Epilepsy emergencies and how the prevent them Juliann

Epilepsy emergencies and how the prevent them Juliann M Paolicchi, MA MD , Northeast Regional Epilepsy Group

Epilepsy emergencies � Breakthrough seizures � Seizure clusters � Prolonged seizures (status epilepticus) � Sudden unexpected death in epilepsy (SUDEP) � Seizure-related injuries � Preparedness plans

Epilepsy emergencies � Perspectives ◦ Person with epilepsy (PWE) ◦ Caregiver ◦ Healthcare provider (nurse, MD) � How to recognize them? � What to do ? � How to prevent them? � How to prepare?

Breakthrough seizures � The severity and urgency depends on seizure type, seizure duration and external circumstances � Some seizures are more dangerous than others � Most seizures are self limited and there is no need for urgent intervention � Only a few need urgent care…and we need to be prepared for those

Breakthrough Partial Seizures � Simple partial (lower risk) ◦ Sensory, motor, auditory, visual, psychic, autonomic � Complex partial (moderate risk)

Breakthrough Generalized Seizures � Absence (lower risk) � Atonic (higher risk) � Tonic (higher risk) � Clonic/myoclonic (moderate risk) � Tonic-clonic (higher risk)

Breakthrough seizures � Safety � When � When measures to worry? to call 911? to go to the hospital? to call your doctor? to use rescue medications?

Safety measures � Clear the way: keep other people out of the way � Move objects that could injure PWE � Important to keep calm and track the time and characteristics of the seizure � Check if there is any information regarding seizure or epilepsy type on bracelet or necklace � Turn PWE on his/her side to keep airway clear � Cushion head � Loosen any tight neckwear 8

Safety measures � Do not try to stop movements related to the seizure or hold down PWE � Do not put anything in the PWE’s mouth during a seizure � After the seizure, remain with PWE until awareness of surroundings is fully regained 9

When to worry � First time seizure (since you do not know this behavior) � Seizures lasting more than a few minutes (5? ) � Repeated seizures without regaining consciousness � Increase in frequency of seizures � Different seizure types occur � PWE is injured, pregnant or with known associated medical condition � Seizure occurs in water � Difficulty breathing 10

What to do � When to call 911? (when you have any of the “worry” signs) � When to go to the hospital? (It is better to call 911 and have EMT trained personnel take care of the PWE) � When to call your doctor? (whenever you do not know what to do. However, if it an emergency call 911 first)

Breakthrough seizure-Rescue medications � What are rescue medications? � What are the side effects and possible complications? � Routes of administration: ◦ ◦ ◦ ◦ Oral medications Buccal Sublingual Rectal Intranasal Intramuscular Intravenous

Breakthrough seizure-Rescue medications � What are rescue medications? ◦ Medications that act very fast and can break seizures ◦ They are usually not effective as routine antiepileptic treatment ◦ It needs to be possible to administer them safely (oral, sublingual, rectal, nasal, intramuscular, intravenous) ◦ They need to be available all the time to PWE and caregivers

Breakthrough seizure-Rescue medications � What are side effects ◦ Sleepiness �May facilitate aspiration ◦ Respiratory depression �May cause breathing problems

Oral rescue medications � Ativan, Valium, Clonazapam � May be difficult to administer during a seizure � Very useful if there is an aura � Risk of injury by trying to put medication in mouth � Risk of aspiration � Never give liquids with medication during a seizure

Buccal rescue medications � Between gums and cheek � Problems: gagging, coughing and aspiration

Buccal route

Sublingual rescue medications � Clonazapam ◦ ◦ ◦ wafers Get absorbed faster than oral medications May be difficult to administer during a seizure Very useful if there is an aura Risk of injury by trying to put medication in mouth Risk of aspiration Never give liquids with medication during a seizure

Sublingual route

Diazepam Rectal Gel � Used in acute or emergency situations to stop a seizure that will not stop on its own � Approved by FDA for use by parents and nonmedical caregivers � State/school district regulations often govern use in schools 20

Emergency Treatment � Rectal Diastat ◦ Clinically proven ◦ Hard to give ◦ Adults don’t like it ◦ Can’t self administer

Intranasal route � Easy to administer � Increases production of nasal mucous and congestion

Intranasal Midazolam � Easy to give � Preferred route � Can be selfadministered or given by caretaker � Under study

Rescue medications-Intramuscular and Intravenous � Valium, Ativan, Midazolam (Versed) ◦ Rapid effect ◦ Needs caregiver to be trained ◦ Only in rare occasions ◦ Midazolam IM

How to prevent them � The seizure threshold ◦ What is the seizure threshold? �The amount of activity necessary to bring a seizure on. �We all have a seizure threshold �It is lower in PWE ◦ What can change it? ◦ The importance of knowing

How to prevent them � Seizure ◦ ◦ ◦ triggers: Missing medication doses (pill organizers, alarms) Alcohol and drugs Stress Environmental temperature Lights Fever/illness

How to prevent them � Seizure triggers: Hormonal changes Hyperventilation Sleep deprivation Medications and supplements (very important to discuss with your doctor every time you take any new medication for any reason or any supplements —many can provoke seizures) ◦ Travel across time zones ◦ ◦

Seizure clusters � What ◦ ◦ are seizure clusters They start and stop but occur one after another The can last a very long time They can lead to injuries and complications They need to be treated aggressively

Seizure clusters � Types ◦ ◦ ◦ ◦ of medications Oral Buccal Sublingual Rectal Intranasal Intramuscular Intravenous

Status epilepticus � What is status epilepticus? � What to do? � Why? � What are the consequences if we do not act in a timely manner?

Status epilepticus � Formal Definition: seizures that do not stop for 30 minutes. Or they happen on and off without regaining consciousness between seizures � Practical definition: 5 minutes

Status epilepticus � Types ◦ ◦ Partial motor status epilepticus Generalized convulsive status epilepticus Non-convulsive status epilepticus Myoclonic status epilepticus

Partial motor status epilepticus � This prolonged seizure involves just one part of the brain. � The person is awake and talking/interacting normally, but has persistent rhythmic jerking on one side of the body, say the hand, arm or face. � It requires emergency treatment, but is not usually as life-threatening as other forms.

Generalized convulsive status epilepticus � This prolonged seizure involves the entire brain, and produces convulsive activity in all four extremities coupled with a lack of responsiveness. � This life-threatening condition requires urgent medical evaluation and treatment.

Non-convulsive status epilepticus � This seizure, which could involve part of the brain or the entire organ, is far less dramatic than generalized convulsive status epilepticus, and produces subtle symptoms such as blinking, staring or confusion – or no obvious signs at all. It is less dangerous than the generalized convulsive type, but still requires prompt recognition and treatment. � A continuous EEG recording is the only way to diagnose non-convulsive status epilepticus.

Myoclonic status epilepticus � Another seizure that involves the entire brain, this form produces prolonged jerking of all four extremities. � It is usually caused by a profound lack of oxygen to the brain due to heart dysfunction, but may also occur in those with myoclonic epilepsy.

Status epilepticus: Epidemiology � One of the most common life-threatening neurological disorders � Incidence: 50, 000 to 200, 000 cases annually in US. � Around 12% of patients with newly diagnosed epilepsy present with status epilepticus � Within 5 years of initial diagnosis of epilepsy, 20% of patients will have status epilepticus � Mortality rate: 3 to 53% (20%) � 55, 000 deaths in U. S. per year

What can cause it? � Cause: � Age: Unknown in 25 to 40% of cases elderly > pediatric > adult

What can cause it? � Most ◦ ◦ ◦ ◦ common causes: Missing medications Stroke Alcohol withdrawal Metabolic disorders Hypoxia Infections Tumors Trauma

What are the consequences? � Effects on the body ◦ Hyper-sympathetic state (increased HR, dysrhythmias, decreased cardiac output, increase in peripheral resistance, increase in BP followed by decreased BP) ◦ Hyperpyrexia (increased body temperature) (central, infection or increase in muscle activity)- neuron damage ◦ PH decreases ◦ Hyperglycemia (increase in catecholamines)

What are the consequences? � Effects on the brain ◦ Early stage: �Increased oxygenation �Increased blood flow (increased BP) ◦ Late stage: �Decreased oxygenation �Decreased blood flow �High requirement of energy with low supply brain injury �Decreased glucose and increased lactate

How is status epilepticus approached? � Clinical assessment: ◦ Exam: trauma, infection ◦ Drugs: ciprofloxacin, baclofen, flumazemil, interferon, ifosfamide, theophyline, isoniazid, alcohol withdrawal, cocaine ◦ Infections: Mycoplasma pneumonia, catscratch encephalopathy, herpes simplex, AIDS ◦ Tumor (metastases), cortical dysplasia

How is status epilepticus approached? � Labs ◦ Glucose level, electrolytes, CBC, toxic screen, LFTs, AEDs levels, urine, ABG (hyponatremia, hypercalcemia, hepatic encephalopathy) ◦ EKG ◦ LP: if infection suspected � Neuroimaging ◦ CT/MRI once stablized

How is status epilepticus approached? EEG and VEEG ◦ Rule out psychogenic seizures ◦ Classify type of SE

How is status epilepticus treated? is an emergency where time is of the essence � Steps: � This ◦ ◦ ◦ Control of airway and ventilation Arterial blood gas monitoring EKG and BP monitoring IV: glucose and Thiamine Blood work: CBC, CPM, electrolytes and AED levels

Treatment � Pharmacological treatment ◦ Benzodiazepines loading �Lorazepam �Diazepam ◦ Phenytoin or Fosphenytoin loading ◦ If no response: �Phenobarbital, Depakon, Keppra, Vimpat ◦ Refractory: �ICU: midazolam, propofol and pentobarbital

Status epilepticus-Conclusion � EMERGENCY � TIME SITUATION IS OF THE ESSENCE � DELAY IN TREATMENT COULD RESULT IN BRAIN DAMAGE OR DEATH

SUPED � What is SUDEP? ◦ SUDEP = Sudden Unexpected Death in Epilepsy. ◦ SUDEP could be the possible cause of death when there is no evidence of trauma or drowning and there is no other clear cause of death (Heart attack, etc. ) ◦ SUDEP is believed to be the cause of approximately 10% of seizure related deaths.

SUPED ◦ Unfortunately, due to the unpredictable nature of SUDEP it remains an understudied phenomena. ◦ Our understanding of this process is very limited and much remains to be investigated about these occurrences and what causes them. ◦ Thankfully, SUDEP is relatively rare, occurring in about 1 out of 1000 patients with epilepsy per year, but its consequences can be catastrophic

Who is at risk? � All patients with Epilepsy are at some risk � Higher risk: ◦ Uncontrolled seizures (risk of 1 in 150) ◦ Patients with generalized tonic-clonic seizures. ◦ SUDEP also appears to typically affect younger adults with epilepsy. � (Approximately 75% of all SUDEP deaths occur in individuals between 20 to 50 years of age) � Children, have a relatively lower risk of SUDEP.

Who is at risk � Patients with varying degrees of cognitive or neurological impairment. � Poor compliance with medications � Use of alcohol or illicit drugs � Nocturnal seizures

What causes it? � Not completely understood, but there are several theories: ◦ There is interruption of cardiac (cardiac arrest, arrhythmias) or respiratory function ◦ The brain is highly interconnected with the heart and respiratory functions ◦ Seizures could disrupt that connections

SUDEP-What can you do � Patients and families must work together with their doctors to obtain optimal seizure control. � Taking anti-seizure medication consistently and regular follow ups with the patient’s health care provider are key. � Autopsies show that many of those who die from SUDEP have low levels of antiepileptic medications in their system.

SUDEP-what can you do? � Maintain a regular sleep schedule (including when traveling across time zones) � Avoid alcohol and illicit drugs � There is a growing number of safety devices that have appeared on the market (none are FDA approved however)

Anything else to prevent it? � Devices to monitor seizures to alert caregivers when a seizure is happening � They achieve this by recognizing rhythmic movements or detecting changes in heart rate which can occur during a seizure � Low tech options include baby monitors to other more sophisticated devices � Seizure service dogs

Limitations of these devices � Failure to detect all seizures � False alarms: ◦ occur when the bed exit alarm function is in use and the patient gets out of bed to go to the bathroom. ◦ Other false alarms can occur if an individual is particularly restless at night

Safety Devices to Prevent SUDEP � There is no device proven to prevent SUDEP � Some devices are marketed but have not been studied � Some � Speak devices are currently under study to your MD before purchasing

Smart. Watch by Smart. Monitor

High Tech?

SUDEP � Knowledge of SUDEP and the factors that are thought to increase the chances of being affected by it are crucial to its prevention. � It is important to develop a management plan for the seizure events, and family and friends should learn basic life support skills.

Seizure-related injuries � Increased incidence of head and soft tissue injuries � Tongue and mouth lacerations � Submersion (10 fold) � Fractures (2 fold) � Burns (3% of burn units admissions) � Car accidents � Sport related injuries

Post seizure-related injuries Confusion: may walk into a dangerous area � Aspiration pneumonia �

THE SEIZURE PREPAREDNESS PLAN � In Case of Seizure: � Please keep calm and stay with me until the seizure ends. � These symptoms/behaviors will tell you that I’m having a seizure: (list specific characteristics of your seizures, for example, falling, jerking limbs, etc. ) ________________________________ __ The things you should do to ensure my safety are: (for example, gently move me away from danger, if possible; loosen any restrictive clothing, etc. ) ______________________________________ � Please do not put anything in my mouth during the seizure! �

THE SEIZURE PREPAREDNESS PLAN � Please observe me carefully so you can describe everything you saw during the seizure. I’ll report what you’ve said to my doctor and it may help with my treatment. � Please call 911 if the seizure is prolonged (lasts longer than two to three minutes), is associated with breathing difficulties, causes injury, or becomes a series of seizures. Brief seizures that end spontaneously without injury do not require a 911 call, but may require a call to my doctor. My doctor’s phone number is: __________________________

THE SEIZURE PREPAREDNESS PLAN � � � If several seizures occur in a row, please give me my rescue seizure medication as follows: (list instructions obtained from your doctor. If you wear a VNS, include instructions to swipe it once over the implant. ) __________________________________________________________________ After the seizure, please help me find a place to rest. It is also important that I get regular meals and take my seizure medications on schedule. If you have a school-age child with epilepsy, the Seizure Preparedness Plan should be given to the school nurse or other appropriate school official, as well as the teachers, coaches, camp director, camp counselor, babysitters and anyone else who may be caring for the child.

Safety at Home � � Make your home as safe as possible by doing the following: Make sure that your floors are carpeted any sharp corners (e. g. , table corners) are padded to reduce the risk of injury due to a fall. � Don’t smoke. � Don’t light a fire or a candle when you are home alone. � � Make sure the drains in your bathtub and shower are working properly to prevent drowning should you lose consciousness while showering. Set your water temperature to a moderate level to avoid being scalded if you lose consciousness while running the hot water.

Safety at Home � � � � Don’t take a bath in deep water, to prevent drowning. Don’t lock the bathroom door, use an “occupied” sign on the doorknob instead. Install a bathroom door that opens outward for easier access, in case you have a seizure and fall against the door. Use plastic glasses and dinnerware instead of glass and china to keep from cutting yourself if you lose consciousness while holding them. Some people also use medical alert systems that notify emergency personnel that they’ve fallen and need assistance. Baby monitors can be helpful to parents of babies or young children who have epileptic seizures during sleep, as they can pick up unusual sounds. I’m often asked about epilepsy detectors, which are devices that monitor breathing, and/or detect urine or vomit in the bed and send warning signals if something is amiss. While it’s an intriguing idea, none of them are FDA-approved for home use.

Safety at home

Safety Away From Home � � Always tell your family and/or friends where you are going and when you expect to return. Wear a Medic-Alert bracelet and/or jewelry printed with your medical information. � Put your emergency contact numbers on speed-dial on your cell phone. � Don’t drive without medical permission. � Keep a supply of rescue medication on hand. � Stay away from the tracks at train and subway stations. � If you fall frequently during seizures, consider taking an elevator instead of the stairs or an escalator.

Safety away from home

General Travel Guidelines � � � Give your friends and family a copy of your travel itinerary, with phone numbers and addresses where you can be reached. Become familiar with the hospitals in the areas you are visiting, in case of emergency. Bring an adequate supply of seizure medication with you. Carry on the plane with you. Do not put in luggage. If the trip is a long one, consider finding a medical provider in the area to provide refills. However, be aware that not all countries have access to every seizure medication prescribed in the United States. Find out in advance which ones are available, and talk to your doctor about other medicines that are acceptable. Longer flights and jet lag can cause disrupted sleep, which can trigger a seizure. Talk to your doctor about getting a prescription sleep aid for the trip.

General Travel Guidelines � Avoid excessive alcohol. � Eat regular meals. � � � Don’t forget to consider time zone changes when taking your medications. Take the medication as close as possible to the time you would be taking it at home. If you travelling to a foreign country, consider learning basic phrases to request medical assistance such as “I need help” and “Where is the hospital? ” Even better, travel with someone who knows the native language. Prepare a plan for an emergency trip back home. Discuss this plan with someone you trust before you go.

Traveling Alone � � � � Wear a Medic-Alert bracelet/necklace that includes a description of your seizure(s). Carry a list on your person of all of your current medications (plus enough medicine to last you from start to end of your trip). Carry emergency contact information on your person, either on an index card or numbers programmed into your cell phone and listed under contacts as ICE (In Case of Emergency). Disclose your seizure history to transportation personnel and provide them with a letter from your doctor. Let the flight attendants, conductor or driver know that you have epilepsy. If you have a VNS device implanted, carry a VNS registration card so that people will know that you cannot get an MRI, should not have deep heat treatment and so on.

SAFETY PRECAUTIONS WHEN ENGAGING IN SPORTS ACTIVITY Baseball Basketball Bike Riding PRECAUTIONS ● Wear protective clothing: � elbow or knee pads � helmet � protective eyeglasses or goggles ● Wear protective clothing: � elbow or knee pads � consider a helmet � protective eyeglasses or goggles ● Avoid busy streets ● Ride on bike paths ● Ride on side streets ● Wear a helmet

Boxing ● High risk activity - should be avoided by all Bungee Jumping ● High risk activity - should be avoided by all Canoeing/ Kayaking Football Gymnastics ● Never canoe/kayak alone; take a “buddy” who knows seizure first aid. ● Always wear a high quality, well-fitting life vest when near the water to prevent drowning. ● Wear protective clothing: � elbow or knee pads � helmet � protective eyeglasses or goggles ● Have a “buddy” when using equipment like balance beams, parallel bars or when vaulting ● ● Consider a helmet when using a balance beam or parallel bars or when vaulting Consider a shock-absorbing mat Take frequent breaks Keep hydrated

*Hang Gliding ● High risk activity - should be avoided by individuals with uncontrolled seizures Horseback Riding ● Wear protective clothing: � elbow or knee pads � helmet �protective eyeglasses or goggles Ice Hockey ● Wear protective clothing: � elbow or knee pads � helmet �protective eyeglasses or goggles Jet Skiing ● High risk activity - should be avoided by individuals with uncontrolled seizures

Martial Arts: Karate, Tai Kwando, Judo ● High risk activity - should be avoided by individuals with uncontrolled seizures *Mountain Climbing ● High risk activity - should be avoided by individuals with uncontrolled seizures Pilates ● Consider a shock-absorbing mat ● Have a ‘buddy” when using equipment ● Take frequent breaks ● Keep hydrated *Rappelling ● High risk activity - should be avoided by individuals with uncontrolled seizures *Rock Climbing ● High risk activity - should be avoided by individuals with uncontrolled seizures Rollerblading ● Wear protective clothing: � elbow or knee pads � helmet �protective eyeglasses or goggles

Rugby ● Wear protective clothing: � elbow or knee pads � helmet � protective eyeglasses or goggles *Scuba Diving ● High risk activity - should be avoided by individual with uncontrolled seizures Skateboarding ● Wear protective clothing: � elbow or knee pads � helmet �protective eyeglasses or goggles Skiing ● ● ● Dress for warmth Wear protective gear Consider a safety strap when riding the t-bar Have a “buddy” Don’t go off open trails

Snorkeling ● High risk activity - should be avoided by individuals with uncontrolled seizures *Skydiving ● High risk activity - should be avoided by individuals with uncontrolled seizures Soccer ● Wear protective clothing: � elbow or knee pads � helmet � protective eyeglasses or goggles

Surfing/Wind Surfing Swimming ● High risk activity - should be avoided by individuals with uncontrolled seizures ● Never swim alone. Have a “buddy” who knows seizure first aid ● Always wear a high-quality, well-fitting life vest when near the water to help prevent drowning. ● Inform the lifeguard about your condition if swimming in a pool Tai chi ● Consider a shock-absorbing mat ● Take frequent breaks ● Keep hydrated Yoga ● Consider a shock-absorbing mat

Driving � Follow � If state laws you have regained your driving privileges, be safe and avoid driving if you are tired or have any known risks for seizures � Don’t hide seizures from your doctor to avoid losing your driver’s license

Final words � Complications � But, and emergencies are rare always be prepared!