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Current strategies for the management of children with sickle cell disease

Mariane de Montalembert1

  • 1Service de Pediatrie Generale, Hopital Necker, 149 rue de Sevres, Paris, France. mariane.demontal@nck.aphp.fr

Expert Review of Hematology
|November 19, 2010
PubMed

Insights

Children with sickle cell disease benefit from early diagnosis and comprehensive care, including screenings and regular check-ups. Modern management significantly improves survival rates, allowing most patients to reach adulthood.

Area of Science:

  • Pediatrics
  • Hematology
  • Genetics

Background:

  • Sickle cell disease (SCD) is a global health concern affecting children worldwide.
  • Early identification and management are crucial for improving outcomes in pediatric SCD.
  • Developed countries utilize neonatal screening for timely SCD diagnosis and intervention.

Purpose of the Study:

  • To outline the comprehensive management strategies for children with sickle cell disease.
  • To highlight key interventions for preventing complications and enhancing survival in pediatric SCD.
  • To describe the evolution of SCD care and its impact on long-term patient prognosis.

Main Methods:

  • Neonatal screening for early detection of SCD.
  • Annual transcranial Doppler ultrasonography for stroke risk assessment (ages 2-16).
  • Regular check-ups to monitor for organ deficiencies and complications.

Main Results:

  • Early detection via screening enables prompt initiation of management, including antibioprophylaxis, immunizations, and parental education.
  • Annual screenings identify high-risk patients for stroke prevention and detect early organ deficiencies.
  • Frequent complications include pain, infections, and acute anemia, sometimes occurring concurrently.

Conclusions:

  • Comprehensive management programs have dramatically increased survival rates for children with SCD.
  • Most individuals with SCD now achieve adulthood due to advancements in care.
  • Ongoing monitoring and interventions are vital for managing SCD and its complications throughout childhood.

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