Care of Infants With Disease: The Ultimate Objective of Newborn Screening

We conclude that newborn screening with a comprehensive follow-up program, emphasizing parental education and the use of penicillin prophylaxis, is effective in reducing morbidity and mortality from pneumococcal infections in infants with sickle cell disease. One of the 159 infants followed-up during 1981 to 1986 died as a result of overwhelming sepsis. This compares with an anticipated mortality rate at that time of 10%. Penicillin prophylaxis should be started at 3 months of age in infants with Hb SS and should also be given to infants with hemoglobin SC and S- β-thalassemia. The duration for which prophylaxis must be given is not known, but it should be continued at least through the age of 5 years. Monthly injections of benzathine penicillin is an inadequate regimen; we recommend that, if penicillin is to be administered by the intramuscular route, it should be given every 3 weeks and supplemented in the last week with oral penicillin. However, we now emphasize that the daily oral administration of penicillin is the preferred method of administration for prophylaxis. Pneumococcal vaccine continues to be given at 2 years of age, with a booster dose recommended at 4 to 5 years of age. H influenzae vaccine conjugates are immunogenic when given at 18 months, and newer vaccines may soon be available that can be given much earlier. Finally, and most important, the program must be structured in such a way as to thoroughly involve the parents in assuming responsibility for their child's continued health and well being.