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Perspectives on Care at the Close of Life: CODA
September 12, 2007

Amyotrophic Lateral Sclerosis: “Prepare for the Worst and Hope for the Best”

JAMA. 2007;298(10):1208. doi:10.1001/jama.298.10.1208

On July 11, 2007,1 authors Mitsumoto and Rabkin presented the unusual case of a patient with amyotrophic lateral sclerosis (ALS) who was a prominent neurologist specializing in ALS. Amyotrophic lateral sclerosis is a devastating, terminal neurodegenerative disease with a highly predictable clinical course: as the authors state, palliative care begins at the time of diagnosis. Virtually all skeletal muscles are eventually affected and the only approved medication for ALS, riluzole, extends life by only about 2 months. Appropriate management includes physical rehabilitation to maintain motor function, nutritional and respiratory support, augmentative communication devices, and psychological support for both patients and families, especially because family members play a central caregiving role, often for extended periods. Early discussion of social, bioethical, and financial issues as well as advance directives is imperative, ideally long before deterioration in nutritional status might call for enteral feeding or decline in respiratory function might call for assistive ventilatory support. Given the variable but inexorable course of the disease, assessing the patient's goals of care is a dynamic and ongoing process. These complex palliative care needs are best addressed by referring patients to multidisciplinary ALS clinics.