Long-term Ventilation for Patients With Duchenne Muscular Dystrophy: Physicians' Beliefs and Practices

Abstract

Although long-term ventilation (LTV) has been shown to extend the lives of individuals with Duchenne muscular dystrophy (DMD), initiating LTV is still considered controversial. The purpose of the study was to describe the LTV-related attitudes and practices of Canadian physicians who follow up patients with DMD. The study consisted of a mail questionnaire supplemented by face-to-face interviews. Forty-five physicians who follow up patients with DMD through Canadian neuromuscular clinics. A mail questionnaire of 66 closed-ended questions related to practice and attitudes was completed by all respondents. Qualitative semistructured interviews were conducted with six volunteer physicians, and were audiotaped and transcribed. The results indicated that 25.0% of physicians do not discuss LTV with all of their DMD patients. The most frequently cited reason for advising against LTV was poor patient quality of life (52.6%). Three themes emerged from the qualitative data: mentioning and discussing LTV are discrete events with different purposes, nighttime and full-time LTV decisions are approached differently, and physicians modify their discussions to influence outcome. The study demonstrated considerable agreement among the physicians regarding disclosure practices. Concerns are raised by the number of physicians who do not disclose to all patients and families and the role of quality-of-life judgments in decision making. It is suggested that because of their subjective nature, quality-of-life judgments should not be made without the participation of the patient and family, and that an initial disclosure is the minimum requirement of informed consent/decision making.