Know what to do when a sickle cell prep is positive in the newborn

Know what to do when a sickle cell prep is positive in the newborn: Excerpt from Avoiding Common Pediatric Errors

Author: Heidi Herrera, MD

What to Do - Take Action

Sickle cell disease (SCD)is the most commonsingle gene disorderin African Americans. Other high-risk infant populations include the Mediterranean countries, Turkey, the Arabian and Indian subcontinent, Hispanic persons in the United States, and people from the Caribbean and South and Central America. SCD can now be diagnosed in the neonatal period and pediatricians should familiarize themselves with their particular state's screening program. A screening sample should always be obtained before blood transfusion regardless of gestational age.

Ahigh-riskinfantnotscreenedatbirthorwhoismissingdocumentation should be screened by hemoglobin electrophoresis as soon as possible. For infants with positive results, confirmatory testing should be performed at least before 2 months of age so that parents can be educated and penicillin prophylaxis started.

Confirmatorytestingrequireshemoglobinseparationbyelectrophoresis (cellulose acetate and citrate agar), isoelectric focusing, or high-performance liquid chromatography. Solubility testing methods (Sickledex, Sicklequik) and sickle cell preparations are inappropriate diagnostic techniques for the newborn. These tests do not differentiate SCD from sickle cell trait. In addition, due to the predominance of fetal hemoglobin in newborns, it can cause false-negative results in infants with SCD. Solubility testing methods can always detect sickle hemoglobin in persons with severe anemia.

Most infants with SCD are healthy at birth and only become symptomatic later in infancy when fetal hemoglobin levels drop. Affected patients can present with painful swelling of the hands and feet (dactylitis), pneumonococcal sepsis or meningitis, severe anemia splenic enlargement, acute chest syndrome, pallor, or jaundice. Therefore, an early diagnosis can alert theparentsbeforethediseasebecomesclinicallyapparent.Prophylacticpenicillin should be started by 2 months of age and the heptavalent conjugated pneumococcal vaccine (Prevan). After 6 months of age, children can begin receiving influenza virus vaccines.

Suggested Readings

American Academy of Pediatrics. Health supervision for children with sickle cell disease. Pediatrics. 2002;109(3):526–535.
Wethers DL. Sickle cell disease in childhood: part I. Laboratory diagnosis, pathophysiology and health maintenance. Am Fam Physician. 2000;62:1013–1020, 1027–1028.

Book Source Details

• Book Title: Avoiding Common Pediatric Errors
• Author(s): Anthony D Slonim MD, DrPH; Lisa Marcucci MD
• Year of Publication: 2008
• Copyright Details: Avoiding Common Pediatric Errors, Copyright © 2008 Lippincott Williams & Wilkins.

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• Back to disease: Neonatal Respiratory Distress Syndrome: Introduction
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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.

More About This Book: Title: Avoiding Common Pediatric Errors Authors: Anthony D Slonim MD, DrPH; Lisa Marcucci MD Publisher: Lippincott Williams & Wilkins Copyright: 2008 ISBN: 0-7817-7489-6

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