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A step-wise approach for establishing a multidisciplinary team for the management of tuberous sclerosis complex: a Delphi consensus report.

Auvin, S; Bissler, JJ; Cottin, V; Fujimoto, A; Hofbauer, GFL; Jansen, AC; Jóźwiak, S; Kerecuk, L; Kingswood, JC; Moavero, R; et al. Auvin, S; Bissler, JJ; Cottin, V; Fujimoto, A; Hofbauer, GFL; Jansen, AC; Jóźwiak, S; Kerecuk, L; Kingswood, JC; Moavero, R; Torra, R; Villanueva, V (2019) A step-wise approach for establishing a multidisciplinary team for the management of tuberous sclerosis complex: a Delphi consensus report. Orphanet J Rare Dis, 14 (1). p. 91. ISSN 1750-1172 https://doi.org/10.1186/s13023-019-1072-y
SGUL Authors: Kingswood, John Christopher

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Abstract

BACKGROUND: Tuberous sclerosis complex (TSC) is a rare autosomal dominant genetic disorder associated with mutations in TSC1 and TSC2 genes, upregulation of mammalian target of rapamycin signaling, and subsequent tumor formation in various organs. Due to the many manifestations of TSC and their potential complications, management requires the expertise of multiple medical disciplines. A multidisciplinary care approach is recommended by consensus guidelines. Use of multidisciplinary teams (MDTs) has been shown to be beneficial in treating other complex diseases, such as cancer. In a lifelong disease such as TSC, an MDT may facilitate the transition from pediatric to adult care. However, little guidance exists in the literature regarding how to organize an MDT in TSC. METHODS: To discuss the best approach to assembling an MDT, this project was initiated in October 2017 with a meeting of 12 physicians from various specialties and various countries. Following this first meeting, the experts generated statements on the most important aspects to implement in establishing an MDT for TSC by 3 rounds of selection using a Delphi process via electronic correspondence. Finally, TSC patient advocates reviewed the findings and provided additional insights from a patient perspective. RESULTS: A 3-step roadmap was recommended, starting with identifying a single individual to begin organizing care (Step 1), then establishing a small core team (Step 2), and finally, establishing a larger multi-disciplinary team (Step 3). Because of the multisystemic nature of TSC, the MDT should include specialists such as a neurologist, a neurosurgeon, a nephrologist, a urologist, a pulmonologist, an ophthalmologist, a cardiologist, a dermatologist, a geneticist, and a psychiatrist/psychologist. The MDT should recommend a care plan for each patient based on the individual's needs and in consultation with him/her or his/her family. Some of the most important aspects of an MDT that were agreed upon included identifying a case manager to help coordinate care, providing access to health care professionals of varying specialties, and including a lead physician who takes medical responsibility for patients' overall care. CONCLUSIONS: The results of our consensus provide guidance to support the initiation of an MDT in TSC.

Item Type: Article
Additional Information: © The Author(s). 2019 Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
Keywords: Multidisciplinary care, Multidisciplinary team, Tuberous sclerosis complex, Tuberous sclerosis complex, Multidisciplinary care, Multidisciplinary team, 1199 Other Medical And Health Sciences, Genetics & Heredity
SGUL Research Institute / Research Centre: Academic Structure > Molecular and Clinical Sciences Research Institute (MCS)
Journal or Publication Title: Orphanet J Rare Dis
ISSN: 1750-1172
Language: eng
Dates:
DateEvent
30 April 2019Published
17 April 2019Accepted
Publisher License: Creative Commons: Attribution 4.0
PubMed ID: 31039793
Web of Science ID: WOS:000466907100001
Go to PubMed abstract
URI: http://sgultest.da.ulcc.ac.uk/id/eprint/110898
Publisher's version: https://doi.org/10.1186/s13023-019-1072-y

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