Learning disabilities and medicines management

What is a learning disability?

A significantly reduced ability to understand new or complex information and to learn new skills (impaired intelligence).

- With a reduced ability to cope independently (impaired social functioning).

- which started before adulthood (in developmental phase), with a lasting effect on development.

How many people in the UK have a learning disability?

1.5 million people in the UK have a learning disability

How do we measure intellectual function?

Through an IQ score and history.

- IQ is measured through WAS-IV (Weschler Adult Intelligence Scale 4)

There are 10 different types of subtests including:

-Verbal Comprehension (VCI)

-Perceptual Reasoning (PRI)

-Working Memory (WMI)

-Processing Speed (PSI)

These then come together to form an IQ score

What IQ score is indicative of a learning disability?

FSIQ < 70 = learning disability

This is the bottom 2 % of the population

What are signs that someone has a learning disability?

1. Often have communication difficulties

2. Limited understanding

3. Limited reading, writing, numeracy skills - no academic qualifications or below GCSE level

4. Needs support with daily living activities or managing household

5. Unlikely to maintain employment without support

6. Likely to be unable to drive

7. Pointers in history: special schooling, delayed developmental milestones

What is the difference between a learning disability and learning difficulty?

Learning disability means the same thing as intellectual disability.

A learning difficulty is a milder level of cognitive impairment.

The IQ for learning difficulty is 80-90

- Learning difficulties can relate to particular elements in learning e.g. reading and writing.

What can cause learning disabilities?

Any insult to developing brain prior to adulthood (any brain damage during childhood).

- This can occur in the antenatal period, prenatal period, or postnatal period.

- The greater the severity of the learning disability, the more likely there is an identifiable cause

Give examples of damage to the brain which can occur in the antenatal period

Genetic conditions, congenital infections/teratogens (e.g. epileptic drugs such as topiramate)

Give examples of damage to the brain which can occur in the prenatal period

Birth asphyxia/hypoxia e.g. birth trauma

Prematurity

Give examples of damage to the brain which can occur in the postnatal period

Childhood meningitis/encephalitis

Traumatic brain injury

Who is in the community team for patient's with learning disabilities?

The MDT team comprises of:

- LD Nursing (Health facilitation and behaviour nurses)

- Psychology

- Physiotherapy

- Occupational Therapy

- Speech and Language Therapy

- (Dietetics)

- (Pharmacy)

Wider team tends to include social workers

(LD = learning disability)

What conditions are people with learning disabilities more at risk of getting?

1. Dementia

2. Epilepsy

2. Dental hygiene

4. Sensory impairments

5. Heart disease

6. Diabetes

7. GI problems e.g. reflux and dysphagia

8. Cancer, particularly GI cancers

Why are people with learning disabilities more likely to get GI problems?

For people with more severe LDs (e.g. cerebral palsy) gastrointestinal complications are more likely. There is an increase in H.pylori infection in those with LDs.

- Often goes undiagnosed as people with LDs have difficulty in expressing symptoms

What do the health problems of patients with LDs (learning disabilities) relate to?

Some health morbidity needs of people with learning disability relate to the underlying cause of the learning disability.

Many of the health morbidities/ health needs of people with learning disabilities relate to difficulty in accessing healthcare and lifestyle factors.

Both of these areas need to be assessed as it is often a combination of these factors.

Describe the relationship between epilepsy and learning disabilities

40% of people with LDs have epilepsy

- Epilepsy often more common due to structural brain abnormalities

Often patients need:

- multiple anti-epileptic drugs

- rescue medications in the community

Epilepsy can worsen cognitive impairment - seizures are epileptogenic themselves. The more seizures, the worse epilepsy gets so more epilepsy.

Many deal with mental illness and challenging behaviour

Be aware of SUDEP (sudden death in epilepsy). Risk factors are much higher in individuals with epilepsy.

Describe the relationship between dementia and learning disabilities

- Risk of dementia is higher in low IQ groups

- This may be due to lower cognitive reserve, starting from a lower baseline in patients with LDs.

- There is an increase in risk of Alzheimer's dementia in patients with Down syndrome - this is due to amyloid precursor gene, results in amyloid plaques presenting at an earlier age.

- Can develop Alzheimer's as early as 40-50 years old in patients with LDs.

How is Alzheimer's dementia managed in Down's syndrome?

Use of anti-dementia medication like normal treatment.

But, the evidence base for people with Down syndrome is non-existent.

Donepezil is the only Ach inhibitor which has been evaluated in Down syndrome

Monoclonal antibody therapies could be ground breaking for patients with Down syndrome. Lecanumab and decanumab are examples which are currently waiting for approval.

Describe mental illness in patients with learning disability

Patients with LD are twice as likely to develop mental illness

Can be harder to diagnose, looking for signs and behaviours as people with LDs struggle more with communication

The risks can be more complex - higher rates of suicide among patients with autism and depression.

What is autism?

Difficulties with social communication, repetitive and restrictive interests. They have sensory differences - greater sensitivity to the environment e.g. noise.

Describe autism in patients with learning disabilities

- 20-30% of people with LD have autism (ASD = autism spectrum disorder)

- Engage in self-soothing behaviour e.g. challenging behaviour, infrastructure destruction, self-injury, aggression to others

- Likely to have comorbid mental illness, particularly anxiety - need to be mindful of epilepsy risk (SSRIs and antipsychotics lower the seizure threshold).

- May be hard to meet physical health needs if they don't want you in their personal space. May require benzodiazepines (sedation) or physical restraints.

- It is important to understand their autistic needs to understand their behaviour.

Describe mortality in LD population

LeDer project investigated lives and deaths of people with a learning disability (LD) and autistic people.

6 in 10 people with a LD will dies before 65 years compared to 1/10th of the population

Main causes of death were similar to the general population

- Covid-19

- Diseases of circulatory system

- Diseases of respiratory system

- Cancer

- Diseases of the nervous system

How many people with a learning disability die from preventable deaths compared to the general population?

People with LD die early and often of treatable health conditions.

49% deaths in LD were 'avoidable' compared with 22% of general population.

What are causes of premature mortality in the LD population?

1. Communication issues: 50% of people with LD have communication difficulties

2. Behaviours

ASD (autism spectrum disorder), dementia, anxiety

3. Capacity

4. Lack of understanding of specialist needs of people with LD

5. Perception of quality of life judgements

- For example, DNR (do not resuscitate) decisions. Just because they have a LD, does not mean their quality of life is poor.

What should we do according to the LeDer project to reduce premature mortality in people with learning disabilities?

1. Make reasonable adjustments to support patients with LD to access healthcare

2. Annual health check: Important for everyone with a LD to access their annual health check (only 75% had their AHC a year before their death)

3. LD liaison nurses are helpful for bridging the gap between principles and provision of good care

What are examples of reasonable adjustments that can be made for patients with a LD?

- reduced waiting time

- quiet environment (side room)

- longer appointments: due to communication difficulties

- accessible information

- understanding how the person communicates

- family/carer input: ask how the patient communicates best

- thinking outside the box/'detective work' (consider whether behaviour demonstrates underlying cause)

- objective measures e.g. NEWS-2 score

What are strategies to check understanding of patients with LD?

1. Ask patient to summarise the information that you've said

2. Ask patient to repeat the key information

3. Use visual aids to supplement verbal information where possible - (e.g. easy read leaflet)

Also explain to family member or carer

Describe how annual health checks are conducted

- RCGP provides a toolkit; a systematic way to check individuals physical health - looking for physical health issues

- Use of the comprehensive geriatric assessment tool kit: this can be useful for the disabled population as well as elderly.

- STOPP-START guidelines: which drugs should be reviewed, discontinued or commenced.

What are the benefits of the annual health checks for people with LDs?

- Good for addressing unmet health needs

- Allows time for medication review and polypharmacy (epilepsy medication, reflux, constipation, anxiety)

What is the role of the LD liaison nurse?

The LD liaison nurses supports understanding of reasonable adjustments and use of mental capacity act in hospitals.

According to the Mental Capacity Act 2005, what criteria must a person meet in order to have capacity?

To have capacity, they must:

1. Understand information relevant to the decision

- They don't need to understand everything, but have the same understanding as the average person.

2. Retain the information

3. Use or weigh-up the information as part of the decision process

4. Communicate their decision

can be non-verbally

What are the 5 key principles of the Mental Capacity Act 2005?

1. Presume capacity

All adults should be presumed to have capacity unless they demonstrate that they don't have capacity

2. Support them to make decisions

3. Unwise decision accepted

If have capacity and make unwise decision, still must accept it

4. Best interest

Any decision made on behalf of those who lack capacity should be done in their best interest

5. Least restrictive option

Anything done for or on behalf of people without capacity should restrict their rights and freedom as little as possible

What are the general principles of prescribing in people with learning disabilities?

- Start at low dose and go slow

- People with LDs have more contraindications with medication

- We must always consider the impact on seizure threshold of medication we are prescribing.

- Blood brain barrier is more permeable

- Genetic condition can cause metabolic differences

- Diagnostic overshadowing

- People with LDs have difficulty in communicating side effects. Careful record keeping is required, recording of adverse effect

- Challenges with monitoring

Why do we prescribe half the BNF recommended dose in people with LDs?

1. Medication could lower their seizure threshold

- If have a seizure, causes more brain damage, worsens cognitive function

2. Impaired blood brain barrier

- Their blood brain barrier is more permeable due to physical brain damage causing the learning disability. Therefore, there is increased risk of CNS side effects from medication

3. Reduced drug metabolism

- Genetic conditions can cause metabolic differences

What is diagnostic overshadowing?

Attributing symptoms of a physical illness to the mental illness

In this case, it would be when symptoms of a drug are attributed to the learning disability. Therefore adverse effects can be hidden. Therefore we should look at changes before and after the medication.

Why may it be difficult to monitor patients with a learning disability?

Co-operation with blood test monitoring can be difficult in patients with LD (especially in autism: challenging behaviour). Must consider what adjustments may be needed

What side effects should we monitor closely in patients with LD?

- Deterioration in seizure control

- Mood and behaviour side effects on antiepileptics

- Cognitive side effects on antimuscarinics

- Mobility impairment and falls (looking for changes, as LD condition can already cause falls)

- Weight gain (especially in Down syndrome)

- Swallowing problems (increased rates of dysphagia in LD)

- NEWT guidelines for enteral feeding tubes or swallowing difficulties

Describe the use of psychotropic medication in people with learning disabilities

- We have now decreased the use of psychotropic medication for people with LDs.

- Need to be clear about whether we are prescribing for challenging behaviour or mental illness.

- Medication should be the last resort for challenging behaviour as historically we over-prescribed antipsychotic drugs in people with LDs.

- Should treat early if it is for mental illness e.g. early intervention in psychosis.

What drugs used to be wrongly used for challenging behaviour?

- Droperidol

- Benperidol

- Thioridazine

- Chlorpromazine

- Zuclopenthixol

- Haloperidol

- Paraldehyde

These medications cause an array of side effects.

We don't want to just sedate people with LDs with high doses of psychotropic drugs.

Describe the STOMP-LD initiative

There has been an NHS initiative called STOMP-LD (stopping over medication of people with learning disability, autism or both)

- Medication should be used for the right reasons, at the right dose, for short time as possible (minimum effective dose used)

- Question and review use of psychotropic drugs in LD patients

- It also focuses on improving understanding of effective non-drug treatments.

- Make sure family and carers should be involved in any decision to start, stop, reduce or continue medication (shared-decision making).

What does NICE recommend in terms of using psychotropic medication in people with autism and people with LDs?

They advise not using psychotropic medication first line for people with autism and behaviour that challenges.

- Only use psychotropic medication as a last resort if behavioural approaches are ineffective.

- Or use of medication to allow delivery of behavioural interventions.

Psychotropic medications should not be used for the core symptoms of autism. It is important to review the risks and benefits of the medication.

What medications can be used for behaviours that challenge (particularly in autistic people)?

1. SSRI antidepressants

If fails, then start...

2. Antipsychotics (low dose) e.g. risperidone for autistic anxiety or over-arousal

3. Anticonvulsants: old treatment; not recommended nowadays

4. Propranolol for symptomatic anxiety

5. Naltrexone for self-injury behaviours (perpetuated by opioid release); prevents release of opioids after self-injury behaviour

Describe the monitoring and reviewal of psychotropic medication in patients with LDs

- Want to discontinue as soon as possible. However, can continue long term if increases QoL of patient and after discussion with patient and carers.

- Weight monitoring and healthy lifestyle input is important with antipsychotics (as side effect of antipsychotic is weight gain).

- Blood test monitoring: hyponatraemia and hyperprolactinaemia is very common. People with LDs are likely to suffer from osteoporosis, therefore raised prolactin levels are likely to make this worse long term by decreasing bone density.

What is a challenge of sodium valproate use in patients with a LD?

Sodium valproate prescribing and risk acknowledgement form relies on the woman signing and accepting the risks of sodium valproate. For women who lack capacity, it is hard to document these decisions - it may require "best interest decisions" to be made. This requires careful documentation.

What is VERA?

VERA is very easy read information for a range of mental health conditions. This can be given to patients with LDs so they can understand their treatment

What are some alternatives to the use of psychotropic medication for people with LDs?

1. VNS: vagal nerve stimulation (anti-epileptic treatment)

2. Busy Brains and busy bodies

3. Environment

4. Talking therapies

5. Mindfulness

6. Consistency of support

Sensory strategies (especially in autism spectrum disorder)

7. Communication support

What should pharmacists to aid people with learning disabilities?

1. Consider how a patient communicates

2. Question and review medication

3. Make reasonable adjustments and go the extra mile (e.g. talking with family, allowing extra time for meetings).

4. Contact liaison nurse or other specialist for help (if needed).