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Standards for the management of sickle cell disease in children

M C Dick1

  • 1Department of Haematology, King's College Hospital, London SE5 9RS, UK. moira.dick@kch.nhs.uk

Archives of Disease in Childhood. Education and Practice Edition
|November 26, 2008
PubMed

Insights

Sickle cell disease (SCD) is England's most common genetic disorder. Newborn screening, implemented by 2006, aims to reduce early-life mortality from pneumococcal sepsis.

Area of Science:

  • Genetics
  • Pediatrics
  • Public Health

Background:

  • Sickle cell disease (SCD) is the most common genetic condition in England, with a birth prevalence of 1 in 2000.
  • SCD causes significant morbidity and mortality, especially in early childhood, without prophylactic measures.
  • Newborn screening for SCD was introduced nationwide in England by 2006.

Purpose of the Study:

  • To provide evidence-based treatment guidelines for children with sickle cell disease.
  • To establish a management plan for healthcare professionals in low-prevalence areas.
  • To support the national newborn screening program for sickle cell disease.

Main Methods:

  • Review of existing evidence for sickle cell disease treatment.
  • Consensus building on current best practices for SCD management.
  • Development of standards and guidelines for pediatric SCD care.

Main Results:

  • The article outlines the current evidence base for treating sickle cell disease.
  • It presents a consensus on good practice for managing children with SCD.
  • Guidelines are provided to support healthcare professionals, particularly in areas with limited resources.

Conclusions:

  • Newborn screening for sickle cell disease is established in England and expanding to Scotland and Wales.
  • Guidelines are crucial for managing SCD, especially in areas with lower prevalence and expertise.
  • Further research is needed to enhance and refine existing treatment and care guidelines for SCD.

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