1. End of Life Care in MND
Dr Sarah Forrest

2. “So Doc, how long have I got?” Prognostication in MND
Prognosticating / estimating survival is complex in predictable diseases and really hard in MND.
Patients can deteriorate rapidly, often with decompensating respiratory failure and some patients die suddenly in the night.
Some patients have a more predictable slower end of life. Perhaps 70% of people have a recognisable end of life phase.
Many have respiratory failure, approx 50% have infection complicating respiratory failure as the mode of death.

3. Case Study: part 1 My chap, C.
First symptoms July 2013, Rt foot, diagnosed August First met him April 2015
“When they find out what’s really wrong with me...”
April 15: Wheelchair, independent transfers, no speech / swallow issues, breathing stable.
Day Hospice, Physio in the gym, OT support.
Muscular pain, low mood, anxiety...but doesn’t like taking medicines.
October 15: Wheelchair bound, fatigue and sleepiness
Discussions about NIV and future PEG, anticipating MND clinic tests.
“When you have so few days left you don’t want to spend them in hospital, even if you are going to get them back later. After all, it’s the days I have now that I want, not later.”
Jan 16: Electric wheelchair, hoist transfers, NIV at night, some dysarthria, some coughing with food. Unstable with rapid symptom change.
Discussed EOL plans: preferred place of care and death, DNA CPR, just in case medications.

4. Triggers for a short prognosis in MND
Breathlessness / respiratory failure
Swallowing issues
Cognitive changes
Weakness
Worsening complex symptoms, including pain, anxiety.
Hussain J, Adams D, Allgar V, Campbell C. Triggers in advanced neurological conditions: prediction and management of the terminal phase.  BMJ Supp Pall Care 2014; 4: 30-37

5. Triggers in months prior to death

6. End of Life Management Future Care Planning in 3 stages
Explaining the diagnosis
Discussing interventions
Talking about the end of life
What do people need to know about dying with MND?

7. Case Study: part 2
January 16: First chest infection, likely aspiration pneumonia.
“How long have I got?”...retracted... “speak to my wife, she’ll tell me anyway”
Establish starting point
Acknowledge change and explain it
Talk about uncertainty in making a prognosis
Ask about concerns (find those fears!)
Focus on symptoms and managing them
Explain that death is usually comfortable and not related to choking or gasping for breath.
Respiratory failure, rising CO2, falling conscious level, medication as needed.

8. Symptoms at the End of Life
End of life care is quite generic;
it’s not that different in MND
Breathlessness
Pain
Anxiety / agitation
Secretions
Nausea
NIV
Feeding

9. 4 A’s of Anticipatory Drugs: “Just in case”
Analgesia
Morphine, diamorphine, oxycodone
Anxiolytic
Midazolam, levomepromazine
Antisecretory
Hyoscine, glycopyrronium
Antiemetic
Cyclizine, haloperidol, levomepromazine

10. Symptoms at the End of Life
End of life care is quite generic; it’s not that different in MND
Breathlessness: Opioids, fans, sometimes O2
Pain: Opioids, positioning, bladder/bowels
Anxiety / agitation: Midazolam, levomepromazine
Secretions: Hyoscine/glycopyrronium, suction, turning
Nausea: usually drug related
NIV: to stop or to continue...
Feeding: reduce volumes as person deteriorates

11. Case Study 3 September 1st: more sleepy, didn’t make Day Hospice
September 8th: seen in DH with wife. Very sleepy but rousable, sleeping much of the time, eating little, drinking from teaspoon, not thirsty. Pain worse and has started using oxynorm. Anxiety bad and needing lots of diazepam. Community Matron has raised question of a syringe driver. Talked about last weeks to days. Discussed and agreed syringe driver: oxycodone 5mg and midazolam 10mg / 24hrs.
“I’ve had enough now....will you run away with me?...Goodbye everyone!”
September 13th: Reassessed at home, deteriorated, pain and anxiety needing extra sc and oral medications. Not bothered by sleepiness, prefers it. Prescribed new doses for syringe driver: oxycodone 20mg, midazolam 20mg and add glycopyrronium 0.2mg / 24hrs.
September 15th: Reviewed by CNS, unsettled night, wants to be sleepy, not eating, just having sips of fluids. Driver doses increased further to oxycodone 30mg, midazolam 40mg and glycopyrronium 0.4mg / 24hrs.
September 17th: Breathing changes, wife called DNs for stat dose midazolam. Died peacefully at home.

12. Taking care
“Emotional toil”: the work/burden we carry as a result of caring.
Young patients
Fit and healthy
Long relationships
Short relationships
Sudden deaths
Unmanaged problems/symptoms
Inability to make plans

13. Resilience
“Psychological resilience is defined as an individual's ability to
successfully adapt to life tasks in the face of social disadvantage
or highly adverse conditions”
Team working and informal support
Debriefing and Schwartz rounds
Supervision
Counselling