EClinicalMedicine (Nov 2023)
Effect of the addition of a mental health specialist for evaluation of undiagnosed patients in centres for rare diseases (ZSE-DUO): a prospective, controlled trial with a two-phase cohort designResearch in context
- Helge Hebestreit,
- Anne-Marie Lapstich,
- Lilly Brandstetter,
- Christian Krauth,
- Jürgen Deckert,
- Kirsten Haas,
- Lisa Pfister,
- Stefanie Witt,
- Christopher Schippers,
- Jan Dieris-Hirche,
- Tim Maisch,
- Oliver Tüscher,
- Lavinia Bârlescu,
- Alexandra Berger,
- Mark Berneburg,
- Vanessa Britz,
- Anna Deibele,
- Holm Graeßner,
- Harald Gündel,
- Gereon Heuft,
- Thomas Lücke,
- Christine Mundlos,
- Julia Quitmann,
- Frank Rutsch,
- Katharina Schubert,
- Jörg Bernhard Schulz,
- Susann Schweiger,
- Cornelia Zeidler,
- Lena Zeltner,
- Martina de Zwaan,
- Federica Akkaya,
- Christine Babka,
- Lisa Bannert,
- Anja Bärsch-Michelmann,
- Leonie Böhm,
- Folke Brinkmann,
- Monika Bullinger,
- Holger Cario,
- Moritz de Greck,
- Klaus-Michael Debatin,
- Katrin Dillmann-Jehn,
- Jutta Eymann,
- Julia Frisch,
- Anja Glode,
- Vega Gödecke,
- Corinna Grasemann,
- Eva Grauer,
- Astrid Haas,
- Lea Haisch,
- Isabell,
- Heinrich,
- Melissa Held,
- Julia Hennermann,
- Stephan Herpertz,
- Anne Herrmann-Werner,
- Julian Hett,
- Peter Heuschmann,
- Bettina Hilbig,
- Laura Holthöfer,
- Christiane Imhof,
- Florian Junne,
- Jan Kassubek,
- Kevin-Thomas Koschitzki,
- Heike Krassort,
- Birgit Kropff,
- Julia Kuhn,
- Philipp Latzko,
- Thomas Loew,
- Albert C. Ludolph,
- Torsten Meyer,
- Isabell Meyer dos Santos,
- Klaus Mohnike,
- Martina Monninger,
- Martin Mücke,
- Susanne Müller,
- Thomas Musacchio,
- Margret Nießen,
- Mariel Nöhre,
- Stephan Ott,
- Andrea Petermann-Meyer,
- Christina Pfeifer-Duck,
- Lea-Sophie Piduhn,
- Carina Rampp,
- Olaf Rieß,
- Kristina Schaubert,
- Annika Schmidt,
- Simone Schneider,
- Ludger Schoels,
- Martina Schwalba,
- Udo Selig,
- Alexandra Sroka,
- Toni Steinbüchel,
- Sebastian Stösser,
- Steffi Suchant,
- Kathrin Ungethüm,
- Matthias Vogel,
- Daniela Volk,
- Christoph Vollmuth,
- Solange Volnov,
- Thomas O.F. Wagner,
- Sabrina Walter,
- Bodo Warrings,
- Kamil Zajt,
- Karola Zenker,
- David Zhang,
- Stephan Zipfel,
- Helge Hebestreit,
- Anne-Marie Lapstich,
- Lilly Brandstetter,
- Christian Krauth,
- Jürgen Deckert,
- Kirsten Haas,
- Lisa Pfister,
- Stefanie Witt,
- Christopher Schippers,
- Jan Dieris-Hirche,
- Tim Maisch,
- Oliver Tüscher,
- Lavinia Aurelia Bârlescu,
- Alexandra Berger,
- Mark Berneburg,
- Vanessa Britz,
- Anna Deibele,
- Holm Graeßner,
- Harald Gündel,
- Gereon Heuft,
- Thomas Lücke,
- Christine Mundlos,
- Julia Hannah Quitmann,
- Frank Rutsch,
- Katharina Schubert,
- Jörg B. Schulz,
- Susann Schweiger,
- Cornelia Zeidler,
- Lena Margarete Zeltner,
- Martina de Zwaan
Affiliations
- Helge Hebestreit
- Centre for Rare Diseases - Reference Centre Northern Bavaria (ZESE), University Hospital Würzburg, Josef-Schneider-Str. 2, 97080, Würzburg, Germany; Corresponding author.
- Anne-Marie Lapstich
- Centre for Health Economics Research Hannover and Institute for Epidemiology, Social Medicine and Health Systems Research, Hannover Medical School, Carl-Neuberg-Strasse 1, 30625, Hannover, Germany
- Lilly Brandstetter
- Institute of Clinical Epidemiology and Biometry, University of Würzburg, Josef-Schneider-Str. 2, 97080, Würzburg, Germany
- Christian Krauth
- Centre for Health Economics Research Hannover and Institute for Epidemiology, Social Medicine and Health Systems Research, Hannover Medical School, Carl-Neuberg-Strasse 1, 30625, Hannover, Germany
- Jürgen Deckert
- Department of Psychiatry, Psychosomatics and Psychotherapy, Centre of Mental Health, University Hospital, Margarete-Höppel-Platz 1, 97080, Würzburg, Germany
- Kirsten Haas
- Institute of Clinical Epidemiology and Biometry, University of Würzburg, Josef-Schneider-Str. 2, 97080, Würzburg, Germany
- Lisa Pfister
- Centre for Rare Diseases - Reference Centre Northern Bavaria (ZESE), University Hospital Würzburg, Josef-Schneider-Str. 2, 97080, Würzburg, Germany
- Stefanie Witt
- Department of Medical Psychology, University Medical Centre Hamburg-Eppendorf, Martinistr. 52, 20246, Hamburg, Germany
- Christopher Schippers
- Center for Rare Diseases and Department of Digitalization and General Practice, RWTH Aachen University Hospital, Pauwelsstraße 30, 52074, Aachen, Germany
- Jan Dieris-Hirche
- Department of Psychosomatic Medicine and Psychotherapy, LWL-University Hospital, Ruhr-University Bochum, Alexandrinenstraße 1-3, 44791, Bochum, Germany
- Tim Maisch
- Centre for Rare Diseases Regensburg, University Hospital Regensburg, Franz-Josef-Strauss-Allee 11, 93053, Regensburg, Germany
- Oliver Tüscher
- Centre for Rare Diseases and Department of Psychiatry and Psychotherapy, University Medical Centre. Johannes Gutenberg University, and Institute for Molecular Biology, 55131, Mainz, Germany
- Lavinia Bârlescu
- Department of Neurology, University of Ulm, Oberer Eselsberg 45, 89081, Ulm, Germany
- Alexandra Berger
- Frankfurt Reference Centre for Rare Diseases, University Hospital Frankfurt, Goethe University Frankfurt, Theodor-Stern-Kai 7, 60590, Frankfurt am Main, Germany
- Mark Berneburg
- Centre for Rare Diseases Regensburg, University Hospital Regensburg, Franz-Josef-Strauss-Allee 11, 93053, Regensburg, Germany
- Vanessa Britz
- Frankfurt Reference Centre for Rare Diseases, University Hospital Frankfurt, Goethe University Frankfurt, Theodor-Stern-Kai 7, 60590, Frankfurt am Main, Germany
- Anna Deibele
- Central German Competence Network for Rare Diseases, University Hospital Magdeburg, Leipziger Straße 44, 39120, Magdeburg, Germany
- Holm Graeßner
- Centre for Rare Disease, University Hospital Tübingen, Calwerstr. 7, 72076, Tübingen, Germany
- Harald Gündel
- Department of Psychosomatic Medicine and Psychotherapy, University Hospital Ulm, Albert.-Einstein-Allee 23, 89081, Ulm, Germany
- Gereon Heuft
- Department of Psychosomatic Medicine Und Psychotherapy, University Hospital Münster, Albert-Schweitzer-Campus 1, 48149, Münster, Germany
- Thomas Lücke
- University Hospital of Paediatrics and Adolescent Medicine, Ruhr-University Bochum, Alexandrinenstr. 5, 44791, Bochum, Germany
- Christine Mundlos
- Allianz Chronischer Seltener Erkrankungen (ACHSE) e.V., c/o DRK Kliniken Berlin Mitte, Dronheimer Str. 39, 13359, Berlin, Germany
- Julia Quitmann
- Department of Medical Psychology, University Medical Centre Hamburg-Eppendorf, Martinistr. 52, 20246, Hamburg, Germany
- Frank Rutsch
- Department of General Paediatrics, Muenster University Children's Hospital and Centre for Rare Diseases, Muenster University Hospital, Albert-Schweitzer-Campus 1, Gebäude A1, 48149, Münster, Germany
- Katharina Schubert
- Central German Competence Network for Rare Diseases, University Hospital Magdeburg, Leipziger Straße 44, 39120, Magdeburg, Germany
- Jörg Bernhard Schulz
- Department of Neurology, RWTH Aachen University, Pauwelsstraße 30, 52074, Aachen, Germany
- Susann Schweiger
- Centre for Rare Diseases and Institute for Human Genetics, University Medical Centre, Johannes Gutenberg University, and Institute for Molecular Biology, 55131, Mainz, Germany
- Cornelia Zeidler
- Centre for Rare Diseases, Hannover Medical School, Carl-Neuberg-Strasse 1, 30625, Hannover, Germany
- Lena Zeltner
- Department of Psychosomatics and Psychotherapy, University Hospital Tübingen, Osianderstraße 5, 72076, Tübingen, Germany
- Martina de Zwaan
- Department of Psychosomatic Medicine and Psychotherapy, Hannover Medical School, Carl-Neuberg-Strasse 1, 30625, Hannover, Germany
- Federica Akkaya
- Christine Babka
- Lisa Bannert
- Anja Bärsch-Michelmann
- Leonie Böhm
- Folke Brinkmann
- Monika Bullinger
- Holger Cario
- Moritz de Greck
- Klaus-Michael Debatin
- Katrin Dillmann-Jehn
- Jutta Eymann
- Julia Frisch
- Anja Glode
- Vega Gödecke
- Corinna Grasemann
- Eva Grauer
- Astrid Haas
- Lea Haisch
- Isabell
- Heinrich
- Melissa Held
- Julia Hennermann
- Stephan Herpertz
- Anne Herrmann-Werner
- Julian Hett
- Peter Heuschmann
- Bettina Hilbig
- Laura Holthöfer
- Christiane Imhof
- Florian Junne
- Jan Kassubek
- Kevin-Thomas Koschitzki
- Heike Krassort
- Birgit Kropff
- Julia Kuhn
- Philipp Latzko
- Thomas Loew
- Albert C. Ludolph
- Torsten Meyer
- Isabell Meyer dos Santos
- Klaus Mohnike
- Martina Monninger
- Martin Mücke
- Susanne Müller
- Thomas Musacchio
- Margret Nießen
- Mariel Nöhre
- Stephan Ott
- Andrea Petermann-Meyer
- Christina Pfeifer-Duck
- Lea-Sophie Piduhn
- Carina Rampp
- Olaf Rieß
- Kristina Schaubert
- Annika Schmidt
- Simone Schneider
- Ludger Schoels
- Martina Schwalba
- Udo Selig
- Alexandra Sroka
- Toni Steinbüchel
- Sebastian Stösser
- Steffi Suchant
- Kathrin Ungethüm
- Matthias Vogel
- Daniela Volk
- Christoph Vollmuth
- Solange Volnov
- Thomas O.F. Wagner
- Sabrina Walter
- Bodo Warrings
- Kamil Zajt
- Karola Zenker
- David Zhang
- Stephan Zipfel
- Helge Hebestreit
- Anne-Marie Lapstich
- Lilly Brandstetter
- Christian Krauth
- Jürgen Deckert
- Kirsten Haas
- Lisa Pfister
- Stefanie Witt
- Christopher Schippers
- Jan Dieris-Hirche
- Tim Maisch
- Oliver Tüscher
- Lavinia Aurelia Bârlescu
- Alexandra Berger
- Mark Berneburg
- Vanessa Britz
- Anna Deibele
- Holm Graeßner
- Harald Gündel
- Gereon Heuft
- Thomas Lücke
- Christine Mundlos
- Julia Hannah Quitmann
- Frank Rutsch
- Katharina Schubert
- Jörg B. Schulz
- Susann Schweiger
- Cornelia Zeidler
- Lena Margarete Zeltner
- Martina de Zwaan
- Journal volume & issue
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Vol. 65
p. 102260
Abstract
Summary: Background: People with complex symptomatology but unclear diagnosis presenting to a centre for rare diseases (CRD) may present with mental (co-)morbidity. We hypothesised that combining an expert in somatic medicine with a mental health specialist working in tandem will improve the diagnostic outcome. Methods: Patients aged 12 years and older who presented to one of the 11 participating German CRDs with an unknown diagnosis were recruited into this prospective cohort trial with a two-phase cohort design. From October 1, 2018 to September 30, 2019, participants were allocated to standard care (SC, N = 684), and from October 1, 2019 to January 31, 2021 to innovative care (IC, N = 695). The cohorts consisted mainly of adult participants with only a minority of children included (N = 67). IC included the involvement of a mental health specialist in all aspects of care (e.g., assessing medical records, clinic visits, telehealth care, and case conferences). Clinicaltrials.gov identifier: NCT03563677. Findings: The proportion of patients with diagnoses established within 12 months after the first visit to the CRD explaining the entire symptomatology (primary outcome) was 19% (N = 131 of 672) in the SC and 42% (N = 286 of 686) in the IC cohort (OR adjusted for centre effects 3.45 [95% CrI: 1.99–5.65]). The difference was mainly due to a higher prevalence of mental disorders and non-rare somatic diseases in the IC cohort. The median time to explaining diagnoses was one month shorter with IC (95% CrI: 1–2), and significantly more patients could be referred to local regular care in the IC (27.5%; N = 181 of 659) compared to the SC (12.3%; N = 81 of 658) cohort (OR adjusted for centre effects 2.70 [95% CrI: 2.02–3.60]). At 12-month follow-up, patient satisfaction with care was significantly higher in the IC compared to the SC cohort, while quality of life was not different between cohorts. Interpretation: Our findings suggested that including a mental health specialist in the entire evaluation process of CRDs for undiagnosed adolescents and adults should become an integral part of the assessment of individuals with a suspected rare disease. Funding: The study was funded by the Global Innovation Fund from the Joint Federal Committee in Germany (Innovationsfonds des Gemeinsamen Bundesausschusses), grant number 01NVF17031.