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The young child with sickle cell disease

D J Preiss1

  • 1St. John's Mercy Medical Center, St. Louis, USA.

Advance for Nurse Practitioners
|August 26, 1998
PubMed
Summary

Sickle cell disease (SCD) is an inherited blood disorder affecting red blood cells, leading to severe complications and organ damage in children. Early recognition and management are crucial for improving outcomes in affected infants.

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Area of Science:

  • Hematology
  • Pediatric Medicine
  • Genetics

Background:

  • Sickle cell disease (SCD) is an inherited hemoglobinopathy affecting approximately 2,000 US infants annually.
  • Pathophysiology involves hemoglobin S, causing chronic hemolytic anemia and vaso-occlusion.
  • Vaso-occlusion leads to ischemic tissue damage and acute/chronic organ failure.

Purpose of the Study:

  • To summarize the pathophysiology and complications of sickle cell disease in infants.
  • To highlight the increased risk of severe infections, such as pneumococcal meningitis.

Main Methods:

  • This is a summary of existing knowledge on sickle cell disease.
  • Information is compiled from established medical literature and clinical observations.

Main Results:

  • Key complications include vaso-occlusive events, splenic dysfunction, and acute chest syndrome.
  • Infants with SCD face significantly higher risks for serious infections, including pneumococcal meningitis.

Conclusions:

  • Sickle cell disease presents with chronic anemia and vaso-occlusion, leading to diverse organ damage.
  • Prompt diagnosis and management are essential to mitigate severe complications and improve long-term health in children with SCD.

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