November 2000
ATLANTA - Sickle cell disease (SCD) affects 3:1,000 black newborns and about 50,000 people in the United States. Because children with SCD, especially those younger than 3, are at increased risk for severe morbidity and mortality, newborn screenings are vital for providing early medical intervention.
From 1995 through 1998, re searchers in California, Illinois and New York conducted a three-year follow-up of all children born and diagnosed with SCD during 1992 and 1993. These states offer universal newborn screening for hemoglobinopathies and accounted for about 20% of all births to blacks in 1992 and 1993.
Parents and physicians of affected children completed surveys that provided follow-up information. State health departments mailed physician surveys to each child's last known provider. Parental surveys were mailed, first, and then telephone contact was made. Repeated attempts were made to locate the children.
During 1992 and 1993, 1,042 newborns were diagnosed with SCD in these three states. Fourteen children died before the study began. Physician surveys were completed for 752 (72%) children; 252 (24%) had parental surveys completed. Both parental and physician surveys were completed for 184 (18%) children.
Seventy-six percent (575) of physicians reported providing antibiotic prophylaxis to their SCD patients but that only 253 (44%) of these children complied with treatment. Twenty-five percent (189) of children received pneumococcal vaccine, and 24% (179) received at least one dose of Haemophilus influenzaetype b conjugate vaccine (Hib).
Parental survey results differed greatly from physician survey results. Forty-four percent (111) of parents said they were informed of SCD services available for their children, and 27% (68) said they used these services. Parents reported that 93% (234) of their children received penicillin prophylaxis. Seventy-five percent (189) said their child had received pneumococcal vaccine, and 65% (164) said their child had received a full series of Hib vaccine.
The researchers noted that data to assess program goals are incomplete for most disorders identified by newborn screening and that study results show just how difficult it is to find and contact families retrospectively.
For more information:
- Pass K, Harris K, Lorey F, et al. Update: newborn screening for sickle cell disease - California, Illinois, and New York, 1998. MMWR. 2000;49(22):729-731.
![[bar]](/art/gradred.gif){kind=small}
You can
express your views on this article, or other relevant themes, in the
Infectious Diseases in Children
Specialty Forums.