Danielle M Andrade1, Anne T Berg2, Veronica Hood3, Kelly G Knupp4, Sookyong Koh5, Linda Laux6, Mary Anne Meskis3, Ian Miller7, M Scott Perry8, Ingrid E Scheffer9, Joseph Sullivan10, Nicole Villas3, Elaine Wirrell11. 1. Adult Epilepsy Genetics Program, Division of Neurology, Krembil Brain Institute, Toronto Western Hospital, University of Toronto, Toronto, Canada. Electronic address: Danielle.andrade@uhn.ca. 2. Ann & Robert H. Lurie Children's Hospital of Chicago, Departments of Pediatrics and Neurological Surgery, Northwestern Feinberg School of Medicine, Chicago, USA. 3. Dravet Syndrome Foundation, Cherry Hill, NJ, USA. 4. Department of Pediatrics and Neurology, University of Colorado Anschutz Campus, Aurora, CO, USA. 5. Department of Pediatric Neurology at University of Nebraska Medical Center, Omaha, NE, USA. 6. Epilepsy Center, Department of Pediatrics, Ann & Robert H. Lurie Children's Hospital, Chicago, IL, USA. 7. Department of Neurology, Nicklaus Children's Hospital, Miami, FL, USA. 8. Jane and John Justin Neurosciences Center, Cook Children's Medical Center, Fort Worth, TX, USA. 9. Epilepsy Research Centre, The University of Melbourne, Austin Health, Royal Children's Hospital, Florey Institute, Murdoch Children's Research Institute, Melbourne, Victoria, Australia. 10. Department of Neurology & Pediatrics, University of California, San Francisco, CA, USA. 11. Child and Adolescent Neurology, Mayo Clinic, Rochester, MN, USA.
Abstract
INTRODUCTION: Dravet syndrome (DS) is still seen as a "pediatric disease", where patients receive excellent care in pediatric centers, but care is less than optimal in adult health care systems (HCS). This creates a barrier when young adults need to leave the family-centered pediatric system and enter the adult, patient-centered HCS. Here we create a guide to help with the transition from pediatric to adult for patients with DS. METHODS: Experts in Dravet syndrome flagged the main barriers in caring for adults with DS and created a 2-page transition summary guide based on their expertise and a literature review. RESULTS: The 2-page guide addresses: DS diagnosis in children and adults; clinical manifestations, including the differences in seizures types and frequencies between children and adults with DS; the natural history of intellectual disability, behavior, gait, motor disorders and dysautonomia; a review of optimal treatments (including medications not commonly used in adult epilepsy settings such as stiripentol and fenfluramine), as well as emergency seizure management; avoidance of triggers, preventive measures, and vaccine administration in adults with DS. CONCLUSION: Several young adults with DS are still followed by their child neurologist. This 2-page transition guide should help facilitate the transition of patients with DS to the adult HCS and should be given to families as well as adult health care providers that may not be familiar with DS.
INTRODUCTION: Dravet syndrome (DS) is still seen as a "pediatric disease", where patients receive excellent care in pediatric centers, but care is less than optimal in adult health care systems (HCS). This creates a barrier when young adults need to leave the family-centered pediatric system and enter the adult, patient-centered HCS. Here we create a guide to help with the transition from pediatric to adult for patients with DS. METHODS: Experts in Dravet syndrome flagged the main barriers in caring for adults with DS and created a 2-page transition summary guide based on their expertise and a literature review. RESULTS: The 2-page guide addresses: DS diagnosis in children and adults; clinical manifestations, including the differences in seizures types and frequencies between children and adults with DS; the natural history of intellectual disability, behavior, gait, motor disorders and dysautonomia; a review of optimal treatments (including medications not commonly used in adult epilepsy settings such as stiripentol and fenfluramine), as well as emergency seizure management; avoidance of triggers, preventive measures, and vaccine administration in adults with DS. CONCLUSION: Several young adults with DS are still followed by their child neurologist. This 2-page transition guide should help facilitate the transition of patients with DS to the adult HCS and should be given to families as well as adult health care providers that may not be familiar with DS.
Authors: Veronica Hood; Anne T Berg; Kelly G Knupp; Sookyong Koh; Linda Laux; Mary Anne Meskis; Quratulain Zulfiqar-Ali; M Scott Perry; Ingrid E Scheffer; Joseph Sullivan; Elaine Wirrell; Danielle M Andrade Journal: Epilepsia Date: 2022-04-20 Impact factor: 6.740
Authors: Elaine C Wirrell; Veronica Hood; Kelly G Knupp; Mary Anne Meskis; Rima Nabbout; Ingrid E Scheffer; Jo Wilmshurst; Joseph Sullivan Journal: Epilepsia Date: 2022-05-12 Impact factor: 6.740