"Extraordinary Burdens and Extraordinary Opportunities"

Susan Jeffrey

Medscape Medical News 2007. © http://www.medscape.com/viewarticle/559781

July 13, 2007 — A patient with amyotrophic lateral sclerosis (ALS) — himself a neurologist and prominent ALS specialist – becomes the focus of a new article looking at the broader principles of palliative care in this devastating disease.

The article, by Hiroshi Mitsumoto, MD, DSc, from the Eleanor and Lou Gehrig ALS/MDA Center at the College of Physicians and Surgeons, Columbia University Neurological Institute, in New York, and Judith G. Rabkin, PhD, from the department of psychiatry at the College of Physicians and Surgeons at Columbia, is published in the July 12, 2007 issue of the New England Journal of Medicine, part of a series called Perspectives on Care at the Close of Life.

Given its predictable terminal course, the authors write, palliative care should begin soon after diagnosis, and the goals of care be assessed on an ongoing basis.

"Each day focus on what you can do," writes the neurologist-patient, identified as Dr. SP. "I've always tried to prepare for the worst and hope for the best."

Extraordinary Burdens, Extraordinary Opportunities

Chronic neurodegenerative disorders such as ALS are "among the most difficult diseases with which clinicians must deal," Drs. Mitsumoto and Rabkin write. The only medication approved for the treatment of ALS in the United States, riluzole (Rilutek, Sanofi-Aventis), prolongs life by about 2 months, but the ultimate outcome is certain in most cases, they note.

"Virtually all skeletal muscles eventually are affected, they write. "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."

In this article, the authors look at a variety of issues related to management and palliative care in ALS, interspersed with perspectives from Dr. SP and his family. For example, they discuss not only how to diagnose the disease but also advise on specific ways to disclose this devastating diagnosis, as well as how to outline the prognosis.

"To deliver the diagnosis of this well-publicized disease requires great sensitivity and care," they write. "Sooner rather than later, the clinician must discuss the nature of the disease and the importance of advance directives, always striving to maintain realistic hopes."

Drs. Mitsumoto and Rabkin provide a broad discussion, describing the symptoms to expect and how to manage them, projected costs of various devices and services required to care for the patient, and the emotional burden that can be expected both for the family and the professional staff who care for them.

"Thus, the physician caring for patients with ALS experiences both the extraordinary burdens and extraordinary opportunities in providing expert care throughout this tragic illness," the authors conclude.

Many of these concepts are applicable not only to ALS, they add, but to other fatal progressive neurologic diseases such as Huntington's chorea and late-stage Parkinson's disease.

This project was partially supported by the Muscular Dystrophy Association's Wings Over Wall Street (Dr. Mitsumoto) and Eleanor and Lou Gehrig MDA/ALS Research Center, Columbia University. Dr. Mitsumoto reported receiving an honorarium from Avanir Pharmaceuticals for a scientific advisory meeting in 2006; being a consultant for Eisai Pharmaceutical from 2005 to 2007; receiving a research grant for a clinical trial at Columbia University from Aeolus; and receiving education grants to Columbia University from Sanofi-Aventis, Ethena Diagnostic, and Bioscrips. Dr. Rabkin reported no financial disclosures.

N Engl J Med. 2007;298:207-216. http://www.ncbi.nlm.nih.gov

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