Care of Epidermolysis Bullosa in Ireland

Advances in the medical care of epidermolysis bullosa (EB) have led to the development of National Service Centers for EB in many countries worldwide. The exemplary model of care to children and adults with EB in the United Kingdom, combined with the knowledge that people with EB were travelling to the United Kingdom for treatment, encouraged the development of the Irish national service. Dystrophic Epidermolysis Bullosa Research Association of Ireland, founded in 1988 played a pivotal role in this development.

Advances in the medical care of epidermolysis bullosa (EB) have led to the development of National Service Centers for EB in many countries worldwide. The exemplary model of care to children and adults with EB in the United Kingdom, combined with the knowledge that people with EB were travelling to the United Kingdom for treatment, encouraged us to develop the Irish national service. Dystrophic Epidermolysis Bullosa Research Association (DEBRA) of Ireland, founded in 1988, played a pivotal role in the development of our service.

Ireland (North and South) is an island of almost 6 million people, and it is conservatively estimated that approximately 200 families in Ireland are affected by EB. The EB service caseload is outlined in Table 1 . Patients living in Northern Ireland (governed by the UK National Health Service) and those living in the south of the country have access to this service. In addition, those patients with skin fragility disorders other than EB have access because of the specialist care required.

Table 1

Irish EB service caseload (adult & pediatric)

Diagnosis	Number of Patients
EB simplex
Localized	40
Dowling-Meara	3
Junctional EB
Herlitz	2 ^a
Non-Herlitz
Dystrophic EB
Recessive	24
Severe generalized	12 ^b
Non–Hallopeau-Siemens	12
Dominant	13
Unclassified	1
Total	83

a Deaths N = 2.

b Deaths N = 7.

The service for children with EB is based at Our Lady’s Children’s Hospital, Crumlin, Dublin, where we have held dedicated clinics since 1996. The adult service is based at St James’ Hospital, Dublin and has been in existence since 2002. The current funding of the core multidisciplinary team for adults and children is outlined in Table 2 . The aims of the multidisciplinary team are to provide high-quality medical care meeting international standards of excellence for our patients, to minimize disabilities, and to offer support necessary to maintain patients’ daily lives as normal as possible.

Table 2

Current provision of core staff

Specialty	Adult Service	Pediatric Service
Specialty	WTE	WTE
EB nurse specialist	1.0	1.0 ^a
Medical social worker	0.5	NDF
Occupational therapy	0.25 ^a	NDF
Physiotherapy	0.2	NDF
Psychology	0.25	NDF
Clinical nutrition	0.5	NDF
Secretary	0.5	NDF