Background information & rationale for the call to action of ePetition e-1888
1 in 100 people worldwide live with epilepsy, a condition characterized by recurrent, unprovoked seizures. It is the most common chronic brain disease, with 1 in 26 people diagnosed with epilepsy at some point in their lifetime. There is an urgent need for accelerated action to make epilepsy a public health priority and this has been highlighted in the 2019 World Health Organization’s first Global Epilepsy Report (WHO 1, 2019).
More than 50 million people live with epilepsy around the world.
People with epilepsy are 2 to 3 times more likely to die prematurely than the rest of the population (Tomson, 2000). Death may be: directly due to epilepsy (such as drowning during a seizure or death due to prolonged seizures, known as status epilepticus); due to the underlying cause of the epilepsy (such as a brain tumor or neurodegenerative disease); or indirectly due to epilepsy (such as from the comorbidities of epilepsy, like depression and suicide, or chronic effects of anti-seizure medications). Sudden death in epilepsy is widely recognized as a global healthcare concern (Levira, 2017).
The most common epilepsy-related cause of death is sudden, unexpected death in epilepsy (known as SUDEP), in which a person with epilepsy dies without warning and with no apparent explanation found at autopsy (Thurman, 2014). Most often the person is discovered dead in bed in the morning, usually with evidence of a recent convulsion. Research suggests 1 in 1000 people living with epilepsy are lost to SUDEP each year (Keller, 2018) and 9 in 1000 for people with frequent seizures that are poorly controlled with medications (Tomson, 2008). Data from the US has demonstrated that SUDEP is the second leading neurologic cause of life-years lost, after stroke (Thurman, 2014).
With a population of just over 37 million in Canada, approximately 370,000 people live with epilepsy and an estimated 370 people lose
their life to SUDEP each year.
Canadian epilepsy-related death statistics
Although there are two national databases that gather cause of death data from death certificates across Canada, neither is currently able to provide an accurate number of lives lost to epilepsy-related deaths:
The Canadian Coroner and Medical Examiner Database (CCMED) is a repository of deaths reported directly to coroners and medical examiners (Stats CDA2, 2019) but does not presently contain data from all the provinces and territories across Canada (including Newfoundland & Labrador and Manitoba, as at April 2019).
The Canadian Vital Statistics Death Database (CVSD) is an administrative survey that annually collects demographic and medical (cause of death) information from all the provincial and territorial vital statistics registries across Canada (Stats CDA1, 2019). All medical certificates are forwarded to a provincial or territorial vital statistics registry. The cause of death listed on the certificate is allocated an "International Statistical Classification of Diseases and Related Health Problems", or ICD, code for upload to the national CVSD. The coding system presently in use is ICD revision 10 (WHO 2, 2019), which does not have a specific code for SUDEP. This means that SUDEP deaths are not readily identifiable in the CVSD.
We currently do not know exactly how many people are lost to epilepsy across Canada. More information about these deaths is critically
needed to aid in the development of prevention strategies.
The latest ICD revision (ICD-11), released on June 18, 2018, does include a code for SUDEP. However, it will take several years for software development, testing, training and migration before the SUDEP category is made available to the coders.
In the interim, in response to requests for SUDEP mortality data, Statistics Canada has just recently implemented a mechanism to help identify SUDEP cases within the CVSD, using a unique combination of ICD-10 codes. This will be applied to cases dating back to 2016.
The accurate and consistent coding of epilepsy-related cause of death within the CVSD is just one consideration towards the gathering of reliable mortality statistics across Canada. There are several barriers to the identification and reporting of epilepsy-related deaths that exist and these pose a variety of challenges to death investigators that lead to the under-recognition, and consequent underestimation, of cases.
Barriers to the IDENTIFICATION of epilepsy-related deaths
Evidence of a seizure (such as blood from a tongue or cheek bite; incontinence) are commonly absent, or of varying specificity, and this can limit consideration of epilepsy as a cause, or contributor, to the death (Middleton, 2018).
Under-recognition of SUDEP is widespread among coroner and medical examiner communities (Devinsky, 2017). As most SUDEP deaths occur during sleep and are unwitnessed, there is a strong reliance on background history and family statements to alert the investigator to past seizures or a diagnosis of epilepsy. For various reasons, such as the social stigma surrounding epilepsy or a lack of appreciation of its significance, families may not be forthcoming with this information. The investigator needs to be aware of SUDEP and other epilepsy-related causes of death to proactively ask relevant questions of families.
Not all members of the death investigation team are familiar with SUDEP. The Canadian system of death investigation includes medical examiners, who are physicians, and coroners, who are not required to be physicians and can come from a variety of backgrounds (for example, law enforcement or nursing). With no nationally recognized training program or credentialing system for coroners and medical examiners, and no national standards for the investigation or classification of death, there is a concern about the consistency and integrity of epilepsy-related death data across Canada. Autopsy rates have been found to vary widely between jurisdictions (Kelsall, 2016).
Coroners who decide whether an autopsy is necessary may do so without knowing its significance in the identification of definite SUDEP cases, if unaware of the term. Were the implementation of mandatory reporting of SUDEP to be introduced in Canada, it would help to educate death investigators about this important cause of death and the role of the autopsy in its determination.
By definition, SUDEP is a diagnosis given after exclusion of all other possible causes of death and this can make it challenging to identify. In cases where more common causes of death cannot be ruled out (for example, age-related causes), the possibility of SUDEP may not even be considered or recorded on the death certificate. Cases are, also, being attributed to concomitant conditions identified at autopsy (for example cardiovascular disease) without mention of the possibility of SUDEP. For these reasons, death certificates tend to significantly under-represent the true number of deaths in which epilepsy has been a factor (Middleton, 2018).
Barriers to the REPORTING of epilepsy-related deaths
Death certificates are an important source of information for epidemiologists and public health authorities when determining epilepsy-related death incidence and identifying cases for research. Data from death certificates is used to populate Canada’s national death statistics database, CVSD. Completion of death certificates varies between jurisdictions, which makes comparison of mortality data difficult (Kelsall, 2016). Studies have also revealed considerable variation in the reporting of epilepsy-related deaths (particularly SUDEP deaths) by coroners and medical examiners, which raises further concern about the integrity of the statistics.
Lack in use of the term ‘SUDEP’ as cause of death is an issue. Deaths instead assigned as ‘undetermined’ or of ‘unknown’ cause are unidentifiable as SUDEP cases and valuable research data is being lost. A US survey found that only a minority of coroners and medical examiners used the term SUDEP on the death certificate (Schraeder, 2006). The investigation of SUDEP cases at two different medical examiner offices revealed only 8 of 74 cases, and just 1 of 28 cases, listed SUDEP on the death certificate (Office of the Chief ME in Baltimore (Zhuo, 2012); Jefferson County Coroner/ME Office (Atherton, 2016) respectively).
Were the implementation of mandatory reporting of SUDEP to be introduced in Canada, it would help to bring greater consistency to the mentioning of SUDEP on death certificates and more essential data for researchers.
Canada is behind other countries in the INVESTIGATION of epilepsy-related deaths
In an effort to ensure accurate and complete evaluation of epilepsy-related deaths, a 2018 position paper of the US National Association of Medical Examiners provides recommendations for the investigation of epilepsy-related deaths, including autopsy, toxicological analysis, interpretation of autopsy and toxicology findings and death certification (Middleton, 2018).
A “Field Investigator Epilepsy Death Form” has also been developed with the aim of assisting investigators in the gathering of specific epilepsy-focused information for coroners and medical examiners (note, this is not presently routinely used throughout all of the states).
In the UK, the Royal College of Pathologists’s “Guidelines on Autopsy Practice Scenario 6: Deaths associated with epilepsy” aids pathologists conducting epilepsy-related death autopsies for coroners (note, this is currently under review and update).
In the US, legislation has been passed in some states to mandate SUDEP surveillance. The states of New York, Illinois and New Jersey require the term ‘SUDEP’ to be used on the death certificate where applicable. Both New York and Illinois ask that these cases are filed with the North American SUDEP Registry. New Jersey goes further, requiring SUDEP education for medical examiners and helps to facilitate tissue donation.
Mandatory reporting of epilepsy-related deaths has several benefits. First, by requiring death investigators to report epilepsy-related deaths, these same professionals will become more aware of SUDEP. Second, mandatory reporting will allow us to determine an accurate incidence of epilepsy-related deaths in Canada. Finally, the data acquired through mandatory reporting of epilepsy-related deaths will allow clinical and research scientists working in this field to develop targeted prevention strategies. Mandatory reporting and surveillance of epilepsy-related deaths is the next critical step to saving the lives of Canadians living with epilepsy.
Epilepsy-related death SURVEILLANCE in Canada
Epilepsy-related mortality, and particularly sudden death in epilepsy, should be a public health priority in Canada. We lack the necessary public surveillance data and need a unified approach to the investigation and reporting of sudden epilepsy-related deaths across the country.
The purpose of ePetition e-1888 is to bring urgent attention to an important issue in essential need of immediate action for all those living with epilepsy and ensure the reliable reporting of sudden epilepsy-related deaths across Canada.
To this aim, and on behalf of all families living with, or bereaved by, epilepsy, the signatories and supporters of ePetition e-1888 respectfully request:
The development, and overseeing, of a standardized approach to the measurement, investigation and reporting of sudden deaths involving epilepsy across Canada; and
The collation of Canadian epilepsy-related death data in a centralized repository.
These actions are the critical next steps required to increase our understanding of this complex issue. Without accurate surveillance, researchers and health policy teams lack the depth of data to study the problem. Only through taking measures to improve the surveillance of epilepsy-related deaths will we be able to develop interventions and strategies to save the lives of people living with epilepsy.
Atherton, D. D. (2016). Implications of Death Certification on Sudden Unexpected Death in Epilepsy (SUDEP) Research. Academic Forensic Pathology, 6(1), 96–102. Retrieved from https://doi.org/10.23907/2016.009
Devinsky, O. F. (2017). Underestimation of sudden deaths among patients with seizures and epilepsy. Neurology, 89(9), 886–892. Retrieved from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5577966/
Keller, A. W. (2018). Incidence of sudden unexpected death in epilepsy in children is similar to adults. Neurology, 91(2), e107-e111. Retrieved from https://www.ncbi.nlm.nih.gov/pubmed/29884734
Kelsall, D. &. (2016). No standards: medicolegal investigation of deaths. CMAJ, 188(3), 169. Retrieved from http://www.cmaj.ca/content/cmaj/188/3/169.full.pdf
Levira, F. T. (2017). Premature mortality of epilepsy in low- and middle-income countries: A systematic review from the Mortality Task Force of the International League Against Epilepsy. Epilepsia, 58(1), 6-16. Retrieved from https://onlinelibrary.wiley.com/doi/epdf/10.1111/epi.13603
Middleton, O. A. (2018). National Association of Medical Examiners position paper: Recommendations for the investigation and certification of deaths in people with epilepsy. Epilepsia, 59(3), 530-543. Retrieved from https://onlinelibrary.wiley.com/doi/epdf/10.1111/epi.14030
Nashef, L. (1997). Sudden unexpected death in epilepsy: terminology and definitions. Epilepsia, 38(Suppl 11), 6-8. Retrieved from https://onlinelibrary.wiley.com/doi/epdf/10.1111/j.1528-1157.1997.tb06130.x
Schraeder, P. D. (2006). Coroner and medical examiner documentation of sudden unexplained deaths in epilepsy. Epilepsy Research, 68(2), 137-143. Retrieved from https://www.sciencedirect.com/science/article/pii/S092012110500238X?via%3Dihub
Stats CDA1. (2019). Vital Statistics - Death Database (CVSD). Retrieved from Statistics Canada: http://www23.statcan.gc.ca/imdb/p2SV.pl?Function=getSurvey&Id=1202355
Stats CDA2. (2019). Canadian Coroner and Medical Examiner Database (CCMED). Retrieved from Statistics Canada: http://www23.statcan.gc.ca/imdb/p2SV.pl?Function=getSurvey&SDDS=5125
Thurman, D. H. (2014). Sudden unexpected death in epilepsy: assessing the public health burden. Epilepsia, 55(10), 1479-85. Retrieved from https://www.ncbi.nlm.nih.gov/pubmed/24903551
Tomson. (2000). Mortality in epilepsy. J Neurol, 247(1), 15-21. Retrieved from https://www.ncbi.nlm.nih.gov/pubmed/10701892
Tomson, T. N. (2008). Sudden unexpected death in epilepsy: current knowledge and future directions. Lancet Neurol, 7(11), 1021-31. Retrieved from https://www.ncbi.nlm.nih.gov/pubmed/18805738
WHO 1. (2019). Improving Access to Epilepsy Care. Retrieved from World Health Organization: https://www.who.int/mental_health/neurology/epilepsy/en/
WHO 2. (2019). Classifications - ICD-10 online versions. Retrieved from World Health Organization: https://www.who.int/classifications/icd/icdonlineversions/en/
Zhuo, L. Z. (2012). Sudden unexpected death in epilepsy: Evaluation of forensic autopsy cases. Forensic Sci Int, 223(1-3), 171-5. Retrieved from https://www.ncbi.nlm.nih.gov/pubmed/22999232
 Status epilepticus is where seizures follow one another without recovery or consciousness in between.
 SUDEP is “the sudden, unexpected, witnessed or unwitnessed, non-traumatic and non-drowning death in patients with epilepsy, with or without evidence for a seizure and excluding documented status epilepticus, in which postmortem examination does not reveal a toxicologic or anatomic cause for death” (Nashef, 1997).
 Email correspondence with Statistics Canada, April 2019.
 Email correspondence with Statistics Canada, April 2019.
 See page 543 of National Association of Medical Examiners position paper: Recommendations for the investigation and certification of deaths in people with epilepsy (Middleton, 2018).
 The Royal College of Pathologists “Guidelines on Autopsy Practice. Scenario 6: Deaths associated with epilepsy”. Currently under review, 2005 version at: https://www.rcpath.org/uploads/assets/uploaded/39f66800-9e17-4336-9b19d6e541baba4e.pdf
 January 2017, the State of New York - https://www.nysenate.gov/legislation/bills/2017/S2422
 January 2014, the State of Illinois - http://www.ilga.gov/legislation/publicacts/fulltext.asp?Name=098-0340
 August 2013, the State of New Jersey - https://www.njleg.state.nj.us/2012/Bills/AL13/91_.HTM