Abstracts

Rationale: New-onset refractory status epilepticus (NORSE) is a rare clinical presentation affecting previously healthy children and adults. Febrile infection-related epilepsy syndrome (FIRES) is a subcategory of NORSE and applies when a preceding fever occurred between two weeks and 24-hr prior to the start of refractory status epilepticus. The NORSE/FIRES Family Registry has been developed to gain insight into possible risk factors for NORSE and FIRES, to assess the spectrum of clinical outcomes and the quality of life of survivors. Given that consensus definitions and approaches to care have only been recently established, the extent and quantity of information families receive during the initial hospitalization period about the diagnosis, prognosis, and quality of communication remains relatively unknown.

Methods: The NORSE/FIRES Family registry is generously supported by the Robert N. Kohn Research Memorial Fund. This registry uses REDCap, a web-based database. People permitted to access the registry include survivors, substitute/surrogate decision makers (for survivors or non-survivors) and physicians. Information collected in this longitudinal cohort study includes past medical history, clinical presentation, disease course, doctor to family communication, clinical sequelae and quality of life, among others. Participants are invited to complete follow-up surveys for up to two years following clinical presentation of seizures. Enrollment is ongoing in English, French, and Spanish. This abstract will focus on communication regarding NORSE/FIRES.

Results: To date, 45 participants have enrolled in this study (2-78 years, mean: 19.6, SD: 16.6, 16 females and 29 males) from 12 different countries across 5 continents. 25/45 participants are survivors of NORSE/FIRES. 27/45 participants were informed by medical personnel that the patients had NORSE or FIRES during hospitalization. Information regarding prognosis was communicated to 28/45 family members. The quantity of prognostic information, on average, was rated 5.9/10 (SD: 3.5), where 10 represents complete information. Satisfaction with communication between medical teams and family was found to be rated at 6.1/10 (SD: 2.1). In a subset of participants surveys, 11/27 patients had a palliative care team involved, with which the quality of information was rated 7.2/10 (SD: 1.8). 7/11 of these patients are survivors of NORSE/FIRES. The quality of life amongst all survivors was rated as 3.9/10 (SD: 2.1).

Conclusions: This data suggests that the term NORSE or FIRES is not universally used in discussions between the health care team and patients’ families. Quality of life for survivors is low, but discussions regarding potential clinical outcomes are also not universally undertaken during the acute stages of hospitalization. Further education regarding the clinical presentation of NORSE/FIRES needs to be disseminated within the medical community. Development of written literature and decision aids for families may help to support prognostic discussions.

Funding: Please list any funding that was received in support of this abstract.: The NORSE/FIRES Family registry is generously supported by the Robert N. Kohn Research Memorial Fund.