Motor Neurone Disease Who Wants to Refuse A Ventilator in Future — Free SCA Practice Case

Character Overview: You are Philippa Fairclough, a 61 year old retired music teacher. You taught for thirty years and still teach two piano pupils at home. You live with your husband Emrys, who is 63 and retired from the council, in a house with a downstairs bedroom. Your daughter Lowri lives forty minutes away and visits each week. Your son Ianto lives in Australia and phones on Sundays. Five months ago you were told you have motor neurone disease, and since then your arms have grown weaker, especially the right. You are articulate, dry and organised, the sort of person who makes lists. You have decided to take charge of what you can. Last week you watched a documentary about people living on ventilators at home, and one man with a tube in his neck, who could only move his eyes, has stayed in your mind. You are not low or hopeless. You want to live every day you have, but on your terms. Your voice is steady and businesslike, and it wavers when you talk about being trapped or about Emrys.

Opening Sentence: "Hello, doctor. I've got MND, and I'd like it written in my notes, today if possible, that I am never to be put on a ventilator. I don't want to end up like the people I saw on that programme."

History if Asked (Data Gathering Phase)

• ●The programme: "It was last Tuesday, a documentary about people living on ventilators at home. There was a man in his fifties with a tube in his neck. He couldn't speak or move anything except his eyes, and his wife did everything for him day and night. She looked exhausted. I haven't stopped thinking about it. I decided that night I wouldn't let that happen to me."
• ●What she means by a machine: "Any machine that breathes for me. I assumed it was all one thing. The nurse at the clinic did mention a mask I might wear at night one day, but I didn't take it in. It's the tube in the neck and being kept going for years that I can't bear."
• ●Why now: "My right hand is going. My handwriting has become shaky and my signature is already not what it was. I want it settled while I can still do it properly. And the clinic only sees me again in three months."
• ●What she has been told about MND: "It's the type that began in my arms. They said it varies a lot from person to person and nobody can say how fast. They said the muscles for breathing and swallowing can be affected later. They started riluzole."
• ●Arms and function: "I can't do buttons or open jars. I can't play octaves any more but the left hand is still good. Walking is normal and I still take the dog out every morning."
• ●Breathing and swallowing: "No breathlessness. I sleep flat on one pillow. No morning headaches and I'm not sleepy in the day. My voice is normal and I eat and drink without trouble. I've lost about two kilos."
• ●Her mood: "Some days are very low. I cried for a week after the diagnosis and again after the programme. But I'm sleeping reasonably and I still enjoy my pupils, my garden and my grandchildren's photos. I'm not hopeless and I haven't thought about ending my life. I want my life, I just want it on my terms."

Ideas, Concerns, Expectations

Gating note: Philippa does not offer these unless the doctor asks what she thinks, what worries her or what she hopes for.

• ●Ideas: She believes that if the doctor writes "no ventilator" in her records, every hospital and ambulance crew will see it and be bound by it. She thinks it is a simple note rather than a formal document. "You just put it in my file, and that's that, doesn't it?"
• ●Concerns: Her greatest fear is being kept alive but locked in, unable to communicate or to move, and becoming a burden. She fears Emrys would end up as that man's wife did. She also worries that in an emergency, before anyone has read her notes, someone will simply put her on a machine. "I'd rather die than be trapped inside my own body for years. And Emrys would never say stop."
• ●Expectations: She wants a clear written record today and wants it respected. She does not want a long discussion and is a little wary that she will be talked out of it. "I've made my mind up. I'd just like it sorted so I can stop thinking about it."

If Asked: Her Wishes in More Detail

Gating note: Philippa gives these only when asked.

• ●If asked about the mask at night: "If it helped me sleep or stopped me being breathless, I might try it. I don't know. I didn't understand that was a different thing. I'd want to be able to stop it."
• ●If asked about resuscitation: "I haven't thought about it. If my heart stopped suddenly I'm honestly not sure. Emrys would want me to have everything. I'd want to hear what the chances really are."
• ●If asked about hospital: "I'd rather be at home with the dog, but I'd go in if it was something that could be fixed."
• ●If asked what matters most: "Being able to talk to my family, to think clearly and to be myself. If I couldn't communicate, I wouldn't want to be kept going."
• ●If asked whether anyone has pressured her: "No. Lowri thinks I'm being too fast. Emrys won't discuss it. This is entirely mine."
• ●If asked whether she has talked to the MND team: "Not about this. The specialist nurse left me a leaflet on planning ahead and I haven't read it."
• ●If the doctor checks she understands what she is deciding: She can explain in her own words that she is asking not to be ventilated even though she might die as a result, and that she knows she can change her mind.

If Asked: Medical History and Medications

Gating note: Philippa answers these plainly when asked.

• ●Other health problems: "My thyroid is underactive and I take levothyroxine. Nothing else. No heart or lung problems."
• ●Medicines: "Riluzole twice a day and levothyroxine. No other tablets or supplements."
• ●Allergies: "None."
• ●Smoking and alcohol: "Never smoked. A glass of wine on Saturdays."
• ●Who would speak for her: "Emrys, I suppose, but he would never say stop. Lowri is more practical."
• ●Previous plans or documents: "I have a will. I haven't done anything about power of attorney, and I've never heard of a document refusing treatment. Nobody at the clinic has talked to me about resuscitation."
• ●Palliative care: "Not involved so far. I assumed that was for later."

Social History and Lifestyle Impact

• ●Home: Emrys does most of the cooking and the lifting. He is anxious and avoids the subject: "Don't be morbid," he says.
• ●Family: Lowri is a primary school teacher with two young children. Ianto is in Australia and is arranging a visit. They know the diagnosis but not her views on ventilation.
• ●Support: Specialist MND nurse by phone, a private physiotherapist once a month, a neighbour who walks the dog on bad days. No carers yet.
• ●Availability: Free any weekday. Emrys could come to a longer appointment.

If Asked: Associated Symptoms

Gating note: Philippa answers these only when asked. Every one is negative unless stated.

• ●If asked about breathlessness, waking breathless, headaches, or sleepiness: "No to all of those."
• ●If asked about choking, coughing on food, or speech changes: "No. My speech is fine."
• ●If asked about memory, personality change or concentration: "No. I'm as sharp as ever, that's the cruel part."
• ●If asked about thoughts of harming herself: "No. I want to live as well as I can."

Responses to Management (The Negotiation Phase)

• ●If the doctor explains what an advance decision is and what makes it legally binding (written, signed, witnessed, and stating that it applies even if life is at risk):
  • ●Reaction: Surprised, then practical. "So a note from you isn't enough?" She is reassured to hear that it can be made without a solicitor and that she can change it. "My signature is getting poor. Would that matter?" Told that someone can sign in her presence and at her direction: "Good. I'd like to get it done soon."
• ●If the doctor explains the difference between invasive ventilation and a mask, and offers to tailor the document:
  • ●Reaction: Thoughtful. "I really did think they were all the same." She says she would refuse a tube in the neck and being kept on a machine permanently, but would like to be able to try a mask if it eased symptoms. If pushed to decide the mask question now: "Can I think about it and talk to the nurse?"
• ●If the doctor explains that it is different from a decision about resuscitation and that a ReSPECT form records preferences and clinician recommendations:
  • ●Reaction: Surprised. "I thought no machine and no resuscitation were the same." She is willing to discuss CPR later but not to decide today. "Can we do that with the MND team? I'd like proper information on the chances."
• ●If the doctor suggests involving Emrys and Lowri:
  • ●Reaction: Hesitant. "Emrys will say I'm giving up." If the doctor offers to sit down with them or suggests she tell them first: "Maybe I could bring them to the next appointment. I'd rather he heard it from me." She agrees to her wishes being shared with the MND team.
• ●If the doctor offers to ask the MND nurse and palliative care team to help, and to record her wishes in the notes now:
  • ●Reaction: Relieved. "That's all I wanted, really. Someone to take me seriously." If told that until the document is complete it may not be binding in an emergency: "Then let's do it quickly. What happens if I end up in hospital before then?"
• ●If the doctor discusses power of attorney:
  • ●Reaction: Curious. "Emrys would be the person, I suppose, but he'd never refuse anything." If told that a power of attorney made after the advance decision could affect it: "Then I'll need advice on the order. I'll ask the nurse."