Promoting Excellence : ALS Report to the Field : Last Hours


аи Executive Summary
аиаReport to the Field

Identification of Existing Resources

The right to discontinue both invasive ventilation and nutritional support is legal and morally valid in the U.S. Every U.S. citizen has the right to stop ventilator therapy and any other type of life-sustaining therapy, as determined by the U.S. Supreme Court in the 1990 Cruzan ruling. The norms and accepted standards are to follow the valid treatment refusal of a competent patient. In ALS, this often requires establishing a communication system with the patient and proactively obtaining Advance Directives concerning discontinuing ventilator support in patients choosing permanent ventilation or NIPPV. However, the issues surrounding the discontinuation of ventilatory support in patients with ALS in practice can be controversial and difficult to manage (Appendices C and D). For this reason, the establishment of Advance Directives is an important step in guiding the decision-making processes surrounding the last hours of life.

Identification of Existing Gaps

To date, current treatment algorithms on withdrawing respiratory support at the end of life are available for some other disease states, but not specifically for ALS. Many clinicians and health care providers are neither experienced nor comfortable with the clinical management of patients with ALS during the last days of life. The impact of the last few hours has not been well studied. Another gap is that the legal and ethical guidelines surrounding withdrawal of respiratory support are not clear, especially in those patients who become "locked-in" after years of ventilator support and have no Advance Directives regarding discontinuing support.

Recommendations to the Field

Practice Recommendations

Management of Patients on Ventilatory Support:

  • Discuss with patients and families when to withdraw ventilatory support and what to expect at this difficult time. Review Advance Directives with the patient and family. Attempt to establish the basis for withdrawal of ventilation prior to initiating ventilation. Discuss the following issues prior to discontinuing respiratory support:
    • The expected manner and time course of death;
    • Medications that will be used to manage symptoms; and
    • Possible use of sedation.
  • Maintain comfort and a physician presence at the bedside for termination of ventilatory support. All arrangements should be in place prior to the removal of support:
    • All family members wishing to be present should be nearby;
    • All cultural or religious rituals should be discussed, planned and implemented;
    • The location should be prepared, if possible (for example, peaceful lighting and music if desired); and
    • If in a home or nursing home setting, all potentially useful medications and suction should be readily available.
  • Parenteral administration of necessary medications will provide a more rapid onset of action (unless there is already an indwelling IV in place, the subcutaneous route is preferred):
    • It is ethically appropriate to sedate to unconsciousness, but as noted in the Practice Parameter, muscle-paralyzing agents should not be used;
    • If oxygen is not already in use, it should not be instituted at this time. If oxygen is being used, flow should not be increased and discontinuation should be considered. For patients with dyspnea, oxygen may be used to alleviate symptoms; and
    • Once comfort has been obtained, positive expiratory pressure can be discontinued, followed by conversion to a T-piece.

Management of Patients Not on Ventilatory Support:

  • The same principles stated above should be used, except that medications should be initiated in a more gradual fashion since there is no specific event to anticipate:
    • The physician should be readily accessible for medication adjustments;
    • Discontinue use of NIPPV completely, if patients are using NIPPV;
    • Appropriate medications for sedation may be given around-the-clock if distress recurs frequently. All other unnecessary medications should be stopped;
    • All monitoring (vital signs, oximetry) should be discontinued. If hospitalized, no further laboratory testing or X-rays should be done; and
    • Oxygen should not be started but may be used to treat signs of dyspnea.

Research Recommendations

  • Develop treatment and management protocols/algorithms on how to manage pain and withdrawal of ventilatory support specifically in patients with ALS at the end of life.
  • Examine how patients die in a natural setting, as compared to those patients on ventilatory support.

Policy Recommendation

The ALS Peer Workgroup calls on health care policy-makers to investigate how to ensure that patients can die at home or in a hospice setting, with or without ventilatory support. In achieving this goal, the patients' comfort should be a priority and their dignity maintained. Establishing these guidelines will ensure that physicians, patients' families and caregivers are comfortable with the interventions employed at the end of life.

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Promoting Excellence in End-of-Life Care was a national program of the Robert Wood Johnson Foundation dedicated to long-term changes in health care institutions to substantially improve care for dying people and their families. Visit for more resources.

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