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Updated: Mar 15, 2026

A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
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Management of Sickle Cell Disease in Children

Suzie A Noronha1, S Christy Sadreameli1, John J Strouse1

  • 1From the Department of Pediatrics, University of Rochester, Rochester, New York, the Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, Maryland, and the Division of Hematology, Duke University School of Medicine, Durham, North Carolina.

Southern Medical Journal
|September 7, 2016
PubMed

Insights

Sickle cell disease (SCD) is an inherited blood disorder causing anemia and organ damage in children. Early screening, preventive care, and evidence-based therapies improve outcomes and manage complications.

Area of Science:

  • Hematology
  • Pediatric Medicine
  • Genetics

Background:

  • Sickle cell disease (SCD) is a complex inherited hemoglobinopathy.
  • It leads to chronic hemolytic anemia, vaso-occlusion, and endothelial dysfunction.
  • Multisystem organ damage occurs from infancy through childhood.

Purpose of the Study:

  • To provide healthcare maintenance guidelines for pediatric patients with SCD.
  • To review common complications associated with SCD in children.
  • To offer recommendations for managing pediatric SCD patients.

Main Methods:

  • Review of current literature and clinical guidelines.
  • Synthesis of evidence-based therapies and preventive strategies.
  • Focus on multidisciplinary care approaches for chronic complications.

Main Results:

  • Screening and preventive measures (prophylaxis, vaccination) have improved pediatric outcomes.
  • Hydroxyurea and transfusions are key therapies for preventing complications.
  • Chronic complications often develop insidiously, requiring vigilant monitoring.

Conclusions:

  • Effective management of pediatric SCD necessitates familiarity with acute and chronic complications.
  • Multidisciplinary care is crucial for addressing the insidious nature of chronic complications.
  • Primary care physicians play a vital role in the comprehensive care of children with SCD.

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