Originally published on The Mighty
Disney Channel star Cameron Boyce died in July at age 20, with an autopsy confirming the cause was sudden unexplained death in epilepsy (SUDEP). Epilepsy is not considered a terminal illness or even a condition that frequently causes death. But in rare cases, it can lead to death.
Naturally, for any person with epilepsy or their parents, news like this is incredibly scary. However, it’s important to remember that SUDEP is rare and education is power. The more you know about SUDEP, the better you can protect your kids (and yourself, if you have epilepsy) against risk factors.
What is SUDEP?
As the name “sudden unexplained death in epilepsy” suggests, the scary nature of SUDEP is that there is no apparent cause of death, not necessarily even a seizure. In most cases, the person is found to have passed away in bed while they were sleeping. An estimated one in 1,000 people with uncontrolled (also called intractable) epilepsy die of SUDEP. The scientific community doesn’t know exactly what causes SUDEP, but it could have to do with how epilepsy affects heart rate, breathing and brain function.
The Mighty talked with Chrystal Reed, M.D., Ph.D., assistant professor of neurology at Cedars-Sinai Medical Center in Los Angeles, about current research into the possible causes of SUDEP, the risks you should be aware of and what you can do to help reduce those risks.
1. SUDEP most often happens during sleep, and you may not have a seizure immediately before.
Reed said one of the biggest misconceptions about SUDEP (besides the fact many people have not even heard about it) is that SUDEP means you had a seizure then fell and hit your head and died. However, a seizure isn’t necessary to diagnose SUDEP.
Most often, SUDEP occurs while a person is sleeping and they are otherwise healthy. They may or may not show any signs they just had a seizure. SUDEP can happen even when there is not a seizure-related accident or emergency and though experts believe most instances of SUDEP are seizure-related, you may not see any obvious signs of seizure.
2. We don’t know for sure what causes SUDEP, but there are a few theories.
Researchers don’t have all the answers yet, but studies of people with intractable or uncontrolled epilepsy who still have seizures despite treatment efforts found some correlations that could explain why SUDEP happens.
For some patients, seizures cause their heart rate to go too low after a seizure or it could cause an arrhythmia, which means their heart beats with an irregular pattern. This can be dangerous and even fatal.
Another possibility is that during a seizure your heart rate goes up, which requires your blood pressure to go down in response to keep you safe. But in some people, that reaction doesn’t happen because your systems aren’t working together as they should.
“There’s a miscommunication with the way that the respiratory system, the heart and the brain talk to each other,” Reed said.
There also may be an issue in the process that regulates your breathing during and after a seizure. Breathing could stop during a seizure, which causes your levels of carbon dioxide to rise. In response to this, the brainstem is supposed to trigger a reflex that wakes you up.
So perhaps in some people with epilepsy, the brainstem does not respond correctly and fails to wake you up when your carbon dioxide levels get to dangerously high levels. Or, maybe the heart stops for too long after a seizure (this is called post-ictal asystole).
3. In order to diagnose SUDEP, there must be no other apparent cause of death.
The only way to know that someone died of SUDEP for sure is to do an autopsy because experts can confirm there wasn’t any other explanation for the death. For example, if an autopsy finds evidence of another condition like pulmonary edema, which is caused by excess fluid in the lungs that makes it hard to breathe, then the diagnosis cannot be SUDEP. Autopsy results won’t find other conditions or complications in people who died of SUDEP.
If you don’t have an autopsy but there is still a strong suspicion the cause of death was SUDEP, then the diagnosis might be “probable” SUDEP. Finally, if the person had another health condition in addition to epilepsy that could have caused the death, but SUDEP is still suspected and there is no autopsy to confirm, then it would be diagnosed as “possible” SUDEP.
“For lots of patients who die of SUDEP, their family says they don’t want an autopsy,” Reed said. “So it’s not clear whether the patient had a cardiac problem prior to it, especially if they’re older, if they had an arrhythmia. It’s really hard to know.”
3. People who died of SUDEP often have a few characteristics in common.
We don’t know the exact mechanism that causes SUDEP so we can’t say definitely what causes it. But Reed said if you go back and talk to patients’ families, a few common qualities emerge, including:
- A history of generalized tonic-clonic seizures (aka grand mal seizures — a seizure in which you lose consciousness and experience powerful muscle contractions). If they’ve had more than three grand mal seizures in a year, that increases the risk of SUDEP
- Being on many medications, which indicates a person most likely has uncontrolled epilepsy — this diagnosis is given to people who have tried at least two anti-seizure medications but they didn’t work
- Sleeping in the “prone” position, which is another way of saying sleeping on your stomach. This position may worsen the breathing and heart rate issues that may be connected to your SUDEP risk
4. People between the ages of 20 and 40 are particularly at risk.
Between the ages of 20 and 40, Reed said, people may stop coming to the doctor for regular appointments. “If they have a tonic-clonic seizure one or two times a year, they don’t really think it’s a big deal,” Reed said.
As adults who are used to living with epilepsy, they also may not have as much social support as they did as kids, or they may live alone. They may not even realize they are having seizures if they happen during their sleep when no one is around. Remember, having three or more grand mal seizures a year is a risk factor.
5. There are a few strategies that can help reduce the risk of SUDEP.
The number one way you can reduce the risk of SUDEP is reducing your seizures. Reed recommended getting evaluated at a level 4 epilepsy center — this is a medical center accredited by the National Association of Epilepsy Centers as providing the highest level of treatment. (You can find one here.) You should work with your doctors and keep regular appointments to determine which medication and even surgery options might be right for you so you experience fewer seizures.
Meanwhile, Reed said she gives her patients and families a few other strategies that can help provide some peace of mind, especially at night. These strategies can help make sure you know about all the seizures that are happening so you’re not under the impression you or your child is having fewer seizures than they are. It can also potentially help someone notice if they are not breathing during or after a seizure.
- Encourage your child or loved one to stop sleeping on their stomach
- Place a baby monitor in the bedroom at night so parents or roommates can more easily monitor for seizures
- Have the patient share a room so someone can more easily notice if they are having a seizure or stop breathing
- Make sure someone in the house knows CPR. If you realize your loved one has stopped breathing, you can potentially save their life by administering CPR
- There are some pillows on the market that have been designed for people with epilepsy. They feature breathable fabric and design to theoretically reduce the risk of suffocation while sleeping. Reed noted there have been no scientific studies regarding their efficacy, but over time, research may prove these pillows to be helpful
- Sleep with a wearable seizure alert device so someone can be alerted if a seizure happens at night. Reed cautioned that these types of devices often send out “false positives,” especially in people with intractable epilepsy who have difficulty sleeping and wake up frequently. But an alert device may provide some reassurance
- Take all medications as directed and avoid any seizure triggers you know of
Finally, Reed recommended people with epilepsy find out if they have any heart-related risk factors. At an epilepsy monitoring unit, doctors can see if you have an irregular heart, bradycardia (heart rate that is too slow) or asystole (flatline, or no electrical activity from the heart) during a seizure, all of which can indicate a higher risk of SUDEP.
“These patients must get a cardiac consultation. They might need a pacemaker device or a defibrillator device so if this ever happens, it can fire and it can prevent the heart from going in asystole. And that will prevent this vicious cycle,” Reed said.
6. Know what to do if you realize someone is having a seizure in their sleep.
Remember, you don’t necessarily have to have a seizure during SUDEP, but if a seizure does happen in someone’s sleep, it’s important you wake them up so they don’t get in a cycle where they have a seizure and then stop breathing. Here’s what you can do.
First, if they are on their stomach, turn them to the side quickly, Reed said. Don’t put anything in their mouth. The most important thing is to wake them up. Reed said you should shout at them and try and shake them awake. Then check their pulse (on the neck is best). And if they’re not breathing, administer CPR because Reed said unlike a heart attack, you may be able to jump start their breathing right away.
Although thinking about and talking about SUDEP with your child or doctor can be scary, you’re ahead of the game by knowing it exists, being aware of the risks and taking steps to prevent potentially life-threatening complications.
“I educate all my patients with epilepsy about SUDEP and encourage medication compliance,” Reed said.