End of Life Care in MND Dr Sarah Forrest

“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.

Case Study: part 1 My chap, C. First symptoms July 2013, Rt foot, diagnosed August 2014. 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.

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

Triggers in months prior to death

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?

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.

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

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

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

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.

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

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