Because primary-care providers manage most patients with epilepsy in the USA and Canada, results of a similar audit in North America would probably only differ in degree. The UK audit should be a wake-up call to the medical profession and result in a targeted campaign for people with epilepsy. While there remains a critical need for more effective treatments ... the UK audit emphasises that many patients with epilepsy perhaps most could benefit substantially if only current knowledge and available therapeutic options were applied effectively.Timothy Pedley, W. Allen Hauser, Lancet 2002
The National Sentinel Clinical Audit of Epilepsy-Related Deaths (Hanna 2002) was led by Epilepsy Bereaved in partnership with medical professional organisations in the UK and with the support of the Joint Epilepsy Council.
This is an emotive topic and groundbreaking study. Firstly, it tackles an important problem that has lain in the shadows far too long. Secondly, it delivers a loud wake up message that demands immediate and vigorous action. Thirdly, it demonstrates a seismic shift in the way we do medical studies the bereaved families have real ownership of the work ... The main role of professionals has been to ensure sound methods and robust analysis. This partnership has allowed key questions to be addressed that have previously been thought too sensitive and remained hidden from view. Professor David Fish
The audit looked at the medical care and post mortem investigation of patients who had died and in whom epilepsy was considered the main cause of death between September 1999 and August 2000. During this time 812 deaths were identified including 81 children under 18 years. A total of 595 deaths were audited for the investigation of death and medical records of 225 were made available and audited to review pre-mortem care. A key aim of the audit was to establish whether deficiencies in the standard of clinical management or overall package of healthcare could have contributed to deaths. A key finding was that 42% of deaths were potentially avoidable.
A majority of adults (54%, 84/158) had inadequate care, which led to the conclusion that 39% of adult deaths were considered potentially or probably avoidable. The main deficiencies identified were (in descending order of frequency): inadequate access to specialist care, inadequate drug management, lack of appropriate investigations, no evidence of a package of care, inadequate recording of histories, adults with learning difficulties lost in transfer from child to adult services, and one or more major clinical management errors.
A majority of children (77%, 17/22) had inadequate care, which led to the conclusion that 59% of deaths in children were considered potentially or probably avoidable. The main deficiencies identified were (in descending order of frequency): inadequate drug management, inadequate access to specialist care, and inadequate investigations. There was concern that documentation was poor in both primary and secondary care; only 1% of hospital records for adults showed that SUDEP had been discussed.
Perhaps the most dismaying disconnect is between doctor and patient the failure by doctors to discuss with the patient the risks of epilepsy and how to minimise them ... or to point the patient in the direction of support from voluntary organisations ... I am sorry to say that this part of the report spoke to me of a basic lack of willingness on the part of doctors to involve patients in decisions about their own care and a ducking out of confronting unpleasant truths ... Lord Howe in Parliament, May 2002
The audit also examined the management of processes following death. 1023 cases were subject to post mortem and the audit reviewed two-fifths of these cases and a tenth of cases which did not go to post mortem. Less than 1 in 8 deaths were fully and properly investigated. In almost half the certificate was incomplete or inappropriate in some way. Communication by the medical profession with bereaved relatives was inadequate. Only 7% of GP notes and 10% of hospital notes indicated contact with the family.
In 2004 as a result of the audit, SUDEP is recognised as a syndrome by national and local policy makers in the UK. In a recent survey of neurologists 66% reported that there had been a definite or probable improvement in managing the risk factors for SUDEP. The audit has a high citation rate with SUDEP highlighted in a wide range of health professional journals and in new NICE epilepsy guidelines in England and Wales (2004). In England there has been an increase in national initiatives on epilepsy including the first epilepsy action plan published by the government in England in response to the audit and in Wales a national implementation group working on an action plan. In Scotland the audit alongside the Findlay Inquiry (Taylor 2002) were important factors in all but two Health Boards creating a new model of integrated epilepsy services.
New guidelines have also been produced on the investigation of epilepsy deaths by the Royal College of Pathologists in 2005 as part of the government action plan. These guidelines and all documents mentioned in this article can be accessed at www.sudep.org/national_report.asp
Jane HannaDirector, Epilepsy Bereaved, UK
We all recognise that most individuals would probably rather not have a post mortem examination performed on themselves, a relative or friend. It can however, be a valuable source of information to society and the medical profession but most importantly to those close to the deceased.
Historically, many sudden deaths occurring in people with epilepsy were attributed by the certifying doctor as being a consequence of choking on vomit or other asphyxial processes. It is now recognised that this is not necessarily the case. The term SUDEP recognises that these deaths can fit a pattern but the exact cause or mechanism of the death is uncertain.
There are no diagnostic features of a SUDEP death; the diagnosis requires exclusion of any other potential cause of death, in association with a typical history and circumstances of the death. Thus a full post mortem examination including toxicology and histology is essential to identify any other natural disease, intoxication or trauma. Deaths could wrongly be attributed to epilepsy when there is another natural disease e.g. cerebral haemorrhage, or drug overdose. Studies have also shown that a neuropathological examination of the brain can provide additional information.
It is important that these deaths are properly investigated to establish the true incidence of SUDEP deaths and to document the findings. The recent National Sentinel Audit of Epilepsy-Related Deaths (Hanna 2002) in the UK sadly identified that there were still many deficiencies in the quality of the examination and in the manner in which the deaths were certified.
The majority of the deaths will undergo a medico-legal post mortem examination, on the instructions of the relevant authority, to determine the cause of death. This should include the retention of small pieces of major organs for processing into histological blocks and slides. It may also involve retention of the brain but pathologists have adapted practice such that a neuropathology opinion can often be obtained either by sampling at the time of the examination or after short fixation and return of the brain before the funeral. In many countries the recent controversy and concerns regarding organ retention have led to changes in the law. Relatives will now be informed of any organ retention. In the UK any research on organs or tissues will now be illegal without the explicit, fully informed consent of the next of kin.
The post mortem examination may provide answers that can help in the grieving process. I am aware from speaking to relatives and support groups that feelings of guilt are not uncommon. For example, if a death is certified as due to inhalation of vomit or asphyxia this may imply that it was preventable. The medical profession must recognise that such a conclusion requires incontrovertible evidence. The mere presence of food in the airways is meaningless (it can occur after death); a histologically proven vital response is required. Similarly petechial haemorrhages within hypostasis in someone found face down is of no diagnostic value. Recognition that these deaths are sudden and unexpected can alone provide some comfort the carer could not have altered the outcome.
A full post mortem examination will identify any other natural disease or pathology this may answer some questions and raise others. In some instances a cause for the epilepsy may be identifiable e.g. traumatic injury or a genetic disease, the latter uncommon but of considerable importance to a family. The pathologist may find another potential cause for the death e.g. ischaemic heart disease. The information available regarding the circumstances of the death may then allow the pathologist to assess whether this, rather than epilepsy, is the likely cause of death.
Epilepsy has long been recognised and invoked as a significant ingredient in the mechanism of sudden unexpected death, particularly in the setting of status seizures, trauma, drownings and aspiration of gastric content. However, a wider appreciation that epilepsy per se may be a major cause of rather than contributory factor to death, is a relatively recent concept which may not be widely comprehended or accepted by the community at large, epileptic patients and their physicians, and perhaps some pathologists. Since these cases present as sudden, unexpected and often unexplained death, they will fall under the jurisdiction of the coroner, and in most circumstances require specialist forensic pathological investigation.
Like that other acronym SIDS (sudden infant death syndrome), the term SUDEP hints at a relatively stereotypical series of circumstances allied to an unascertained cause of death; but unlike SIDS (or perhaps the more controversial SADS (sudden adult death syndrome)), the field of potential causative mechanisms appears narrower and is arguably better delineated, holding the promise of effective intervention strategies.
Much research over the past few years has pointed to complex cerebral and cardiorespiratory factors, which individually or in concert may result in death during or shortly after a seizure. If the task of clinicians is to predict and intervene, the role of the forensic pathologist and coroner might best be seen as recognition and comprehensive investigation so that the true incidence (at various points in time) is documented, and effective multidisciplinary remedies implemented. A vital first step along this path is uniformity of approach, but many factors need to be addressed before this pathological nirvana is attained, some of which may be subject to considerable regional and situational constraints:
A full appreciation and documentation of the circumstances surrounding the death including a comprehensive medical and medication history, and details of death scene examination by a knowledgeable investigator (including position and attitude of body, details of scene disturbance, witness statements etc). Also required are further enquiries when the Police Report is inadequate, ready and timely access to medical records when necessary, consultation with clinicians if ECG or EEG available, and consideration and investigation of possible epilepsy in the unascertained autopsy.
A full and thorough autopsy examination including recording stigmata suggestive of epilepsy (e.g. tuberous sclerosis, gum hypertrophy, unusual scalp or facial scars), asphxyial signs, and factors that may indicate a recent seizure (oral injuries, urinary or faecal incontinence, collapse injuries etc).
Consideration given to retention of the brain for formal neuropathological examination and alternate strategies for enhancing brain examination when permission for retention is denied.
Full toxicological analysis, (including antiepileptic medications and vitreous biochemistry).
Uniformity of approach in formulation of cause of death (rationalisation of the terms Epilepsy, SUDEP, Unascertained, Sudden Adult Death Syndrome, Suggestive of (or Probable) Epilepsy, Seizure, Status Epilepticus).
Adopting a rational and consistent approach to certification when significant co-pathology exists.
Implementing mechanisms for accurate retrieval of data and dissemination of information to families, physicians, and interested parties.
Developing lines of communication with epilepsy bereavement services and epileptologists.
In his 2001 report, the Chief Medical Officer of England recommended that an action plan be developed by the Government to improve epilepsy services and address the findings of the National Sentinel Clinical Audit of Epilepsy-Related Deaths (Hanna 2002). In 2003 the Government published the first action plan on epilepsy and this included the proposed publication of a clinical guideline for the diagnosis, management and treatment of epilepsy.In England and Wales the body responsible for the development of evidence-based clinical guidelines is the National Institute for Health and Clinical Excellence (NICE). The guidelines were to address how care should be improved for children and adults with epilepsy, including areas in primary and secondary care where improvements in epilepsy services could reduce the risks of seizure-related deaths.
A development group was convened consisting of experts nominated by a wide range of organisations. The group included nine medical experts nominated by the Association of British Neurologists, Society of British Neurological Surgeons, Royal College of Physicians, Royal College of General Practitioners, Royal College of Paediatrics and Child Health, Royal College of Nursing, Neonatal and Paediatric and Child Health, and four patient representatives nominated by the Joint Epilepsy Council, Epilepsy Action, National Society for Epilepsy, and Epilepsy Bereaved. The group identified key clinical questions and reviewed the evidence identified by a methodology team based at the Clinical Governance Research and Development Unit at the Department of Health Sciences, University of Leicester. This review led to recommendations for clinical practice. Other experts were involved as co-opted members and there was widespread consultation with all stakeholders at each draft of the guidelines.
The clinical guidelines offer recommendations on diagnosis, classification, investigations, treatment, information, and support. Key priorities include timely assessment by a specialist within two weeks of a suspected first seizure, a consulting style that enables a partnership between patients and clinicians about management decisions, the need for a comprehensive care plan, individualised medication, and a regular structured review. All of these recommendations reflect the findings of the audit. The importance of the need for information to reduce the risk of SUDEP is a core theme. An example of the importance of information provision about SUDEP is found in the women and pregnancy section where the guideline recommends that women considering stopping their medication should be specifically warned about SUDEP.
The guidelines also emphasise why preventing seizures is important to reduce the risk of epilepsy-related death. The Epilepsy Be Aware card has recently been published by the government. This card has essential information on epilepsy, helpline details, and a leaflet setting out the importance of annual review. Both the card and the leaflet include information about SUDEP. Research showed that this was valued by patients.
Tailored information on the individuals risk of SUDEP should be part of the counselling checklist for people with epilepsy and their families and/or carers taking account of the small but definite risk of SUDEP. The guidelines recognise that there may be factors that increase the risk of SUDEP. These include poor seizure control, having convulsive or nocturnal seizures, having a learning disability, being a young adult male, not taking treatment as prescribed or having abrupt or frequent changes to medication.
The guidelines also recognise particular issues relating to young people and the learning disabled. Both groups are at increased risk of SUDEP. Lifestyle issues and the importance of taking medication are particularly important issues to discuss with young people. Health professionals should be aware of the higher risks of mortality for people with learning disabilities and epilepsy and discuss these with individuals, their families and/or carers. SUDEP is also an essential part of a risk assessment of people with learning disability.
The time at which information about SUDEP should be given is after confirmation of the diagnosis, and tailored to the individual. The discussion is best made during a consultation where there is a partnership between clinician and patient. It is important that essential information on how to recognise a seizure, first aid measures, and the importance of reporting further attacks, should be given after the first suspected seizure.
A series of guidelines recommending advising health professionals to contact families and/or carers to offer their condolences, invite them to discuss the death, and offer referral to bereavement counselling and a SUDEP support group after a bereavement, was the result of research carried out with bereaved families.
For further information there are quick reference guides for health care professionals at www.nice.org.uk
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