JAMA : the journal of the American Medical Association
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Living wills have been strongly endorsed in principle. Unfortunately, existing living wills are rarely used in clinical practice because they are vague and difficult to apply. To remedy this, we propose a new advance care document: the Medical Directive. ⋯ Finally, there is a section for a statement of wishes regarding organ donation. The Medical Directive provides an opportunity for significant improvement in the documentation of patients' preferences regarding life-sustaining care in states of incompetence. As an expression of a patient's wishes, the Medical Directive should be honored by courts and should facilitate physician-patient discussions of critical and terminal care options.
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Of 18 nonblack patients with sickle cell disease, 14 had sickle cell anemia, 2 had hemoglobin SC disease, and 2 had hemoglobin S-beta o-thalassemia. The beta s gene cluster haplotypes that were determined in 7 patients were of African origin and were identified as Central African Republic, Central African Republic minor II, Benin, and Senegal. The haplotype Central African Republic minor II was present on the beta o-thalassemia chromosome in 2 patients. ⋯ These data strongly support the concept that the beta s gene on chromosome 11 of these individuals is of African origin and that the alpha-gene locus on chromosome 16 is of white or native American origin. The clinical severity of the disease in these nonblack patients is appropriate to their haplotype without alpha-thalassemia-2 and is comparable with that of black patients. All persons with congenital hemolytic anemia should be examined for the presence of sickle cell disease regardless of physical appearance or ethnic background.