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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. |
