A.M. Lynn, M.D.
The live birth rate in the United States in 1990 was 4,158,212. Major congenital defects that will require surgical corrections or palliation in the first year of life will affect 0.02% (for tracheoesophageal fistula) or 0.04% for imperforate anus as reported in the Congenital Malformations surveillance 1982-1985 from the Center for Disease Control (CDC) in 1988. Infants affected by these major congenital defects necessitating surgery in infancy comprise an orphan population of less that 100,000. For these infants, surgery offers the best (or only) potential for correction and a good quality of life. Postoperative pain control should be given to these infants, as it would be adults undergoing similar surgeries, despite their limited ability to communicate their needs.
Infants and children first received the label therapeutic orphans in 1968 when Shirkey pointed out they were excluded from the labeling of most drugs. Kauffman's editorial in 1991 presented the lack of rigorous study of drugs in infants and children as a continuing problem. More recently in 1996 Kauffman, Cote, and others anticipated the "adoption" of the pediatric therapeutic orphan, as the Food and Drug Administration implemented a more proactive stand encouraging pediatric investigation of pharmacologic agents.