A step-wise approach for establishing a multidisciplinary team for the management of tuberous sclerosis complex: a Delphi consensus report
Por:
Auvin, S, Bissler, JJ, Cottin, V, Fujimoto, A, Hofbauer, GFL, Jansen, AC, Jozwiak, S, Kerecuk, L, Kingswood, JC, Moavero, R, Torra, R, Villanueva, V
Publicada:
30 abr 2019
Resumen:
BackgroundTuberous 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.MethodsTo 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.ResultsA 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.ConclusionsThe results of our consensus provide guidance to support the initiation of an MDT in TSC.
Filiaciones:
Auvin, S:
Robert Debre Childrens Hosp, AP HP, Serv Neurol Pediat & Malad Metabol, 48 Blvd Serurier, F-75019 Paris 19, France
INSERM, U1141, Paris, France
Bissler, JJ:
St Jude Childrens Res Hosp, 848 Adams Ave, Memphis, TN 38103 USA
Le Bonheur Childrens Hosp, 848 Adams Ave, Memphis, TN 38103 USA
Cottin, V:
Claude Bernard Univ Lyon 1, Louis Pradel Hosp, Reference Ctr Rare Pulm Dis, UMR754, F-69677 Lyon, France
Fujimoto, A:
Seirei Hamamatsu Gen Hosp, Naka Ward, 2 Chome 12-12 Sumiyoshi, Hamamatsu, Shizuoka, Japan
Hofbauer, GFL:
Univ Hosp Zurich, Ramistr 100, CH-8091 Zurich, Switzerland
Jansen, AC:
Vrije Univ Brussel, UZ Brussel, Laarbeeklaan 101, B-1090 Brussels, Belgium
Jozwiak, S:
Warsaw Med Univ, Dept Child Neurol, Banacha 1, PL-02097 Warsaw, Poland
Childrens Mem Hlth Inst, Dept Neurol & Epileptol, Warsaw, Poland
Kerecuk, L:
Birmingham Womens & Childrens NHS Fdn Trust, Birmingham B15 2TG, W Midlands, England
Kingswood, JC:
St George Hosp, Blackshaw Rd, London SW17 0QT, England
Moavero, R:
Tor Vergata Univ Hosp, Via Columbia 2, I-00133 Rome, Italy
Bambino Gesu Pediat Hosp, IRCCS, Rome, Italy
Torra, R:
Univ Autonoma Barcelona, Fundacio Puigvert, REDINREN, Carrer Cartagena 340-350, Barcelona 08025, Spain
Villanueva, V:
Hosp Univ & Politecn La Fe, Avinguda Fernando Abril Martorell 106, Valencia 46026, Spain
Gold, Green Published, Green Accepted
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