Patient Resources
Supportive Listeners
While patients are in the ICU, their families are often overwhelmed by the acute, surreal situation of NORSE. Having someone to talk with who knows the experience of waiting for the uncertain outcome of NORSE can be helpful.
Listeners are matched to family members asking for a sympathetic ear. Listeners do not offer medical advice, simply the willingness to listen while the family members vent their anguish and repeat the details of the illness. Finding the words to tell their story helps the family to process the seemingly unreal situation of NORSE.
Families can request a listener on the contact page.
A printable brochure about NORSE is available here for your families.
Planning Guide and Glossary of terms for life after hospitalization
(The NORSE Institute is grateful to Erica Sieg, PsyD, Northwestern University Feinberg School of Medicine for providing this discharge planning content.)
Doctors often cannot make specific predictions regarding prognosis, outcomes, or long term needs of NORSE and FIRES patients. They simply don’t know enough about the illness. But NORSE and FIRES patients almost always need some form of medical care after being discharged from the hospital. The type and extent of care differs for each patient.
Patients and families may not expect that after hospitalization, another “battle” or struggle often begins. Many patients will have acquired some degree of brain injury during the acute phase of NORSE or FIRES as a result of their brain seizing for a long period of time. Most patients continue to have seizures. Even though they did not have epilepsy prior to NORSE or FIRES, these continuing seizures mean that they have developed epilepsy. This epilepsy often combined with other lasting effects of brain injury can change the lives of patients and their families in radical and difficult ways. The following is a guide to resources that can help patients and their families move forward across a range of situations. It is best to read through the checklist prior to the patient’s discharge from the hospital so you can enlist the help of medical staff. They may be harder to reach once you leave the hospital.
Recovery is a difficult to predict on an individual basis for patients with NORSE and FIRES. And there are always individual exceptions. You should ask the doctors most involved in your patient’s case to give you their best estimate of prognosis, or how they expect the patient’s mental, emotional and physical abilities to be affected by the illness. Ask them in what areas they expect improvement and over what time frame.
At the end of this section there is a glossary of terms you may find helpful in your discussion with medical staff
Hospital Discharge Planning Checklist
Epilepsy Team
A physician from the patient’s Epilepsy team, familiar with the case should continue to provide care after hospital discharge. That person will help with seizure management and coordinate care in the event of relapse. If that physician is not able to continue to provide continued care, another epilepsy specialist who can provide continued care to the patient must be identified prior to leaving the hospital.
An outpatient follow-up appointment, prescription information, and epilepsy staff contact information (such as a nurse you can contact with questions or updates) should be arranged before the patient leaves the hospital.
Ask about the need for your patient to have any at-home tests or monitoring that in the future (e.g. at-home or “ambulatory” EEG to make sure seizures are still under control once out of the hospital, blood work to ensure accurate medication levels etc.) and how to obtain those resources
Ask about “red flags” to look out for which might indicate worsening neurologic status or the need to come back to the hospital urgently or call 911.
Ask about relevant health and safety procedures to be used at home (such as modifications to living or sleeping arrangements, use of pill boxes, dietary restrictions, driving and activity restrictions, recommended level of oversight, etc.).
Social Work Resources
Social Workers probably have been involved in your patient’s case. They can be a valuable resource to help find services, providers, and information regarding medical, financial, legal, insurance, and social needs specific to the patient and particular to the geographic area where the patient lives
Because NORSE and FIRES are rare conditions, there are no established resources specifically aimed at this disorder. Social workers can help these patients utilize well established resources for neurological conditions such as stroke, traumatic brain injury, or dementia.
Often, a specific diagnosis like a “Major Neuro-cognitive Disorder” or “Acquired Brain Injury” is necessary to receive services or qualify for certain legal recourse. This diagnosis can be found in the Neuropsychological Assessment Report, or by requesting it from the Epilepsy doctor.
Ask Social worker to obtain specific diagnosis if relevant and necessary to receive these medical and legal services.
Financial burden after such a significant medical event like NORSE or FIRES can be crippling to patients and families.
Ask Social worker to help apply for institutional and governmental aid to help ease some of the financial burden or to make a more manageable financial plan. Sometimes the hospital’s Department of Financial Affairs may suggest solutions and options.
Ask what medication and physical equipment the patient will need the first week upon discharge from the hospital or rehab center. Make sure you have those items in hand before the patient leaves the hospital or rehab center.
Legal Services
Patients and families may already be aware of Health Care Power of Attorney (POA) stipulations during the hospital stay. After discharge, further legal actions may be needed if a patient is at or beyond the age of legal adulthood and has sustained a moderate to severe brain injury and is need of assistance more globally or long term. Sometimes Guardianship (where a person becomes legal guardian of another) is a reasonable course of action. Similarly, applying for and obtaining legal disability, is often necessary for a period of time, or permanently.
Rehabilitation
Rehabilitation is overseen by a medical doctor specializing in physical medicine and rehabilitation (PM&R), often called a Physiatrist. It typically includes physical therapy (PT), occupational therapy (OT), and speech and language pathology (SLP), all working together. Generally, a patient is evaluated for rehabilitation needs, including a brief screening of cognitive functioning (thinking abilities) prior to leaving the hospital.
Purpose: To improve cognitive and physical functioning where possible, gain an understanding of safety needs, and implement safety and activities of daily living (ADL) strategies. Area’s addressed include basic functions like walking, talking, and taking care of oneself (using the bathroom, eating, getting dressed, etc.).
Inpatient Rehabilitation: Patient lives in a rehabilitation facility. Typically, for patients with moderate to severe injuries who need more help to establish safe mobility and basic activities of daily living (ADL’s) prior to returning home. This can be due to body de-conditioning (strength is lost from being physically immobilized for so long or brain damage associated with prolonged seizures.
Outpatient Day Rehabilitation: Patient lives at home and receives care at a medical facility. Typically for patients with mild to moderate injuries where a person is safe to return home, but would still benefit from additional comprehensive PT, OT, SLP therapies and strategies for daily living.
Outpatient Cognitive Rehabilitation: Patient lives at home. Typically for patients who can already complete all prior activities independently from a mobility standpoint. Cognitive Rehabilitation focuses on higher level skills such as attention, memory and language deficits.
Referrals can be requested from the Epilepsy team, Epilepsy Social Worker, or Rehabilitation Hospital. Cognitive rehabilitation specific to NORSE and FIRES is unlikely, but services for brain injury, stroke, and dementia can be appropriate treatment for NORSE and FIRES patients.
Neuropsychological Assessment
This assessment is overseen by a Neuropsychologist, a psychologist who specializes in assessing brain function, typically in the setting of neurologic illness or disease. The assessment is non-invasive, there are no physical side effects or dangers. A technician administers paper and pencil, question and answer test, and some computerized tasks designed to make the patient’s brain use attention, memory, language, and visual spatial abilities. A patient’s performance is statistically compared to a registered group of “normal” healthy individuals to determine if there are any deficits, and the information is provided to patients and their families during a feedback session at a later date.
Purpose: To understand the nature and extent of any residual brain dysfunction, guide treatment planning and make recommendations for additional recovery and interventions to optimize functioning. Based on level and type of impairment, this can include referrals to Social Work, Neuropsychiatry, Cognitive Rehabilitation programs, Vocational Rehabilitation programs etc. Areas addressed generally include returning to school, work, ability to live or raise children independently, drive, make complex medical decisions, remain in charge of complex finances, evaluate behavioral and psychiatric needs, etc.
Timing: In rare settings, neuropsychological assessment can be available while a patient is hospitalized, but it is most typically completed after a person leaves the hospital in an outpatient setting. This is because medical stability is necessary, or neuropsychological results will not be reliable or relevant for any length of time. The timing of outpatient neuropsychological assessment often depends on the severity of initial injury. For mild injuries, neuropsychological assessment can occur right after hospitalization and be the first step to guide recovery and return to functioning. For moderate to severe injuries, rehabilitation is often attempted first allowing time for some initial recovery, with full neuropsychological assessment at 3-6 months to then further guide more long-term planning.
If not provided, patients and families can ask their Epilepsy team about referrals to a neuropsychologist in their hospital or region.
Neuropsychiatry Assessment
A Neuropsychiatrist is a Psychiatrist (medical doctor) who specializes in prescribing medications to support thinking, mood and behavior of individuals who have neurological illness. They have the training to assess the unique needs of NORSE and FIRES patients and their families. They can prescribe medication to support rehabilitation whereas the neuropsychologist cannot.
Timing: In some cases, a Neuropsychiatrist can be consulted prior to the patient leaving the hospital. Generally, this visit is completed as outpatient follow-up appointments every 3-6 months intervals of 3-6 months.
Discuss with the primary epilepsy doctor whether the patient might need a neuropsychiatry consult. If the answer is yes, get a referral for one.
Family & Caregiver Supports
Patients and families impacted by NORSE and FIRES often benefit from counseling to adjust to the changes and receive additional emotional and problem-solving support. Acceptance and Commitment Therapy (ACT) is a form of psychotherapy that teaches healthy adaptation to difficult circumstances, and may be particularly beneficial for family members, and high functioning patients. Patients with more moderate to severe injuries may need to work with a dedicated Neuropsychologist, or Rehabilitation Psychologist specialized in helping patients and families with the cognitive, emotional, and psychosocial aspects following a major neurologic event, often through behavioral and environmental modifications.
Sometimes, the patient’s needs are so significant, family structures are entirely up-ended in heart breaking and unforeseen ways (e.g. an adult child is now dependent on their aging parents, divorce occurs from a now disabled individual, young children need to be raised by others because the parent is disabled, a young child ). Navigating these challenges and decisions is difficult, time consuming, and often slow moving. A final “structure” can take a few years to establish before it becomes a more normal and settled routine.
Caregiver burn out is very applicable to families who have cared for patients with NORSE or FIRES. Caregivers need to remember to attend to their own medical and health needs, in order to continue to support their loved one. Caregivers are encouraged to obtain their own support, divide responsibilities with other family members or friends, utilize all available social or government agencies or funds. Support groups for epilepsy, brain injury, stroke, and dementia may all be applicable as they share commonalities.
Making long term plans for a patient with NORSE or FIRES is often recommended. Planning ahead for the event of relapse, disability, or further decline in terms of family structure and response can ease the panic and burden at that difficult time should it ever occur and give families peace of mind.
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. 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 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, but also have comorbidities of depression, anxiety, or cognitive impairment. Comorbidities can be physical, cognitive, or psychiatric.
Electroencephalography (EEG) Monitoring: EEGs are 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 devise for brain waves. It does not transmit, shock, or produce any kind of electrical current. Electrodes (small metal recording devises) are placed on the surface of the scalp and held on 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 has provided visual information about the structure of the brain. This includes brain scans such as magnetic resonance imaging (MRI), computed tomography (CT), or single positron emission 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.
