Epidemiological, clinical, pathological and genetic characteristics of epidermolysis bullosa in New Zealand.

OBJECTIVE: To establish the epidemiological, clinical, pathological and genetic characteristics of epidermolysis bullosa (EB) in New Zealand (NZ). METHODS: Participants were recruited through the Dystrophic Epidermolysis Bullosa Research Association of New Zealand (DEBRA NZ). Dedicated EB nurse medical records, Genetic Health Service NZ (GHSNZ) records and, where available, public hospital records were manually reviewed for relevant clinical data. RESULTS: Ninety-two participants took part in the study (56% participation rate). Forty-nine (53%) participants had EB simplex (EBS), 40 (43%) had dystrophic EB (DEB), and 3 (3%) had junctional EB (JEB). Point prevalence for EB of all types was 19.5 per million, and 10.4, 8.6 and 0.9 per million for EBS, DEB and JEB, respectively. Thirty-four participants had intermediate or severe EB. There were 29 paediatric cases and almost even numbers of males and females. Compared to NZ European and Maori, prevalence rates were lower for Pacific and Asian people and higher in the Middle Eastern/Latin American/African population. Eight out of 14 skin biopsy results were informative, and 14 of 15 genetic test results were informative. CONCLUSION: New Zealand has similar prevalence rates of EB compared with other national cohorts. This is likely to be an underestimate due to methodological limitations. Recent advancements in genomic testing have resulted in an improved diagnostic rate in our population. Further research into ethnic differences in prevalence, and exploring the characteristics of lethal forms of EB, is warranted. A dynamic registry may be helpful for the EB community in NZ.

Delivery of a psychological intervention to assess and reduce workplace stress among intensive care staff.

INTRODUCTION: Intensive care staff have high levels of stress. We conducted a service improvement initiative to assess workplace stress levels among staff in one adult general intensive care unit and deliver a stress management intervention. METHODS: A psychological intervention of four stress management sessions, and fortnightly staff support drop-in groups, was developed and delivered within a year. Pre- and post-intervention, workplace stress in the unit was assessed using a Health and Safety Executive tool. RESULTS: Pre-intervention assessment of 76 (47.2%) staff indicated that improvement was needed in all domains of workplace stress. 125 staff (77.6%) participated in the intervention and gave positive ratings for content, relevance, practicality and personal value (median 4 (1-5); interquartile range 3.8-4.6). Post-intervention assessment of 71 staff (41.3%) demonstrated improvements in all workplace stress domains. CONCLUSION: A reduction in workplace stress was observed following a service improvement intervention in one intensive care unit although no causality can be assumed. Similar interventions should be evaluated using robust study designs.