Epilepsy Management in People with a Learning Disability

1. Epilepsy Management in People with a Learning Disability Mark Scheepers Consultant Psychiatrist in Learning Disability Gloucestershire

2. Theme for today • Why me, why here, why now? • Guidelines • Treatment options • Morbidity & Mortality • Rescue medication

3. Are people with LD special? • Epidemiology • Aetiology • Communication • Behaviour/Psychiatric symptomatology • Co-morbidity with other conditions • Morbidity and mortality

4. Objective evaluation of the facts FINISHED FILES ARE THE RE- SULT OF YEARS OF SCIENTIF- IC STUDY COMBINED WITH THE EXPERIENCE OF MANY YEARS…

5. Differential diagnosis of seizure • Hallucinations • Panic attacks • Behaviour Disorders • Physical illness • Epilepsy - partial (simple, complex, 2° generalised) - generalised (absence, clonic, tonic, tonic- clonic, myoclonic & atonic) • NEAD (non-epileptic attack disorder)

6. Epilepsy management • Diagnosis - when, who, valid still? - are all attacks seizures? • Physical injuries • Psychological factors • Social factors • Educational needs • Status Epilepticus

7. Guidelines

8. Evidence Table Considered Judgement Graded Recommendation

10. The Individual Investigations Seizure & Syndrome diagnosis Treatment History The Care Context Impact of Epilepsy Treatment Options Drug Monitoring Rescue Treatment Standards for Services Risk Assessment Categories

11. Expanded contents • The Individual • Treatment Options • Standards for Service

12. The person with LD & epilepsy • Needs a comprehensive evaluation of their current abilities, health status and co-morbidity • Needs an assessment of current psychiatric or behavioural problems • Needs a review of their ability to understand and communicate in order to make an informed choice • Needs an assessment of impact of the epilepsy and the treatment on the patient and the carers before changes are carried out

13. When the drugs don’t work…. What to do, what to do, what to do…

14. Principles of Medication Use • Ensure the patient receives the correct drug for their seizure type or syndrome • In patients still having seizures despite the correct drug -review diagnosis - review treatment adherence - check that maximum tolerated dose has been used III. If the first drug remains ineffective, introduce a second drug slowly, without tapering the first. If the patient becomes seizure free, consider gradual withdrawal of the first drug.

15. Principles of Medication Use II • If reasonable options for monotherapy have been explored and the acceptable symptom control has not been achieved, long-term two drug therapy should be tried • If the first add-on drug is not effective, add a third drug and then slowly withdraw the second (ineffective) drug. This can be repeated with other drugs • If symptoms are still not controlled with two drugs, some patients may benefit from an additional third drug

16. Treatment Pathways Generalised Seizures Unclassifiable Partial Seizures Treatment choices Treat as generalised initially Monotherapy Treatment choices Carbamazepine Lamotrigine Sodium Valproate Monotherapy Treatment choices LamotrigineSodium Valproate Monotherapy drugs should be used in rotation with titration to effect or side effect MONOTHERAPY FAILURE Add-on choice Add-on choice Levetiracetam Topiramate Levetiracetam Tiagabine Topiramate Pregabalin Gabapentin Zonisamide

17. Byzantine treatmentAs soon as the patient gets up in the morning and has emptied his bowels, he should drink an infusion of hyssop, which will do him a lot of good, as many have been healed simply by drinking this, and were only taken ill two or three times.It is forbidden to drink undiluted wine after taking a bath as nothing can set off a seizure more easily than this - and indeed undiluted wine is in general dangerous for all epileptics."

18. Service standards • Medication, where prescribed, is given • Adequate training occurs for the provision of rescue medication • Experienced staff should attend health professional contacts where management decisions are to be made • Risk assessments should be undertaken to reduce the impact of epilepsy on QOL

19. Summary points • Epilepsy has a great impact on PWLD and their families • A multiprofessional approach is essential to reduce this impact • Seizure reduction remains the primary aim as this significantly improves QOL • The care setting impacts on the treatment application • Few studies are LD specific and many interventions are non-LD based

20. Medicine in the Middle Ages: Falling Sickness Blessing (14th/15th century): "As convulse and bewitch are walking across the heath,they meet the Holy Virgin Mary.the Virgin Mary asks convulse and bewitch:'convulse and bewitch, where are you going?'convulse and bewitch say: 'We are going to him and him.'The Virgin Mary asks: 'What are you going to do there?'convulse and bewitch say'We're going to tear flesh, drink blood and break legs.'The Virgin Mary says: 'You must not do that:you must go where there are bare rocks,there you can tear flesh, drink blood and break legs.'May god the father, god the son and god the holy ghost help us. Amen."

21. Morbidity & Mortality • Published data are limited • Clinicians report an increased number of injuries in PWLD – higher attendance to A&E since the closure of the institutions • PWLD have a reduced life expectancy (SMR 1.6), this is further reduced if they also have epilepsy (SMR 5) and even further reduced if they have epilepsy and cerebral palsy • SUDEP

23. What is rescue medication and what would be the drugs’ ideal properties? • Medication used intermittently for the treatment of acute seizures including status • May require different drugs for different seizure types (clusters, prolonged, cyanotic) • The drugs used should be effective at terminating seizures • They should be easy to administer • They shouldn’t have significant side effects

24. Rescue Medication • May be used to stop acute seizures, preventing progression to status epilepticus in those at risk • To prevent repeated seizures that may be short lived but these clusters may lead to injury etc. • To terminate seizures with cyanotic episodes • To prevent seizures that occur at specific events, cycles or situations • May need to be administered to conscious and unconscious patients

25. What drug and when? • What is the context of the use of rescue medication (status, clusters,cyanotic)? • Is the patient conscious or unconscious? • Where, when and by whom is the drug being administered? • Is there a way of reviewing medication? • How often can the drug dose be repeated?

26. Diazepam • Low potency, long duration benzodiazepine • Effective via oral, rectal & intravenous routes • Rectal quicker than oral absorption, but variable (between 20-30minutes to peak plasma level) • Highly lipophillic so quickly crosses BBB • Short duration of anti-epileptic action as redistributed to peripheral fat, large volume of distribution & long half-life (1-2 days) • May cause behavioural difficulties

27. Rectal DiazepamUses and application • Gold standard treatment for acute seizures • Easy and safe to administer with an appropriate licence for use in epilepsy • Long half-life leads to hangover effect, sedation and behavioural difficulties • Reports of schools & day centres being unwilling to administer a rectal treatment • Possibly reduces quality of life due to reduced opportunities (difficult to administer in public places, long period of recovery )

28. Midazolam • High potency, short duration benzodiazepine • Effective via buccal, intranasal, IV & IM routes • Mucosal absorption very good with peak blood concentration after 5-10 minutes • Short half life (3 hours) and small volume of distribution • Acidic and therefore bitter to taste, irritant to nose

29. MidazolamUses and application • As effective as Diazepam for acute seizures • Administered via mucosal surfaces (mouth & nose) in various settings, but unlicensed • Significant implications on impact of epilepsy as can be administered in public places • No hangover effect so therefore no significant loss of opportunities & improved QOL • Can be repeatedly administered with no significant problems

30. Lorazepam • High potency, short duration benzodiazepine • Effective via oral, IV and sublingual routes • Intermediate absorption when given orally, long plasma half life (8-24 hours) • Longer duration of anti-epileptic action • IV drug used as first line treatment of status • Oral drug may be used for short term treatment of clusters or repeated/ breakthrough seizures

31. LorazepamUses and application • Effective in management of status epilepticus • Primary use as an anxiolytic, but also licensed for status epilepticus • Effective as short term treatment of breakthrough or repeated seizures where the patient can take oral medication • Less hangover effect as not as lipophillic as other benzodiazepines, possibly improving QOL

32. Clonazepam • High potency, long duration benzodiazepine • Effective via oral & intravenous routes • Intermediate absorption when given orally, long half life (20-80 hours) • Dependent on acetylation so individual variability in individual dose • May be used as an adjunct in severe epilepsy and may be given alternate days to avoid tolerance

33. ClonazepamUses and application • Effective in the management of repeated or breakthrough seizures when given orally • Can precipitate the emergence of different seizures • Tolerance may develop with prolonged use • Quite significant levels of sedation with poor psychomotor co-ordination • Levels of sedation a concern in ID as sedation can reduce opportunities

34. Clobazam • High potency, long duration 1,5 benzodiazepine • Only available as an oral preparation • Intermediate absorption when given orally, long half life (10-55 hours) • Effective in all types of epilepsy • Fewer side effects than other benzodiazepines (particularly in psychomotor effects) • Higher incidence of tolerance

35. Clobazam Uses and application • Effective in the management of breakthrough or repeated seizures when given orally • Less side effects than conventional benzo’s with potentially less sedation • Widespread use as an adjunctive therapy • Most problematic is the development of tolerance

36. Protocol for rescue medication • Rescue medication may often be required when managing epilepsy in LD • Clear care plans including the reason for drug use, the dose, the timing of administration & total dose in 24 hours should be drawn up & reviewed regularly • Where the patient is conscious, a choice of oral formulations exist & should be used • Where unconscious, there is a choice of drugs: Rectal diazepam remains the standard, but Midazolam has potential

38. Conclusions • People with a learning disability commonly have epilepsy as an associated condition • This may be complex and difficult to treat • The aim of a service should be to try to have outcomes which are as close to the general epilepsy population as possible • These patients should have the full range of investigations and treatments available to them