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) GROUP ASC (Augmented Symptom Control) 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. 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!