Threats to "informed" advance directives for the severely physically challenged? 2003

John R Bach
Department of Physical Medicine and Rehabilitation, University of Medicine and Dentistry of New Jersey-New Jersey Medical School, Newark, NJ 07103-2406, USA.

The neuromuscular diseases, such as infantile spinal muscular atrophy, Duchenne's muscular dystrophy, and amyotrophic lateral sclerosis, are widely considered to be terminal illnesses. However, as with many neuromuscular and neurologic diseases, morbidity and mortality are caused by dysfunction of inspiratory, expiratory, and bulbar musculature. This article will discuss how inspiratory and expiratory musculature can be supported by simple, noninvasive means that are rarely considered when, as with the general population, individuals with disabilities are counseled about advance directives. Failure to use noninvasive aids almost invariably results in respiratory failure, intubation, and tracheostomy or death. When noninvasive aids are available, invasive measures referred to in advance directives (eg, intubation) are often needed only temporarily. Yet, ill-informed patients are often advised to refuse intubation and die or to be intubated and left to decide whether to undergo tracheostomy for long-term ventilatory support. Further, despite severe disability, ventilator users with neuromuscular disease report normal life satisfaction. Health care professionals, on the other hand, tend to ignore the patient's life satisfaction and consider quality of life measures not designed for the disabled to justify withholding life-saving interventions. Advance directives, although sometimes appropriate for patients with irretractable pain and advanced cancer, are inappropriate for patients with severe disability because of muscle weakness, and virtually no patients are appropriately counseled about all therapeutic options.

UI MeSH Term Description Entries
D007258 Informed Consent Voluntary authorization, by a patient or research subject, with full comprehension of the risks involved, for diagnostic or investigative procedures, and for medical and surgical treatment. Consent, Informed
D008020 Life Support Care Care provided patients requiring extraordinary therapeutic measures in order to sustain and prolong life. Extraordinary Treatment,Prolongation of Life,Care, Life Support,Extraordinary Treatments,Life Prolongation,Treatment, Extraordinary,Treatments, Extraordinary
D009468 Neuromuscular Diseases A general term encompassing lower MOTOR NEURON DISEASE; PERIPHERAL NERVOUS SYSTEM DISEASES; and certain MUSCULAR DISEASES. Manifestations include MUSCLE WEAKNESS; FASCICULATION; muscle ATROPHY; SPASM; MYOKYMIA; MUSCLE HYPERTONIA, myalgias, and MUSCLE HYPOTONIA. Amyotonia Congenita,Oppenheim Disease,Cramp-Fasciculation Syndrome,Fasciculation-Cramp Syndrome, Benign,Foley-Denny-Brown Syndrome,Oppenheim's Disease,Benign Fasciculation-Cramp Syndrome,Benign Fasciculation-Cramp Syndromes,Cramp Fasciculation Syndrome,Cramp-Fasciculation Syndromes,Fasciculation Cramp Syndrome, Benign,Fasciculation-Cramp Syndromes, Benign,Foley Denny Brown Syndrome,Neuromuscular Disease,Oppenheims Disease,Syndrome, Cramp-Fasciculation,Syndrome, Foley-Denny-Brown,Syndromes, Cramp-Fasciculation
D010344 Patient Advocacy Promotion and protection of the rights of patients, frequently through a legal process. Patient Ombudsmen,Patient Representatives,Clinical Ombudsman,Patient Ombudsman,Advocacy, Patient,Ombudsman, Clinical,Ombudsman, Patient,Ombudsmen, Patient,Patient Representative,Representative, Patient,Representatives, Patient
D010353 Patient Education as Topic The teaching or training of patients concerning their own health needs. Education of Patients,Education, Patient,Patient Education
D010548 Personal Health Services Health care provided to individuals. Health Services, Personal,Services, Personal Health,Health Service, Personal,Personal Health Service,Service, Personal Health
D010549 Personal Satisfaction The individual's experience of a sense of fulfillment of a need or want and the quality or state of being satisfied. Life Satisfaction,Satisfaction,Life Satisfactions,Satisfaction, Life,Satisfaction, Personal,Satisfactions, Life
D011788 Quality of Life A generic concept reflecting concern with the modification and enhancement of life attributes, e.g., physical, political, moral, social environment as well as health and disease. HRQOL,Health-Related Quality Of Life,Life Quality,Health Related Quality Of Life
D012121 Respiration, Artificial Any method of artificial breathing that employs mechanical or non-mechanical means to force the air into and out of the lungs. Artificial respiration or ventilation is used in individuals who have stopped breathing or have RESPIRATORY INSUFFICIENCY to increase their intake of oxygen (O2) and excretion of carbon dioxide (CO2). Ventilation, Mechanical,Mechanical Ventilation,Artificial Respiration,Artificial Respirations,Mechanical Ventilations,Respirations, Artificial,Ventilations, Mechanical
D003657 Decision Making The process of making a selective intellectual judgment when presented with several complex alternatives consisting of several variables, and usually defining a course of action or an idea. Credit Assignment,Assignment, Credit,Assignments, Credit,Credit Assignments

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