END OF LIFE CARE IN DUCHENNE MUSCULAR-DYSTROPHY

End of life care for patients with Duchenne muscular dystrophy (DMD) h as become increasingly complex because of new technologies, changes in medical personnel over periods of time, emergence of home health care systems, and increasing patient and family autonomy in decision-makin g. In this review, we discuss the medical problems, particularly respi ratory and cardiac failure, faced by DMD patients. Current concepts co ncerning the evaluation and options for treatment of these problems ar e presented as well as the ethical issues involved in the care of the DMD patient. These issues include the medical indications for treatmen t, patient preferences, quality-of-life issues, and contextual feature s related to legal, institutional, religious, geographic, cultural, so cial, and financial factors. We also present our experience at Loma Li nda University Medical Center over the past 10 years in the developmen t of a home mechanical ventilation program for DMD patients and an alg orithm for the evaluation of these patients. Many patients with DMD do well on long-term ventilation, but some find that their quality of li fe is less than desirable and choose to discontinue this method of lif e-prolongation. Many of these new options are very expensive, making t he decision to use them a difficult one. Ultimately, these are societa l issues that require clear reflection on matters of resource allocati on that should be performed by health care professionals, citizens, an d health planners.