About FIRES
FIRES, Febrile Infection Related Epilepsy Syndrome, is a rare and catastrophic neurological condition that affects the brain. It strikes suddenly causing explosive, relentless and difficult to treat seizures. It typically affects healthy children, although adults can be affected, with no history of epilepsy and they can experience hundreds of seizures a day in the acute phase of the condition.
It’s a life-threatening medical emergency. Prolonged seizures (over 30 minutes) can cause permanent neurological damage.
FIRES is considered a one-in-a-million condition. It’s rare and poorly understood making it exceptionally difficult to identify and treat, and right now, there’s still no cure. More funding is urgently needed to better understand it, find more effective treatments, and ultimately a cure. Increased awareness is key to advancing research and public awareness of it.
When a child is struck down by FIRES the impact is profound, turning lives upside down in an instant. The condition is cruel, relentless and drastically under-researched.
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What are SEIZURES?
A seizure is a sudden burst of electrical activity in the brain. It can cause changes in behaviour, movements, feelings and levels of consciousness. How a seizure affects you depends on what area of the brain is involved in the intense electrical activity. There are different types of seizures – head over to Epilepsy.org.uk to read more.
Epilepsy is defined as having two or more seizures at least 24 hours apart, that don’t have a known cause. Epilepsy is most commonly treated with medications that can prevent seizures.
Most seizures last from 30 seconds to two minutes. A seizure lasting longer than five minutes is normally considered a medical emergency. Seizures over 30 minutes in length can cause permanent neurological damage from the protracted abnormal electrical activity in the affected area of the brain.
Studies show the longer a seizure lasts, the more difficult it is to stop. The effectiveness of seizure medication declines as seizure duration increases. During prolonged seizures the brain releases pro-inflammatory cytokines leading to neuroinflammation and this inflammation can make the brain more prone to future seizures and increase the risk of developing drug-resistant epilepsy.
What Is FIRES?
FIRES a type of rare epilepsy that develops suddenly and explosively in children (although adults can be effected) following a minor febrile illness that quickly escalates into life threatening, treatment resistant, non-stop seizures. It’s thought to be an overwhelming inflammatory response to a minor viral infection, but the cause is yet to be identified.
FIRES is considered a sub-set of NORSE (New-Onset Refractory Status Epilepticus). The only difference between the two conditions is that FIRES occurs after a febrile illness, but without a clear infection or other known cause.
There is no specific treatment pathway that is universally effective. Children require weeks or months in a medically induced coma to try and protect the brain, undergoing intensive and complex treatments. Despite these efforts, most treatments have poor results, and in many cases the seizures can't be stopped.
Survival rates vary depending on the severity of the condition and the response to treatment. One in five children won’t survive the acute stage of the disease. Children who do survive are frequently left with significant neurological disability.
Little is known about FIRES. There is no clear cause, no definitive treatment and - most heartbreakingly - no cure. Whilst it is thought to be a rare immune/inflammatory response - research is only just beginning to uncover its mechanisms.
Because it's so rare, many people, including doctors, researchers and scientists are not familiar with FIRES. Raising awareness of FIRES is critical for early recognition, faster diagnosis, and timely intervention, which may help reduce its devastating impacts.
How Common Is FIRES?
FIRES is an extremely rare and considered a one-in-a-million condition.
It typically occurs in children, with an average age of 8 years old, although it can range from 2 years through to adulthood. Boys are slightly more commonly affected than girls. It can occur in adults, but more commonly impacts children.
Despite its rarity, the impact of FIRES is profound, and because awareness among the public and medical professionals is low, this can lead it to go unrecognised delaying effective treatments.
Stages of FIRES
Acute Phase (Initial Stage)
During the acute phase, seizures begin suddenly and explosively rapidly escalating into non-stop seizure activity that becomes increasingly difficult to stop. There is no obvious cause to the seizures, and they don’t respond to conventional first line treatments.
Children often require weeks or months in a medically induced coma to protect the brain, undergoing intensive, aggressive and complex treatments to try and stop the seizures. This phase can last for weeks or months, and patients will frequently develop numerous complications from not only the seizures, but the treatments.
FIRES is complex and life-threating and its management requires an aggressive, coordinated and individualised approach to improve survival and long-term outcomes. It involves multiple specialities working together to control seizures, manage complications and provide supportive care. It can include specialities from neurology, critical care, immunology and rheumatology, infectious disease specialists, anaesthesiology, nutrition and metabolic specialists, and palliative care.
Survival rates vary depending on the severity of the condition and the response to treatment. One in five children won’t survive the acute stage of the disease.
Chronic Phase (Long-term)
Surviving children and adults enter the chronic phase when the seizure activity decreases, and this is only the beginning of an exceptionally difficult road of recovery. The children frequently enter it with significant neurological disability and chronic life-long, difficult to control epilepsy, along with other complications that present significant neurological, psychological, and social challenges.
It's essential to understand that every person's experience with FIRES can be different.
Why Can’t FIRES Be Stopped Easily?
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Standard epilepsy treatments fail because the underlying mechanism is not a typical seizure disorder and is not definitively known, making treating it exceptionally difficult.
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The immune system itself appears to be causing damage, so suppressing seizures alone is not enough.
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By the time FIRES is diagnosed, brain damage may have already started, making it hard to reverse.
Why is FIRES so Damaging?
FIRES can lead to catastrophic, irreversible brain damage due to uncontrolled, prolonged seizure activity. This is further compounded by inflammatory processes as a result of the seizure activity. It is a neurological emergency and early recognition and aggressive treatment are critical. Brain damage can be the result of many factors such as:
Prolonged Seizures: Continuous seizure activity, longer than 30 minutes can cause permanent damage to brain cells (neurons) resulting in memory loss, cognitive decline, and motor impairment. Brain neurons simply can't handle prolonged electrical hyperactivity from extended seizures. The longer a seizure, the more damage it will do, and the more difficult it is too stop.
Brain Inflammation: Cytokines are part of the immune system. However, if the immune system overreacts (as is thought to happen in FIRES) it can produce a cytokine storm - releasing harmful inflammatory cytokines. These inflammatory cytokines can lead to brain swelling causing further neuronal damage.
Brain Shrinking & Scarring: Over time, the seizures from FIRES can cause shrinkage of brain structures (atrophy), and scar formation (gliosis), which can lead to permanent neurological disability and chronic drug-resistant epilepsy.
Long-Term Neurological Damage: Survivors often face lifelong drug-resistant epilepsy, cognitive disability, behavioural problems, and motor dysfunction - all significantly affecting quality of life.
Diagnosing & Treating FIRES
FIRES is not a standalone diagnosis but a collection of symptoms and clinical features that occur together but not necessarily have a single known cause. There is no clear biomarker to diagnose it – it is made clinically, based on a careful history. In summary:
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FIRES has a distinct clinical presentation - febrile illness followed by relentless seizures
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The underlying cause remains unknown in most cases (although immune and metabolic mechanisms are suspected)
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It is a diagnosis of exclusion - tests must be carried out to rule out other potential conditions or infections before considering FIRES.
There is no specific treatment pathway that is universally effective. Children require weeks or months in a medically induced coma to try and protect the brain, undergoing intensive and complex treatments. Despite these efforts, most treatments have poor results, and in many cases the seizures can't be stopped.
The NORSE Institute, in the United States, has a guideline for the investigation of FIRES which you can read here. They also have a recommended treatment algorithm for generalised convulsive status epilepticus in adults and children which you can read here.
These algorithms are also included in the paper published by Department of Neurology, King's College Hospital, London, UK which you can read here.
Living with FIRES: Ongoing Care
Children (and adults) who do survive are frequently left with long-term neurological disabilities including developmental delays, cognitive challenges, and motor difficulties and often need ongoing care such as physical, cognitive, and speech therapy. The are frequently also left with chronic, difficult to control epilepsy.
Managing drug-resistant epilepsy, cognitive impairment, and mental health issues requires multidisciplinary care to improve the quality of life for patients and their families.
But it’s not just the child who needs support. Families and caregivers go through so much during what will be a life-long journey, and emotional and psychological help is vital to help manage the stresses and challenges. Support groups or counselling can provide a space to connect with others in similar situations, offering comfort and understanding.
The child and their family are also at risk of post-intensive care syndrome (PICS). A concept whereby the critical care experience can have long-term deleterious health outcomes in both the patient and the family. Click here to read the article by Nora Wong, Founder of the NORSE Institute and Raquel Farias-Moeller.
Apply for Family Grants
If your child is experiencing FIRES, we want to help. Families can apply for a grant to support ongoing care and get the resources they need.
Reach out to us today to find out how we can assist you during this difficult time.
To apply for a family grant, please contact us on rachel@samssuperheroes.co.uk
NORSE Institute
The NORSE Institute, located in the United States, is a non-profit organisation dedicated to advancing understanding, research, and treatment of New-Onset Refractory Status Epilepticus (NORSE) and Febrile Infection-Related Epilepsy Syndrome (FIRES). They are the leading global body on NORES and FIRES. Their vision is to find an effective treatment for NORSE, and its subcategory FIRES. It was founded by Nora Wong, who lost her son to NORSE.
They have a medical and scientific advisory board supporting their work and a range of published resources to support both medical professionals and patients. This includes diagnostic evaluations, treatment recommendations, treatment algorithms, patient resources, family registry for patients to support research, and family support meetings.
Sam's Superheroes Foundation works closely with Nora Wong from the NORSE Institute, supporting their work in anyway we can. See our Resources and Support for Families section to learn more about their available resources.
Help Us Change This
Your donation can drive groundbreaking research, offer animal-assisted therapy, deliver creative therapy toolkits to children in hospitals, and provide essential financial grants to families navigating life with FIRES.
Every contribution matters. Whether it's a one-time gift or ongoing support, you're helping us bring hope, care, and progress to those who need it most.
Join us in honouring Sam’s legacy—together, we can make a real difference.
Resources and Support for Families
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FIRES Investigation GuidelinesClinical guidance for diagnosing and managing FIRES. Click here
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Treatment AlgorithmRecommended approach for treating status epilepticus in adults and children. Click here
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Discharge Planning GuideSupport for post-hospital recovery and long-term care. Click here
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NORSE Institute PodcastExpert discussions on FIRES, research, and patient experiences. Click here
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NORSE Institute Family RegistryA global database collecting patient data to improve understanding of NORSE/FIRES. Click here
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NORSE/FIRES BiorepositoryA Yale-led research initiative collecting biological samples for genetic and pathology studies. Click here
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NORSE RoundtableExpert discussions from the American Epilepsy Society Conference. Watch here
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NORSE/FIRES Conference 2023Family Conference (June 6, 2023) – Watch here
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NORSE/FIRES Symposium 2023Scientific Symposium (June 7, 2023) – Watch here
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Consensus Definitions (Epilepsia 2018)Standardised definitions of NORSE and FIRES. Click here
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About the Epilepsy Research InstituteThe central hub for epilepsy research, connecting experts, patients, and funders to drive innovation. Click here
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UK FIRES Research StudyLed by Dr. Sukhvir Wright and Dr. Marios Kaliakatsos, this study aims to develop new treatments and guidelines for FIRES. Click here
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Sam’s StoryMeet Sam, a FIRES Superhero, and learn about his journey with epilepsy. Click here
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Epilepsy Action (UK)General information on epilepsy and seizures. Visit site
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Epilepsy Foundation (USA)FIRES information and resources. Click here
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National Organisation for Rare Disorders (NORD)Advocacy and support for rare conditions. Click here
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ILAE Podcast (Sept 2020)Dr. Lawrence Hirsch and Dr. Nicolas Gaspard discuss NORSE research. Listen here