ABSTRACT
Degenerative neuromuscular diseases are characterized by a gradual decline of motor
function leading to respiratory collapse, while the patients retain consciousness
and cognition. The ethical challenges of caring for such patients result from the
need to implement various combinations of initiating, withholding, and withdrawing
life-sustaining interventions.
In caring for this population of patients physicians should adhere to the ethical
principles of autonomy, beneficence, nonmaleficence, and justice. A central goal of
care is to avoid a decisional impasse by anticipating end-of-life issues in discussion
with patients and families. The evolution of these diseases is usually slow enough
to allow ample patient education, and thus physicians should foster early and frank
discussions and encourage the patient to set up advance directives, designate a durable
power of attorney for health care, and plan end-of-life care.
Competent patients have the right to accept or refuse life-sustaining therapies, and
such requests should be honored. In delivering palliative care, adequate sedation
and analgesia must be provided when needed. If a decision to withhold or withdraw
life support is made, patient comfort and dignity are the ultimate objectives.
KEYWORDS
Ethics - neuromuscular diseases - end-of-life care - palliative care
