Ventilation/End of Life

Neuromuscular Disorders Dr Emma Husbands

Consultant Palliative Medicine

Emma.Husbands@glos.nhs.uk

Contents

Cases

NIV and palliation and ethical bits

APM guidelines

Important bits

Lessons from ICU

Terence

54 year old man with progressive MND

Used nocturnal NIV for 3 months

Admitted to hospice for symptom control

increasing constipation/fatigue and intermittent nausea

On settling, declined NIV when nurses tried to apply it

Stated he felt more comfortable with the staff around

Died overnight……..

Unexpected but not surprising death

Patient had capacity

Staff were devastated

Family were relieved

John

61 year old man with MND

Totally paralysed neck down but managing oral intake with

effort.

Increased use of NIV steadily at home such that he was 24/7

dependent.

Wanted to be at home

Did not want to have mask removed BUT clear he was less

well and symptoms increasing despite NIV

Discussion with wife

Explained likelihood of dying while NIV on

Chest would still move but heart would have stopped beating

Family all felt that trying to remove the NIV would cause too much distress to John

Died at home, peacefully

NIV was the norm for him and gave him reassurance

It is OK to die with the mask on

He was in control

Family understood the machine was symptom control

and not stopping the disease progressing

Jane

70 year old woman with MND

Acute admission

BiPAP on ICU

Asking for it to stop

ICU asked for support

Discussed with patient again to confirm her wishes

She understood death may happen quickly, did not want to go

home as felt safe

Stats diamorphine 5mg, midazolam 5mg given s/c

Pump commenced with diamorphine 10mg and midazolam

10mg over 24hrs

Pressures turned down

Mask removed that evening

PRN’s available but no more required.

Died peacefully within 6 hrs with family present

An acute situation but patient able to take control

Use of s/c route was adequate

Doses individually titrated

Staff felt acceptable situation

ICU have some experience of withdrawing treatments

Pall care team felt clear this was what the patient

wanted

NIV – assisted ventilation – has been shown to be beneficial in

a number of neuromuscular conditions.

Improves mortality

Improves morbidity

It’s use has become much more routine

It’s use continues to evolve and change

Tracheal ventilation

Significant carer impact

Prolonging life has seen new stages of illnesses

NIV is a symptom control measure

BUT it doesn’t feel quite the same as giving a dose of oramorph for breathlessness

Ethical concerns

Withdrawal of ventilation

cause of death is the underlying condition not the act of

withdrawal

The ‘active’ nature of withdrawal can make if feel causative

Potential of rapid death after withdrawal can add to this

‘Undignified’ to die on BiPAP

Withholding and withdrawing treatment

Primary aim of starting a treatment is to provide a health

benefit to the patient

The same applies to continuing a treatment already started

A(?THE) key measurement of the health benefit is what the

patient says it is

Psychologically may be easier to withhold a treatment than

to withdraw it……

Newish ground for non-icu and community services

Your experiences??

How do we practically approach withdrawing

ventilation?

Endorsed by GMC/Coroners asc.

Standard 1

“A patient should be made aware that assisted ventilation is a form of treatment and they can choose to stop it at any time.

They should be in no doubt that this is legal and that their health care teams will support them”

Start this discussion at commencement of ventilation

Offer them the opportunity to discuss future scenarios when assisted ventilation is being considered.

Not everyone is ready for this discussion, conversations should be tailored to individual patients

Assess and discuss capacity for the decision about ventilation and it’s discontinuation

Standard 2

Senior clinicians should validate the patient’s decision and lead the withdrawal

Is there an advanced care directive?

Does the patient have capacity for this decision?

Respiratory/ palliative care consultants or suitably experienced general practitioners should lead this process

Standard 3

Withdrawal should be

undertaken within a

reasonable timeframe

after a validated request.

When?

Within a few days

Where?

Home? Hospice? Hospital?

Who?

Who will lead? Who will

manage ventilator? Who will

be there?

Standard 4

Symptoms of

breathlessness and

distress should be

anticipated and

effectively managed.

What drugs should be used,

does the patient need

sedation before the ventilator

is removed or augmented

symptom control?

Medicating Symptoms

GROUP S (Sedation)

Patients highly dependent

on assisted ventilation

Become very breathless or

distressed within minutes of

not having this in place.

Will require sedation before

assisted ventilation is stopped.

GROUP ASC (Augmented

Symptom Control)

Patients who can tolerate

longer periods of time

without assisted ventilation

Develop symptoms after a

longer period of time

will require augmented

symptom control.

Medications to have available

Opioids

Diamorphine/Morphine are most commonly used.

Benzodiazepines

Midazolam has the most flexibility in routes of administration but lorazepam is an alternative.

Levomepromazine

May be a useful second line sedative, especially if a patient is benzodiazepine tolerant or already on large doses.

Suggested initial dose 25mg SC.

Doses

Depends on

Route of administration

sc/iv

Current medication of patient (are they already on high doses of opioids?)

Age/ weight/ physical status of patient

Some examples and recommendations are given in APM guidelines

Other factors

Oxygen

Position of patient

Ventilator settings

Ability to adjust the ventilator

Reduce back up resp rate

Then reduce pressures

Ensure alarms are OFF

Standard 5

After the patients death,

family members should

have appropriate

support and

opportunities to discuss

the events with the

professionals involved.

Who will provide support for

family after death?

Arrange debrief for

professionals.

Submit data set and share key

learning.

Dying with NIV in situ

Ensure family/Carers understand this can happen and

patient’s chest may continue to move despite them

having died.

If expected, may be appropriate to leave instructions on

how to remove the mask and turn off the NIV machine.

Sudden death is not uncommon in neuromuscular

conditions

Might become something we talk about more often with families

Lessons

Be prepared

Patients know their options

Staff know its an option

Locally, we need to be able to act quickly so should have ‘plans’

SPC teams/Resp teams likely to need to collaborate in relation to this

Expect the unexpected

Families/patient/staff need to know timeframes can vary etc

Thanks for listening!