Register now for upcoming event:
NORSE/FIRES Family Conference : Research Updates, Transition of Care, Post-Intensive Care Syndrome
Wednesday, May 1, 2024 10am – 12:30 pm ET
Target Audience: Patients, Families, Clinicians, and Scientists
To Register, click here.
Leads: Krista Eschbach, MD, (Children's Hospital-Colorado)
Teneille Gofton, MD, FRCPC, (Western Univ. Canada)
Transition from pediatrics to adulthood, Sena Yoon, LCSW, (Children’s Hospital Colorado)
Post-intensive care unit syndrome, Mary Hartman, MD. MPH, (Washington Univ. St. Louis)
Breakout discussion groups:
1. Communication with your doctor about treatment and medications
2. Transition from pediatric to adult care: resources and insights
3. Transition home from the hospital and rehab: resources and insights
4. Opportunities to connect with others: Bereavement group – for those who have lost a loved one to NORSE or FIRES
5. Opportunities to connect with others: Adolescent and adult survivors of NORSE
6. Opportunities to connect: Caregivers only - what about me?
Whether their loved ones have passed away or continue to live with NORSE--several parents and spouses have spoken of developing physical, cognitive, and psychological impairments in themselves after NORSE changed their lives. the term Post-Intensive Care Syndrome or PICS refers to a patient’s new and persistent declines in physical, cognitive, and psychological functioning following an ICU stay separate from or worsening the initial cause of the ICU hospitalization (for all ICU cases: cancer, TBI, infection, etc.) A link to an overview of post-intensive care syndrome is here: https://www.ncbi.nlm.nih.gov/books/NBK558964/
The term Post-Intensive Care Syndrome-Families or PICS-F refers to the close family members of ICU patients who experience subsequent adverse changes in cognitive, behavioral, and mental health because they have witnessed their loved ones' treatment in the ICU.
Our two Family meetings in September were on this topic. Alison Kukla, MPH, who leads bereavement support and self-management services at the Epilepsy Foundation shared the Epilepsy Foundation's Bereavement Guide (see link below) and also a link to the National Center for Post-Traumatic Stress Disorder: https://www.ptsd.va.gov/ sponsored by the US Veterans Association,) not only military focused.)
Families may want to review the most recent publications and recordings on the identification and treatment for NORSE and FIRES and/or forward it to relevant professionals.
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Recordings available now of our 2023 NORSE Family Conference and Scientific Symposium
NORSE/FIRES Family Conference, June 6, 2023: https://youtu.be/QicS6txJoZ4
Target audience: Patients, Families and Clinicians
Presentations and panel discussion among a rheumatologist, neuropharmacologist, and neurologist on treatments in the acute phase of NORSE/FIRES, and therapies in the chronic phase to address seizures, cognitive and behavioral outcomes. Breakout sessions offer an opportunity for families to connect with clinicians and other families living a similar experience whether it be bereavement or coping with survival.
Recording of Medical & Scientific Symposium, June 7, 2023: https://youtu.be/hIHMjSOBGEY
Target audience: Clinicians and Scientists
Latest findings on the genetic landscape, cytokine profiling, peripheral blood monocytes, animal models and possible seasonality of cryptogenic NORSE/FIRES, and an update on the open NORSE/FIRES biorepository.
Journal articles
Wickstrom R, Taraschenko O, Dilena R, Payne ET, Specchio N, Nabbout R, Koh S, Gaspard N, Hirsch LJ; International NORSE Consensus Group. International consensus recommendations for management of New Onset Refractory Status Epilepticus (NORSE) incl. Febrile Infection-Related Epilepsy Syndrome (FIRES): Statements and Supporting Evidence. Epilepsia. 2022 Aug 23;63(11):2840–64. doi: 10.1111/epi.17397. Epub ahead of print. PMID: 35997591; PMCID: PMC9828002. This is a free article (click on link above)
Patients with NORSE/FIRES require specialized care.
This publication addresses that process. Vinette SA, Young GB, Khosravani H. Early identification of NORSE and transfer to care setting with appropriate supports: A proposed algorithm. Front Neurol. 2023 Apr 11;14:1072020. doi: 10.3389/fneur.2023.1072020. PMID: 37114231; PMCID: PMC10126407.
*Important notice about recent changes to Medicaid*
Rachel Patterson, Senior Director, Federal Relations & Policy, Epilepsy Foundation, shares that Medicaid eligibility renewals were paused during the COVID-19 pandemic but are re-starting soon. This list from CMS (Centers of Medicare & Medicaid) allows you to find the date renewal notices will be sent out and the date by which coverage may be lost if there is no response to the renewal notice.
Recordings of the NORSE Medical/Scientific Symposium and Family Conference on June 15 & 16, 2022 are now available. Please read the following before viewing.
Many families found both the Scientific Symposium and the Family Conference to be informative, especially the breakout sessions at the end of the Family Conference where people had a chance to connect with others whose experiences were similar to their own. From feedback on the followup survey, we will increase the time in the breakout sessions next year. A few families found the family perspectives on organ donation and the scientific presentation on neuropathology research distressing. We will find ways to minimize that discomfort in the future while preserving the opportunity for everyone to learn from each other.
Usually, families are not invited to attend scientific conferences. Our intention at the NORSE Institute is different. The illness is rare, information about it is scarce even among professionals, and so little of that information is communicated to families. We hold that families deserve the opportunity to learn and share what they know with scientists, as they choose. We will work to make that learning and sharing experience as positive and effective as possible.
In the meantime, the detailed agenda of the symposium and the family conference are posted below to help those who would like to navigate their viewing of the recordings to skip certain parts.
Clinical Symposium: https://www.youtube.com/watch?v=59dVveERN38
NORSE/FIRES Family conference: https://youtu.be/gV9m9YhBMvs
NORSE Scientific Symposium and NORSE/FIRES Family Conference Agenda
Medical/Scientific Symposium, Wednesday, June 15th
Target audience: clinicians and scientists
10:00 – 10:05 Welcome: Nicolas Gaspard, MD, PhD (Université libre de Bruxelles)
10:05 - 10:20 Conclusions from the NORSE/FIRES Delphi study
Points of consensus and implications for practice and research.
Speaker: Ronny Wickström, MD, PhD (Karolinska Univ. Hospital)
10:20 - 11:10 Case management of 17-year-old patient with expert commentary
Panelists: Jim Riviello, MD (Baylor, pediatric neurology); Emily Gilmore, MD (Yale, adult neurointensive care); Ingo Helbig, MD (CHOP, neurogenetics), Eyal Muscal, MD (Baylor, rheumatology) and Mike Wilson, MD (UCSF, metagenomics). Case by Larry Hirsch, MD (Yale, epilepsy)
11:10 - 11:20 Family message on palliative care and donation of organs for research, Sarah Mussetter, Esq. (Finn’s mom) and Judith K. Weiss, MA (Gabe’s mom)
11:20 -11:25 Communication of NORSE on social media
Raquel Farias-Moeller, MD (Children's Wisconsin)
11:35 – 12:20 Data Blitz Presentations
Immune dysregulation in NORSE/FIRES patients
Aurélie Hanin, PharmD, PhD, Department of Neurology & Immunobiology, Yale School of Medicine, New Haven in Collaboration with Pitié-Salpêtrière Hospital, Paris, France
Neuropathology in NORSE: Current knowledge and ongoing research efforts
Eleonora Aronica, MD, PhD (U of Amsterdam) and Anita Huttner, MD, (Yale)
Seasonality of NORSE
Jorge Cespedes, MD, (Yale)
12:20 – 12:30 Biorepository and Closing: Lawrence Hirsch, MD (Yale)
NORSE/FIRES Family Conference, Thursday, June 16th
Target audience: Patients, Families, and Clinicians
10:00 – 10:05 Welcome: Nora Wong, PhD (NORSE Institute)
10:05-10:50 Ask the Experts Panel Discussion
Moderator: Krista Eschbach, MD (Children's Colorado)
Panelists: James Riviello, MD (Baylor); Raquel Farias-Moeller, MD (Children's Wisconsin) Nicolas Gaspard, MD, PhD (Université libre de Bruxelles) and Marissa Kellogg, MD, MPH (OHSU)
Questions pre-submitted by patients and families
10:50 -11:05 This is my life: What patients and families want doctors to know
Doctor-family communication, Judith K. Weiss, MA (Gabe’s mom)
Caring for Peds survivor, Aurelie Schiessl
My life now, Luke Boulianne
11:15 – 11:25 Early results from NORSE/FIRES Family Registry
Teneille Gofton, MD (Western U. Canada)
11:25 – 11:35 NORSE/FIRES Biorepository
Lawrence Hirsch, MD (Yale)
11:45 – 12:15 Breakout sessions
• Bereavement: Teneille Gofton, MD (Western Canada); Cassie Matz, LCSW, (Children's Colorado)
• Families with surviving adult patients: Erica Sieg, PsyD (Northwestern) and Steve VanHaerents, MD (Northwestern)
• Families with surviving pediatric patients: Lauren Treat, MD (Children's Colorado)
• Adult and teen patients: Alison Kukla, MPH, Epilepsy Foundation
12:25-12:30: Closing: Krista Eschbach
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The NORSE Institute held a scientific symposium for lab scientists and clinical scientists and a NORSE Family Conference for patients, families, clinicians and scientists on March 4-5, 2021. Recordings of the Symposium and the Family Conference are available below.
Scientific Symposium Part 1 https://youtu.be/65LfnBGx_iI
Scientific Symposium Part 2 https://youtu.be/3lA9xFTJtCo
Family Conference Day 1 https://youtu.be/1gWGynjkDFA
Family Conference Day 2 (Part 1) https://youtu.be/_KLsqdbKo50
Family Conference Day 2 (Part 2) https://youtu.be/Z6w_6fs_8HQ
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NORSE in plain English: Hear Dr Lawrence J Hirsch (Yale) and Dr Nicolas Gaspard (Erasme Hospital, Belgium) explain what NORSE is and where the research is going in an interview with ILAE (International League Against Epilepsy) here.
To get a medical summary of NORSE and FIRES and related research and readings, a link to a recent article in the medical journal, Seizure is here.
To provide doctors information to evaluate NORSE, give them this diagnostic checklist:
If you would like to talk with someone who has gone through the NORSE experience, we have NORSE family members who are volunteer NORSE navigators. These are untrained, sympathetic listeners who may offer the outlet you need given the social isolation during COVID. Contact us through the Comments page.
Check the Professionals side of this website to view NORSE research and researchers.
What is NORSE?
NORSE is the abbreviated term or acronym for new onset refractory status epilepticus. In everyday language, it is a life-threatening condition requiring emergency treatment that comes seemingly from out of the blue (new onset), characterized by a prolonged seizure or a cluster of seizures without recovery in between (status epilepticus) without clear cause despite extensive medical testing. The seizures do not respond to the first and second lines of established medical treatments (refractory). Although the cause for the illness is eventually found in about half the cases (usually autoimmune or paraneoplastic encephalitis), in the balance of the cases, the cause is never found and is often termed “cryptogenic” NORSE or NORSE of unknown etiology.
After at least two anti-seizure drugs fail to stop the seizures, patients are typically placed in a coma with anesthetic drugs to protect their brains from the effects of the seizures (often referred to as a medically-induced coma). The hope is that after this period of quiet, the brain has calmed. At some point, doctors carefully reduce the anesthetic drugs, checking for the return of seizures, a process sometimes referred to as "weaning" the patient away from the anesthetics.
The consequences of NORSE vary from rare, full recovery, to mild to significant neurological deficits (morbidity) and death (mortality). Most survivors require extensive rehabilitation therapy and develop epilepsy despite no previous history of epilepsy.
NORSE vs FIRES
According to the newly-proposed consensus definitions of NORSE and FIRES, FIRES, Febrile Infection-Related Epilepsy Syndrome, is a subtype of NORSE that is preceded by a fever. (Article of proposed definitions is here.)
Important: FIRES is not the same as simple febrile seizures common in childhood which are considered benign and self-limiting. Previously, the term NORSE was used to refer to new onset refractory status epilepticus in adults, and the term FIRES was referred to the same condition in children. The thinking set forth in the newly-proposed definitions of NORSE and FIRES is that there are no age limits to either condition. Children can have NORSE and adults can have FIRES. Everyone with FIRES, by definition, also has NORSE. The distinguishing characteristic of FIRES is a fever starting between 2 weeks and 24 hours prior to the onset of refractory status epilepticus. NORSE has been observed in all ages but is seen predominantly in young adults and children.
FIRES had been considered a separate entity from NORSE until recently. Even though the proposed consensus definitions linking the two and placing FIRES as a subtype of NORSE have been published and reviewed and endorsed by the Critical Care EEG Monitoring Research Consortium, the diffusion of information is slow. There are still physicians who have never heard of NORSE or FIRES; there are physicians who still think FIRES occurs only in children.
Other terms for NORSE and FIRES
The term NORSE is a relatively new one. The first reference to NORSE in a medical journal was in 2005. Since then researchers have used various terms to describe this syndrome (a condition characterized by a set of associated symptoms): super-refractory status epilepticus, autoimmune encephalitis, de novo refractory status epilepticus or presumed encephalitis with refractory status epilepticus. The terms devastating epileptic encephalopathy in school-aged children (DESC), acute encephalitis with refractory, repetitive partial seizures (AERRPS) and infantile hemiconvulsion-hemiplegia and epilepsy syndrome (IHHES) have been used to describe pediatric illnesses similar to FIRES. The common element in all these terms is the cascades of seizures that seem to come from out of the blue in healthy people with no history of epilepsy, and the refractory nature of these seizures—they do not respond to traditional anti-epileptic drugs.
A report about NORSE written by Drs Gaspard and Hirsch filed with the National Organization for Rare Disorders (NORD) can be found here.
You can listen to an interview of Dr. Nicolas Gaspard about the nuanced changes in the conceptualization of NORSE reflected in the newly-proposed consensus definitions. We are grateful to Dr. Joseph Sirven, Editor-in-Chief of the Epilepsy Foundation's website, epilepsy.com, for conducting this interview as part of his Hallway Conversations series.
This site has integrated most of the research in abbreviated form for non-medical readers. You can read what physicians are reading about NORSE by going onto Pubmed which is an archive of the biomedical and life science journal articles at the U.S. National Institute of Health National Library. You can start your search here. A list of relevant publications curated by Dr Gaspard is also available on the Medical Professionals segment of this website.
Things to Consider:
Diagnostic Checklist, Medical Conferences, Palliative Care
NORSE is not a specific diagnosis. The proposed consensus definition defines NORSE as a “clinical presentation.” This assessment of NORSE is arrived at by a process of observations of the patient, extensive medical testing and elimination of other causes. This process takes time for tests to be administered, sent to laboratories (often off premises) and then the wait for results. The frustrating thing is that even if or when doctors conclude that the patient has NORSE, the precise cause of NORSE may not ever be identified, and a specific treatment protocol to follow has not been formally established for NORSE.
Many hospitals have their own protocol for treating refractory status epilepticus. The Medical Advisory Board of the NORSE Institute has generated a preliminary Diagnostic Checklist for NORSE. Both the checklist and a treatment protocol for convulsive status epilepticus developed at Yale-New Haven Hospital are available on the website’s Medical Professional resources which may be shown to your doctors. As a disclaimer, it should be clear that each patient requires treatment unique to that patient. These protocols developed by doctors who have experience treating NORSE are offered only as a starting reference point other centers may wish to consider.
Request Periodic Medical Conferences. A patient with NORSE symptoms will most likely be seen by a number of doctors: neurologists, specialists in critical care, epilepsy, nephrology and cardiology as the seizures, the anti-seizure and anesthetic drugs and the prolonged immobility can cause negative effects on the body. Try to identify the one or two doctors who lead the case to help you integrate and interpret their information. Ask for a medical conference with this lead doctor and other doctors key to the case to assess what is currently known and expected to happen. Understand that early in the case, the doctors are still struggling to identify the cause of the illness to determine the best treatment. That identification process is largely a process of elimination that takes time. Keep a daily record of your own observations and questions to ask at the conference. The condition of your loved one will likely change over time. Request additional medical conferences to be informed of the patient’s current condition and planned course of treatment.
Consider Involving a Palliative Care Team. A palliative care team can help support communication among the medical teams and between the doctors and the family. There are misconceptions on the part of both the public and doctors about palliative care. Many times a palliative care team or a palliative care approach is not called in until death is imminent. As a result, palliative care is often erroneously associated exclusively with end of life care. Palliative care is relevant when illness is life-threatening and life-altering for survivors. The World Health Organization’s definition of palliative care is “an approach that improves the quality of life of patients and their families facing the problem associated with life-threatening illness, through the prevention and relief of suffering by means of early identification and impeccable assessment and treatment of pain and other problems, physical, psychosocial and spiritual.” It “intends neither to hasten or postpone death “and is applicable early in the course of illness, in conjunction with other therapies that are intended to prolong life, such as chemotherapy or radiation therapy, and includes those investigations needed to better understand and manage distressing clinical complications.” Read full definition here
Full recovery from NORSE is possible. Requesting palliative care services does not negate that hope. Morbidity and mortality are also possible. Early involvement of a palliative care team can help doctors and families be on the same page regarding the patient’s current condition, and what to hope and plan for given the expected outcomes. It can be upsetting to hear that doctors are uncertain of the cause and the outcomes for a patient. NORSE is full of uncertainty for both families and doctors. You can read about the differing perspectives on communication here. Frequent and frank discussion of what is known and what remains uncertain may help doctors and families plan together for the best course of action for the patient.
Why did this happen?
No one knows for certain why NORSE strikes some people and not others. The exact cause of the disorder is similarly unknown and the best medical treatments for it are also not yet established. NORSE is a rare disorder with one estimate being around 3,200 cases per year in the U.S. Underreporting and misdiagnoses are likely since the term itself is new and not well established. There is no evidence as yet for a genetic basis for NORSE; and no reports of NORSE striking more than once in a family. As of now, there is no established biological marker that distinguishes NORSE as a specific disease. It’s possible that this presentation of symptoms is a syndrome resulting from a number of different causes.
NORSE Glossary of Terms
The following are terms that may come up in your conversations with medical staff that you should know. (The NORSE Institute is grateful to Erica Sieg, PsyD, Northwestern University Feinberg School of Medicine for providing this content.)
Terms often used in medical conferences
Acquired brain injury vs Traumatic Brain Injury: Acquired brain injury is an umbrella term for any type of injury to a person’s brain. Traumatic brain injury is a specific type of acquired brain injury that refers to some force external to the person that causes injury such as a car accident, fall, assault, gun shot etc. that damages the brain. Some examples of acquired non-traumatic brain injury (that occur from within a person) include stroke, brain tumors, encephalitis, and NORSE and FIRES among others.
Breakthrough Seizures: Sudden unexpected seizures in someone who previously had achieved reliable seizure control with medications.
Cognition: Broad term for mental abilities (“thinking”) related to information processing such as attention, processing speed, working memory, language, visual spatial, problem solving, planning, and memory.
Comorbidities: Additional illnesses or all the illnesses present in a person, not just the primary one being discussed. For example, someone may have epilepsy and also have comorbidities of depression, anxiety, or cognitive impairment. Comorbidities can be physical, cognitive, or psychiatric.
Electroencephalography (EEG) Monitoring: the most common way to monitor brain functioning for seizure activity. It does not hurt and is not dangerous as it is only a recording device for brain waves. It does not transmit, shock, or produce any kind of electrical current. Electrodes (small metal recording devices) are placed on the surface of the scalp and secured with a safe glue or tape. Once in place, electrodes measure the electrical activity in the brain through the skull. The electrodes send the information by attached wires to a computer to be recorded and analyzed by doctors.
Epilepsy: Epilepsy is a disease characterized by an enduring predisposition to generate epileptic seizures. A seizure is one event (see below) and epilepsy is the disease involving recurrent, unprovoked seizures.
Function: What someone can do; how well they can perform or complete tasks.
Neurobehavior: Behavioral, emotional, or personality changes associated with changes in brain structure or function. Examples are aggression, irritability, apathy, crying, sadness, hallucinations, eating more/less, sleeping more/less, repeating the same activity multiple times, or not engaging in normal activities.
Neuroimaging: Any procedure that provides visual information about the structure of the brain. This includes brain scans such as magnetic resonance imaging (MRI), computed tomography (CT), positron emission tomography (PET) scan or single-photon emission computerized tomography (SPECT) among others.
Neurologic state: The state of a person’s central nervous system which includes the brain and spinal cord. When a change in this system occurs, immediate medical intervention is needed because the central nervous system regulates important aspects of the body’s internal environment like breathing and heart rate, processes all sensory information such as sight and sound, and is responsible for conscious awareness, voluntary body movement, thinking, learning, and feeling.
Seizure: Abnormal excessive or synchronous neuronal activity in the brain. In other words, an episode when the cells of the brain are too active or start acting together in ways that are atypical. This interrupts normal brain functioning and produces the associated symptoms of changes in awareness, thinking, moving, speaking, or feeling (the brain’s normal processes). Most seizures do not last longer than 2 minutes.
Status Epilepticus (“Status”): A medical emergency defined as a continuous seizure lasting more than 5 minutes, or two or more seizures without full recovery of consciousness between any of them. Essentially, this is when seizures last too long or don’t stop. This is dangerous because it can cause significant brain damage or even death.
