Longitudinal evaluation of quality of life in 288 patients with Neurofibromatosis 2

Advances in molecular biology have resulted in novel therapy for neurofibromatosis 2-related (NF2) tumours, highlighting the need for robust outcome measures. The disease-focused NF2 impact on quality of life (NFTI-QOL) patient questionnaire was assessed as an outcome measure for treatment in a multi-centre study. NFTI-QOL was related to clinician-rated severity (ClinSev) and genetic severity (GenSev) over repeated visits. Data were evaluated for 288 NF2 patients (n = 464 visits) attending the English national NF2 clinics from 2010 to 2012. The male-to-female ratio was equal and the mean age was 42.2 (SD 17.8) years. The analysis included NFTI-QOL eight-item score, ClinSev graded as mild, moderate, or severe, and GenSev as a rank order of the number of NF2 mutations (graded as mild, moderate, severe). The mean (SD) 8.7 (5.4) score for NFTI-QOL for either a first visit or all visits 9.2 (5.4) was similar to the published norm of 9.4 (5.5), with no significant relationships with age or gender. NFTI-QOL internal reliability was good, with a Cronbach’s alpha score of 0.85 and test re-test reliability r = 0.84. NFTI related to ClinSev (r = 0.41, p < 0.001; r = 0.46 for all visits), but weakly to GenSev (r = 0.16, p < 0.05; r = 0.15 for all visits). ClinSev related to GenSev (r = 0.41, p < 0.001; r = 0.42 for all visits). NFTI-QOL showed a good reliability and ability to detect significant longitudinal changes in the QOL of individuals. The moderate relationships of NFTI-QOL with clinician- and genetic-rated severity suggest that NFTI-QOL taps into NF2 patient experiences that are not encompassed by ClinSev rating or genotype.

Managing the transition to work: the role of the planning network in British town planning education

The development of town planning education in the United Kingdom can be traced back over at least sixty years and has always enjoyed a close relationship with practitioners, employers and the professional body, the Royal Town Planning Institute (RTPI). In order to ensure an intake of sufficient quality to a growing profession, the Institute offered its own exams until the 1980s and then initiated the current system of accrediting both undergraduate and postgraduate programmes of study. This system of accreditation emphasises the importance of relevant knowledge, skills and values as well as core and specialised studies. The vocational nature of town planning requires that graduates have the breadth of understanding as well as the practical skills in order to practice effectively. Thus accredited courses have over time developed strong links with employers and practitioners. Rapid developments in the scope and range of planning, and the skills needed to work in it, have reflected changes in public policy and growing number of agencies concerned with the built environment. The system of regular quinquennial visits to accredit courses has helped ensure that this acceptance of change has become part of the culture of planning schools.