2013 DEBRA International Congress

 

This was the official website for the 2013 DEBRA International Congress.
DEBRA International congress, held 20-22 September, 2013 in Rome. The venue for the conference was the brand new Auditorium San Paolo of the Bambino Gesù Children's Hospital.
The DEBRA International Congress is an annual event which first took place in Strasbourg, France in 1992.
Content is from the site's 2013 archived pages.

To learn more about the DEBRA International Congress go to their current website at: www.debra-international.org/homepage.html

DEBRA International is a worldwide network of national groups working on behalf of those affected by the genetic skin blistering condition epidermolysis bullosa (EB). 
On our site, you will find information about the condition, appropriate medical care, research into EB and living with EB as well as links to the national DEBRA groups around the world. 
Our vision is to ensure that people living with EB have access to the best quality support and medical care, while also driving the development of effective treatments and cures.

 

Epidermolysis bullosa

What is EB?

Epidermolysis Bullosa (EB) is a group of rare genetic skin conditions, which is characterised by extremely fragile skin and recurrent blister formation, resulting from minor mechanical friction or trauma.

The skin has two layers; the outer layer is called the epidermis and the inner layer the dermis. Normally, there are 'anchors' between the two layers that prevent them from moving independently from one another. In people with EB, the two skin layers lack the anchors that hold them together, and any action that creates friction between the layers (like rubbing or pressure) will create blisters and painful sores. Sufferers of EB have compared the sores to third-degree burns.

Over the past 15-20 years, 13 major genes responsible for the majority of cases of EB have been identified. The genetic errors in EB result in defects in the proteins that adhere the epidermis to the dermis.

In many countries, Butterfly Children is a term often used to describe younger patients because their skin is as fragile as a butterfly’s wings. Sometimes, children with the condition are also described as Cotton Wool Babies. And in South America, Crystal Skin Children is the term used.

Clinical symptoms

EB skin is never able to ever heal properly with normal strength: chronic open wounds and extensive scarring develop with attendant pain. Each time EB skin is damaged, the damage is irreversible, and disfigurement and disability accrue over a lifetime. Some severe forms of EB are fatal in infancy; others in older children and young adults.

The chronic wounds of EB can result in decreased mobility owing to pain and the extensive scar tissue that forms. Scarring in turn results in constriction of the mouth or throat, or 'mitten' deformities of the hands and feet: benefits of surgery to release fingers, for example, are of limited duration as scar tissue starts to form again immediately.

For some types of EB, the internal mucosa is also affected: nutrition can be compromised, resulting in osteoporosis, and general failure to thrive: quite young children can depend on gastrostomies or require highly specialised diets.

A type of skin cancer, squamous cell carcinoma (SCC), is a major cause of death for recessive dystrophic (RDEB) teenagers and young adults: surgery, radio- or chemotherapy are not effective. It is particularly aggressive and invasive in EB patients and the need is to prevent development or slow the spread.

 

  • Help people with EB

    Your support of DEBRA International or a national EB group enables us to provide the best quality of life for families and individuals affected by EB.

     

 



An aside: Our neighbor's daughter has a relatively mild form of EB. When they moved next door and we discovered our daughters were the same age, I read as much information as possible about EB to understand what I should be aware of and help educate my kids. Likewise, my husband who is part of a team that provides support for Salesforce implementation to various businesses and organizations asked his co workers about EB. Turned out that RTS Labs, the progressive software development firm where my husband works has had several clients that were medical practices with patients that were benefiting from the research being done at Columbia University, Stanford University, andd the University of Colorado Anschutz on cell reprogramming of autologous cells as treatment strategies for RDEB using Immune System Programming (ISPTM). I'm always amazed at what a small interconnected world we live in.

My daughter Elsa and Anna became good friends. Right now they are at the age when playing princess is important. The girls looked with me at online stores that sold girls princess costumes and all the accessories that are available to complete the princess look. Because of Anna's sensitivity to certain fabrics, her mother and I decided we would make the girl's princess dresses using as reference the examples found on a website called Everything Princesses. They offered such delightful costumes. We bought wands and other accessories from the site to augment the princess dresses we had made from derma silk and cotton. It's such a delight watching the girls play outside, twirling, giggling, and dancing among the flower garden in their princess dresses. I highly recommend checking out www.debra-international.org/homepage.html. It's a most informative site as is the EB Research Partnership site found at: www.ebresearch.org/institution.html.

 



 

Countdown to Congress

WELCOME IN ROME!

DEBRA International Congress 2013
Living with EB: the voice of patients and families

Co-chairs
Claudio Notarantonio, Rainer Riedl

How to improve the quality of life in those who are suffering from Epidermolysis Bullosa? How to help and support them and their families? What are the best treatments (medical and surgical, nutritional, physiotherapical, etc.)?

In the International Congress organized by DEBRA International, patients of all ages (children, adolescents and adults), families, researchers, and health professionals (physicians, surgeons, nutritionists, etc.) respond to these questions by providing complete and updated answers. During the five thematic sessions, that cover all ages (from neonates to adults), both the testimonies of patients and parents as well as the expertise of researchers and medical professionals provide a comprehensive approach and stimulate constructive dialogue. In such a way, for every patient with Epidermolisi Bullosa, DEBRA continues its mission of providing the most updated information, promoting the access to the best health care, and stimulating innovative research.

 

Family Reduction

  • 1st registrant: € 150,00

Registration fee family riduction:

  • 2nd parent, children, brother or sister: € 100,00
  • 3rd+ parents, children, brothers and sisters: € 50,00
  • accompanying persons (grandparents): € 50,00
  • children under 2 years: free
  • children from 2 to 12 years: € 50,00

example:

  1. father: € 150
  2. mother: € 100
  3. 5 years old children: € 50
  4. 1 year old children: free

    TOTAL € 300 (instead of € 600)

Schedule

FRIDAY, SEPTEMBER 20

  • Registration
  • OPENING CERIMONY - Welcoming remarks
  • Session I: EB TODAY
  • Session II: THE NEWBORN AND HIS FAMILY


SATURDAY, SEPTEMBER 21

  • Session III: THE CHILD AND HIS FAMILY
  • Session IV: THE TEENAGER AND THE ADULT
  • ROUND TABLE / HEALTHCARE EQUITY


SUNDAY, SEPTEMBER 22

  • Session V: Conclusions

Congress Venue

Hall / Auditorium San Paolo

Bambino Gesù Children’s Hospital / Ospedale Pediatrico Bambino Gesù   

Via Ferdinando Baldelli, 38   –  Rome

 

FRIDAY, SEPTEMBER 20


 

Friday, 20th September 2013 / Venerdì, 20 settembre 2013

13:30

Registration / Registrazione

 

14:00

OPENING CERIMONY - Welcoming remarks:

 

President of BAMBINO GESU’ CHILDREN’S HOSPITAL

 

President of DEBRA ITALY

 

President of DEBRA INTERNATIONAL    

 

 

Giuseppe Profiti

 

Claudio  Notarantonio

 

Rainer  Riedl

 

I SESSION / I SESSIONE: EB TODAY – L’EPIDERMOLISI BOLLOSA OGGI

Chairmen / Moderatori: B. Dallapiccola, A. Vicini

14:40

What’s new in clinics / Aggiornamenti in clinica

Christine Bodemer

15:00

What’s new in research / Aggiornamenti nella ricerca

Giovanna Zambruno

15:20 

Ethical challenges and resources / Sfide e risorse etiche

Andrea Vicini

15:40

Discussion / Discussione

 

 

II SESSION / II SESSIONE: THE NEWBORN AND HIS FAMILY

IL NEONATO E LA SUA FAMIGLIA

Chairmen / Moderatori: D. Bonamonte, ….

16:00

Testimony of Clarissa and Gaetano’s parents

Testimonianza dei genitori di Clarissa e Gaetano

….

16:20

Taking care of the newborn / Presa in carico del neonato

Annalisa Ciasulli

Andrea Diociaiuti

16:50

Therapeutic patient education / Educazione terapeutica del paziente

Patrizia  Amadio

17:10

Discussion / Discussione

 

17:30

GENERAL ASSEMBLY DEBRA INTERNATIONAL
ASSEMBLEA GENERALE DEBRA INTERNATIONAL

 

20:00

Welcome cocktail / Cocktail di benvenuto

 

 

 

SATURDAY, SEPTEMBER 21


 

Saturday, 21st September 2013 / Sabato, 21 settembre 2013

III SESSION / III SESSIONE: THE CHILD AND HIS FAMILY

IL BAMBINO E LA SUA FAMIGLIA

Chairmen / Moderatori:  S. Esposito, I.M. Faccin

9:30

Testimony of Riccardo’s parents / Testimonianza dei genitori di Riccardo    

Davide Serafini

9:40

Testimony of Tommaso

Testimonianza di Tommaso

Tommaso Serafini

9:50

EB and the family / La famiglia e l’EB

Cristiana De Ranieri

10:05

Hand surgery: the experience of an italian centre

Chirurgia della mano: l’esperienza di un centro italiano

Antonio Cuzzocrea

10:20

Discussion / Discussione

 

10:40

Coffee break / pausa caffè

 

 

IV SESSION / IV SESSIONE:

 THE TEENAGER AND THE ADULT

L’ADOLESCENTE  E ADULTO

Chairmen / Moderatori:  ……………..

11:00

Testimony of Angela and Anna’s adolescence

Testimonianza dell’adolescenza di Angela ed Anna

Angela Falco

Anna Faccin

11:20

Testimony of Valeria / Testimonianza di Valeria

Valeria Manca

11:30

Growing up with EB / Crescere con l’EB

Elisabetta Andreoli

11:45

The Nutritional aspects in childhood / Aspetti nutrizionali dell’infanzia

Alberto Villani

12:00  

Gastrostomy / Gastrostomia

Christine Bodemer

12:15

Esophageal dilation / Dilatazione esofagea

TamaraCaldaro

Raffaella De Angelis

12:30

Gluten sensitivity / Sensibilità al glutine

Giuseppina Annicchiarico

12:45 

Discussion / Discussione  

 

13:30

Lunch / Pranzo

 

 

15:00

ROUND TABLE / TAVOLA ROTONDA

HEALTHCARE EQUITY/ EQUITA’ DELL’ASSISTENZA

Christine Bodemer

Elena Burlando

John Dart

May El Hachem

Majdy Naim

Rainer Riedl

Daniela Riva

Ivelina Yordanova

17:00- 18:30

CONCURRENT SESSION / SESSIONE PARALLELA

Management of nutrition / Gestione della nutrizione

Lynne Hubbard

Rosie Jones

Michelle McKay

17:00 -18.30

CONCURRENT SESSION / SESSIONE PARALLELA

The management of  infected and non infected wounds

Gestione delle lesioni cutanee infette e non

Jane Clapham

Jackie Denyer

 Angela Ranati

20:00

Getting together / Serata conviviale

 

 

 

 

SUNDAY, SEPTEMBER 22


 

Sunday, 22nd September 2013 / Domenica, 22 settembre 2013

V SESSION / V SESSIONE

Chairmen / Moderatori: C. Pilo, R. Riedl

09:30

The relationship with the patient Association and the Region

Rapporti con la Regione e l’Associazione dei pazienti

Raffaella De Angelis

09:45

An italian model of interdisciplinary approach

Un modello italiano di approccio interdisciplinare

Sophie Guez

10:00

Occupational therapy / Terapia occupazionale

Florian Prinz

Hedwig Weiss

10:20

Platelet –rich plasma for wound treatment /Gel piastrinico per il trattamento delle ulcere

Gianluca Tadini

10:35

RDEB therapeutic developments: Update on recombinant collagen VII

Prospettive terapeutiche per l’EBDR: aggiornamenti sul collagene VII ricombinante

Marcia Séllos-Moura

10:50 

Prioritization of therapy uncertainties in Dystrophic EB: where should research direct to? an example of priority setting partnership in very rare disorders

Evanina Morcillo-Makow

11:10 

Coffee break / pausa caffè

 

11:30

EBCare patient-report Registry / Registro EBCare patient-reported

John Dart                          

11:50

EB-CLINET – Linking Clinical Expertise in EB

EB-CLINET – mettere in rete l’esperienza clinica

Gabriele Pohla-Gubo

12:10

Priorities in support services for people with EB

Rosie Gowran

12:30

2014 Congress Announcement / Annuncio del Congresso 2014

Rainer Riedl

12:45

Closing remarks / Saluti e conclusione lavori 

Claudio Notarantonio

13:00

Light lunch / Saluto conviviale

 

 

 

DebraCongress2013.com