Navigating Life after New-onset Refractory Status Epilepticus (NORSE) and Febrile-infection Related Epilepsy Syndrome (FIRES): Insights from Caregiver Interviews
Krista Eschbach1, Julia Reedy2, Teneille Gofton3, Margaret Gopaul4, Raquel Farias-Moeller5, Marissa Kellogg6, Lawrence Hirsch7, Nora Wong8, Brooke Dorsey Holliman2
1Pediatrics, Section of Neurology, University of Colorado Anschutz Medical Campus, Children's Hospital Colorado, 2Adult and Child Center for Health Outcomes Research and Delivery Science (ACCORDS), University of Colorado Anschutz Medical Campus, 3London Health Sciences Centre, 4Yale School of Medicine, 5Medical College of Wisconsin, 6VA Portland Healthcare System, Dept of Neurology, 7Yale University Comprehensive Epilepsy Center, 8NORSE Institute
Objective:
To describe the long-term lived experience of patients and their caregivers after NORSE.
Background:
NORSE is a rare condition characterized by refractory status epilepticus, often of unknown cause, in a previously neurologically healthy individual. It is associated with a risk of acute mortality and significant neurocognitive sequela.
Design/Methods:
We completed semi-structured interviews with caregivers of adult and pediatric patients with NORSE. Interviews were audio recorded, professionally transcribed, and continued until emerging themes identified. We coded and analyzed transcripts using a thematic content analysis approach.
Results:
Seven interviews (1 NORSE; 6 FIRES) were completed between April - August 2023. NORSE onset occurred a median of 7.5 years ago (IQR 6-10). While experiences varied based on NORSE outcomes and amount of time post-illness onset, we identified the following themes: 1) Changes in life experience: All participants described significant disease impact with reduced quality of life (QOL); however, perceived QOL was not always consistent throughout the chronic phase. Some participants described improvements and others reported a decline in QOL. For some caregivers, there was a significant financial impact and roles within the home changed including stopping work to become the primary caregiver for their child. 2) Medical concerns: Patients are often impacted by ongoing seizures and challenges posed by antiseizure medications, as well as other health concerns (e.g., gastrointestinal symptoms, mood and behavior issues). 3) Need for help and support: Caregivers described feelings of isolation and loneliness for themselves and for their child. Some described unmet needs, such as requiring more assistance during the transition out of the hospital and challenges accessing behavioral and mental health resources.
Conclusions:
NORSE has significant life-altering impacts on patients and caregivers. These findings highlight a need to evaluate outcomes and provide support extending beyond seizure management, as well as the importance of multidisciplinary care models for patients with NORSE and their families.