National bundle of care for children and young people with epilepsy: annex 1

Standards of care for children and young people with epilepsy.

Addressing variation in care between epilepsy services

Rationale 

Since the establishment of Epilepsy12 in 2009, there have been improvements in aspects of care such as input from epilepsy specialist nurses and evidence of comprehensive care planning. Despite this, variation in key aspects of care provided persist between epilepsy services.

Paediatric epilepsy specialist nurses (ESNs) provide specialist care and management for Children and Young People (CYP) with epilepsy. NG217, 11.1.1 states that all CYP with epilepsy should have access to an epilepsy specialist nurse. There is clear evidence to show that access to ESNs results in:

  • A decrease in emergency paediatric epilepsy admissions
  • An improvement of knowledge, self-care and quality of life for CYP
  • Supports continuity of care between settings, and
  • Cost savings, both long-term and within the first year of care.

Despite this, access remains variable between Trusts as well as at Integrated Care Board (ICB) level.

Epilepsy12 Cohort 4 data shows variation between Trusts in the completion of care plans and the delivery of key information relating to Sudden Unexpected Death in Epilepsy (SUDEP). Only 70% of CYP with epilepsy in England had an agreed care plan in place in the first year of care, and over 40% did not have any evidence of receiving information relating to SUDEP and risk minimisation.

In addition, there is currently significant variation between ICBs in the time taken for an electroencephalogram (EEG) and magnetic resonance imaging (MRI) investigations being undertaken. According to Epilepsy12 Cohort 4 audit data, only 52% of CYP diagnosed with epilepsy in England and Wales obtained an EEG within the recommended four weeks of request.

Young people living with epilepsy have said:

Having access to an Epilepsy Specialist Nurse would really help with communication and make it easier to get in contact with a [healthcare] professional.
I have never had a conversation with a healthcare professional about Sudden Unexpected Death in Epilepsy. You end up looking on the internet and doing your own research and using social media to find information.
Care plans would be really helpful for parents and educational settings, it would reduce the amount of explaining you would have to do when attending school.

Variation in care: standards

V1 All CYP with epilepsy should have access to an epilepsy specialist nurse (ESN) as per NICE guidelines (NG217, 11.1.1).

V2 All CYP should receive timely access to appropriate electroencephalogram (EEG) and magnetic resonance imaging (MRI) investigations as per NICE guidelines (NG217, 1.2 and 1.3).

V3 All CYP should have an agreed and comprehensive written care plan provided to them as per NICE guidelines (NG217, 2.1.7). This should be for home and education settings with evidence of involvement from the CYP and their parent/carer.

V4 All CYP/parents/carers should receive personalised and developmentally appropriate information on the risks of Sudden Unexpected Death in Epilepsy (SUDEP). This should include discussions on minimising individual risks listed in NG217, 10.1. This should be documented and revisited annually as part of the care planning process.

V5 CYP should be referred to a paediatrician with expertise in assessing first seizures and diagnosing epilepsy following a first seizure for an appointment within two weeks, as per NICE guidelines (NG217, 1.1.1). There should be clear and explicit referral pathways from primary care to secondary care services with a paediatrician with expertise in epilepsy.

V6 CYP with epilepsy alongside a learning disability, autism or both should receive co-ordinated care using a multidisciplinary team approach (NG217, 9.1). This may involve community paediatricians and ESNs working across acute and community care providers.

V7 Integrated Care Boards (ICBs) should develop and maintain links with any relevant CYP epilepsy networks (for example regional network group affiliated to Organisation of Paediatric Epilepsy Networks in the UK (OPENUK) within their geographical area.

Supporting the mental health and wellbeing of CYP with epilepsy

Rationale

CYP with epilepsy have an increased incidence of mental health conditions including anxiety and depressive disorders when compared to the general population and those with other long-term conditions. Some studies have indicated that 37% of CYP with epilepsy have a mental health condition, compared to 11% of CYP with diabetes, and 9% of the general population. Without early identification and support, this can lead to increased morbidity, treatment outcomes and educational attainment.

In 2021, a survey by Young Epilepsy found 77% of CYP said living with epilepsy has had a significant impact on their mental wellbeing, including their thoughts, feelings and how they are able to cope with everyday life. Furthermore, less than half of young people said their epilepsy doctor or nurse spoke to them about how the condition could impact on their mental health.

Data from Epilepsy12 Cohort 4 audit shows that in England only 20% of Trusts formally screened for mental health disorders. In addition, only 20% of Trusts had mental health provision for CYP with epilepsy, and this was co-located in 10%. Nearly 30% of Trusts did not have agreed referral pathways into mental health services. Without early identification through screening and subsequent referral/management, these problems can lead to increased morbidity and reduced quality of life, treatment outcomes and educational attainment. Epilepsy can also affect the family of children and young people, with parents/carers more vulnerable to anxiety and depression.

In 2023/24, NHS England will pilot models of integrating mental health care within epilepsy services in several ICBs. This will enable an evidence base to be generated for effective models of mental health support for CYP with epilepsy.

Young People living with epilepsy have said:

I’ve never been asked about my mental health during an epilepsy clinic…I think that it is important that everyone has the opportunity to express their emotions and feelings they have due to epilepsy.
Epilepsy has completely changed who I am as a person, affected my confidence and I have developed a life-long battle with anxiety.

Supporting the mental health of CYP with epilepsy: standards

MH1 CYP over the age of five years old with epilepsy should have their mental health screened routinely within epilepsy clinics.

MH2 CYP with epilepsy who have been identified as requiring additional support with their mental health and wellbeing should be referred to an appropriate service to manage their mental health care. Self-help resources to support wellbeing should be made available to CYP with epilepsy. Providers should have access to clear referral pathways and an up-to-date directory of local services of providers/organisations offering the appropriate level of support. This should include services within healthcare, education and Voluntary Sector and Community Enterprise (VCSE) organisations. It may include Child and Adolescent Mental Health Services (CAMHS), NHS Talking Therapies for Anxiety and Depression, Mental Health Support Teams/educational psychology, psychology support, specialist neuropsychology and neuropsychiatry services, and VCSE mental wellbeing support depending on the level of need.

In addition, CYP with identified neurodevelopmental needs (e.g. Attention Deficit Hyperactive Disorder, or Autism Spectrum Disorder) should be referred into relevant local neurodevelopmental pathways.

MH3 Providers should aim for mental health care to be integrated within the epilepsy service. This may comprise of epilepsy clinics where mental health professionals can provide direct input into clinical care, and meetings where epilepsy and mental health professionals can discuss individual patients

MH4 Conversations around mental wellbeing should be routinely incorporated into regular epilepsy review consultations and a part of integrated care planning.  Providers should demonstrate a proportionate response to worsening mental health patterns in CYP with epilepsy and make use of appropriate pathways to access additional support.

MH5 Mental wellbeing support should be available for parents/carers/families of CYP with epilepsy.

MH6 CYP with epilepsy should have access to appropriate self-management strategies to support mental wellbeing, taking  into account age and cognitive ability.

MH7 Epilepsy services should support the review of CYP’s neurodevelopment and cognitive function as part of routine management and reviews.    

Improving referrals into tertiary services and the Children’s Epilepsy Surgery Service (CESS)

Rationale 

Epilepsy surgery in infancy and early childhood is increasingly recommended because it may prevent many years of unnecessary seizures. Surgery can help prevent or reduce the impaired development and behaviour that is sometimes caused by early onset seizures. Children and adolescents with drug-resistant epilepsy who had undergone epilepsy surgery have a significantly higher rate of freedom from seizures and better scores with respect to behaviour and quality of life, compared to those who continued medical therapy alone at 12 months.

NHS England Specialised Commissioning commissions the Children’s Epilepsy Surgery Service (CESS) to provide specialist pre-surgical evaluation and surgery for children in four specialised CESS centres across England, should they meet the acceptance criteria in line with NICE guidelines. In England, it is estimated that 780 children should be evaluated per year, and from these 405 should have a resective surgical procedure. In 2019, the CESS service only received 543 referrals and undertook 398 evaluations and 235 surgical procedures.  

Epilepsy12 Cohort 4 audit data showed that in England only 35% of CYP with epilepsy that met the criteria for surgery were referred to the epilepsy surgery service for assessment within the first year of care. In addition, only 53% of children and young people in England who met paediatric neurology referral criteria had either input from a paediatric neurologist, or had been referred to CESS, during their first year of care.  

In addition to surgical assessment and referral into CESS, CYP may be referred into tertiary care for assessment for vagal nerve stimulation (VNS) insertion or ketogenic dietary therapy in line with NICE guidelines.

Physically he is stronger and more alert [following epilepsy surgery 2 years ago]. His development in his learning and understanding continues to exceed our expectations when we set out on this path. Parent of 11-year old child.
I have seen a [ketogenic diet] specialist…but only after I tried five different medications and my mum had to keep insisting [as] none of them [were] working. Young Person.

Improving referrals into tertiary services and the children’s epilepsy surgery service: standards

TC1 All CYP who meet criteria for tertiary neurology referral should have timely access to the relevant tertiary specialist with expertise in managing complex epilepsy. This should be within two to four weeks from referral depending on criteria set out in NICE guidelines (NG217, 3.1.3 and 3.1.4). Assessment will include consideration of all potential treatment modalities including a Children’s Epilepsy Surgery Service (CESS) evaluation. The initial appointment in tertiary care may be held at the tertiary centre or through a locally held paediatric neurology outreach clinics.

TC2 All CYP meeting criteria for epilepsy surgery should be referred into a tertiary paediatric neurology service for consideration of referral to the Children’s Epilepsy Surgery Service (CESS) for an initial assessment as per NICE guidelines (NG217, 8.2).

TC3 There should be a defined model of shared care between tertiary and secondary care epilepsy services.

TC4 CYP with certain childhood-onset epilepsy syndromes, or drug-resistant epilepsy if other treatment options have been unsuccessful or are not appropriate, should be considered for ketogenic dietary therapy under the guidance of a tertiary epilepsy specialist as per NICE guidelines (NG217, 8.1)  There should be appropriate access across a system to ketogenic dietary therapy to facilitate this. Reasonable adjustments to the approach and implementation should be made in relation to any sensory needs and restrictive preferences for CYP with a learning disability, autism or both.

TC5 All CYP meeting criteria for vagal nerve stimulation (VNS) implantation as per NICE guidelines (NG217, 8.3) should be referred into the CESS for review of investigations and agreement that VNS is appropriate. An outcome of this discussion should be expected within four weeks.

TC6 Where a second opinion is sought, the existing paediatrician with specialist interest and/or paediatric neurologist should be informed to avoid duplication of investigations. Primary care providers should not refer directly to tertiary specialist epilepsy services unless a second opinion is sought.

Improving the transition from paediatric to adult epilepsy services

Rationale 

The need for continuity of care during transition from paediatric to adult services is particularly important for young people managing the physical and mental transition from adolescence to adulthood. Effective management of this transition period by healthcare professionals is vital to develop and maintain the self-esteem and confidence of the adolescent with epilepsy. It also provides an important opportunity to review the diagnosis, classification, cause and management of a young person’s epilepsy before they enter adulthood.

Despite this, Epilepsy12 Cohort 4 audit data shows that nearly 40% of NHS trusts in England do not have an outpatient epilepsy service involving both adult and paediatric professionals. In addition, 65% of Trusts did not have a specific outpatient clinic for young people with epilepsy. A 2021 Young Epilepsy survey showed that 32% of young people said their transition experience had a negative impact on their mental health.

Young People living with epilepsy have said:

Worth knowing the age that you are going to transition to adult services from the day you get diagnosed so people are prepared and there are no shocks or surprises.
Just before I was 16, I got a letter about moving to adult services with a questionnaire. Then a phone call to check my answers, then one joint clinic, which was not enough because [it involves] big changes including you.

Improving the Transition from Paediatric to Adult Epilepsy Services: Standards

T1 All CYP with epilepsy should have a designated named worker responsible for initiating and planning for transition, taking into account any co-existent conditions, as per NICE guidelines (NG43, 1.2.5).

T2 Transition provision and resources should include key essential components and take into account any co-existing conditions and the complexity of epilepsy. The information provided should be individually tailored to the CYP with epilepsy.

T3 During the transition period, paediatric and adult multidisciplinary teams should jointly review the person’s diagnosis and management plan as per NICE guidelines (NG217, 11.2.4). Services should involve the young person and their family or carers in planning and making decisions about their care. 

T4 Transfer into adult services should be developmentally appropriate and at a time of ‘relative stability’ as per NICE guidelines (NG43, 1.2.3).

T5 Service design, delivery and evaluation related to transition (NICE guidelines, NG43, 1.1.1) should involve CYP, their families and carers.

T6 All Young People (YP) with epilepsy should continue to have access to an Epilepsy Specialist Nurse (ESN) for support following transfer to adult services, as per NICE guidelines (NG217, 11.1.1). For autistic patients or patients with a learning disability, there should be access to support from community learning disability teams. 

T7 YP with epilepsy and a learning disability, and autistic YP, should be managed as per Royal College of Psychiatrists Good Psychiatric Practice CR203 . This involves adult learning disability psychiatry teams supported by adult neurology services.

T8 All YP who meet criteria for complex epilepsy should be able to continue to access ongoing specialist epilepsy care (for example specialist treatments such as vagal nerve stimulation (VNS), ketogenic diet or epilepsy surgery) after transfer to adult neurology services as per NICE guidelines. This includes robust protocols during transfer for ongoing prescribing of Sodium Valproate and specialist medications (for example Fenfluramine, cannabidiol (CBD), and Everolimus). 

References

  1. Epilepsy12 – National organisational audit and clinical audit – 2022 [Internet]. November 2022 [cited 2023 April 11]. Available from: www.rcpch.ac.uk/resources/epilepsy12-national-organisational-audit-clinical-audit-2022
  2. Epilepsies in children, young people and adults (NICE Guideline NG217) [Internet]. April 2022 [cited 2023 April 11]. Available from: www.nice.org.uk/guidance/ng217
  3. Scot, A.J., Sharpe, L., Loomes, M., Gandy, M. Systematic Review and Meta-Analysis of Anxiety and Depression in Youth With Epilepsy. Journal of Paediatric Psychology [Internet]. 2020 [cited 2023 April 11], 45(2):133-144. Available from: https://doi.org/10.1093/jpepsy/jsz099
  1. Davies S, Heyman I, Goodman R. A population survey of mental health problems in children with epilepsy. Developmental Medicine and Child Neurology. 2003 [cited 2023 April 11], 49: 292-5. Available from: https://pubmed.ncbi.nlm.nih.gov/12729141/
  1. Young Epilepsy. Young people’s experiences of epilepsy and mental wellbeing 2021 [Internet]. November 2021 [cited 2023 April 11]. Available from: www.youngepilepsy.org.uk/reports/young-peoples-experiences-epilepsy-and-mental-wellbeing-2021
  1. Young Epilepsy. Young people’s experiences of epilepsy care survey 2021 [Internet]. March 2021 [cited 2023 April 11]. Available from: www.youngepilepsy.org.uk/reports/young-peoples-experiences-epilepsy-care-survey-findings-2021
  1. Royal College of Psychiatrists. Management of epilepsy in adults with intellectual disability. Good Psychiatric Practice: College Report CR203 [Internet]. May 2017 [cited 2023 April 11]. Available from: www.rcpsych.ac.uk/docs/default-source/improving-care/better-mh-policy/college-reports/college-report-cr203.pdf?sfvrsn=c534ff08_2
  1. NHS England. Children’s Epilepsy Surgery Service (CESS) [Internet]. January 2018 [cited 2023 April 11]. Available from: www.england.nhs.uk/publication/childrens-epilepsy-surgery-service-cess/
  1. Lukmanji, S., Manji, S.A., Kadhim, S., Sauro, M.K., Wirrell, E.C., Kwon, C.S., Jette, N. The co-occurrence of epilepsy and autism: A systematic review. Epilepsy & Behaviour [Internet]. 2019 [cited 2023 April 11], 98:238-248. Available from: https://doi.org/10.1016/j.yebeh.2019.07.037.
  1. Strasser, L., Downes, M., Kung, J., Cross., J.H., De Haan, M. Prevalence and risk factors for autism spectrum disorder in epilepsy: a systematic review and meta-analysis. Developmental Medicine and Child Neurology [Internet]. 2017 [cited 2023 April 11], 60(1):19-29. Available from: https://doi.org/10.1111/dmcn.13598.
  2. Autistica, Epilepsy Research UK, Young Epilepsy. Autism and Epilepsy: Laying out the evidence [Internet]. October 2021 [cited 2023 April 12]. Available from: www.youngepilepsy.org.uk/reports/autism-and-epilepsy-laying-out-evidence

Publication reference: PRN00318_ii