RT Journal
A1 Mitsumoto H, Rabkin JG
T1 Palliative care for patients with amyotrophic lateral sclerosis: “prepare for the worst and hope for the best”
JF JAMA
JO JAMA
YR 2007
FD July 11
VO 298
IS 2
SP 207
OP 216
DO 10.1001/jama.298.2.207
UL http://dx.doi.org/10.1001/jama.298.2.207
AB 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.