Little attention was given to sickle cell screening until the 1970s, when Dr. Roland Scott, in a letter to the New England Journal of Medicine, called for mass premarital carrier screening (Scott, 1970). Scott argued that although it was more prevalent in African Americans than cystic fibrosis, PKU, and other conditions of concern, little public health effort was directed at SCD. Scott noted that there was no cure for SCD, but suggested that it could be the first hereditary illness to be controlled by genetic counseling (that is, by encouraging carriers not to marry or have children). Scott's appeal was echoed in a public awareness campaign, and in 1971, President Nixon singled out SCD for special attention in a health message to Congress, calling for an increase in federal spending on sickle cell research, education, and screening.
Also in 1971, Connecticut passed the first sickle cell screening legislation, which other states quickly followed. These laws were typically introduced by African-American legislators and passed by unanimous vote. Screening was typically mandatory for some groups, but the legislation did not always specify which populations should be targeted; some included newborns, preschool children, individuals seeking marriage licenses, or inmates. Some laws called for carrier screening and some for disease screening.
Initial supporters of SCD screening were spurred on by the success of PKU screening, but the clear difference between SCD and PKU was not fully appreciated until later. There was no intervention for SCD at this time other than counseling to avoid marriage or pregnancy (prenatal SCD screening was not feasible). In addition, questions about whether and how programs should be targeted led to the potential for stigmatization. Some states explicitly targeted only African Americans. The New York statute required urban schoolchildren to be screened, but not rural children. The lack of attention to the eugenic implications of informing someone that he carries sickle cell trait led to charges of racism and growing opposition to screening programs. Most of the laws that were passed in the 1970s lacked confidentiality provisions, and, as a result, there were many documented cases of job discrimination, especially in the military, even for those having asymptomatic sickle cell trait. Eventually, the National Sickle Cell Anemia Control Act, passed in 1972, said federal funds could be used for screening only if programs were voluntary.
Studies published in the 1980s demonstrated that a prophylactic regimen of penicillin in infants significantly reduced the morbidity and mortality of SCD, and in 1987 a National Institutes of Health (NIH) consensus conference called for universal (not targeted) newborn screening for hemoglobinopathies (NIH, 1987). As a result of this recommendation and increased federal funding, 29 states have reinstituted non-targeted newborn screening programs.
The experience with SCD screening in the 1970s illustrates the difficulties that can arise when the goals of screening programs are not clearly specified, when there is no treatment that improves health outcomes, and when the intervention is not acceptable to the target population because of stigma and discrimination.