Parental and physician beliefs regarding the provision and content of written sudden unexpected death in epilepsy (SUDEP) information

Purpose: The 2007 UK National Institute for Health and Clinical Excellence (NICE) guidelines for epilepsy recommend disclosing the risk of sudden unexpected death in epilepsy (SUDEP) to patients. This recommendation is not undertaken routinely, and considerable variation in individual physician practice exists. Literature indicates wariness of causing distress and anxiety, particularly to children/young people and their families through disclosing a nonpreventable risk. There has been no systematic pediatric study examining parent/guardian information needs and beliefs for risk of SUDEP and its impact on seizure management. It is important to first address these concerns before routinely imparting SUDEP information to parents following NICE recommendations. Methods: Two questionnaire surveys: a questionnaire examining the provision by pediatric neurologists of SUDEP information, and questionnaires examining parental beliefs and implications at two time points regarding SUDEP information provided in a leaflet. Participants were included in the study if their child had an established diagnosis of epilepsy. Factors for exclusion were single unprovoked seizure, absence seizures, patients in remission, and active discontinuation of treatment. Results: The majority (74%) of pediatric neurologists provided SUDEP information only to a select group of children with epilepsy and were uncertain about the effect such information would have upon the parent and child. Conversely, 91% of parents expected the pediatric neurologist to provide SUDEP risk information. The provision of this information did not have a significant immediate and longer‐term negative impact. Discussion: The majority of parents wanted to know about SUDEP and its associated risks. Whenever possible, SUDEP information should be given by the physician accompanied by an information leaflet.     

Sudden unexpected death in epilepsy (SUDEP): don't ask,don't tell?

BACKGROUND: The National Institute for Clinical Excellence in the UK has issued guidelines stating all individuals with epilepsy be given information about sudden unexpected death in epilepsy (SUDEP). METHODS: We conducted a survey of current practice among UK neurologists, using a questionnaire sent to all practising neurologists in the UK listed on the Association of British Neurologists database, asking under what circumstances they told patients about SUDEP. RESULTS: Of the validated respondents, 5% discussed SUDEP with all patients, 26% with a majority, 61% with a few, and 7.5% with none. The commonest reasons for SUDEP to be discussed were the patient asking about it and the neurologist counselling people with known risk factors for SUDEP. CONCLUSIONS: The variation we found, although not necessarily in tune with the guidelines, reflects the variation in patients' need for knowledge about their condition.     

Impact on Priority Setting

Summary: It is now acknowledged that an excess mortality is associated with epilepsy, over and above that of the underlying disease. This risk is even greater in uncontrolled epilepsy. Because sudden unexplained death in epilepsy (SUDEP) appears to be seizure-related, it is likely that this excess mortality would be reduced if seizures were better controlled or, ideally, stopped. Patients and physicians are in the fortunate position of being able to choose from more effective treatments for epilepsy than at any time in the past. Reductions in mortality, however, must go hand in hand with improved epilepsy care in general. In the past, this area has been overlooked and underfunded. There is some evidence that specialist epilepsy clinics are better at managing cases of epilepsy than general neurology clinics or general medical services. Investing in dedicated epilepsy services could arguably lead to a reduction in mortality. Such investment would also lead to improved provision of counseling and information services for people with epilepsy. It is not yet possible to determine the effects of these interventions or to set targets for a reduction in mortality in people with epilepsy, because there is a lack of accurate data on the prevalence of epilepsy and the rate of SUDEP. Obtaining these figures should perhaps be our first aim. The relevance of these issues to the management of SUDEP are discussed with regard to the United Kingdom Health Service.     

Abbreviated report of the NIH/NINDS workshop on sudden unexpected death in epilepsy

Sudden unexpected death in epilepsy (SUDEP) is a devastating complication of epilepsy and is not rare. The NIH and National Institute of Neurological Disorders and Stroke sponsored a 3-day multidisciplinary workshop to advance research into SUDEP and its prevention. Parallel sessions were held: one with a focus on the science of SUDEP, and the other with a focus on issues related to the education of health care practitioners and people with epilepsy. This report summarizes the discussions and recommendations of the workshop, including lessons learned from investigations of sudden infant death syndrome (SIDS), sudden cardiac death, autonomic and respiratory physiology, medical devices, genetics, and animal models. Recommendations include educating all people with epilepsy about SUDEP as part of their general education on the potential harm of seizures, except in extenuating circumstances. Increasing awareness of SUDEP may facilitate improved seizure control, possibly decreasing SUDEP incidence. There have been significant advances in our understanding of the clinical and physiologic features of SIDS, sudden cardiac death, and SUDEP in both people and animals. Research should continue to focus on the cardiac, autonomic, respiratory, and genetic factors that likely contribute to the risk of SUDEP. Multicenter collaborative research should be encouraged, especially investigations with direct implications for the prevention of SUDEP. An ongoing SUDEP Coalition has been established to facilitate this effort. With the expansion of clinical, genetic, and basic science research, there is reasonable hope of advancing our understanding of SUDEP and ultimately our ability to prevent it.     

Provision of sudden unexpected death in epilepsy (SUDEP) information among Malaysian parents of children with epilepsy

Sudden unexpected death in epilepsy (SUDEP) is an important cause of mortality in epilepsy. To date, there is only one published UK study evaluating information provision of SUDEP among parents of children with epilepsy (CWE), and there are no studies published from Asia. Although SUDEP information provision is recommended among parents of CWE, it is uncertain if these recommendations are applicable to Asian countries due to the different cultural attitude towards epilepsy. Our prospective cohort study consisted of multiethnic parents of children with epilepsy (CWE) seen in a tertiary hospital in Malaysia. Information on SUDEP was delivered to parents using an epilepsy educational software program. Participants completed a set of standardized questionnaire and Depression Anxiety Stress Scales–Short Form (DASS-21) immediately after and retested 3–6 months after the SUDEP information provision. A total of 127 parents (84 mothers) participated in the study. The CWE consisted of 3 ethnic groups (38% Malay, 30% Chinese, 32% Indian) with a mean age of 9.6 years. Majority (70.9%) felt positive after SUDEP information provision, 90.6% wanted SUDEP discussion for themselves with 70.1% wanted SUDEP discussion with their child, and a lower proportion (58.3%) would discuss SUDEP with their child. None of the participants reported increased symptoms of depression, stress or anxiety attributed to SUDEP information provision. Most parents took steps to reduce SUDEP risk, and most parents did not report an impact on their own functioning. However, there was an increase in parental report over time of impact on their child's functioning following SUDEP information (P < 0.05). In conclusion, most Malaysian parents of CWE wanted SUDEP information. Following SUDEP information disclosure, majority did not report negative emotions; however, an increase in parents over time reported an impact on their child. Our findings reiterate that provision of SUDEP information should form part of care of CWE and parents should receive ongoing support as they undergo a period of parenting adjustment when dealing with the information provided.     

Sudden unexpected death in epilepsy (SUDEP): What do patients think?

ObjectivesSudden 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 cross-sectional study to evaluate the awareness and perspectives on SUDEP among adult patients with epilepsy.MethodsOne 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.ResultsAwareness 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.ConclusionsOur 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.     

Information on Sudden Deaths from Epilepsy

Summary: Epilepsy Bereaved? is a charity representing those who have experienced the sudden death of a loved one due to epilepsy. Many individuals with epilepsy, as well as their partners, relatives, and friends, are unaware of the risk for sudden unexpected death in epilepsy (SUDEP). Much of the literature available does not include information on SUDEP. When information is given, the risk for SUDEP may be underestimated. Epilepsy Bereaved? believes that people with epilepsy and their partners, families, and friends have the same right to be informed as any others dealing with a chronic condition, unless they express a wish to the contrary. Information should include the risk for SUDEP. Bereaved families agree that they would rather have been informed of the risks than left ignorant. Increased awareness and understanding of this condition may help improve treatment compliance and enable individuals to take measures to reduce the risk for sudden death.     

SUDEP counseling: Where do we stand?

Sudden unexpected death in epilepsy (SUDEP) is the leading cause of epilepsy-related death in children and adults living with epilepsy. Several recent clinical practice guidelines have recommended that all individuals living with epilepsy and their caregivers be informed about SUDEP as a part of routine epilepsy counseling. Furthermore, several studies over the last two decades have explored the state of SUDEP counseling. Patients with epilepsy and their families want to be informed about the risk of SUDEP at or near the time of diagnosis, and preferably in person. Despite guideline recommendations, many pediatric and adult neurologists do not routinely inform individuals with epilepsy and their families about SUDEP. Some neurologists discuss SUDEP with only a subset of patients with epilepsy, such as those with risk factors like frequent generalized or focal to bilateral tonic–clonic seizures, nocturnal seizures, noncompliance, or medically refractory epilepsy. Proponents of routine SUDEP counseling argue that patients with epilepsy and their families have a “right to know” and that counseling may positively impact epilepsy self-management (i.e., behavioral modification and risk reduction). Some neurologists still believe that SUDEP counseling may cause unnecessary stress and anxiety for patients and their families (although this is erroneous) and that they also have a “right not to know.” This narrative review explores the current gaps in SUDEP counseling, patients' and caregivers' perspectives of SUDEP counseling, and SUDEP prevention.     

Sudden Unexpected Death in Epilepsy: Addressing the Challenges

Epilepsy is associated with a higher rate of premature death than the general population, and the commonest cause of epilepsy mortality is sudden unexpected death in epilepsy (SUDEP). It is difficult to quantify because of the variable reporting of this cause of death. Death occurs due to autonomic deregulation of cardio-respiratory pathways as a result of seizures. Measures to reduce cardio-respiratory dysfunction are discussed together with the importance of seizure control in preventing SUDEP. The role of seizure detection devices, antiepileptic drugs and the importance of providing information about SUDEP to people with epilepsy are highlighted. There is increasing interest in SUDEP and some current initiatives are discussed.     

Epilepsy and Sudden Death: A Personal View

Summary: Epilepsy Bereaved? is a charity that aims to inform, support, and empower partners, relatives, and friends of people affected by sudden unexpected death in epilepsy (SUDEP). It also works toward preventing mortality due to SUDEP through raising awareness and promoting research. The aim of Epilepsy Bereaved? is to enable bereaved families to be heard. The charity represents more than 200 families in the United Kingdom and overseas. This article presents some of their experiences. All the deaths involved young people, many of whom had been recently diagnosed with epilepsy or had experienced only infrequent seizures. Their families report that they have been unaware of the risk for sudden death. Explanations and counseling from health professionals were rare. This was often felt by families of the victims to be symptomatic of a continuing failure to provide adequate information and support to people with epilepsy. In many cases, distress was exacerbated by the belief that death certificates were inaccurate. In supporting this workshop, Epilepsy Bereaved? hopes that further progress will be made to prevent deaths and suffering from this tragic syndrome.     

Coroner and medical examiner documentation of sudden unexplained deaths in epilepsy

BACKGROUND: Prevalence data for sudden unexplained death in epilepsy (SUDEP) are hampered by its underuse as a final diagnosis on death certificates in appropriate cases. Few data exist about how coroners (COs) and medical examiners (MEs) in the United States use the diagnosis of SUDEP. METHODS: A survey instrument that addressed demographics, professional background, annual cases of epilepsy, seizure history, percentage of post-mortem examinations, cause of death, and use of SUDEP as a diagnosis was sent to all COs and MEs in the United States. Unadjusted comparisons between categorical variables used chi2 tests. A multiple regression model examined the odds of respondents considering SUDEP to be a valid diagnosis. RESULTS: Of 2995 surveys, 80.7% went to COs and 19.3% to MEs. The response rate was 15.9% for COs and 21.8% for MEs. Acknowledgment of SUDEP as a valid entity was greatest among pathologists (83.5%) versus other physicians and non-physicians (P< .001) and correlated with higher autopsy rates and seeing more cases of epilepsy. In actual practice, SUDEP was not used routinely as a death certificate diagnosis in most cases with no cause of death found at autopsy by any group in the survey regardless of title, educational background, location, autopsy rate, or number of seizure cases seen annually. CONCLUSIONS: SUDEP appears to be an underused final diagnosis by COs and MEs throughout the United States. There is a need to educate officials at all levels about this diagnosis in persons who have epilepsy with no other cause of death.     

Knowing the risk of SUDEP: Two family's perspectives and The Danny Did Foundation

There is much debate in the epilepsy community about whether neurologists should discuss the risk of sudden unexpected death in epilepsy (SUDEP) with their patients and family members. Those in favor purport that patients have a right to know about SUDEP. Opponents say the risk is so low that discussions only worry patients and families, especially if there is nothing that can be done to prevent SUDEP. North American surveys show that the epilepsy community knows little about SUDEP and neurologists are unlikely to talk about it. However, surveys of those bereaved by SUDEP show that an overwhelming majority of the parents, spouses, and family members want to be told about SUDEP immediately after the diagnosis of epilepsy. This article is written by two families bereaved by SUDEP and their strong belief that neurologists should have the discussion about the risk of SUDEP soon after the diagnosis of epilepsy.     

Sudden unexpected death in epilepsy (SUDEP): what every neurologist should know

This review aims to empower general neurologists to provide better informed person‐centred advice on sudden unexpected death in epilepsy (SUDEP) to people with epilepsy in order to help keep them safe. Past and present evidence is consolidated in order to inform readers about SUDEP, and up‐to‐date insights into the epidemiology, diagnostic classification, pathophysiology, risk factors, influence of co‐morbidity, and importance of sensitive person‐centred communication are outlined. This review provides “fingertip” information to the practicing neurologist with regards to identifying and communicating risks for SUDEP and suggests practical measures for managing these risks in partnership with the patient.     

A qualitative study of the reactions of young adults with epilepsy to SUDEP disclosure,perceptions of risks,views on the timing of disclosure,and behavioural change

ObjectiveWe examined young adults' responses to information about sudden unexpected death in epilepsy (SUDEP).MethodConsecutive patients attending a specialist epilepsy clinic, aged 16–30 years, with a Hospital Anxiety and Depression Scale (HADS) score of < 11, and who had been told about SUDEP at least 2 weeks previously, were invited to the study. Semistructured in-depth interviews were conducted by an experienced qualitative interviewer until data saturation was achieved. An inductive thematic analysis of the data was conducted, facilitated by the use of the qualitative data analysis software (NVivo). The study was approved by the South East Scotland Research Ethics Committee.ResultsTwenty-seven patients (15 females) aged 18–29 years were interviewed. All said that they were satisfied with the information that they had received about SUDEP. However, at the interview, their understanding of SUDEP was often limited and incorrect. Many were not aware of their own SUDEP risk status. Twenty-two (81%) said that everyone should be told about SUDEP. Most thought that disclosure should happen at the time of the diagnosis or soon after, preferably in a face-to-face consultation; clinician judgment on timing was highlighted. Few reported seeking further information on SUDEP for themselves beyond the initial consultation. Most were either untroubled by the initial disclosure of SUDEP or reported initial anxiety that was not long-lasting. A minority reported changing their behavior, for example, improving medication adherence, but most reported no change. The majority expressed a fatalistic view of SUDEP as something outside of their control.SignificanceOur results suggest that clinicians can be more confident that most of their young adult patients want to know about SUDEP, want to be told early, and do not have long-lasting anxiety due to its disclosure. Getting information on SUDEP will not, on its own, increase medication adherence in most patients. Methods to increase patient understanding of SUDEP are needed.     

Sudden unexpected death in epilepsy (SUDEP) disclosure in pediatric epilepsy: An Italian survey on “to tell or not to tell”

Background and objectivesAlthough there has recently been significant debate regarding the importance of disclosing the risk of SUDEP, professional societies and clinical practice guidelines currently recommend that the risk of SUDEP be disclosed as part of a comprehensive epilepsy education program. Therefore, the primary aim of the present study was to examine whether healthcare providers treating pediatric patients with epilepsy in Italy would disclose the risk of SUDEP to the parents of children with epilepsy.MethodsThe present study assessed data from a questionnaire that collected sociodemographic information and clinicians' attitudes towards SUDEP. The survey was available online from September to December 2015. Chi-squared (χ²) tests and multivariate logistic regression analysis were performed when appropriate, and a qualitative content analysis of open-ended questions was performed.FindingsA total of 114 medical doctors (71 females and 43 males) completed the questionnaire. Of the respondents, 18 (16.2%) stated that all patients should be counseled about SUDEP, 22 (19.8%) thought that the majority should be, 58 (52.3%) said that only a minority should be, and 13 (11.7%) believed none should be. With respect to physicians' experience in counseling about SUDEP, only 2 (1.8%) counseled all their patients. A univariate logistic regression analysis showed that the factors associated with “not counseling about SUDEP at all” were the low number of patients with epilepsy they took care of (p < 0.01), fewer years of experience (p = 0.03), and the belief that it was safe from a legal point of view (p < 0.001), The main reasons for counseling about SUDEP were refractory course of epilepsy (79%) and if the parent/patient requested information (65%). Additionally, the findings of the qualitative analysis highlighted the emotional difficulties that neuropediatricians encounter when dealing with the disclosure of SUDEP.ConclusionsThe present findings showed that a minority of neuropediatricians in Italy counseled all parents of their patients about SUDEP. Educational training may help physicians better communicate with the patient/parents regarding this difficult issue.     

Incidence of sudden unexpected death in epilepsy in community-based cohort in China

ObjectiveSudden unexpected death in epilepsy (SUDEP) is associated with the high premature mortality observed among people with epilepsy. It is, however, considered a rare event in China, probably because of lack of awareness and limitation of studies in the country. We aimed to provide some initial estimation of the burden of SUDEP in China.MethodsWe established a large Chinese community-based cohort of people with epilepsy between January 2010 and December 2011. For any participant who died during follow-up, detailed information on cause of death was obtained using a specifically designed Verbal Autopsy Questionnaire. All cases were reviewed by a multidisciplinary expert panel and reinvestigated if necessary. Sudden unexpected death in epilepsy incidence rates were estimated and case details provided.ResultsThe cohort consisted of 1562 people and during a median 5 years follow-up, 72 deaths were reported. The all-causes death incidence was 11.23 (95% CI 8.86–14.07) per 1000 person-years. Fifteen died suddenly and unexpectedly in a reasonable state of health in the week preceding death. We recorded detailed information of these 15 deaths. Thirteen were considered to be probable SUDEP and two possible SUDEP. The incidence of probable SUDEP was 2.03 (95% CI 1.13–3.38) per 1000 person-years, and the incidence of all suspected (probable and possible) SUDEP was 2.34 (95% CI 1.36–3.77) per 1000 person-years.SignificanceThe incidence of SUDEP was relatively high among Chinese people with epilepsy when compared with that in previous community-based studies from high-income countries. The burden of SUDEP in China requires further assessments.     

SUDEP and epilepsy‐related mortality in pregnancy

Although data are limited, research in 2004 estimated a 10‐fold increase in mortality in pregnancy in the United Kingdom in women with epilepsy (WWE) compared to women without epilepsy. We highlight epilepsy mortality in pregnancy based on the 2011 report of the United Kingdom Confidential Enquiries into Maternal Deaths, relating its findings to previous reports and epilepsy‐rates in pregnancy. Among 2,291,493 maternities (2006–2008), we estimated 0.6% or 13,978 were in WWE. Fourteen deaths were epilepsy‐related, of which 11 (79%) were sudden and unexpected (SUDEP). Nine occurred during pregnancy and five were postpartum. Nine (64%) were in women taking lamotrigine, seven as monotherapy. We estimated that 1:1,000 women died from epilepsy (mostly SUDEP) during or shortly after pregnancy. Epilepsy‐related mortality is a significant risk in pregnancy. Antiepileptic drug–related factors may be relevant. The high proportion of women taking lamotrigine may reflect United Kingdom prescribing practice. Recent observations from the European and International Registry of Antiepileptic Drugs and Pregnancy (EURAP), whereby women on lamotrigine, the levels of which significantly decrease in pregnancy, had more difficulties with epilepsy control, argue against this being the sole explanation. Given the potential risks, every attempt should be made to prevent seizures, particularly convulsive, during pregnancy and postpartum. This, we believe, includes being proactive in maintaining lamotrigine levels during pregnancy. A PowerPoint slide summarizing this article is available for download in the Supporting Information section here .     

Sudden unexpected death in epilepsy. Risk factors, possible mechanisms and prevention: a reappraisal

People with epilepsy are more likely to die prematurely than those without epilepsy. The most common epilepsy-related category of death is sudden unexpected death in epilepsy (SUDEP), accounting for up to one fifth of epilepsy deaths in some series. SUDEP is more common in populations of people with intractable epilepsy, the annual incidence being as high as one in 200 patient years in these settings. The majority of people dying with SUDEP have a history of generalised tonic clonic seizures, and high seizure frequency and polytherapy also seem to be risk factors. The goal of treatment should therefore be seizure freedom, using the lowest effective number and dosage of AEDs. Evidence for many other risk factors is conflicting. The most commonly suggested mechanisms for SUDEP are cardiac abnormalities and apnoea, but the cause of SUDEP is still unknown. Clarification of risk factors and establishment of the mechanisms of SUDEP are important so that as many people as possible can be saved from SUDEP.     

SUDEP: overview of definitions and review of incidence data.

The classification, occurrence, and predictors of sudden unexpected and unexplained death in individuals with epilepsy (SUDEP) have received considerable attention over the last few years. Specific criteria for the classification of definite, probable, possible, and not SUDEP implemented in United States epidemiologic studies are presented. The incidence of SUDEP in different epilepsy populations is presented. SUDEP is a real phenomenon, because the occurrence of such deaths, especially at relatively young ages, among individuals with epilepsy is far greater (perhaps 40-fold) than among those without epilepsy. SUDEP incidence rates are lower in population-based studies, higher in referral populations and clinical trials of adjunct drugs for complex partial epilepsy, and highest for surgical series. Seizure severity appears to be the strongest risk factor for SUDEP because higher rates are reported from studies of individuals with intractable epilepsy. Other potential risk factors, including sex, seizure etiology, younger age at onset, and partial-onset seizures, are unresolved.     

Cardiorespiratory abnormalities during epileptic seizures

Sudden unexpected death in epilepsy (SUDEP) is a leading cause of death in young and otherwise healthy patients with epilepsy, and sudden death is at least 20 times more common in epilepsy patients as compared to patients without epilepsy. A significant proportion of patients with epilepsy experience cardiac and respiratory complications during seizures. These cardiorespiratory complications are suspected to be a significant risk factor for SUDEP. Sleep physicians are increasingly involved in the care of epilepsy patients and a recognition of these changes in relation to seizures while a patient is under their care may improve their awareness of these potentially life-threatening complications that may occur during sleep studies. This paper details these cardiopulmonary changes that take place in relation to epileptic seizures and how these changes may relate to the occurrence of SUDEP.