Epilepsy, like other long-term conditions such as asthma or diabetes comes with certain risks which include accidents, injuries and occasionally death. The Australian Bureau of Statistics has recorded around 300 epilepsy related deaths per year in Australia, with up to half of these a result of SUDEP. However it is thought that recording has not been precise and that epilepsy deaths have been significantly under reported. Based on international research it is estimated that up to 250 deaths per year can be attributed to SUDEP alone in Australia.
Dying is not an easy topic to talk about, but premature, sudden death is devastating. Having an awareness, knowing the risks and talking about it, and knowing what to do in the case of a seizure, can help prevent these unnecessary deaths.
What is SUDEP?
Sudden Unexpected Death in Epilepsy (SUDEP)[1] is an uncommon condition where a person with epilepsy dies suddenly and prematurely, and no reason for death can be found. It predominantly occurs overnight and is often not witnessed. There may sometimes be obvious signs a seizure has happened, though this isn’t always the case.
SUDEP is one of the leading causes of death related to epilepsy yet still poorly understood.
The cause of SUDEP is not yet known. It is likely that there is no single explanation and researchers are investigating a range of possibilities such as the effect seizures can have on breathing and the heart and failing to arouse after a seizure.
How common is it?
- For adults the risk is low. In one year, SUDEP typically affects 1 person in 1000 who have epilepsy
- For children with epilepsy the risk of SUDEP is rare. In one year, SUDEP typically affects 1 child in 4500 who have epilepsy
SUDEP is more common than SIDS but far fewer people know about it
How does it happen? [2],[3]
Death will likely happen if you stop breathing or your heart stops. If one occurs, then the other is going to follow. There is no doubt that seizures can sometimes cause abnormal heart rhythms (arrhythmias) or slow shallow breathing (respiratory depression). Research to date shows all SUDEP is not caused by a single mechanism, but that different mechanisms can occur in different people.
Most SUDEP cases occur after a tonic-clonic seizure[4]. However, the means that lead from seizures to death are still unknown.
Among the proposed reasons for SUDEP include heart and breathing abnormalities or changes during and after a seizure, plus difficulties with arousal or awakening after a seizure. [5]
Are some seizures higher risk than others?
Having active seizures can put you at risk of injury and death, and there are certain types of seizures which can increase a person’s risk of SUDEP.
A major risk factor for SUDEP is the presence and frequency of tonic clonic seizures. People with 3 or more tonic clonic seizures per year have an increased risk of SUDEP and this risk is likely to increase if they happen at night or when asleep. The more frequent the tonic clonic seizures, the higher the risk.
Although SUDEP is more common in people with frequent seizures it has also occurred in people who have had very few seizures.
People who do not have tonic clonic seizures are still at risk of SUDEP.
People with only absence or myoclonic seizures are not known to have an increased risk for SUDEP. However, people who have these seizures may be at risk for tonic-clonic seizures if they do not take medication as prescribed.
It is important you discuss your risks and concerns with your doctor who can help assess your own risks and discuss steps to reduce them. This is where the SUDEP and Seizure Safety Checklist will be used.
What is my risk of SUDEP? [6]
There are some recognised risk factors and some suggested risk factors for SUDEP. The main risk factors are:
- Tonic clonic seizures
- Poorly controlled or frequent seizures
Although the evidence is low, other suggested risk factors may include
- A history of seizure related injury
- A history of status epilepticus and/or prolonged seizures
- Seizures during sleep (nocturnal seizures)
- Sleeping alone
- Not taking medication as prescribed
- Medication changes
- Alcohol or substance abuse
- Also having depression or other psychiatric illness
- Presence of intellectual disability
- Pregnancy
- Unclear seizure frequency or treatment history
- The person has had epilepsy for more than 15 years
- The seizures begin at less than 16 years of age
- The person is male gender, and younger adult age 20-40 years
Can the risk of seizures be reduced?
Of course, many seizure risks can be lessened. The most important step to avoid injury or death in epilepsy is to try to control seizures or at least reduce how often they happen. There is plenty of safety information on our website which can help reduce the risk of injury.
Although a third of people with epilepsy continue to strive for seizure control, a few actions can help reduce individual risks such as seeing an epilepsy specialist, taking medication as prescribed and discussing a treatment review or changes if necessary.
Sometimes other treatment options may be possible such as surgery or vagus nerve stimulation, if they are suitable for your particular type of epilepsy.
Other conditions that may contribute
It is no secret that having one health issue can exacerbate or worsen another. Here we discuss two conditions that may contribute to increasing the risk of SUDEP in people with epilepsy.
Long QT Syndrome (LQTS)
An area that has been researched considerably in relation to SUDEP is LQTS and the genes around this disorder. Long QT syndrome is a heart rhythm condition that can potentially cause fast, chaotic heartbeats. These rapid heartbeats might trigger a sudden faint or seizure. In some cases, the heart can beat erratically for so long that it causes sudden death.[7] Some people with epilepsy carry the LQTS genes, and inherited LQTS can often go undiagnosed or is misdiagnosed as a seizure disorder, such as epilepsy. 3,5
For more about Long QT Syndrome click here
Obstructive sleep apnoea (OSA) [8]
SUDEP frequently occurs in bed, presumably from sleep, and people with epilepsy are more likely to have a sleep disorder than the general population, so this topic is worth raising.
There are several types of sleep apnoea, but the most common is obstructive sleep apnoea. OSA occurs when the throat muscles intermittently relax and block the airway during sleep, interrupting breathing. Noticeable signs of OSA are loud snoring, episodes of stopping breathing during sleep, followed by abrupt awakenings accompanied by gasping or choking sounds.[9] These periods of stopping breathing are called apnoea and they can significantly slow the heart rate and increase blood pressure. OSA is associated with stroke, heart disease or arrhythmias, hypertension, diabetes, obesity, and sudden death.
OSA is found in as many as 30-40% of people with epilepsy with possibly many undiagnosed.
Alternatively, OSA (and other sleep disorders) can be the primary reason for poor seizure control. This is because snoring and apnoea’s disrupt sleep, creating chronic sleep deprivation and therefore increasing the risk of seizures. When treated, seizure control improves.
For more about Obstructive Sleep Apnoea click here
Choices
Your lifestyle and treatment choices can influence your risk. For example, you may be in a high-risk group but may have options to reduce that risk, such as surgery for epilepsy or improved medications. Or you may be at low risk, but your treatment or lifestyle choices put you at greater risk, for instance deciding to make medication changes without speaking to your doctor or drinking excessive alcohol. Have a look at our self-management factsheet to help with good lifestyle choices.
Can safety devices help?
Night-time supervision often isn’t sustainable or even possible in most cases, but some people feel more reassured knowing their loved-one is monitored overnight. Some people choose to do this with technology.
There are several devices and monitors available for night-time seizure monitoring in the home. They are designed differently. Some recognise abnormal movements that a seizure may cause, some recognise a fall out of bed, whilst others recognise changes in either breathing patterns, oxygen levels or heart rate. An alarm is triggered so that help – either a family, friend or emergency services – can be notified.
Because many epilepsy-related deaths often occur in bed during the night, there is an assumption that suffocation or breathing difficulties may contribute to the deaths. There are special ‘anti-suffocation’ pillows available. While these pillows do seem to allow more airflow, they cannot guarantee the safety of a person having nocturnal seizures. The use of such pillows is a personal choice.
Some families have found monitors to be a useful part of a risk reduction plan. No technology is perfect, you can’t be with your loved one all the time but monitoring devices can alert you or someone else to provide more immediate assistance.
Speak to your doctor or epilepsy nurse about whether a device is something that you might choose to use.
Read more about individual devices on our Epilepsy Products page
Monitors, alarms and safety products don’t guarantee safety or detection of all seizures, but they can provide peace of mind for some people.
SUDEP and Seizure Safety Checklist [10]
What is it?
The SUDEP and Seizure Safety Checklist has been designed for health practitioners as a clinical tool to support risk communication with their patients with epilepsy, and to help develop a plan to assess and reduce the risks.
It came about after a large UK study over 9 years showed that 80% of people with epilepsy who died suddenly had not been in contact with specialist services in the year prior, and 90% had a worsening of seizures in the 3-6 months prior to the death. One half had a record of alcohol misuse, and a quarter had been taking drugs to treat depression or anxiety. This highlighted the fact that there may be modifiable risk factors that can be acted upon to avoid SUDEP.
A common theme was the lack of awareness and the underestimation of risk, indicating that risk assessment and risk management are fundamental in reducing avoidable deaths.
As a result of these findings, SUDEP Action UK and Cornwall Partnership NHS Foundation Trust, developed a SUDEP and Seizure Safety Checklist. Epilepsy Action Australia has worked in collaboration to adapt this Checklist for people with epilepsy in Australia.
The SUDEP and Seizure Safety Checklist is useful to both health practitioners and people with epilepsy leading to a step-up of care and better awareness of safety. \Health practitioners have reported that the Checklist raised awareness of management of patients at risk of epilepsy-related death.
Whilst the Safety Checklist was originally developed for SUDEP, risk factors such as medication non-adherence and depression can be picked up, and are relevant to epilepsy mortality generally.
Steps to reduce the risk of SUDEP
If you have epilepsy, ask your doctor to discuss the risk of SUDEP with you.
Medications
The first and most important step to reduce your risk of SUDEP is to take your anti-epileptic medication as prescribed.
- Never make any changes without consultation with your neurologist or doctor
- Discuss any unwanted medication side effects with your doctor if you are not happy
- If you miss a dose, take it as soon as possible, but if it is close to the next dose time, then don’t take a double dose
- If you are still having seizures when taking medication, discuss your options with your doctor
- If seizures continue, consider seeing a neurologist who is an epilepsy specialist, if you have not already done so.
Lifestyle changes
Other possible steps to reduce the risk of SUDEP may include
- If you know what triggers your seizures, try to avoid them. This is not always possible but lessen them as much as you can
- Develop an awareness of triggers and lifestyle choices that affect your seizures. This way you can learn how to better control your seizures with simple changes in things like diet, exercise and stress management
- Go easy on the alcohol. If you like to have an alcoholic drink or two, then take the low-alcohol version if you can
- Get enough sleep. Not only is this good health practice for everyone, it can help with seizure control
- Having someone around reduces your risk significantly. However, many people live alone, so you may need to have a seizure alert system in place so someone can get to you quickly. This can be life saving
- Make sure people close to you know how to manage your seizures and what to do in an emergency.
Summary
Epilepsy, like other long-term conditions such as asthma or diabetes comes with certain risks. If left unchecked these can become very serious but these risks can be reduced with increased awareness.
“Since sudden death can happen to anyone with epilepsy, doctors need to discuss this rare possibility with people with epilepsy and their families,” Professor Orin Devinsky, Director NYU Comprehensive Epilepsy Centre, says. “Education of patients and caregivers would be critical to avoid potential risk factors…” [11]
Risks associated with seizures need to be discussed, no matter what type of epilepsy you have. Some of it can be difficult to talk about but it is important to be informed and ask questions. This way you can understand your risk, learn how to stay healthy, work to control your seizures, and reduce your risk as much as you can.
References:
[1] https://n.neurology.org/content/neurology/88/17/1674.full.pdf Accessed Sept 2019
[2] Teran, FA, Massey C & Richerson GB. Overview of section: current research on causes of SUDEP. In: Hanna J, Panelli R, Jeffs T, editors. Continuing the global conversation [online]. SUDEP Action & SUDEP Aware; 2018 [retrieved 7/8/2019]. Available from: www.sudepglobalconversation.com.
[3] Sowers LP, Massey CA, Gehlbach BK, Granner MA, Richerson GB. Sudden unexpected death in epilepsy: fatal post-ictal respiratory and arousal mechanisms. Respir Physiol Neurobiol 2013;189:315-23.
[4] Ryvlin, Prof. P. et al. (2013) The Lancet Neurology Volume 12, Issue 10, October 2013, Pages 966-977
[5] https://www.ncbi.nlm.nih.gov/pubmed/30905388 Buchanan, G.F. (2019) Impaired CO2 Induced Arousal in SIDS and SUDEP (2019) accessed August 2019
[6] Hesdorffer, D.C., Tomson, T., Benn, E., Sander, J.W., Nilsson, L., Langan, Y., Walczak, T.S., Beghi, E., Brodie, M. J., Hauser, A. (2011), Combined analysis of risk factors for SUDEP. Epilepsia, 52: 1150-1159. doi:10.1111/j.1528-1167.2010.02952.x Accessed August 2019
[7] https://www.mayoclinic.org/diseases-conditions/long-qt-syndrome/symptoms-causes/syc-20352518 Accessed August 2019
[8] Lino Nobili, Paola Proserpio, Guido Rubboli, Nicola Montano, Giuseppe Didato Carlo A.Tassinari. (2011) Sudden unexpected death in epilepsy (SUDEP) and sleep. Sleep Medicine Reviews. Volume 15, Issue 4, August 2011, Pages 237-246 https://doi.org/10.1016/j.smrv.2010.07.006. Accessed August 2019
[9] https://www.mayoclinic.org/diseases-conditions/obstructive-sleep-apnea/symptoms-causes/syc-20352090 Accessed August 2019
[10] https://sudep.org/checklist-development Accessed August 2019
[11] https://n.neurology.org/content/93/3/e227 SUDEP in the North American SUDEP Registry 2019
