What discussion should the Adult-Gerontology Acute Care Nurse Practitioner (AGACNP) have with a patient with amyotrophic lateral sclerosis (ALS) who is admitted with respiratory failure and requires high-flow nasal canula?

Advance Directives, Mechanical Ventilation, and Tracheostomy Discussion

The AGACNP must initiate a discussion about advance directives, mechanical ventilation options (both non-invasive and invasive), and tracheostomy with this ALS patient and family immediately, as respiratory failure represents a critical decision point requiring urgent clarification of goals of care. 1

Why This Discussion is Critical Now

• ALS patients requiring high-flow nasal cannula are at a critical juncture where decisions about escalation to non-invasive ventilation (NIV) or invasive mechanical ventilation via tracheostomy must be made urgently. 1, 2

• Respiratory failure in ALS can progress suddenly, particularly in association with respiratory tract infections, making it essential to have advance directives established before emergency situations arise. 1

• The patient's cognitive status and decision-making capacity must be assessed now while they can still participate meaningfully in these discussions, as up to 50% of ALS patients have cognitive impairment that can affect their ability to make informed decisions later. 3

Essential Components of the Discussion

Ventilation Options and Their Implications

• Non-invasive ventilation (NIV) should be discussed as the next step if high-flow nasal cannula fails, with clear explanation that NIV may only provide temporary support and has high failure rates in ALS patients with bulbar dysfunction. 1, 2

• Tracheostomy with invasive mechanical ventilation must be presented as an option, with honest disclosure that while it can prolong survival (median 75 months vs 32 months natural course), it does not alter the underlying disease progression and the patient will remain completely or partially ventilator-dependent. 1, 4

• The family must understand that 70% of ALS patients who undergo tracheostomy leave the hospital completely dependent on mechanical ventilation, with only 28% partially dependent and rare cases achieving ventilator independence. 1

Advance Care Planning Specifics

• Document the patient's values, preferences, and any decisions in an accessible site in the medical record, including completion of MOLST/POLST forms if applicable. 1

• Identify a healthcare proxy or medical power of attorney immediately, as the patient's ability to communicate preferences may deteriorate rapidly. 1

• Explore the patient's fears about dying and address anxiety while assessing their understanding of disease progression and prognosis. 1

Critical Information to Convey

• Tracheostomy does not alter the prognosis of ALS itself—it only prolongs the dying process while the underlying neuromuscular disease continues to progress. 1

• Quality of life considerations are paramount: Studies show that ALS patients with tracheostomy can have similar quality of life to those without, but this requires adequate family support, home care resources, and patient acceptance of complete dependence. 1, 4

• The impact on family and caregivers must be addressed, including the need for 24-hour care, potential need for institutionalization, financial implications, and caregiver burden. 1, 2

Common Pitfalls to Avoid

• Do not delay this discussion based on assumptions about the patient's quality of life—healthcare professionals often underestimate the quality of life of ventilator-dependent ALS patients and may inappropriately withhold information about long-term ventilation options. 1

• Avoid using your own perceptions of quality of life as the main factor in deciding whether to offer information about invasive ventilation—this is both a legal and ethical violation. 1

• Do not proceed with tracheostomy without explicit informed consent that includes discussion of the patient's likely complete ventilator dependence, inability to reverse the decision easily, and the progressive nature of ALS affecting all motor functions. 1

• Recognize that patients with cognitive impairment or behavioral changes (present in up to 50% of ALS patients) may have poor compliance with ventilation and are at higher risk of complications, requiring careful individualized assessment. 1, 3

Timing and Approach

• This discussion should occur immediately given the patient's current respiratory failure—waiting until further deterioration occurs may eliminate the patient's ability to participate in decision-making. 1

• Provide anticipatory guidance about what dying from respiratory failure looks like if the patient chooses to forgo invasive ventilation, and ensure palliative care consultation is available. 1

• Ensure the patient and family understand that refusing tracheostomy is a valid choice and that excellent palliative care can provide comfort and dignity at end of life. 1