Contents

Acknowledgements

Program

Commentary (Saturday)

Commentary (Sunday)

Observations

Acknowledgements

It would not have been possible for myself or any other delegate from Australia to attend this conference if it was not for the generous support of the following. Their efforts and generous contributions are greatly appreciated and DebRA Australia looks forward to working with these people and their organizations for many years to come.

Kjetil Moen - Mölnlycke Health Care Pty Ltd

Nigel Sinclair - Nu Skin Enterprises Australia

DebRA Australia was also supported in this project by all the state bodies that contributed funds to make this trip possible. I would also like to thank the DebRA Australia committee, which is made up of representatives from each state, for their ongoing support and efforts in the creation of DebRA Australia.

My employer Energy Australia who granted me paid special leave to attend the conference on behalf of DebRA Australia. They have always been supportive of my need for work and family life balance when required.

Saturday Program - Annual Meeting / Stockholm April 22. – 24. 2005

08.00 – 08.45	DebRA Europe Conference, May, 2005.	Registration
08.45 – 09.00		Opening
09.00 – 09.30	Smith & Nephew	Dressings
09.30 – 10.00	Mølenycke	Dressings
10.00 – 10.15		Break
10.15 – 11.30		Questions/panel practical use of dressings
11.30 - 12.00		Break
12.00 – 12.30	Robert Snookes	What can we learn from people with EB? -
12.30 – 13.00	Nina Benan & Nina Rambæk ;	What do you tell your child? How can we handle the difficult questions?
13.00 – 14.00		Lunch
14.00 – 14.50	Carina Hvalsted & Andreas Tallborn	Family dynamics
14.50 – 15.20	Nina Braathen	Nursery school and school (mobility)
15.20 – 15.35		Break
15.35 – 16.00	Kari Anne Bø	Teens – teenager first, forget your EB?
16.00 – 16.30	Øyvind Alster	Leisure time/Rise to the challenge
16.30 – 18.00		Parallel sessions Office managers forum and Fundraisers forum.
19.30		Formal dinner.

Sunday - Program Cont'd

09.00 – 10.00	Janice Carrera/Elizabeth Pillay	Relationships /EB and pregnancy
10.00 – 11.00	Lesley Haynes	Nutrition
11.15 – 11.30		Break.
11.30 – 11.45	Gabor Koranyi	EB and eyes
11.45 – 12.15	Claes Hultling	See the possibilities
12.15 – 12.30		End of conference / Lunch

Commentary

Friday 22 April 2005

Delegates where able to register for the conference on the Friday afternoon and where invited to a reception buffet that evening. During this evening delegates from the 23 countries attending the conference had the opportunity to meet each other in an informal setting. As this was the first international conference I was attending this was a great chance for myself to meet many people I had only conversed with via email. I was also able to meet many other people with various interests in EB from people with the condition, parents and those who work as paid officers for DebRA in the different countries. The networking was great and many business cards were exchanged. I am looking forward to the possibilities that may come from this conference from the networking alone that occurred.

Saturday 23 April 2005

Welcome

The day started off with registrations for the people who were not able to attend the function on the Friday night. This was followed by an official welcome by dEBra Nordic the organizing group for the conference outlining the program for the weekend.

The delegates were then broken into 2 groups. Delegates who needed to attend the Annual General Meeting of DEBRA Europe left for another room while the remainder stayed to hear presentations on dressings. Representatives from Smith & Nephew as well as Mölnlycke conducted this presentation on dressings.

Dressings

Iver Helgaker from Smith & Nephew spoke about the history of his company and their range of dressings commonly used in EB care. This included Allevyn, Exu Dry, Acticoat fixation products and hydrogels. Smith & Nephew now have an Acticoat Absorbent, which is an alginate. All of these products are available here in Australia.

Margareta Uggla from Mölnlycke Health Care spoke about their products that use Safetac Technology and the history of Safetac, which has now been around for 15 years. The products presented where the Mepilex range which included Mepilex, Transfer, Lite, Border and the new one Mepitac. This new product Mepitac is a fixation tape which uses the Safetac Technology to hold dressings and IV’s etc in place which minimizes trauma to the skin when removed. It does not require any other products to remove it and leaves no residue. All these products are available in Australia and if DebRA members are unable to get them through their state health service they can be purchased direct from Mölnlycke Health Care. Mölnlycke Health Care offers a special price to DebRA members to help keep the cost down.

Panel Session on Dressings Questions and Practical uses

This panel session included the representatives from the companies mentioned previously along with EB specialist nurses. I have included some of the questions and responses to give an idea of the discussion, however the manufacturers and medical staff guidelines should be followed on a case-by-case basis in conjunction with the individual. As we know EB is very individual and what works for one person may have different results for another. This information should not be used to override that advise. Some of the questions included:

How do I know when to change the dressing?

The answers here depended on the specific product being used however most of the products represented ranged from 3 days to 7 days. This would also depended on the amount of discharge from the wound.

What is the best way to apply that dressing?

Mölnlycke have developed a cutting guide that gives examples on how to prep their dressings depending on the area concerned. EB specialist nurses and other medical professionals can also help people with EB on how to use dressings and their recommendations on what they think might work in the individual. The advice also from the nurses was that medical professionals should also listen to the EB patient on how they found a specific dressing and make adjustments if required or requested.

I have tried Acticoat but found it dries out and can damage the area when being removed?

The advice was to try the Acticoat Absorbent, the alginate, or try placing the Acticoat under the first layer of Exu Dry to avoid damage to the wound area.

Other then the Mepitac there was no other dressings presented that I have not seen or heard of before. The representatives also pointed out that there is continued research and development going on into dressings by these companies and they do welcome feedback on their individual products.

What can we learn from people with EB

Presenter: Robert Snookes, Social Worker DebRA UK

This was a presentation from Robert on what he had learnt from working with adults who have EB and how best to be an advocate for them and provide support when required. This presentation was intended for other professionals working with people who have EB or people representing the EB community. How the use of words such as "disease" and "sufferer" can cause offence even when used with the best intentions. The use of words like these can make the person with EB feel patronized and undervalued. The use of jargon should only be used within the realm of other professionals and not in forums where there is a wider audience. The most important person/people change throughout the individuals life i.e. their parent(s) the person themselves, their life partner and then as is happening more and more the children of the couple. This is the same process for almost anyone with E.B. or not.

Robert broke his presentation down around the following key words.

• "LISTEN"

• "TRUST"

• "DETERMINATION"

• "NEVER"

• "FRIENDSHIP"

• "ILL NOT STUPID"

• "TIME"

• "PATIENCE"

• "FREEDOM"

EB is a condition, NOT a way of life.

The most independent of people make EB live with them, they do not live with EB.

If all these are taken into account the relationship between professionals and people with EB is more productive and is a two way street.

Family Dynamics

Presenter: Carina Hvalsted

This was a presentation about the centre where Carina works called Agrenska. This center is an interdisciplinary team that works with family unit who have a child diagnosed with a rare condition. They work with the family by focusing on the medical and psycho-social issues. The goal of the program is to assist the families to achieve the greatest possible independence and participation in society. This is done through a number of weekend workshops where the family stays at the center and has access to the different specialists within the unit. Their website is ww.agrenska.se

They run programs that look at the needs for:

• The individual person with a rare condition
• Children
• Parents
• Siblings

This is followed up by newsletters letting the families know about any new information on rare disorders. Included is case studies and follow up weekend workshops that are available to the family.

What do you tell your child? / How can we handle the difficult questions?

Presenters Nina Benan / Nina Rambaek : Centre for Rare Disorders University Hospital Norway

This was a presentation about the centre where they work in Norway. They work with the family to develop coping strategies.

They look at the following areas:

• Impairment
• Disability v Ability
• Empowerment
• Quality of Life
  • Aron Antonovsky: Sense of coherence
  • Manageability - Solutions
  • Meaningfulness – daily living is meaningful
  • Comprehensibility – acceptance of one’s feelings (Control)
• Interaction

How to handle the difficult questions

This will always depend on the characteristics of the individual, the family and the society we live in.

• Communication
  • In communication with children we must be aware of some assumptions
  • Children always reflect the attitude of their parents
  • Children always want to interact and be responsive
  • Children always want to be responsible
  • Children can always see through a lie
  • Children have very good intuition

What to tell your child, coping and some strategies for parents:

• Coping
  This is a universal phenomenon. Living with a chronic condition seriously challenges our coping ability. To cope is a balance between our personal abilities and the environmental demands. Learn to see the difference between what we have to live with and what we can change.

• Strategies
  There was many strategies discussed for parents in this session and I have listed a few that appeared to get the most positive responses from the group.
  • Avoid situations
  • Protect
  • Social support
  • Informed
  • Acceptance
  • Share feelings
  • Pride to succeed

Some feedback and hopes from parents who have participated in the program

• Live as normal as possible.
• Leave the responsibility to someone else
• Nature is good medicine
• Motion makes you feel better
• See the person not the diagnosis

Nursery School and School (mobility)

Presenter Nina Braathen

Nina spoke about her experiences with families living with EB and what Occupational Therapists and Physiotherapists could offer to families, individuals and school environments. That they are a resource that can be called upon to review home, nursery schools and schools for any potential issues. They offer possible solutions and recommendations for changes to the environment that suite the individual with EB. It is then up to the various people involved as to whether or not they accept, amend or take no action in regards to the recommendations. There was many scenario’s presented with lots of detailed options, too many to include in this report. The main thing is that this is a resource that is available for families and individuals and that they should adopt the recommendations they feel comfortable with.

Teens-Teenager first, forget your EB

Presenter Keri Anne Bo

Keri Anne lives with EB Simplex type Ogna. There is a strong history of this in her family and Keri Anne spoke about her experiences at school and her first job’s and how EB impacted on these events in her life. The strategies she employed to not let people know about her EB, or to let people know about it when she thought this was necessary. She feels fortunate to have been allowed to try out things she wanted. Sometimes it has gone ok and others not so well.

Keri Anne really dreaded being made to do sports at school or go on excursions. These were the times that the consequences of having EB Simplex really become visible. She believes she has knocked a number of teachers off their feet when they have seen what a bad EB day could be like. This is regardless of the warnings she tried to give them of what these activities could cause her.

In everyday life nobody can see that she has this condition and most people find it difficult to understand what this condition means to her and how it impacts her life, as it does not always show.

Her EB has been not disclosed to some employers in the beginning due to the fear of not getting the job or being fired. The difficulties of trying to work around this and eventually having to explain what this meant. She has found that the best thing is to approach things as a challenge and to try and push boundaries. Keri Anne enjoys traditional dancing and if that means she has to be off her feet for a couple of days due to blisters and pain that is the price she is prepared to pay as she enjoys this so much. Her EB does not stop her from doing the things she wants and she will balance the consequences against her desire to do something and then go ahead and do it.

Leisure Time / Rise to the Challenges

Presenter Øyvind Alster

This presentation was based on his 17 years experience working with disabled people in Norway. He currently works at a camp for able body and disabled persons where the activities are adapted to the individuals need so as they can participate and share the experience. One of the people who attend the camp regular has EB.

Is leisure time for a disabled person different from not disabled people? If so in what way and what can be done about it? Persons with handicaps have the same needs for leisure time as everybody else. However they may need some type of adapted activities. It is very important that the leaders of these activities have knowledge in how to adapt the activity based on a good knowledge of the condition the individual has. There is also a problem with reaching out to the families with information about the activities they can take part in. Leisure time should be done apart from the individual’s normal routine and not just fitted into; it should have specific time set aside.

Some of the solutions he presented may be things such as computer interests, swimming and common interest groups that require little or no changes for the individual. Outdoor activities, which include an experienced leader to start with to assist in adapting or changes that maybe required so the person, can share the experience with others. This also encourages them to continue with the activity and not give up because it maybe just to hard or not adapted correctly in the first place.

DebRA Europe Fundraisers Forum

Discussion Group

This was a discussion group chaired by Mark Smith director of fundraising and communication for DebRA UK. Many of the DebRA Europe countries were represented along with others from the America, Canada, Chile and myself from Australia. The items that were discussed included:

• Branding
  

  DEBRA, debra, DebRA, dEBra which variation should be used, logo colours and style. There are many variations in use internationally. After much discussion it was decided to develop a brief and ask a PR and Marketing Company to come back with some suggestions. A working group was formed which also included Suzanne Cohen the new director for DebRA America so as to include an international perspective. The group is to develop the brief and review recommendations. This group would then forward their recommendations to all for discussion.

• PR campaign
  

  This was were some countries presented ideas on what was working for them. I will go into more detail on some of the ideas further on.

  At this time there was also a discussion around using children to promote EB and DebRA. How much is too much, where should the line be drawn. It was agreed that it was up to the individual families involved as to how much was shared with the media and marketing people. DebRA must also be supportive of the families involved and ensure that their best interests are kept in mind.

• DebRA UK support for Europe
  

  Marketing material has been developed by the UK and this material is available to other DebRA Europe countries, which can be adapted to their specific needs fairly easily. This also applies to other countries outside Europe the material is available to all DebRA countries international who wish to use it.

  DebRA UK is also establishing a new database system, which they can keep track of all their activities on. One feature of this product is that there can be sub groups. These sub groups can be other countries that can store their activities and information on such as membership lists, record of activities, funds and mailing lists. Each group’s information would not be able to be accessed by other groups and would be in a similar format for all. Countries interested in participating can advise Mark Smith who will arrange the access when the package is being installed in the near future.

• Mutual Fundraising Opportunities. What are they and Making them work?
  

  DebRA Ireland commissioned Pilgrim Jewellery to design a piece of jewellery. They then order 500 to start with that they launched and sold at their DebRA ball, just prior to mother’s day and just shortly after the running of the documentary "The boy whose skin fell off". Retailers that sold Pilgrim Jewelry also agreed that all proceeds from the sale of the item would go to DebRA Ireland. With each piece sold the purchaser also got and information leaflet on EB and the work DebRA does. They have sold all 500 pieces and have orders for more. DebRA Ireland has order another 500 pieces to be made. If other countries are interested in doing a similar thing DebRA Ireland is available to assist with ideas and set up.

  DebRA Austria went to an advertising company and asked them to produce a campaign. This campaign was for newspapers and TV to raise awareness of EB and raise funds for a DebRA house they wished to build. The advertising company delivered a campaign that did not include any pictures of EB. Please refer to attachments 1, 2 and 3 for a preview of these posters. They also did a TV ad along the same lines of the poster.

  The advertising company was so touched by the stories of people with EB that they did the campaign for next to nothing. The TV advertisement has been nominated for awards and we wait to hear the results. Other countries are welcome to approach the company involved who would like to see the advertising campaign in other countries. As the artwork for the posters and the shooting of the TV advertisements have been done it would not be a lot of work to adapt to other countries logos and information.

Sunday 24 April 2005

Relationships EB and Pregnancy

Presenter Janice Carrera & Liz Pillay

Senior EB Nurse specialist for Adults (DebRA UK)

We were fortunate that these presenters had included a detailed summary of their talk in the program. I have included this word for word from that document written jointly by the above presenters as I feel it describes their presentation better then I could.

When a child with EB is born it affects the relationships within the immediate and extended family. There is often a feeling of loss and grief and the family’s hopes for the future.

One of the first questions a family asks me is "will my son/daughter be able to have normal relationships with the opposite sex – will they be able to marry and have children of their own?"

Our protective feeling towards affected children may prevent them from enjoying normal experiences, which lead to a healthy cognitive and emotional development. Social isolation may set in as families lose contact with friends and the community becoming preoccupied with EB and shutting out normal experiences.

Mothers must learn to accept outside help as soon as possible so that they are not just looked on as the main carer and a more healthy relationship can develop within the family.

This is why we firmly believe that people with EB, however severe, should have the widest possible experience of everyday life with all its ups and downs.

A major problem is that statistics show that often the parent’s relationship has failed due to the pressure of bringing up a child with EB so there is no role model of a "normal" family unit whatever that means.

Towards puberty a teenager with EB has all the same thoughts and urges as their non-affected friends and these should be encouraged not suppressed. If they start having sexual experiences the usual contraceptive advice and help should be offered as is to all their peers.

Often a rebellious streak sets in, the EB Teenager perhaps trying to have more sexual experiences just to prove that they are like their friends. They often hide the fact that they are having a relationship. Parents are often horrified when it is revealed so require a lot of support themselves. They are fearful that their teenager will be in danger, especially of skin damage. When the new relationship becomes permanent there is often a sense of loss as they feel their caring role may be diminished and taken over by the new partner. However it is most important that the partner does not become the nurse.

When the EB daughter becomes pregnant there is an even greater fear of the future. We now have experience of several people with EB who have had successful relationships.

Becoming pregnant, giving birth and caring for a child are normal aspirations for most young people, as is the experience of being in a loving sexual relationship. In the past those with the severer forms of EB were often, for various reasons denied this experience. Over the past few years some of the young men and women we have come to know, who have relatively severe forms of EB have achieved happy relationships with a normal sex life. As a result of this we now know women who have become mothers despite their EB, and for some despite advice given by both professionals and their own families. In the UK (and indeed globally) we now have a number of women with RDEB who have successfully given birth to healthy babies, both by caesarean section and normal vaginal delivery. We also know men with RDEB who have become fathers to healthy children. Participating in a sexual relationship, becoming pregnant, giving birth and subsequently caring for a child when you have a condition such as RDEB is not without anxiety for all concerned. Concerns are often expressed by the person with EB, partner’s families and the medical team. Many of these anxieties can be overcome by the provision of information and the sharing of experiences. We aim to share some of these experiences with you this morning and hope you will be able to participate.

There was then an interview shown of 2 ladies from the UK who have both had successful pregnancies. They spoke about their concerns and fears during the pregnancy and labor, the good and the bad.

Nutrition

Presenter Lesley Haynes

Specialist Paediatric Dietitian

GOSH Hospital London

Great Ormond Street Hospital (GOSH) in the UK cares for around 250 EB patients with all degrees of severity mainly from the UK with some from overseas. Lesley has worked in this position for 16 years, working solely with EB families. Every EB child is different but when one sees large numbers of patients, similarities and trends emerge. Lesley has got to know what works and what does not, and so can minimize re-inventing the wheel. EB is a many faceted group of conditions and, no dietitian can help families single handedly. Lesley works as part of a multi disciplinary team at GOSH. They recognize that whilst many factors influence a child’s nutritional status, equally many aspects of the child’s life are influenced by nutrition and it is vital all professionals work together.

Children with milder forms of EB are much less likely to have nutritional problems, but in severe EB, 2 main factors hinder nutritional status:

• Reduced nutritional intake as a result of oral, esophageal and gastro-intestinal complications.
• Increased requirements due to losses of blood and nutrients in blister fluids and the high cost of infection healing.

These are tough adversaries, but it is possible significantly to reduce their impact. They try to tackle the oral, esophageal and gastro-intestinal complications by:

• Painstaking dentistry to minimize scarring in the mouth
• Carefully controlled serial esophageal dilations
• Early use of medications to minimize acid reflux
• Pro-active gastrostomy placement with individualized feeding regimes
• Balancing of laxatives, stool softeners, pre-biotic and fibre

Quality of life for both EB children and their families is paramount and the role of nutrition support should be part of a holistic approach to EB care.

See the Possibilities

Presenter: Gabor Koranyi

This final presenter spoke about his personal experiences and challenges. Twenty years ago he dived into shallow water and ended up in a wheel chair for life. As a doctor he states, "you think I would have known better." After the accident he become a patient in the hospital in which he worked. As a doctor he made a terrible patient.

He had a young family and his career was going places now he was in a hospital bed and did not necessarily like the way in which the system treated him. After 3 months he could no longer stand the hospital and its environment and wanted to be discharged. His doctors were against this but he discharged himself anyway and with the help of friends in the emergency services he was flown by helicopter to his families summer holiday location.

Here he felt much more relaxed away from the hospital system, with old friends and in a safe familiar environment. The fisherman from the village who he had grown up with over his summer holidays were used to their fair share of accidents and tragedy in their industry. They did not feel sorry for him or pity him for his situation but did acknowledge it like this "shit happens do you want to have a drink." Gabor adopted this as his motto as these men who he had known most of his life saw him as himself first and his condition second.

He has since traveled the world looking at how people in wheelchairs are treated and the services they have access to. After his return he started campaigning for a separate spinal cord injury unit in Stockholm. Not just a ward of the hospital. After some years they got a site that needed a lot of work. They raised funds and built the unit with the input from people in wheelchairs. It is a welcoming environment for people in wheelchairs. For example the kitchen has no cupboards under the benches or sinks. Cook tops and stoves are all at the right height; storage space is accessible to all. Meeting rooms are spacious and not filled with furniture.

Gabor loves sailing and cooking he has also written a cookbook called. "The Cook Book for Crips." His publisher and others were not big fans of the title and thought some might take offense. He would not budge on this and his book ended up being one of the best selling cook books in Sweden that year and has now been translated into a number of languages. He is also still a practicing doctor.

Observations

It was a great opportunity and I only wish more people involved with EB had the opportunity to participate in a conference like this. It was great to see people with EB and professionals interacting on a number of levels from common interest chats to EB in general.

There are a number of views out there on how EB is treated and how awareness of the condition is promoted to the wider community. It was interesting to note that some of the more conservative and extreme views came from people living with EB. It is for this reason alone that care must be taken when doing anything to represent the EB community to ensure that there is widespread support for the planned action what every it might be.

The networking opportunities with other DebRA support groups is just to good an opportunity not be involved in events like this. It was great to talk to representatives from other countries to see what new treatments there might be, what awareness and fundraising campaigns work for them. The services available and not available across the different countries.

One thing I did find, as well as representatives from other countries also noticed, was that the countries that are successful with services, awareness campaigns, fundraising and research were the countries who employed professionals to work for DebRA. This is something I think that DebRA Australia might want to consider in the future. It is a big step however the model from other countries is out there to be duplicated and these countries are very supportive in advise they can offer to others should they wish to do the same. These countries do also appear to have good involvement from the wider EB community in their activities as a result.

DebRA Europe has recently secured funding from the EU for 3 research projects expected to run a number of years to the value of 4.8 million Euros. It will be interesting to see the work being done and updates on their progress at conferences in the coming years.