“This meeting lays bare the true risk of living with epilepsy.” – PAME co-chair Gardiner Lapham

I attended my first PAME meeting in 2022. That was a tough meeting, and I cried. Several parent advocates spoke about their own experiences of losing a child to Sudden Unexpected Death in Epilepsy (SUDEP). However, in 2023, I was more emboldened by their voices. These caregivers come to this meeting year after year to beg medical practitioners to inform their patients about the risks of death due to epilepsy.

Are they listening?

This is (some of) what I learned:

Nobody wants to hear that death is a risk, but anyone can die from epilepsy. The real tragedy is if the child dies and the parent feels like there’s something that they could have done that they didn’t do.

Professor Helen Cross, President of the International League Against Epilepsy, led the session with her keynote address. She highlighted that epilepsy is a significant contributor to premature mortality (early death) worldwide – a person with epilepsy has a 2-4 times greater risk of dying compared with the general population. Even people with no known cause for their epilepsy, people with epilepsy have reduced life expectancy.

Why do people die due to epilepsy?

• Increased mortality may be related to the underlying cause of epilepsy.
• There are higher rates of suicide among people with epilepsy, adverse reactions to drugs, and side effects that can cause psychiatric or medical disorders.
• Status epilepticus, which is a prolonged convulsive seizure lasting more than five minutes or a nonconvulsive seizure of more than 10 minutes, can result in death.
• Sudden Unexplained Death in Epilepsy (SUDEP) – As many as one in 833 people living with epilepsy each year die of SUDEP. Higher seizure frequency, tonic-clonic seizures, and nocturnal seizures place you at higher risk, as do genetic mutations like Dravet Syndrome. However, SUDEP can occur across the spectrum of epilepsies. Emergency medical personnel (EMTs, ER doctors) may not be well-versed in therapies to stop seizures or may fear administering them.  
• Seizures cause loss of breathing in 33% of cases.
• Seizures can lead to a loss of awareness and body control, which can lead to accidental deaths such as falls, burns, car accidents, aspiration pneumonia, choking, or drowning. After SUDEP, drowning in the bathtub was the most frequent epilepsy-related cause of death. The most significant risk factor for motor vehicle accidents is not complying with the physician’s recommendation not to drive.
• Children are at the highest risk of SUDEP or other causes of epilepsy-related death when they have more than one condition (such as epilepsy and cerebral palsy or epilepsy and intellectual disability).
• Most deaths in children with epilepsy are pneumonia-related: aspiration (choking) may occur between seizures and every time they have a seizure, they can have microaspiration, which results in chronic lung disease and can lead to acute aspiration.

SUDEP rates are higher in patients with drug-resistant epilepsy. It is estimated that 1 in every 150 people with drug-resistant seizures die of SUDEP each year.

How do we prevent this?

The American Academy of Neurology and the American Epilepsy Society recommend that people with epilepsy and their caregivers are informed about SUDEP soon after an epilepsy diagnosis. There was some debate about what “soon after diagnosis” means. When would you want to know about this risk? 

Clinicians have been resistant to informing patients about SUDEP and other causes of death due to epilepsy due to fear of causing anxiety or because they don’t understand that SUDEP is not that rare. Self-management skills improve when the patient and their family know about the risks. And ultimately, patients have a right to know. 

Seizure control remains the cornerstone of prevention!

The number one way to prevent epilepsy-related mortality is to stop the patient’s epilepsy, especially convulsive tonic-clonic seizures. This means exploring all of the options to treat the patient’s epilepsy. 

Receiving care at a comprehensive epilepsy center can decrease mortality risk by 50%. 

These centers focus on treating children who continue to have seizures after trying multiple medications. They can have a more comprehensive view of the patient by accessing multidisciplinary subspecialty clinics (think about genetic testing; pre-surgical evaluations with MEG, SEEG, SPECT, WADA, and more; dietary therapies; orthopedists; neuropsychologists, and other specialists).

Epilepsy surgery and SUDEP.

At the very least, patients should be referred to a Level 4 epilepsy center for a surgical evaluation as quickly as possible once thought to be drug-resistant (meaning that they have failed to achieve seizure control after two appropriate medication trials or after they have failed one medication if they have brain malformations or lesions).

• Epilepsy surgery results in a 2x lower risk of death and a 3x lower risk of SUDEP.
• Long-term, vagus nerve stimulation may have an additional cardioprotective effect.
• Neuromodulation lowered rates of SUDEP.

Suggestions to protect your loved one with epilepsy:

• Ensure that they are taking their medications as prescribed;
• If they are drug-resistant, make sure they get a surgical evaluation at a Level 4 epilepsy center to learn about surgical options that may stop or reduce their seizures. Some patients require second or third opinions to find the right treatment option – this stuff is complicated!
• Have rescue medications and a seizure action plan – and use them! 
• Improve sleep, reduce sleep apnea and sleep-disordered breathing; have a sleep study if unsure;
• Ensure a seizure-safe home. Enhance seizure monitoring for high-risk individuals such as sharing a bedroom, using seizure detection devices or pulse oximeters, using anti-suffocation pillows or no pillows;
• Take precautions outside the home. If seizures are uncontrolled, they should not drive or operate heavy equipment, swim alone, or ride a bicycle.

There was a long discussion about the side effects of anti-seizure medications. We know that seizures are not just bad for the brain – they can lead to autonomic dysfunction and more (as highlighted above). Treating seizures is good for the brain and body.

But no decision you make is without risk. Epilepsy medications can result in comorbid psychiatric or somatic disease, premature mortality, heart arrhythmias, cardiac diseases, metabolic disorders, psychiatric adverse effects, and impact bone health. We need to learn more about the effects of these anti-seizure medications, and patients need to be informed about the risks.

STOP SUDEP campaign

Iris-Maria Killinger of the Oskar Killinger Foundation opened the 2022 PAME meeting with the story of her son’s tragic death due to SUDEP. She is a German human rights attorney who has launched a powerful STOP SUDEP campaign.

“These patients need to be heard, to be seen, and to be taken seriously.” – Iris-Maria Killinger

Finally – what about research? 

Epilepsy is the lowest funded of all neurological conditions. How do we move the needle forward on this potentially deadly condition without sufficient funding? Groups like the Epilepsies Action Network are trying to drive more funding, but more work needs to be done.

For more information:

• Program from the 2023 PAME meeting
• Partners Against Mortality in Epilepsy website
• Creating a Safe Home Environment for Seizures, Epilepsy Foundation
• STOP SUDEP campaign
• Preventing Epilepsy Deaths: Clinician Toolkit (includes a patient handout with questions to ask)
• Epilepsy Surgery Improves Survival In Children With Drug-Resistant Seizures, Monika Jones, Pediatric Epilepsy Surgery Alliance