Younger adult with intellectual disability in a care home

Case summary

Background (age, sex, occupation, baseline function)

38-year-old female
24-hour care in group care home due to severe intellectual disability

History of presentation/reason for review

Care Programme Approach (CPA) called to discuss deterioration in presentation
She had become very drowsy, has recurrent vomiting, increased seizure frequency, intermittent episodes of stupor/unresponsiveness, appears pale and clammy

Current medical history and relevant co-morbidities

Non-oral: medications and sustenance given via gastrostomy
Recurrent aspiration risk (including silent aspiration from secretions)
Recurrent chest infections/pneumonia - exacerbates seizures and requires out-of-hours GP contacts
Different seizure types – tonic-clonic, tonic, focal, myoclonic, status epilepticus (including non-convulsive status epilepticus (NCSE))
Lennox-Gastaut Syndrome (LGS)

Current medication and drug allergies (include over the counter (OTC) preparation, traditional medicines and herbal remedies)

Allergies or adverse drug reactions (ADRs): nil

Clobazam 5mg/5ml suspension: 10mg (10mls) at night (regular treatment of seizures)
Lamotrigine 100mg dispersible tablets: one tablet twice a day
Lamotrigine 25mg dispersible tablets: two tablets twice a day
Metoclopramide 5mg/5ml solution: 10mg (10mls) three times daily
Mirtazapine 15mg/5ml solution: 30mg (10mls) at night
Phenytoin 30mg/5ml solution: 270mg (45mls) once daily at night
Rufinamide 200mg/5ml suspension: 200mg (5mls) twice a day
Sodium valproate (Epilim®) 200mg/5ml liquid: 1gram (25mls) twice a day

As is common in those with intellectual disability, the medication regime is static, with minimal changes in recent years, other than addition of mirtazapine.

Over the counter medicines: nil

Lifestyle and current function

Non-smoker, nil alcohol, diet entirely comprised of enteral feed
Has normal periods of alertness and interactivity, reduced when physically unwell or has had a recent seizure

“What matters to me” (ideas, concerns and expectations of treatment)

She is non-verbal
Elderly father and sister have ongoing contact. They are distressed but also accept that she had been given a limited life expectancy as a child.
Fully prepared to take recommendations from the Health and Social Care Team
Family feel that antiseizure medications (ASM) are most important, and acknowledge the anxiety caused by the introduction of the Valproate Pregnancy Prevention Programme (“Prevent”) for women of childbearing age

Observations, examinations and results

Note: local laboratory reference ranges may vary

Weight 47kg, Height 155cm, BMI 19.6kg/m²
Routine bloods are similar to her normal baseline (e.g. elevated LFTs aligned with treatment with sodium valproate and rufinamide, albumin levels within range and no recent changes)
Chest clear on auscultation
Urinalysis normal

Most recent relevant consultations

Two months ago: antidepressant therapy started for signs and symptoms indicative of a depressive episode, with moderate improvement
Four weeks ago: Valproate Pregnancy Prevention Programme annual risk assessment form completed by intellectual disability psychiatrist
Meeting convened to discuss commencement of palliative care pathway:
- Discussion with medical team, welfare power of attorney, family and carers around immediate commencement of palliative care, including a decision not to aggressively treat symptoms of physical health, admit to hospital or provide Hospital at Home care
- Evident from case records that recent ASM therapeutic drug monitoring (TDM) has not taken place with documentation that regular drug monitoring is not indicated. Epilepsy nurse specialist advises that given complicated profile, it would be indicated in this instance
- Concerns raised about some aspects of the presentation being similar to neurotoxicity and/or non-convulsive status epilepticus
- Acknowledgement and documentation of medication regime including contra-indications (metoclopramide) and pharmacological agents that are difficult to manage or outwith scope of best practice guidelines (phenytoin for management of LGS)

7 steps: Person specific issues to address

1. Aims: What matters to the individual about their condition(s)?

Review diagnoses and consider:

Therapeutic objectives of drug therapy
Management of existing health problems
Prevention of future health issues, including lifestyle advice

Ask person to complete PROMs (Questions to answer before medicines review) before their review

Person specific issues to address

Improving quality of life: over sedation potentiates poorer/recurrent physical health issues
To review polypharmacy and potential interactions

2. Need: Identify essential drug therapy

Identify essential drugs (not to be stopped without specialist advice)

Drugs that have essential replacement functions (e.g. levothyroxine)
Drugs to prevent rapid symptomatic decline (e.g. drugs for Parkinson’s disease, heart failure)

Person specific issues to address

All antiseizure medications (ASM) require specialist input. Rufinamide requires enhanced specialist consideration and referral
Legislative requirements met for ongoing use of sodium valproate
Changes to non-ASM could destabilise physical health and thus create additional provoking factors for increased seizures

3. Does the individual take unnecessary drug therapy?

Identify and review the continued need for drugs

what is medication for?
with temporary indications
with higher than usual maintenance doses
with limited benefit/evidence for use
with limited benefit in the person under review (see Drug efficacy & applicability (NNT) table)

Person specific issues to address

Mirtazapine – confirm indication. Are there sufficient signs of mood disorder/moderate depression to balance the risk of over-sedation (with clobazam). Consider if signs of low mood caused by frequency of seizures
Consider sedation profile

4. Effectiveness: Are therapeutic objectives being achieved?

Identify the need for adding/intensifying drug therapy to achieve therapeutic objectives

to achieve symptom control
to achieve biochemical/clinical targets
to prevent disease progression/exacerbation
is there a more appropriate medication to achieve goals?

Person specific issues to address

Seizures and respiratory conditions have known linear connection. Sedation can contribute to overall poorer outcome
Assess effectiveness by combination of:
- Review drug history and consider cautious reduction in overall drug load
- Review for evidence of neurotoxicity from ASM and consider pharmacokinetic and pharmacodynamic interactions
- Increase sensory and interactive activities to monitor improved cognitive response

5. Safety: Does the individual have or is at risk of ADR/ side effects? Does the patient know what to do if they’re ill?

Identify individual safety risks by checking for

appropriate individual targets e.g. HbA1c, BP
drug-disease interactions
drug-drug interactions (see ADR table)
monitoring mechanisms for high-risk drugs
risk of accidental overdosing

Identify adverse drug effects by checking for

specific symptoms/laboratory markers (e.g. hypokalaemia)
cumulative adverse drug effects (see ADR table)
drugs used to treat side effects caused by other drugs

Medication Sick Day guidance

Ensure discussion and clear information on which medicines to withhold at times of dehydrating illness

Person specific issues to address

Risk of inappropriate palliation
Risk of over-sedation and worsening of chest health, leading to risk of morbidity/mortality
Mirtazapine has the potential to increase seizure frequency
ASM TDM to monitor for potential toxicity, then ongoing to monitor variance in levels
Note phenytoin has non-linear pharmacokinetics and will need at least 21 days following dose changes to reach steady state again
Ongoing consideration of potential for reactive neurotoxicity when prescribing, deprescribing, or alterations of dose
Metoclopramide contra-indicated in epilepsy and ADRs may cause misdiagnosis/inappropriate prescribing (see additional information regarding metoclopramide below)
Care staff should be trained in the administration of any rescue medication
Staff education on identification of likely symptoms of non-convulsive status epilepticus and neurotoxicity
Include pro-forma / symptom checklist to support when to escalate

6. Sustainability: Is drug therapy cost-effective and environmentally sustainable?

Identify unnecessarily costly drug therapy by

considering more cost-effective alternatives, formulary choices, safety, convenience

Consider the environmental impact of

Inhaler use
Single use plastics
Medicines waste
- over-ordering
- disposal
Water pollution

Person specific issues to address

Medicines should be prescribed by brand for epilepsy
Clear rationale for non-tablet formulation
Care staff advised to dispose of medicines through community pharmacy and advised to only order what is needed

7. Person-centredness: Is the person willing and able to take drug therapy as intended?

Does the person understand the outcomes of the review?

Consider Teach back

Ensure drug therapy changes are tailored to individual’s preferences. Consider

is the medication in a form they can take?
is the dosing schedule convenient?
what assistance is needed?
are they able to take medicines as intended?

Agree and communicate plan

discuss and agree with the individual/carer/welfare proxy therapeutic objectives and treatment priorities
include lifestyle and holistic management goals
inform relevant health and social care providers of changes in treatments across the transitions of care

Ask person to complete PROMs (Questions to answer after medicines review) after their review

Agreed plan

Phenytoin: implement two weekly serum level check – most unpredictable pharmacokinetic (non-linear) and highly interactive and highly protein bound
If changes to medication do not resolve altered consciousness and CT/ MRI alongside blood tests (U&Es, calcium, etc) are clear, consider EEG to exclude neurological basis of deterioration (non-convulsive status epilepticus NCSE)
ASM: referral to neurology and joint working with epilepsy and intellectual disability team to rationalise current ASM
Use of formal care/support structures (Care Programme Approach) to potentiate inter-professional collaboration and mapping of outcomes
Share information to support parents/family/carers e.g. local carers centre, Epilepsy Scotland website, Quarriers fieldworker service (this should have occurred via epilepsy specialist services input).

Outcomes

Blood tests showed severe neurotoxic levels of phenytoin 33mg/L (normal range: 10-20mg/L)
Phenytoin cautiously reduced in 25mg increments every four weeks and levels returned to baseline

Key concepts in this case

Diagnostic overshadowing in people with intellectual disability. This occurs when a health professional makes the assumption that the behaviour of a person with intellectual disability is part of their disability without exploring other factors such as biological determinants
People with intellectual disability often remain on medication longer than necessary and are more likely to accrue polypharmacy
Long-standing polypharmacy: a changing presentation requires investigation due to various factors that have been subject to change e.g. biophysiology secondary to natural ageing; addition of medication due to age related physical health needs
Role for proactive monitoring of blood serum levels in complex clinical scenario
Reactive prescribing of anti-emetic added to sedating profile, was potentially pro-epileptic and required treatment of neurotoxicity symptoms

Additional information regarding metoclopramide:

People with intellectual disability are more likely to have acute episodes of gastrointestinal distress and are often prescribed short courses of metoclopramide.
Metoclopramide adverse effects include movement disorders and extrapyramidal side effects which can complicate differential diagnosis and/or lead to diagnostic overshadowing of stereotypy, subtle epileptogenic activity (i.e. the side effects of metoclopramide may be confused with signs of epilepsy). In individuals with communication deficit, this increases the risk of emergent behaviours or changes of presentation being misdiagnosed or diagnostically overshadowed (i.e. side effects of metoclopramide could be interpreted as normal behaviour in someone with intellectual disability).
Metoclopramide is contra-indicated in epilepsy; people with intellectual disability have a 30-33% prevalence rate for epilepsy. This sub-population are more likely to develop late onset or epilepsy of older age due to atypical cerebral structures.
All of the above may be underpinned by ongoing or longer than indicated therapy with metoclopramide.