Butterfly children to get specialist nurse service

The Government is to fund part-time specialist nurses who will travel around the country to support treatment for the debilitating skin condition, Epidermolysis Bullosa (EB).

The National Health Board has arranged for three specialist EB nurses to be employed through Capital and Coast DHB and located at Auckland, Wellington and Christchurch. They will provide clinical advice when a new baby is born with EB, manage ongoing care and support local medical professionals. 

EB is a condition which can have an enormous impact on those who live with it and their families.

Depending on the severity of the condition, everyday activities can be very difficult and some people are completely incapacitated by it, as their skin breaks down and blisters at the slightest touch.

Young people with the disorder are known as butterfly children as their skin is likened to the fragility of a butterfly's wings.

For eight years the support organisation representing people with EB, DEBRA New Zealand, has funded three part-time EB specialist nursing positions from its own resources. However, following a serious decline in fundraising income this essential service was in danger.

A coordinated EB specialist nursing team has the potential to improve patient care and outcomes and reduce hospital admissions.

"It is crucial to have expert advice on the best care and treatment for EB, especially when a new baby is born," says DEBRA director Anna Kemble Welch.

"EB is so rare that most doctors or nurses would have no experience with the condition or how to look after a baby with skin so fragile it can't even be picked up without causing blisters and wounds, and can't suck without blisters and skin shearing off from mouth and tongue.

"Now with the funding secure for the specialist EB nurses, they will be able to travel to any corner of New Zealand when a new baby is born with blistering skin or when an older child or adult needs expert help. The EB nurses will be available to support the medical professionals, the person with EB or parents, families and carers in the very best way to manage the at times overwhelming task of treating the blisters and wounds that form, while also making sure that family life can be as normal as possible.

"Knowing this service will be available now and in the future is the answer to a dream that DEBRA NZ has worked hard on for many years. It will make an enormous difference to everyone affected by EB and their families and communities, including the medical professionals involved in the care of someone with this complex, rare condition." 

In providing the Services, the EB specialist nurses will:

• provide appropriate clinical advice when a new baby is born with EB;
• organise for the appropriate diagnostic biopsies;
• provide appropriate assistance for EB patients who may require surgery;
• work with the two specialist dermatologists trained and experienced in EB management to provide multi disciplinary clinics
• coordinate multi disciplinary clinic appointments and maintain patient records;
• manage the integrated care plans for EB patients' ongoing care;
• provide an outreach service from the multi disciplinary clinics that shall involve visiting patients in their own local community;
• liaise with local health care professionals and care givers about EB patients' care and provide appropriate advice and support;
• provide ongoing education, development and peer support for health care professionals
• provide advice on the efficient use of appropriate dressings
• provide support for families, whanau and care givers

Key Facts:

• Epidermolysis Bullosa, known as EB, is a dermatological disease and is a group of genetic disorders causing painful wounds like burns with blistering and shearing of the skin.  In patients with EB anchoring of the skin is deficient.  The clinical type of EB depends on where skin separation occurs - the painful delaminating occurs either at key points within the layers of skin or at the interface with the tissue below.  Genetically EB can be recessive, dominant or occur as a new mutation.

• For those with severe EB, skin blistering and shearing will be caused by even the gentlest friction such as touch, a hug, clothes rubbing, sucking and even swallowing food.  Severe EB requires special protective dressings and hours of wound care and bandaging every day using techniques and products that avoid any friction on the skin.

• In New Zealand there are about 120 children and adults with EB.  While most have moderate to mild symptoms all patients affected require specialist and ongoing clinical care to varying degrees.  Severe EB has an incidence of 1 in 300,000 and currently 11 New Zealanders live with severe EB.