Coping with Complex Epilepsy Whilst Striving for a Quality of Life for the Whole Family – Facilitating a Parent’s Perspective

A fascinating look at recent findings from a survey that uncovered some intriguing views on the treatment of complex epilepsy.

Emma Williams MBE – Founder and Chief Executive for the Matthew’s Friends Charity for Ketogenic Dietary Therapies and Director of the Matthew’s Friends Clinics for the management of medical Ketogenic Dietary Therapy. Emma has worked in this field for over 16 years after her own son Matthew responded so well to the ketogenic diet. Setting up the charity that bears his name and supporting families from all over the world, she is a co-author of many medical papers and presents at conferences globally. The Matthew’s Friends mission is to make medical ketogenic dietary therapies available to all those who may need them with the appropriate medical support and information.

Clare Szwec BSc RD – Medical Affairs Advisor for Nutricia Advanced Medical Nutrition. Clare is a registered dietitian with eleven years of experience working in the Medical Nutrition industry. Clare’s role specifically supports the epilepsy area of Nutricia by providing scientific and nutritional direction based on critical evaluation and appraisal of clinical information and research. Clare’s role also involves the provision of education programmes for health care professionals raising awareness of complex epilepsy and how it can be effectively managed.

Nia Emlyn-Jones BSc MSc – Senior Strategy and Insight Manager for Nutricia Advanced Medical Nutrition. Nia has worked in policy, stakeholder and market research for fifteen years, across consultancy, public sector and commercial organisations. Nia is responsible for designing, executing and analysing a wide variety of market research, with a focus on supporting the business to translate findings into activity and focus that best meets the needs of patients.

For families receiving an initial diagnosis of epilepsy the focus is all about stopping the seizures. Parents take their child to the doctor and expect them to be able to ‘cure’ the seizures by giving them medicine. However, in the UK 36% of epilepsy patients have inadequate control of seizures with AEDs1. These families for whom seizure freedom is not achieved by medication endeavour to cope with complex drug resistant epilepsy (DRE). For them the seizures become ‘just’ seizures and it is everything else that surrounds them that takes over day to day living. ‘Normal’ life has become a thing of the past and it is finding a quality of life (QoL) between seizures that is crucially important for the continued survival of these families.

We also know QoL is an important measure of treatment efficacy yet there are no validated assessment tools available to take into consideration the subtle changes in QoL in children with DRE that maybe considered by their families as huge improvements but cannot be quantified. A recent survey was developed to attempt to better understand exactly what QoL looks like to these parents and in particular how the side effects of management options impact this.

The survey was scripted with 37 questions of differing types including open, closed and free text. The questions were rigorously reviewed by an expert panel of specialist healthcare professionals and families with children with epilepsy to check for sensitivity and content. The survey was then distributed via charity contacts and social media.

51 responses were received from parents of children with epilepsy, 37 of which experienced the ketogenic diet therapy (KDT) as a management option. The responses were very detailed and over half of parents leaving contact details (unusual for so called anonymous market research which clearly demonstrates the need of these vulnerable families for support and for their voices to be heard)

Key observations from the responses2:

42% of responders rated their child’s QoL as poor or very poor and QoL when related to seizure reduction, happiness and enjoyment, ability to have social interactions with peers and achieve a normal everyday life. The language used was not aspirational and to these families QoL was more so around being normal and being able to do all the things that everyone else does.

Only a quarter of responders reported an improvement in QoL with the introduction of anti-epileptic drugs (AEDs) with 82% reporting negative side effects which impact the whole family. Over a half (59%) reported that doctors did not recognise the full extent of AED side effects. One family even reported that these side effects were sometimes worse than the seizures themselves.

Keto AED infographic

Side effects related to AEDs, including poor attention span, low energy levels, poor memory and sleepiness were reported in over two thirds of the responders.

49% of parents with children on KDT reported that KDT had improved their child’s QoL. Over half reported an improvement in those same measures during dietary management; attention, energy levels and memory, with less anxiety, sadness and depression. Parents also commented that they welcomed the opportunity of KDT to do something for/take part in the care of their child. They felt it was empowering at a time when they felt helpless and unable to do anything to make their child’s life happier/better.

Discussion and Conclusion

Epilepsy continues to be considered a taboo illness and QoL is poor in almost half of responders to this survey. The responses indicate that AEDs impact on QoL more than other therapies like the ketogenic diet which has a perception (rightly or wrongly) as ‘difficult’. Despite the challenging nature of the initial introduction of KDT it is linked to an improvement in QoL with parent reported outcome measures which are not currently measured as part of management efficacy. These families need a QoL in between seizures and so does the child. However, if, when attempting to control the seizures, the extent to which AED side effects impact the child and family could be more fully recognised, and the HCPs and families could find a management plan where improving QoL is the aim, balancing seizure control with AED side effects then Social Service involvement and support would be eased. This is because families would be in a better position to cope and therefore savings would be generated.

The finding from this survey demonstrates that KDT can have a positive impact on QoL over and above seizure reduction and therefore, it begs the question – why is it that the KDT is not considered or offered as a credible epilepsy management an earlier stage?

Furthermore, despite the NICE Guideline3 recommending referral to a tertiary centre for consideration of other management such as KDT, VNS or surgery after failure of 2 or more appropriately prescribed drugs; over 60% of the children in this survey group had been prescribed 5 or more AEDs. This finding also contradicts the recently updated global consensus statement from the world’s leading experts in KDT which state that if the first 2 medications fail then the diet should at least be discussed with the family. This suggests there is a treatment gap whereby some children are being denied or having delayed access to options that can be as effective as drugs or could indeed be curative such as surgery. This is perhaps corroborated by a third of these families reporting that they had researched and initiated conversations about KDT with their doctor. Research shows that at least 10% of children that go on the diet WILL become seizure free for the rest of their lives with two thirds of children going on the diet having at least a drop of 50% in their seizures and for a child that is having 100’s of seizures a week a 50% drop is massive and means there is more of a chance of a life between seizures.

Complex epilepsy is by definition very complicated and requires expert management so for ease of identification of those patients to refer without delay to a tertiary centre for consideration of KDT the recently updated International Ketogenic Diet Study Group consensus guideline4 lists the types of syndromes that are known to respond well to KDT and are listed in the following tables 1 and 2.

Although this is a small survey with some limitations as the target population surveyed could have an inherent bias for non-pharmacological management it does serve to highlight the importance of using parent reported outcome measures in research and clinical practice to evaluate treatment efficacy. It further demonstrates that KDT can have a positive impact on QoL over and above seizure reduction and should therefore be considered earlier. With such potential benefits the KDT should no longer be considered as a last resort and should be spoken about in a more positive and proactive way.

Download this article as a PDF from here.

The scientific poster from this survey was accepted and presented at the 2019 British Paediatric Neurology Association’s annual conference. To view the poster click here.

Acknowledgements

Matthew’s Friends Charity for Ketogenic Dietary Therapies
The Daisy Garland Charity
Epilepsy Ireland
Epilepsy Society
Epilepsy Action
Young Epilepsy
Dr Anita Devlin, Consultant Paediatric Neurologist, Great Northern Children’s Hospital in an advisory role
Birmingham Women’s and Children’s Hospital

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Date of Preparation: December 2018