The Funds Raised for DebRA by the Cannonball Charity Ride will go the support the EB Nurses project.

Cost of Project: Cost $160,000 per year (for year 2 of the project from June 2011‚ 2012)

Background Information

Dystrophic Epidermolysis Bullosa Research Association Inc. (DebRA Australia) is a non for profit organisation established in 2005. It is the only EB support group in Australia and is dedicated to funding essential support services to children and adults with Epidermolysis Bullosa (EB), and give hope to people who desperately need it. EB is a painful genetic skin disorder, where the skin layers and other body linings separate and blister at the slightest touch. Normal day to day life can cause severe pain that leads to disfigurement and disability. Imagine your skin is a fragile as a butterfly's wings; this is the reality of people living with EB. There are approx. 1,000 people in Australia with EB ranging from mild to severe in condition. We are seeking $75,000 to support year 2 of a new project, to enable services to be continued so that a meaningful evaluation can be completed.

Existing Project

EB care needs are very specific, and knowledge among the national medical community is very limited. DebRA Australia has identified there is currently a huge gap in access to specialised advice and support in the early years of life for babies born with EB. There is currently only one specialised part time paediatric EB Nurse in Australia, employed by the Sydney Royal Children's Hospital . DebRA has gained funding of $100,000 in 2010 from the nib Foundation to assist in the establishment of a two year program costing $160,000 per year to employ 2 part time national Rapid Response EB Nurses. This service will enable early intervention for newborns and provide equity of access nationally, ensuring improved quality of life for families with babies born with EB. DebRA aims to operate this specialist nursing service for 2 years and during that time collect statistical data to substantiate the necessity of State based EB Nurses throughout Australia. This date will be used to provide tangible evidence of the benefits of such a service to State and Federal governments, with the aim of ultimately having nurses based in all States to ensure there is national coverage of EB care via the public health system.

The EB Nurses will travel to newly diagnosed patients and provide early education on dressings, support, medical care and nutrition to parents, schools, professionals and relevant community groups. This will improve community involvement, lead to improved patient wellbeing and equity of care on a national basis.

To date a memorandum of understanding (MOU) is in place with the Sydney Children's Hospital for placement of the first national EB Nurse to commence duties on the 1^st June 2010. Negotiations are underway with the Brisbane Royal Children's Hospital for placement of the second EB Nurse who we anticipate will commence 1^st Jul y 2010. Due to the rarity of the condition and specialist skills required, the EB Nurse position is classified as a Clinical Nurse Consultant Level 3. Part of the project includes a Project Coordinator to liaise with the hospitals, implement the new project, organise travel and accommodation, evaluate statistics and lobby for continued services.

DebRA International and DebRA UK will be utilised as mentors to ensure best practice EB Care guidelines are implemented. A national survey has been completed as a baseline to allow evaluation of the impact of the new services. Further audits and a satisfaction survey will be developed to evaluate outcomes and identify areas for improvement. The national EB Nurses will liaise with other health professionals involved with EB care.

Key performance measures which will be used to evaluate progress include:

• Percentage of new EB babies born in Australia contacted within 72 hours of notification (target 100%)
• Percentage of identified EB patients have access to suitable evidenced based dressing protocols (target 100%)
• Percentage of Hospitals with a maternity ward, contacted to increase awareness of EB diagnostic methods and access to care (target 100% contacted annually)
• Amount of additional funds attracted to enable continuity of the service

Program risks include:

• Inability to attract/retain suitably qualified staff
• Significant increase in incidence of sufferers, which could outstrip supply of allocated nursing staff
• Project Coordinator unable to secure sufficient funding to maintain continuity of service

Program will be evaluated by:

• Adherence with the project plan and the key performance measures will be assessed quarterly by DebRA and due diligence provided to funding bodies by an annual audited report
• Quarterly reviews will be conducted with employees
• Six monthly reviews will be conducted with the institutions involved
• EB Database Registry statistics will be used in conjunction with the reviews
• Feedback will be obtained from patients/carers via surveys
• Review of strategic plan annually

Dissemination of information will be conducted by sharing information via:

• The International and National DebRA Conferences
• DebRA Australia's Website
• National newsletter to DebRA members and supporters twice yearly
• DebRA Committee Meetings
• St George Hospital where EB Database Registry is maintained
• Liaison with Federal and State health officials

Who would benefit from the project:

There are approx. 1,000 people in Australia with EB and it is their families and extended families that are impacted by this rare distressing condition. Implementation of the program will ultimately impact and support 100% of people in Australia with EB, improving the quality of life for these individuals and their families.

Organisation's Ability to Carry out the Project by June 30^th 2012:

The progress made in starting the National EB Nurse Program was outlined earlier and we are confident that it will be implemented as envisaged. Furthermore, although a small charity for a rare disease, DebRA has achieved great milestones since its conception five years ago, and has an excellent track record considering its limited volunteer base and resources. Previous lobbying has resulted in the allocation by the Australian government of $16.4 million to fund a national EB Dressing Scheme for 4 years. A Sydney based company called Bright Sky has won the tender to administer the scheme, which will result in EB patients receiving subsidised monthly dressing orders delivered to their door. Other achievements and programs in place include:

'We are small, rare, but we do count.'

Who is DebRA Australia?

Dystrophic Epidermolysis Bullosa Research Australia (DebRA Australia) is a professionally-run, non-profit organisation that receives no Government funding. DebRA was set up in 2005 to fight for Aussie children born with a rare, genetic skin condition called Epidermolysis Bullosa (EB). This organisation is run by a team of committed and hard-working volunteers and one part-time employee, located all around Australia, each closely connected to the cause.

Connor's Story

Imagine your skin is as fragile as butterfly wings. It falls away from your body at the slightest touch leaving painful blisters and over time scarring so severe that your fingers and toes may fuse together. Imagine having to live your life wrapped in bandages and in constant pain. Even lying down or rolling over hurts your skin. Now imagine the thought of living your entire life in this state with no hope of a cure.

Hi my name is Connor and I am 11 years old. I have EB (Epidermolysis Bullosa) this means that my skin does not work properly and I have sores over my body and inside. Sometimes I can not eat my favourite food like chocolate or fruit because it is like eating barbed wire and hurts my mouth, can you imagine that? I have to be feed through a tube in my tummy. Sometimes I can't play with my friends because I have sore eyes and have to stay in the dark for days until they get better. I have to breathe through a tube in my neck called a trache. I hate having a shower because it hurts my skin and it takes me and mum 2-3 hours to take off the old dr essings and put on new ones to protect my skin. My favourite things are motorbikes and I have a quad bike that I ride when I can as well as playing my X-box 360.

DEBRA Australia has a vision working for a life free of pain. We provide a network of support for kids like Connor. With access to specialist nursing services, provision of vital medical equipment. Holding camps so people can learn the latest about EB, make friends with others living with EB so they do not feel isolated. DEBRA Australia also lobby's the government at all levels for improved services and funds research so that we can ultimately find a cure. Please help us support kids like Connor.

What is EB?

DebRA Australia advocates for the 'Butterfly Children' who live with EB, born with skin as fragile as a butterfly's wings. There is currently no cure for EB. These brave children;

- have skin that blisters and peels at the slightest touch, internally and externally.

- live in constant pain, often likened to the pain of living with third degree burns.

- bandage their skin every day to protect and heal the blisters and wounds.

- often have their young lives cut cruelly short by secondary diseases, such as skin cancers.

How DebRA helps its members

Without DebRA Australia, our members would have nowhere to turn. Working together, DebRA;

- lobbies for improved EB services

- raises money for research into a cure for EB

- researches best practice treatments for EB

- provides emotional & financial support and advocacy for their members, and

- provides members with important social and medical support networks

Why DebRA needs help

We are small: Although DebRA Australia is a powerhouse of a charity, we are very small, and often struggle to get our message out to the public and to the government.

We are rare: DebRA Australia is the only charity of its kind in Australia because EB is rare, affecting only 1000 Australians. It is hard to secure funding for research into a cure for EB when people are often not directly, or even indirectly, affected by EB.

But we do count: DebRA is the only EB support group for families with EB, we are making a difference.

How DebRA Australia is changing the world

For three long years we have battled the political heavyweights of Canberra to get a National EB Dressings Scheme off the ground to fund dressings which often cost families and DebRA thousands of dollars a month. We have attended meetings at Parliament House, sent numerous letters, meet with local politicians, and conducted surveys with our members to present current data to the Federal Health Minister.

Our hard work finally paid off late l ast year when it was announced that DebRA Australia had brought about the FIRST EVER Australian National EB Dressings Scheme, worth $16.4 million over 4 years!! Meaning EB patients now receive monthly dressing orders delivered straight to their door. This is a huge step forward for EB patients and their families, but there is much more to be done.

DebRA Australia's recent successes

A federally-funded National EB Dressings Scheme - $16.4 million over 4 years starting January 2010. This is a first for Australia. Similar schemes have been around in the UK and NZ for 10-20 years.

2 part-time National Rapid Response EB nurses funded for 2 years from 2010. This is a first for Australia. We are already applying for more financial support to keep this crucial nursing service going long-term.

DebRA's current challenges

1. Sustain federal funding to ensure the EB Dressings Scheme stays a permanent service.

2. Secure permanent federal funding for EB specialist nurse services Australia-wide.

3. Spread the DebRA message through social networking and national events.

4. Further fund research into a cure for EB.

5. Secure funding for the Biennial EB Conference and Kids Camp in 2012 for all DebRA members.

6. Increase the exposure of the DebRA brand.

How do I find out more?

Visit our website www.debra.org.au.

'We are small, rare, but we do count.'

What type of bike do I need to have?

On previous Cannonball Rides we have had everything from 250s to 990s. Ideally though 450s and up better suited to the long dist ances travelled each day.

How long do we ride each day?

We average about 500kms each day.

What fuel range do I need?

200km

Does the bike need to be Registered?

Yes. All bikes are registered and must be roadworthy for the event. Bikes will also go through pre-event scrutineering.

Do I need to carry everything myself?

No - a luggage vehicle will carry your gear between each nights stop.

What do I need to take?

• Bike Gear: including Boots, Gloves, Goggles, Clothing (nylons/draggin jeans, jacket and jersey) and any other protective gear you feel necessary
• Clothes for the night
• A check bag to send your riding gear back in (ie $2 Crazy Clarks bag)
• A 20l jerry can with your bike number on it
• Tool kit to carry on the bike and ideally tubes so we can get you going without waiting for the support vehicle
• Oils, Filters and any other consumables for the service day
• And above a sense of humour

What about tyres?

Closer to the ride date we will provide you with an address to send tyres to. We recommend Cannonball Supporter, Tyres for Bikes, they provide tyres at competitive prices and will send them direct to the service location for you. Contact Dave on 07 3262 4377.

Do I need to be a mechanic?

No, each ride has a collection of McGuyer types and we can usually someone running again. On our last ride we even rebuilt the engine of a 640KTM.

Are there any hidden costs on the ride?

If you don't count your dignity, No...only thing you need to pay for after the Entry Fee and Donation Fee is - any servicing of your bike that is required, Alcohol and souvenirs.

Can I get my deposit back?

No, however if you cannot make the ride for factors outside your control (eg loss of employment, injury) a $1000 credit will be applied to an entry in your name for the following ride.

 How do I get home?

Your bike is automatically covered to get back to Brisbane, however you will need to find your own way home. The reason for this is that alot of the wives and families meet up at the finish line and riders stay on for a short break with the family, so it is easier to make your own arrangements to get home.

How does the charity get it funds?

The ride doesn't rely on 'turning a profit' to raise funds, rather your ride fee is broken up into 2 parts: a donation to our chosen Charity and a fee to cover the cost of the ride.  Even if the ride organiser encounters a large unexpected cost the charity will always get the full amount of your donation.

Can I raise more than the minimum Donation Fee?

Yes - we encourage it!