Subscribe to RSS
DOI: 10.1055/s-0032-1301396
The NFTI-QOL: A Disease-Specific Quality of Life Questionnaire for Neurofibromatosis 2
Publication History
19 June 2011
11 October 2011
Publication Date:
06 February 2012 (online)
Abstract
The objective of this study was to develop a reliable, validated disease-specific score measuring quality of life (QOL) in clinical practice and treatment trials in Neurofibromatosis 2 (NF2) individuals. In NF2 patients, qualitative interviews (n = 15) and a focus group session (n = 30) generated items for a pilot questionnaire. This was tested and refined (n = 20). The final version (NFTI-QOL) was validated (n = 50) with two generic QOL questionnaires (SF-36 and EuroQOL). The NFTI-QOL was also administered to patients with solitary vestibular schwannoma (SVS) (n = 30) and normal controls (n = 30). The participants were NF2 patients, SVS patients, and normal controls. NFTI-QOL score, SF-36 score, and EuroQOL score were the main outcome measures. Mean NFTI-QOL score was 9.4 (range: 0 to 20, maximum possible score = 24). The NFTI-QOL score correlated strongly with EuroQOL (r = 0.71, p < 0.001) and SF-36 (r = 0.81, p < 0.001). NF2 individuals were significantly worse than the SVS patients, who in turn were worse than the controls on the NIFTI-QOL. The NFTI-QOL showed good internal reliability (Cronbach's α = 0.87). We developed an eight-item disease-specific QOL score for NF2 patients, validated against SF-36 and EuroQOL. It correlated strongly with clinician-rated disease severity in NF2, with better correlation than the SF-36 in this regard.
-
References
- 1 Evans DGR, Howard E, Giblin C , et al. Birth incidence and prevalence of tumor-prone syndromes: estimates from a UK family genetic register service. Am J Med Genet A 2010; 152A (2) 327-332
- 2 Ferner RE. Neurofibromatosis 1 and neurofibromatosis 2: a twenty first century perspective. Lancet Neurol 2007; 6 (4) 340-351
- 3 Baser ME, Friedman JM, Wallace AJ, Ramsden RT, Joe H, Evans DG. Evaluation of clinical diagnostic criteria for neurofibromatosis 2. Neurology 2002; 59 (11) 1759-1765
- 4 Plotkin SR, Stemmer-Rachamimov AO, Barker II FG , et al. Hearing improvement after bevacizumab in patients with neurofibromatosis type 2. N Engl J Med 2009; 361 (4) 358-367
- 5 Mautner VF, Nguyen R, Kutta H , et al. Bevacizumab induces regression of vestibular schwannomas in patients with neurofibromatosis type 2. Neuro-oncol 2010; 12 (1) 14-18
- 6 Ammoun S, Flaiz C, Ristic N, Schuldt J, Hanemann CO. Dissecting and targeting the growth factor-dependent and growth factor-independent extracellular signal-regulated kinase pathway in human schwannoma. Cancer Res 2008; 68 (13) 5236-5245
- 7 Fayers PM, Machin D. Quality of Life. 2nd ed. Wiley; 2007
- 8 Neary WJ, Hillier VF, Flute T, Stephens SD, Ramsden RT, Evans DG. The relationship between patients' perception of the effects of neurofibromatosis type 2 and the domains of the Short Form-36. Clin Otolaryngol 2010; 35 (4) 291-299
- 9 Patel CM, Ferner R, Grunfeld EA. A qualitative study of the impact of living with neurofibromatosis type 2. Psychol Health Med 2011; 16 (1) 19-28
- 10 Ware JE, Snow KK, Kosinski M, Gandek B. SF-36 Health survey. Manual & Interpretation Guide 1993;
- 11 The Euroqol Group. EuroQol—a new facility for the measurement of health-related quality of life. The EuroQol Group. Health Policy 1990; 16 (3) 199-208
- 12 Kissil JL, Blakeley JO, Ferner RE , et al. What's new in neurofibromatosis? Proceedings from the 2009 NF Conference: new frontiers. Am J Med Genet A 2010; 152A (2, Issue 2) 269-283
- 13 Kind P, Dolan P, Gudex C, Williams A. Variations in population health status: results from a United Kingdom national questionnaire survey. BMJ 1998; 316 (7133) 736-741
- 14 Jenkinson C, Layte R, Wright L, Coulter A. The UK SF-36: An Analysis and Interpretation Manual. University of Oxford; 1996
- 15 Bronstein AM, Golding JF, Gresty MA , et al. The social impact of dizziness in London and Siena. J Neurol 2010; 257 (2) 183-190