcare.com1.888.322.4348

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.


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We are often asked how research is funded atDebRA. DebRA ofAmerica contributes toan International EBResearch Consortiumthat represents EBorganizations all over theworld. EB researchers

and clinicians apply to the Consortium to fundtherapies that can increase quality of life for EBpatients and promising work toward a cure for EB.The applications are reviewed by an internationalMedical Scientific Advisory Board (MSAP). Twomembers of DebRA’s Scientific Advisory Board siton this prestigious panel. There are usually tworounds of applications annually with fifteen toeighteen applications each round. In the last round,five were awarded funding. The amount of theaward depends on the quality of the proposal andthe available funds for each round. The Consortiumis rigorous in selection, reporting, and tracking grantoutcomes. We believe this structure offers the bestuse of funds. It is objective, accountable, anddedicated to finding a cure for EpidermolysisBullosa.

I’ve been thinking a lot about how challenging it isto raise awareness about rare/orphan diseases,such as EB. An orphan or rare disease is generallyconsidered to affect fewer than 200,000 people inthe United States. There are more than 7,000 raredisorders that impact the lives of 25,000,000Americans and their families. Epidermolysis Bullosais one, and its impact is physical, emotional, andfinancial. Over its 30 year history, DebRA hasoffered support to the 30,000 patients, theirfamilies and caregivers with knowledge, comfortand care. We are also an important resource forthe medical community who may never have seenan Epidermolysis Bullosa case.

This June, DebRA will host the 2010 Patient CareConference. We have a sparkling agenda–the topresearchers, the best in care and treatment, and funevents. Hope to see you in Cincinnati from June16th through June 19th!

Warmest Regards,

Mary SpragueExecutive Director of DebRA

Sophie’s Wish Story Continued from page 1.

Message from the Executive Director

The very next month we all flew to New York. Itwas 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 andthe whole Yankees team. Sam got to go into thedugout and meet the players during battingpractice.

He got lots of players’ autographs and his picturetaken. He was so excited to meet Derek Jeter. Theboys were in heaven. We also went to the CentralPark Zoo and saw Mary Poppins on Broadway. Itwas amazing. I said to my Dad as we were cominghome," We went to LA and NY in the samesummer, and we are all eight and under."

For a brief moment in time the answer was alwaysyes. All our dreams came true, except for a miraclethat is. My summer was very uplifting, filling myheart with hope, joy and strength. My family and Iwere truly blessed. We made memories that willlast a lifetime.

Schulz Family Left to Right: Sadie, Simon, Sam (Front), Mom, Lori, Dad, Patrick and Sophie.

Spring 2010 3

DebRA ofAmericaBoard ofTrustees

PresidentFaith Daniels

Vice-PresidentWarren T. Buhle, Esq.

TreasurerRichard Gallagher, Jr.

SecretaryCharlee Miller

Chairman SABAlan 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


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TopicsThe agenda has a range of interesting topics overthe three day conference. Some topics include:

RegistrationAll registration materials are on the DebRAwebsite: www.debra.org. When you registerthrough our site you are guaranteed our specialroom rate of $99 per night, plus tax. Special needsaccommodations should be arranged with thehotel when you call to book your rooms.

In addition to reserving your room, please registeronline at Debra.org. Indicate your name, number ofchildren attending, special meal requests, and if youwill participate in any planned activities.

Activities and Other InformationOn Thursday evening, Hollister Wound Care isplanning an Aquarium adventure for children andtheir families. They will provide a light dinner andtransportation to and from the Aquarium.

This year we are also adding short private medicalconsultations. Families can sign up to discuss topicsthat are of special interest to them.

National Rehab will be taping the PCC for thosewho can’t join us in June.

2010 Patient CareConference This year DebRA’s Patient Care Conference will beco-hosted by the excellent team at the CincinnatiChildren’s Hospital Medical Center. The mostcurrent information on EB research will bepresented by four of the top researchers in theUSA. They each have different approaches tosolving the same puzzle…how to cure EB.

Did YouKnow?The Kakkis EverylifeFoundation(curetheprocess.org) is anew colleague.

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.

Wednesday, June 16 through Saturday, June 19at the Hyatt Regency Cincinnati (151 West Fifth Street Cincinnati, OH 45202)

We Look Forward to Seeing You There!

Save the Date!The 12th Annual Mats Wilander CelebrityTennis & Golf Classic

Monday October 18, 2010Westchester Country ClubRye, New York

Sponsorships Available

To reserve your foursomeand/or your place on the court:

Call: 212.868.1573Email: [email protected]

4 Spring 2010

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 FundingInsurance, Medicare, SSI, Make-A-Wish

For Adults Only - Emotional Issues, Marriage,Genetic Counseling and Pregnancy


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Dear Nutritionally Challenged,Eating and taking in enough calories to promotegrowth and development and wound healing canbe a difficult task in EB. Many families depend onsoft diet options and liquid nutrition (i.e., PediaSure,Boost) because of structural issues with chewingand swallowing. Working with a registered dietitian,you can maximize calories with some of thefollowing ideas that were put together by theNutrition team at Cincinnati Children’s HospitalMedical Center.

I hope some of these suggestions work for you.

Geraldine Kelly-Mancuso, RN DebRA’s EB Nurse Educator866-322-7276, [email protected]

Dear DebRA Nurse,My 10 year old son is having a hard time taking inenough nutrition, and we are looking for ideas toincrease his daily calorie intake. Any suggestions?

-Nutritionally challenged

Ask the EB Nurse

Spring 2010 5 10

Butter or Margarine (45 Calories/Tsp.)Add to eggs, casseroles, vegetables, rice, cookedcereals, and pastas.

Mayonnaise (45 Calories/Tsp.)Use liberally, add extra to salads.

Salad Dressing/Dips (45 Calories/Tsp.)Use liberally on salads, as a dip for raw vegetablesor 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.)Use on pudding, jell-o, pies and other desserts.Use as a dip with fresh fruit.

Wheat Germ (25 Calories/Tsp.)Add a tablespoon or two to cereal. Mix into cookie batter, casseroles, etc.

Meet Debra’s EBNurseGeri 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.


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DebRA welcomes Ronald "Randy" J.Schwartz to the Debra of AmericaBoard of Directors. Randy is theVice President of Marketing atMolnlycke Health Care, US, LLC.Prior to joining Molnlycke in 2007,Randy served as Director ofMarketing/ Commercial Develop-ment at COLOPLAST CorporationSkin Health Division and has heldseveral key positions with tophealthcare firms.

His extensive charitable workincludes: Becca's Closet, thePhoenix Society and Burn SummerCamps and he is a long standingmember of the Association for theAdvancement of Wound Care(AAWC).

Randy's experience is invaluable to DebRA as we continue tostrengthen our capacity andcommitment to finding a cure forEB. In his words, "I am delighted andexcited to join the board of DebRA.I have seen the devastating effectsof EB and understand the pain andfrustration patients and familymembers feel when a child isstricken with EB. With over 25 yearsof wound care marketing and salesexperience, I look forward tohelping the organization in any waypossible to ensure that we meet themission and values of DebRA."

New Board Member

Rays of Hope in Battling an Agonizing DiseaseBy Sabin Russell

It wasn’t until Ileana Peralta was in junior highschool that she summoned the courage to Googleher own disease.

The teenager from Livermore knew almosteverything about her inherited condition,Epidermolysis Bullosa, a tongue twister evendoctors call just EB. The disease is caused by thelack of the collagen gene that makes the glue thattethers layers of skin together. It makes her skinseem as fragile as a butterfly’s wing.

Ileana and other children with EB go through lifewith much of their bodies wrapped in gauze, like aburn patient. Their skin, at the slightest trauma, isprone to form blisters, which are vulnerable toinfection. Scarring eventually curls fingers and toes,fusing them. To varying degrees, these children arecondemned to a life of pain and disfigurement.

EB is rare. Ileana’s severe form of it, called recessivedystrophic, affects between one and four per onemillion in the United States, said Dr. Alfred T. Lane,a pediatric dermatologist. Dr. Lane was recruitedto Stanford University Medical School 19 yearsago to search for treatments for EB, and has beenworking on it ever since.

“It’s the worst skin disease that I know of,” he said.“This disease just cries out for help.”

The handful of doctors in the United States whodeal with EB are experimenting with some of the

most advanced technologies in medicine — genetherapy, stem cell therapy and bone marrowtransplantation — as a means of eventualtreatment.

Last fall, the California Institute of RegenerativeMedicine, the state agency that runs the voter-approved $3 billion stem cell research program,allotted $11.7 million to Stanford to find a way toharness the newest stem cell technologies againstEB. The goal is a clinical trial by 2014.

Under Dr. Lane’s direction, the California stem cellmoney will pay for an approach using newtechniques that transform ordinary skin cells intoall-purpose stem cells. In theory, these stem cellscan be engineered to carry the missing collagengene, and can then be coaxed to grow largeamounts of healthy skin grafts, immunologicallycompatible with each patient, like Ileana.

When Ileana trolled the Web for informationabout her disease, she discovered that childrenwho have it may not survive to adulthood. “Thepart that scares me is that some kids died from it,”she said. “I’m glad I’m still here.”

EB in the News: New York Times Article

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

Excerpt – Originally Published March 14, 2010

To read the full article visithttp://nytimes.com/2010/03/14/health/14sfblister.html

You may also view a link to the article on DebRA’sFacebook page: www.facebook/DebraofAmerica


Spring 2010 7

2010 Local Events Schedule

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4th Annual Grant Taylor Fishing RodeoWaterville Baptist ChurchTrion, GAFebruary, 2010

Emma Kinley Mack Chasing A Cure for EBTaylor Armstrong, GAFebruary 6, 2010

Ella Furjes Trivia NightGwen Furjes, Ballwin, MOFebruary 13, 2010

Jonah’s Online EB AuctionKatie McNalley, February 23 - 27, 2010

“Applause for A Cause”One in A Million - University of Miami, Miami FloridaMarch 22, 2010

Butterfly BenefitLeslie Rader, Louisville, KYMarch 27, 2010

Weston Zucha’s Walk-A-ThonDarlene Vantine, Crosby, TXApril 10, 2010

Butterfly Wishes for EllieShawn and Andrew TavaniAtlanta, GAApril 22, 2010

Eagles’s Bowl-A-ThonHelen Barnhart, Hagerstown,MDApril 24, 2010

EB Awareness WalkOne in A Million - University of Miami, Miami, FLApril 25, 2010

“Pub Fundraiser”Colleen SchindledeckerHarrisburg, PAMay 2, 2010

Winos and DinosLindsey Gregg, Eaton, OHMay 22, 2010

Lancaster Golf FundraiserDave Cornman, Lancaster, PAJune 9, 2010

“Birthday Fundraiser”Elizabeth Largay, Westbrook, ME

July 24, 2010

Tyler Fisher EB Awareness:Open House and SpaghettiDinnerTrisha Wirick, Lancaster, OHAugust 24, 2010

EB Walk/RunJennifer Searle, Syracuse, NYOctober 10, 2010

“Cookbook Fundraiser”Dawn Kuznik, Buffalo, NYFall 2010

If you would like to host a LocalEvent to benefit DebRA andhelp raise awareness forEpidermolysis Bullosa, pleasecontact the office at 212-868-1573 or [email protected].

DebRA thanks all of ourdedicated supporters for theirtime and generosity.


To make a donation today, send a check payable to DebRA of America or youcan make a gift online at www.debra.org.

We deeply appreciate your support!

Combined Federal Campaign #11990

Dystrophic Epidermolysis BullosaResearch Association of America

16 East 41st Street3rd FloorNew York, NY 10017

Phone: 212-868-1573Fax: 212-868-9296E-mail: [email protected]

The DebRA Team How You Can HelpDebRA of America not only supports researchtowards finding a cure for EB, we also providedirect services to families coping with the dailyhardships of the disease. Your donations to DebRAhelp to brighten the lives of these families and easesome of the emotional and financial hardships ofliving with EB. Your dollars support:

Family Crisis FundProvides emergency assistance to alleviate thedifficulties of unreimbursed medical costs.

Nurse EducatorResident EB expert who manages over 3,000inquiries annually, answers medical questions andmakes referrals.

New Family Advocate ProgramProvides a critical support system for familieslearning to care for a newborn with EB.

Wound Care Clearing House Provides free medical supplies to EB patients.Each year, over $100,000.00 worth of productsare sent directly to EB families from DebRA.

Until there is a cure...There is...

Mary SpragueExecutive Director

Alecia BakerBusiness Manager

Pennie CannonProject Manager

Ro-Ann RabanilloDevelopment Assistant

Maxine SilentDevelopment Manager

Maxine, Alecia, Pennie, Mary, Ro-Ann

(Left to Right)

Make a DifferencePurchase a LimitedEdition Butterfly PinOnly 30 left!

Go to www.debra.org

Did you know?

of every dollar donated to DebRA is used tosupport EB patients and their families, and fundresearch towards a cure.

89.4¢


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DEBRA CURRENTS - Spring 2010

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