Ethical considerations in the management of individuals with severe neuromuscular disorders

There have been many recent advances in improving the quality of life and prolonging life for individuals with advanced neuromuscular disease. These include the use of physical medicine techniques to balance extremity muscle strength and improve range of motion and noninvasive techniques to provide inspiratory and expiratory muscle assistance to prolong life without resort to tracheostomy. Such advances help eliminate the "crisis" decision making about "going on a respirator" and sophisticated assistive equipment and robotic aids. Physicians and society in general use quality of life issues inappropriately derived by questioning physically able individuals to justify withholding or implementing life-sustaining therapeutic interventions for these individuals. Informed decisions about ethically and financially complex matters such as long-term ventilator use should be made by examining the life satisfaction of competent individuals who have already chosen these options. The great majority of severely disabled ventilator-assisted individuals with neuromuscular disease are satisfied with their lives despite the inability to achieve many of the "usual" goals associated with quality of life in the physically able population. Their principle life satisfaction derives from social relationships, the reorganization of goals and from their immediate environment. Although the Americans with Disabilities Act is seen as an important step to prevent discrimination against disabled individuals, it does little or nothing for the self-directed disabled individual who is not informed by his/her physicians regarding potentially vital therapeutic options nor does it help those who are warehoused in institutions because of lack of a national personal assistance services policy.(ABSTRACT TRUNCATED AT 250 WORDS)