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
9
(No Transcript)
10
Categories

• 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

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 dont 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 Lamot
rigine Sodium Valproate
Monotherapy Treatment choices Lamotrigine
Sodium 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 Gaba
pentin 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 thatyou 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 AE 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

22
(No Transcript)
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 shouldnt 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
Midazolam Uses 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
Lorazepam Uses 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
Clonazepam Uses 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 benzos 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

37
(No Transcript)
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

39
(No Transcript)
40
(No Transcript)