Butterfly Children Receive GG's Patronage
Butterfly Children Receive Governor General’s Patronage
Children with a rare genetic disorder, known as EB or Epidermolysis Bullosa, in which skin breaks down and blisters at the slightest touch, today (subs 28 November) received the Governor General’s patronage at a special event to mark the beginning of the first EB awareness appeal.
Ms Anna Kemble Welch, secretary of DEBRA-NZ, (Dystrophic Epidermolysis Bullosa Research Association) said public understanding of EB was essential if those with the condition were to be helped.
“Until now, we have been a network of families and friends of EB children struggling to raise awareness of this cruel and painful skin disorder. Today, DEBRA-NZ becomes a wider-known organisation, informing the public and medical fraternity and fundraising to assist people with this painful and debilitating condition.
“We are thrilled that the Governor General, Dame Silvia Cartwright, has given her support to EB sufferers and their families,” she said.
Guests of honour at today’s Government House reception, attended by 250 friends and supporters of DEBRA, will be four of the five New Zealand children who have a severe strain of EB and others - adults and children - with varying degrees of severity of the skin disorder.
Children with the severe strain of EB live in constant pain with their skin falling off at the slightest abrasion. They are often called ‘butterfly children’ because of the fragile nature of their skin.
Severe EB sufferers are covered in layers of scars and blisters and their internal membranes are damaged by abrasive food. Their throats slowly close up with scar tissue. Operations to open the oesophagus are common, as is plastic surgery to straighten limbs and fingers.
Ms Kemble Welch, a parent of an EB child, said that the chronic skin disorder has huge implications for those inflicted with EB and also for their families and cited bath-time as an example.
“While bath-time for most children is a fun activity, for EB sufferers and us, as parents, the daily bathing regime, in a saline solution and the application of ointments and sterile bandages, is agonizing. The three-hour process is sometimes undertaken by ‘bathers’, who relieve parents of the anguish of bathing their own children,” she said.
EB has no cure and, if EB children don’t die at a relatively young age from complications like septicaemia, they run a high risk of developing skin cancer at a later age, because of the severity of their skin damage.
Despite coping with extraordinary pain, EB children are remarkable and, like all children, want to live their lives as fully and normally as possible.
DEBRA-NZ is appealing for support and hopes to raise $150,000. Bright pink donation boxes, decorated with butterflies, will be at Amcal pharmacies throughout New Zealand until Christmas.
Funds raised will assist with projects that
will significantly help the children and adults in
New Zealand who live with the daily pain of EB. Among the projects are:
Part-time specialist EB nurse
Funding of a part-time specialist nurse to give support and advise to all EB sufferers in New Zealand. The nurse will also to be available to provide expert postnatal care for new babies born with EB.
It is important to note that a parent has no forewarning that their baby may be born with severe EB and it is highly unlikely that attending medical professionals will have experience or expertise in dealing with EB.
A life-experience camp:
Organising a camp for EB sufferers in the Oceania region, at the Outdoor Pursuits Centre in the central North Island of New Zealand.
Holding a national conference for EB families and the medical fraternity, bringing to New Zealand leading overseas-based medical specialists to share recent developments in research and the treatment of EB abroad.
USA skin disorder
Sending each of our EB children to a camp in the USA. Every year, members of the American Academy of Dermatology organise summer camps in the USA for children with rare skin disorders where specialist dermatologists are on-hand to assist and advise the children. The camps are a rare opportunity for children to spend time with others of a similar age and condition.
Contribution to international
The most promising potential remedy for EB is in the field of genetic research. International research efforts are being co-ordinated to avoid duplication and to speed up the discovery of a cure. At this stage, New Zealand is not involved but DEBRA-NZ aims to build sufficient funds to make a positive contribution to research efforts to find a cure.
Annual international EB
Every year DEBRA groups around the world meet to share ideas and information. It is important for all EB sufferers (children and adults) in New Zealand to be part of the global EB network to share in new development and support systems.