Managing Respiratory Failure in ALS

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Managing Respiratory Failure in ALS

• John Cunnington, MD, FRCPC
• McMaster University

2
Pathophysiology

• Respiratory muscle weakness
• the patient expired
• Failure to eliminate CO2
• Acidosis
• Confusion CO2 narcosis
• Hypoxia-late
• Coma and death

3
Clinical Issues

• How bad is it?
• Preserving what youve got
• Decision making
• Self determination for patient and caregiver
• Managing respiratory failure
• Prognosis and the patient

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How bad is it?

• Symptoms
• Clinical exam
• Spirometry
• Blood gases

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How bad is it? Symptoms

• Shortness of breath
• With activities
• At rest
• At night
• Smothering
• Panic
• Confusion

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How bad is it? Clinical exam

• The fish out of water
• Pulse
• Respiratory rate
• Air entry
• (Cyanosis)

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How bad is it?Spirometry

• Measuring breathing capability
• Vital capacity
• How much can you breath out?
• Normal 3-4 litres
• If you can only breath out 1 litre you have
  severe impairment

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How bad is it?Blood gases

• Oximetry
• Warning value lt90 (late stage)
• Arterial Blood Gases
• Carbon dioxide PCO2
• Pt has respiratory failure if PCO2 is gt45
• Acidosis

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Keeping what youve got

• Drugs
• Chest physio--generally not indicated
• Breath stacking
• Assisted coughing

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Decision making

• When is the time to talk about end of life
  decisions?
• Honesty in talking about a fatal disease
• Planning for the end of life

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Self determination for the Patient

• Providing the information
• Allowing the patient to reflect
• Letting the patient decide
• The importance of the point of view of health
  care workers

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Self determination for the Caregiver

• Should the patient decide how the partner is to
  live?
• Helping the caregiver realize the legitimacy of
  their own dilemma

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Managing respiratory failure

• Palliative care
• Reassurance
• Relief of breathlessness (with narcotics)
• Oxygen generally not helpful
• Pumps for moving air (ventilation)
• Ventilation
• Bipap

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BiPap

• The temporary solution
• In your face
• When could it help? Resp. failure
• Nocturnal use
• Continuous use
• Comfort and tolerability
• Palliative use

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Tracheostomy and Ventilation

• Mechanical ventilation
• At home? Your partner as ICU nurse
• Or, institutional care
• When does it seem to make sense?
• Complications
• The downside of being kept alive
• Disease progression
• Inability to communicate
• Quality of life

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Prognosis and the Patient

• Travel
• Honesty
• How long have I got?
• What will it be like at the end?
• 911?
• Typical ends and support to the caregiver