Back to Journals » Journal of Pain Research » Volume 12

Pain symptomology, functional impact, and treatment of people with Neurofibromatosis type 1

Authors Buono FD, Grau LE, Sprong ME, Morford KL, Johnson KJ, Gutmann DH

Received 23 March 2019

Accepted for publication 6 August 2019

Published 22 August 2019 Volume 2019:12 Pages 2555—2561


Checked for plagiarism Yes

Review by Single anonymous peer review

Peer reviewer comments 2

Editor who approved publication: Dr E Alfonso Romero-Sandoval

Frank D Buono,1 Lauretta E Grau,2 Matthew E Sprong,3 Kenneth L Morford,4 Kimberly J Johnson,5 David H Gutmann6

1Department of Psychiatry, Yale School of Medicine, New Haven, CT, USA; 2Department of Epidemiology of Microbial Diseases, Yale School of Public Health, New Haven, CT, USA; 3Northern Illinois University, Dekalb, IL, USA; 4Department of Internal Medicine, Yale School of Medicine, New Haven, CT, USA; 5Institute of Public Health, Brown School, Washington University in St Louis, St Louis, MO, USA; 6Department of Neurology, Washington University School of Medicine, St. Louis, MO, USA

Correspondence: Frank D Buono
Department of Psychiatry, Yale School of Medicine, 300 George Street, New Haven, CT 06510, USA
Tel +1 203 801 3499
Fax +1 203 801 3442

Introduction: Neurofibromatosis type 1 (NF1) is a neurogenetic disorder affecting 1 in 3000 people worldwide, where individuals are prone to develop benign and malignant tumors. In addition, many people with NF1 complain of pain that limits their daily functioning. Due to the complexity of the disorder, there are few options for treating pain symptoms besides surgery and medications. Moreover, the spectrum of pain symptomatology and treatment, as well as the mechanisms underlying NF1-associated pain, has been understudied.
Methodology: To address this knowledge gap, we conducted a survey of 255 adults with NF1, leveraging the Washington University NF1 Patient Registry Initiative (NPRI) database. Demographic and pain data were collected using a Qualtrics survey.
Results: All participants had at least one surgical procedure, with 55% reporting having at least one surgery within the last year and 17% being currently prescribed opioid medication. A positive relationship was shown (p<0.001) between those prescribed prescription pain medication, and their pain severity and interference. Moreover, there was a significant relationship (p=0.049) between the usage of complementary treatments and pain severity and interference.
Conclusion: The current study demonstrates that individuals with NF1 report a higher incidence of pain severity and interference than observed in NF1 previous studies, with pain symptoms not localized to any specific region of the body. The consideration for alternative treatments and careful monitoring of current treatments that are more conservative or have less potential adverse side effects may improve pain management and reduce the risk of developing medication dependence.

Keywords: Neurofibromatosis type 1, complementary treatment, adults, chronic pain

Creative Commons License This work is published and licensed by Dove Medical Press Limited. The full terms of this license are available at and incorporate the Creative Commons Attribution - Non Commercial (unported, v3.0) License. By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed. For permission for commercial use of this work, please see paragraphs 4.2 and 5 of our Terms.

Download Article [PDF]  View Full Text [HTML][Machine readable]