Download PDF
Journal of Pediatric Epilepsy 2020; 09(04): 135-142
DOI: 10.1055/s-0040-1716670
Review Article

Challenges in the Transition of Care Process for Patients with Dravet and Lennox–Gastaut Syndromes

Cemal Karakas
1   Department of Neurology and Neurophysiology, Baylor College of Medicine, Houston, Texas, United States
2   Section of Pediatric Neurology and Developmental Neuroscience, Department of Pediatrics, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas, United States
,
Rebecca J. Schultz
2   Section of Pediatric Neurology and Developmental Neuroscience, Department of Pediatrics, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas, United States
3   Nelda C. Stark College of Nursing, Texas Woman's University, Houston, Texas, United States
,
Jay R. Gavvala
1   Department of Neurology and Neurophysiology, Baylor College of Medicine, Houston, Texas, United States
› Author Affiliations Funding None.
› Further Information
    Permissions and Reprints

Abstract

Epileptic encephalopathies such as Dravet syndrome (DS) and Lennox–Gastaut syndrome (LGS) present unique challenges in the transition of care not only for the providers but also for the patients and families. Some of these challenges include the complexity of disease process, differences in medication management between children and adults, high incidence of comorbidities such as psychosocial issues, a lack of structured transition process from pediatric to adult care, and the lack of parental knowledge and reluctance to transition to an adult provider. Improving transition readiness and transfer of care are essential to long-term management and continuity of care. Studies show that patients/families who possess transition readiness skills have better health outcomes. Furthermore, participation in a structured transition intervention has been shown to improve transition readiness and utilization of ambulatory care in the adult setting. Reported benefits of implementation of transition planning include increased self-esteem, improved health literacy, fewer emergency room visits, decreased hospitalizations and comorbidities, and fewer school absences. Nevertheless, there is a lack of evidenced-based, family/patient-centered transition model of care. This review's primary goal is to provide an overview of challenges in the transition of care and recommendations for an ideal transition for patients with DS and LGS.

Keywords

transition of care - Dravet syndrome - Lennox–Gastaut


Publication History

Received: 28 July 2020

Accepted: 06 August 2020

Article published online:
11 September 2020

© 2020. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 

  • References

  • 1 Dravet C. The core Dravet syndrome phenotype. Epilepsia 2011; 52 (Suppl. 02) 3-9
    CrossrefPubMedGoogle Scholar
  • 2 Anwar A, Saleem S, Patel UK, Arumaithurai K, Malik P. Dravet syndrome: an overview. Cureus 2019; 11 (06) e5006
    PubMedGoogle Scholar
  • 3 Khan S, Al Baradie R. Epileptic encephalopathies: an overview. Epilepsy Res Treat 2012; 2012: 403592
    PubMedGoogle Scholar
  • 4 Martin P, Rautenstrauβ B, Abicht A, Fahrbach J, Koster S. Severe myoclonic epilepsy in infancy - adult phenotype with bradykinesia, hHypomimia, and perseverative behavior: report of ive cases. Mol Syndromol 2010; 1 (05) 231-238
    CrossrefPubMedGoogle Scholar
  • 5 Claes L, Del-Favero J, Ceulemans B, Lagae L, Van Broeckhoven C, De Jonghe P. De novo mutations in the sodium-channel gene SCN1A cause severe myoclonic epilepsy of infancy. Am J Hum Genet 2001; 68 (06) 1327-1332
    CrossrefPubMedGoogle Scholar
  • 6 Arzimanoglou A, French J, Blume WT. et al. Lennox-Gastaut syndrome: a consensus approach on diagnosis, assessment, management, and trial methodology. Lancet Neurol 2009; 8 (01) 82-93
    CrossrefPubMedGoogle Scholar
  • 7 VanStraten AF, Ng YT. Update on the management of Lennox-Gastaut syndrome. Pediatr Neurol 2012; 47 (03) 153-161
    CrossrefPubMedGoogle Scholar
  • 8 Nabbout R, Camfield CS, Andrade DM. et al. Treatment issues for children with epilepsy transitioning to adult care. Epilepsy Behav 2017; 69: 153-160
    CrossrefPubMedGoogle Scholar
  • 9 Office of Title V & Family Health, Maternal Child Health (MCH) Title V 2015 Needs Assessment Findings, Division of Family and Community Services, Editor. Austin, Texas: Texas Department of State Health Services; 2015
  • 10 Schultz RJ. Parental experiences transitioning their adolescent with epilepsy and cognitive impairments to adult health care. J Pediatr Health Care 2013; 27 (05) 359-366
    CrossrefPubMedGoogle Scholar
  • 11 Warnell P. The transition experience of epilepsy patients/families: results of a telephone survey. Axone 1998; 20 (02) 31-33
    PubMedGoogle Scholar
  • 12 Camfield PR, Gibson PA, Douglass LM. Strategies for transitioning to adult care for youth with Lennox-Gastaut syndrome and related disorders. Epilepsia 2011; 52 (Suppl. 05) 21-27
    CrossrefPubMedGoogle Scholar
  • 13 Sawicki GS, Lukens-Bull K, Yin X. et al. Measuring the transition readiness of youth with special healthcare needs: validation of the TRAQ--Transition Readiness Assessment Questionnaire. J Pediatr Psychol 2011; 36 (02) 160-171
    CrossrefPubMedGoogle Scholar
  • 14 Camfield PR, Bahi-Buisson N, Trinka E. Transition issues for children with diffuse cortical malformations, multifocal postnatal lesions, (infectious and traumatic) and Lennox-Gastaut and similar syndromes. Epilepsia 2014; 55 (Suppl. 03) 24-28
    CrossrefPubMedGoogle Scholar
  • 15 Thomson J, Shah SS, Simmons JM. et al. Financial and social hardships in families of children with medical complexity. J Pediatr 2016; 172: 187-193.e1
    CrossrefPubMedGoogle Scholar
  • 16 Gilliam F, Penovich PE, Eagan CA. et al. Conversations between community-based neurologists and patients with epilepsy: results of an observational linguistic study. Epilepsy Behav 2009; 16 (02) 315-320
    CrossrefPubMedGoogle Scholar
  • 17 Borlot F, Tellez-Zenteno JF, Allen A, Ali A, Snead III OC, Andrade DM. Epilepsy transition: challenges of caring for adults with childhood-onset seizures. Epilepsia 2014; 55 (10) 1659-1666
    CrossrefPubMedGoogle Scholar
  • 18 Andrade DM, Bassett AS, Bercovici E. et al. Epilepsy: Transition from pediatric to adult care. Recommendations of the Ontario epilepsy implementation task force. Epilepsia 2017; 58 (09) 1502-1517
    CrossrefPubMedGoogle Scholar
  • 19 Nabbout R, Arzimanoglou A, Chin RFM, Grinspan Z, Speechley K, Camfield P. The evaluation and costs of transition programs for youth with epilepsy. Epilepsy Behav 2019; 93: 133-137
    CrossrefPubMedGoogle Scholar
  • 20 Carrizosa J, An I, Appleton R, Camfield P, Von Moers A. Models for transition clinics. Epilepsia 2014; 55 (Suppl. 03) 46-51
    CrossrefPubMedGoogle Scholar
  • 21 Barron DA, Molosankwe I, Romeo R, Hassiotis A. Urban adolescents with intellectual disability and challenging behaviour: costs and characteristics during transition to adult services. Health Soc Care Community 2013; 21 (03) 283-292
    CrossrefPubMedGoogle Scholar
  • 22 Camfield P, Camfield C. Transition to adult care for children with chronic neurological disorders. Ann Neurol 2011; 69 (03) 437-444
    CrossrefPubMedGoogle Scholar
  • 23 Rajendran S, Iyer A. Epilepsy: addressing the transition from pediatric to adult care. Adolesc Health Med Ther 2016; 7: 77-87
    CrossrefPubMedGoogle Scholar
  • 24 Devinsky O. Transition to adult care for children with epilepsy--a call for action. Epilepsia 2014; 55 (Suppl. 03) 54-55
    CrossrefPubMedGoogle Scholar
  • 25 Beghi E, Camfield PR, Camfield CS. Epidemiologic aspects: lost in transition. Epilepsia 2014; 55 (Suppl. 03) 3-7
    CrossrefPubMedGoogle Scholar
  • 26 Brown LW, Camfield P, Capers M. et al. The neurologist's role in supporting transition to adult health care: a consensus statement. Neurology 2016; 87 (08) 835-840
    CrossrefPubMedGoogle Scholar
  • 27 Schwartz LA, Tuchman LK, Hobbie WL, Ginsberg JP. A social-ecological model of readiness for transition to adult-oriented care for adolescents and young adults with chronic health conditions. Child Care Health Dev 2011; 37 (06) 883-895
    CrossrefPubMedGoogle Scholar
  • 28 Ferris M, Cohen S, Haberman C. et al. Self-Management and transition readiness assessment: development, reliability, and factor structure of the STARx questionnaire. J Pediatr Nurs 2015; 30 (05) 691-699
    CrossrefPubMedGoogle Scholar
  • 29 Adams S, Nicholas D, Mahant S. et al. Care maps for children with medical complexity. Dev Med Child Neurol 2017; 59 (12) 1299-1306
    CrossrefPubMedGoogle Scholar
  • 30 Hatcher-Martin JM, Adams JL, Anderson ER. et al. Telemedicine in neurology: Telemedicine Work Group of the American Academy of Neurology update. Neurology 2020; 94 (01) 30-38
    CrossrefPubMedGoogle Scholar