Laurent M Willems1, Susanne Schubert-Bast2, Janina Grau1, Christoph Hertzberg3, Gerhard Kurlemann4, Adelheid Wiemer-Kruel5, Thomas Bast5, Astrid Bertsche6, Ulrich Bettendorf7, Barbara Fiedler8, Andreas Hahn9, Hans Hartmann10, Frauke Hornemann11, Ilka Immisch12, Julia Jacobs13, Matthias Kieslich14, Karl Martin Klein15, Kerstin A Klotz16, Gerhard Kluger17, Markus Knuf18, Thomas Mayer19, Klaus Marquard20, Sascha Meyer21, Hiltrud Muhle22, Karen Müller-Schlüter23, Anna H Noda1, Susanne Ruf24, Matthias Sauter25, Jan-Ulrich Schlump26, Steffen Syrbe27, Charlotte Thiels28, Regina Trollmann29, Bernd Wilken30, Johann Philipp Zöllner1, Felix Rosenow1, Adam Strzelczyk31. 1. Epilepsy Center Frankfurt Rhine-Main and Department of Neurology, Goethe-University Frankfurt, Frankfurt am Main, Germany; Center for Personalized Translational Epilepsy Research (CePTER), Goethe-University Frankfurt, Frankfurt am Main, Germany. 2. Epilepsy Center Frankfurt Rhine-Main and Department of Neurology, Goethe-University Frankfurt, Frankfurt am Main, Germany; Center for Personalized Translational Epilepsy Research (CePTER), Goethe-University Frankfurt, Frankfurt am Main, Germany; Department of Neuropediatrics, Goethe-University Frankfurt, Frankfurt am Main, Germany. 3. Department of Neuropediatrics, Vivantes Klinikum Neukölln, Berlin, Germany. 4. St. Bonifatius Hospital, Lingen, Germany. 5. Epilepsy Center Kork, Clinic for Children and Adolescents, Kehl-Kork, Germany. 6. Department of Neuropediatrics, University Hospital for Children and Adolescents, Rostock, Germany. 7. Neuropediatric Practice, Hirschaid, Germany. 8. Department of General Pediatrics, Division of Neuropediatrics, University Hospital Münster, Germany. 9. Department of Neuropediatrics, Justus-Liebig-University Gießen, Gießen, Germany. 10. Clinic for Pediatric Kidney, Liver and Metabolic Diseases, Hannover Medical School, Hannover, Germany. 11. Department of Neuropediatrics, Leipzig University Hospital for Children and Adolescents, Leipzig, Germany. 12. Epilepsy Center Hessen and Department of Neurology, Philipps-University Marburg, Marburg, Germany. 13. Department of Neuropediatrics and Muscle Disorders, Center for Pediatrics, Medical Center, University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg i.Br., Germany; Department of Pediatrics and Clinical Neurosciences, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada. 14. Epilepsy Center Frankfurt Rhine-Main and Department of Neurology, Goethe-University Frankfurt, Frankfurt am Main, Germany; Department of Neuropediatrics, Goethe-University Frankfurt, Frankfurt am Main, Germany. 15. Epilepsy Center Frankfurt Rhine-Main and Department of Neurology, Goethe-University Frankfurt, Frankfurt am Main, Germany; Center for Personalized Translational Epilepsy Research (CePTER), Goethe-University Frankfurt, Frankfurt am Main, Germany; Departments of Clinical Neurosciences, Medical Genetics and Community Health Sciences, Hotchkiss Brain Institute & Alberta Children's Hospital Research Institute, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada. 16. Department of Neuropediatrics and Muscle Disorders, Center for Pediatrics, Medical Center, University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg i.Br., Germany; Berta-Ottenstein-Programme, Faculty of Medicine, University of Freiburg, Freiburg i.Br., Germany. 17. Clinic for Neuropediatrics and Neurorehabilitation, Epilepsy Center for Children and Adolescents, Schön Clinic Vogtareuth, Germany; Research Institute, Rehabilitation, Transition and Palliation, PMU Salzburg, Salzburg, Austria. 18. Department of Pediatrics, Klinikum Worms, Worms, Germany; Department of Pediatrics, University Medicine Mainz, Mainz, Germany. 19. Epilepsy Center Kleinwachau, Dresden-Radeberg, Germany. 20. Department of Pediatric Neurology, Psychosomatics and Pain management, Klinikum Stuttgart, Stuttgart, Germany. 21. Department of Neuropediatrics, University Children's Hospital of Saarland, Homburg, Germany. 22. Department of Neuropediatrics, Christian-Albrechts-University Kiel & University Hospital Schleswig-Holstein, Campus Kiel, Germany. 23. Epilepsy Center for Children, University Hospital Neuruppin, Brandenburg Medical School, Neuruppin, Germany. 24. Department of Neuropediatrics, University Hospital Tübingen, Tübingen, Germany. 25. Klinikum Kempten, Klinikverbund Allgäu, Kempten/Allgäu, Germany. 26. Department of Neuropediatrics, University of Witten/Herdecke, Herdecke, Germany. 27. Division of Pediatric Epileptology, Centre for Paediatrics and Adolescent Medicine, University Hospital Heidelberg, Heidelberg, Germany. 28. Department of Neuropediatrics and Social Pediatrics, Ruhr University Bochum, Bochum, Germany. 29. Department of Neuropediatrics, Friedrich-Alexander University of Erlangen-Nürnberg, Erlangen, Germany. 30. Department of Neuropediatrics, Klinikum Kassel, Kassel, Germany. 31. Epilepsy Center Frankfurt Rhine-Main and Department of Neurology, Goethe-University Frankfurt, Frankfurt am Main, Germany; Center for Personalized Translational Epilepsy Research (CePTER), Goethe-University Frankfurt, Frankfurt am Main, Germany; Epilepsy Center Hessen and Department of Neurology, Philipps-University Marburg, Marburg, Germany. Electronic address: strzelczyk@med.uni-frankfurt.de.
Abstract
OBJECTIVE: This study aimed to measure health-related quality of life (HRQOL) in children and adolescents with tuberous sclerosis complex (TSC) and quality of life (QOL) and depressive symptoms among caregivers. METHODS: Adequate metrics were used to assess HRQOL in children and adolescents with TSC (4-18 years, KINDLR) as well as QOL (EQ-5D) and symptoms of depression (BDI-II) among caregivers. Predictors for reduced HRQOL and depressive symptoms were identified by variance analysis, ordinal regression, and bivariate correlation. RESULTS: The mean HRQOL score was 67.9 ± 12.7, and significantly lower values were associated with increasing age, attending special needs education, TSC-associated psychiatric symptoms, and drug-related adverse events. The mean QOL of caregivers was 85.4 ± 15.7, and caregiver's sex, TSC mutation locus, familial TSC clustering, special needs education, degree of disability, care dependency, presence of TSC-associated psychiatric symptoms, and TSC severity were significant predictors of lower QOL. Depressive symptoms were identified in 45.7% of caregivers, associated with female sex of the caregiver, familial TSC clustering, special needs education, and presence of TSC-associated psychiatric symptoms of the child. Multivariate regression analysis revealed adolescence and drug-related adverse events as significant predictors for lower HRQOL in TSC children, and TSC2 variants predicted lower QOL and depressive symptoms in caregivers. CONCLUSION: Compared with other chronic diseases, such as headache, diabetes or obesity, children with TSC have significantly lower HRQOL, which further decreases during adolescence. A decreased HRQOL of patients correlates with a lower QOL and increased symptoms of depression of their caregivers. These results may improve the comprehensive therapy and care of children and adolescents with TSC and their families and caregivers. TRIAL REGISTRATION: DRKS, DRKS00016045. Registered 01 March 2019, http://www.drks.de/DRKS00016045.
OBJECTIVE: This study aimed to measure health-related quality of life (HRQOL) in children and adolescents with tuberous sclerosis complex (TSC) and quality of life (QOL) and depressive symptoms among caregivers. METHODS: Adequate metrics were used to assess HRQOL in children and adolescents with TSC (4-18 years, KINDLR) as well as QOL (EQ-5D) and symptoms of depression (BDI-II) among caregivers. Predictors for reduced HRQOL and depressive symptoms were identified by variance analysis, ordinal regression, and bivariate correlation. RESULTS: The mean HRQOL score was 67.9 ± 12.7, and significantly lower values were associated with increasing age, attending special needs education, TSC-associated psychiatric symptoms, and drug-related adverse events. The mean QOL of caregivers was 85.4 ± 15.7, and caregiver's sex, TSC mutation locus, familial TSC clustering, special needs education, degree of disability, care dependency, presence of TSC-associated psychiatric symptoms, and TSC severity were significant predictors of lower QOL. Depressive symptoms were identified in 45.7% of caregivers, associated with female sex of the caregiver, familial TSC clustering, special needs education, and presence of TSC-associated psychiatric symptoms of the child. Multivariate regression analysis revealed adolescence and drug-related adverse events as significant predictors for lower HRQOL in TSC children, and TSC2 variants predicted lower QOL and depressive symptoms in caregivers. CONCLUSION: Compared with other chronic diseases, such as headache, diabetes or obesity, children with TSC have significantly lower HRQOL, which further decreases during adolescence. A decreased HRQOL of patients correlates with a lower QOL and increased symptoms of depression of their caregivers. These results may improve the comprehensive therapy and care of children and adolescents with TSC and their families and caregivers. TRIAL REGISTRATION: DRKS, DRKS00016045. Registered 01 March 2019, http://www.drks.de/DRKS00016045.
Authors: Adam Strzelczyk; Gerhard Kurlemann; Thomas Bast; Ulrich Bettendorf; Gerhard Kluger; Thomas Mayer; Bernd A Neubauer; Tilman Polster; Sarah von Spiczak; Regina Trollmann; Markus Wolff; Toby Toward; Jens Gruenert; Eddie Gibson; Clive Pritchard; Joe Carroll; Felix Rosenow; Susanne Schubert-Bast Journal: Neurol Res Pract Date: 2022-06-06