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Perspectives on Care at the Close of Life
Clinician's Corner
July 11, 2007

Palliative Care for Patients With Amyotrophic Lateral Sclerosis: “Prepare for the Worst and Hope for the Best”

Author Affiliations

Perspectives on Care at the Close of Life Section Editor: Margaret A. Winker, MD, Deputy Editor, JAMA.


Author Affiliations: Eleanor and Lou Gehrig ALS/MDA Center, College of Physicians and Surgeons, Columbia University, Neurological Institute (Dr Mitsumoto) and Clinical Psychology in Psychiatry, Department of Psychiatry, College of Physicians and Surgeons, Columbia University and New York State Psychiatric Institute, (Dr Rabkin) New York.

JAMA. 2007;298(2):207-216. doi:10.1001/jama.298.2.207

Amyotrophic lateral sclerosis (ALS) is a devastating terminal neurodegenerative disease with a highly predictable clinical course such that palliative care should begin at or soon after diagnosis. The outcome is certain in most cases. The only medication approved for treatment in the United States, riluzole, extends life by about 2 months. Virtually all skeletal muscles eventually are affected. Multiple problems require a multidisciplinary approach including aggressive symptomatic management, rehabilitation to maintain motor function, nutritional and respiratory support, augmentative communication devices, and psychological support for both patients and families because family members so often play a central role in management and care. Social, bioethical, and financial issues as well as advance directives should be addressed long before enteral feeding or assistive ventilatory support might be considered. Goals of care should be assessed on an ongoing basis. Presenting the unusual case of a patient with ALS who is also a prominent neurologist specializing in ALS, we enumerate issues in management and palliative care applicable to ALS but also to other fatal, progressive neurologic diseases such as Huntington's chorea and late-stage Parkinson disease.

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