- Email: [email protected]
Sudden unexpected death in epilepsy (SUDEP): What do patients think?
Epilepsy & Behavior 42 (2015) 29–34
Contents lists available at ScienceDirect
Epilepsy & Behavior journal homepage: www.elsevier.com/locate/yebeh
Sudden unexpected death in epilepsy (SUDEP): What do patients think? Zheyu Xu a, Sujith Ayyappan a, Udaya Seneviratne a,b,⁎ a b
Department of Neuroscience, Monash Medical Centre, Clayton, Victoria 3168, Australia Department of Medicine, Monash University, Melbourne 3800, Australia
a r t i c l e
i n f o
Article history: Received 10 August 2014 Revised 6 November 2014 Accepted 8 November 2014 Available online xxxx Keywords: Sudden unexpected death in epilepsy Epilepsy Patients' perspectives Mortality
a b s t r a c t Objectives: Sudden unexpected death in epilepsy (SUDEP) is a major cause of mortality in epilepsy. Despite its devastating consequences, SUDEP appears to be poorly discussed with patients by health professionals. The risk of causing psychological distress to the patient is highlighted as a reason for not discussing SUDEP. However, no studies have assessed the adult patients' views on this important question. We conducted this crosssectional study to evaluate the awareness and perspectives on SUDEP among adult patients with epilepsy. Methods: One hundred five consecutive adult patients with epilepsy, referred to the Epilepsy Clinic of a tertiary hospital between October 2012 and November 2013, were surveyed to ascertain their views and understanding of SUDEP. The data were analyzed using logistic regression to explore the association between patients' awareness of SUDEP and characteristics such as age, gender, duration of epilepsy, level of education, and employment. Results: Awareness of SUDEP among adult patients with epilepsy was poor (14.3%). However, the vast majority (89.5%) wished to be informed about SUDEP, and 59% requested detailed information. The treating neurologist was considered to be the most appropriate source of SUDEP information by 85.6% of patients. Multivariable analysis of the data showed no association between characteristics of patients (age, gender, duration of epilepsy, level of education, and employment) and their awareness of SUDEP or desire to get SUDEP-related information. Conclusions: Our study suggests that the majority of adult patients wish to be informed about SUDEP. This is in contrast to the general reluctance of medical professionals to inform all patients routinely about this condition. Crown Copyright © 2014 Published by Elsevier Inc. All rights reserved.
1. Introduction The most devastating consequence of epilepsy is death. Long-term, population-based, prospective studies have shown an increased risk of premature mortality in patients with epilepsy by more than twice that of the general population [1–3]. The mortality risk remains elevated irrespective of the degree of seizure control [1]. Cause-specific mortality analyses in the cohort with epilepsy demonstrate a higher proportion of deaths from malignant neoplasms, pneumonia and other respiratory diseases, cerebrovascular diseases, and cardiovascular causes. While some of the causes of mortality such as aspiration pneumonia have an obvious causal relationship, the mechanism of increased mortality in other situations remains unclear. The postulations include a higher degree of somatic and psychiatric comorbidities, socioeconomic deprivation, effect of long-term antiepileptic medications, common genetic predispositions, and role of inflammatory mechanisms [1,2]. Causes of mortality as a direct consequence of seizures and epilepsy include sudden unexpected death in epilepsy (SUDEP), accidental deaths due to epileptic seizures, and deaths from status epilepticus. Sudden Abbreviations: SUDEP, sudden unexpected death in epilepsy. ⁎ Corresponding author at: Department of Neuroscience, Monash Medical Centre, Clayton, Victoria 3168, Australia. Tel.: +61 395942240; fax: +61 395946241. E-mail address: [email protected] (U. Seneviratne).
http://dx.doi.org/10.1016/j.yebeh.2014.11.007 1525-5050/Crown Copyright © 2014 Published by Elsevier Inc. All rights reserved.
unexpected death in epilepsy is the commonest cause of death directly attributable to epilepsy [4]. Definite SUDEP is defined as “sudden, unexpected, witnessed or unwitnessed, nontraumatic and nondrowning death, occurring in benign circumstances, in an individual with epilepsy, with or without evidence for a seizure and excluding documented status epilepticus, in which postmortem examination does not reveal a cause of death” [5]. Patients with epilepsy have 20 times higher risk of sudden unexpected death compared with the general population [6]. A recent systematic review of pooled data from multiple populationbased epidemiological studies reported an annual incidence of 1.16 SUDEP cases per 1000 patients with epilepsy [7]. The risk is increased to 5.9 per 1000 person-years in patients with chronic drug-refractory epilepsy and up to 9.3 per 1000 person-years in patients who continue to have seizures after epilepsy surgery [8]. Sudden unexpected death in epilepsy is second only to stroke in terms of the years of potential life lost from neurological conditions, underscoring the public health significance of this condition [7]. The unexpected occurrence of death results in an immense traumatic impact on the families of victims. It remains controversial whether all patients should be routinely informed of SUDEP [9]. Arguments against routinely informing patients about SUDEP largely ride on the fact that the risk of SUDEP is low, and information may cause undue distress impairing quality of life in some patients [10]. A patient has been reported to have developed psychogenic nonepileptic seizures after being
30
Z. Xu et al. / Epilepsy & Behavior 42 (2015) 29–34
informed about SUDEP during the initial consultation [11]. On the other hand, not informing about SUDEP would conflict with the patients' ethical right to know about all aspects of their medical condition. There is no consensus on the optimal way of communicating the risk of SUDEP to patients and caregivers [12]. The push to inform patients about SUDEP has largely been driven by patient support groups. The National Institute for Clinical Excellence (NICE) and the Scottish Intercollegiate Guidelines Network (SIGN) in the United Kingdom recommend disclosure of adequate information on SUDEP to every patient or their family or caregivers as a requisite of good practice [13,14]. The American Epilepsy Society and the Epilepsy Foundation Joint Task Force on SUDEP have taken a similar approach, recommending that information regarding SUDEP should be given as part of a comprehensive epilepsy education and that the timing and extent of discussion should be individualized [15]. However, the task force acknowledges the uncertainty about the optimal method of conveying this information [15]. Currently, the discussion of SUDEP has not been incorporated as one of the epilepsy quality measures suggested by the American Academy of Neurology [16,17]. Despite the prevalent recommendations on discussing SUDEP with every patient with epilepsy, the actual clinical practice varies considerably. According to a study, only 5% of the UK neurologists discussed SUDEP with every patient with epilepsy, while the majority (61%) limited the SUDEP discussion to patients with high risk factors or to patients who requested the information [18]. A survey of American neurologists noted that 35% never discussed SUDEP with their patients. While 29% of neurologists discussed SUDEP with every patients [17], the majority (38%) conducted the discussion only when the patient was at high risk of SUDEP [17]. Only 8% of Italian epileptologists discussed SUDEP with all patients, and 62% followed the practice of discussing SUDEP only with selected patients [19]. In Brazil, only 14% discussed SUDEP with all patients, 76% with a minority, and 10% did not discuss SUDEP with any of their patients [20]. The available data on the extent of SUDEP discussion have been derived from the physician's perspective, dependent on physician self-reporting, which is likely to be biased by an overestimate of actual clinical practice [19]. A recent retrospective case note review of patients with epilepsy found documentation of SUDEP discussion only in 4% of cases, implying the low prevalence of this practice in actual clinical care [21]. In general, neurologists are excellent at addressing the medical aspects of the disease (83%–94%) but underperform in counseling patients regarding epilepsy-specific safety measures (70%) including SUDEP [17]. The perceived risk of generating unnecessary anxiety among patients and families or caregivers is highlighted as the main reason for the reluctance among clinicians to discuss SUDEP with all patients [9,12,22]. However, surprisingly, the most pertinent question of whether patients with epilepsy wish to be informed about SUDEP has not been adequately addressed. In studies on pediatric patients with epilepsy, almost all parents wished to be informed about SUDEP [22,23]; however, the majority (61%) wished their child not to be informed [22]. To the best of our knowledge, no study has evaluated the adult patients' perspectives on SUDEP, in particular their degree of awareness and desire to be informed about the condition. Against this backdrop, we conducted a cross-sectional survey to explore these aspects from the patients' perspective. 2. Materials and methods Adult patients (18 years of age and above), referred to the tertiary Epilepsy Clinic at Monash Medical Centre, Melbourne, Australia between October 2012 and November 2013, were invited, after confirming the diagnosis of epilepsy, to participate in a survey to ascertain their understanding of SUDEP, and their wish to be informed of the condition. Patients who consented for the study were given a brief, 16-point survey questionnaire (Supplementary file) incorporating demographic data, education and employment status as per the Australian Bureau of Statistics
classification [24,25], duration of epilepsy, level of SUDEP awareness, preferences on receiving SUDEP information, and individual perceptions of their major concerns in relation to epilepsy. All patients wishing to get more SUDEP-related information from the treating neurologist were given the opportunity to discuss SUDEP during the subsequent consultation. Data were statistically analyzed using the IBM SPSS (Version 21) software package (IBM Corporation, New York, USA). Descriptive statistics included frequencies/percentages for categorical variables and mean/median and standard deviation for continuous variables. Multivariable analysis was performed with logistic regression to determine the variables among patients (age, gender, education level, duration of epilepsy, occupation) associated with (a) awareness of SUDEP and (b) willingness to be informed about SUDEP. The study was approved by the Human Research Ethics Committee of Monash Health. 3. Results A total of 112 adult patients were invited to participate, and 105 completed the survey questionnaire. Seven people did not consent to take part in the study. Responses from the patients are summarized in Table 1. 3.1. Demographics The study population consisted of 40 males and 65 females (M:F ratio = 1:1.3), with ages ranging from 18 to 79 years (mean = 41.4 ± 17.7). The large majority of patients (90.5%) had secondary education or above, which included a certificate (29.5%), bachelor degree (10.5%), and postgraduate degree (5.7%). The participants included clerical and administrative workers (20%), sales workers (13.3%), professionals (10.5%), technicians and trade workers (10.5%), laborers (7.6%), community and personal service workers (3.8%), and machinery operators and drivers (2.9%). Nearly a quarter of patients in the survey were unemployed or retired. 3.2. Disease factors Epilepsy duration varied widely from 2 to 840 months (mean = 108.3 ± 121.8 months). 3.3. Extent of information desired The majority of patients (62.9%) desired to know all information appropriate to their medical condition, while 32.4% only wanted to know a reasonable amount. A small minority of patients (4.7%) opted for a minimal amount of information. More than half of the patients (52.4%) thought that epilepsy was not associated with a higher risk of sudden death; however, most of the patients (89.5%) expressed the wish to get more information if the mortality risk is higher. 3.4. SUDEP awareness Patient awareness of SUDEP was poor, with only 15 (14.3%) patients of the group having previously heard of SUDEP. Of the patients with prior knowledge of SUDEP, 5 obtained the information from their neurologist, 2 from the general practitioner, 2 from epilepsy support groups, 1 from a nurse, and the rest from media and friends. A total of 15.2% of the patients thought that SUDEP was preventable, but the majority (79.1%) were unsure. Only 5 (4.8%) patients had personally known of another patient who had died from SUDEP. 3.5. Perceptions as to how information about SUDEP should be conveyed The vast majority of patients (89.5%) wished to be informed about SUDEP, with 59% requesting detailed information and 32.4% wishing basic information. Only 8.6% of the patients preferred not to be informed
Z. Xu et al. / Epilepsy & Behavior 42 (2015) 29–34
31
Table 1 Participants' responses to the survey questionnaire. Variable
Response
n (total: 105)
%
1
Age (years)
2
Gender
3
Current occupation
4
Education level
5
How long have you had epilepsy? (years)
6
With regard to your epilepsy, you would generally like to know …
7
Do you think that people with epilepsy have a higher risk of sudden death compared with people without epilepsy? If there is a higher risk, would you prefer to know the details?
18–25 26–50 51–75 N75 Male Female Managers Professionals Technicians and trades workers Community and personal service workers Clerical and administrative workers Sales workers Machinery operators and drivers Laborers Unemployed/retired Postgraduate degree Graduate diploma and graduate certificate Bachelor degree Advanced diploma and diploma Certificate Secondary education Primary education Preprimary education b1 1–5 5–10 10–20 N20 Everything possible A reasonable amount of information A minimal amount of information Yes No Yes No Yes No Neurologist General practitioner Emergency department doctor Internet Television/radio/newspaper/magazine Nurse Epilepsy support groups Friend Family member Other Detailed information Basic information None Neurologist General practitioner Emergency department doctor Internet Television/radio/newspaper/magazine Nurse Epilepsy support groups Friend Family member Other During the 1st consultation with the neurologist During the second consultation During subsequent consultations When seizure control worsens Yes No Do not know Yes No Driving restrictions Work restrictions Lifestyle restrictions Social stigma Side effects of medications Risk of seizures Sudden unexpected death in epilepsy (SUDEP)
22 51 30 2 40 65 0 11 11 4 21 14 3 8 33 6 1 11 5 31 41 10 0 17 42 19 19 8 66 34 5 50 55 94 11 15 90 5 2 0 6 4 1 2 4 0 0 62 34 9 89 8 1 5 0 1 0 0 0 0 43 11 23 24 16 6 83 5 100 52 16 21 11 25 38 16
21.0 48.6 28.5 1.9 38.1 61.9 0 10.5 10.5 3.8 20 13.3 2.9 7.6 31.4 5.7 0.9 10.5 4.8 29.5 39.1 9.5 0 16.2 40.0 18.1 18.1 7.6 62.9 32.4 4.7 47.6 52.4 89.5 10.5 14.3 85.7 33.3 13.3 0 40 26.7 6.7 13.3 26.7 0 0 59.0 32.4 8.6 85.6 7.7 0.9 4.8 0 0.9 0 0 0 0 42.6 10.8 22.8 23.8 15.2 5.7 79.1 4.8 95.2 49.5 15.2 20.0 10.5 23.8 36.2 15.2
8 9 10
Have you ever heard of a condition called sudden unexpected death in epilepsy (SUDEP)? If your answer is yes, where did you get this information from? (single or multiple responses)
11
How much information regarding SUDEP would you like to receive?
12
In the ideal situation, whom would you prefer to obtain this information from? (n = 104 as one patient did not give response)
13
If you feel that you need to know the details about SUDEP, when do you think this information should be provided? (n = 101 as 4 patients did not give response)
14
Do you think that SUDEP is preventable?
15
Are you personally aware of anyone who has suffered from SUDEP (sudden unexpected death in epilepsy?) What concerns you most in relation to epilepsy?
16
32
Z. Xu et al. / Epilepsy & Behavior 42 (2015) 29–34
of SUDEP. Among the patients wishing to receive information on SUDEP, 85.6% preferred to gain knowledge from their neurologist, 7.7% from their general practitioner, and 4.8% from the internet. A discussion on SUDEP would be preferred during the first consultation by 42.6% of the patients, while 33.6% wished to have the discussion in subsequent visits. A total of 23.8% of the patients wished to discuss SUDEP only when the seizure control worsened. 3.6. Relative significance of SUDEP from the patients' perspective Sudden unexpected death in epilepsy ranked only fifth in the patients' concerns in relation to their epilepsy. Driving restriction was raised as the most important epilepsy-related concern, followed by risk of seizure recurrence, medication side effects, and other lifestyle restrictions. 3.7. Factors influencing the SUDEP awareness Logistic regression analysis of patient characteristics such as age (p = 0.631), gender (p = 0.136), level of education (p = 0.997), occupation (p = 0.984), and duration of epilepsy (p = 0.301) found no significant association with the patients' awareness of SUDEP. 3.8. Factors influencing the willingness to be informed of SUDEP None of the patient characteristics such as age (p = 0.257), gender (p = 0.224), level of education (p = 0.439), occupation (p = 0.970), and duration of epilepsy (p = 0.554) was significantly associated with the patients' willingness to be informed of SUDEP on logistic regression analysis. 3.9. Adverse effects of participating in the study None of the participants experienced any undue stress or anxiety from participating in the study. 4. Discussion To our knowledge, the current study is the first to determine the adult patients' perspective on SUDEP. Despite serious personal and public health implications, the awareness of SUDEP among patients was surprisingly low (14.3%). The overwhelming majority of the patients (89.5%) in our surveyed population wished to be informed about sudden death in epilepsy. The vast majority wanted to receive information on SUDEP from the treating neurologist (85.6%), and many of them preferring to do so during the first consultation (42.6%). Both SUDEP awareness and patients' perspective on SUDEP education did not depend on any of the patient characteristics such as age, gender, duration of epilepsy, level of education, and occupation. Our results on the patients' eagerness to get medical information on SUDEP markedly contrasts with the general reluctance of the medical profession to inform patients of SUDEP due to the perceived risks of causing psychological harm to the patient [9,22]. In particular, ethical arguments hinge on the need to respect the patient's right to autonomy and unwanted consequences of informing patients [12]. The high proportion of adult patients wishing to be informed may seem like a surprising result. However, our results are similar to data obtained from the pediatric population exploring parental perceptions of SUDEP [22,23]. It is interesting to note that the majority of parents in these surveys chose to withhold the SUDEP information from their children, probably reflecting their desire to “protect” their child from potential distress due to this knowledge [22]. It may be argued that the medical professionals are choosing to behave in a paternalistic fashion, protecting their own patients from the distress of knowing about SUDEP, which is akin to how parents are choosing to withhold information from their child.
Our results are concordant with previously published data that patients with epilepsy and their carers are generally of the view that insufficient information about epilepsy and SUDEP is conveyed to them [22,23,26]. A qualitative study of 38 UK adult patients with epilepsy and their carers reported lack of information on SUDEP as a particular concern [26]. Similarly, in a study discussing the impact of bereavement on relatives, most relatives expressed that they would have preferred to know that epilepsy could be fatal [27]. The seriousness and the rarity of SUDEP did not dissuade the relatives from retrospectively wishing to be informed of the condition, with the authors stating that “many relatives had been either categorically told or led to believe that epilepsy could not be fatal. Most stated in retrospect that they would have preferred to know of the possibility of premature death, however, remote” [27]. However, it must be stressed that this particular group of relatives is likely to be biased by their personal experience of sudden death in the patient, which in itself, is a very rare event. We also explored patient perspectives on the best approach to convey the information on SUDEP. Most (76.2%) of our patients preferred to be informed about SUDEP during the first or during the subsequent consultation, whereas only a small proportion wanted information when the seizure control worsened. Our patients' wishes to be informed early are in line with the practice among American neurologists, where 30% provided this information on the first consultation and 10% on the second consultation [17]. This was a cross-sectional study, and patients were surveyed at different stages of their epilepsy ranging from early diagnosis to chronic. In order to study the influence of ongoing seizures on patients' willingness to know about SUDEP, we evaluated the treatment responsiveness at the time of survey based on medical records. We followed the consensus proposal on the definition of drug-resistant epilepsy by the International League Against Epilepsy [28]. Twenty-one participants qualified for the definition of drug-resistant epilepsy at the time of the survey (Table 2). Among them, 20 wished to have information on SUDEP, while only 1 did not want to get the information. The risks of incurring harm from providing information about SUDEP to patients and their carers may perhaps be an unwarranted alarm from the medical profession's desire to be paternalistic. Among pediatric neurologists, 74% chose to provide only selected information about SUDEP and were uncertain of the impact that this information had on their patients and carers [22]. However, the study also showed that although parents experienced initial short-term anxiety, the information did not result in significant immediate and long-term negative impact [22]. Many investigators have argued that disclosure of the SUDEP information should be on a case-by-case basis and limited to a selected group of patients with increased risk of mortality such as patients with refractory epilepsy and those noncompliant with treatment [3,9,10,12]. It has also been suggested that SUDEP could be discussed with patients without the need to provide specific details or use of the ‘death’ in routine discussion [29]. It is also of interest to note that patients in our survey had several other concerns in relation to their epilepsy. Driving restrictions topped the list of epilepsy-related concerns, followed by the risk of recurrent seizures, work restrictions, and lifestyle restrictions. Sudden unexpected death in epilepsy was reported as a concern by 15.2% of the patients. Previous studies have also identified restrictions on driving, independence, and employment as the top epilepsy-related concerns from the patients' perspective, even though sudden death is a worry [30,31]. Table 2 Preference for SUDEP discussion in patients with drug-responsive and drug-resistant epilepsy. Seizure control
Wish to discuss SUDEP information Yes
No
Total
Drug-responsive Drug-resistant
74 20
10 1
84 21
Z. Xu et al. / Epilepsy & Behavior 42 (2015) 29–34
We acknowledge some limitations of the study. The sample was from a tertiary center, introducing a potential sampling bias. It is possible those patients were motivated and willing to know more about their epilepsy. The survey was conducted in the clinic setting. One might argue that patients could have responded positively to impress the treating neurologist. However, the survey was conducted before the consult without any influence from the neurologist. Since the proportion of patients with SUDEP awareness was small and the proportion of patients wishing SUDEP information was high, a small sample size might have affected the analysis of statistical correlations. In a survey of this nature, a larger sample is likely to yield more robust results. Our study utilized a brief survey questionnaire to explore patients' perceptions. A mixed method using a combination of qualitative and quantitative data would have been more informative. Additional survey questions such as the preferred mode of SUDEP discussion (face-to-face vs. telephone, verbal vs combined verbal and pamphlets); the patients' preference for the presence of a relative, a nurse, or a social worker in the room; the particular aspects of SUDEP to be discussed; and the questions to assess the patients' understanding of SUDEP would have been useful. However, a longer questionnaire may adversely affect the reliability of responses and participation rate. We acknowledge the possible role of memory problems impacting on the patients' ability to comprehend and recall previous discussions on SUDEP. In our crosssectional survey design, we utilized information from medical records to evaluate the treatment responsiveness at the time of survey. It is possible that the drug-responsive state may have 275 changed on followup in some cases. We did not review the medical records for disparities with information from the survey. It could be argued that patients with medically refractory epilepsy were keener to obtain information on SUDEP. However, our analysis does show that patients' knowledge and willingness to receive information on SUDEP do not depend on the duration of epilepsy. Finally, we did not evaluate the potential for long-term psychological distress following disclosure of information in a naturalistic setting. Hence, we suggest that these findings should be retested in larger samples from multiple centers to help formulate policy decisions on disclosure of SUDEP information to patients with epilepsy. 5. Conclusions Sudden unexpected death in epilepsy deserves more attention from treating clinicians, public health professionals, and researchers because of its substantial personal, family, and public health burden. Although guidelines from various professional bodies have recommended imperative disclosure of SUDEP information to all patients diagnosed with epilepsy, there has been a general reluctance in the medical profession to discuss SUDEP, probably because of fear of causing psychological harm. Our study is the first of its kind in the adult population with epilepsy, demonstrating that the overwhelming majority of patients want to be informed about SUDEP and that they prefer to receive detailed information from the treating neurologist earlier than later. It might still be argued that given the sensitive nature of this topic, the treating clinician is in the best position to decide when and how to inform the patient about SUDEP depending on each individual case. We hope our results highlight the importance of disclosure of SUDEP information from the patients perspective and encourage health professionals and policy makers to incorporate SUDEP discussion in their practice and as a quality measure of clinical practice, respectively. Author contributions Dr. Xu: study design, data collection, and manuscript writing. Dr. Ayyappan: study design and manuscript writing. Dr. Seneviratne: study concept and design, data collection, data analysis, and critical review of the manuscript.
33
Disclosures and conflicts of interest Dr. Seneviratne has received an educational grant and speaker honoraria from UCB Pharma. Doctors Xu and Ayyappan report no disclosures. Source of funding None. Appendix A. Supplementary data Supplementary data to this article can be found online at http://dx. doi.org/10.1016/j.yebeh.2014.11.007. References [1] Neligan A, Bell GS, Johnson AL, Goodridge DM, Shorvon SD, Sander JW. The longterm risk of premature mortality in people with epilepsy. Brain 2011;134:388–95. [2] Nilsson L, Tomson T, Farahmand BY, Diwan V, Persson PG. Cause-specific mortality in epilepsy: a cohort study of more than 9,000 patients once hospitalized for epilepsy. Epilepsia 1997;38:1062–8. [3] Mohanraj R, Norrie J, Stephen LJ, Kelly K, Hitiris N, Brodie MJ. Mortality in adults with newly diagnosed and chronic epilepsy: a retrospective comparative study. Lancet Neurol 2006;5:481–7. [4] Duncan S, Brodie MJ. Sudden unexpected death in epilepsy. Epilepsy Behav 2011;21: 344–51. [5] Nashef L, So EL, Ryvlin P, Tomson T. Unifying the definitions of sudden unexpected death in epilepsy. Epilepsia 2012;53:227–33. [6] Ficker DM, So EL, Shen WK, Annegers JF, O'Brien PC, Cascino GD, et al. Populationbased study of the incidence of sudden unexplained death in epilepsy. Neurology 1998;51:1270–4. [7] Thurman DJ, Hesdorffer DC, French JA. Sudden unexpected death in epilepsy: assessing the public health burden. Epilepsia 2014;55:1479–85. [8] Shorvon S, Tomson T. Sudden unexpected death in epilepsy. Lancet 2011;378: 2028–38. [9] Brodie MJ, Holmes GL. Should all patients be told about sudden unexpected death in epilepsy (SUDEP)? Pros and cons. Epilepsia 2008;49:99–101. [10] 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. [11] Ng YS, Davenport R, Duncan S, Derry C. Complications of the SUDEP (sudden unexpected death in epilepsy) discussion. J Neurol Neurosurg Psychiatry 2013;84:e2. [12] Miller WR, Young N, Friedman D, Buelow JM, Devinsky O. Discussing sudden unexpected death in epilepsy (SUDEP) with patients: practices of health-care providers. Epilepsy Behav 2014;32:38–41. [13] The epilepsies: the diagnosis and management of the epilepsies in adults and children in primary and secondary care. Natl Inst Clin Excell 2013(1-116) [http://publications.nice.org.uk/the-epilepsies-the-diagnosis-and-management-of-the-epilepsies-in-adults-and-children-in-primary-and-cg137 (accessed June 9, 2014)]. [14] Scottish Intercollegiate Guidelines Network. Diagnosis and management of epilepsy in adults: a national clinical guideline. Edinburgh: Scottish Intercollegiate Guidelines Network; 2003. [15] So EL, Bainbridge J, Buchhalter JR, Donalty J, Donner EJ, Finucane A, et al. Report of the American Epilepsy Society and the Epilepsy Foundation joint task force on sudden unexplained death in epilepsy. Epilepsia 2009;50:917–22. [16] Fountain NB, Ness PCV, Swain-Eng R, Tonn S, Bever CT. Quality improvement in neurology: AAN epilepsy quality measures: Report of the Quality Measurement and Reporting Subcommittee of the American Academy of Neurology. Neurology 2011; 76:94–9. [17] 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. [18] Morton B. Sudden unexpected death in epilepsy (SUDEP): don't ask, don't tell? J Neurol Neurosurg Psychiatry 2006;77:199–202. [19] Vegni E, Leone D, Canevini MP, Tinuper P, Moja EA. Sudden unexpected death in epilepsy (SUDEP): a pilot study on truth telling among Italian epileptologists. Neurol Sci 2011;32:331–5. [20] Abdalla IG, Scorza CA, Cavalheiro EA, de Albuquerque M, de Almeida A-CG, Scorza FA. Attitudes of Brazilian epileptologists to discussion about SUDEP with their patients: truth may hurt, but does deceit hurt more? Epilepsy Behav 2013;27:470–1. [21] Waddell B, McColl K, Turner C, Norman A, Coker A, White K, et al. Are we discussing SUDEP? — a retrospective case note analysis. Seizure 2013;22:74–6. [22] Gayatri NA, Morrall MCHJ, Jain V, Kashyape P, Pysden K, Ferrie C. Parental and physician beliefs regarding the provision and content of written sudden unexpected death in epilepsy (SUDEP) information: parental beliefs regarding SUDEP information. Epilepsia 2010;51:777–82. [23] RamachandranNair R, Jack SM, Meaney BF, Ronen GM. SUDEP: what do parents want to know? Epilepsy Behav 2013;29:560–4. [24] Australian and New Zealand Standard Classification of Occupations (ANZSCO). Version 1.2 http://www.abs.gov.au/ausstats/[email protected]/Latestproducts/1220.0Main%
34
Z. Xu et al. / Epilepsy & Behavior 42 (2015) 29–34
20Features22013,%20Version%201.2?opendocument&tabname=Summary&prodno= 1220.0&issue=2013,%20Version%201.2&num=&view=; 2013. [accessed March 5, 2012]. [25] Australian Standard Classification of Education (ASCED). http://www.abs.gov.au/ ausstats/[email protected]/Latestproducts/1272.0Contents12001?opendocument&tabname= Summary&prodno=1272.0&issue=2001&num=&view=; 2001. [accessed March 5, 2012]. [26] Prinjha S. Many people with epilepsy want to know more: a qualitative study. Fam Pract 2005;22:435–41. [27] Nashef L, Garner S, Sander J, Fish DR, Shorvon SD. Circumstances of death in sudden death in epilepsy: interviews of bereaved relatives. J Neurol Neurosurg Psychiatry 1998;64:349–52.
[28] Kwan P, Arzimanoglou A, Berg AT, Brodie MJ, Allen Hauser W, Mathern G, et al. Definition of drug resistant epilepsy: consensus proposal by the ad hoc Task Force of the ILAE Commission on Therapeutic Strategies: definition of drug resistant epilepsy. Epilepsia 2010;51:1069–77. [29] Leach JP. SUDEP discussions with patients and families. Pract Neurol 2012;12:103–6. [30] Gilliam F, Kuzniecky R, Faught E, Black L, Carpenter G, Schrodt R. Patient-validated content of epilepsy-specific quality-of-life measurement. Epilepsia 1997;38:233–6. [31] Martin R, Vogtle L, Gilliam F, Faught E. What are the concerns of older adults living with epilepsy? Epilepsy Behav 2005;7:297–300.
Contents lists available at ScienceDirect
Epilepsy & Behavior journal homepage: www.elsevier.com/locate/yebeh
Sudden unexpected death in epilepsy (SUDEP): What do patients think? Zheyu Xu a, Sujith Ayyappan a, Udaya Seneviratne a,b,⁎ a b
Department of Neuroscience, Monash Medical Centre, Clayton, Victoria 3168, Australia Department of Medicine, Monash University, Melbourne 3800, Australia
a r t i c l e
i n f o
Article history: Received 10 August 2014 Revised 6 November 2014 Accepted 8 November 2014 Available online xxxx Keywords: Sudden unexpected death in epilepsy Epilepsy Patients' perspectives Mortality
a b s t r a c t Objectives: Sudden unexpected death in epilepsy (SUDEP) is a major cause of mortality in epilepsy. Despite its devastating consequences, SUDEP appears to be poorly discussed with patients by health professionals. The risk of causing psychological distress to the patient is highlighted as a reason for not discussing SUDEP. However, no studies have assessed the adult patients' views on this important question. We conducted this crosssectional study to evaluate the awareness and perspectives on SUDEP among adult patients with epilepsy. Methods: One hundred five consecutive adult patients with epilepsy, referred to the Epilepsy Clinic of a tertiary hospital between October 2012 and November 2013, were surveyed to ascertain their views and understanding of SUDEP. The data were analyzed using logistic regression to explore the association between patients' awareness of SUDEP and characteristics such as age, gender, duration of epilepsy, level of education, and employment. Results: Awareness of SUDEP among adult patients with epilepsy was poor (14.3%). However, the vast majority (89.5%) wished to be informed about SUDEP, and 59% requested detailed information. The treating neurologist was considered to be the most appropriate source of SUDEP information by 85.6% of patients. Multivariable analysis of the data showed no association between characteristics of patients (age, gender, duration of epilepsy, level of education, and employment) and their awareness of SUDEP or desire to get SUDEP-related information. Conclusions: Our study suggests that the majority of adult patients wish to be informed about SUDEP. This is in contrast to the general reluctance of medical professionals to inform all patients routinely about this condition. Crown Copyright © 2014 Published by Elsevier Inc. All rights reserved.
1. Introduction The most devastating consequence of epilepsy is death. Long-term, population-based, prospective studies have shown an increased risk of premature mortality in patients with epilepsy by more than twice that of the general population [1–3]. The mortality risk remains elevated irrespective of the degree of seizure control [1]. Cause-specific mortality analyses in the cohort with epilepsy demonstrate a higher proportion of deaths from malignant neoplasms, pneumonia and other respiratory diseases, cerebrovascular diseases, and cardiovascular causes. While some of the causes of mortality such as aspiration pneumonia have an obvious causal relationship, the mechanism of increased mortality in other situations remains unclear. The postulations include a higher degree of somatic and psychiatric comorbidities, socioeconomic deprivation, effect of long-term antiepileptic medications, common genetic predispositions, and role of inflammatory mechanisms [1,2]. Causes of mortality as a direct consequence of seizures and epilepsy include sudden unexpected death in epilepsy (SUDEP), accidental deaths due to epileptic seizures, and deaths from status epilepticus. Sudden Abbreviations: SUDEP, sudden unexpected death in epilepsy. ⁎ Corresponding author at: Department of Neuroscience, Monash Medical Centre, Clayton, Victoria 3168, Australia. Tel.: +61 395942240; fax: +61 395946241. E-mail address: [email protected] (U. Seneviratne).
http://dx.doi.org/10.1016/j.yebeh.2014.11.007 1525-5050/Crown Copyright © 2014 Published by Elsevier Inc. All rights reserved.
unexpected death in epilepsy is the commonest cause of death directly attributable to epilepsy [4]. Definite SUDEP is defined as “sudden, unexpected, witnessed or unwitnessed, nontraumatic and nondrowning death, occurring in benign circumstances, in an individual with epilepsy, with or without evidence for a seizure and excluding documented status epilepticus, in which postmortem examination does not reveal a cause of death” [5]. Patients with epilepsy have 20 times higher risk of sudden unexpected death compared with the general population [6]. A recent systematic review of pooled data from multiple populationbased epidemiological studies reported an annual incidence of 1.16 SUDEP cases per 1000 patients with epilepsy [7]. The risk is increased to 5.9 per 1000 person-years in patients with chronic drug-refractory epilepsy and up to 9.3 per 1000 person-years in patients who continue to have seizures after epilepsy surgery [8]. Sudden unexpected death in epilepsy is second only to stroke in terms of the years of potential life lost from neurological conditions, underscoring the public health significance of this condition [7]. The unexpected occurrence of death results in an immense traumatic impact on the families of victims. It remains controversial whether all patients should be routinely informed of SUDEP [9]. Arguments against routinely informing patients about SUDEP largely ride on the fact that the risk of SUDEP is low, and information may cause undue distress impairing quality of life in some patients [10]. A patient has been reported to have developed psychogenic nonepileptic seizures after being
30
Z. Xu et al. / Epilepsy & Behavior 42 (2015) 29–34
informed about SUDEP during the initial consultation [11]. On the other hand, not informing about SUDEP would conflict with the patients' ethical right to know about all aspects of their medical condition. There is no consensus on the optimal way of communicating the risk of SUDEP to patients and caregivers [12]. The push to inform patients about SUDEP has largely been driven by patient support groups. The National Institute for Clinical Excellence (NICE) and the Scottish Intercollegiate Guidelines Network (SIGN) in the United Kingdom recommend disclosure of adequate information on SUDEP to every patient or their family or caregivers as a requisite of good practice [13,14]. The American Epilepsy Society and the Epilepsy Foundation Joint Task Force on SUDEP have taken a similar approach, recommending that information regarding SUDEP should be given as part of a comprehensive epilepsy education and that the timing and extent of discussion should be individualized [15]. However, the task force acknowledges the uncertainty about the optimal method of conveying this information [15]. Currently, the discussion of SUDEP has not been incorporated as one of the epilepsy quality measures suggested by the American Academy of Neurology [16,17]. Despite the prevalent recommendations on discussing SUDEP with every patient with epilepsy, the actual clinical practice varies considerably. According to a study, only 5% of the UK neurologists discussed SUDEP with every patient with epilepsy, while the majority (61%) limited the SUDEP discussion to patients with high risk factors or to patients who requested the information [18]. A survey of American neurologists noted that 35% never discussed SUDEP with their patients. While 29% of neurologists discussed SUDEP with every patients [17], the majority (38%) conducted the discussion only when the patient was at high risk of SUDEP [17]. Only 8% of Italian epileptologists discussed SUDEP with all patients, and 62% followed the practice of discussing SUDEP only with selected patients [19]. In Brazil, only 14% discussed SUDEP with all patients, 76% with a minority, and 10% did not discuss SUDEP with any of their patients [20]. The available data on the extent of SUDEP discussion have been derived from the physician's perspective, dependent on physician self-reporting, which is likely to be biased by an overestimate of actual clinical practice [19]. A recent retrospective case note review of patients with epilepsy found documentation of SUDEP discussion only in 4% of cases, implying the low prevalence of this practice in actual clinical care [21]. In general, neurologists are excellent at addressing the medical aspects of the disease (83%–94%) but underperform in counseling patients regarding epilepsy-specific safety measures (70%) including SUDEP [17]. The perceived risk of generating unnecessary anxiety among patients and families or caregivers is highlighted as the main reason for the reluctance among clinicians to discuss SUDEP with all patients [9,12,22]. However, surprisingly, the most pertinent question of whether patients with epilepsy wish to be informed about SUDEP has not been adequately addressed. In studies on pediatric patients with epilepsy, almost all parents wished to be informed about SUDEP [22,23]; however, the majority (61%) wished their child not to be informed [22]. To the best of our knowledge, no study has evaluated the adult patients' perspectives on SUDEP, in particular their degree of awareness and desire to be informed about the condition. Against this backdrop, we conducted a cross-sectional survey to explore these aspects from the patients' perspective. 2. Materials and methods Adult patients (18 years of age and above), referred to the tertiary Epilepsy Clinic at Monash Medical Centre, Melbourne, Australia between October 2012 and November 2013, were invited, after confirming the diagnosis of epilepsy, to participate in a survey to ascertain their understanding of SUDEP, and their wish to be informed of the condition. Patients who consented for the study were given a brief, 16-point survey questionnaire (Supplementary file) incorporating demographic data, education and employment status as per the Australian Bureau of Statistics
classification [24,25], duration of epilepsy, level of SUDEP awareness, preferences on receiving SUDEP information, and individual perceptions of their major concerns in relation to epilepsy. All patients wishing to get more SUDEP-related information from the treating neurologist were given the opportunity to discuss SUDEP during the subsequent consultation. Data were statistically analyzed using the IBM SPSS (Version 21) software package (IBM Corporation, New York, USA). Descriptive statistics included frequencies/percentages for categorical variables and mean/median and standard deviation for continuous variables. Multivariable analysis was performed with logistic regression to determine the variables among patients (age, gender, education level, duration of epilepsy, occupation) associated with (a) awareness of SUDEP and (b) willingness to be informed about SUDEP. The study was approved by the Human Research Ethics Committee of Monash Health. 3. Results A total of 112 adult patients were invited to participate, and 105 completed the survey questionnaire. Seven people did not consent to take part in the study. Responses from the patients are summarized in Table 1. 3.1. Demographics The study population consisted of 40 males and 65 females (M:F ratio = 1:1.3), with ages ranging from 18 to 79 years (mean = 41.4 ± 17.7). The large majority of patients (90.5%) had secondary education or above, which included a certificate (29.5%), bachelor degree (10.5%), and postgraduate degree (5.7%). The participants included clerical and administrative workers (20%), sales workers (13.3%), professionals (10.5%), technicians and trade workers (10.5%), laborers (7.6%), community and personal service workers (3.8%), and machinery operators and drivers (2.9%). Nearly a quarter of patients in the survey were unemployed or retired. 3.2. Disease factors Epilepsy duration varied widely from 2 to 840 months (mean = 108.3 ± 121.8 months). 3.3. Extent of information desired The majority of patients (62.9%) desired to know all information appropriate to their medical condition, while 32.4% only wanted to know a reasonable amount. A small minority of patients (4.7%) opted for a minimal amount of information. More than half of the patients (52.4%) thought that epilepsy was not associated with a higher risk of sudden death; however, most of the patients (89.5%) expressed the wish to get more information if the mortality risk is higher. 3.4. SUDEP awareness Patient awareness of SUDEP was poor, with only 15 (14.3%) patients of the group having previously heard of SUDEP. Of the patients with prior knowledge of SUDEP, 5 obtained the information from their neurologist, 2 from the general practitioner, 2 from epilepsy support groups, 1 from a nurse, and the rest from media and friends. A total of 15.2% of the patients thought that SUDEP was preventable, but the majority (79.1%) were unsure. Only 5 (4.8%) patients had personally known of another patient who had died from SUDEP. 3.5. Perceptions as to how information about SUDEP should be conveyed The vast majority of patients (89.5%) wished to be informed about SUDEP, with 59% requesting detailed information and 32.4% wishing basic information. Only 8.6% of the patients preferred not to be informed
Z. Xu et al. / Epilepsy & Behavior 42 (2015) 29–34
31
Table 1 Participants' responses to the survey questionnaire. Variable
Response
n (total: 105)
%
1
Age (years)
2
Gender
3
Current occupation
4
Education level
5
How long have you had epilepsy? (years)
6
With regard to your epilepsy, you would generally like to know …
7
Do you think that people with epilepsy have a higher risk of sudden death compared with people without epilepsy? If there is a higher risk, would you prefer to know the details?
18–25 26–50 51–75 N75 Male Female Managers Professionals Technicians and trades workers Community and personal service workers Clerical and administrative workers Sales workers Machinery operators and drivers Laborers Unemployed/retired Postgraduate degree Graduate diploma and graduate certificate Bachelor degree Advanced diploma and diploma Certificate Secondary education Primary education Preprimary education b1 1–5 5–10 10–20 N20 Everything possible A reasonable amount of information A minimal amount of information Yes No Yes No Yes No Neurologist General practitioner Emergency department doctor Internet Television/radio/newspaper/magazine Nurse Epilepsy support groups Friend Family member Other Detailed information Basic information None Neurologist General practitioner Emergency department doctor Internet Television/radio/newspaper/magazine Nurse Epilepsy support groups Friend Family member Other During the 1st consultation with the neurologist During the second consultation During subsequent consultations When seizure control worsens Yes No Do not know Yes No Driving restrictions Work restrictions Lifestyle restrictions Social stigma Side effects of medications Risk of seizures Sudden unexpected death in epilepsy (SUDEP)
22 51 30 2 40 65 0 11 11 4 21 14 3 8 33 6 1 11 5 31 41 10 0 17 42 19 19 8 66 34 5 50 55 94 11 15 90 5 2 0 6 4 1 2 4 0 0 62 34 9 89 8 1 5 0 1 0 0 0 0 43 11 23 24 16 6 83 5 100 52 16 21 11 25 38 16
21.0 48.6 28.5 1.9 38.1 61.9 0 10.5 10.5 3.8 20 13.3 2.9 7.6 31.4 5.7 0.9 10.5 4.8 29.5 39.1 9.5 0 16.2 40.0 18.1 18.1 7.6 62.9 32.4 4.7 47.6 52.4 89.5 10.5 14.3 85.7 33.3 13.3 0 40 26.7 6.7 13.3 26.7 0 0 59.0 32.4 8.6 85.6 7.7 0.9 4.8 0 0.9 0 0 0 0 42.6 10.8 22.8 23.8 15.2 5.7 79.1 4.8 95.2 49.5 15.2 20.0 10.5 23.8 36.2 15.2
8 9 10
Have you ever heard of a condition called sudden unexpected death in epilepsy (SUDEP)? If your answer is yes, where did you get this information from? (single or multiple responses)
11
How much information regarding SUDEP would you like to receive?
12
In the ideal situation, whom would you prefer to obtain this information from? (n = 104 as one patient did not give response)
13
If you feel that you need to know the details about SUDEP, when do you think this information should be provided? (n = 101 as 4 patients did not give response)
14
Do you think that SUDEP is preventable?
15
Are you personally aware of anyone who has suffered from SUDEP (sudden unexpected death in epilepsy?) What concerns you most in relation to epilepsy?
16
32
Z. Xu et al. / Epilepsy & Behavior 42 (2015) 29–34
of SUDEP. Among the patients wishing to receive information on SUDEP, 85.6% preferred to gain knowledge from their neurologist, 7.7% from their general practitioner, and 4.8% from the internet. A discussion on SUDEP would be preferred during the first consultation by 42.6% of the patients, while 33.6% wished to have the discussion in subsequent visits. A total of 23.8% of the patients wished to discuss SUDEP only when the seizure control worsened. 3.6. Relative significance of SUDEP from the patients' perspective Sudden unexpected death in epilepsy ranked only fifth in the patients' concerns in relation to their epilepsy. Driving restriction was raised as the most important epilepsy-related concern, followed by risk of seizure recurrence, medication side effects, and other lifestyle restrictions. 3.7. Factors influencing the SUDEP awareness Logistic regression analysis of patient characteristics such as age (p = 0.631), gender (p = 0.136), level of education (p = 0.997), occupation (p = 0.984), and duration of epilepsy (p = 0.301) found no significant association with the patients' awareness of SUDEP. 3.8. Factors influencing the willingness to be informed of SUDEP None of the patient characteristics such as age (p = 0.257), gender (p = 0.224), level of education (p = 0.439), occupation (p = 0.970), and duration of epilepsy (p = 0.554) was significantly associated with the patients' willingness to be informed of SUDEP on logistic regression analysis. 3.9. Adverse effects of participating in the study None of the participants experienced any undue stress or anxiety from participating in the study. 4. Discussion To our knowledge, the current study is the first to determine the adult patients' perspective on SUDEP. Despite serious personal and public health implications, the awareness of SUDEP among patients was surprisingly low (14.3%). The overwhelming majority of the patients (89.5%) in our surveyed population wished to be informed about sudden death in epilepsy. The vast majority wanted to receive information on SUDEP from the treating neurologist (85.6%), and many of them preferring to do so during the first consultation (42.6%). Both SUDEP awareness and patients' perspective on SUDEP education did not depend on any of the patient characteristics such as age, gender, duration of epilepsy, level of education, and occupation. Our results on the patients' eagerness to get medical information on SUDEP markedly contrasts with the general reluctance of the medical profession to inform patients of SUDEP due to the perceived risks of causing psychological harm to the patient [9,22]. In particular, ethical arguments hinge on the need to respect the patient's right to autonomy and unwanted consequences of informing patients [12]. The high proportion of adult patients wishing to be informed may seem like a surprising result. However, our results are similar to data obtained from the pediatric population exploring parental perceptions of SUDEP [22,23]. It is interesting to note that the majority of parents in these surveys chose to withhold the SUDEP information from their children, probably reflecting their desire to “protect” their child from potential distress due to this knowledge [22]. It may be argued that the medical professionals are choosing to behave in a paternalistic fashion, protecting their own patients from the distress of knowing about SUDEP, which is akin to how parents are choosing to withhold information from their child.
Our results are concordant with previously published data that patients with epilepsy and their carers are generally of the view that insufficient information about epilepsy and SUDEP is conveyed to them [22,23,26]. A qualitative study of 38 UK adult patients with epilepsy and their carers reported lack of information on SUDEP as a particular concern [26]. Similarly, in a study discussing the impact of bereavement on relatives, most relatives expressed that they would have preferred to know that epilepsy could be fatal [27]. The seriousness and the rarity of SUDEP did not dissuade the relatives from retrospectively wishing to be informed of the condition, with the authors stating that “many relatives had been either categorically told or led to believe that epilepsy could not be fatal. Most stated in retrospect that they would have preferred to know of the possibility of premature death, however, remote” [27]. However, it must be stressed that this particular group of relatives is likely to be biased by their personal experience of sudden death in the patient, which in itself, is a very rare event. We also explored patient perspectives on the best approach to convey the information on SUDEP. Most (76.2%) of our patients preferred to be informed about SUDEP during the first or during the subsequent consultation, whereas only a small proportion wanted information when the seizure control worsened. Our patients' wishes to be informed early are in line with the practice among American neurologists, where 30% provided this information on the first consultation and 10% on the second consultation [17]. This was a cross-sectional study, and patients were surveyed at different stages of their epilepsy ranging from early diagnosis to chronic. In order to study the influence of ongoing seizures on patients' willingness to know about SUDEP, we evaluated the treatment responsiveness at the time of survey based on medical records. We followed the consensus proposal on the definition of drug-resistant epilepsy by the International League Against Epilepsy [28]. Twenty-one participants qualified for the definition of drug-resistant epilepsy at the time of the survey (Table 2). Among them, 20 wished to have information on SUDEP, while only 1 did not want to get the information. The risks of incurring harm from providing information about SUDEP to patients and their carers may perhaps be an unwarranted alarm from the medical profession's desire to be paternalistic. Among pediatric neurologists, 74% chose to provide only selected information about SUDEP and were uncertain of the impact that this information had on their patients and carers [22]. However, the study also showed that although parents experienced initial short-term anxiety, the information did not result in significant immediate and long-term negative impact [22]. Many investigators have argued that disclosure of the SUDEP information should be on a case-by-case basis and limited to a selected group of patients with increased risk of mortality such as patients with refractory epilepsy and those noncompliant with treatment [3,9,10,12]. It has also been suggested that SUDEP could be discussed with patients without the need to provide specific details or use of the ‘death’ in routine discussion [29]. It is also of interest to note that patients in our survey had several other concerns in relation to their epilepsy. Driving restrictions topped the list of epilepsy-related concerns, followed by the risk of recurrent seizures, work restrictions, and lifestyle restrictions. Sudden unexpected death in epilepsy was reported as a concern by 15.2% of the patients. Previous studies have also identified restrictions on driving, independence, and employment as the top epilepsy-related concerns from the patients' perspective, even though sudden death is a worry [30,31]. Table 2 Preference for SUDEP discussion in patients with drug-responsive and drug-resistant epilepsy. Seizure control
Wish to discuss SUDEP information Yes
No
Total
Drug-responsive Drug-resistant
74 20
10 1
84 21
Z. Xu et al. / Epilepsy & Behavior 42 (2015) 29–34
We acknowledge some limitations of the study. The sample was from a tertiary center, introducing a potential sampling bias. It is possible those patients were motivated and willing to know more about their epilepsy. The survey was conducted in the clinic setting. One might argue that patients could have responded positively to impress the treating neurologist. However, the survey was conducted before the consult without any influence from the neurologist. Since the proportion of patients with SUDEP awareness was small and the proportion of patients wishing SUDEP information was high, a small sample size might have affected the analysis of statistical correlations. In a survey of this nature, a larger sample is likely to yield more robust results. Our study utilized a brief survey questionnaire to explore patients' perceptions. A mixed method using a combination of qualitative and quantitative data would have been more informative. Additional survey questions such as the preferred mode of SUDEP discussion (face-to-face vs. telephone, verbal vs combined verbal and pamphlets); the patients' preference for the presence of a relative, a nurse, or a social worker in the room; the particular aspects of SUDEP to be discussed; and the questions to assess the patients' understanding of SUDEP would have been useful. However, a longer questionnaire may adversely affect the reliability of responses and participation rate. We acknowledge the possible role of memory problems impacting on the patients' ability to comprehend and recall previous discussions on SUDEP. In our crosssectional survey design, we utilized information from medical records to evaluate the treatment responsiveness at the time of survey. It is possible that the drug-responsive state may have 275 changed on followup in some cases. We did not review the medical records for disparities with information from the survey. It could be argued that patients with medically refractory epilepsy were keener to obtain information on SUDEP. However, our analysis does show that patients' knowledge and willingness to receive information on SUDEP do not depend on the duration of epilepsy. Finally, we did not evaluate the potential for long-term psychological distress following disclosure of information in a naturalistic setting. Hence, we suggest that these findings should be retested in larger samples from multiple centers to help formulate policy decisions on disclosure of SUDEP information to patients with epilepsy. 5. Conclusions Sudden unexpected death in epilepsy deserves more attention from treating clinicians, public health professionals, and researchers because of its substantial personal, family, and public health burden. Although guidelines from various professional bodies have recommended imperative disclosure of SUDEP information to all patients diagnosed with epilepsy, there has been a general reluctance in the medical profession to discuss SUDEP, probably because of fear of causing psychological harm. Our study is the first of its kind in the adult population with epilepsy, demonstrating that the overwhelming majority of patients want to be informed about SUDEP and that they prefer to receive detailed information from the treating neurologist earlier than later. It might still be argued that given the sensitive nature of this topic, the treating clinician is in the best position to decide when and how to inform the patient about SUDEP depending on each individual case. We hope our results highlight the importance of disclosure of SUDEP information from the patients perspective and encourage health professionals and policy makers to incorporate SUDEP discussion in their practice and as a quality measure of clinical practice, respectively. Author contributions Dr. Xu: study design, data collection, and manuscript writing. Dr. Ayyappan: study design and manuscript writing. Dr. Seneviratne: study concept and design, data collection, data analysis, and critical review of the manuscript.
33
Disclosures and conflicts of interest Dr. Seneviratne has received an educational grant and speaker honoraria from UCB Pharma. Doctors Xu and Ayyappan report no disclosures. Source of funding None. Appendix A. Supplementary data Supplementary data to this article can be found online at http://dx. doi.org/10.1016/j.yebeh.2014.11.007. References [1] Neligan A, Bell GS, Johnson AL, Goodridge DM, Shorvon SD, Sander JW. The longterm risk of premature mortality in people with epilepsy. Brain 2011;134:388–95. [2] Nilsson L, Tomson T, Farahmand BY, Diwan V, Persson PG. Cause-specific mortality in epilepsy: a cohort study of more than 9,000 patients once hospitalized for epilepsy. Epilepsia 1997;38:1062–8. [3] Mohanraj R, Norrie J, Stephen LJ, Kelly K, Hitiris N, Brodie MJ. Mortality in adults with newly diagnosed and chronic epilepsy: a retrospective comparative study. Lancet Neurol 2006;5:481–7. [4] Duncan S, Brodie MJ. Sudden unexpected death in epilepsy. Epilepsy Behav 2011;21: 344–51. [5] Nashef L, So EL, Ryvlin P, Tomson T. Unifying the definitions of sudden unexpected death in epilepsy. Epilepsia 2012;53:227–33. [6] Ficker DM, So EL, Shen WK, Annegers JF, O'Brien PC, Cascino GD, et al. Populationbased study of the incidence of sudden unexplained death in epilepsy. Neurology 1998;51:1270–4. [7] Thurman DJ, Hesdorffer DC, French JA. Sudden unexpected death in epilepsy: assessing the public health burden. Epilepsia 2014;55:1479–85. [8] Shorvon S, Tomson T. Sudden unexpected death in epilepsy. Lancet 2011;378: 2028–38. [9] Brodie MJ, Holmes GL. Should all patients be told about sudden unexpected death in epilepsy (SUDEP)? Pros and cons. Epilepsia 2008;49:99–101. [10] 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. [11] Ng YS, Davenport R, Duncan S, Derry C. Complications of the SUDEP (sudden unexpected death in epilepsy) discussion. J Neurol Neurosurg Psychiatry 2013;84:e2. [12] Miller WR, Young N, Friedman D, Buelow JM, Devinsky O. Discussing sudden unexpected death in epilepsy (SUDEP) with patients: practices of health-care providers. Epilepsy Behav 2014;32:38–41. [13] The epilepsies: the diagnosis and management of the epilepsies in adults and children in primary and secondary care. Natl Inst Clin Excell 2013(1-116) [http://publications.nice.org.uk/the-epilepsies-the-diagnosis-and-management-of-the-epilepsies-in-adults-and-children-in-primary-and-cg137 (accessed June 9, 2014)]. [14] Scottish Intercollegiate Guidelines Network. Diagnosis and management of epilepsy in adults: a national clinical guideline. Edinburgh: Scottish Intercollegiate Guidelines Network; 2003. [15] So EL, Bainbridge J, Buchhalter JR, Donalty J, Donner EJ, Finucane A, et al. Report of the American Epilepsy Society and the Epilepsy Foundation joint task force on sudden unexplained death in epilepsy. Epilepsia 2009;50:917–22. [16] Fountain NB, Ness PCV, Swain-Eng R, Tonn S, Bever CT. Quality improvement in neurology: AAN epilepsy quality measures: Report of the Quality Measurement and Reporting Subcommittee of the American Academy of Neurology. Neurology 2011; 76:94–9. [17] 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. [18] Morton B. Sudden unexpected death in epilepsy (SUDEP): don't ask, don't tell? J Neurol Neurosurg Psychiatry 2006;77:199–202. [19] Vegni E, Leone D, Canevini MP, Tinuper P, Moja EA. Sudden unexpected death in epilepsy (SUDEP): a pilot study on truth telling among Italian epileptologists. Neurol Sci 2011;32:331–5. [20] Abdalla IG, Scorza CA, Cavalheiro EA, de Albuquerque M, de Almeida A-CG, Scorza FA. Attitudes of Brazilian epileptologists to discussion about SUDEP with their patients: truth may hurt, but does deceit hurt more? Epilepsy Behav 2013;27:470–1. [21] Waddell B, McColl K, Turner C, Norman A, Coker A, White K, et al. Are we discussing SUDEP? — a retrospective case note analysis. Seizure 2013;22:74–6. [22] Gayatri NA, Morrall MCHJ, Jain V, Kashyape P, Pysden K, Ferrie C. Parental and physician beliefs regarding the provision and content of written sudden unexpected death in epilepsy (SUDEP) information: parental beliefs regarding SUDEP information. Epilepsia 2010;51:777–82. [23] RamachandranNair R, Jack SM, Meaney BF, Ronen GM. SUDEP: what do parents want to know? Epilepsy Behav 2013;29:560–4. [24] Australian and New Zealand Standard Classification of Occupations (ANZSCO). Version 1.2 http://www.abs.gov.au/ausstats/[email protected]/Latestproducts/1220.0Main%
34
Z. Xu et al. / Epilepsy & Behavior 42 (2015) 29–34
20Features22013,%20Version%201.2?opendocument&tabname=Summary&prodno= 1220.0&issue=2013,%20Version%201.2&num=&view=; 2013. [accessed March 5, 2012]. [25] Australian Standard Classification of Education (ASCED). http://www.abs.gov.au/ ausstats/[email protected]/Latestproducts/1272.0Contents12001?opendocument&tabname= Summary&prodno=1272.0&issue=2001&num=&view=; 2001. [accessed March 5, 2012]. [26] Prinjha S. Many people with epilepsy want to know more: a qualitative study. Fam Pract 2005;22:435–41. [27] Nashef L, Garner S, Sander J, Fish DR, Shorvon SD. Circumstances of death in sudden death in epilepsy: interviews of bereaved relatives. J Neurol Neurosurg Psychiatry 1998;64:349–52.
[28] Kwan P, Arzimanoglou A, Berg AT, Brodie MJ, Allen Hauser W, Mathern G, et al. Definition of drug resistant epilepsy: consensus proposal by the ad hoc Task Force of the ILAE Commission on Therapeutic Strategies: definition of drug resistant epilepsy. Epilepsia 2010;51:1069–77. [29] Leach JP. SUDEP discussions with patients and families. Pract Neurol 2012;12:103–6. [30] Gilliam F, Kuzniecky R, Faught E, Black L, Carpenter G, Schrodt R. Patient-validated content of epilepsy-specific quality-of-life measurement. Epilepsia 1997;38:233–6. [31] Martin R, Vogtle L, Gilliam F, Faught E. What are the concerns of older adults living with epilepsy? Epilepsy Behav 2005;7:297–300.