Development and Preliminary Evaluation of a Quality‐of‐Life Questionnaire for Adults with Neurofibromatosis Type 1 (NF1‐AdQOL)

2021 ◽  
Author(s):  
H. Crawford ◽  
K. North ◽  
M.J. Wilson ◽  
Y. Berman ◽  
V. McKelvey‐Martin ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Preliminary Evaluation ◽  
Life Questionnaire ◽  
Quality Of Life Questionnaire

scholarly journals Executive functions and quality of life in children with neurofibromatosis type 1

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Arnaud Roy ◽  
Jean-Luc Roulin ◽  
Christèle Gras-Le Guen ◽  
Marie-Laure Corbat ◽  
Sébastien Barbarot
Keyword(s):  
Quality Of Life ◽  
Executive Function ◽  
Executive Functions ◽  
Neurofibromatosis Type 1 ◽  
School Environment ◽  
Daily Life ◽  
Neurofibromatosis Type ◽  
Life Questionnaire

Abstract Background To examine the impact of executive function disorders on health-related quality of life (QoL) in children with neurofibromatosis type 1 (NF1), we conducted a prospective single-center study among 40 children with NF1 aged 8–12 years (mean = 9.7, SD = 1.4) and their parents, comparing them with 56 healthy control children matched for age, sex, parental education level, and handedness. We collected children’s self-reports and parents’ proxy reports of QoL with the Kidscreen-52 questionnaire, and measured executive functions by combining seven performance-based tests and a daily life questionnaire completed by parents and teachers. Results Several QoL domains were significantly impaired in the children with NF1, compared with healthy controls, mainly according to their parents’ reports (3 out of 9 scales; Cohen’s d: 0.57–0.76), with particularly low scores in the social support and peers and school environment domains. Executive function difficulties (Cohen’s d: 0.64–1.72) significantly predicted the impairment of QoL domains as perceived by the children or their parents, regardless of the indirect indicators of learning disabilities. Conclusions Both performance-based executive function scores and behavioral ratings of executive functions in daily life by parents and teachers were associated with low QoL levels in the children with NF1. The school environment and social integration appear to be particularly affected and should therefore be targeted in the management of the disease.

scholarly journals A Cross-Sectional Study of Gender Differences in Quality of Life Domains in Patients With Neurofibromatosis Type 1

2021 ◽  
Author(s):  
Geohana Hamoy-Jimenez ◽  
Hadiya Elahmar ◽  
Meg Mendoza ◽  
Raymond Kim ◽  
Vera Bril ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Neurofibromatosis Type 1 ◽  
Physical Appearance ◽  
Neurofibromatosis Type ◽  
Cross Sectional Study ◽  
Cross Sectional ◽  
Appearance Anxiety ◽  
Sf 36

Abstract Background. There is limited data regarding gender differences in quality of life between women and men with Neurofibromatosis type 1. We aimed to study differences in quality of life domains between women and men with Neurofibromatosis type 1 living in Canada.Methods: This is a cross sectional study of adults with Neurofibromatosis type 1 attending a tertiary NF centre at Toronto General Hospital between January 2016 to December 2017. Demographic and clinical data were collected. We compared scores of generic measures (SF-36, EQ-5D-5L, pain interference) and a disease-specific measure (PedsQL-NF1 module) between women and men. We also assessed the relationship between disease visibility scored by an examiner (Ablon’s visibility index) and self-reported perceived physical appearance, stratified by gender.Results. One hundred and sixty-two participants were enrolled, 92 females and 70 males. Ablon’s index score 1 was in 43% and score 2 in 44%, while only 13% of patients had a score 3. Women had worse scores on the total PedsQL-NF1 scales, and also in the perceived physical appearance, anxiety and emotional health domains. In women, there was a significant association between Ablon’s index and perceived physical appearance, with lower scores with increments in Ablon’s class (ANOVA p<0.001). In men, there was no difference in self-reported physical appearance by Ablon’s index. There were no differences between men and women in the SF-36 or EQ-5D-5L scores.Conclusion. Women with NF1 reported worse NF1-related quality of life than men, with worse perceived physical appearance, anxiety, and mental health. Perceived physical appearance did not always correlate to disease visibility; therefore, healthcare providers should inquire about body image, physical appearance concerns, and mental health, especially among women with NF1.

scholarly journals Quality of life in patients with neurofibromatosis type 1 and 2 in Canada

2020 ◽  
Vol 2 (Supplement_1) ◽  
pp. i141-i149
Author(s):  
Geohana Hamoy-Jimenez ◽  
Raymond Kim ◽  
Suganth Suppiah ◽  
Gelareh Zadeh ◽  
Vera Bril ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Neurofibromatosis Type 1 ◽  
Physical Appearance ◽  
Well Being ◽  
Neurofibromatosis Type ◽  
Pain Interference ◽  
Sf 36 ◽  
Tertiary Center

Abstract Background There is scarce data on the quality of life of people with neurofibromatosis type 1 (NF1) and type 2 (NF2) in Canada. Methods A cross-sectional study of adults with NF1 and NF2 attending a tertiary center. Patients completed generic measures (SF-36, EQ-5D-5L, and PROMIS pain interference) and disease-specific questionnaires (PedsQL NF1 module and the NFTI-QOL for NF2). We compared generic scores between NF1 and NF2 individuals and used regression models to assess factors associated with quality of life. Results Hundred and eighty-four participants were enrolled. Mean age was 33 years in NF1 and 40 years in NF2. NF1 and NF2 individuals had lower employment rates and lower scores in all domains of the SF-36 compared to the general Canadian population (P &lt; .005). Using the EQ-5D-5L, there was a high proportion of pain (64% in NF1 and 74% in NF2) and anxiety/depression (60% in NF1 and 68% in NF2). Pain interference correlated with poor quality of life in NF1 and NF2; perceived physical appearance was the main predictor of mental well-being in NF1. Conclusions Individuals with NF1 and NF2 have low quality of life, and this correlates with pain, anxiety, and depression, which are prevalent in NF1 and NF2. Perceived physical appearance predicts quality of life in NF1. A multidisciplinary approach is necessary for patients with NF1 and NF2, including mental health and pain management.

Parental reports of health-related quality of life in greek children with neurofibromatosis type 1

2009 ◽  
Vol 155 (3) ◽  
pp. 453 ◽  
Author(s):  
Athanasios Vardarinos ◽  
Dimitrios I. Zafeiriou ◽  
Euthymia Vargiami ◽  
Polyxeni Pratsidou-Gertsi ◽  
Eleutherios Kontopoulos ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Health Related Quality ◽  
Related Quality ◽  
Health Related ◽  
Parental Reports

scholarly journals Depression among adults with neurofibromatosis type 1: prevalence and impact on quality of life

2015 ◽  
Vol 88 (5) ◽  
pp. 425-430 ◽  
Author(s):  
J.S. Cohen ◽  
H.P. Levy ◽  
J. Sloan ◽  
J. Dariotis ◽  
B.B. Biesecker
Keyword(s):  
Quality Of Life ◽  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type

scholarly journals Executive functions and quality of life in children with neurofibromatosis type 1

2021 ◽  
Author(s):  
Arnaud Roy ◽  
Jean-Luc Roulin ◽  
Christèle Gras-Le Guen ◽  
Marie-Laure Corbat ◽  
Sébastien Barbarot
Keyword(s):  
Quality Of Life ◽  
Executive Function ◽  
Executive Functions ◽  
Neurofibromatosis Type 1 ◽  
School Environment ◽  
Daily Life ◽  
Neurofibromatosis Type ◽  
Self Reports

Abstract Background. To examine the impact of executive function disorders on health-related quality of life (QoL) in children with neurofibromatosis type 1 (NF1). Prospective single-center study among 40 children with NF1 aged 812 years (mean = 9.7, SD = 1.4) and their parents, comparing them with 56 healthy control children matched for age, sex, parental education level, and handedness. We collected children’s self-reports and parents’ proxy reports of QoL with the Kidscreen-52 questionnaire, and measured executive functions by combining seven performance-based tests and a daily life questionnaire completed by parents and teachers. Results. Several QoL domains were significantly impaired in the children with NF1, compared with healthy controls, according to both their self-reports (3 out of 9 scales; Cohen’s d: .40.42) and their parents’ reports (6 out of 9 scales; Cohen’s d: .34.75), with a systematic decrease in the social support and peers and school environment domains. Executive function disorders (Cohen’s d: .641.72) significantly predicted the impairment of QoL domains as perceived by the children or their parents, regardless of the indirect indicators of learning disabilities. Conclusions. Both performance-based executive function scores and behavior ratings of executive functions in daily life by parents and teachers were associated with low QoL levels in the children with NF1. The school environment and social integration appear to be particularly affected, and should therefore be targeted in the management of the disease. 227 words

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