Withdrawing Non-Invasive Ventilation in ALS
Mr. R is 62 with bulbar-onset ALS, 18 months from diagnosis. He has a percutaneous endoscopic gastrostomy and uses non-invasive ventilation (NIV) at night and increasingly during the day. He has a forced vital capacity of 28% predicted. He presents with pneumonia and is now on continuous NIV, unable to speak more than a few words, and using accessory muscles. He has an advance directive stating he does not want intubation. He is drowsy but wakes when spoken to and nods. His wife says he told her last week 'I don't want to live like this.' The team asks whether to continue NIV and whether to start morphine.
1. How do you assess his wishes and decide about continuing NIV?
Read the worked answer
This is a capacity and goals-of-care assessment under pressure. His advance directive refusing intubation does not automatically address NIV, which is a different intervention and may be a comfort measure or a life-sustaining treatment depending on context.
Assessment:
- Determine decision-specific capacity: can he understand that stopping NIV will likely shorten his life, retain the information, weigh it, and communicate a choice? His drowsiness and communication difficulty make this hard but not impossible; use yes/no questions, a communication board, or a speech device, and involve someone skilled in ALS communication.
- Clarify his prior statement with his wife and, if possible, with him directly. 'I don't want to live like this' is meaningful but ambiguous; explore what 'like this' means (dependency, breathlessness, inability to speak).
- If he has capacity and wants to stop NIV, that is his decision and should be honored, with a plan for symptom control.
- If capacity is uncertain, treat reversible contributors (hypoxia, infection, medication) and reassess; use the substitute decision-maker based on his known wishes.
- Document the discussion and the decision, and inform the team.
2. If NIV is withdrawn, how do you manage his breathlessness and distress, and what do you tell the family?
Read the worked answer
Withdrawal of NIV is a legitimate, ethical decision when it aligns with the patient's wishes. The goal is a peaceful death, not to hasten it, and symptom management is titrated to comfort.
Management:
- Premedicate before withdrawal: an opioid (e.g., morphine) titrated to relieve breathlessness, and a benzodiazepine (e.g., midazolam) for anxiety and air hunger. Give a dose before stopping NIV so distress does not escalate.
- Stop NIV in a planned way, with the family present if they wish, and with a nurse at the bedside.
- Continue opioids and benzodiazepines as needed, titrated to symptoms; do not withhold because of fear of respiratory depression when the goal is comfort.
- Treat secretions (anticholinergics, positioning, suction only if helpful), keep him warm and clean, and provide mouth care.
- Continue to involve the ALS team and palliative care.
What to tell the family:
- This is what he wanted, and we will keep him comfortable.
- He may breathe differently and may not wake; that is expected.
- We will not leave him in distress and will adjust medicines as needed.
- Reassure them that stopping NIV is not 'giving up' but following his wishes.
Document the premedication, the withdrawal plan, and the family conversation.