"Untold Distress" - How did the COVID-19 pandemic affect those who had previously experienced an epilepsy-related bereavement?

PURPOSE: This study explores the impact of the COVID-19 pandemic and lockdown on people with lived experience of sudden bereavement as a consequence of an epilepsy-related death. METHOD: We developed an online survey with fixed choice and open-ended response formats to collect data on grief symptoms and well-being during the pandemic. A total of 275 people bereaved by epilepsy-related deaths between 1980-2020 participated in this study: with 79 participants providing free-text responses for inductive thematic analysis. RESULTS: In total, 84% of participants reported a bereavement following a sudden death of a person aged under 40, with 22% aged 19 and under. The majority (77% of participants) reported they had been thinking more about the person who died compared to before the COVID-19 outbreak and 54% had experienced more distressing flashbacks to the time of death. Additionally, 61% reported more difficulties falling asleep and staying asleep and 88% of participants reported that the outbreak and response measures had negatively impacted upon their mental health. Medication was being taken for a diagnosed mental health condition by 33% of participants at the time of the study. We categorized these negative experiences during COVID in to four main-themes - 'Family', 'Lifestyle', 'Personal Well-being' and 'Health Services and Shielding Populations'. The 'Personal Well-being' theme was inextricably linked to grief symptoms including 'reflection on the death', 're-exposure to feeling', 'grief', 'salience of sudden deaths in the media' and 'inability to commemorate anniversaries and rituals'. These findings were consistent for bereaved people irrespective of the recency of the death. CONCLUSION: This study highlights the impact of the disruption caused by the pandemic on the grief-management of those bereaved by epilepsy-related death. Grief is not static and its management is connected to the psychosocial and formal support structures that were disrupted during the pandemic. The removal of these supports had an adverse effect upon the mental health and well-being of many bereaved. There is an urgent need for a system-wide transformation of epilepsy and mental health services to be inclusive of the needs and experiences of people impacted by sudden deaths in epilepsy and the contribution of the specialist service developed by families and clinicians to meet this gap.

Paediatric sudden unexpected death in epilepsy: A parental report cohort.

BACKGROUND: Sudden unexpected death in epilepsy (SUDEP) accounts for a large percentage of deaths in children with epilepsy. Contributing factors to paediatric SUDEP are incompletely understood. AIMS OF STUDY: The Epilepsy Deaths Register (EDR) is an anonymized register that compiles information on deaths related to epilepsy, across all ages and epilepsy classifications. Using the EDR, we sought to identify key risk factors for SUDEP in children to assist the development of preventive measures. METHODS: All registrations between the ages of 1 and 16 years were reviewed to identify definite or probable SUDEP. These cases were analysed to identify common demographics, comorbidities, monitoring, treatments and circumstances near to the deaths. RESULTS: We identified forty-six cases (27 males) of definite or probable SUDEP. Paediatric SUDEP is more common in a 12- to 16-year age group and in those with neuro-disability. Most paediatric SUDEP occurs during apparent sleep. There were four cases with a vagus nerve stimulator. SUDEP can occur early after the onset of seizures. CONCLUSIONS: This is the largest single cohort of SUDEP reported in children. Reports from caregivers can augment population data. Surveillance in sleep is a priority area of development.

Educational attainment of children born to mothers with epilepsy.

OBJECTIVE: Small prospective studies have identified that children exposed to valproate in utero have poorer scores on cognitive testing. We wanted to identify whether children exposed to antiepileptic drugs (AEDs) in utero have poorer school performance. METHODS: We used anonymised, linked, routinely collected healthcare records to identify children born to mothers with epilepsy. We linked these children to their national attainment Key Stage 1 (KS1) tests in mathematics, language and science at the age of 7 and compared them with matched children born to mothers without epilepsy, and with the national KS1 results. We used the core subject indicator (CSI) as an outcome measure (the proportion of children achieving a minimum standard in all subjects) and the results in individual subjects. RESULTS: We identified 440 children born to mothers with epilepsy with available KS1 results. Compared with a matched control group, fewer children with mothers being prescribed sodium valproate during pregnancy achieved the national minimum standard in CSI (-12.7% less than the control group), mathematics (-12.1%), language (-10.4%) and in science (-12.2%). Even fewer children with mothers being prescribed multiple AEDs during pregnancy achieved a national minimum standard: CSI (by -20.7% less than the control group), mathematics (-21.9%), language (-19.3%) and science (-19.4%). We did not observe any significant difference in children whose mothers were prescribed carbamazepine or were not taking an AED when compared with the control group. CONCLUSIONS: In utero exposure to AEDs in combination, or sodium valproate alone, is associated with a significant decrease in attainment in national educational tests for 7-year-old children compared with both a matched control group and the all-Wales national average. These results give further support to the cognitive and developmental effects of in utero exposure to sodium valproate as well as multiple AEDs, which should be balanced against the need for effective seizure control for women during pregnancy.

Validating epilepsy diagnoses in routinely collected data.

PURPOSE: Anonymised, routinely-collected healthcare data is increasingly being used for epilepsy research. We validated algorithms using general practitioner (GP) primary healthcare records to identify people with epilepsy from anonymised healthcare data within the Secure Anonymised Information Linkage (SAIL) databank in Wales, UK. METHOD: A reference population of 150 people with definite epilepsy and 150 people without epilepsy was ascertained from hospital records and linked to records contained within SAIL (containing GP records for 2.4 million people). We used three different algorithms, using combinations of GP epilepsy diagnosis and anti-epileptic drug (AED) prescription codes, to identify the reference population. RESULTS: Combining diagnosis and AED prescription codes had a sensitivity of 84% (95% ci 77-90) and specificity of 98% (95-100) in identifying people with epilepsy; diagnosis codes alone had a sensitivity of 86% (80-91) and a specificity of 97% (92-99); and AED prescription codes alone achieved a sensitivity of 92% (70-83) and a specificity of 73% (65-80). Using AED codes only was more accurate in children achieving a sensitivity of 88% (75-95) and specificity of 98% (88-100). CONCLUSION: GP epilepsy diagnosis and AED prescription codes can be confidently used to identify people with epilepsy using anonymised healthcare records in Wales, UK.

Epilepsy and deprivation, a data linkage study.

OBJECTIVE: To investigate whether the link between epilepsy and deprivation is due to factors associated with deprivation (social causation) or factors associated with a diagnosis of epilepsy (social drift). METHODS: We reviewed electronic primary health care records from 2004 to 2010, identifying prevalent and incident cases of epilepsy and recording linked deprivation scores. Logistic and Poisson regression models were used to calculate odds ratios and incidence rate ratios. The change in deprivation was measured 10 years after the initial diagnosis of epilepsy for a cohort of people. RESULTS: Between 2004 and 2010, 8.1 million patient-years of records were reviewed. Epilepsy prevalence and incidence were significantly associated with deprivation. Epilepsy prevalence ranged from 1.13% (1.07-1.19%) in the most deprived decile to 0.49% (0.45-0.53%) in the least deprived decile (adjusted odds ratio 0.92, p < 0.001). Epilepsy incidence ranged from 40/100,000 per year in the most deprived decile to 19/100,000 per year in the least deprived decile (adjusted incidence rate ratio 0.94, p < 0.001). There was no statistically significant change in deprivation index decile 10 years after a new diagnosis of epilepsy (mean difference -0.04, p = 0.85). SIGNIFICANCE: Epilepsy prevalence and incidence are strongly associated with deprivation; the deprivation score remains unchanged 10 years after a diagnosis of epilepsy. These findings suggest that increasing rates of epilepsy in deprived areas are more likely explained by social causation than by social drift. The nature of the association between incident epilepsy and social deprivation needs further exploration.

The importance of the experiences of initial diagnosis and treatment failure when switching antiepileptic drugs.

We sought to understand the issues that lead from the need to change antiepileptic drugs (AEDs) and how this may influence someone's feelings regarding swapping to another drug. We conducted semistructured interviews with 14 people with epilepsy, four months after changing from AED monotherapy. Interviews were digitally recorded, transcripts were coded independently, and thematic analysis was undertaken through group work. There were seven major themes: failure, the reason behind the failure, and the experience itself; expectations; previous experience; personality and life events; side effects; impact of diagnosis; and outcome. Clinical outcome and patients' ideas of outcome were often discordant. Each drug change arises from a position of failure that elicits strong feelings of loss of control and vulnerability in participants. Recognizing the need for counseling of targeted individuals undergoing AED change is key. Unresolved emotional issues surrounding biographical disruption following diagnosis were potent modifiers of the change process.

International consensus clinical practice statements for the treatment of neuropsychiatric conditions associated with epilepsy.

In order to address the major impact on quality of life and epilepsy management caused by associated neuropsychiatric conditions, an international consensus group of epileptologists met with the aim of developing clear evidence-based and practice-based statements to provide guidance on the management of these conditions. Using a Delphi process, this group prioritized a list of key management areas. These included: depression, anxiety, psychotic disorders, nonepileptic seizures, cognitive dysfunction, antiepileptic drug (AED)-related neurobehavioral disorders, suicidality, disorders in children and adolescents, disorders in children with intellectual disability, and epilepsy surgery. Clinical practice statements were developed for each area and consensus reached among members of the group. The assessment and management of these conditions needs to combine knowledge of psychiatric disorders, knowledge of the impact of epilepsy and its treatment on psychopathology, and an ability to deliver care within epilepsy services. The aim of these statements is to provide guidance on quality care for people with epilepsy that have a range of neuropsychiatric disorders.

Implications for families of advances in understanding the genetic basis of epilepsy.

Investigations into families with a large number of individuals with epilepsy have led to the discovery of epilepsy-causing (or epilepsy associated) gene mutations. These discoveries offer advantages and insights for the patient, family, healthcare professionals and biomedical scientists. Despite these benefits, there is little evidence about the impact of participation in genetic research for families with epilepsy. Here we report on the reflections of individuals who have participated in epilepsy genetic research through the Wales Epilepsy Research Network (WERN). Undergoing genetic investigation for inherited epilepsy has extensive emotive impact, both positive and negative, on individuals and families. Recognising these impacts is imperative to researchers working with families; having implications for study design, research consent and the provision of appropriate support.

Sampling and ethical issues in a multicenter study on health of people with intellectual disabilities.

OBJECTIVES: To study health inequalities in persons with intellectual disabilities, representative and unbiased samples are needed. Little is known about sample recruitment in this vulnerable group. This study aimed to determine differences in ethical procedures and sample recruitment in a multicenter research on health of persons with intellectual disabilities. Study questions regarded the practical sampling procedure, how ethical consent was obtained in each country, and which person gave informed consent for each study participant. STUDY DESIGN AND SETTING: Exploratory, as part of a multicenter study, in 14 European countries. After developing identical guidelines for all countries, partners collected data on health indicators by orally interviewing 1,269 persons with intellectual disabilities. Subsequently, semistructured interviews were carried out with partners and researchers. RESULTS: Identification of sufficient study participants proved feasible. Sampling frames differed from nationally estimated proportions of persons with intellectual disabilities living with families or in residential settings. Sometimes, people with intellectual disabilities were hard to trace. Consent procedures and legal representation varied broadly. Nonresponse data proved unavailable. CONCLUSION: To build representative unbiased samples of vulnerable groups with limited academic capacities, international consensus on respectful consent procedures and tailored patient information is necessary.

Examining the prevalence of epilepsy and delivery of epilepsy care in Ireland.

PURPOSE: To examine the prevalence of epilepsy and delivery of epilepsy care in Ireland. METHODS: A multiple case ascertainment methodology was employed examining five nationwide data sources. Data were obtained via (1) a nationwide population-based health survey, (2) secondary data analysis of all reimbursements for prescriptions of antiepilepsy medication nationwide from 2002 to 2005, (3) a nationwide survey of primary care community-based physicians, (4) a nationwide survey of specialist care hospital-based neurologists, and (5) secondary data analysis of inpatient discharges from acute hospitals nationwide for persons with a diagnosis of epilepsy between 2000 and 2005. RESULTS: The findings indicate that nationwide approximately (1) 10 per 1,000 persons (n = 31,000) 18 years and older have self-reported lifetime prevalence of epilepsy, (2) 8.3-9 per 1,000 (approximately 33,000-36,000) persons 5 years and older are being treated for epilepsy using antiepilepsy medication, (3) the typical general practitioner provides care to an average of 13 patients with active epilepsy and epilepsy in remission with treatment, (4) specialist care is provided on a weekly basis to approximately 442 persons with active epilepsy and epilepsy in remission with treatment, and (5) approximately 67 discharges for persons with a primary or secondary diagnosis of epilepsy are reported from acute hospitals weekly. DISCUSSION: This study is distinguished as the first nationwide prevalence study of epilepsy in Europe and provides previously unavailable data on this population in Ireland. Moreover, this study illustrates the merits of secondary data analysis of existing sources when access to clinical sources for the purpose of identifying epidemiologic samples is limited.

Psychopathology in children and adolescents with epilepsy: an investigation of predictive variables.

This epidemiological study was aimed at determining the prevalence of behavioral and emotional problems in a UK community-based population of children and adolescents with epilepsy aged 4-17 using a postal questionnaire survey. The intent was to identify, through this survey, those epilepsy-related and demographic factors predictive of the presence of psychopathology and diminished health-related quality of life, and to distinguish whether such factors differ for differing types of psychopathology. Outcomes were measured using the Strengths and Difficulties Questionnaire (SDQ), Moods and Feelings Questionnaire (MFQ), Impact of Paediatric Epilepsy Scale (IPES), and Quality of Life in Epilepsy Inventory for Adolescents (QOLIE-AD-48). Information was obtained from main carers for 56 children (25 males and 31 females, mean age=12 years, SD=3 years 9 months, range=5-17). Parent report identified that 23 (47.9%, 95% confidence interval [CI] 34.5-61.7%) children met psychiatric caseness criteria and 32 (61.5%, 95% CI 48-73.5%) had chronic distress and social impairment. Regression analyses identified seizure severity as a risk factor for emotional problems and depression (odds ratio [OR]=1.09, P<0.05), whereas cognitive impairment was associated with behavioral problems, specifically conduct problems (OR=14.0, P<0.05), hyperactivity/inattention (OR=9.4, P<0.01), and peer problems (OR=28.5, P<0.01). Cognitive impairment and high seizure frequency were significantly related to increased IPES scores (R(2)=0.33, P<0.001) and diminished QOLIE-AD-48 scores (R(2)=0.39, P<0.01). In conclusion, children with epilepsy experience considerable psychopathology and reduced quality of life. Epilepsy-related factors appear more closely associated with emotional well-being, and cognitive factors with behavior problems.

A community study of the presence of anxiety disorder in people with epilepsy.

Anxiety represents a major problem for people with epilepsy, and it is important to understand why it arises and how to reduce its potential debilitating and adverse effects. The aim of this study was to determine the prevalence of anxiety in a community-identified sample of people with epilepsy and to identify which demographic and clinical factors are most closely associated with anxiety and which factors predict the presence of anxiety among people with epilepsy. Adults with epilepsy in the community (n=515) were identified through primary care records and sent validated questionnaires, which included the Hospital Anxiety and Depression Scale (HADS). The mailout also included items on demographic and clinical variables. The prevalence of anxiety (HADS score >11) in this sample was 20.5% (95% CI: 16.9-24.1%) and was associated with a current history of depression, perceived side effects of antiepileptic medication, lower educational attainment, chronic ill health, female gender, and unemployment. It was not associated with the duration of epilepsy. The findings from this study suggest that anxiety disorders in a community population with epilepsy are most strongly predicted by factors independent of epilepsy-related variables, with the exception of patient-reported side effects. It is important to be aware of these factors when evaluating an individual with epilepsy.

Behavioral assessment in mentally retarded and developmentally disabled patients with epilepsy.

Behavioral disturbance is common in many individuals with mental retardation. Its presence in those with coexisting epilepsy provides a further challenge to the individual, caretakers, and family members. In particular, concerns that antiepileptic medication is causing such behavioral disturbances are often raised. For the clinician, assessment is crucial to minimize morbidity by appropriately identifying the cause of the behavioral disturbance. The assessment should aim to delineate whether the disturbance is due to a direct effect of epilepsy, its treatment, social impact, or factors unrelated to the epilepsy. However, before being able to make this differentiation, the clinician needs to understand the behavior itself. Assessing behavior in people with mental retardation requires a multifactorial approach. These factors include an objective and reliable description of the behavior and an assessment of its function. Detailed description requires a functional analysis of behavior. Such analysis may indicate the behavior is associated with epilepsy treatment or represents a seizure disorder in itself. In the majority of cases, though, it is likely that any disturbance seen is independent of epilepsy or its management.