کیفیت زندگی در گروهی از بیماران ایرانی مبتلا به نوروفیبروماتوز نوع یک، با تاکید بر مشکلات پوستی

Background and purpose: Neurofibromatosis type 1 (NF1) is a dominant genetic disorder of the skin and the nervous system that is seen in equal proportions in both sexes. The incidence of NF1 is about one in every 3,000. Chronic skin diseases have a significant impact on the individual's mental image and also affect mental health and quality of life. The aim of this study was to investigate the quality of life in a group of Iranian patients with NF1 suffering from skin problems. Materials and methods: A cross-sectional, descriptive-analytical study was conducted in 2015-2017 in 193 patients with NF1 attending the Iranian Neurofibromatosis Society, using convenient sampling. Data were collected by Skindex-16 quality of life measure which is composed of 16 questions in three dimensions (emotions, symptoms, and performance). Data were analyzed using SPSS applying Pearson Correlation and ANOVA. Results: Of 193 patients with NF1, 69.5% (n=132) were females. The mean age of patients was 31.58±9.21. The mean score for quality of life was 44.88±22.06. In the domains of symptoms, emotions, and performance the mean scores were 6.57±4.99, 24.76±11.64, and 13.64±8.86, respectively. Quality of life was not statistically correlated with age and sex, but the relationship between the domain of symptoms with age was found to be significant (P=0.01). Conclusion: The results showed that patients with neurofibromatosis have moderate quality of life and lower quality in terms of emotions and performance. These findings can be used to provide better health care for this group of patients and improve their quality of life.