Patients With Amyotrophic Lateral Sclerosis Receiving Long-term Mechanical Ventilation

Advance Care Planning and Outcomes

¹ From the Center for Health Ethics and Law, Robert C. Byrd HSC of West Virginia University, Morgantown
² From the California Kaiser Permanente Medical Care Program, Los Angeles
³ From the MacLean Center for Clinical Medical Ethics, University of Chicago Pritzker School of Medicine, Chicago
⁴ From the Amyotrophic Lateral Sclerosis Association of Eastern Ohio, Canton
⁵ From the Department of Neurology, University of Chicago Pritzker School of Medicine, Chicago

Objective: To examine advance care planning and outcomes of patients with amyotrophic lateral sclerosis (ALS) receiving long-term mechanical ventilation (LTMV).

Design: Case series.

Setting: Population-based study in homes and chronic care facilities in four states, and Home Ventilator Program of California Kaiser Permanente.

Patients: Seventy-five ALS patients receiving LTMV were identified; 11 died prior to interview, and 6 were totally locked in; 50 of 58 (86%) who were able to communicate consented to structured interviews, of whom 36 lived at home and 14 in an institution.

Results: Thirty-eight patients (76%) had completed advance directives, and 96% wanted them. Thirty-eight patients wished to stop LTMV in certain circumstances, of whom 30 had completed advance directives. Those who had completed advance directives were more likely to have communicated their preference to stop LTMV to family and physician than those who had not (76 vs 29%; p=0.05). Patients living at home rated their quality of life on a 10-point scale better than those in an institution (7.2 vs 5.6; p=0.0052), and their yearly expenses were less ($136,560 vs $366,852; p=0.0018).

Conclusions: Most ALS patients receiving LTMV would want to stop it under certain circumstances, and the process of advance care planning enhances communication of patient preferences to family and physicians. Home-based LTMV is less costly and associated with greater patient satisfaction.

Key Words: advance directives • amyotrophic lateral sclerosis • decision making • ethics • mechanical ventilation • outcomes

Submitted on January 17, 1996
Accepted on March 26, 2007

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