Historical review of legislative and national initiatives for sickle cell disease.
The genes responsible for the transmission of sickle cell syndromes from one generation to the next were introduced into the new world during the 17th century. However, this disease was not recorded in the medical literature in the United States until 1910 by Herrick of Chicago. During the next 40 years, many additional cases were reported and a fairly large bibliography developed which dealt essentially with descriptive, clinical and pathological aspects of the disease. New interest in the syndrome occurred in 1949 when Pauling and his associates, employing chemical and electrophoretic techniques, showed that an abnormal hemoglobin was responsible for the sickling phenomenon. In the same year, Neel and Beet, working independently of each other, clarified the inheritance of the disease on the basis of the heterozygous-homozygous hypothesis. In 1958, Ingraham combined the techniques of electrophoresis, chromatography, and trypsin digestion ("fingerprinting") to show that the difference between hemoglobins A, C, and S was in the amino acid sequence of the polypeptide chains which make up the hemoglobin molecule. However, despite these notable advances, interest in the disease remained at a relatively low scientific and health care priority until February 1971, when President Nixon in his message to Congress indicated that greater attention and support for sickle cell disease should be made available at the national level. This paper will review some of the important legislative, political, and organizational initiatives which have had a significant impact on the development and implementation of the current national sickle cell disease program in the United States.
D000755:Anemia, Sickle Cell / Q000188:drug therapy* / Q000266:history; D049673:History, 20th Century; D006801:Humans; D007883:Legislation, Medical; D012109:Research Support as Topic; D014481:United States