Professor Helen Cross and Drs Colin Dunkley and Margaret Jackson explain how an integrated approach to epilepsy care could avoid misdiagnosis and improve outcomes for patients
- A suspected seizure has significant implications for anyone, regardless of age
- Diagnosis of epilepsy depends on an accurate clinical history and specialist assessment
- Early specialist assessment is the key to accurate diagnosis and good management
- Important outcomes in epilepsy include gaining control of seizures, improving developmental and learning outcomes and quality of life
- Magnetic resonance imaging is the recommended mode of imaging for detection of intracranial abnormalities associated with epilepsy
- A written care plan promotes better self-management, sharing of information, and improved links between patients and health professionals
- Early treatment of prolonged seizures, in accordance with an emergency individual care plan, can prevent status epilepticus and hospital admission
- Epilepsy specialist nurses reduce avoidable admissions and are essential for the delivery of a comprehensive care plan
- At annual review, there should be access back to secondary care for adults with epilepsy to manage problems outside the expertise of most general practitioners
- Adults and children with persistent seizures despite medical treatment require specialist review in specialist epilepsy (tertiary) centres
- A period of joint adult and paediatric working is needed to avoid gaps in care for patients between childhood and adulthood.
Epilepsy is a common neurological disorder in which individuals are prone to recurrent epileptic seizures. More accurately, it is a group of disorders with many causes and should be referred to as ‘the epilepsies’. Accurate estimates of incidence and prevalence are difficult to obtain because identification of people who may have epilepsy is challenging. It has been estimated that between 362,000 and 415,000 people in England are affected by epilepsy.1 In addition, there may be up to another 124,500 people who have been diagnosed with epilepsy, but in whom the diagnosis is incorrect.1
Two-thirds of people with active epilepsy have their condition satisfactorily controlled with anti-epileptic drugs (AEDs).1 Other approaches may include surgery, or specific dietary therapy (in children). Optimal management improves health outcomes and can also help to minimise other, often detrimental, impacts on social, educational, and employment activity.
The epilepsies are an area of health where there are significant opportunities for services to make a difference. Important outcomes in epilepsy include gaining control of seizures, lessening their impact and adverse effects, enhancing developmental and learning outcomes, and improving quality of life. There is potential to improve quality of life, education, and employment opportunities, as well as minimising the risk of death.
Variation in standards of care
Several reports commissioned since 2000 have reported epilepsy management to be suboptimal in the UK, with deaths that could potentially have been avoided.2 Furthermore, the annual estimated cost of established epilepsies is £2 billion (including direct and indirect costs).1
NICE guidance on the diagnosis and management of the epilepsies was first published in 2004, and updated (with regard to pharmacological treatment) in 2012 as NICE Clinical Guideline CG137 on epilepsy (see www.nice.org.uk/cg137). 1 Although this guidance has acted as a spur to improve services, it is recognised that many people with epilepsy (or suspected epilepsy) do not receive care in accordance with this guidance.
The many types of epilepsies, and the number of different professionals and services required in their management, mean that treatment and provision needs can differ considerably from one individual to the next. Children and adults with epilepsy are often managed in non-designated clinics and, as a population, are often less visible and under-resourced. Although the individual professional, carer, and patient may be subjectively aware of how good care is, those with a broader provider or commissioning role can have significant difficulty in objectively determining quality of care. The Royal College of Paediatrics and Child Health (RCPCH) UK childhood audit epilepsy12 concluded that:3
- there remain significant gaps between national recommendations and care received
- there is variation in quality between providers
- individual services have strengths and weaknesses
- a formal system of capturing, measuring, and communicating quality can be used to generate improvement plans
- young people and parents have a role in reporting the quality of care received.
The NICE quality standards for the epilepsies in adults, and in children and young people
The NICE quality standards for the epilepsies in adults (QS26; see www.nice.org.uk/guidance/qs26),4 and children and young people (QS27; see www.nice.org.uk/guidance/qs27),5 set out measurable parameters by which the quality of services can be judged (see Tables 1 and 2). They should be helpful both to those commissioning services and to care providers, in improving local services for adults, children, and young people. The two standards were developed by a Topic Expert Group and were open for consultation before publication.
Both quality standards should contribute to improved outcomes along the patient pathway for individuals with epilepsy and their carers, by promoting:4,5
- rapid, accurate diagnosis from a specialist
- appropriate investigations
- patients’ awareness of plans for their management
- timely access to tertiary care or re-referral back to a specialist, when needed.
|1||Adults presenting with a suspected seizure are seen by a specialist in the diagnosis and management of the epilepsies within 2 weeks of presentation.|
|2||Adults having initial investigations for epilepsy undergo the tests within 4 weeks of them being requested.|
|3||Adults who meet the criteria for neuroimaging for epilepsy have magnetic resonance imaging.|
|4||Adults with epilepsy have an agreed and comprehensive written epilepsy care plan.|
|5||Adults with epilepsy are seen by an epilepsy specialist nurse who they can contact between scheduled reviews.|
|6||Adults with a history of prolonged or repeated seizures have an agreed written emergency care plan.|
|7||Adults who meet the criteria for referral to a tertiary care specialist are seen within 4 weeks of referral.|
|8||Adults with epilepsy who have medical or lifestyle issues that need review are referred to specialist epilepsy services.|
|9||Young people with epilepsy have an agreed transition period during which their continuing epilepsy care is reviewed jointly by paediatric and adult services.|
|NICE website. The epilepsies in adults. Reproduced with kind permission. Available at: www.nice.org.uk/guidance/qs26|
|1||Children and young people presenting with a suspected seizure are seen by a specialist in the diagnosis and management of the epilepsies within 2 weeks of presentation.|
|2||Children and young people having initial investigations for epilepsy undergo the tests within 4 weeks of them being requested.|
|3||Children and young people who meet the criteria for neuroimaging for epilepsy have magnetic resonance imaging.|
|4||Children and young people with epilepsy have an agreed and comprehensive written epilepsy care plan.|
|5||Children and young people with epilepsy are seen by an epilepsy specialist nurse who they can contact between scheduled reviews.|
|6||Children and young people with a history of prolonged or repeated seizures have an agreed written emergency care plan.|
|7||Children and young people who meet the criteria for referral to a tertiary care specialist are seen within 4 weeks of referral.|
|8||Children and young people with epilepsy have a structured review with a paediatric epilepsy specialist at least annually.|
|9||Young people with epilepsy have an agreed transition period during which their continuing epilepsy care is reviewed jointly by paediatric and adult services.|
|NICE website. The epilepsies in children and young people. Reproduced with kind permission. Available at: www.nice.org.uk/guidance/qs27|
Diagnosis and investigation—statements 1–3
A suspected seizure has significant implications for any individual, regardless of age. An adult or young person suspected of having a seizure is not allowed to drive, causing some individuals severe social and financial difficulties, as well as a loss of confidence and fear of further seizures. A seizure may be a sign of serious brain disease (e.g. tumours, venous thrombosis). Other conditions (e.g. convulsive and cardiac syncope, functional non-epileptic attacks) may mimic seizures and be difficult to differentiate from them. Diagnosis of epilepsy is dependent on an accurate clinical history: 20%–30% of adults and children diagnosed with epilepsy by non-specialists do not have epilepsy.6,7
Prolonged delay in diagnosis causes avoidable distress, so early assessment by a specialist is the key to good management. A 2011 audit, the National Audit of Seizures in Hospitals (NASH), showed that management of people presenting with seizures to accident and emergency services, or acute medical admissions units, was inadequate in:8
- onward referral to specialist services
- information given to patients.
Quality statement 1, that patients with suspected first seizures are seen within 2 weeks by a specialist in epilepsy, should improve this situation.4,5
Services will need to have clear pathways for referral if children are to be reviewed by a paediatrician with appropriate competencies within 2 weeks of presentation of a suspected seizure. The Epilepsy12 audit showed that 21% of children with epilepsy had no evidence of this type of input within 12 months of presentation.3 NICE QS27 is therefore particularly challenging in its emphasis on early specialist input from initial presentation.5 Epilepsy12 also demonstrated that 64% of children received magnetic resonance imaging (MRI), and 60% tertiary referral by 12 months, when indicated.3 With increasing input from healthcare professionals with expertise, more children should receive appropriate investigations and referral to tertiary care. Radiology and neurophysiology services will need adequate capacity and reporting systems in place in order to achieve the maximum 4-week waiting times required by quality statement 2.
Investigations for epilepsy have limited sensitivity but can help with prognosis and classification of the condition. The decision to start treatment with AEDs, and length of suspension of a driver’s licence after a single seizure, may be influenced by results of an MRI and electroencephalogram (EEG) as the presence of abnormalities raises the risk of further seizures. Magnetic resonance imaging is more sensitive than computed tomography (CT) scanning in detecting primary brain tumours and other intracranial abnormalities associated with epilepsy, and is therefore the preferred mode of imaging recommended in both QS26 and QS27.4,5
Initial management and care planning—statements 4–6
Many people with epilepsy are unaware what type of epilepsy they have, and repeated surveys have shown that people with epilepsy do not believe they have been given sufficient information about their condition. Epilepsy is associated with 1000 deaths a year due to accidents and sudden unexplained death in epilepsy (SUDEP),2 and it is one of the main causes of unscheduled care. Many women are unaware of interactions between AEDs and oral contraceptives and are not informed of the risks to their unborn child of taking AEDs in pregnancy.9 To promote better self-management, sharing of information, and improved links between the patient and their healthcare professionals, a written care plan is recommended in both NICE QS26 and QS27 (quality statement 4).4,5 The written emergency plan for treatment of prolonged or repeated seizures, in quality statement 6, is of particular importance. Early treatment of prolonged seizures (usually with buccal midazolam), given according to an individual care plan, can prevent status epilepticus, avoiding hospital admission.1
Epilepsy specialist nurses
Epilepsy specialist nurses (ESNs) are valued by people with epilepsy and their carers for their support, and as sources of information and advice. However, many areas in England, usually those without specialist epilepsy services, do not have ESNs. Although data to demonstrate the cost effectiveness of nurse specialists are lacking, there is no doubt that such healthcare professionals enhance the quality of care and improve the experience of people with epilepsy. The Epilepsy12 national audit showed that 47% of paediatric services had no ESNs,3 and 54% of children with epilepsy had no evidence of input by an ESN within 12 months of diagnosis. The Royal College of Nursing recommends at least one ESN per 250 children with epilepsy.10,11 Providers and commissioners need to increase ESN numbers and access. It is likely that appropriate ESN access reduces avoidable admissions.12 The quality statements outlining the requirement for comprehensive care plans (quality statement 4) will also be very difficult to deliver for adults and children without sufficient ESN provision.3
Since April 2013, the best practice tariff for paediatric epilepsy has allowed providers to attract additional income for planned activity that includes contact with an ESN and a paediatrician with expertise.13 Adequate ESN input is essential to support the provision of comprehensive care plans for children with epilepsies. Manageable caseloads are needed to allow ESNs to support improved plans of care that extend into educational, respite, and other care settings.
Later management and review—statements 7 and 8
Nearly 50% of people with epilepsy will respond to their first AED and will be discharged back to primary care.14
NICE CG137 (see www.nice.org.uk/cg137) recommends that these people have an annual review to identify issues such as stopping medication, planning a pregnancy, or side-effects from medication.1 The quality standards recommend that there should be access back into secondary care for management of these issues because they are outside the expertise of most GPs.4,5
Conversely, 30% of adults and children with epilepsy have seizures that persist despite medical treatment.14 This may be refractory epilepsy, or the consequence of inaccurate diagnosis, or inappropriate treatment. These individuals require specialist review and diagnostic tests that are only available in specialist epilepsy centres. It is envisaged that there should be local networks through which non-specialist secondary care can refer people to tertiary epilepsy centres for diagnostic tests, such as videotelemetry. Many tertiary centres will provide assessment for, and undertake, surgery for epilepsy.
Transitional services—quality statement 9
There are many models of transitional care from child to adulthood currently in the UK but there remain gaps in provision.15 NICE QS26 and QS27 recognise the need for transitional services that include a period where there is joint working between adult and paediatric care. This will require paediatric services to integrate with appropriately resourced adult services. New commissioning arrangements for this type of joint working need to be considered.
NICE QS26 and QS27 for the epilepsies in adults, and in children and young people, envisage that services should be commissioned from and coordinated across all relevant agencies, encompassing the whole pathway of epilepsy care.4,5 An integrated approach to provision of services is fundamental to the delivery of high-quality care. The quality standards should be:
- delivered by multidisciplinary teams through a local epilepsy clinical network
- interpreted in the context of national and local guidelines on training and competencies.
Since the NICE guideline on epilepsy was published in 2004, there have been many initiatives to support its implementation. The British Paediatric Neurology Association has successfully established paediatric epilepsy training courses16 aligned to NICE recommendations, and the UK chapter of the International League Against Epilepsy runs biannual training weekends for trainees. Paediatric epilepsy courses deliver pragmatic education tailored to different professionals within an epilepsy care pathway. They support the development of required professional competencies for delivering care aligned with NICE QS26 and QS27. Many regions within the UK have evolving clinical networks supporting collaborative multi-professional epilepsy care. Quality standards can be used by networks to share good practice and organise regional interventions. Regional clinical epilepsy networks need recognition, support, and funding in order to fulfil their role.
Funding for specialist epilepsy care in adults is variable, with some commissioners and providers unwilling to recognise the additional costs that such services demand. Now with NICE CG137, as well as NICE QS26 and QS27, comes the challenge of genuine implementation. The potential for better clinical outcomes, as well as for reducing the costs of misdiagnosis and avoidable admissions, should act as incentives for providers and commissioners.
Primary care has at the very least a responsibility to be aware of individuals with epilepsy within their practice. Care of children lies exclusively within secondary care, although primary care needs to be aware of the care pathway, particularly what should happen with a first event. Adults will seek advice from primary care to a variable degree, and even if well controlled on medication may require more specialised advice at certain times; they will definitively require regular review. The quality standards are a set of tools by which standards of care can be optimised in individuals with epilepsy of all ages.
- Proportion of adults, or children and young people with epilepsy:
- presenting with a suspected seizure who are seen within 2 weeks by a specialist in the diagnosis and management of the epilepsies
- having initial investigations for epilepsy who undergo the tests within 4 weeks of them being requested
- who meet the criteria for neuroimaging for epilepsy, who have an MRI scan
- who have seen a named epilepsy specialist nurse at diagnosis
- Proportion of adults with epilepsy:
- with a history of prolonged or repeated seizures who have an agreed written emergency care plan.
MRI=magnetic resonance imaging
- CCGs should look to benchmark their current commissioned service provision against the NICE quality standards for the epilepsies in adults (QS26), and children and young people (QS27)
- CCG epilepsy leads working alongside local providers should map local care pathways to meet these standards, and look to commission new services where gaps exist
- These care pathways should be shared with local ambulance services, primary care, minor injury units, and walk-in centres to ensure patients who present with suspected symptoms of epilepsy are assessed and referred appropriately
- CCGs should look to commission epilepsy specialist nurses but should consider employment models that avoid expensive tariff charges each time healthcare professionals see or contact a patient, or else agree an annual budget with a local provider
- CCGs could ask practices to use annual epilepsy reviews to check that patients with active epilepsy have up-to-date care plans, and adequate medication for prolonged seizures if appropriate
- CCGs should monitor claims on the best-practice tariff for paediatric epilepsy to ensure that services meet the quality specification for this enhanced payment
- Tariff costs for outpatients:a
- paediatric epilepsy = £215 (new), £125 (follow up)
- neurology (non-mandatory) = £222 (new), £128 (follow up)
- best-practice tariff paediatric epilepsy = £173 (follow up)
CCG=clinical commissioning group
- NICE. The epilepsies: the diagnosis and management of the epilepsies in adults and children in primary and secondary care. Clinical Guideline 137. Available at: www.nice.org.uk/cg137
- Hanna N, Black M, Sander J et al. The national sentinel clinical audit of epilepsy-related death: epilepsy—death in the shadows. London: The Stationery Office, 2002. Available at: www.archive2.official-documents.co.uk/document/reps/nscaerd/nscaerd.pdf
- Royal College of Paediatrics and Child Health. United Kingdom collaborative clinical audit of health care for children and young people with suspected epileptic seizures, Epilepsy12 National Report. London: RCPCH, 2012. Available at: www.rcpch.ac.uk/system/files/protected/page/Epilepsy_web.pdf
- NICE website. The epilepsies in adults. Quality standard 26. www.nice.org.uk/qs26 (accessed 28 May 2013).
- NICE website. The epilepsies in children and young people. www.nice.org.uk/qs27 (accessed 28 May 2013).
- Smith D, Defalla B, Chadwick D. The misdiagnosis of epilepsy and the management of refractory epilepsy in a specialist clinic. QJM 1999; 92 (1): 15–23.
- Uldall P, Alving J, Hansen L et al. The misdiagnosis of epilepsy in children admitted to a tertiary epilepsy centre with paroxysmal events. Arch Dis Child 2006; 91 (3): 219–221.
- Marson T. UK epilepsy audit shows major deficiencies in care: who should respond and how? Practical Neurol 2013; 13: 2–3.
- Fairgrieve S, Jackson M, Jonas P et al. Population based prospective study of the care of women with epilepsy in pregnancy. BMJ 2000; 321 (7262): 674–675.
- Epilepsy Action. Best care: the value of epilepsy specialist nurses. A report on a study by researchers at Liverpool John Moores University on behalf of Epilepsy Action, June 2010. Leeds: Epilepsy Action, 2010. Available at: www.esna-online.org.uk/documents/FV%20Best%20care%20Jul2010.pdf
- Royal College of Nursing. Competencies: a competency framework and guidance for developing paediatric epilepsy nurse specialist services. London: RCN, 2005. Available at: www.rcn.org.uk/__data/assets/pdf_file/0004/78673/002792.pdf
- Johnson K, McGowan T, Dunkley C. A review of an epilepsy specialist’s clinical activity and impact on paediatric admissions. Sherwood Forest Hospitals NHS Foundation Trust, 2012. Available at: www.cewt.org.uk/CEWT/Research_files/ILAE%20poster%202012.pdf
- Department of Health. Payment by results guidance for 2013–14. DH: London, 2013. Available at: www.gov.uk/government/uploads/system/uploads/attachment_data/file/141396/Mental-Health-PbR-Guidance-for-2013-14.pdf
- Kwan P, Brodie M. Early identification of refractory epilepsy. N Engl J Med 2000; 342 (5): 314–319.
- Iyer A, Appleton R. Transitional services for adolescents with epilepsy in the UK: a survey. Seizure 2013. [Epub ahead of print] G