Navigating life after New-onset refractory status epilepticus (NORSE) and Febrile infection-related epilepsy syndrome (FIRES): Insights from caregiver and patient interviews

Abstract

Objective

To describe the lived experience of patients with NORSE and explore quality of life (QOL) for patients and their caregivers.

Background

NORSE is a rare condition characterized by refractory status epilepticus, often of unknown cause, in a previously neurologically healthy individual. Febrile infection-related epilepsy syndrome (FIRES) is a subset of NORSE. NORSE is associated with a risk of mortality, acute morbidity, and neurocognitive sequela with limited data about the long-term impacts.

Methods

We conducted in-depth individual or dyadic semi-structured interviews with patients and caregivers. Interviews explored the long-term experiences after NORSE and its impacts on patient and caregiver QOL. Data were analyzed using a thematic content analysis approach.

Results

Fourteen interviews, comprising 5 patients and 15 caregivers, were completed between April 2023 – February 2024. The median age at NORSE onset was 17 years (IQR: 11.00 – 20.25) with a median of 5.60 years (IQR: 3.88 – 8.50) since onset. While experiences varied based on NORSE outcomes and time since onset, we identified the following themes: 1) Seizures, medication side effects, and comorbid health concerns impact patient independence and QOL; 2) Mental and behavioral health concerns compounded by loneliness and isolation impact patient QOL; 3) Friend and family relationship changes impact patient social and emotional QOL; 4) Family members take on care of the patient and experience significant life changes.

Conclusion

NORSE has significant life-altering impacts on patients and caregivers. These findings highlight a need to evaluate outcomes and provide support that extends beyond seizure management.