Epilepsy & Behavior 44 (2015) 251–252
Contents lists available at ScienceDirect
Epilepsy & Behavior journal homepage: www.elsevier.com/locate/yebeh
Editorial
Sudden unexpected death in epilepsy
Sudden unexplained death in epilepsy (SUDEP) has become a focus of attention for epileptology as it is one of the most disturbing aspects of patient care. A provocative paper, published a decade ago, advocated discussion of SUDEP on a case-by-case basis, reserving the right not to discuss it when patients had not raised the topic, thereby respecting the patient's right to avoid the additional distress in an already stigmatizing condition [1]. In common law jurisdictions, it is imperative to discuss ‘material risks’ to which patients would attach specific value when deciding to accept, or reject, treatment [2]. This departed from the Bolam Principle, which had placed less importance on ‘material risk’, favoring compliance with those practices accepted by a significant body of the therapist's peers [3]. The fundamental risks for SUDEP include the following: potential genetic predisposition [4,5], male gender, early age at onset, convulsive seizures, being in bed at the time of SUDEP [6,7], and polypharmacy [1]. No particular medication has been impugned [6], thus making none of the accepted risk factors ‘material risks’ in the treatment of epilepsy as none are truly modifiable. It follows that warning about SUDEP is a potential tool, to be used to enhance adherence and compliance with medical advice for the treatment of epilepsy, as the identified risks are beyond intervention. A review of what constitutes usual practice by clinicians indicates that most neurologists adopt a ‘need to know’ approach, dealing with the discussion of SUDEP on a case-by-case basis, with only a very small minority discussing it with all their patients [8–11]. Most are selective, discussing it with those who ask and those whom they consider ‘need to know’. The study by Xu and colleagues [12] (details published in the last issue of Epilepsy & Behavior) has sought to determine the attitudes of patients regarding the discussion of SUDEP [12]. They surveyed more than 100 patients attending a tertiary referral hospital epilepsy clinic over a period of 1 year and concluded that most patients want to be “informed about SUDEP” and that their treating neurologist “was the most appropriate source” of the information. This interpretation by Xu et al., of their findings, is perhaps a little over zealous when considering the questions posed within the survey. Question 6 asked, “With regards to your epilepsy, would you generally like to know: everything possible (~ 63% concurring); a reasonable amount of information (~32% so identified); and a minimal amount of information (~ 5% agreeing)”. There was a 50:50 split when patients were asked if “people with epilepsy have a higher risk of sudden death compared to people without epilepsy”. The survey then asked, “If there is a higher risk, would you prefer to know details?” to which ~90% agreed while only ~14% were aware of SUDEP. Of the 14% who were aware, one-third had heard about it from their neurologist (5 patients) and 13% (2 patients) from their family
http://dx.doi.org/10.1016/j.yebeh.2014.12.001 1525-5050/© 2015 Published by Elsevier Inc.
physician. The majority, two-thirds (10 patients), obtained their knowledge from either the Internet or the popular press. When asked how much information the patients “would like to receive”, 59% (62 patients) expected detailed information, ~ 32% (34 patients) wanted basic information, while ~9% (9 patients) were happy to receive no information at all. When asked when the provision of such information was most appropriate, ~47% of respondents felt that this was best delivered at subsequent consultations (3rd consultation or later) or “when seizure control worsens”. Of the 7 preferred options, regarding the most important concerns about epilepsy, SUDEP ranked last on the list, with a 15% response rate. Driving, risk of seizures, side-effects of medications, and lifestyle issues were ranked in the top 4 places of concerns for patients. Surveys, such as the one conducted by Xu and colleagues [12], are an important source of information but need to be interpreted carefully, with particular reference to the questions being asked. Asking people, the majority of whom (~ 84%) had experienced epilepsy for N 1 year, comprising ~ 44% of those who had epilepsy for N 5 years, whether they would like to know everything possible about their epilepsy, with only ~62% indicating same, is an important finding. This suggests that one-third of people with a long-standing epilepsy wish to know only a “reasonable amount” or a “minimal” amount, as demonstrated by the survey results. When posed the question, “If there is a higher risk (of sudden death), would you prefer to know details?”, what is surprising, and perhaps more informative, is that ~11% said “No”. Posing such a question, immediately after asking if there was an increased risk, is inviting a response in the affirmative and hence, a self-fulfilling response to such survey. Responses still included N 10% of those who did not want to receive such information, the reasons for same being open to conjecture. The application of this alternative interpretation, of the results of the survey, provides strong support for the conclusions advocated within the publication, now a decade old [1]. Despite that almost half of the sample had epilepsy for N 5 years, b 15% were aware of SUDEP, indicating that the majority of clinicians saw no reason to discuss it and patients failed to ask about it. With the plethora of information available on the Internet and in the popular press, b 15% had sought such information about SUDEP, and of those who were familiar with SUDEP, twothirds had obtained their knowledge from this source, thereby confirming its availability. No one should deny patients their right to know as much about their illness as they choose to know. Similarly, no one has the right to frighten patients about potential consequences of their illness, unless such approach is offered with the aim of improving patient well-being and ultimately protecting that patient from harm. The risk of SUDEP may encourage clinicians to use it as a motivating tool to enhance compliance
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Editorial
with treatment and adherence to lifestyle expectations, which attach to epilepsy. The data provided by Xu et al. [12] confirm that neurologists do discuss SUDEP when they consider it appropriate, as do family physicians. It would also suggest that this is done on a case-by-case basis, even within a tertiary referral epilepsy center. Such approach respects the right of the ~ 10% who indicated, even within a structured survey perceived to be leaning towards complete disclosure, that they do not want to know. Sudden unexplained death in epilepsy remains an emotive issue, and its discussion with patients remains controversial, which was one of the principle issues within the report by Xu et al. [12]. Little appears to have changed in the last decade since the opposite view was proffered [1]. What is imperative for all concerned is to respect the patient as an individual, to provide optimal care to treat that patient's epilepsy, and to address any concerns as they may arise. No patient should be refused access to information which is sought, and clinicians must appreciate each patient's needs. This has not changed nor hopefully will change. Conflict of interest The authors declare they have no conflicts of interest. References [1] Beran RG, Weber S, Sungaran R, Venn N, Hung A. Review of the legal obligations of the doctor to discuss Sudden Unexplained Death in Epilepsy (SUDEP) — a cohort controlled comparative cross-matched study in an outpatient epilepsy clinic. Seizure 2004;13:523–8. [2] Rogers v Whittaker [1992] HCA 58, 59; (1992) 175 CLR 479. [3] Bolam v Friern Hospital Management Committee [1957] WLR 582.
[4] Aurlien D, Leren TP, Tauboll E, Gjerstad L. New SCN5A mutation in a SUDEP victim with idiopathic epilepsy. Seizure 2009;18:158–60. [5] Le Gal F, Korff CM. A case of SUDEP in a patient with Dravet syndrome with SCN1A mutation. Epilepsia 2010;51:1915–8. [6] Hesdorffer DC, Tomson T, Benn E, Sander JW, Nilsson L, Langan Y, Walczak TS, Beghi E, Brodie MJ, Hauser A. Do antiepileptic drugs or generalized tonic–clonic seizure frequency increase SUDEP risk? A combined analysis. Epilepsia 2012;53:249–52. [7] Tomson T, Walczak T, Sillanpaa M, Sander JW. Sudden unexpected death in epilepsy: a review of incidence and risk factors. Epilepsia 2005;46(Suppl. 11):54–61. [8] Panelli R. SUDEP counselling: How and when? Neurol Asia 2011;16(Suppl. 1):51–4. [9] Wasade VS, Spanaki M, Iyengar R, Barkley GL, Schultz L. AAN Epilepsy Quality Measures in clinical practice: a survey of neurologists. Epilepsy Behav 2012;24: 468–73. [10] Vegni E, Leone D, Canevini MP, Tinuper P, Aldo Moja E. Sudden unexpected death in epilepsy (SUDEP): a pilot study on truth among Italian epileptologists. Neurol Sci 2011;32:331–5. [11] Waddell B, McColl K, Turner C, Norman A, Coker A, White K, Roberts R, Heath CA. Are we discussing SUDEP? A retrospective case not analysis. Seizure 2013;22:74–6. [12] Xu Z, Ayyappam S, Scneviratne U. Sudden unexpected death in epilepsy (SUDEP): what do patients think? Epilepsy Behav 2014. http://dx.doi.org/10.1016/j.yebeh. 2014.11.007.
Roy G. Beran M.D., F.R.A.C.P., F.R.C.P., F.R.A.C.G.P., F.A.C.L.M., B.Leg.S., M.H.L., Professor, Conjoint Associate Professor School of Medicine, Griffith University, Australia UNSW, Australia Suite 5, Level 6, 12 Thomas Street, Chatswood, NSW 2067, Australia. Tel.: +61 2 9415 3800; fax: +61 2 9413 1353. E-mail address: [email protected]. 4 December 2014
Contents lists available at ScienceDirect
Epilepsy & Behavior journal homepage: www.elsevier.com/locate/yebeh
Editorial
Sudden unexpected death in epilepsy
Sudden unexplained death in epilepsy (SUDEP) has become a focus of attention for epileptology as it is one of the most disturbing aspects of patient care. A provocative paper, published a decade ago, advocated discussion of SUDEP on a case-by-case basis, reserving the right not to discuss it when patients had not raised the topic, thereby respecting the patient's right to avoid the additional distress in an already stigmatizing condition [1]. In common law jurisdictions, it is imperative to discuss ‘material risks’ to which patients would attach specific value when deciding to accept, or reject, treatment [2]. This departed from the Bolam Principle, which had placed less importance on ‘material risk’, favoring compliance with those practices accepted by a significant body of the therapist's peers [3]. The fundamental risks for SUDEP include the following: potential genetic predisposition [4,5], male gender, early age at onset, convulsive seizures, being in bed at the time of SUDEP [6,7], and polypharmacy [1]. No particular medication has been impugned [6], thus making none of the accepted risk factors ‘material risks’ in the treatment of epilepsy as none are truly modifiable. It follows that warning about SUDEP is a potential tool, to be used to enhance adherence and compliance with medical advice for the treatment of epilepsy, as the identified risks are beyond intervention. A review of what constitutes usual practice by clinicians indicates that most neurologists adopt a ‘need to know’ approach, dealing with the discussion of SUDEP on a case-by-case basis, with only a very small minority discussing it with all their patients [8–11]. Most are selective, discussing it with those who ask and those whom they consider ‘need to know’. The study by Xu and colleagues [12] (details published in the last issue of Epilepsy & Behavior) has sought to determine the attitudes of patients regarding the discussion of SUDEP [12]. They surveyed more than 100 patients attending a tertiary referral hospital epilepsy clinic over a period of 1 year and concluded that most patients want to be “informed about SUDEP” and that their treating neurologist “was the most appropriate source” of the information. This interpretation by Xu et al., of their findings, is perhaps a little over zealous when considering the questions posed within the survey. Question 6 asked, “With regards to your epilepsy, would you generally like to know: everything possible (~ 63% concurring); a reasonable amount of information (~32% so identified); and a minimal amount of information (~ 5% agreeing)”. There was a 50:50 split when patients were asked if “people with epilepsy have a higher risk of sudden death compared to people without epilepsy”. The survey then asked, “If there is a higher risk, would you prefer to know details?” to which ~90% agreed while only ~14% were aware of SUDEP. Of the 14% who were aware, one-third had heard about it from their neurologist (5 patients) and 13% (2 patients) from their family
http://dx.doi.org/10.1016/j.yebeh.2014.12.001 1525-5050/© 2015 Published by Elsevier Inc.
physician. The majority, two-thirds (10 patients), obtained their knowledge from either the Internet or the popular press. When asked how much information the patients “would like to receive”, 59% (62 patients) expected detailed information, ~ 32% (34 patients) wanted basic information, while ~9% (9 patients) were happy to receive no information at all. When asked when the provision of such information was most appropriate, ~47% of respondents felt that this was best delivered at subsequent consultations (3rd consultation or later) or “when seizure control worsens”. Of the 7 preferred options, regarding the most important concerns about epilepsy, SUDEP ranked last on the list, with a 15% response rate. Driving, risk of seizures, side-effects of medications, and lifestyle issues were ranked in the top 4 places of concerns for patients. Surveys, such as the one conducted by Xu and colleagues [12], are an important source of information but need to be interpreted carefully, with particular reference to the questions being asked. Asking people, the majority of whom (~ 84%) had experienced epilepsy for N 1 year, comprising ~ 44% of those who had epilepsy for N 5 years, whether they would like to know everything possible about their epilepsy, with only ~62% indicating same, is an important finding. This suggests that one-third of people with a long-standing epilepsy wish to know only a “reasonable amount” or a “minimal” amount, as demonstrated by the survey results. When posed the question, “If there is a higher risk (of sudden death), would you prefer to know details?”, what is surprising, and perhaps more informative, is that ~11% said “No”. Posing such a question, immediately after asking if there was an increased risk, is inviting a response in the affirmative and hence, a self-fulfilling response to such survey. Responses still included N 10% of those who did not want to receive such information, the reasons for same being open to conjecture. The application of this alternative interpretation, of the results of the survey, provides strong support for the conclusions advocated within the publication, now a decade old [1]. Despite that almost half of the sample had epilepsy for N 5 years, b 15% were aware of SUDEP, indicating that the majority of clinicians saw no reason to discuss it and patients failed to ask about it. With the plethora of information available on the Internet and in the popular press, b 15% had sought such information about SUDEP, and of those who were familiar with SUDEP, twothirds had obtained their knowledge from this source, thereby confirming its availability. No one should deny patients their right to know as much about their illness as they choose to know. Similarly, no one has the right to frighten patients about potential consequences of their illness, unless such approach is offered with the aim of improving patient well-being and ultimately protecting that patient from harm. The risk of SUDEP may encourage clinicians to use it as a motivating tool to enhance compliance
252
Editorial
with treatment and adherence to lifestyle expectations, which attach to epilepsy. The data provided by Xu et al. [12] confirm that neurologists do discuss SUDEP when they consider it appropriate, as do family physicians. It would also suggest that this is done on a case-by-case basis, even within a tertiary referral epilepsy center. Such approach respects the right of the ~ 10% who indicated, even within a structured survey perceived to be leaning towards complete disclosure, that they do not want to know. Sudden unexplained death in epilepsy remains an emotive issue, and its discussion with patients remains controversial, which was one of the principle issues within the report by Xu et al. [12]. Little appears to have changed in the last decade since the opposite view was proffered [1]. What is imperative for all concerned is to respect the patient as an individual, to provide optimal care to treat that patient's epilepsy, and to address any concerns as they may arise. No patient should be refused access to information which is sought, and clinicians must appreciate each patient's needs. This has not changed nor hopefully will change. Conflict of interest The authors declare they have no conflicts of interest. References [1] Beran RG, Weber S, Sungaran R, Venn N, Hung A. Review of the legal obligations of the doctor to discuss Sudden Unexplained Death in Epilepsy (SUDEP) — a cohort controlled comparative cross-matched study in an outpatient epilepsy clinic. Seizure 2004;13:523–8. [2] Rogers v Whittaker [1992] HCA 58, 59; (1992) 175 CLR 479. [3] Bolam v Friern Hospital Management Committee [1957] WLR 582.
[4] Aurlien D, Leren TP, Tauboll E, Gjerstad L. New SCN5A mutation in a SUDEP victim with idiopathic epilepsy. Seizure 2009;18:158–60. [5] Le Gal F, Korff CM. A case of SUDEP in a patient with Dravet syndrome with SCN1A mutation. Epilepsia 2010;51:1915–8. [6] Hesdorffer DC, Tomson T, Benn E, Sander JW, Nilsson L, Langan Y, Walczak TS, Beghi E, Brodie MJ, Hauser A. Do antiepileptic drugs or generalized tonic–clonic seizure frequency increase SUDEP risk? A combined analysis. Epilepsia 2012;53:249–52. [7] Tomson T, Walczak T, Sillanpaa M, Sander JW. Sudden unexpected death in epilepsy: a review of incidence and risk factors. Epilepsia 2005;46(Suppl. 11):54–61. [8] Panelli R. SUDEP counselling: How and when? Neurol Asia 2011;16(Suppl. 1):51–4. [9] Wasade VS, Spanaki M, Iyengar R, Barkley GL, Schultz L. AAN Epilepsy Quality Measures in clinical practice: a survey of neurologists. Epilepsy Behav 2012;24: 468–73. [10] Vegni E, Leone D, Canevini MP, Tinuper P, Aldo Moja E. Sudden unexpected death in epilepsy (SUDEP): a pilot study on truth among Italian epileptologists. Neurol Sci 2011;32:331–5. [11] Waddell B, McColl K, Turner C, Norman A, Coker A, White K, Roberts R, Heath CA. Are we discussing SUDEP? A retrospective case not analysis. Seizure 2013;22:74–6. [12] Xu Z, Ayyappam S, Scneviratne U. Sudden unexpected death in epilepsy (SUDEP): what do patients think? Epilepsy Behav 2014. http://dx.doi.org/10.1016/j.yebeh. 2014.11.007.
Roy G. Beran M.D., F.R.A.C.P., F.R.C.P., F.R.A.C.G.P., F.A.C.L.M., B.Leg.S., M.H.L., Professor, Conjoint Associate Professor School of Medicine, Griffith University, Australia UNSW, Australia Suite 5, Level 6, 12 Thomas Street, Chatswood, NSW 2067, Australia. Tel.: +61 2 9415 3800; fax: +61 2 9413 1353. E-mail address: [email protected]. 4 December 2014
