Quality of life of patients with neurofibromatosis 1 – physical disability does not necessarily result in inferior mental health.

Abstract

Abstract Introduction Neurofibromatosis 1 (NF1) is a chronic neurocutaneous disease with tremendous impact on quality of life (QoL). We have performed an analysis of disease severity, mental and physical QoL and compared the different severity classes of patients with neurofibromatosis 1 (NF1). Patients and methods We performed a prospective analysis of 89 patients with NF-1 which are included in the period of 1/2016 - 3/2018. Local records served as data source including demographic data, employment status, level of education, and marital status were evaluated. All patients received 36-Item Short Form Health Survey (SF-36). Additionally, numerical pain rating scale (NPS) Patients were stratified according to different severity of NF-1 and different visibility and severity of disease. Results: Out of 89 patients, severity grad 4 was identified in 42 (47.2%), moderate in 17 (19.1%), mild in 23 (25.8%) and minimal in 7 (7.9%) cases. According to visibility scale, severe grade 3 was found in 28 (31.5%), moderate grade 2 in 26 (29.2%) and mild grade in 35 (39.3%) cases. All SF-36 related data except for pain showed significantly lower values, if compared to the standard German population (p<0.001, physical component summary p=0.045). Sex, marital status and level of education showed no significant differences. Employment was significantly associated with better mental and physical status (p=0.028 and p=0.01 respectively) and age >40 was significantly associated with lower physical (p=0.027) but not mental component (p=0.362). According to the numerical pain rating scale, 7-10 were noted in 9 cases (10,1%), 5-6 in 10 patients (11.2%), 1-4 in 26 patients (29.2%) and no pain in 44 cases (49.4%). Physical component showed significant difference between different NPS grades (p<0.001) but no significance in mental component summery (p=0.06). Finally, we found no significant difference between different severity grades and visibility grades in mental component summery. Conclusion: The severity grade and visibility grade of patients with NF-1 does not necessarily results in decreased mental components and physical limitations in comparison with lower grades, so that symptomatic treatment should be considered even in patients with severe disability, since these still may have comparable QoL to less severe disabled patients with NF-1. Employment was associated with better QoL according to our results.